Sjogren - Larsson Syndrome: a case report.

Two male siblings aged 8 and 11 years, born of non-consanguineous parents, presented with classical triad of congenital ichthyosis, mental retardation and spastic diplegia. They had no eye, hair, nails and teeth changes. MRI showed diffuse dysmyelination.

Arsenic poisoning in a family.

A family of six members is reported, who accidently consumed arsenic, during preparation of indigenous medicine in their home, and developed arsenic poisoning.

Periodic limb movement disorder : a clinical and polysomnographic study.

Periodic limb movement disorder (PLMD) is one of the commonest neurological disorders and causes significant disability, if left untreated. However, it is rarely diagnosed in clinical practice, probably due to lack of awareness and/or lack of necessary diagnostic facilities. Restless leg syndrome (RLS), aging, pregnancy, uraemia, iron deficiency, polyneuropathy are some of the common causes of secondary PLMD. Clinical presentation, polysomnographic findings and management of six patients of PLMD have been discussed in this report.

Febrile status epilepticus as the first presentation of cortical developmental malformation: report of 2 cases.

Cortical developmental malformations are an important cause for epilepsy. The epileptic disorder usually begins with sporadic seizures, followed by gradually increasing seizure frequency and culminating in epilepsia partialis continua. We report the de-novo occurrence of focal status epilepticus accompanying hyperpyrexia in two male children, aged 18 months and 4 years. One child had prior neuro-developmental delay, while in the other, developmental milestones were normal. In both instances, there were no seizures prior to the presenting neurological illness. Cerebrospinal fluid examination was normal in both the cases. Magnetic resonance imaging revealed extensive pachygyria in one patient and focal cortical thickening in the other. Therefore, cortical developmental malformation may be suspected as an underlying aetiology in children presenting with de-novo status epilepticus and hyperpyrexia.

Seizures after stroke : a prospective clinical study.

Stroke is one of the most common causes of epilepsy in elderly. However, there have been very few prospective studies to define the incidence, pattern and outcome of seizures in stroke. Most studies are based on retrospective analysis of hospital records. Hence, we planned this prospective study to see the clinical, radiological and electroencephalographic characteristics of seizures in stroke and their outcome, from a north Indian tertiary care centre. Over a span of approximately 6 years, 269 consecutive patients with stroke were studied and followed up. Thirty-five (13%) of these developed seizures, primarily related to stroke, during mean follow up period of 15.9 months. Twenty of these had infarctions while 15 had haemorrhages. Involvement of the cortical region was seen in most of the patients with seizures. In these patients, 86% of the lesions involved cortical areas exclusively or in addition to subcortical areas on CT scan of the brain. Twenty-seven (77%) developed early seizures, two third of them had immediate post-stroke seizures. None of the patients with early onset seizures developed recurrent seizures or epilepsy, while 50% of late onset seizures developed epilepsy. No specific EEG pattern was found in those who later developed epilepsy. In the present study, early onset seizures after stroke were rather common and did not affect outcome and did not recur even when not treated with anti-epileptics. Late onset seizures were less common but were associated with recurrent seizures.

Cysticercus immunoblot assay in patients with single, small enhancing lesions and multilesional neurocysticercosis.

A single, small (< 20 mm), ring or disc shaped contrast enhancing lesion located at the cortical-subcortical junction with minimal or no surrounding edema on computed tomography is the commonest mode of presentation of neurocysticercosis in the Indian subcontinent. Serum samples of 37 patients with these single, small enhancing lesions (SSEL's) and five patients with typical multilesional parenchymal neurocysticercosis were tested by the electro-immunoblot transfer (EITB) assay and the enzyme linked immunosorbent assay (ELISA). EITB was positive in 18 patients (48.64%) and ELISA was positive in 21 patients (56.76%) with SSEL's. On the other hand EITB was positive in all five patients (100%) and ELISA was positive in four patients (80%) with multilesional neurocysticercosis. The low sensitivity of the EITB in the SSEL's is probably linked to an insufficient immune stimulation provided by a single cysticercus cyst.