A 5-year-old marasmic male child was referred to our institute for multiple lymphadenopathy. His mother in the same household had pulmonary tuberculosis. On admission, the child had no history of cough, dyspnea or night sweats. However, he was lethargic and unable to stand or sit without support. His general examination was quite remarkable for the evidence of severe protein energy malnutrition (PEM) and rickets. He had marked pallor, generalized xerosis, crusted lesions with boil on the lower back, and hyperpigmented friction dermatosis on buttocks. His crawling movement was further complicated by valgus deformity of legs due to nutritional rickets. Cervical [] and groin examination showed multiple matted lymph nodes forming a large cervical and inguinal lymphadenopathy. His ocular examination revealed nebular keratopathy in both eyes. His initial evaluation at the previous institute did not reveal any malignancy as a possible etiology for large cervical and inguinal lymphadenopathy. His detailed clinical neurological evaluation did not reveal any positive findings. Hematological investigation revealed severe anemia (Hemoglobin - 7.3 gm/dl), leukocytosis (Total Leukocytes Count - 15,700/mm3; 84% neutrophils, 12% lymphocytes, 3% monocytes, and 1% eosinophils), and mild thrombocytopenia (87,000/mm3). Biochemical investigation for rickets showed hypocalcemia (serum calcium - 6.31 mg/dl), normal phosphate (serum phosphate - 4 mg/dl), higher alkaline phosphate - 310, and low Serum 25 (OH) D - 8 ng/ml. He was seronegative for human immunodeficiency virus (HIV) on Enzyme-Linked Immunosorbent Assay (ELISA) test; Mantoux test was also negative. The chest X-ray revealed miliary Koch's infiltration with a widening of the costochondral junction of the ribs due to rickets in the bony thorax []. Abdominal and thoracic ultrasound revealed mild hepatomegaly, mild splenomegaly, and enlarged mesenteric, inguinal, peripancreatic, celiac axis, periportal, and bilateral iliac lymph nodes. Corroborative radiological evaluation for rickets (X-ray both knee and wrist joint) showed metaphyseal widening with splaying and fraying of epiphysis and changes related to osteoporosis, and pencil line thinning of the epiphyseal cortex, suggesting rickets []. Fine needle aspiration cytology of cervical lymph node showed features of tuberculous lymphadenitis with the presence of acid-fast bacilli.
Antituberculosis therapy including Streptomycin injection (according to CAT-1 of the National Tuberculosis Control Program) and oral steroids were started. Cytology report confirmed the Koch's etiology. Severe PEM and vitamin deficiency were aggressively managed in the hospital with supervised high protein diet and oral therapeutic supplementation of Vitamin A and Vitamin D with calcium. During 2 weeks of observation in the pediatric ward, the child recovered gradually and was discharged home with oral medicines. Within 3 weeks of discharge, the patient presented again to the pediatric intensive care unit with generalized tonic-clonic convulsions. There was no focal neurological sign on examination. The computed tomography (CT) scan of brain revealed multiple small tuberculomas in the brain parenchyma scattered like millets in the cerebral hemispheres, bilateral cerebellum, and brain stem []. Few of them were conglomerate in the left cerebellum, right parietal region, and pons with periventricular hypodensity. Anticonvulsant therapy was started and Streptomycin and steroid were added to the chemotherapy. Afterward, the child was symptom free.


A 42-day-old baby boy who underwent mechanical ventilation in the second day of life gradually weaned off from the respirator but while extubated, he could not tolerate extubation. He had severe tracheomalacia on rigid bronchoscopy. Patient had Echocardiography and spiral computed tomography scan for ruling out of extrinsic pressure, after being certain of abnormal vasculature or mass, we decided to insert some kinds of stent which available. T-tube tracheal stent was rigid and not suitable in size and pliability for trachea of neonate. T-tube cholangiogram has the same shape but a very pliable and suitable size #16 (SUPA/Iran) with low cost for small neonate. We performed 1-2 cm incision above sternal notch and through a tracheotomy and trimming the catheter to appropriate size was placed []. He tolerated the tube and discharged and 9 months later tube can be discontinued.


A 25 year-old woman was transferred to the The Heart Center of Chonnam National University Hospital because of shortness of breath and hypotension for one day. Four days before admission, the patient complained of fever and sore throat, but took no medications. Three days earlier, the patient had nausea, vomiting, loose stools, lower abdominal pain and a general feeling of weakness. The patient visited a local clinic and was admitted for conservative treatment for acute gastroenteritis. At that time of admission, her body temperature was normal and blood pressure was 100/60 mmHg. On hospital day 2, the patient complained of feeling dizzy, had chest discomfort and progressive shortness of breath; the blood pressure dropped to 80 mmHg in systole. The patient was then transferred to our hospital.
The patient was a student and resided in a local area. There was a history of occasional alcohol. There was no history of smoking, drugs or allergies prior to admission. On physical examination, the patient was drowsy, but oriented, and had shortness of breath. The blood pressure was 85/40 mmHg with a continuous intravenous infusion of dopamine (15 µg/kg/min) and norepinephrine (0.02 µg/kg/min), the pulse 150 beats per minute, the respiratory rate 24 breaths per minute, and the body temperature 36℃. The oxygen saturation was 100 percent with oxygen supplied at 3 L/min by nasal cannula. The lung sounds were coarse on both lower lung fields, the heart was rapidly beating without murmur, and the r neck veins were slightly engorged. The skin was cool and clammy, but had no eruption, and no pitting edema. The remainder of the physical examination including neurological examinations revealed no abnormalities. The laboratory findings showed a complete blood count with increased white blood cells (17,500/mm3 (neutrophil 75%, eosinophil 2.6%)) and normal hemoglobin (12.7 g/dL), hematocrit (37.9%), and platelet count (267,000/mm3). Blood chemistries showed the following: aspartate aminotransferase 299 U/L, alanine aminotransferase 422 U/L, alkaline phosphatase 44 U/L, total protein 5.6 g/dL, albumin 3.0 g/dL, and lactate dehydrogenase 1,226 U/L, blood urea nitrogen 26.7 mg/dl, creatinine 1.2 mg/dL, amylase 23 U/L, sodium 135 mEq/L, and potassium 4.1 mEq/L. Cardiac enzymes were elevated (myoglobin 148 ng/mL, creatine kinase 215 U/L, CK-MB 14.3 U/L, troponin I 4.49 ng/mL), and NT-proBNP >35,000 pg/mL and C-reactive protein 9.0 mg/dL were also increased. A chest radiograph revealed increased pulmonary vascular markings and a normal cardiac silhouette (). Electrocardiogram revealed a sinus tachycardia (heart rate 147 bpm), low voltage and right axis deviation, but no definite ST-T change was noted (). Titers of serum neutralizing antibodies for coxsackievirus, adenovirus, Epstein-Barr virus, cytomegalovirus, herpes virus, hepatitis B and C virus, and HIV were all negative. Echocardiography demonstrated a mild hypokinesis of left ventricular (LV) anteroseptal wall with LV wall thickening, and hyperdynamic LV function with reduced stroke volume, moderate amount of pericardial effusion, dilated inferior vena cava with increased hepatic echogenicity, and no significant valvular abnormalities (, ). The endomyocardial biopsy specimen from the right ventricle revealed extensive interstitial edema associated with focal necrosis of myocytes, diffuse infiltration of inflammatory cells with lymphocytes, neutrophils, and eosinophils suggesting acute eosinophilic myocarditis (). Skin tests for allergy and stool ova were negative.
With conservative treatment, the blood pressure was normalized on day 3 after the admission, and the cardiac enzymes were also normalized during the hospitalization. However interestingly, the peripheral blood eosinophil count increased through hospital day 8, from an initial count 450/mm3 to peak 4,300/mm3 (). The patient complained of chest pain on motion, and the electrocardiogram was sinus rhythm with T inversions on all leads (), but no definite friction rub was auscultated. We treated the patient with oral prednisolone, 1 mg/kg of body weight, for the symptoms of pericarditis and it subsided 2 days later. Blood eosinophil count was normalized over 6 days after administration of steroids. The follow up chest radiograph was clear, () and the echocardiography showed good LV wall motion and function with decreased LV wall thickening and normal LV cavity size, and a minimal amount of pericardial effusion remaining (, ). The patient was discharged and followed up at the outpatient clinic with a tapering dose of prednisolone over next 3 months. After 3 months, the electrocardiogram had returned to normal, and the pericardial effusion cleared (, ).


A 39-year-old man was admitted with severe right upper quadrant abdominal pain. The history was significant for partial small bowel resection for intestinal infarction about 5 years prior to this presentation. The patient was otherwise stable without any further investigations or management, and had been asymptomatic until the current episode.
On admission, his vital signs were as follows: blood pressure 160/100 mmHg, pulse rate 72/min, respiratory rate 18/min, temperature 36.3℃. He had minimal conjunctival injection and icteric sclerae. Abdominal examination revealed normal bowel sounds with no palpable abdominal mass or organomegaly. There was tenderness to palpation over the right upper quadrant and epigastrium. He did not have any rebound tenderness and Murphy's sign was negative. Laboratory studies showed WBC 5700/mm3, Hb 12.3 g/dL, hematocrit 35.0%, and platelets 183,000/mm3. Liver function tests revealed a total bilirubin of 5.2 mg/dL, AST 175 IU/L, ALT 185 IU/L, alkaline phosphatase 899 IU/L, and GTP 615 IU/L. Abdominal CT demonstrated multiple high attenuation areas in the biliary duct and gallbladder (). Endoscopic retrograde cholangiopancreatography (ERCP) showed a mild but active blood leak and adherent clots at the ampulla of Vater (). Cholangiography revealed multiple tubular filling defects in the common bile duct as well as the intrahepatic ducts (). Endoscopic sphincterotomy and nasobiliary drainage were performed. During the procedure, hemobilia spontaneously stopped. He reported an improvement in his abdominal pain after the procedure. However, the following day, the patient developed a massive hematemesis and hematochezia, with a drop in his blood pressure to 70/50 mmHg and Hb to 7.5 g/dL. Emergency selective hepatic angiogram demonstrated multiple microaneurysms in the branches of the hepatic arteries () and a slow leak of the contrast medium into the bile duct from the arteriobiliary fistula (). This fistulous tract was blocked with coil and gelfoam (). Following this procedure, his blood pressure stabilized rapidly. There was no evidence of rebleeding. Superior mesenteric, inferior mesenteric and renal artery angiography revealed multiple stenoses, smooth occlusions and microaneurysmal changes in small- to mid-sized vessels. Antinuclear antibody, antineutrophil cytoplasmic antibodies, rheumatoid factor, antiphospholipid antibody and lupus anticoagulant were all negative. A clinical diagnosis of polyarteritis nodosa was made and he was managed with prednisolone 25 mg per day, which was tapered to 5 mg per day, in combination with cyclophosphamide 50 mg per day. He is entirely asymptomatic at six month follow up.


A 56-year-old man was admitted to the otorhinolaryngology department, complaining of multiple palpable masses in the right cervical and submandibular areas, which slowly progressed in size during the past 8 years. The patient had neither any remarkable past medical history, nor a family history relevant to such a complaint. On physical examination, no palpable lymph nodes or masses in any other area of the body were noted. Initial laboratory values revealed mild anemia (Hb 11.6 g/dL), but normal leukocyte and platelet counts. Blood urea nitrogen (BUN), creatinine, calcium, inorganic phosphate, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and serum electrolyte levels were found to be normal. Serum albumin was 3.3 g/dL, and total protein was 8.9 g/dL. There were no abnormalities on the routine urine analysis. Neck and paranasal sinus computed tomography (CT) scans were conducted as part of an evaluation for surgical resection. Numerous enlarged lymph nodes were found to be conglomerated in the right submental and submandibular areas (level II, III, IV, V). The largest of these measured about 6.7×4.7 cm, and the right internal jugular vein was appeared to have been severely compressed by multiple enlarged lymph nodes. The tonsil, salivary gland, paranasal sinus, and nasal cavity yielded no abnormal findings (). The patient underwent surgery for the removal of the masses, but some small remnant masses persisted. Upon the gross examination of the removed lymph nodes, multiple grayish-white masses were seen, ranging from 2.5×1.8×1.5 cm to 6.5×5×4.5 cm in dimensions. Upon sectioning of the lymph nodes, the cut surfaces were grayish-white with focal hemorrhaging (). Histologically, the masses were composed of dense and diffuse infiltrations of atypical plasma cells, with a few residual atrophic lymphoid follicles. These secreted immunoglobulin (Lambda positive) aggregates with extensive amyloid deposits (Congo Red positive, Lambda positive/Kappa negative) in both the stroma and blood vessel walls (). After the operation, the patient was transferred to the hemato-oncologic department, for further evaluation and treatment. A full work-up was conducted in order to ascertain whether there was any other evidence of plasma cell dyscrasia. Serum and urine protein electrophoresis (PEP) and immunoelectrophoresis (IEP) were also conducted. Serum PEP revealed an M-spike in the gamma region, and serum IEP revealed abnormal arcs against anti-IgG and anti-Lambda antisera (), but urine PEP was not performed due to the small amount of available protein, and urine IEP revealed no abnormal arcs. The β2-microglobulin level was found to be normal (1.6 mg/L), and no osteolytic lesions were noted on the skeletal evaluation, which included a skull series. Rouleux formations were seen on the peripheral blood smear. However, bone marrow aspiration and biopsy from the iliac crest revealed trilineage elements of normal hematopoiesis, as well as no evidence of plasma cell dyscrasia. Accordingly, the patient was diagnosed with primary lymph node plasmacytoma, and was treated via radiotherapy (total dose of 50 Gy in 25 fractions). The residual mass is about 1×2 cm sized, so we are planning to monitor the patient with PET CT scans, as well as a neck CT, in order to assess the viability of the remaining mass.


A 70-year-old man was admitted to our hospital due to general weakness for 2 days. He did not have any specific medical history. However, he had a history of heavy alcohol drinking for more than 30 years. The laboratory tests at the time of admission revealed that elevated serum levels of total bilirubin (6.3 mg/dL), direct bilirubin (5.1 mg/dL), aspartate aminotransferase (450 IU/L), alanine aminotransferase (254 IU/L), gamma-glutamyl transpeptidase (399 IU/L), alkaline phosphatase (180 IU/L), and lactate dehydrogenase (890 IU/L). Serologic tests for hepatitis A, B and C were negative. The complete blood count showed decreased level of white blood cell count (3,200 mm-3) and platelet count (61,000 mm-3). However, the level of hemoglobin was within normal limits. Coagulation profiles and C-reactive protein were also within normal limits. The test for serum tumor markers revealed marked elevated level of CA19-9 (462.2 U/mL) and normal levels of alpha-fetoprotein (5.15 IU/mL) and CEA (3.45 ng/mL).
The dynamic contrast-enhanced CT images showed a 6 cm well-encapsulated mass in the right hepatic lobe, with the enhancement pattern of early arterial enhancement and delayed washout. And also, CT images showed enlargement of right and caudate lobe of the liver, and wall thickening and enhancement of both intrahepatic bile ducts (). However, the biliary duct was not dilated. Based on these CT findings in addition to the increased level of CA19-9, the initial tentative diagnosis was a hepatic malignant tumor such as HCC or mass-forming cholangiocarcinoma with underlying alcoholic liver disease and intrahepatic cholangitis. Dynamic gadolinium-enhanced MR imaging was subsequently performed. On fat-suppressed T1-weighted image (), the mass consisted of large area of hypointensity and small area of hyperintensity, which looked reversed with respect to signal intensity on fat-suppressed T2-weighted image (). Small portion of T1 hyperintensity within the mass was interpreted to represent the presence of hemorrhage. The hypointense area of the mass on pre-contrast fat-suppressed T1-weighted image was hyperintense, slightly hypointense and hypointense, respectively, on gadolinium-enhanced T1-weighted arterial, portal, and delayed-phase MR images that were obtained at 30 seconds, 75 seconds and 180 seconds, respectively, after the injection of gadolinium (). According to various imaging findings, such as early arterial enhancement and delayed washout, mosaic pattern of enhancement, internal hemorrhagic component, well defined tumoral capsule, and findings indicative of underlying alcoholic liver disease, the tumor was radiologically diagnosed as a HCC.
Subsequently, right hepatectomy was decided to perform as a curative treatment. In addition, conservative treatment such as administration of hepatotonic agents and antibiotics to treat underlying alcoholic liver disease and cholangitis had been done for 1 month before operation. The laboratory test performed just before the operation revealed that serum levels of total bilirubin (0.8 mg/dL), direct bilirubin (0.4 mg/dL), aspartate aminotransferase (51 IU/L), alanine aminotransferase (40 IU/L), gamma-glutamyl transpeptidase (101 IU/L), alkaline phosphatase (60 IU/L), and lactate dehydrogenase (441 IU/L) and CA19-9 (14.7 U/mL) were markedly improved. Right hepatectomy was performed at 6 weeks after admission, according to the initial treatment plan.
The gross surgical specimen showed that a 6 cm brown to reddish lobulated mass with both solid and cystic portions was surrounded by whitish fibrous capsule, and included hemorrhagic necrosis, necrotic debris and internal whitish septa (). And also, multifocal irregular surface of the liver was demonstrated which was suggestive of chronic alcoholic liver disease. Microscopic examination revealed extensive areas of coagulative necrosis, infiltrations of chronic inflammatory cells including lymphoplasma cells and some eosinophils, fibrosis and focal areas of hemorrhage and hemosiderin pigments (). These histologic features resulted in the diagnosis of hepatic abscess and chronic alcoholic liver disease. He had an uneventful postoperative course, and has been alive for 59 months after surgery.


A 25-year-old male with schizophrenia developed a hypomanic episode 1 week after the initiation of blonanserin at 8 mg/day. He was first diagnosed with schizophrenia in 2003 at the age of 16 years with symptoms of auditory hallucination and delusions of reference and persecution and has been on various antipsychotic medications for 9 years. The patient did not achieve symptomatic remission, and his course was characterized by intermittent worsening and softening of the referential delusion that others were talking behind his back. He has been on a stable regimen of olanzapine 5-20 mg (final dose 10 mg), aripiprazole 15 mg, clonazepam 0.5 mg, and benztropine 1 mg since 2010. In February 2011, he experienced increased appetite and weight gain; thus, fluoxetine (20 mg) was added to his regimen for 7 months until September 2011 with no mood change.
In March 2012, the patient's ideas of reference began to increase and in May, he complained that his neighbors said bad things about him and that the rumors had spread throughout the community. Following this exacerbation, blonanserin 8 mg/day was added to his regimen. Ten days later, the intensity of his delusions decreased, but he developed new symptoms indicative of a hypomanic episode, such as euphoria, elated mood, grandiosity, and hyperactivity. The patient expressed his plan to become a psychiatrist and he wrote poems for several hours per day with the intention of becoming a professional poet. He had a score of 19 on the Young Mania Rating Scale (YMRS). One week later blonanserin was withdrawn and the episode ended 4 weeks after discontinuation (YMRS score: 4).


A 38-year-old male developed a manic episode after the initiation of blonanserin treatment. He first visited our outpatient department in May 2012 with complaints of persecutory and referential delusions that had developed 2 weeks earlier. He claimed that his colleagues at work bullied him and had hatched a plot to get him fired. His family reported that he accused his brother of being a spy from the company and called his father a North Korean spy. Furthermore, he showed bizarre behaviors such as wandering and picking up garbage. Schizophreniform disorder was suspected, and he was initially administered blonanserin 6 mg, alprazolam 0.375 mg, and benztropine 2 mg/day. Shortly after receiving the medication, he displayed a slightly argumentative attitude and increased activity, but his behavior was considered to be within the normal range. Two weeks later, at a dose of 8-mg blonanserin, the patient reported that his persecutory and referential delusions were no longer valid, and he displayed a slight euphoria and increased psychomotor activity that met the criteria for a hypomanic episode.
Two weeks later, at 16 mg/day blonanserin, the patient showed full-blown manic symptoms including talkativeness, hyperactivity, irritable and expansive mood, and flight of idea. He was considered for hospitalization but refused. He scored 27 on the YMRS. Valproate 500 mg/day was added to his regimen, and the dose of blonanserin was reduced to 12 mg/day. His manic episode continued for 2 additional weeks; however, when blonanserin was discontinued his manic symptoms disappeared within 2 weeks (YMRS score: 2).


A 33-year-old male presented to our institution with a left submandibular abscess secondary
to dental infection. He had a three-day history of left jaw pain, lethargy, fever and
decreased PO intake. He had no significant medical history. Examination findings included
moderate to severe trismus and marked left submandibular swelling, and his 38th tooth was
tender on palpation. His initial WCC was 18.7 with a neutrophilia and CRP was 153. Contrast
CT demonstrated localized abscess in the left submandibular space.
Ultrasound reported a small hypoechoic mass on the medial aspect of the left submandibular
gland. He was admitted and commenced on IV ceftriaxone, metronidazole and dexamethasone. His
symptoms and biochemical markers improved over the following three days and he was
discharged home on oral cephalexin and metronidazole. Drainage was initially considered
unnecessary due to the small size of the collection and excellent clinical response.
Forty-eight hours after discharge, the patient re-presented to the same institution with
right-sided weakness, confusion and sudden expressive aphasia. On examination, he was
afebrile, had a BP of 159/92 and PR of 70. His GCS was 11, and he had a right upper motor
neuron seventh nerve palsy and had decreased power in his right arm with hemi-sensory loss.
His WCC was 14, CRP 45 and ESR 61. A non-contrast head CT scan was normal.
A CT angiogram of the head and neck was performed which demonstrated a tight stenosis
(greater than 90%) involving the mid-left internal carotid artery over a distance of
approximately 2 to 3 mm ().
A 1 cm-segment of thrombus was noted in the superior branch of the left MCA at the level of
the anterior end of the Sylvian fissure and an abscess was seen involving the floor of mouth
and submandibular space ().
MRI showed acute left inferior frontal cortical infarct in the distribution of the left MCA
(). It also showed further
enlargement of the left submandibular space fluid collection, consistent with abscess
formation with a focus of gas.
The patient was taken to theatre for a left transcervical incision and drainage of abscess
and removal of the left submandibular gland. The findings at operation included a large left
submandibular gland, adherent to the surrounding fascia. There was a large quantity of
necrotic tissue surrounding the gland with fistula extending to the gingival mucosa adjacent
to the 38th tooth. The tooth was removed.
Subsequent MRA of the neck performed one week postoperation demonstrated a stable stenosis
of the distal left ICA at the level of C1 and, to a lesser extent, the right ICA. There was
resolution of the left-sided submandibular fluid collection and the associated inflammatory
change. ECHO/Holter monitor screen, thrombophilia screen and cholesterol studies were
unremarkable.
Two months later, the patient was admitted for an elective left carotid endarterectomy via
a combined transcervical and mandibular osteotomy approach. The findings at operation were a
small internal carotid artery with an obvious stenosis, with intimal hyperplasia, 1 cm away
from the base of the skull. His postoperative progress was complicated by a 10th, 11th and
12th cranial nerve paresis secondary to postoperative oedema. This improved throughout his
postoperative stay with some mild deficit remaining at discharge.


A 54-year-old primiparous woman presented with a 6-month history of mild abdominal pain and distension of the right portion of the abdomen. Her family history was not contributory. Her past surgical history included a total thyroidectomy for papillary carcinoma followed by radioiodine therapy 3 years before.
Physical examination revealed a slightly tender, ill-defined mass over the left abdomen. The remaining systemic examination did not reveal any coexistent lesion. Laboratory tests, including complete blood count, chemistry profile, urinalysis and chest X-ray, were all within normal limits. Serum tumor markers were negative.
An abdominal ultrasound (US) documented the presence of a coarse, partially cystic mass in the right retroperitoneal region. Abdominopelvic CT and MRI revealed a large unilocular cystic mass measuring 11 cm with an enhancing solid portion and partial water content, located in the retroperitoneal space adjacent to the lower pole of the right kidney, with wall calcifications (fig. ). There was no evidence of extracystic extension or distant metastasis. On suspicion of a hydatid cyst, serological tests were performed and yielded negative results. A gynecological examination, including hysteroscopy and Pap smear, was also performed. It was negative for utero-adnexal pathology.
The patient underwent laparotomy with subcostal transverse incision. On opening the peritoneal cavity, ascitic fluid was found and sampled. Cytological examination of the fluid did not reveal the presence of malignant cells. Abdominal cavity exploration revealed no abnormal findings in the liver, stomach, large bowel and spleen, and the uterus, ovaries, fallopian tubes and appendix were all normal. Sequential exploration of the small intestine revealed the presence of an exophytic lesion about 1 cm in diameter, which was removed and whose definitive histological examination was compatible with fibroma of the small intestine. A large, encapsulated cystic mass was found in the right retroperitoneum, below the lower pole of the right kidney that was displaced posteriorly and cranially to the mass. The cecum, right colon and right colic flexure were displaced medially to the mass. The mass was dissected from the surrounding structures. A fragment of the cystic wall was sent for extemporaneous histological examination and it was compatible with a fibrous wall denuded of lining epithelium. Complete excision of the cyst was performed. The postoperative period was uneventful, and the patient was discharged home 5 days after surgery.
The gross pathological findings showed a 12 × 10-cm cystic mass with a smooth surface. The cystic wall measured 0.3 cm at maximum thickness. The tumor was composed of malignant epithelial and stromal components. Microscopically, the fibrous wall of the cyst delimited an extensively necrotic lesion containing chronic inflammation, calcification, cholesterol crystals and hemorrhagic extravasation. On hematoxylin and eosin staining, the entire tumor showed cellular whorls dispersed amidst pale sarcomatous stroma. The cells in whorls were round to short, spindle shaped and had moderate mitotic activity. The sections, however, revealed whorled glandular areas.
A panel of immunohistochemical tests was performed: whorled glandular areas were positive for cytokeratin (CK19) and negative for vimentin, confirming their epithelial nature; they were also negative for CK20 and calretinin. The stromal component of the tumor, highlighted on vimentin stain (vimentin), was positive for CD10. No immunoreactivity to estrogen and progesterone receptors was found. The presence of necrosis, cytonuclear atypia and immunophenotypic features were compatible with PRMA (fig. ).
Six courses of adjuvant chemotherapy were administered with carboplatin at a dose of 5 AUC and taxol 175 mg/mq/ev. A follow-up abdominal CT, performed 9 months later, was negative for tumor recurrence. Written informed consent was obtained from the patient for publication of this case report and all accompanying images.


The patient was a 41 year-old, unemployed, divorced male, with high school diploma, residing in Kerman (one of the central Iranian cities). According to his biography concerning sexual background, he experienced his first ejaculation in 14 years old by masturbation and frequently masturbated through the age of 22 years. He married in 22 and experienced frequent intercourses with his wife from which he gained pleasure and it was his preferable method. He commenced drug abuse from the age of 20 years and was involved in different types of substances. Her wife left ten years ago. The patient has started crystal (glass) administration through insufflations since seven months ago and has been taking crystal only since. Simultaneous with crystal abuse, wearing women’s underwear has been the only medium through which the patient reached sexual orgasm. The desire rose with increasing crystal administration. He has masturbated since, wearing women’s underwear, as a preferable method of sexual arousal. Subsequent to crystal (glass) dependency, he has had no desire to have intercourse with women. No instances of paraphilia were seen in his previous records, he is not suffering from any specific psychiatric disease nor is he taking specific medications.


This was a case report of a 38-year-old male patient who was admitted to neurosurgery with a history of headache and neck pain since 2 months. No history of antecedent head trauma, febrile illness, photophobia, seizures or previous diagnosis of any malignancy was present. He didn′t have lymphadenopathy or hepatosplenomegaly. Neurologic examination was not revelatory as no abnormality was found. His peripheral smear examination was normal. He was negative for human immunodeficiency virus (HIV).
MRI of the brain revealed a well-defined lobulated focal mass measuring 4.5 cm × 3.6 cm × 3.5 cm with extensive adjacent white matter edema in right parieto-occipital lobe causing mass effect on right lateral and third ventricle and basal ganglia region with contralateral midline shift of 9 mm. On T1-weighted images mass was isointense to gray matter []. On T2-weighted and fluid-attenuated inversion recovery images, it appeared iso to mildly hyperintense []. There was mild restricted diffusion. The lesion showed intense homogeneous enhancement on post contrast study [Figure and ]. Small area of central necrosis was seen within the mass. MR spectroscopy revealed an elevated choline and lipid lactate. MRI findings were concluded as glioma.
Patient underwent craniotomy for micro-neurosurgical complete resection by aspiration of the suspected glioma. Squash preparations were made from tissue received for frozen section and stained with hematoxylin and eosin. Monomorphic lymphoid tumor cells were seen without any fibrillary background []. The cells had large nuclei with prominent nucleoli and scant to moderate cytoplasm. Further, lymphoid cells having a blast like appearance with cytoplasmic vacuoles were recognized better on Leishman stain []. Tingible body macrophages and lymphoglandular bodies were seen. The frozen sections showed the tumor cells in sheets, but the cytologic details were not clear.
With intraoperative diagnosis of lymphoma infiltration on squash smears, on table bone marrow aspiration was done from the sternum to rule out hemopoietic malignancy in the marrow. The bone marrow smears showed normal marrow cells.
A squash unstained smear was further studied with immunocytochemistry for CD45 (with a satisfactory positive control). The smears showed cytoplasmic CD45 positivity in intact tumor cells [].
The paraffin sections revealed the typical lymphoma cells displaying perivascular pattern and invading the brain [Figure and ]. The starry sky appearance was also seen []. The diagnosis of PCNSL was conferred. The immunohistochemical studies showed CD20 positivity [] in lymphoma cells while they were negative for CD3, CD10, Bcl2, Bcl6 [Figure –] and CD99. CD3 positive reactive T cells were seen amidst the lymphoma cells []. The negativity of lymphoma cells for CD10, Bcl2, Bcl6 and positivity to CD20 indicate the non-germinal center B-cell phenotype.
The final diagnosis of diffuse large B cell primary CNS lymphoma (DLBCL) was given. Post-operative contrast CT showed complete removal of the lesion and the patient received chemo and radiotherapy. MRI after 2½ years follow-up showed no evidence of recurrence.


A 67-year-old male presented to the emergency department with a 10-day history of left-sided testicular pain. Symptoms were of gradual onset, and were initially treated with antibiotics from his general practitioner. There was no history of trauma, injury, pyrexia or voiding symptoms. Urine dipstick test was negative.
Examination revealed an acutely tender left groin and left testicle with a markedly enlarged, erythematous left hemi-scrotum. Antibiotics were changed to doxycycline from ciprofloxacin, and the patient was referred for an urgent ultrasound which was performed the following day. Full blood count revealed white cell count of 8.2×109/L with a C-reactive protein (CRP) of 48 mg/L. All other blood tests were normal.
An ultrasound scan revealed no vascularity within the left testicle. The patient was taken for scrotal exploration under general anaesthetic. This revealed a torted left testicle with no pus present. A left orchidectomy was performed along with right-sided testicular fixation. The patient had an uneventful post-operative course, and was discharged home the following day.


A 51-year-old male received a penetrating stab wound to the abdomen and underwent an immediate emergency laparotomy. Findings at laparotomy were splenic hilar injury, a through-and-through enterotomy of the stomach and transection of the left renal vein. The stomach was repaired, a splenectomy was performed and ligation of the left renal vein was undertaken with temporary clamping of the aorta. The abdomen was packed, and a temporary topical negative pressure closure of the abdomen was undertaken. The patient required 24 units of packed red cells, 20 units of fresh frozen plasma and 4 units of platelets to achieve resuscitation. Twenty-four hours later, the patient was returned to the operating room for a relook laparotomy. The transverse colon had patchy ischaemia, so a damage control resection of the colon was undertaken. Minor bleeding points on the abdominal wall were controlled. The topical negative pressure closure was reapplied. On the fourth postoperative day, a second relook laparotomy was undertaken. The colon was anastomosed. The abdomen was unable to be closed, and the topical negative pressure dressing was reapplied. On the ninth postoperative day, a further attempt was made to close the abdomen but this was not successful. The topical negative pressure closure was reapplied.
On the 11th postoperative day, a component separation was performed. The topical negative pressure dressing was removed leaving the defect shown with the anterior sheath retracted away from the midline (see Fig. . The overlying skin was released bilaterally from the abdominal wall, and the anterior rectus sheath was released. To achieve this, the external oblique was incised at Point A, and the incision taken high to the costal margin and as low as possible to allow maximal release. The internal oblique is then exposed (Point B), and the released edge is visible at Point C (see Fig. ).
A posterior sheath release was then performed to provide medial movement of the sheath, so that it could be closed in the midline (see Fig. . A 25 × 25-cm biologic mesh (Strattice™ Reconstructive Tissue Matrix,–LifeCell Corp.) was rotated to a diamond shape and secured in the pre-peritoneal space, to the lateral fascia (see Fig. . The rectus muscle was closed, and the component separation allowed for the anterior sheath to be closed in the midline (see Fig. . Primary skin closure was undertaken, and the patient returned to the ward.
On the 16th postoperative day, he suffered a witnessed ventricular fibrillation cardiac arrest. He was resuscitated and subsequently anticoagulated. The following day a computed tomography scan revealed a large abdominal wall haematoma anterior to the rectus sheath. He returned to theatre on the 17th postoperative day. The fascia was found to be intact, and the haematoma evacuated. Topical negative pressure was reapplied. On the 21st postoperative day, the skin was reclosed. He returned to the ward and was discharged on the 49th postoperative day, following implant of a cardiac defibrillator, with an intact wound (see Fig. ).


A 30-year-old male patient was suffering from herpes labialis, for which he consulted local physician and was prescribed tablet carbamazepine 200 mg two times a day, tablet acyclovir dispersible 200 mg five times a day, tablet pregabaline 75 mg twice a day and tablet methylcobalamine 500 μg thrice a day. He improved initially from herpes labialis lesions but after 10 days, developed lesions in oral mucosa which spread to the face, upper extremities, trunk and ultimately all over the body for which he consulted the same doctor. He was treated with steroids, cetirizine, and erythromycin and the initial treatment was continued as such. He was suspected to be having reactivation of the herpes virus or drug reaction. But the severity of the lesion was pointing more toward drug allergic reaction. Moreover he was continuing the tablet acyclovir in routine prescribed doses, so possibility of virus reactivation was very minimal. The condition of the patient worsened after 5 days and was referred to dermatology department of Dhiraj Hospital, a tertiary care teaching rural hospital. On cutaneous examination, multiple hyper-pigmented lesions and some target lesions were present all over the body, creamy-curdy lesions over the tongue, oral mucosa, and palate []. On ophthalmic examination, multiple scaly lesions in lids, gross lid swelling, purulent discharge, congested conjunctiva, ulceration in conjunctiva, and lower fornix. The patient was suspected of having AHS due to drug allergic reaction. Blood investigations revealed WBC count 6800/mm3, neutrophils 78%, lymphocytes 15%, urine albumin 4+ and SGPT 66 IU/l, suggestive of liver and kidney involvement.
Carbamazepine was stopped and the patient was treated with injection dexamethasone 8 mg and injection pheniramine maleate 4 mg intramuscularly once a day for 3 days, tablet azithromycin 500 mg once a day for 6 days, injection ranitidine i.v. two times a day. He improved after 5 days of treatment and his SGPT levels returned to normal.


A 41-year-old male patient had complaints of skin lesions over the trunk with shooting pain at the back, radiating to the front. He was diagnosed as having herpes zoster by a local physician and treated with tablet acyclovir 800 mg five times a day, tablet mecobalamine 500 μg thrice a day, tablet folic acid 5 mg once a day and tablet carbamazepine 300 mg once a day.
The lesions of herpes zoster subsided within 5 days of treatment but on the 7th day, he developed new reddish skin lesions over the trunk. He discontinued all medications and the new lesions subsided within 2 days. The patient had again consulted the doctor for persisting shooting pain and was prescribed tablet carbamazepine 300 mg once a day, tablet fexofenadine 60 mg once a day, tablet clonazepam 0.5 mg once a day and tablet aceclofenac 100 mg daily. But after 9 days, reddish-colored lesions reappeared over the trunk spreading to upper and lower limbs so he was referred to department of skin and venereal diseases of Dhiraj Hospital, Piparia, Vadodara. On examination, the lesions were flat, reddish in color with well-demarcated margin present all over the body []. Laboratory investigations were sent immediately to rule out internal organ involvement. Investigations showed hemoglobin (Hb) 13.4 gm%, total leukocyte count (TLC) 5300/mm3, neutrophils 86%, lymphocytes 09%, monocytes 02%, eosinophil 03%, basophil 00%, random blood glucose (RBS) 128 mg%, blood urea 21mg%, serum creatinine 0.9 mg%, SGPT 50 IU/L, SGOT 23 IU/L and serum bilirubin 0.6 mg%. The level of SGPT was borderline high suggesting of mild liver involvement. Carbamazepine was discontinued and he was given injection dexamethasone 8 mg and injection pheniramine maleate 4 mg intramuscularly once a day for 3 days. After 4 days of treatment, lesions subsided without any sequelae and liver enzymes came back to normal.


A 50-year-old male patient was suffering from trigeminal neuralgia and was on tablet carbamazepine 300 mg once a day for last 1 week. He developed reddish-colored skin lesions all over the body, spreading into oral cavity with fever after 8 days of starting the treatment. He was suspected of having drug reaction and referred to department of skin and venereal diseases of Dhiraj hospital, Piparia. On clinical examination, skin lesions were present all over the body with itching and fever []. The lesion appeared first in oral cavity and spread to the trunk and extremities. He was diagnosed as having maculopapular eruptions due to carbamazepine. Blood investigations revealed WBC count 6300/mm3, neutrophils 78%, lymphocytes 15%, urine albumin 3+ and SGPT 60 IU/l, suggestive of liver and kidney involvement. Tablet carbamazepine was stopped immediately and he was given oral prednisolone 30 mg once a day for 1 week and tapered by 5 mg per week along with injection pheniramine maleate 4 mg intramuscularly. He was on regular follow-up with good improvement in his clinical condition.


A forty three-year-old gentleman presented to us after eight days of suicidal ingestion of one tube (35 g) of rodenticide paste (Ratol, containing 3% YP). He had history of daily alcohol intake of about 50-60 grams for the past 5 years without any history suggestive of underlying chronic liver disease or cirrhosis. He had no past history of jaundice. He denied history of ingesting any other drugs or toxins in the recent past.
After self-limiting nausea and vomiting on the day of ingestion, he remained asymptomatic for 4 days. He observed dark-coloured urine and generalized itching on day 5 after ingestion. Pruritus was severe and disturbing his sleep. After being detected to have jaundice, by his local physician, he was referred to our institute on day 8 for further investigation.
Examination revealed clinical jaundice with excoriation marks all over the body secondary to severe pruritus. His liver was palpable 4 cm below the costal margin and was firm in consistency. Detailed examination failed to show any stigma of underlying chronic liver disease, splenomegaly, or ascites. Cardiovascular and nervous system examination was observed normal and there were no bleeding diathesis. He was not in hepatic encephalopathy as assessed by normal mini-mental state examination score (MMSE). He reported the pruritus as 8 on a visual analogue score of 0 to 10.[]
Evaluation showed a normal hemogram, with bilirubin of 22 mg/dL (0.2-2.0 mg/dL) with a direct fraction of 18 mg/dL, serum alanine amino transferase (ALT) of 58 IU/L (0-40 IU/L), aspartate amino transferase (AST) level of 100 IU/L (0-40 IU/L), alkaline phosphatase (AP) value of 277 IU/L (50-150 IU/L), and gamma glutamyl transferase (GGT) of 140 IU/L (20-40 IU/L). Prothrombin time was prolonged by 4 seconds, which normalized after vitamin K injection. Electrocardiogram and renal functions were normal.
Other causes of liver disease were ruled out by negative HbsAg, anti-HCV, IgM anti-HEV, and IgM anti-HAV serology. His serum ceruloplasmin and ferritin levels were normal. Ultrasound scan of the abdomen was unremarkable, except for a fatty liver. Upper Gastrointestinal (GI) endoscopy showed no varices. After normalization of prothrombin time, liver biopsy was done on day 14 of ingestion, which showed enlarged hepatocytes, giant cell transformation, intrahepatic cholestasis and spotty hepatocyte necrosis with infiltration by lymphocytes and few eosinophils []. Histopathology has not shown any evidence of architectural distortion of hepatic lobule, fibrosis, cirrhosis, or regenerating nodules.
The patient never developed any clinical signs of acute liver failure. Pruritus was controlled with emollients, ursodeoxycholic acid, cholestyramine and sertraline. Liver function tests normalized and pruritus also improved over the next 3 weeks. Patient is currently asymptomatic after 4-months of follow-up.


An eight-year-old boy was visited in endocrinology clinic of the Kashan University of Medical Sciences. He was 15.5 kg in weight (3.5 standard deviation below mean) and his height was 92 cm (7 standard deviation below mean). Based on the available documents, he weighed 3.65 kg (50th centile) at birth, with a crown to heel length of 47 cm (10th centile). He had shown abdominal distention and vomiting in his neonatal period and eventually diagnosed asHirschsprung disease that was confirmed using biopsy.
In physical examination he had severe short stature, disproportionate short-limbed dwarfism, short and puffy hands, and genu varum deformity. The sternum was abnormally prominent. Hyperextensibility of the joints was not detected.
His scalp hairs, eyebrows, and eyelashes were fair, very fine and sparse (). While no nail dysplasia or dental abnormality was found, intellectual development was normal for his age and no clinical evidence of immunodeficiency was observed. However he developed hypothyroidism in the age of 6 years. A 3-mm kidney stone was detected when he was 3 years old that enlarged to 8mm in size at the age of 8 and treated by lithotripsy.
Skeletal X-rays revealed typical metaphyseal dysostosis, including widening, cupping, and defective mineralization in the metaphysis of the tubular bones (). Vesicouretral reflux was detected in his voiding vesicocyctogram. Complete blood count, electrolytes, and renal function tests were normal. Sterile pyuria was found in several samples of urinalysis that may be as a result of the vesicouretral reflux or renal stone. Microscopic stool examination detected no fat globules. The patient had been assessed for celiac disease and cystic fibrosis during his infancy that was negative.


A 47-year-old woman visited our hospital in January 2003 because of a painful right breast mass with skin dimpling. On initial evaluation, chest CT revealed the presence of a heterogeneous enhancing breast mass measuring 9 cm with skin invasion and multiple conglomerated lymph nodes in the right axillary area. In addition, multiple metastatic pleural masses with malignant pleural effusion and mediastinal lymph node enlargement were also observed. A bone scan showed increased uptake in the sternum and right sixth anterior rib. There was no evidence of liver metastases on the initial CT scan (). Fine-needle aspiration cytology of the breast mass showed atypical malignant cells. Accordingly, she was diagnosed with stage IV right breast cancer with multiple pleural and bone metastases. She subsequently received palliative chemotherapy with docetaxel and epirubicin. Follow-up CT scan performed after four cycles of chemotherapy showed partial regression of the breast mass and multiple metastatic masses in the pleura and axillary area. She then underwent palliative total mastectomy of her right breast because of an ulcerated skin lesion. Pathological examination showed invasive ductal carcinoma with nuclear grade 2 and lymphatic and perineural invasion. Immunohistochemistry studies showed positive staining for the estrogen receptor (ER) protein, progesterone receptor protein, and human epidermal growth factor receptor 2 (HER2) (score 3). She received two additional cycles of chemotherapy with docetaxel and epirubicin. A follow-up CT scan showed stable disease and she then started taking tamoxifen (20 mg daily) in July 2003.
Two years later, in November 2005, a surveillance breast ultrasound showed an irregular circumscribed mass measuring 1 cm on theright mastectomy site. The patient underwent a wide local excision and pathology confirmed an invasive ductal carcinoma. Although previous metastatic lesions showed stable disease, a new chest wall lesion had developed; therefore, her treatment was switched from tamoxifen to the non-steroidal aromatase inhibitor anastrozole. At that time, she was postmenopausal, based on her serum follicle-stimulating hormone levels. In January 2007, a bone scan showed new increased uptake in the right second rib, the third anterior rib, and the right acetabulum. She had progressive disease of the bone; therefore, capecitabine (an oral prodrug of 5-fluorouracil; 2,500 mg/m2/day) was started (two weeks on, one week off). After nine cycles of chemotherapy, she had stable disease and was off chemotherapy for approximately four years with no evidence of progression.
However, follow-up CT scan performed in June 2011 showed multiple newly developed peripheral enhancing nodules in the liver (). She received six cycles of chemotherapy until November 2011, and CT scan showed that her metastatic hepatic lesions were stable. Since then, her chemotherapy has been discontinued because of grade 4 neutropenia and osteomyelitis of the mandible. Four months later, follow-up CT scan showed ill-defined heterogeneous enhancing lesions in the entire liver with surface nodularity. The liver had a nodular contour consistent with cirrhosis, as well as moderate ascites (). F18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET-CT) also showed disseminated and innumerable lesions throughout almost the entire liver with increased FDG uptake (maximum standardized uptake value [SUVmax], 7.3), suggesting diffuse liver metastasis (). Palliative chemotherapy with docetaxel and epirubicin was started again. Endoscopy showed grade 3 large esophageal varices with a red color sign. She denied a history of liver disease, alcohol use, and risk factors for viral hepatitis. Serologies for viral or autoimmune etiologies of cirrhosis were negative and the serum level of alpha-fetoprotein was normal. An ultrasound-guided liver biopsy was performed in order to confirm the cause of the cirrhotic changes. Pathological examination showed that the hepatic parenchyma was diffusely infiltrated by poorly differentiated carcinoma cells. Hepatocytes were almost replaced by carcinoma cells and extensive fibrosis between clusters of cancer cells was observed (). Immunohistochemical staining was positive for gross cystic disease fluid protein-15, MOC 31, ER, and HER2 (score 3), but negative for hepatocytes (). Follow-up PET-CT showed progression of liver metastases with increased FDG uptake (SUVmax, 9.0) since the scan performed in June 2011. Therefore, we concluded that the hepatic metastases from breast cancer had progressed and led to cirrhotic changes in the liver.
Subsequently, her chemotherapy regimen was changed to trastuzumab and docetaxel. However, bleeding of esophageal varices occurred on the sixth day after initiation of chemotherapy, and hepatic failure progressed gradually. Therefore, the patient decided to discontinue chemotherapy.


A 69-year-old man visited our clinic in July 2010 with complaints of blurred vision and night blindness in both eyes. Our initial examination found that his best-corrected visual acuity in decimal units was 1.0 in the right eye and 0.6 in the left eye. His refractive error was +3.0 D and +3.5 D for the right eye and left eye, respectively. Full-field rod ERGs were absent and cone-rod mixed responses were the negative type, indicating ON bipolar cell dysfunction (). From the symptoms and ophthalmic examination, he was suspected of having PR.
A general physical examination revealed small cell carcinoma of the lung and Western blot of the patient’s serum showed autoantibodies against TRPM1. We diagnosed this patient with melanoma-associated retinopathy and retinal ON bipolar dysfunction due to TRPM1 autoantibody and have reported his findings in more detail elsewhere. After diagnosis, chemotherapy was started using cisplatin and etoposide combined with radiation for the lung cancer in August 2010. The patient achieved a complete remission, but underwent cranial irradiation in March 2011 to prevent brain metastasis.
We have followed this patient ophthalmologically for more than 2 years since his initial visit and his symptoms have not changed. His best-corrected visual acuity decreased slightly to 0.8 in the right eye and 0.5 in the left eye due to progression of cataracts. His visual field has not constricted and his ERGs have not changed (see ). Rod responses were nonrecordable in 2010 and 2012. The amplitudes for a-wave of cone-rod mixed maximum response were 240 μV and 220 μV in 2010 and 2012, respectively, and those for b-wave were 150 μV and 120 μV, respectively. The amplitudes of cone response were about 25 μV for the a-wave and 50 μV for the b-wave at both times in 2010 and 2012. The amplitudes of the 30 Hz flicker ERGs were 14 μV in 2010 and 11 μV in 2012. The difference in the ERGs was most likely within the error of measurement and not due to differences in retinal function. However, fundus photographs taken in October 2012 showed diffuse choroidal hypopigmentation in both eyes (). We also found that the choroidal vessels were more visible than those obtained at the initial examination ().
Spectral domain optical coherence tomography (Heidelberg Engineering, Heidelberg, Germany) showed a slight reduction in retinal thickness during the 2 years of follow-up, but the morphological architecture of the retina appeared almost normal at both times (). Consistent with the choroidal hypopigmentation of the fundus, spectral domain optical coherence tomography showed a marked decrease in choroidal thickness over the 2-year period (, arrowhead). Subfoveal choroidal thickness in the right eye measured manually was 250 μm in 2010, 180 μm in 2011, and 110 μm in 2012, and for the left eye was 380 μm in 2010, 230 μm in 2011, and 100 μm in 2012.


A previously healthy 8-month-old boy presented with a 3×3 cm mass in the right lower leg, which was surgically excised. Pathological analysis confirmed the diagnosis of infantile fibrosarcoma and the surgical margin was not clear. His parents refused reexcision but accepted monitoring by regular imaging. However, a mass was detected again in the same area two years after surgery. Therefore, the patient then underwent complete excision, and recurrent sarcoma was confirmed histologically. After the second resection, the patient had monthly follow-up and no evidence of disease was observed on an ongoing one year.
A surveillance whole-body fludeoxyglucose positron emission tomography scan conducted 3 years after the initial presentation demonstrated a recurrent mass in the right lower leg, one micrometastatic lesions at the left proximal fibula and another macrometastasis in the 4th lumbar vertebral column, respectively. Lumbar spine magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion of 1.6 cm in the posterior aspect of the 4th lumbar vertebral column without spinal canal compression (). To preserve right lower leg, debulking surgery of the recurrent lesion was performed, and the histological features were found to be consistent with recurrent sarcoma with rhabomyosarcomatous differentiation. Two weeks after the surgery, the patient started to receive chemotherapy with a VAC regimen composed of vincristine (1.5 mg/m2), dactinomycin (0.045 mg/kg), and cyclophosphamide (2.2 g/m2) for a total of 44 weeks treatment course.
Given the potential risk of spinal pathologic fracture and myelopathy, radiotherapy was chosen to treat the 4th lumbar vertebral metastatic lesion. Cyberknife® hypofractionated radiotherapy was decided due to the solitary nature of the metastasis and fewer sessions are needed, which would decrease the frequency of general anesthesia for radiotherapy. The patient was immobilized in a vacuum mattress. Fused enhanced computed tomography (CT) (Accuray) and MRI scans were used in the treatment planning. The delineation of the gross tumor volume (GTV) and the organs at risk was carried out on the planning CT. Given the potential risk of kyphosis and scoliosis by asymmetric irradiation of the spine, the whole involved vertebra was contoured as the GTV. Clinical target volume was equal to the GTV and no margin was added to obtain the planning target volume (PTV), which was 8.84 mL. No fiducial or other markers were implanted to localize the irradiated area. A total of 26 Gy was prescribed to the 75% isodose line, covering 92.64% of the PTV with a maximal spinal cord dose of 744.26 cGy (). Cyberknife® stereotactic body radiotherapy was performed on the 15th, 16th, 19th, and 20th of October 2009 with a mean procedure time of 83 min (range 56 min to 128 min). The radiotherapy course was conducted in a manner concomitant with the first cycle of chemotherapy. During the radiotherapy, immobilization was aided by general anesthesia, and X-sight spine tracking (Accuray Inc.) was used to minimize setup error.
The patient tolerated the anesthesia and immobilization well and remained still during the radiotherapy. Up to the date of this report, i.e., 33 months after radiotherapy, the patient remains neurologically stable and 23% reduction in tumor volume was achieved on the surveillance MRI scans (). No acute radiation toxicity was found.


A 66-year-old male was referred to our center by his primary care physician because of the increase of proteinuria. He was diagnosed with diabetes at the age of 50 years but remained untreated. At the age of 60 years, he started voglibose at the diabetes center of our hospital. At the age of 66 years, urinary abnormality of protein 3+ and occult blood 1+ was pointed out, combined with simple diabetic retinopathy and a serum creatinine level of 0.96 mg/dl. He was referred to our center for further investigation, and a renal biopsy was performed. One of 11 glomeruli showed global sclerosis, and the other glomeruli showed massive foam cell infiltration in the glomerular capillaries and mildly expanded mesangium (fig. ). These foam cells immunohistochemically stained positive for cluster differentiation 68 (CD68) (not shown). Transmission electron microscope images also revealed foam cell infiltration mainly located in the capillaries containing scattered lipid droplets and lamellar bodies in their cytoplasm (fig. ). The glomerular basement membrane (GBM) showed mild thickening, and nodular lesions were formed in some glomeruli. The interstitium and tubules showed mild fibrosis and atrophy (25% of the cortical region), and a moderate degree of arteriolar hyalinosis was also found. No interstitial foamy cells or foamy tubular degeneration was found. Immunofluorescence study revealed no significant glomerular deposits. The patient's lipid profile showed type III HLP (table ). Apolipoprotein phenotype analysis showed a homozygosity of apoE2. A further DNA sequence analysis was performed as described previously [] and showed a nucleotide substitution of cysteine for arginine at codon 158 (Arg158Cys) of the apoE gene and no other mutations. Eicosapentaenoic acid was added to his statin therapy, but he progressed to end-stage renal failure and was started on dialysis 8 years after the diagnosis.


A 58-year-old male was referred to our center by his primary care physician due to nephrotic syndrome. He had massive proteinuria (7.38 g/day) and hypoalbuminemia (1.9 g/dl). He had been treated for diabetes since the age of 50 years and had been started on insulin therapy at the age of 55 years. He had been well-managed (HbA1c, 5.3%), although a pre-proliferative retinopathy and mild renal dysfunction (sCr, 1.38 mg/dl) became evident. His renal biopsy revealed 5 of 22 glomeruli with global sclerosis. The residual glomeruli showed massive foam cell infiltration in the glomerular capillaries and severely widened mesangium (fig. ). These foam cells stained positive for CD68 (not shown). Transmission electron microscope images also revealed foam cell infiltration mainly located in the capillaries containing scattered lipid droplets and lamellar bodies in their cytoplasm (fig. ). The glomerular tufts were enlarged with hyalinosis, mesangiolysis and microaneurysmal formation, which were consistent with diabetic glomerulosclerosis. The interstitium and tubules showed moderate fibrosis and atrophy (50% of the cortical region). The foamy change was seen in some tubular epithelial cells, but no interstitial foamy cells were found. The interlobular arteries showed a moderate degree of fibrous intimal thickening, and the arterioles also showed a moderate degree of subendothelial hyalinosis. Immunofluorescence studies revealed nonspecific linear IgG deposits along the GBM; however, no significant deposit was found by electron microscopy. The patient's lipid profile showed type III HLP (table ). He had an apoE2 homozygote phenotype and the Arg158Cys genotype with no other mutations. Three years after the diagnosis, he progressed to renal failure and was started on hemodialysis.


A 78-year-old male with nephrotic syndrome was referred to our center by his primary care physician. The patient had a history of hypertension since the age of 45 years, hypothyroidism since the age of 68 years and diabetes mellitus since the age of 70 years. He also had a history of percutaneous coronary intervention for coronary artery disease and femoropopliteal bypass surgery for peripheral arterial disease at the age of 74 years, and a history of Wallenberg syndrome since the age of 77 years. He was being treated with an angiotensin II receptor blocker, a calcium-channel blocker, pitavastatin, aspirin and levothyroxine sodium hydrate. Diabetic control by diet and an oral α-glucosidase inhibitor were sufficient. There was no evidence of retinopathy or albuminuria before the episode of nephrosis. His urinalysis on admission was positive for proteinuria (2.5 g/day) and red blood cells (1–4/hpf). His blood chemistry findings were as follows: albumin 3.2 g/dl, creatinine 1.11 mg/dl and HbA1c 6.1%. A kidney biopsy revealed 21 glomeruli, of which 2 were globally sclerotic. The glomeruli showed increased mesangial matrices and mild GBM thickening. Massive foam cells had infiltrated into the glomerular capillaries and mesangial region (fig. ). These foam cells stained positive with Oil Red O (fig. ) and immunohistochemically stained positive for CD68 (fig. ). Transmission electron microscope images also revealed foam cell infiltration mainly located in the capillaries, and some mesangial cells showed scattered lipid droplets in their cytoplasm (fig. ). Mild interstitial fibrosis and tubular atrophy (20% of the cortical region) as well as moderate arteriosclerosis and arteriolar hyalinosis were found. The foamy change was seen in some tubular epithelial cells but no interstitial foamy cells were found. Immunofluorescence studies revealed segmental deposits of IgM and C3 in the mesangial area and slightly peripheral IgG deposits in the glomeruli; however, no significant deposit was found by electron microscopy. He had an apoE2 homozygote phenotype and the Arg158Cys genotype with no other mutations. After the diagnosis, ezetimibe was added to his statin therapy. The patient's most recent serum creatinine was increased to 2.1 mg/dl after 2 years.


A 51-year-old Caucasian man was brought into the Emergency Department following a mechanical fall. His past medical history was significant for rheumatic heart disease, mechanical mitral valve replacement at the age of 29 years, repeat replacement at the age of 33 years, and left-sided stroke. He was on warfarin for anticoagulation (mechanical valve). His social history was significant for smoking (2–3 cigarettes/day) and occasional use of alcohol, while family history was significant only for diabetes mellitus.
On physical examination, his vitals were stable with a temperature 98° F, blood pressure 113/79 mm Hg, pulse rate 88 beats/min, and respiratory rate 18 breaths/min. There was no rash except for mild ecchymosis over the right eye and helix of the right ear. His conjunctiva was pale but anicteric. There was mild pedal edema. A cardiac examination demonstrated a mechanical heart valve click, normal second heart sound, absent murmur, and no pericardial rub. His neurological exam revealed mild confusion and right-sided weakness. The rest of the examination was unremarkable.
A computed tomography of the head without contrast, performed in the Emergency Department, showed a right subdural hematoma. He was subsequently transferred to the Intensive Care Unit for observation and neurological monitoring. Laboratory tests showed a very high prothrombin time. Serum creatinine on the day of admission (day 1) was 70.72 μmol/l (normal range: 53.04–97.24 μmol/l). He received 4-factor (II, VII, IX, and X) prothrombin complex concentrate for reversal of the high international normalized ratio in view of his subdural hematoma. By the next morning (day 2), he had become oliguric, and serum creatinine increased to 247.52 μmol/l. Other laboratory results on day 2 are shown in table . Urine sediment showed granular casts. There were no dysmorphic red blood cells or cellular casts.
In view of the drop in hemoglobin and platelets, elevated lactate dehydrogenase (LDH), indirect hyperbilirubinemia, and acute kidney injury, the possibility of TMA secondary to hemolytic uremic syndrome/thrombotic thrombocytopenic purpura was considered. Complement studies showed C3 0.70 g/l (normal range: 0.82–1.85 g/l) and C4 0.19 g/l (normal range: 0.15–0.53 g/l). ANA, anti-dsDNA, hepatitis panel, HIV, anti-scl-70, ANCA and anticardiolipin antibody were all negative.
A peripheral smear demonstrated the presence of schistocytes consistent with microangiopathic hemolytic anemia. A renal ultrasound was unremarkable. A Tc-99m MAG3 scan demonstrated decreased perfusion to both kidneys and severely decreased cortical uptake. Renal Doppler study of the right kidney showed a resistive index of 0.69. Blood flow to the left kidney could not be assessed completely due to abdominal gas.
On day 4, a renal biopsy was performed which conformed TMA. The findings are outlined below (fig. , fig. , fig. ).
A diagnosis of aHUS was made based on biopsy findings, serological data, and clinical history. A normal ADAMTS 13 and an equivocal antibody test to ADAMTS 13 ruled out thrombotic thrombocytopenic purpura. On day 3, hemodialysis was initiated due to anuria and hyperkalemia. Plasma exchange with fresh frozen plasma was initiated on day 5 for treatment of aHUS (60 ml/kg of fresh frozen plasma for restitution). Fresh frozen plasma was selected as the restitution fluid in view of the initial concern about genetic or inherited complement defects. Subsequent quantitative assays looking for genetic or inherited complement defects [factor B 239.7 μg/ml (127.6–278 μg/ml), MCP CD46 positive, factor H 253 μg/ml (160–412 μg/ml) and factor I 33.5 μg/ml (29.3–58.5 μg/ml)] were within normal limits. Factor H antibody was negative. A genetic mutational analysis for the above described complement factors was unable to be performed due to denial from the patient's insurance company. Plasma exchange was discontinued after 2 days, and eculizumab was started after administration of a meningococcal vaccine (day 7). By day 8, the patient's platelets improved to 142 × 10/l and LDH decreased to 945 U/l. Hemoglobin also stabilized. Eculizumab was continued at 900 mg every week for a total of 5 weeks and then transitioned to 1,200 mg every 2 weeks. Urine output gradually improved and hemodialysis was terminated after 2.5 months. His estimated glomerular filtration rate at 6 months was 41 ml/min/1.73 m2 as determined by the MDRD formula. The trends in LDH and platelets over time are represented in fig. . A computed tomography scan of the chest/abdomen/pelvis was also done to rule out infection or malignancy as precipitants for aHUS.
Informed consent was obtained from the patient for the publishing of this work.


A 26-year-old male reported to the Dental Department, with the chief complaint of swelling and difficulty in breathing for about 5 months. The extraoral clinical examination revealed a diffuse swelling on the left side of the face obliterating the nasolabial fold. The swelling was associated with slight pain and continuous headache. Patient also complained of continuous tear discharge from the left eye. Computed tomography scan revealed a soft tissue lesion completely obliterating the left maxillary sinus and extending up to the nasal cavity; right maxillary sinus was normal. The swelling extended anteriorly up to external nasal orifices and posteriorly to the nasopharynx. The nasal septum showed deviation to the right side. Left ethmoid sinus was also found to be involved. The (orthopantomogram) showed similar radiolucency of the lesion which was interrupted by some flecks of radiopacitites [Figures and ]. Also, both the maxillary canines were impacted with the left maxillary permanent canine involved with the lesion and were closely associated with the small flecks of radioopacity.
The presence of keratin and cholesterol crystals in the fine needle aspiration cytology report directed toward the cystic lesion having keratin in the lumen. A provisional diagnosis of OKC was arrived upon. Complete enucleation of the lesion was carried out under general anesthesia, which was followed by extraction of all the left maxillary teeth. Along with the lesion frontoethmoid sinus was also excised as the lesion had involved the same. After completion of the surgical procedure the entire cavity was completely cleaned and was packed with Gel foam. Prosthesis (obturator) was made from the presurgical records and was delivered to the patient after surgery. Following the surgery, the healing of the surgical wound was uneventful and the patient was kept under observation to check for recurrence.
The histopathological examination revealed the cystic space which was lined by a continuous layer of stratified epithelium of 6-8 cell layer thickness with prominent basal cell layer and the absence of rete ridges. The stratified epithelium was orthokeratinized in nature with prominent stratum granulosum layer. Abundance of orthokeratin was present in the cystic lumen. The connective tissue showed minimal or no inflammatory response. One portion of the lesion was hard and solid which was processed by decalcification. The decalcified section showed presence of dentine and pulp like tissue arranged in an irregular pattern. Enamel spaces were seen in some areas. On the basis of thorough histopathological examination, the final diagnosis of orthokeratinizing odontogenic cyst associated with complex odontoma was given [Figures and ].


A 46 years old man had a closed proximal left humeral fracture following a road traffic accident one and half year back. After that he was referred to our hospital where fixation of fractured fragments was done by internal screw fixation followed by pre-emptive treatment with cefoperazone-sulbactum combination and ceftazidime for 10 days. The patients was initially fine for two weeks after that he suffered from persistent pain over affected region and six months later a sinus tract developed along with purulent discharge. [] The patient received various combinations of antimicrobials (mainly second and third generation cephalosporins) to control the disease. Conventional radiographs showed subperiosteal thickening in earlier stage. Bone scintigraphy showed increased uptake in the humeral diaphysis. Laboratory investigations revealed hemoglobin 12 mg/dl, leucocyte count 12 × 103/cmm, erythrocytic sedimentation rate 30 mm/1st hr, random plasma glucose level 130 mg/dl and CRP 180 ug/ml. The expressed pus from the sinus tract was aseptically collected. The direct gram stained smear of collected pus showed gram positive cocci (GPC) arranged in small clusters. The other part of collected sample seeded on blood agar (BA) and McConkey's agar (MA) plate and both the plates were incubated aerobically at 37°C for 48 hours. After 48 hours of incubation non-pigmented small 0.1 mm diameter non haemolytic colonies grew on BA plate. [] MA plate showed no growth. The colonies were further sub cultured on Nutrient Agar (NA) and grew as small non-pigmented colonies. Gram stained smear from colony of BA and NA revealed GPC arranged in small clusters. The colonies were catalase positive and oxidase negative. Slide coagulase test from the isolated colonies was negative and tube coagulase test was positive after 18 hours. Antibiotic susceptibility test result showed the isolated strain was only susceptible to vancomycin and resistant to oxacillin, azithromycin, co-trimoxazole, and cefpodoxime. [] A lawn culture was put on NA plate along with 20 ug and 40 ug menadione impregnated discs over it. The colonies around menadione discs grew as large colonies thereby confirming menadione auxotrophy. Subculture from this large colony revealed yellow pigmented normal Staphylococcal colony. [] These large colonies were catalase, coagulase (slide and tube) tests positive and oxidase negative.


A 45-year-old male patient underwent partial penectomy and distal urethrostomy for erythroplasia of Queyrat (i.e. carcinoma in situ of squamous cell carcinoma) in 2008. Since he was extremely dissatisfied with the remaining penile stump, he consulted our center about the option of phallic reconstruction. On June 14, 2010, phallic reconstruction was performed. The native urethra was lengthened by means of a radial forearm free flap. The phallus was reconstructed with an anterolateral thigh flap. The urethra was positioned inside the phallus according to the ‘tube-in-a-tube’ principle. In October 2012, the patient presented with obstructive voiding symptoms. A diagnostic urethroscopy showed a suspicious lesion in the remaining native, bulbar urethra, and biopsy confirmed the recurrence of a poorly differentiated squamous cell carcinoma of the urethra. On December 13, 2012, he underwent excision of the remaining native, bulbar urethra with the creation of a perineal urethrostomy at the membranous urethra. The perineal urethrostomy was performed using the technique as described by Blandy et al. []. Unfortunately, the postoperative period was complicated by bleeding, wound dehiscence and infection (fig. ). Finally, the perineal urethrostomy became completely obliterated and a suprapubic catheter was placed (fig. ). As the patient accepted this only as a temporary solution, we proposed the revision of the perineal urethrostomy using a meshed split-thickness skin graft. This procedure was performed on April 23, 2013. The patient was placed in the lithotomy position and a midline perineal incision was made. Dissection was continued up to the level of the urogenital diaphragm. A flexible cystoscope was inserted along the suprapubic tract and brought through the bladder neck into the posterior urethra. This marked the place of obliteration as the light of the cystoscope could be seen through the perineal incision. The urethra was opened on the tip of the cystoscope. All fibrotic tissue was further excised until only healthy urethral mucosa remained. The urethral stump was spatulated over 1.5 cm. Vicryl 4.0 stay sutures were placed at the mucosa to facilitate identification and to prepare the anastomosis for the graft (fig. ). A split-thickness graft (10 × 15 cm) was harvested from the skin of the left thigh and meshed 1:1. In the middle of the graft, an opening was made to anastomose with the urethral opening. The edges of the graft were further tailored and anastomosed with the perineal skin edges. Several additional quilting sutures were placed to fix the graft against the perineal graft bed (fig. ). A compressive dressing and a 20-Fr urethral catheter were left in place for 5 days. After 5 days, the dressing was removed with satisfactory graft take. Subsequently, the catheter was also removed. At present (6 months of follow-up), the patient is voiding well. Maximum and average urinary flow are 22 and 13 ml/s, respectively. His International Prostate Symptom score is 5/35. The perineal wound has healed well, with minimal contraction of the grafted area (fig. ). Through the neomeatus, a 20-Fr catheter can be passed without any difficulties, indicating a sufficient diameter.
This study was approved of by the local ethics committee.


A 45 year-old female patient was referred to our department with right ear fullness which was precipitated by changes in position. Her symptom was initiated about 3 months ago. She has been well controlled hypertension. In physical examination, air fluid level was found in her right tympanic membrane, and the level increased when she hung her head down. Pure tone audiometry examination showed the right conductive hearing loss. Impedance test showed that the right was C type and the left was A type. In order to distinguish CSF otorrhea, her middle ear fluid was aspirated, and its glucose level noted 88 mg/dL. Computed tomogram (CT) of temporal bone demonstrated a bony defect adjacent to the soft tissue lesion of posterior wall of the temporal bone with an effusion showing high attenuation in the right mastoid cavity (). Thus, we presumed that CSF otorrhea might be caused by arachnoid granulation. She underwent mastoidectomy under general anesthesia. During a procedure for canal wall-up mastoidectomy, in accordance with the findings of the CT scan, the tissue which was suspected to be arachnoid granulation was observed with the bony defect of the posterior wall. The surrounding bone was removed by a diamond drill. The granulation tissue was placed at the defect of meninx (). The tissue was obtained for a biopsy. The remained granulation tissue was shrunk by bipolar cauterization. The bony defect was reconstructed by the auricular cartilage and perichondrium complex graft. Subsequently, fibrin glue was placed over the graft. The biopsy demonstrated a fibrillary tissue (). Moreover, glial fibrillary acidic protein stain indicated a positive reaction, and we confirmed arachnoid granulation which contained a portion of parenchyma of the cerebrum (). The patient has not had any symptoms of the disorder. In addition, 1 year and 3 months after the surgery, she's been continuing follow up in the outpatient clinics without any complications or recurrence.


A 53-year-old male presented with cough, sputum, and headache for 2 weeks.
Five years previously, he had been diagnosed with HIV. He noted the onset of cough, sputum, and headache. The cough worsened, his sputum turned more yellowish and headaches accompanied by neck stiffness were related to coughing.
Over the first five years following the diagnosis of HIV, he was treated with antiretroviral medications (lamivudine and zidovudine; Combivir, Ritonavir; Norvir, atazanavir sulfate; Reyataz).
Vital signs were as follows: temperature, 38.0℃; blood pressure, 110/70 mm Hg; pulse rate, 90/min; respiratory rate, 20/min. He was alert mentally. Auscultation revealed rales in the right lower lung field. A Brudzinski-Kernig test was positive.
Complete blood count results were white blood cell 18.43×103/µL (neutrophils 91.3%, lymphocytes 3.5%), hemoglobin 14.0 g/dL, hematocrit 40.3%, and platelets 290×103/µL. Serum chemistry showed blood urea nitrogen 14 mg/dL, creatinine 0.66 mg/dL, aspartate aminotransferase 25 IU/L, alanine aminotransferase 23 IU/L, total bilirubin 1.92 mg/dL, and C-reactive protein 38.51 mg/dL. Cerebral spinal fluid (CSF) showed a white blood cell count of 108/µL, red blood cell count 0/µL, glucose 53 mg/dL, and protein 123 mg/dL. CSF and sputum bacterial cultures were negative. A CD4 T-cell count of 139/µL and HIV RNA of 20.7 copies/mL were recorded.
Plain chest radiography showed ground glass opacity and consolidation in both lung fields (especially the right lower) (). Contrast-enhanced chest computed tomography showed multiple irregular nodular infiltration in both lungs and patchy consolidation with ground glass opacity of the right middle and lower lung (). Contrast-enhanced cervical spine magnetic resonance imaging (MRI) showed a posterior epidural abscess: a peripheral rim-enhancing lesion at the posterior C2-3 epidural space (); osteomyelitis; mild scanning enhancement of the clivus and cervical spine; early leptomeningitis; and prominent leptomeningeal enhancement ().
The patient was administered vancomycin (1 g q 12 hr IV), ceftriaxone (2 g q 24 hr), clindamycin (600 mg q 12 hr) and fluconazole (400 mg q 24 hr). The patient's fever reduced 3 days following admission. His epidural abscess was discussed with the neurosurgeon in our hospital. There were no neurological symptoms, no surgical treatment was performed and antibiotic therapy was maintained. At follow-up 5 days after admission, a plain chest radiograph showed improvement, and S. pneumoniae was detected in two blood cultures that showed sensitivity to most antibiotics. The patient was thus switched from vancomycin plus ceftriaxone to ampicillin (2 g) plus sulbactam (1 g q 6 hr IV). Fifteen days after admission, neck stiffness had improved and cough and sputum production reduced. A plain chest X-ray showed decrease in both ground glass opacity and consolidation in both lung fields (). Twenty-six days after admission, laboratory analysis was as follows: white blood cell count 5.42×103/µL, neutrophils 55.9%, lymphocytes 25.9%, and C-reactive protein 0.26 mg/dL. The patient wished to be discharged, so antibiotic therapy was switched from intravenous to oral (amoxicillin/clavulanate 625 mg PO tid). Since discharge, no recurrence of symptoms or evidence of pneumonia according to plain chest radiography have been observed. One year after discharge, there is no abnormal finding on cervical MRI ().


A 31-year-old man who had no significant medical history presented with status epilepticus. He had headache, fever of 39° and generalized tonic clonic seizure without recovery of consciousness. His vital sign was stable except fever. On neurological examination, there was no abnormality except stuporous mental status between frequent generalized tonic clonic seizures. Initial blood tests showed no leukocytosis and normal level of inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Cerebrospinal fluid (CSF) was clean and contained 108 leukocytes/mm3 (83% polymor-phonuclear leukocytes and 17% lymphocytes) with normal glucose (78 mg/dL) and protein (42 mg/dL). Serologic testing of blood and CSF was normal. Initial brain MRI showed T2 hyperintensity and swelling of isolated bilateral hippocampus, especially CA1 region without any abnormal lesion in other areas ().
On the day of admission, we assumed that the patient had herpes encephalitis, and started maximum dosage of acyclovir (15 mg/kg three times a day) which is the hospital protocol for treatment of herpes encephalitis. Electroencephalography (EEG) showed frequent spike and waves on bilateral fronto-temporal areas dominantly on temporal areas, so we started antiepileptic drugs (initial phenytoin loading with subsequent add-on of levetiracetam, oxcarbazepine and topiramate) and midazolam-coma therapy. Further serologic tests including polymerase chain reaction (PCR) for herpes species were negative and no bacteria and viruses were grown. He recovered from status epilepticus three weeks later, but afterwards he developed KBS which is characterized by hyperphagia (he ate a large amount of food six to seven times a day), hypersexuality (he wanted to have a sex with the nurses, his ex-girlfriend and his mother), hypermetamorphosis, global amnesia (memory registration was normal, but recall was impaired) and dysosmia. On the 46th day of hospitalization, the score of his Korean-version of mini-mental status examination was 20/30 (orientation to time- 3/5, orientation to place- 2/5, immediate recall- 3/3, delayed recall- 0/3, attention and calculation- 3/5). Since we considered that his symptoms occurred due to secondary temporal lobe epilepsy, we performed an EEG, but his EEG showed no definite epileptiform discharges. Forty-five days later, follow-up brain MRI showed isolated bilateral hippocampal atrophy (). Finally, we concluded that he had a KBS, and the patient was discharged on the 58th day of hospitalization and his cognitive dysfunction is persistent with minimal improvement for six months follow-up.


A 73-year-old, right-handed woman visited our hospital in September 2010, complaining of right foot drop and numbness on right lower leg in the previous month. There was no history of diabetes, trauma or any other polyneuropathy. She had undergone a lumbar disc operation five years previously. In particular her first son and grandson had a history of transient monoparesis of upper limbs that spontaneously resolved.
Neurological examination revealed weakness of right foot dorsiflexion and eversion (Medical Research Council scale grade 3) and reduced pinprick and touch sensations on the dorsal side of the right foot and the lateral aspect of the lower leg. Deep tendon reflexes at both knees and ankles were decreased.
Electrodiagnostic studies showed a right peroneal neuropathy around the knee level and chronic bilateral L5 radiculopathy. The sonographic examinations demonstrated swelling of right common peroneal nerve around the fibular head level (cross-sectional area [CSA], 18 mm2), compared with contralateral side (CSA, 9.6 mm2).
Six months later, while she was recovering from foot drop, she had another presentation with wrist drop on the right side. Electrodiagnostic studies were performed to rule out radial neuropathy. Motor nerve conduction studies showed prolonged distal latency and reduced compound muscle action potential amplitude in the right median nerve and no response in the right peroneal nerve. The ulnar nerve's segmental conduction velocities across the elbow were decreased (). In sensory nerve conduction studies, sensory nerve action potentials (SNAP) of both median nerves and right superficial peroneal nerve were not generated. The distal sensory latency was prolonged, and SNAP amplitude was reduced in both ulnar nerves (). In needle electromyography, ulnar innervated muscles including the abductor digiti minimi, first dorsal interosseous, and flexor carpi ulnaris showed large amplitude, long duration and polyphasic motor unit action potentials (MUAP) with reduced recruitment. In the forearm muscles innervated by radial nerve, extensor digitorum communis and extensor indicis proprius showed no MUAP. However brachioradialis and extensor carpi radialis longus muscles did not show any abnormalities. Median innervated muscles, such as abductor pollicis brevis and flexor carpi radialis, showed long duration and polyphasic MUAP. In lower limb muscles, abnormal spontaneous activities and large amplitude, long duration and polyphasic MUAP with reduced recruitment were demonstrated in right peroneal innervated muscles, such as tibialis anterior and peroneus longus (). These electrodiagnostic studies suggested not only right radial neuropathy, but also bilateral median, ulnar neuropath and right common peroneal neuropathy. The clinical findings and electrodiagnostic findings suggested HNPP, and the diagnosis was confirmed by genetic test demonstrating a PMP22 gene deletion.
Peripheral nerves of the upper and lower extremities were examined by ultrasonography with an HD15 ultrasound system (Philips Ultrasound Inc., Bothell, WA, USA) using a 7-12 MHz linear array transducer. We traced most of the peripheral nerves, which were typically measured in previous studies about normal reference values of CSA in sonography, including median nerve at the level of carpal tunnel inlet and outlet in the forearm at the antecubital fossa and mid-humerus, ulnar nerve at the wrist and elbow, mid-humerus and radial nerve in the forearm and spiral groove level, common peroneal nerve at the fibular head level, tibial nerve at the popliteal fossa, sciatic nerve in the distal thigh and cervical roots at the C5-8 level. All measurements were done on the right side. CSA was measured using a direct tracing method just inside the hyperechoic rim of each nerve, and the CSA findings were compared with normal reference values [-].
The CSA of the median nerve at the carpal tunnel inlet and outlet (CSA, 10.8 mm2 and 9.8 mm2; normal value <6.7 mm2) and in the forearm (CSA, 12.7 mm2; normal value <10.7 mm2) was enlarged (). However the CSA of median nerve at the antecubital fossa (CSA, 13.1 mm2; normal value <13.2 mm2) and mid-humerus level (CSA, 10.9 mm2; normal value <13.1 mm2) was not swollen. The CSA of ulnar nerve was enlarged at the elbow (CSA, 9.3 mm2; normal value <8.3 mm2), but not in the wrist (CSA, 4.6 mm2; normal value <8.1 mm2), and not in the mid-humerus level (CSA, 5.0 mm2; normal value <7.9 mm2). The radial nerve was not swollen in the forearm (CSA, 5.5 mm2; normal value <14.3 mm2) or at the spiral groove level (CSA, 5.1 mm2; normal value <13.3 mm2). In the lower limb, the CSA of common peroneal nerve at the fibular head level was enlarged (CSA, 16.2 mm2; normal value <12.1 mm2) (). However, other nerves of lower extremity were not swollen: the tibial nerve at the popliteal fossa (CSA, 23.5 mm2; normal value <55.9 mm2) and the sciatic nerve in the distal thigh (CSA, 49.9 mm2; normal value <80.6 mm2). The cervical nerve roots (C5-8) were not enlarged at any root level: C5 root (CSA, 6.8 mm2; normal value <7.8 mm2), C6 root (CSA, 10.3 mm2; normal value <12 mm2), C7 root (CSA, 11.6 mm2; normal value <14.2 mm2) and C8 root (CSA, 9.8 mm2; normal value <14.4 mm2) ().


A 29-year-old woman presented to our clinic with a 3-month history of left knee pain. The patient had no significant previous medical history or trauma. Pain was aggravated after going up and down the stairs. On physical examination, she had localized tenderness in the lateral aspect of the patella. However, there was no evidence of a soft-tissue mass and overlying skin lesion. Further, no joint effusion was noted. She had full range of motion and the neurocirculatory status was normal. Laboratory studies indicated that all inflammatory markers were within the normal limits. Radiographic examination revealed a well-defined osteolytic lesion with a sclerotic margin in the lateral aspect of the patella, and there was no pathologic fracture or periosteal reaction (). A technechium-99m bone scan showed an increased uptake isolated to the lateral aspect of the left patella. Magnetic resonance imaging (MRI) was also conducted, which showed a partially multilobulated and septated mass in the lateral aspect of the patella. A fluid-fluid level was noted in this mass with T1-low and T2-high signal intensity. The margins of the lesion were well defined and a cortical break was not seen in the anterior and posterior cortex of the patella (). Based on the imaging studies, the preoperative diagnosis was foremost aneurysmal bone cyst or simple bone cyst.
At surgery, there was no abnormality in the soft tissue around the patella. The lesion was aspirated using a needle and about 2 mL bloody fluids were discharged from the cavity. The lesion was a cystic cavity and was surrounded by a gray or brownish tissue. Multiple fragments of hemorrhagic soft tissue admixed with recognizable bone fragments were revealed. Curettage was completely performed and cauterization of the inner wall of the cavity was thoroughly done. After massive saline irrigation, the cavity was filled with autogenous iliac bone chips.
Microscopically, sheets of chondroblasts were intermingled with fine eosinophilic chondroid matrix. The chondroblasts showed oval shaped nuclei with moderate eosinophilic cytoplasm. Several multinucleated giant cells were scattered. Occasional mitotic figures were also identified. The indented nuclear grooves were characteristic nuclear features. Blood-filled cystic cavities were present in the chondroblastoma, consistent with secondary aneurysmal bone cyst ().
The final diagnosis was chondroblastoma with a secondary aneurysmal bone cyst. Post-operatively, the patient was ambulating without a limp and had a normal, pain-free range of motion. At the 3-month follow-up, she had returned to her usual daily and recreational activities. Radiologically, the grafted bones were well incorporated and there were no other abnormal symptoms and no recurrent evidence in the patella at the post-operative 1.5-year follow-up ().


A 27-year-old Saudi female, living in Makkah, was referred from ophthalmology clinic, with severe retro-orbital headache and neck pain started 3 days before admission. There was no myalgia, arthralgia, bleeding manifestation, cutaneous rash, or any similar or significant past medical history. On examination, she was in pain, febrile with a temperature of 38.3°C, respiratory rate of 20/min, heart rate of 112/min, and blood pressure 116/63 mmHg. Her chest, cardiovascular, abdomen, and neurological examinations including fundus were unremarkable, except of mild neck rigidity. She was admitted with professional diagnosis of acute meningitis and started on intravenous dexamethasone, vancomycin, ceftriaxone, and acyclovir with good hydration. Brain CT scan was normal. CSF was normal except of pleocytosis (85% lymphocytes). The complete blood count was as follows: WBC 4.5 cell/mm3, 60% lymphocytic, Hb 12.1 gm/dL, platelets count 135/mm3. The blood tests for malaria, brucella, cytomegalovirus (CMV), Epstein-Barr virus (EBV), human immunodeficiency virus (HIV), and swine flu virus (H1N1) in the blood were negative. Dengue serology sent to Makkah central laboratory due to relative low platelets and low grade fever. Upon these result dexamethasone, vancomycin, and ceftriaxone were stopped on the next day and the patient was kept on intravenous acyclovir only. Throughout the 7 days admission period, the patient was almost afebrile but showed bizarre headache appearing as persistent diffuse headache with attacks of severe retro-orbital pain, eye tearing, and blurring vision mainly on left eye that respond only to pethidine intravenous injection in spite of trial of multi analgesic and neuropathic medication including amitriptyline, topiramate, pregabaline, and tramadol. The headache attacks predominantly appeared between 3 and 11 pm, associated with anxiety and fear of death in addition to episodes of arrhythmia range between bradycardia down to 45/min and supraventicular tachycardia up to 180/min, that required many cardiology and psychiatry on call review. On 4th day of admission, serum dengue IgM reported to be positive. To further strengthen the diagnosis, we repeated the CSF test for dengue IgM. On the 7th day, the patient was so much upset and asked discharge against medical advice to seek abroad management. 20 days later, the patient was seen in neurology out-patient clinic complaining of persistence migraine like headache and the CSF test for dengue IgM was found to be positive. Over several months later, the patient was on amitriptyline and tramadol on need. Then gradually and slowly, headache attacks diminished in frequency and severity, resolving almost completely 4 months after onset.