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A case of paraganglioma of parasellar origin in a 7-year-old girl is described. She presented with hemibase syndrome with involvement of a majority of the left cranial nerves. Three times in the past 2 years she had recurrent ophthalmoplegia with complete recovery. She was treated surgically and with postoperative radiotherapy. The site of origin of this paraganglioma as well as the clinical presentation are uncommon features in this case.

The objective of this study was to elucidate an association between Apo- Ee4 allele and CSF biomarkers Aβ42 and tau for the diagnosis of Alzheimer's Disease (AD) patients. Aβ42 and tau protein concentrations in CSF were measured by using ELISA assays. The levels of Aβ42 were found to be decreased where as tau levels increased in AD patients. Moreover in AD patients Apo-Ee4 allele carriers have shown low Aβ42 levels (328.86 ± 99.0 pg/ml) compared to Apo-Ee4 allele non-carriers (367.52 ± 5 7.37 pg/ml), while tau levels were higher in Apo-Ee4 allele carriers (511 ± 44.67 pg/ml) compared to Apo-Ee4 allele non-carriers (503.75 ± 41.08 pg/ml). Combination of Aβ42 and tau resulted in sensitivity of 75.38% and specificity of 94.82% and diagnostic accuracy of 84.30% for AD compared with the controls. Therefore low Aβ42 and elevated tau concentrations in CSF may prove to be a better diagnostic marker for AD along with the Apo-Ee4 allele.

A total of 251 young patients (less than 40 years) with stroke were admitted during a 7-year period and investigated. These constituted 30% of the total stroke patients admitted to this hospital. Among the young, 63% of strokes were of nonembolic occlusive group. Hemorrhagic strokes were observed in 26% cases. This study revealed that concurrent infection, nonspecific arteritis, diabetes mellitus and hypertension contribute very little to the stroke in younger population. However, cholesterol, beta-lipoproteins, triglycerides, platelet adhesiveness and platelet aggregation were significantly increased in some of the clinical groups.

Forty four cases of the neurological complications of acute haemorrhagic conjunctivitis (AHC) seen in India during 1981 epidemic are reported. The disease predominantly affected adult males. The preceding attack of AHC, a latent period, prodromal symptoms of fever, myalgia and root pains followed by acute onset of lower motor neurone paralysis of limbs and/or cranial nerves formed the classical picture of neurological involvement. The recovery was poor and nearly half of the patients remained severely handicapped. Electrophysiological studies showed early appearance of widespread fibrillations and fasciculations, large polyphasic potentials of increased amplitude and reduced interference pattern. Nerve conduction studies were normal in most of the cases. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis and rise in protein content. Significant antibody titres against enterovirus type 70 (EV 70) were demonstrated in the serum and the CSF. HLA studies showed low occurrence of A2 and B15 HLA antigens. Muscle biopsies revealed neurogenic atrophy and sural nerve biopsies were histologically unremarkable. The similarities of this disease with poliomyelitis and its pathogenesis are discussed.

The cases of identical twins with ataxia telangiectasia, early intellectual impairment and progressive spasticity are reported. Immunological tests revealed reduced levels of serum and salivary IgA, increased B cells, reduced T cells and raised alpha-fetoprotein. CT scan performed in one of the twins was normal. The pathogenesis of the spasticity is discussed.

Background More than three billion populations are living under the threat of Japanese encephalitis in South East Asian (SEA) countries including India. The pathogenesis of this disease is not clearly understood and is probably attributed to genomic variations in viral strains as well as the host genetic makeup. The present study is to determine the role of polymorphism of TNF-alpha promoter regions at positions -238G/A, -308G/A, -857C/T and -863C/A in the severity of Japanese encephalitis patients. Methods Total of 142 patients including 66 encephalitis case (IgM/RT-PCR positive), 16 fever cases (IgM positive) without encephalitis and 60 apparently healthy individuals (IgG positive) were included in the study. Polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) using site specific restriction enzymes were implemented for polymorphism study of TNF alpha promoter. Results Following the analysis of the digestion patterns of four polymorphic sites of the TNF- alpha promoter region, a significant association was observed between the allele -308A and -863C with the patients of Japanese encephalitis. Conclusions TNF- alpha 308 G/A has been shown to be associated with elevated TNF- alpha transcriptional activity. On the other hand, polymorphism at position -863C/A in the promoter region has been reported to be associated with reduced TNF- alpha promoter activity and lower plasma TNF levels. As per the literature search, this is the first study to identify the role of TNF- alpha promoter in JE infection. Our results show that subjects with - 308A and -863C alleles are more vulnerable to the severe form of JE infection.

Acute cerebellar ataxia as an isolated neurological manifestation of enteric fever is very rare. Three cases of acute cerebellar ataxia associated with enteric fever are reported. The diagnosis of enteric fever was confirmed by positive blood culture, strongly positive Widal test and rising antibody titres. The major clinical features were rapid development of gait ataxia, limb ataxia and dysarthria. None of the patients had altered sensorium. The cerebellar involvement was noticed on the second or third day of fever which progressed for one to two days. The symptoms remained static for one to two weeks and thereafter all the patients showed gradual recovery in a few weeks. Acute onset of cerebellar lesion, self limiting course and cerebrospinal fluid pleocytosis suggest par- or post-infectious demyelinating pathology in these patients, who were not related to each other.