The index patient is a 9-year old male who presented with complaints of progressively slurred speech, progressive weakness of right upper limb and both lower limbs and mouth deviation to the left side, of two weeks and vomiting of one hour - all prior to presentation. Onset of symptoms was insidious. Slurred speech progressed to aphasia and limb weakness progressed to inability to walk. No history of fever, catarrh, cough, leg swelling, difficulty in breathing, skin rash, haematuria, facial swelling, convulsions, headache, yellowness of the eyes, abdominal swelling, weight loss, night sweats or joint pains. No antecedent trauma to the head or ingestion of drugs or herbal preparations. His haemoglobin genotype was unknown. He presented to a private hospital where he was admitted and given subcutaneous low molecular weight heparin for two days on suspicion of stroke. His clinical state improved briefly and then deteriorated, necessitating referral to Federal Medical Centre, Owerri. General physical examination findings on admission were normal. Neurological examination showed a conscious child, cooperative with normal comprehension of speech but expressive aphasia, right sided facial weakness, no signs of meningeal irritation, normal muscle bulk, hypertonia in both upper and lower limbs, weakness of the limbs (power grade 3 on the Rt. sided limbs and grade 4- on the Lt. side), hyperreflexia bilaterally in both upper and lower limbs, absent abdominal reflexes, sustained ankle clonus and positive Babinski sign bilaterally. There were signs of incoordination (dysmetria, dysdiadochokinesia). Findings on examination of other systems were normal. Admitting diagnosis was encephalopathy 2oC encephalitis with differential diagnoses of space-occupying lesion (SOL) with raised intracranial pressure (RICP) and sickle cell CNS infarctive crisis.
Investigations done included: haemoglobin genotype, Full Blood Count (FBC), Erythrocyte Sedimentation Rate (ESR), Random Blood Sugar (RBS), Fasting Blood Sugar (FBS), blood film for Malaria Parasite (MP), Serum Electrolytes, Urea & Creatinine (SEUC), Magnetic Resonance Imaging (MRI) of the brain, Clotting profile with Activated Partial Thromboplastin Time (APTT). Results of investigations are shown in Table 1 and figures below.
The MRI brain showed multifocal areas of irregular abnormal signal intensities in the parietal and occipital regions suggestive of multiple subacute cerebral infarction (Figure 1, Figure 2, Figure 3). Following further review of patient's clinical features, investigation results and MRI brain, the diagnosis was modified to Primary Angiitis of the CNS (PACNS). Due to vomiting on admission, patient was commenced initially on intravenous fluids for 24hrs and intravenous dexamethasone 4mg 6hrly for 48hrs. By the second day on admission, vomiting had subsided and patient commenced oral feeds and tabs prednisolone 20mg two times daily (1mg/kg/d). An intravenous antibiotic (Ceftriaxone 50mg/kg/d) was commenced while awaiting FBC result. An antiheliminthic (tab Albendazole 400mg stat) was administered. Physiotherapy was also instituted. Three days into admission, patient had become emotionally labile (cried easily when spoken to and was frustrated by his inability to talk) but general and neurological examination findings remained same. He received tabs Artemeter-Lumefantrin (480/80) due to a positive blood film for MP. By the seventh day on admission, remarkable improvement was noted. Patient's speech had become audible though poorly fluent, and power had improved to grade 4- on the right side similar to the left side. Gait was broad based and ataxic and he could not tandem walk. This improvement was steady and sustained. By the 4th week on admission, power in all limbs had increased to grade 4+ but there was still subtle Rt. facial weakness, hyperreflexia, weakness of the hand muscles (Rt. > Lt.) with impaired fine motor function (handwriting, dressing). Signs of incoordination also persisted and increased appetite, weight gain of 6kg (35kg to 41kg) and "moon face" were noted. However, Blood Pressure (BP) (90/60mmHg) and vital signs (temperature, pulse rate, respiratory rate and oxygen saturation) remained normal.
Treatment was then modified with reduction of tabs prednisolone to 20mg on alternate days and addition of tabs cyclophosphamide 50mg on alternate days to synchronize with steroid-off days. Patient and his mother were counselled on the nature of the diagnosis, the possible prolonged therapy required, the possibility of residual deficits and the need for continued physiotherapy and occupational therapy. Due to the absence of trained occupational therapists in our locality, patient was provided with a small soft ball to squeeze frequently and advised to pick grains of rice from a tray repeatedly at home. He was to copy words, figures or diagrams from textbooks many times a day. All these were to improve his fine motor function especially handwriting. He was subsequently discharged and given weekly follow-up appointments in our neurology clinic. He was monitored for side effects of the immunosuppressive treatment with weekly FBC, ESR and BP monitoring and these remained normal all through the follow-up period.
Subsequent follow-up visits (weeks 5-9) showed steady improvement with normal power returning to the limbs grossly, improving fine motor function (handwriting, performance of activities of daily living) and resolution of facial weakness, expressive aphasia and signs of upper motor neuron lesion. However, increased appetite, weight gain (41kg-53kg) and signs of incoordination remained. Further weekly tapering of prednisolone was done (10mg alternate days to 4mg alternate days to 2mg alternate days). By the 12th week following diagnosis, hand grasp had become normal and handwriting legible and speed of writing good. Weight dropped to 42kg and he no longer had "moon face". He resumed schooling with unimpaired participation. However, signs of incoordination such as dysmetria (past pointing) and impaired tandem walk persisted. Both drugs were finally stopped (after 3 months) and a repeat Magnetic Resonance Imaging (MRI) requested. Due to financial constraints and patient's remarkable recovery, reimaging was delayed. Patient was seen again seven months later, about one year after admission and by then those signs of incoordination that persisted after discharge had all disappeared and patient was completely neurologically normal. A repeat brain MRI done at this time (one year after onset of illness) showed marked improvement with complete resolution of the some of the cortical lesions in the parietal and occipital regions bilaterally and residual hyperintensity in the periventricular and supraventricular cerebral white matter bilaterally involving the parieto-occipital regions predominantly; there were no new lesions (Figure 4, Figure 5, Figure 6).