Sudden bilateral reversible vision loss: a rare presentation of acute intermittent porphyria.

Acute intermittent porphyria is a rare disorder, characterised clinically by variable patterns of neurological and metabolic disturbances. We report a rare presentation with sudden onset painless bilateral reversible vision loss of cerebral origin along with a brief review of the underlying pathophysiology.

Clinicodiagnostic features of tuberculous meningitis and the role of CSF PCR in early diagnosis: a study from north-west India.

The objective of this study was to study the clinical features and laboratory profile including acid fast bacillus microscopy, cerebrospinal fluid (CSF) culture and M. tuberculosis polymerase chain reaction (PCR) for an early diagnosis of tuberculous meningitis. Fifty consecutive patients, fulfilling the clinical criteria of tuberculous meningitis, were included in the study. All patients were subjected to detailed clinical and laboratory evaluation and radiological tests. CSF acid fast bacillus microscopy, culture and PCR were carried out in all and results were analysed. Majority of the patients presented with fever, headache, vomiting and signs of meningeal Irritation. Ziehl-Neelsen staining for acid-fast bacillus was positive in 4% cases, culture in 18% cases and CSF PCR for Mycobacterium tuberculosis was positive in 68%. Miliary pattern on chest x-ray was seen in 16% and CNS tuberculomas were seen in 14% cases. The CSF PCR estimation in clinically suspected cases of tuberculous meningitis may be an extremely valuable test for early diagnosis and institution of specific therapy.

Recurrent stroke in a case of left atrial myxoma masquerading vasculitis.

We report a case of left atrial myxoma presenting as recurrent neurological deficits with absent peripheral pulses mimicking systemic vasculitis. Due to absence of cardiac signs and symptoms, there was one year delay in diagnosis from initial symptom. Left atrial myxomas are rare but treatable cause of recurrent stroke.

Parkinsonism and recovery in central and extrapontine myelinolysis.

Central pontine myelinolysis is a demyelinating affection of central pons diagnosed on the basis of characteristic MRI finding in an appropriate clinical setting. The condition has been described as universally fatal; however, recent reports of recovery have been documented. We report a case of central pontine and extra pontine myelinolysis, which presented with parkinsonian features apart from bulbar symptoms and made a remarkable recovery. A short review of the literature follows.

Juvenile myoclonic epilepsy--an experience from north western India.

OBJECTIVES: The clinical data on cases of Juvenile Myoclonic Epilepsy (JME) were analysed. Response to initial small dosages (lower than usual) of sodium valproate and further lower maintenance dosages in patients who were seizure free for 2 years on drug were assessed. MATERIAL AND METHODS: Seventy-six patients who were diagnosed to have Juvenile Myoclonic Epilepsy on definite criteria were studied. All patients were put on sodium valproate in dosages (lower than usual) for initial control and further lower maintenance dosage and response evaluated. RESULTS: The clinical profile was found to be similar as in other parts of India. There was a female preponderance and average delay of 4.9 years in final diagnosis. Forty-eight (63.1%) patients showed good control on 15 mg/kg/day dosages of sodium valproate. After a seizure free interval of 2 years, 58% of patients could be maintained on small dosages ranging from 3-5 mg/kg/day to 6-8 mg/kg/day. CONCLUSION: The majority of JME patients responded well not only to sodium valproate in dosages lower than usually prescribed but required very small dosages for maintenance after a seizure free period of 2 years.

Herpes simplex encephalitis in North West India.

All patients admitted with provisional diagnosis of an encephalitic illness over a period of 30 months, were studied. Special investigations included CSF analysis, EEG, CT scan and MRI. Herpes simplex virus (HSV) antibody estimation in CSF and blood was done simultaneously using ELISA. Patients with diagnosis of cerebral venous thrombosis, cerebral malaria, tubercular meningitis etc, who resembled herpes simplex encephalitis (HSE), were excluded systematically with relevant investigations. 28 patients showed electroencephalographic, serologic and/or neuroradiological evidence of herpes simplex encephalitis. Males were affected more than females. Age ranged from 4 years to 65 years. Main clinical features included altered sensorium (100%) and seizures (89%). Serological test for HSV antibody in CSF and blood was positive in 14 patients. Fronto-temporal localisation was seen in EEG of 18 patients. CT and MRI were fairly characteristic with bilateral asymmetric fronto-temporal lesions. Patients with mild disease and who reported earlier responded well to treatment with acyclovir. Mortality was higher if treatment was delayed or if the disease was severe. Delayed treatment even in less severe cases produced neurological deficit in many survivors. Despite limitations of non-availability of CSF-PCR and serial estimation of HSV antibodies, the study is an attempt to highlight the value of high index of suspicion of HSE on clinical grounds, systematically excluding cases with different aetiologies resembling HSE and planning early antiviral therapy to reduce both mortality and morbidity associated with this fatal disease.

Antiphospholipid antibody positive young stroke: an analysis of 12 cases.

AIMS AND OBJECTIVES: To study clinico-investigative profile of 12 young (<45 years) patients with stroke who tested positive for anti phospholipid antibodies (APLA). SUBJECTS AND METHODS: The diagnostic, clinical, laboratory and radiologic features in 12 APLA positive young patients who presented with stroke were studied. The APLA analysis included estimation of anticardiolipin (aCL) antibodies and lupus anticoagulant (LA). Other relevant tests included anti-nuclear antibody, human immunodeficiency virus, Venereal Diseases Research Laboratory, platelet count, echocardiography and carotid Doppler. APLA positive strokes were those cases where either the immunoglobulin G (IgG) and immunoglobulin M (IgM) were raised or LA was positive, and other known causes were excluded. RESULTS: Levels of IgG (aCL) was raised in 11 cases (mild 7, moderate 1, high 3), IgM was elevated in all the 12 cases (moderate 2, high 10). Of the two LA positive cases both were IgM positive but in one IgG was negative. Five patients showed small multiple bilateral cerebral infarcts on computerised tomography (CT) scan. 5 patients had history of recurrent strokes. Hemiparesis was more frequent than hemiplegia. None presented with dense hemiplegia. All patients recovered to normal functional capacity and did not have recurrence on drugs. CONCLUSION: A preliminary study on APLA positive young strokes showed certain clinical and radiological features, mild to moderate stroke, pre-treatment recurrences, multiple smaller infarcts on CT, which could be clustered in a subgroup of stroke in young. Incidentally these patients showed a good prognosis in terms of long term outcome.

Mega dose intravenous methylprednisolone in bilateral simultaneous optic neuritis.

OBJECTIVES: The study was carried out to assess the efficacy of megadose intravenous methylprednisolone in patients of bilateral simultaneous optic neuropathy (BSON) of unknown etiology. METHODS: Fifteen consecutive patients admitted in neurology unit were included in the study. These patients were subjected to various investigations including MRI and CSF exam to exclude all known causes of optic neuropathy including multiple sclerosis. All patients were put on single dose intravenous methylprednisolone, 1 gm/day in adults and 500 mg/day in children for three days. Pre and post-treatment visual parameters including visual evoked potentials (VEP) were carried out. RESULTS: There was a female preponderance and the average age was 28 years. The visual acuity and P100 latences of visual evoked potentials (VEP) improved in all cases. The improvement was statistically significant (P < 0.001). CONCLUSION: Use of intravenous methylprednisolone is a preferred drug in bilateral simultaneous optic neuropathy (BSON) as compared to oral or retrobulbar steroids.

Clinical profile and spectrum of epilepsy in rural Rajasthan.

OBJECTIVE: The study was conducted at a rural primary health centre of Rajasthan with the aim to study the clinical profile of epilepsy, to assess the efficacy and side effects of antiepileptic drugs and to educate the patients. METHODS: Two hundred and eighty four patients were studied over a period of one year. Seizures were classified according to International League Against Epilepsy Classification (1981). Detailed clinical history and neurological examination was done in all patients. Routine blood tests, computerised tomography of head and electroencephalogram (EEG) were done only in few patients due to non-availability and limited resources. All patients were given free medicines and followed up monthly for assessing the compliance, the response and side effects of anti-epileptic drugs. RESULTS: Male:Female ratio was 2:1. Mean age of onset was 9.8 yrs. Generalized tonic and clonic seizures was the commonest type (82%) of seizures. Eighty one percent patients had onset of seizures before 20 years of age. Sixty eight percent were fully controlled on drugs. Fifty percent were controlled on monotherapy and 15% required more than two drugs. CONCLUSION: Most of the epileptic patients can be managed at rural centre without sophisticated investigations.