As long as fever persists in an evolving pneumonia, there is a risk of empyematization, especially if a small effusion has already occurred at the beginning of the picture. In principle, antibiotic treatment is correct and before considering changes in treatment, the possibility of empyematization should be evaluated.

As long as fever persists in an evolving pneumonia, there is a risk of empyematization, especially if a small effusion has already occurred at the beginning of the picture. In principle, antibiotic treatment is [HIDDEN] and before considering changes in treatment, the possibility of empyematization should be evaluated.

A 56-year-old woman with a history of well-controlled schizophrenia and no toxic habits. Admitted for middle lobe pneumonia with a small associated metaneumonic pleural effusion and on treatment with levofloxacin 500 mg/24h . She presented good clinical evolution except for persistent febrile fever and leukocytosis on the sixth day of treatment. Microbiological studies are not available. The most appropriate course of action is:

The evolution is normal, treatment should be maintained until completing 10 days.

It is considered a therapeutic failure and antibiotic treatment should be modified.

Perform thoracentesis to rule out empyema.

Add corticosteroids at a dose of 0.5 mg/Kg/day to antibiotic treatment.

Bronchoscopy with biopsy, aspiration and bronchoalveolar lavage.

[Failure of levofloxacin therapy in two cases of community-acquired pneumonia caused by fluoroquinolone-resistant Streptococcus pneumoniae and complicated with empyema].

Community acquired pneumonia (CAP) due to Streptococcus pneumoniae is a frequent cause of morbidity and mortality. We communicate two cases of CAP with complications. In both cases levofloxacin-resistant S. pneumoniae was isolated in pleural effusion. Patient 1: A 51-year-old man who had not received previous treatment with quinolones was admitted to the hospital for CAP and initially treated with levofloxacin (500 mg/24h iv). Four days later pleural effusion developed and fluid culture isolated levofloxacin-resistant S. pneumoniae (MIC > 32 .g/ml). The outcome was favorable following chest tube placement and treatment with beta-lactam antibiotics. Patient 2: A 73-year-old man with a history of chronic obstructive pulmonary disease was admitted due to CAP and was initially treated with levofloxacin (500 mg/24 h iv). He was transferred to our hospital after 10 days of treatment with this antibiotic, following the development of pleural effusion with isolation of levofloxacin-resistant S. pneumoniae (MIC = 12 .g/ml). The patient was treated with chest tube placement and beta-lactam antibiotics with a favorable outcome. Patients with CAP treated empirically must be closely followed, both clinically and radiologically, to facilitate early detection of complications due to bacterial resistance to the prescribed antibiotic. Patients with CAP who have received quinolones in the weeks before the development of pneumonia should not been treated empirically with these antibiotics because of the risk of resistance development.

Severe Pulmonary Infection in a 20-Month-Old Female.

Community-Acquired Pneumonia (CAP) is a common reason for hospitalization of a pediatric patient. We report a 20-month-old female admitted for suspected CAP. History included a week-long cough, fever, dyspnea, single occurrence of seizure-like activity, and a sick contact. Initial chest X-ray (CXR) showed left lower lobe pneumonia and parapneumonic effusion with a complex left pleural effusion. Ultrasound findings prompted the need for contrast-enhanced computed tomography (CT) of the chest. Contrast-enhanced CT of the chest confirmed a large pleural effusion with major atelectasis and mediastinal shift. The patient was treated with empiric antibiotics, video-assisted thoracoscopic surgical (VATS) decortication of empyema, and chest tube placement. Due to intraoperative complications, the VATS decortication was aborted and patient was transferred to the pediatric intensive care unit (PICU). A thoracentesis with culture failed to isolate a bacterial organism. Dexamethasone was started after repeat CXR showed persistent infiltrate. Subsequent contrast-enhanced CT of the chest showed a large collection of air and persistent consolidation. The patient received repeat VATS decortication and reinsertion of a chest tube. Repeat pleural fluid cultures failed to isolate a bacterial organism. Infectious disease (ID) consult recommended linezolid 140 mg Q8H for 4 weeks. Seven days after second VATS, a respiratory pathogen panel was positive for rhinovirus/enterovirus. With resolution of leukocytosis and clinical improvement, the patient was discharged with the chest tube in place and pediatric surgery outpatient follow-up. After three months, sequalae from both the infection and interventions presented .

Hospital-acquired pneumonia

Although gram-negative bacilli are a common cause they are rarely found in the respiratory tract of people without pneumonia, which has led to speculation of the mouth and throat as origin of the infection. Diagnosis In hospitalised patients who develop respiratory symptoms and fever, one should consider the diagnosis. The likelihood increases when upon investigation symptoms are found of respiratory insufficiency, purulent secretions, newly developed infiltrate on the chest X-Ray, and increasing leucocyte count. If pneumonia is suspected material from sputum or tracheal aspirates are sent to the microbiology department for cultures. In case of pleural effusion, thoracentesis is performed for examination of pleural fluid. In suspected ventilator-associated pneumonia it has been suggested that bronchoscopy or bronchoalveolar lavage is necessary because of the risks of incorrect clinical diagnoses.

Aspiration pneumonia

Treatment is typically with antibiotics such as clindamycin, meropenem, ampicillin/sulbactam, or moxifloxacin. For those with only chemical pneumonitis, antibiotics are not typically required. Among people hospitalized with pneumonia, about 10% are due to aspiration. It occurs more often in older people, especially those in nursing homes. Both sexes are equally affected. Signs and symptoms The person may have an insidious course with increased respiratory rate, foul-smelling sputum, hemoptysis, and fever. Complications may occur, such as exudative pleural effusion, empyema, and lung abscesses. If left untreated, aspiration pneumonia can progress to form a lung abscess. Another possible complication is an empyema, in which pus collects inside the lungs. If continual aspiration occurs, the chronic inflammation can cause compensatory thickening of the insides of the lungs, resulting in bronchiectasis.

Pleural empyema

and oral therapy. Switching to oral antibiotics can be considered upon clinical and objective improvement (adequate drainage and removal of chest tube, declining CRP, temperature normalization). Oral antibiotic treatment should then be continued for another 1–4 weeks, again based on clinical, biochemical and radiological response.

Borrelia turicatae

Treatment for relapsing fever can include various antibiotics. TBRF spirochaetes are susceptible to penicillin and other β-Lactam antibiotics, as well as tetracyclines, macrolides, and possibly fluoroquinolones. Although the CDC has not yet developed specific treatment guidelines for TBRF, experts generally recommend tetracycline 500 mg every 6 hours for 10 days as the preferred oral regimen for adults. If tetracyclines are contraindicated, erythromycin, 500 mg (or 12.5 mg/kg) every 6 hours for 10 days is an effective alternative. For patients with central nervous system involvement, parenteral therapy with ceftriaxone 2 g/day for 10–14 days is preferred. All patients treated with antibiotics should be observed during the first 4 hours of treatment for a Jarisch-Herxheimer reaction, which is a worsening of symptoms characterized by rigors, hypotension, and high fever. The reaction occurs in over 50% of cases and may be difficult to distinguish from a febrile crisis. Given appropriate

[Etiologic aspects and therapeutic problems of purulent pleurisy in Abidjan (Côte d'Ivoire)].

We present the results of a retrospective study of 127 cases of empyema admitted to the pneumophtisiology department of the Centre hospitalier universitaire de Treichville (Abidjan), between January 1985 and December 1989. We present the pathogens identified in the pleural fluid and the course of the disease during treatment by repeat thoraco-centesis and systemic antibiotics. During the study period, pleural empyema represented 2.7% of all admissions to the pneumophtisiology department, and 20.5% of those presenting with pleural effusions. Bacteriological examination was recovered in 88 of the 127 patients, and was positive in 57 cases (64.7% of those examined). Of those with positive bacteriology, 50 (56.8%) had non-tuberculous bacterial infections, and 7 (7.9%) had tuberculous infection. Among the non-tuberculous bacterial infections, Gram-negative bacilli were most common (72%), and Pseudomonas was the species most frequently identified (48%). The mean stay in hospital was 47 days (range 10-143) and in 82 patients (64.6%), the outcome was favourable. The presentation was complicated by encystment in 36 cases (28.4%) and 9 patients (7%) died in hospital.

[Treatment of community-acquired pneumonia by pristinamycin (Pyostacine 500). Results of a non comparative open study].

Activity of natural streptogramin (NSG) appears well adapted to pathogens responsible for CAP. The goal of this multicenter pilot study was to bring first data about efficacy of NSG in treatment of CAP. PATIENTS METHOD: Ten days of a NSG (1 gr b.i.d. or t.i.d.) regimen was administered to 46 hospitalized adult patients for CAP defined with fever > 38 degrees C, respiratory symptoms and X-ray opacity. Severely ill patients were excluded. A broncho-pulmonar sample (expectoration or trantracheal aspiration or protected distal sample) was performed in all patients. two patients were excluded because of pulmonary embolism (n = 1) or tuberculosis (n = 1) and 44 patients were analyzed. 50% of them had associated disease, 20% had failure of prior antibiotherapy. At inclusion, mean fever was 39.2 +/- 0.7 degrees C, respiratory rate was 22 +/- 5/mn, PaO2 was 74 +/- 10 mmHg, chest X-ray showed bilateral opacity in 16%, unilateral in 84% and pleural fluid level in 6 cases. Etiological diagnosis was determined in 70% of cases. Streptococcus pneumoniae (n = 14), Haemophilus influenzae (n = 5), Legionella pneumophila (n = 2), Mycoplasma pneumoniae (n = 2) and Chlamydia psittaci (n = 1) were the most frequent isolated pathogens. 40 patients (91%) were cured with NSG and delay to obtain apyrexia was 4.4 +/- 3.9 days. NSG was stopped in 4 patients: 1 clinical and bacteriological failure (Klebsiella pneumoniae), 2 clinical failures (1 pneumococcus with purulent pleurisy, 1 pneumococcus with worsening of respiratory status), 1 patient with resistant H. influenzae strain in spite of favourable clinical evolution. NSG was well tolerated in 86% of patients. these data invite to carry on evaluation of first line therapy of CAP with NSG.

[Pneumonia caused by Fusobacterium necrophorum: is Lemierre syndrome still current?].

Fusobacterium necrophorum is a non-spore-forming gram-negative anaerobic bacillus that may be the causative agent of localized or severe systemic infections. Systemic infections due to F.necrophorum are known as Lemierre's syndrome, postanginal sepsis or necrobacillosis. The most common clinical course of severe infections in humans is a progressive illness from tonsillitis to septicemia in previously healthy young adults. A septic thrombophlebitis arising from the tonsillar veins and extending into the internal jugular vein leads to septicemia and septic emboli contributing to the development of necrotic abscesses especially in lungs and other tissues such as liver, bone and joints. In this case report, a previously healthy man with pneumonia and empyema due to F.necrophorum has been presented. A 22 year-old man suffering from sore throat for seven days was admitted to emergency department with ongoing fever and dysphagia for three days. On admission he was already taking amoxicillin-clavulanic acid and his complaints were relieved with continuation of therapy to a total of 10 days. However, five days after the cessation of treatment he developed productive cough, fever and generalized myalgia. On physical examination, there were crackles on right lower lung, and chest X-ray revealed pulmonary consolidation on the right middle lobe. Levofloxacin therapy was started based on the diagnosis of pneumonia. While polymorphonuclear leucocytes and intracellular gram-negative bacilli were seen in Gram stained sputum smear, sputum culture was reported as normal flora. Although the patient's status had started to improve with treatment, his condition deteriorated with development of fever and dyspnea. Chest X-ray revealed consolidation, pulmonary infiltrates, pleural effusion and air-fluid level on the right. Meropenem, clarithromycin and linezolid were initiated and a chest tube was inserted with the preliminary diagnosis of necrotizing pneumonia, empyema and type-1 respiratory failure. While there was no growth on bronchoalveolar lavage fluid culture, thoracentesis material inoculated into thioglycolate broth revealed turbidity. Further inoculation onto Schaedler agar which was incubated under anaerobic conditions, yielded growth of catalase negative, indol positive, gram-negative anaerobic bacilli identified as F.necrophorum by BBL Crystal system (Becton Dickinson, USA). The detailed history of the patient revealed that fish bone had stuck in his throat a week ago. Clarithromycin and linezolid were discontinued and he was recovered within six weeks of meropenem treatment. F.necrophorum infection should be considered in the differential diagnosis of persistent head and neck infections with rapidly progressive metastatic necrotic lesions especially in healthy young adults and clindamycin or metranidazol should be added to the treatment protocols.

[Retrospective study of 77 cases of purulent pleurisy].

77 cases of non-tuberculous empyema were studied retrospectively. The data of past history, laboratory, radiological, bacteriological and outcome were gathered. The mean delay between initial symptoms and clinical diagnosis was 2.6 weeks in this study. It was longer for those anaerobic features (p less than 0.02) whose details were unravelled. "Blind" antibiotic therapy before the first pleural aspirate is still common (46.7% of cases) and did not alter the frequency with which the causal bacteria was found, nor the distribution of the bacterial population. Pleural aspirate enabled the germ or germs responsible to be identified in 63% of cases. Streptococcus pneumoniae (n = 15) and anaerobic bacterias (n = 26) infections were predominant. Early pleural drainage in a trained unit care is the essential element in the treatment in association with appropriate antibiotics and prolonged physiotherapy. This triple therapy approach leads to a medical cure in practically all the patients and avoids a recourse to surgery.

Selective norepinephrine reuptake inhibitor

In the use of atomoxetine in children (6 years or older up to 70 kg) with attention-deficit hyperactivity disorder, acute treatment should be started with approximately 0.5 mg/kg orally daily. The dose should be increased after a minimum of 3 days up to approximately 1.2 mg/kg daily (target dose) as a single or two divided doses (in the morning and late afternoon). For children older than 6 years old, over 70 kg, acute treatment should be started with 40 mg/day orally and increased up to 80 mg/day after a minimum of 3 days. The dose can be taken as a single dose in the morning or in two divided doses (in the morning and late afternoon). After 2–4 weeks the dose can be increased to 100 mg/ daily.

[Clinical course and treatment of pleural empyema in children].

Purulent pleurisy has become rare. It is often masked by previous antibiotic treatment so that functional prognosis may be poor. Twenty children with purulent pleurisy of the large cavity admitted from 1987 to 1993 were included in the study: there were nine infants (age 5 to 18 months) with pleuro-pulmonary staphylococcal infection (group I) and 11 children (4-13 years) (group II). Clinical, biological, bacteriological and radiologic findings were analysed retrospectively as was the outcome. Patients of group I were admitted in poor general condition. X-ray showed moderate effusion and characteristic signs of staphylococcal infection. The bacteria identified in seven patients (77%) was S aureus. Recovery was rapid with antibiotics and simple local treatment. X-rays were normal two months after hospital discharge in seven patients (77%). One infant presented cicatricial bullous emphysema which required segmental resection. Patients of group II were admitted for moderate respiratory signs after a relatively long delay (14 days) since the onset of symptoms. X-rays showed considerable effusion in all and mediastinal shift in five patients (45%). Streptococcus pneumoniae was identified in one patient only. Local treatment of empyema was difficult; the effusion, already fibrinous, required repeated use of chest tubes in eight cases and surgical decortication in three. X-rays, performed 2 months after hospital discharge, were normal in only three patients. Long-term course was nevertheless favorable since chest X-rays at 5 months were normal in all children of both groups. Early recognition of purulent pleurisy is important in children aged over 3 years to ensure effective drainage before the effusion becomes fibrinous. All patients in whom the first tube was inserted after more than 10 days had a difficult follow-up requiring repeated chest drainages or surgery. Ultrasonography was a useful aid for diagnosis and local treatment. Computed tomography was useful for adapting treatment after several days of course.

Vesicoureteral reflux

Medical treatment Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include: Amoxicillin or ampicillin – infants younger than 6 weeks Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months After 2 months the following antibiotics are suitable: Nitrofurantoin {5–7 mg/kg/24hrs} Nalidixic acid Bactrim Trimethoprim Cephalosporins Urine cultures are performed 3 monthly to exclude breakthrough infection. Annual radiological investigations are likewise indicated. Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics.

[Current persistance of pleural empyema. Study of 31 observations (author's transl)].

Antibiotic treatment of pleuro-pulmonary infectious diseases with ordinary germs brought a notable decrease in the frequency of pleural empyema without preventing them completely. The authors report 31 cases observed over the last 10 years. The initial signs corresponded to the classical picture but were rapidly masked by antibiotic treatment; it resulted in delay in hospital admission of over one month for more than half of the cases, for diagnosis was overlooked for a long time. In 19 patients, the pleural liquid was aseptic probably because of the antibiotics and of the lack of identification of anaerobic germs. The authors insist on the practical modalities of a medical treatment by puncture-lavage which, when associated with general antibiotic treatment, cured patients in 22 cases. On the other hand, the existence of bronchopleural fistula entailed surgery.

Cladribine

Two main approaches to multiple sclerosis treatment maintenance therapy are used – immunomodulation and immunosuppression and alternatively, immune reconstitution therapy. Classified as the latter, cladribine tablets are administered intermittently as a short treatment course without continuous immunosuppression. In contrast to maintenance therapies, clinical efficacy extends beyond the dosing period. Cladribine tablets are administered as 2 courses separated by 1 year (a maximum of 20 days of treatment). The recommended cumulative dose is 3.5 mg/kg weight over 2 years, administered as 1 treatment course of 1.75 mg/kg per year. Each treatment course consists of 2 treatment weeks, one at the beginning of the first month and one at the beginning of the second month of the respective treatment year. Each treatment week consists of 4 or 5 days on which a patient receives 10 mg or 20 mg (1 or 2 tablets) as a single daily dose based on body weight.

[Pleuropulmonary staphylococcal infections in the infant].

In a study of 44 infants and young children with staphylococcal pneumonia and pleurisy, cases were found to be divisible into three clinical groups: those with predominantly digestive tract symptomatology (seven); those in acute respiratory distress (28); and those with signs of central nervous system disorder (nine). Characteristic radiographic findings often permitted an early etiologic diagnosis, confirmed later by bacteriologic culture or by the course of the disease. The length of hospitalization was found to be directly proportional to the length of time between the onset of symptoms and the start of adequate therapy. Nineteen patients required thoracotomy, two were drained by a Küss trocar, 13 required repeated pleural aspiration, five underwent spontaneous resorption of the empyema and five had no pleural effusion. In several instances antibiotics were used in very high dosage (three to four times the suggested maximal dose). The main agents used were erythromycin, chloramphenicol and ristocetin; the dosage of the last named never exceeded the maximal recommended dose. Twenty-eight patients were less than one year old; of these seven died. Sixteen were over one year of age, the oldest being six years; all of these were cured.

A severe case of Streptococcal pyogenes empyema following influenza A infection.

Any immunological mechanisms induced by influenza virus could cause severe secondary bacterial superinfection such as those by Streptococcus pyogenes [group A streptococcus (GAS)], Streptococcus pneumoniae or Staphylococcus aureus. Over recent years, the frequency of pleural empyema has increased in children with influenza infection. We present a severe case of acute empyema caused by S.pyogenes after influenza A infection. A previously healthy 39-year old woman was diagnosed as influenza A and received oral Oseltamivir 75 mg twice daily for 5 days. She had no vaccination of influenza A. Although her influenza A infection improved, she complained of fever and cough to our institute. Chest radiography showed encapsulated pleural effusion of the left lung and pleural effusion which was consistent with acute empyema. Then, she was diagnosed as having acute empyema and was admitted to our institute. Streptococcus pyogenes was identified by pleural fluid culture on day 4. thus, MNZ was changed to clindamycin (CLDM) 600 mg three times a day. While thoracic drainage with intrapleural urokinase and combination antibiotic therapy of ceftriaxone and CLDM were performed, her general condition and chest radiographic findings were not improved. She received video-assisted thoracic debridement on day 10. After the operation, the antibiotic therapy was changed to ABPC 6 g daily iv. Due to good clinical course, the antibiotic therapy was switched to oral amoxicillin 500 mg three times daily on day 28. Then, she was discharged. Influenza A virus infection could lead to severe GAS infection, while the latter can occur in otherwise healthy individual as well. Physician must consider the possibility of severe GAS infection after influenza A infection.

Thoracic empyema - a review based on three cases reports.

Complicated parapneumonic effusion is one in which an invasive procedure is necessary for its resolution and empyema means pus in the pleural space. An early diagnosis and therapy of these conditions results in less morbidity and mortality. CT of the chest is important to study complex pleural effusions. Loculated effusions, those occupying more than 50% of the thorax, or which show positive Gram stain or bacterial culture, or a purulent effusion with a pH below 7.20, with a glucose level below 60 mg/dl or a LDH level more than three times the upper limit of normal for serum, are indications for an invasive procedure. These characteristics result from the evolution of a not well treated parapneumonic effusion, through the three stages: (1) exsudative; (2) fibrinopurulent; (3) fibrotic. Depending on the stage therapeutic methods vary from therapeutic thoracentesis, insertion of a chest tube with or without instillation of fibrinolytics, video-assisted thoracoscopic surgery, and lung decortication. A review of all these aspects are done based on a series of three cases reports with very different clinical presentation: one patient with empyema by Streptococcus pyogenes and that died rapidly due to massive hemoptysis; a patient with empyema due to acute pneumonia developing during an airflight; a patient with empyema and bacteraemia by Streptococcus pneumonia leading to the diagnosis of an unknown HIV infection.

Chancroid

Antibiotics Macrolides are often used to treat chancroid. The CDC recommendation is either a single oral dose (1 gram) of azithromycin, a single IM dose (250 mg) of ceftriaxone, oral (500 mg) of erythromycin three times a day for seven days, or oral (500 mg) of ciprofloxacin twice a day for three days. Due to a paucity of reliable empirical evidence it is not clear whether macrolides are actually more effective and/or better tolerated than other antibiotics when treating chancroid. Data is limited, but there have been reports of ciprofloxacin and erythromycin resistance. Aminoglycosides such as gentamicin, streptomycin, and kanamycin has been used to successfully treat chancroid; however aminoglycoside-resistant strain of H. ducreyi have been observed in both laboratory and clinical settings.[7] Treatment with aminoglycosides should be considered as only a supplement to a primary treatment.

[Pneumonia caused by Haemophilus influenzae. Study in a series of 58 patients].

Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5% of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69%) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90%). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48%). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality.

Aspiration pneumonia

Treatment The main treatment of aspiration pneumonia revolves around the use of antibiotics to remove the bacteria causing the infection. Broad antibiotic coverage is required to account for the diverse types of bacteria possibly causing the infection. Currently recommended antibiotics include clindamycin, meropenem, ertapenem, ampicillin/sulbactam, and moxifloxacin. Treatment with piperacillin/tazobactam, cefepime, levofloxacin, imipenem, or meropenem is recommended in cases of potential antibiotic resistance. The typical duration of antibiotic therapy is about 5 to 7 days. If there is a large accumulation of fluid within the lungs, drainage of the fluid may also aid in the healing process.

Pleural effusion in an immunocompetent host with cryptococcal pneumonia: A case report.

Pulmonary cryptococcosis is an opportunistic infection that mainly occurs among immunocompromised patients although it can sometimes occur in immunocompetent individuals. However, the imaging findings of pulmonary cryptococcosis in immunocompetent hosts differ from those in immunosuppressed patients. In addition, the most common imaging findings of isolated pulmonary cryptococcosis are single or multiple nodules. Cavities and the halo sign are, however, prevalent in immunosuppressed patients. In immunocompetent patients, lung consolidation, pleural effusion or cavities are scarce. A 29-year-old Asian male was admitted to our hospital with complaints of cough and fever that had persisted for a month. As a chest computed tomography scan showed consolidation in his left lower lobe, he was initially diagnosed with pneumonia and received antibiotic treatment. A second review of the chest computed tomography image revealed multiple cavities and pleural effusion. Flexible fiberoptic bronchoscopy was subsequently performed, bronchoalveolar lavage fluid and serum cryptococcal antigen tests were positive. Cryptococcus capsules were observed in bronchoalveolar lavage fluid ink stain. Histopathological examination of a percutaneous lung biopsy from the left lower lobe further revealed granulomatous inflammation, and periodic acid-Schiff staining showed red-colored yeast walls, signifying pulmonary cryptococcosis. The patient was then treated with a daily dose of fluconazole (0.4 g), but the cough and fever still persisted. We therefore changed treatment to voriconazole (0.2 g, twice a day), and the patient's clinical outcome was satisfactory. Although rare, clinicians should not disregard the possibility of cavities and pleural effusion occurring in immunocompetent hosts without underlying diseases.

Are there any differences in the community acquired pneumonias admitted to hospital over the past decade?

The past few years have seen a decline in community acquired pneumonia (CAP) in children in the western world, although this has gone hand-in-hand with more serious cases needing hospital admission. Our study characterises cases of CAP admitted to hospital and compares this data with a 2001 study. We collected data on 63 admissions over a six-month period. The majority were aged 0-2 years old. Chest X-ray showed consolidation/atelectasy in 58 (92.1%) and pleural effusion (PE) in 17 (27.0%), of which 11 were empyema (17.4% of all admissions). The bacterial agent was isolated in five cases: Streptococcus pyogenes (two, pleural fluid), Streptococcus pneumoniae (two, blood culture) and Haemophilus influenzae (one, blood culture). Sixty-one children (96.8%) were prescribed antibiotherapy. The median length of hospital stay was five days. Patients with PE were older, had a longer course of fever, higher inflammatory parameters, longer hospital stay and longer course of iv antibiotics. Compared to the prior study we found greater severity of CAP, with higher prevalence of PE and empyema. Nevertheless there was a shorter course of fever during hospital stay and shorter hospital stay. We also noticed less antibiotic prescription prior to admission and greater prescription of ampicillin during hospital stay. In the literature, the higher severity of CAP has been partially attributed to the emergence of more aggressive serotypes of Stretococcus pneumoniae not included in the heptavalent vaccine. There is therefore a greater interest in new vaccines containing them. Complicated CAP should be referred to centres specialising in its diagnosis and management.

Invasive diagnostic techniques for pneumonia: protected specimen brush, bronchoalveolar lavage, and lung biopsy methods.

We suggest the following strategy for managing patients with pneumonia. For nonventilated patients with either CAP or HAP, empiric antibiotic treatment should be started according to approved guidelines, and if the clinical evolution of the patient is not adequate, fiberoptic bronchoscopy including PSB and BAL could be considered, with modification of the antibiotic treatment accordingly. In ventilated patients with either CAP or HAP, respiratory secretion sampling using noninvasive techniques should be conducted upon clinical suspicion of VAP and before starting a new antibiotic treatment. Antibiotic therapy according to approved guidelines should be started as soon as possible and maintained during the first 48 hours if the patient's evolution is satisfactory and condition has stabilized. Then, initial antibiotic treatment should be adjusted according to cultures. If there is a clear diagnostic alternative to VAP and cultures are negative, this is the only case in which antibiotic treatment could be withdrawn. If the patient's clinical evolution is inadequate (persistence of fever, leukocytosis, increasing infiltrates, and respiratory failure), fiberoptic bronchoscopy with PSB and BAL and modification of the initial antibiotic regimen should be sought. Open lung biopsy may be indicated in patients with diffuse pulmonary infiltrates in whom a diagnosis has not been achieved by other methods, including bronchoscopy. Transbronchial lung biopsy should not be viewed as a diagnostic technique for pneumonia except in immunosuppressed patients with diffuse alveolar infiltrates.

[Pneumonia due to Haemophilus influenzae.Study in a series of 58 patients]

Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5&#37; of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69&#37;) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90&#37;). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48&#37;). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality.

Pharmacology of cyproterone acetate

The effective dosage of CPA for inhibition of ovulation in women, this being an antigonadotropic effect, is 1 mg/day. CPA alone has been found to suppress ovulation in 3 of 5 women at a dose of 0.5 mg/day and in 5 of 5 women at a dose of 1 mg/day in studies. Ovulation inhibition with 1–2 mg/day CPA in combination with 1–2 mg/day estradiol valerate as a birth control pill (brand name Femilar) occurred in 94.4% of 108 women during the third treatment cycle in one study and in almost 100% of 26 women over 12 treatment cycles in another study (except for one woman who ovulated during her first treatment cycle).

[Comparative course of pneumonia with suppuration].

Certain clinical, biochemical, bacteriologic and radiographic parameters are compared, as well as the evolution and treatment of two groups of purulent pneumonias followed during the two trienniums 1968-1970 and 1980-1982. In the more recent triennium stands on the decrease in the incidence of this type of pneumoniae as well as a shorter hospital admittance and a lesser need for surgical therapy.

Cavernous sinus thrombosis

Differential diagnosis Orbital cellulitis Internal carotid artery aneurysm Stroke Migraine headache Allergic blepharitis Thyroid exophthalmos Brain tumor Meningitis Mucormycosis Trauma Treatment Recognizing the primary source of infection (i.e., facial cellulitis, middle ear, and sinus infections) and treating the primary source expeditiously is the best way to prevent cavernous sinus thrombosis. Antibiotics Broad-spectrum intravenous antibiotics are used until a definite pathogen is found. Nafcillin 1.5 g IV q4h Cefotaxime 1.5 to 2 g IV q4h Metronidazole 15 mg/kg load followed by 7.5 mg/kg IV q6h Vancomycin may be substituted for nafcillin if significant concern exists for infection by methicillin-resistant Staphylococcus aureus or resistant Streptococcus pneumoniae. Appropriate therapy should take into account the primary source of infection as well as possible associated complications such as brain abscess, meningitis, or subdural empyema.

[The influence of the initial antibacterial treatment effectiveness, clinical and etiological factors on non-resolvent severe pneumonia and outcome].

To evaluate the influence of the initial pneumonia treatment effectiveness, clinical and etiological factors, local innate immune response intensity on pneumonia non-resolution and mortality. One hundred one bronchoscopy and bronchoalveolar lavage performed to 68 severe community (CAP) or hospital-acquired pneumonia patients survived during initial 5-7-day treatment period. Initial treatment effectiveness, age, pneumonia type, presence of mechanical ventilation, comorbidities, coma, antibacterial treatment, pathogen isolation and treatment changes were assessed. Logistic regression analysis was performed to detect factors associated with non-resolving pneumonia and mortality. Etiotropical treatment was administered to 30.9% of patients. Initial antibacterial treatment was corrected in 64.7% of all cases. After 21-30 days from the pneumonia onset survived 66.2% of patients (n=43). Pneumonia course could be evaluated in 52 cases. Delayed resolution of the pneumonia was stated in 32.7% of cases (n=17). Pneumonia was cured or condition improved in 64.3% of the CAP patients (n=9) and 68.4% of the hospital-acquired pneumonia patients (n=26). Only ineffective initial pneumonia treatment significantly increased probability of non-resolving pneumonia on multivariate analysis (OR 16.92, 95% CI 2.02-141.72, p<0.05). Influence of etiotropic treatment was not significant. Isolation of two or more pathogens from bronchoalveolar lavage fluid after the initial treatment was significantly associated with mortality on multivariate analysis (OR 6.25, CI 1.06-36.74, p<0.05). The other analyzed variables had no significant influence on pneumonia resolution and outcome. In conclusion, the initial pneumonia treatment failure increases probability of the non-resolving pneumonia. Etiotropical treatment has no impact on pneumonia outcome when adequate empirical antibacterial treatment is administered, however, it allows to reduce unnecessary use of the broad-spectrum antibacterials. Mortality is associated with the presence of the polymicrobial infection after the initial pneumonia treatment.

Aggressive periodontitis

Scale and Polish Root Surface Debridement (RSD) Antibiotics: There is evidence that the additional use of systemic antibiotics in conjunction with non-surgical periodontal treatment results in a more favourable clinical response, as compared to just periodontal treatment alone, as it helps to suppress pathogenic bacteria and create a health-associated biofilm. There have been many antibiotic regimes proposed for the treatment of AgP. However, the combination of choice according to current research is a combination of amoxicillin (500 mg, thrice/day) and metronidazole (200 mg, thrice/day), for 7 days, starting on the day of the final debridement. Doxycycline (100 mg, once/day, with a starting first dose of 200 mg) is the choice of antibiotics for patients allergic to penicillin. Light Amplification by Stimulated Emission of Radiation (LASER) Therapy

[Jacopo's chest (bad history of a pleural empyema). A case report].

We present a case of pleural empyema, occurred in a healty 7 years boy. He was admitted to our hospital because of a lobare pneumonitis. The patient was administered with a 2 degrees generation Cefalosporine given intramuscularly and with Corticosteroid (1 mg/kg/die). After an initial improvement of his clinical conditions, he got worse so that he underwent a TC scan which showed the presence of a left pleural empyema requiring the insertion of an intercostal tube drainage followed by an intervention of decortication. The boy had some evidence of a staphylococcal etiology such as the evolution in empyema itself, the augmentation of antistafilolisinic title found during the illness, and the typical finding of blebs on chest radiograph. As cultures from both blood and drainage liquid samples remained sterile, we were unable to demonstrate a clear bacterial etiology of the empyema. It remains doubtful if corticosteroid administration could contribute to the severity of the pneumonia evolution.

Surgery_Schwartz

can be avoided in patients with small effusions associated with resolving pneumonia. These patients typically present with cough, fever, leukocytosis, and uni-lateral infiltrate, and the effusion is usually a result of a reactive, parapneumonic process. If the effusion is small and the patient responds to antibiotics, a diagnostic thoracentesis may be unneces-sary. If the effusion is large and compromising respiratory efforts, or if the patient has a persistent white blood cell count despite improving signs of pneumonia, an empyema of the pleural space must be considered. In these patients, early and aggressive drainage with chest tubes is required, possibly with surgical intervention.Once the decision is made to access a pleural effusion, the next step is to determine if a sample of the fluid or complete drainage of the pleural space is desired. This step is influenced by the clinical history, the type and amount of fluid present, the Brunicardi_Ch19_p0661-p0750.indd 73601/03/19


4 is the same as absences,

A patient of adolescent age with myoclonus ("violent jumps") at breakfast time will almost always lead us to juvenile myoclonic epilepsy (answer 5 correct), a very well characterized entity. Among other features is the previous history of seizures or absences, the worsening with nighttime departures, and the EEG with generalized polyp spike discharges at a higher frequency than that of absences. Of the remaining answers, Lennox Gastaut develops at an early age with other symptomatology, 4 is the same as absences, and 3 usually presents with complex partial seizures and either way, the EEG is clearly distinct from a temporal focus.

A patient of adolescent age with myoclonus ("violent jumps") at breakfast time will almost always lead us to juvenile myoclonic epilepsy ([HIDDEN]), a very well characterized entity. Among other features is the previous history of seizures or absences, the worsening with nighttime departures, and the EEG with generalized polyp spike discharges at a higher frequency than that of absences. Of the remaining answers, Lennox Gastaut develops at an early age with other symptomatology, 4 is the same as absences, and 3 usually presents with complex partial seizures and either way, the EEG is clearly distinct from a temporal focus.

An 18-year-old female patient with a history of absences between 6 and 9 years of age, generalized tonic-clonic seizures of recent onset and violent jumping of the upper limbs at breakfast. The clinical manifestations worsen with nighttime weekend outings. An EEG shows acute polyp spike discharges at 6 cycles/second. The most likely diagnosis is:

Symptomatic epilepsy due to mesial temporal sclerosis.

[Clinical and electroencephalographic characteristics of epilepsy with myoclonic absences].

Epilepsy with myoclonic absences (EMA) is a type of childhood epilepsy characterized by a specific seizure type, i.e. myoclonic absences (MA). This study aimed to investigate the clinical and electrophysiological characteristics of EMA. Video-EEG monitoring was carried out in 6 patients with EMA, and 2 of them were examined with simultaneous deltoid muscle surface electromyogram (EMG). The clinical and EEG characteristics, treatment and prognoses of EMA were analyzed. Of the 6 patients, 3 were female, and 3 were male. The age of onset was from 2 years and 3 months to 11 years (average 5 years and 2 months). MA was the sole seizure type in 5 patients. One patient presented generalized tonic clonic seizures (GTCS) at the onset and then switched to MA. The manifestations of MA included an impairment of consciousness of variable intensity, rhythmic myoclonic jerks with evident tonic contraction mainly involving the upper extremities, a deviation of head and body to one side or asymmetrical jerks observed in some cases, a duration ranging from 2 to 30 s, an abrupt onset and termination, a high frequency of attacks, at least several times to over 30 times per day, and easily provoked by hyperventilation. The ictal EEG consisted of rhythmic 3 Hz spike and wave discharges that were bilateral, synchronous and symmetrical in all patients. The deltoid muscle EMG recording in 2 patients showed rhythmic myoclonus at the same frequency as the spike and waves. The interictal EEG showed generalized spike and wave discharges in all patients, and focal discharges in some patients. Valproate was the drug of choice, which was often combined with other antiepileptic drugs. The ages at follow up ranged from 6 years and 4 months to 19 years. Seizures were controlled from 8 months to 3 years in 4 cases. The treatment at the onset was late in one case and was irregular in another who had GTCS during the course of the disease. These two cases were followed up for 2 years and 6 months and 5 years, respectively. Seizures could not be controlled in the 2 patients with intellectual impairment. EMA was a rare type of childhood epilepsy characterized by MA. Clinical observation and ictal video-EEG and EMG were essential to diagnose EMA. Valproate alone or combined with other antiepileptic drugs given early could have a favorable effect to EMA. Delayed therapy and the presence of GTCS might suggest poor prognosis.

[Juvenile myoclonic epilepsy].

We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, "mini mental test," EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures.

[Differential diagnosis in idiopathic generalized epilepsies with tonic-clonic seizures: assessment of the use of ambulatory EEG and video/EEG monitoring].

The value of long-term cassette EEG (24-EEG) and Video/EEG for differential diagnosis and classification of idiopathic epilepsies with generalised tonic-clonic seizures (GTCS) was evaluated in twenty-eight patients. The analysis of clinical and EEG features allowed proper classification of epileptic syndrome in twenty-two (79%) patients. In twelve cases absences or myoclonic seizures appeared beside GTCS after 1-9 years from epilepsy onset (mean 3.3 yrs). EEG and clinical data allowed to classify epilepsy in nine (75%) of those patients: in six patients as juvenile absence epilepsy and in three as juvenile myoclonic epilepsy. Sixteen patients suffered from GTCS only (mean duration of epilepsy 10.6 years); in thirteen of them (81%) the diagnosis of epilepsy with GTCS on awakening (AGM) could be established. 24-EEG and Video/EEG helped to demonstrate (a) interictal generalized spike/polyspike-wave discharges (SW/PSW) 3-6 Hz not present in routine EEG in 25% of patients, (b) typical circadian distribution of discharges in AGM patients and (c) absences and myoclonic seizures in 32% of patients. Slow spike-wave variants and focal changes in EEG which could suggest secondarily generalized GTCS were the main diagnostic problem.

Clinical observations of juvenile myoclonic epilepsy in 131 patients: a study in South India.

We studied clinical features of 131 patients with juvenile myoclonic epilepsy (JME). The prevalence was 7.7% among the epileptic patients registered. The mean age at onset was 13.37+/-4.93 years and the diagnosis was established at a mean age of 19.53+/-7.85 years. Absence seizures were reported by 27 (20.6%) patients, myoclonic jerks by 131 (100%) and generalized tonic-clonic seizures (GTCS) by 111 (84.7%). The triad of absence seizures, myoclonic jerks and GTCS was noted in 23 (17.5%) patients, 88 (67.2%) had myoclonic jerks and GTCS, 4 (3%) had absence seizures and myoclonic jerks and 16 (12.2%) had only myoclonic jerks. Early onset absences were seen in 21 (16%) patients and the onset was late in 6 (4.6%). Absences antedated other types of seizures in all the patients. Myoclonic jerks were predominantly unilateral or had unilateral onset in 22 (16.8%). In 17 (13%) patients GTCS antedated myoclonic jerks. Myoclonic jerks had characteristic circadian distribution in 112 (85.5%) patients. On awakening GTCS occurred in 87 (78.4%) patients and in 4 (3.6%) patients they were purely nocturnal. Sleep deprivation was the most important precipitating factor (54.2%). Initial electroencephalogram (EEG) showed classical generalized spike or multiple-spike slow-wave paroxysms in 81% of records. Focal EEG abnormalities were noted in 20.6% of records. The most common focal abnormality was voltage asymmetry. A family history of epilepsy was noted in 31 (23.6%) probands. Diagnosis of JME was made in all the cases in the clinic. The factors responsible for delay in diagnosis of the 36 patients seen by neurologists included failure to ask or interpret the history which was otherwise suggestive of myoclonic jerks in all 36 (100%) cases, the type of seizure for which the patients sought medical attention, and misinterpretation of EEGs in 28 patients. Diagnosis of partial epilepsy was made in seven patients. The factors responsible for such diagnoses were, unilateral jerks in one patient, unilateral jerks and absence seizures in three patients and focal EEG abnormalities in three patients.

Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy.

Benign myoclonic epilepsy in infancy (BME) is characterized by the occurrence of brief myoclonic attacks in normal infants aged 4 months to 3 years. There is no prior personal history, although in some patients 1 or 2 isolated febrile convulsions may occur prior to the onset of myoclonias. A family history of epilepsy or febrile convulsions is present in 30% of cases. Myoclonic attacks are short and mild, they involve mainly the head and upper limbs. The psychomotor development continues normally after the onset of seizures. The EEG shows a normal background activity and generalized spike-wave or polyspike-wave discharges associated with the myoclonias. These abnormalities are activated by drowsiness and during the first stages of sleep. A clinical and EEG photosensitivity is present in one-third of the patients. Myoclonias can be easily controlled by valproate monotherapy. Rare grand mal seizures can occur during adolescence, after withdrawal of drug treatment. The psychomotor evolution is good if treatment is started early. When myoclonias begin during the first year of life, the diagnoses of cryptogenic infantile spasms and of non-epileptic benign infantile myoclonus must be eliminated. In cases with a later onset, the following diagnoses can usually be easily discarded: cryptogenic Lennox-Gastaut syndrome, myoclonic-astatic epilepsy and unclassified epilepsies with the association of myoclonias and other types of seizures.

[Characteristics of clinical manifestations and EEG of Lennox-Gastaut syndrome].

Lennox-Gastaut syndrome (LGS) is one of the most severe and refractory form of childhood epilepsy. The purpose of this study was to investigate the clinical and EEG characteristics of patients with LGS. Sixty-two patients with LGS, including 37 males and 25 females, were followed-up regularly per three months or per six months, therapy was adjusted according to the changes in seizures and EEG, and the clinical data were analyzed in detail. The onset occurred between the age of 8 months and 12 years, with the peak at 1-4 years of age, accounting for 61%; a late onset which occurred after 8 years of age, was unusual. Furthermore, one patient who developed LGS at the age of 13 years and remained to have all the features of seizures and EEG at 35 years of age was identified as adult's LGS. Forty-three patients were classified as symptomatic, perinatal events were the predominant factors in this group. The others were cryptogenic. It was noted that 11 cases had a history of West syndrome. A transformation process from West syndrome to LGS was observed in another 7 cases. Every patient had two or more seizure types during the course of the disease; tonic seizure, atypical absence seizure, head drop or sudden falls were the characteristic types. The degree of mental deficit was variable from slight to profound deterioration, but mental and behavioral disturbances existed in every case as a rule. In all cases electroencephalogram (EEG) background was abnormal and consisted of diffuse slow spike-and-waves (1-1.5CPS), predominant in frontal and temporal regions. Twenty-four cases had the polyspike-wave. Bursts of fast rhythms (10-14CPS) were observed in 29 patients during sleep. The choice of antiepileptic drugs (AEDs) was based on the seizure types; routinely, 2 or more kinds of AEDs were used in combination, the classic drugs, valproate and clonazepam were firstly recommended; the other drugs, such as lamotrigine and topiramate that are used as add-on therapy were further consideration. Although the total effect was not satisfactory, the severity and frequency of seizures in almost all cases had lessened to some extent. LGS shows diverse manifestations; comprehensive diagnosis is crucial, active and efficacious treatment can improve the mental and behavioral development and prognosis as a whole.

Lennox-Gastaut's syndrome--prognosis of the secondary generalized epilepsies.

On 62 cases with Lennox-Gastaut's syndrome aged four to 31, the clinical-electroencephalographic findings were summarized as follows; (1) Age of onset was over 10 years in 10 cases (16.1%). (2) Mental deficiencies were more severe in those with onset earlier than age three. (3) Behavioral problems were observed in 34 cases (54.8%); 21 with hyperactive and 13 with hypoactive ones -- 18 hyperactive cases (85.7%) with the onset taking place before age six, and 12 hypoactive cases (92.3%), all whose age is now over 10. (4) The number of clinical seizures showed a tendency in which monoictal manifestation decreased from 25 to three whereas polyictal one increased from 13 to 59 cases during the course of a decade. (5) Interictal EEG findings were pseudorhythm of slow spike-wave with or without focal spikes, and so-called runs of rapid spikes during sleep recording. Focal spikes were observed in 25 cases (40.3%); mainly in the frontal area among those under 10 years old, and in the anterior temporal area among those over 20. The rapid spikes were demonstrated in 19 cases (30.6%) in the over-15 age group and appeared to be correlated with epileptic drop seizures and atypical complex absences.

West syndrome followed by juvenile myoclonic epilepsy: a coincidental occurrence?

West syndrome is an age-dependent epilepsy with onset peak in the first year of life whose aetiology may be symptomatic or cryptogenic. Long-term cognitive and neurological prognosis is usually poor and seizure outcome is also variable. Over the past two decades a few patients with favourable cognitive outcome and with total recovery from seizures were identified among the cryptogenic group suggesting an idiopathic aetiology. Recent research has described two children with idiopathic WS who later developed a childhood absence epilepsy. We reviewed the medical records of patients with West syndrome admitted to the our Child Neuropsychiatry Unit in the last 15 years in order to know the clinical evolution of infantile spasms.We report a child with West syndrome with onset at 8 months of age followed by some clusters of bilateral, arrhythmic myoclonic jerks of the upper limbs, mainly on awakening, synchronous with the generalized discharges of 4 Hz spike-wave occurring at 12 years of age and by co-occurrence of a later generalized tonic-clonic seizure at 14 years and four months, both sensitive to Levetiracetam suggesting a juvenile myoclonic epilepsy. This unusual evolution, never previously reported, suggests that both electroclinical features mentioned above may share some pathophysiological processes genetically determined which produce a susceptibility to seizure and emphasizes that the transition between different age-related epileptic phenotypes may involve also the West syndrome.

Epilepsy syndromes

Juvenile myoclonic epilepsy Juvenile myoclonic epilepsy (JME) is a genetic generalised epilepsy that occurs in patients aged 8 to 20 years. Patients have normal cognition and are otherwise neurologically intact. The most common seizure is myoclonic jerks, although generalized tonic-clonic seizures and absence seizures may occur as well. Myoclonic jerks usually cluster in the early morning after awakening. The EEG reveals generalized 4–6 Hz spike wave discharges or multiple spike discharges. These patients are often first diagnosed when they have their first generalized tonic-clonic seizure later in life, when they experience sleep deprivation (e.g., freshman year in college after staying up late to study for exams). Alcohol withdrawal can also be a major contributing factor in breakthrough seizures, as well. The risk of the tendency to have seizures is lifelong; however, the majority have well-controlled seizures with anticonvulsant medication and avoidance of seizure precipitants.

Epilepsy syndromes

Lennox-Gastaut syndrome Lennox-Gastaut syndrome (LGS) is a generalized epilepsy that consists of a triad of developmental delay or childhood dementia, mixed generalized seizures, and EEG demonstrating a pattern of approximately 2 Hz "slow" spike-waves. Onset occurs between two and 18 years. Epilepsy is consider a chronic (meaning it lasts for a long time) condition that is defined by seizures. Lennox-Gastaut syndrome (LGS) is a rare and severe form of epilepsy. As in West syndrome, LGS result from idiopathic, symptomatic, or cryptogenic causes, and many patients first have West syndrome. Authorities emphasize different seizure types as important in LGS, but most have astatic seizures (drop attacks), tonic seizures, tonic-clonic seizures, atypical absence seizures, and sometimes, focal seizures. Anticonvulsants are usually only partially successful in treatment.

["Benign" form of myoclonic epilepsy in children].

Among 62 children with myoclonic epilepsy who had first seizures between 1 and 10 years, without clinical or radiological evidence of brain lesion, we selected the 16 patients who had exhibited several types of fits and had stopped having seizures for over two years. First seizures occurred between 18 months and 4 years, and they were generalized clonic, tonic-clonic or tonic. After a mean 3 months' period, patients started also to have absence and myoclonic fits. During the period with various types of seizures, that lasted a mean 10 months, patients were ataxic and hyperkinetic, and 11 of them suffered myoclonic absence status for several hours or days. The EEG showed a high voltage rhythmic slow-wave activity with spikes, differing from the slow spike wave tracing of the Lennox-Gastaut syndrome, and there was no photosensitivity. The mean duration of the epilepsy was 1 year and 4 months and the last seizures were convulsive, occurring mainly during sleep. The clinical and EEG pattern, the high familial incidence are shared by the Doose syndrome, of which the present series seems to be a subgroup, as are other well-defined syndromes: benign and severe myoclonic epilepsies of infancy.

Eye closure related spike and wave discharges: clinical and syndromic associations.

Precipitation of spike and wave (SW) discharges in some epileptic patients by eye closure (EC) has rarely been reported. To disclose the clinical characteristics and classification of syndromes of epileptic patients with SW discharges induced by EC, we investigated 10 patients (1 M, 9 F) showing this peculiar EEG feature. The patients aged between 9-39 years (mean 20.6 +/- 9.058), underwent short-term (1-3.5 hr) video-EEG investigations in order to document the appearance of the SW discharges within 3 seconds of the act of EC, in at least two occasions. Clinical analysis showed that 5 female patients who had the syndrome of juvenile myoclonic epilepsy (JME) had a later onset of epilepsy (13-15 years) than the 3 patients (3 girls) with eyelid myoclonia with absences (EMA) (3-8 years of age at onset). The remaining 2 patients who were diagnosed as childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) according to the international classification, did not show photosensitivity on the video-EEG. All but one of the 5 JME patients had experienced myoclonic seizures in intermittent photic stimulation (IPS) at the time of EC, associated with multiple spike and wave discharges. Two of the 3 EMA patients exhibited typical absences with eyelid myoclonia during the act of EC. The high rate of family history of epilepsy in first degree relatives of our patients was an outstanding feature, which could have future implications in research of the genetic basis of epilepsy patients with ECS.

Juvenile myoclonic epilepsy: a 5-year prospective study.

We made a long term prospective study of 66 patients with juvenile myoclonic epilepsy (JME). Prevalence was 10.2% among 672 patients with epilepsies. Sex distribution was equal. Sixty-three were not diagnosed on referral; JME was not initially recognized in the epilepsy clinic in 22. Clinical typical absence seizures were reported in 33.3%, myoclonic jerks in 97% and generalized tonic-clonic seizures (GTC) in 78.8% of the patients. Mean age (+/- SD) at onset was 10.5 +/- 3.4 years (range 5-16 years) for absence seizures, 15 +/- 3.5 years (range 8-26 years) for myoclonic jerks, and 16 +/- 3.5 years (9-28) years (range 1-9 years) and GTC by 4.4 +/- 2.7 years (range 1-8 years) in 14 (21.2%) patients who manifested all three types of seizure. Absence were never antedated by myoclonic jerks or GTC. Myoclonic jerks occurred on awakening in 87.5% of the patients. GTC occurred mainly on awakening, but other patients had nocturnal or diurnal GTC with no circadian distribution. Neurologic examination was normal for all patients except for tremor of the hands similar to essential tremor, noted in 35% of patients. Computed tomography (CT) brain scans were normal: 93% of patients had precipitating factors: sleep deprivation (89.5%), fatigue (73.7%), photosensitivity (36.8%; television and video games 8.8%), menstruation (24.1% of women), mental concentration (22.8%), and stress (12.3%). Incidence of JME among siblings (13 of 41 examined families) implies an autosomal recessive mode of inheritance for this Arab population. EEGs were frequently normal in treated patients. At least one abnormal EEG was recorded in 56 (84.9%) patients. Abnormalities consisted mainly of generalized discharges of spike/double spike and/or polyspike and slow wave. Frequent multiple spikes and discharge fragmentations varied from 0.5- to 20-s duration (mean 6.8 s). Twenty (30.3%) had focal abnormalities, and 18 (27.3%) had photoconvulsive discharges. Eighty-eight percent of patients remained seizure-free for > or = 3 years of follow-up. Effective treatment was achieved with valproate (VPA); control of myoclonic jerks was improved with clonazepam (CZP). CZP monotherapy did not consistently prevent GTC. Adding small doses of CZP with simultaneous reduction of VPA was the most effective and better tolerated form of medication, particularly in patients demonstrating an adverse reaction or requiring a large VPA dosage. VPA dosage was successfully reduced in 15 patients who were seizure-free for > 2 years and had infrequent seizures before treatment, but 9 of 11 patients relapsed after VPA discontinuation.(ABSTRACT TRUNCATED AT 400 WORDS)

[Electroclinical features of myoclonic-atonic epilepsy].

To summarize the electroclinical characteristics of myoclonic atonic epilepsy (MAE) in children. The clinical data, video electroencephalogram (EEG) and simultaneous electromyography (EMG) of MAE patients were analyzed. The treatment and its effects were followed up. In 47 MAE patients, 25 had a history of febrile seizures (FS), 20 had a family history of FS or epilepsy. All patients had a normal development before the illness. The age of afebrile seizure onset was between 1.4 years to 5.8 years. The first seizure was generalized tonic-clonic seizure (GTCS) in 41 patients (87.2%). All patients had multiple seizure types, including 47 GTCS (97.9%), 34 myoclonic atonic seizures (72.3%), 47 myoclonic seizures (100%), 32 atonic seizures (68.1%), 36 atypical absences (76.6%) and 3 tonic seizures (6.4%). EEG backgrounds were slow or parietal θ rhythm, interictal EEG showed 1-4 Hz (predominant 2-3 Hz) generalized spike and wave or poly spike and wave discharges in all cases. Seizures were controlled by antiepileptic drugs (AEDs) in 41 patients (87.2%). Valproate was used in 37. Lamotrigine was used in 26. Mild mental retardation was observed in 10 children after the onset of the illness. The clinical features of MAE included the following: the development was normal before the onset of the illness; the onset of seizure type was often GTCS. All patients had multiple generalized seizure types. Myoclonic atonic seizure was its characteristic seizure type. EEG showed generalized discharges. Early diagnosis and rational choice of AEDs are important for getting a better prognosis.

Myoclonic-astatic epilepsy of early childhood--clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome.

The aim of this study is to elucidate the clinical and neurophysiological characteristics of the myoclonic, myoclonic-astatic, or astatic seizures in patients with myoclonic-astatic epilepsy (MAE) of early childhood, and to discuss on the nosology of this unique epileptic syndrome. The subjects included 30 patients, who fulfilled the following modified International League Against Epilepsy (ILAE) criteria for MAE, and whose main seizures were captured by video-electroencephalographs (EEG) or polygraphs. The modified ILAE criteria includes: (1) normal development before onset of epilepsy and absence of organic cerebral abnormalities; (2) onset of myoclonic, myoclonic-astatic or astatic seizures between 7 months and 6 years of age; (3) presence of generalized spike- or polyspike-wave EEG discharges at 2-3 Hz, without focal spike discharges; and (4) exclusion of severe and benign myoclonic epilepsy (SME, BME) in infants and cryptogenic Lennox-Gastaut syndrome based on the ILAE definitions. The seizures were investigated precisely by video-EEG (n=5), polygraph (n=2), and video-polygraph (n=23), which identified myoclonic seizures in 16 cases (myoclonic group), atonic seizures, with or without preceding minor myoclonus, in 11 cases (atonic group), and myoclonic-atonic seizures in three cases. All patients had a history of drop attacks, apart from ten patients with myoclonic seizures. Myoclonic seizures, involving mainly the axial muscles were classified into those with mild intensity not sufficient to cause the patients to fall (n=10) and those that are stronger and sufficient to cause astatic falling due to flexion of the waist or extension of the trunk (n=6). Patients in the atonic group fell straight downward, landed on their buttocks, and recovered immediately. Analysis of the ictal EEGs showed that all attacks corresponded to the generalized spike or polyspikes-and-wave complexes. In the atonic form, the spike-and-wave morphology was characterized by a positive-negative-deep-positive wave followed by a large negative slow wave. In two patients, the intensity of the atonia appeared to correspond to the depth of the positive component of the spike-and-wave complexes. We did not detect any significant differences in the clinical and EEG features and prognosis, between the atonic and myoclonic groups. Although the determination of exact seizure type is a prerequisite for diagnosing an epileptic syndrome, the strict differentiation of seizure type into either a myoclonic or atonic form, does not appear to have a significant impact on the outcome or in delineating this unique epileptic syndrome. At present, we consider it better to follow the current International Classification of Epileptic Syndromes and Epilepsies until a more appropriate system than the clinico-electrical approach for classifying patients with MAE is available.

Clinical and EEG characteristics of Juvenile Myoclonic Epilepsy.

Objective : To determine the clinical and electroencephalographic characteristics of patients with Juvenile Myoclonic Epilepsy (JME). In this descriptive case series study, 60 patients of Juvenile myoclonic epilepsy (JME) were included. After detailed history clinical examination, Electroencephalography (EEG) with standard protocol was performed in all patients and was analyzed by a neurologist. Out of 60 patients, 26 (43.3%) were males and 34 (56.6%) were females. Mean age at the onset of myoclonic jerks (MJ) and generalized tonic clonic seizures (GTCS) was 13.7 ± 2.12 years and 14.15 ± 1.79 years respectively. Average delay in the diagnosis was 5.2 years. Myoclonic jerks (MJ) were present in all patients, GTCS in 52 (86.6%), and absence seizures in 8 (13.33%) patients. 6 (10%) had only Myoclonic Jerks. First seizure type was MJ in 52 (86.6%) and absence in 8 (13.3%). Most common precipitating factors were sleep deprivation in 80% and fatigue in 66.6%. Family history for epilepsy was positive in 20%. Diagnosis by referring physicians was JME in only 6 (10%) patients. EEG was abnormal in 42 patients (70%) showing generalized , 4- to 6-Hz polyspike and wave in 27 (45%), generalized single spike/ sharp waves in 7 patients (11.6%), 8 (13.3%) patients had 3-Hz spike-and-wave (SW) activity in addition to the polyspike-and-wave (PSW) pattern. Independent focal EEG abnormalities were noted in 12 patients (20%). Many of our patients were misdiagnosed by the referring physicians and were prescribed inappropriate antiepileptic drugs. Factors causing misdiagnosis were failure to elicit history of myoclonic jerks, misinterpreting myoclonic jerks as partial seizures and misinterpretation of EEG abnormalities.

Epilepsy syndromes

Childhood absence epilepsy Childhood absence epilepsy (CAE) is a genetic generalized epilepsy that affects children between the ages of 4 and 12 years of age, although peak onset is around five to six years old. These patients have recurrent absence seizures, brief episodes of unresponsive staring, sometimes with minor motor features such as eye blinking or subtle chewing. The EEG finding in CAE is generalized 3 Hz spike and wave discharges. Some go on to develop generalized tonic-clonic seizures. This condition carries a good prognosis because children do not usually show cognitive decline or neurological deficits, and the seizures in the majority cease spontaneously with ongoing maturation.

Panayiotopoulos syndrome

In Panayiotopoulos’ original study, ictal vomiting occurred in only 24 children out of 900 patients of all ages with epileptic seizures. Twenty-one were otherwise normal children (idiopathic cases constituting what is now considered Panayiotopoulos syndrome), and 3 had symptomatic epilepsies. Half of the seizures were lengthy, lasting for hours (autonomic status epilepticus). The EEG of the 21 idiopathic cases showed great variations: 12 had occipital paroxysms or spikes alone or with extraoccipital spikes; 2 had central spikes and giantsomatosensory evoked spikes; 2 had midline spikes; 1 had frontal spikes; 1 had brief generalized discharges; and 3 had consistently normal EEG. Subsequent attention was focused on the predominant group with occipital spikes, which was established as "early onset benign childhood epilepsy with occipital paroxysms". The other group of 9 children with extraoccipital spikes or normal EEGs was reevaluated much later; their clinical manifestations and

Epilepsy with myoclonic absences.

Among the epileptic syndromes that are defined mainly on the basis of a characteristic seizure type, epilepsy with myoclonic absences (EMA) stands out as a somewhat controversial entity. This is because the sound and evident clinical characteristics on which it was identified some 30 years ago have evolved, mostly as a consequence of changes in the practical management of epilepsies and to the description of myoclonic components in a variety of other generalised epilepsies with absences. Myoclonic absences (MA) are described as typical absences with sudden onset and offset that are associated with generalised spike and wave (SW) discharges on the ECG, with distinctive traits. Clinically, absences are associated with axial hypertonia (the subject usually bends forward and slightly raises their shoulders and arms), and jerks synchronous with the SW discharges. Neurophysiologically, axial hypertonia and rhythmic jerks may be recorded on polygraphic surface electromyogram leads in association with the typical SW discharges; as such, despite an ECG, the diagnosis may be missed in the absence of video documentation of the seizure and/or adequate polygraphy. MA need to be distinguished from absences with other types of prominent myoclonic accompaniment (perioral, eyelid, limbs).The prognosis of EMA remains variable. Modern therapeutic combinations, such as valproic acid and ethosuximide, or valproic acid and lamotrigine, are usually effective; however, in a proportion of patients, seizures are resistant to drug treatment. These patients may experience cognitive deterioration and, in some cases, evolution towards a more severe form of epilepsy, including the Lennox-Gastaut syndrome. The more benign cases usually present with MA as the only seizure type, while patients who experience other seizures, especially generalised tonic-clonic seizures, in association with MA may have a less favourable outcome.

Diagnostic issues and treatment of cryptogenic or symptomatic generalized epilepsies.

To clarify the diagnostic issues and treatment of patients with cryptogenic or symptomatic generalized epilepsies, not including West syndrome (WS), we investigated electroclinical change during the clinical course, and treatment effects in these patients. The selection criteria were minor generalized seizures as their main seizure type and diffuse epileptic discharges as their main EEG findings. Regarding EEG, we included EEGs that predominantly displayed multifocal spike-waves because of the inclusion of severe epilepsy with multiple independent spike foci (SE-MISF). We divided the subjects into two groups according to their main seizure types: Group A (54 patients) with brief tonic seizures and Group B (24 patients) with myoclonic seizures and/or atypical absences. The main epileptic syndromes were considered to be Lennox-Gastaut syndrome and SE-MISF in Group A, and epilepsy with myoclonic-astatic seizures in Group B. A history of WS was often seen in Group A, but it was exceptional in Group B. During the clinical course, seizure types did not basically change in Group A. EEG patterns were changeable in both groups. Although there was some overlap in electroclinical manifestations among epileptic syndromes, a transition between the two groups was not seen. High-dose valproate and ethosuximide were the most effective in Groups A and B, respectively. Long-term prognosis was significantly more favorable in Group B than in Group A.

Idiopathic generalised epilepsies with 3 Hz and faster spike wave discharges: a population-based study with evaluation and long-term follow-up in 71 patients.

For several years we have been following patients with intractable, childhood-onset idiopathic generalised epilepsies with > or = 3 Hz spike-wave discharges. Our need to find explanations for their intractability was the starting point for this study. We were interested in identifying characteristics, which would predict intractability; evaluating how these patients were treated and whether polytherapy was useful. We identified patients with > or = 3 Hz spike-wave discharges by reviewing EEG reports recorded between 1983 and 1992. Data were collected from medical records and through personal interviews. We identified 82 patients with tentative idiopathic generalised epilepsy. Eleven were excluded. Thirty-eight patients had childhood absence epilepsy, 18 had juvenile absence epilepsy, 13 had juvenile myoclonic epilepsy and two had eyelid myoclonia with absences: 89.5, 78, 38 and 0% of the patients in each group, respectively, had been seizure free for more than 2 years. Twenty percent of the patients had intractable seizures. All intractable patients with juvenile absence epilepsy had rhythmic, random eyelid blinking and generalised tonic-clonic seizures. A history of more than ten generalised tonic-clonic seizures was associated with intractability in juvenile myoclonic patients. Monotherapy with ethosuximide or valproate resulted in seizure control in 65% of patients. Seventeen patients (24%) were treated with polytherapy, six achieved remission. These six patients had childhood absence epilepsy and juvenile absence epilepsy. Positive outcome was found in childhood absence epilepsy and juvenile absence epilepsy. Intractable seizures were more frequent among patients with juvenile myoclonic epilepsy. None of them benefited from polytherapy with conventional anti-epileptic drugs.

Myoclonus

Juvenile myoclonic epilepsy (JME) usually consists of jerking and muscle twitches of the upper extremities. This may include the arms, shoulders, elbows, and very rarely, the legs. JME is among the most common types of epilepsy and can affect one of every 14 people with the disease. These seizures typically occur shortly after waking up. Onset for JME can be seen around puberty for most patients. Administration of medications that also treat multiple seizure types is usually the most effective form of treatment. Lennox-Gastaut syndrome (LGS), or childhood epileptic encephalopathy, is a rare epileptic disorder accounting for 1–4% of childhood epilepsies. The syndrome has much more severe symptoms ranging from multiple seizures daily, learning disabilities, abnormal findings in electroencephalogram (EEG). Earlier age of seizure onset is correlated with higher risk of cognitive impairment.

Juvenile Myoclonic Epilepsy -- Evaluation

History provides most of the clues necessary for a diagnosis of JME. Physical examination is generally unremarkable. An electroencephalogram (EEG) provides supporting evidence for diagnosing JME. An interictal EEG is abnormal in the majority of patients with JME. [16] If the routine EEG is normal, an overnight and sleep-deprived EEG is abnormal in almost every patient. The abnormalities are seen mostly during the transition from sleep to awakening. [17] The typical EEG in JME shows diffuse, symmetric, bilateral 4 to 6 hertz (Hz) polyspike and wave discharges with a fronto-central predominance. The background has normal alpha rhythm, and approximately half of the patient population will have focal or asymmetric abnormalities. [18] Ictal or continuous EEG recording shows 10-16 Hz polyspike discharges with myoclonic jerks. The spikes correlate with myoclonic jerks. [19] The EEG findings in GTC (low voltage fast activity with spike and wave discharges) and absence seizures (generalized 3 Hz spike and wave discharges) associated with JME are similar to those seen with other epilepsies. Since it is known to be photosensitive epilepsy, photic stimulation provides enhanced yield on EEG in JME.

Epilepsy syndromes

Febrile infection-related epilepsy syndrome Febrile infection-related epilepsy syndrome (FIRES) Frontal lobe epilepsy Frontal lobe epilepsy, usually a symptomatic or cryptogenic localization-related epilepsy, arises from lesions causing seizures that occur in the frontal lobes of the brain. These epilepsies can be difficult to diagnose because the symptoms of seizures can easily be confused with nonepileptic spells and, because of limitations of the EEG, be difficult to "see" with standard scalp EEG. Juvenile absence epilepsy is an idiopathic generalized epilepsy with later onset than CAE, typically in prepubertal adolescence, with the most frequent seizure type being absence seizures. Generalized tonic-clonic seizures can occur. Often, 3 Hz spike-wave or multiple spike discharges can be seen on EEG. The prognosis is mixed, with some patients going on to a syndrome that is poorly distinguishable from JME.

Myoclonic astatic epilepsy

The outcome is unfavorable if generalized tonic-clonic, tonic, or clonic seizures appear at the onset or occur frequently during the course. Generalized tonic-clonic seizures usually occur during the daytime in this disorder, at least in the early stages. Nocturnal generalized tonic-clonic seizures, which may develop later, are another unfavorable sign. If tonic seizures appear, prognosis is poor. Status epilepticus with myoclonic, astatic, myoclonic-astatic, or absence seizures is another ominous sign, especially when prolonged or appearing early. Failure to suppress the EEG abnormalities (4- to 7-Hz rhythms and spike-wave discharges) during therapy and absence of occipital alpha-rhythm with therapy also suggest a poor prognosis (Doose 1992a).

Idiopathic generalized epilepsy

Juvenile absence epilepsy Juvenile absence epilepsy is similar to CAE but has an onset between ages 9 and 13. Other differences are that patients with this disorder have less frequent but longer absence seizures than those with CAE. There are a number of possible genetic loci for this disorder, though no causative genes have been demonstrated. Juvenile myoclonic epilepsy Also known as Janz syndrome, juvenile myoclonic epilepsy (JME) is a common form of epilepsy, accounting for ~10% of all cases and ~25% of cases of idiopathic generalized epilepsies. Many children with CAE go on to develop JME. JME first presents between the ages of 12 and 18 with prominent myoclonic seizures. These seizures tend to occur early in the morning. Patients with JME may also have generalized tonic-clonic seizures and absence seizures. Linkage of this disorder has been shown to mutations in the genes GABRA1, CACNB4, CLCN2, GABRD2, EFHC1, and EFHC2.

Some clinical and EEG aspects of benign juvenile myoclonic epilepsy.

Twelve patients with benign juvenile myoclonic epilepsy (BJME) representing 4% of our population of epileptics (n = 275) are presented. Only two patients (17%) had myoclonic jerks as the only seizure type. Seven (58%) had generalized tonic-clonic seizures (GTCS) and myoclonus. Three patients (25%) had absence seizures (AS), GTCS, and myoclonic jerks. Electroencephalographic evidence of photosensitivity was found in four (33%). Auditory precipitation of seizures was found in one patient. As is the case with other primary generalized epilepsies, the onset of BJME seems to be age specific. In our series the mean age of onset in years was 4.3 for AS, 14.75 for myoclonic jerks, and 16.4 for GTCS. It took an average of 8.5 years from the onset of BJME (range, 2-20 years) and 6.5 years from the onset of GTCS (range, 2 months-6 years) until the condition was properly recognized. Five patients experienced at least one episode of myoclonic status epilepticus. Generalized, paroxysmal, symmetric polyspike and slow wave discharges are the typical EEG finding. These complexes, however, showed considerable interpatient variability. Sleep deprivation proved to be the most valuable activating procedure. Valproic acid monotherapy effectively controlled myoclonic jerks as well as associated GTCS in most patients.

[Janz's juvenile myoclonic epilepsy: a little-known frequent syndrome. A study of 85 patients].

Juvenile myoclonic epilepsy (JME) constitutes 10% of all epilepsies. Despite this syndrome being well defined, its diagnosis is usually delayed. The aim of this study was to analyze the clinical and electroencephalographic characteristics to facilitate guidelines to contribute to its recognition. From January 1986 to July 1993 the clinical and EEG data of 85 patients with JME were prospectively studied. In 68 cases (80%) the polygraphic study of sleep was also analyzed during a nap period. The series included 44 males and 41 females of a mean age of 28 years (range: 13-63). Fifty-six percent of the cases showed family history of epilepsy and/or febrile convulsions. All the patients had myoclonic crisis with the age of 15 being the mean age of initiation (range: 8-27). Eighty-seven percent also had generalized tonic-clonic crisis and 18% typical absences. Myoclonias were presented daily up the administration of adequate treatment in 60% of the cases with 21% having myoclonic status. The mean interval from the initiation of the myoclonic crisis to diagnosis of JME was of 10.6 years. On monotherapy with valproic acid and following a mean follow up period of 23.8 months, 86% of the patients remained free of crisis. Nonetheless, the rate of recurrence was 100% in the 19 patients who discontinued the treatment. Surveillance EEG was normal on some occasion in 88% of the cases. The most characteristic paroxysms were the following: wave-point at 4-5 Hz and generalized rapid wave-polypoint. Light stimulation provoked a paroxysmal response in one third of the cases. Sleep EEG was abnormal in all the patients. An activation of the paroxysms during non-REM sleep in 78% of the cases and on waking up in 25%. Juvenile myoclonic epilepsy is a well defined syndrome. Its diagnosis is based on directed anamnesis allowing myoclonic jerks to be collected which often remain unperceived, and EEG exploration with sleep tracing in which the characteristic outbreaks of wave-point or generalized rapid wave-polypoints may be discovered.

[Clinical-electrophysiological pattern of juvenile myoclonic epilepsy].

18 patients (9 females, 9 males) with juvenile myoclonic epilepsy (JME) were studied. Despite a fairly long duration of the disease (from 1 to 34 years--mean 9.5 years) the patients had not been properly diagnosed. In all cases routine serial EEG examinations, 24-hour EEG with Medilog System 9000, and polygraphic Video/EEG/EMG recording were done (Videometry Processor, Glonner). Typical attack triads occurred in 7 cases (39%), grand mal seizures and myoclonic attacks in 10 cases (56%), one patient had only myoclonic seizures. EEG demonstrated already in routine recording occurrence of seizure activity with spike/polyspike-slow wave 3-6 Hz complexes. 24-hour EEG made possible demonstration of these complexes in the remaining 4 cases. Seizure activity during clinical myoclonic seizures were recorded in 8 cases, and during absences in 2 cases. A valuable method was also Video/EEG/EMG recording which showed occurrence of both myoclonic seizures (in 5 cases) and absences (9 cases). Three patients with absences were not aware of their seizures. In 8 patients EEG findings demonstrated focal abnormalities which contributed to previous diagnostic errors. The described clinical and electrophysiological features met fully the diagnostic criteria of JME. In the Polish literature this is the first report on such a large groups of JME cases. Attention is called to diagnostic difficulties due to poor knowledge of this disease and its manifestations which leads to inaccurate history taking. Another cause is non-availability of full neurophysiological diagnostic facilities in many centers.

Severe idiopathic generalized epilepsy of infancy with generalized tonic-clonic seizures.

While the literature on infantile epilepsies with minor and major seizures is extensive, little consideration has been given to infantile epilepsy with generalized tonic-clonic seizures (GTCS) alone. The aim of the present study was to analyze the data of a large group of patients and their families to obtain further insight into the clinical picture and pathogenesis of this type of epilepsy. The 101 children (58 boys, 43 girls) met the following inclusion criteria: onset of the epilepsy with febrile or afebrile GTCS in the first 5 years of life, absence of primary organic brain lesion or progressive brain disease, severe course with frequent febrile and/or afebrile GTCS, failure of conventional anticonvulsive therapy. The epilepsy predominantly afflicts normally developed infants, boys and girls being about equally affected. The epilepsy begins with frequent febrile or afebrile GTCS, characteristically of long duration and often with alternating lateralization. In half of the cases additional myoclonic or myoclonic astatic seizures and/or absences occur. The initial GTCS phase is the same in epilepsies with and without minor seizures. Erratic myoclonias are especially characteristic. With advancing age, the symptomatology becomes increasingly polymorphic due to the occurrence of additional simple and complex focal and tonic seizures. Severe impairment of mental development soon after onset is a leading symptom. The overall death rate was 9%. Only 11% of the patients had been seizure-free for at least two years at final examination. The EEG was initially normal and subsequently exhibited diffuse 4-7/s rhythms, and only later spikes and waves of irregular shape (87%). Focal sharp waves occurred transiently in 26%. The family history and EEG of probands and relatives showed the pathogenesis to be decisively determined by genetic factors. Early infantile GTCS epilepsy represents a genetically determined (idiopathic) epileptic encephalopathy. It overlaps with other forms of early childhood epilepsy such as severe myoclonic epilepsy, severe type of myoclonic astatic epilepsy, as well as early childhood absence epilepsy with GTCS.

Spike-and-wave

Lennox-Gastaut syndrome Epileptic encephalopathies are a group of conditions that result in deterioration of sensory, cognitive, and motor functions due to consistent epileptic activity. Lennox-Gastaut syndrome (LGS) is a childhood epileptic encephalopathy characterized with generalized seizures and slow spike-wave activity while awake. LGS is a combination of atonic absences, tonic seizures, cognitive deterioration, and slow spike-wave activity in the EEG. This syndrome usually results from focal, multifocal, or diffuse brain damage and can be divided into symptomatic and cryptogenic types. Cognitive deterioration with high-frequency spike-wave activity affects most patients 2–9 years old with generalized seizures. The age of onset for LGS is between 1 and 10 years, between 2 and 6 years for symptomatic cases and 5 and 8 years for cryptogenic cases. Episodes can be triggered by modifications of treatment, which usually involves benzodiazepines, or changes in the conditions of life.

Usefulness of a morning routine EEG recording in patients with juvenile myoclonic epilepsy.

To evaluate if a standard awake EEG recording in the morning is superior to afternoon awake EEG session in detecting generalized epileptiform discharges (GEDs) in patients with juvenile myoclonic epilepsy (JME). The study group included 29 consecutive patients (23 women; mean age 22.3+/-6.3 years; age at onset of JME 15.4+/-3.4 years) with JME. Out of 29 patients 5 were untreated, 9 patients were treated with valproate, 8 with lamotrigine, 6 with levetiracetam and 1 patient with valproate plus phenobarbital. Two routine consecutive interictal EEG recordings were performed at 9a.m. and at 3p.m., respectively, while the subject was awake, on the same day after a a regular nocturnal sleep at own home. The morning EEG recording showed GEDs (i.e., generalized polispike and waves, photoparoxysmal response, or both). in 20/29 patients. In 15 of these 20 patients, the afternoon recording was normal and this difference was statistically significant (p < or = 0.001). Moreover, there was a striking reduction of GEDs in three of the remaining five patients. Nine/29 patients had both morning and afternoon EEG recording normal. The results of this study have illustrated a significant greater rate of detection of generalized epileptiform abnormalities by performing standard awake EEG in the morning in comparison with an afternoon session.