The neuroleptic malignant syndrome: A report of 14 cases from North India.

Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. This study was performed to investigate the clinical spectrum, antecedent events and outcome of NMS patients admitted in the Neurology department of a large teaching hospital of North India. Fourteen cases of NMS were taken after a thorough search during a three-year period (May 2000 to April 2003). The Incidence of NMS was 1.40/ 1000 patients treated with neuroleptics and mortality rate was 14.28%. Amongst the neuroleptics Haloperidol (parenteral) was implicated as a most common drug for NMS in 57% of patients. An association with coexisting precipitating illness was clearly recorded in 71.4% patients. All the recorded patients of NMS received 500-700 mg CPZ equivalent/day of neuroleptics. NMS as an indiosyncratic phenomenon was noticed in 28% patients. 85.7% responded to dopaminergic drugs along with supportive treatment and showed partial or complete recovery within 7-14 days. In those with partial recovery residual deficits included Parkinsonian features, depression and diaphoresis in a small percentage of patients.

Pseudotumor--like presentation of neurobrucellosis.

Brucellosis is bacterial zoonoses. In endemic areas brucellosis can present with clinical features of nearly any neurological illness. Meningitic presentation is most common, with patient presenting with either acute or chronic meningitis. Pseudotumor--like presentation is also documented and accounts for only 4% of cases of neurobrucellosis. Here we are documenting a case of neurobrucellosis with presentation similar to pseudotumor cerebri but with abnormal CSF. This highlights the fact that being a potentially treatable condition brucellosis should always come in the differential diagnosis of neurotuberculosis especially if there are atypical features e.g. pseudotumor presentation with abnormal CSF.

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.

CADASIL in a family from north-west India.

We here with report a family with two sibs having history of recurrent familial stroke. Neuroimaging revealed diffuse hyperintense signals in subcortical white matter and basal ganglia on MR images in younger sib suggestive of cerebral autosomal dominant arteriopathy with sub-cortical infarcts and leucoencephalopathy (CADASIL). The diagnosis was further strengthened on skin biopsy showing presence of PAS positive granules with thickening of dermal vessels.

Juvenile amyotrophic lateral sclerosis with unusual presentation: a case report.

A case of juvenile amyotrophic lateral sclerosis with wasting confined to the distal part of one lower limb and the proximal part of the contralateral upper limb is being presented. A brief review of the literature is carried out.

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.

Lacunar syndromes due to small haemorrhagic lesions.

OBJECTIVE: To study the frequency, clinical presentations and prognostic aspects of haemorrhagic lacunar syndromes. MATERIAL AND METHODS: 375 patients of cerebrovascular accidents admitted in neurology wards over 2 years were studied. Based on CT findings, 114 patients were diagnosed as intra cerebral haemorrhage (ICH). They were assessed in detail by careful history and examination of neurological status. Coagulation studies, MRI and angiography were done in selected cases. RESULTS: In 17 (15%) patients, size of hematoma was < 2 cms as seen in CT scan. The sites of these small haemorrhages were in basal ganglia (6 patients), internal capsule (3 patients), thalamus (5 patients) and pons (3 patients). Depending on their location they presented as pure motor stroke, pure sensory stroke, ataxic hemiparesis, sensorimotor stroke and clumsy hand-dysarthia syndrome. Three patients showed interesting phenomena like transient ischemic attack caused by bleed or previous disease showing improvement after bleed. Prognosis of these small ICH was excellent with 100% survival rate and lesser disability as shown by good functional recovery. CONCLUSIONS: Haemorrhagic lacunar syndromes are not just the statistical curiosities but could have significant prognostic bearing.

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.

A preliminary trial of serratiopeptidase in patients with carpal tunnel syndrome.

OBJECTIVES: This study was planned to assess the response of serratiopeptidase in patients with carpal tunnel syndrome (CTS). METHODS: Twenty patients with CTS were evaluated clinically. After baseline electrophysiological studies, these patients were given serratiopeptidase 10 mg twice daily with initial short course of nimesulide. Clinical and electrophysiological reassessment was done after 6 weeks. RESULTS: Mean age was 43.9 years with male to female ratio of 1:2.33. Sixty five percent cases showed significant clinical improvement which was supported by significant improvement in electrophysiological parameters. Recurrence was reported in four cases. No significant side effect was observed. CONCLUSIONS: Serratiopeptidase therapy may proved to be a useful alternative mode of conservative treatment. Larger study may be further helpful to establish the role of serratiopeptidase in CTS.

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.

Stroke like presentation of Creutzfeldt Jakob disease: an unusual variant.

Creutzfeldt-Jakob disease (CJD) is a degenerative process of the brain, induced by novel infectious agent or prion, and is usually characterized by a rapidly progressive dementia in association with myoclonus. However different patterns of disease presentation have been identified. The authors describe three probable cases of CJD. None of them had positive family history or any known modes of iatrogenic transmission. Interestingly, all the cases presented like a stroke. This is the first series of CJD cases from this part of country.

Cerebral venous thrombosis in pregnancy and puerperium--a prospective study.

During a prospective study of cerebrovascular accidents we evaluated 64 cases of cerebral venous thrombosis (CVT) in pregnancy and puerperium. CVT made up approximately half of the young strokes and 40% of strokes occurring in females. Majority of them were below 25 years of age, multipara women from rural areas who were delivered at home by untrained dais. While 79.6% cases occurred during first two weeks after delivery, the incidence of antepartum CVT was also higher at 7.8%. Majority of the patients had meningoencephalitic (70.32%) presentation. Others in the spectrum had either acute fulminant type (18.7%), neuropsychiatric (6.25%) or pseudotumour cerebri like presentation (4.68%). Patients with neuropsychiatric and pseudotumour cerebri like presentation had a better prognosis while those with acute fulminant type had a poorer outcome. The patients with bilateral hemorrhagic infarcts and diffuse cerebral edema on CT scan had a poorer prognosis. A lower mortality rate (18.75%) could be achieved with early diagnosis and quick institution of planned therapy.