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Over the last two decades, an increase in the incidence of PCNSL cases has been reported in the West, both among immunosuppressed and immunocompetent patients. The present study was undertaken to assess the trend of incidence of PCNSL cases in India. To the best of our knowledge, only a single such report is available from India. All biopsy proven PCNSL cases obtained from the Neurosurgical databases of two large referral hospitals, one in Northern India (AIIMS, New Delhi) and another in Southern India (NIMHANS, Bangalore) from the period 1980 to 2003, were reviewed. Immunophenotyping was done and relevant clinical details collected. Appropriate statistical analysis was done to assess any change in trend of incidence or age at presentation. PCNSL cases constituted 0.95% and 0.92% of the total intracranial neoplasms at AIIMS and at NIMHANS, respectively. The mean age for cases diagnosed at AIIMS was 44.35 years, while that for NIMHANS was 39.51 years. Statistical analysis to evaluate any change in trend either of incidence or of age at presentation, over the study period, did not reveal any significant change. All the cases occurred in immunocompetent patients, except one case of HIV positive at NIMHANS, and one case of renal transplant at AIIMS. Frontal lobe was the most common site of involvement. Majority of the cases were diffuse, high grade, large cell lymphoma, B-cell immunophenotype. Thus, this multicentric hospital based study did not reveal any increase in incidence of PCNSL cases in India over the past 24 years. Further, in contrast to the West, majority of the cases in this Indian study were immunocompetent and a decade younger than in the West. The association of PCNSL with HIV/AIDS has been low in India, possibly due to early death in AIDS on account of opportunistic infections.

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Object Intraventricular and paraventricular tumors resulting in hydrocephalus commonly require a CSF diversion procedure. A tumor biopsy can often be performed concurrently. Although the tissue samples obtained during endoscopic biopsy procedures are small, a diagnosis can be made in most cases. In the present study the authors analyzed the efficacy of concurrent endoscopic biopsy and CSF diversion procedures using a single bur hole and trajectory. Methods Eighty-seven patients with intraventricular and paraventricular tumors were treated with endoscopic biopsy and CSF diversion procedures using a rigid rod-lens endoscope or a rigid fiberscope during a 10-year period. All patients underwent a tumor biopsy and an endoscopic third ventriculostomy (ETV), aqueductal stenting (AS), or ventriculoperitoneal (VP) shunting, depending on the tumor location and site of obstruction. A single bur hole for both procedures was used in all patients. Results Among the 87 patients, the biopsy was diagnostic in 72 (83%) and merely suggestive in 7 (8%); in 8 patients (9%) the sample was nondiagnostic. Among the 22 patients who underwent an initial endoscopic biopsy and subsequent procedures, the specimen obtained at the second surgery was concordant with the initial endoscopic biopsy sample in 13 patients; it was somewhat similar in 4 patients. In the other 5 patients, either a microsurgical or stereotactic approach was used to correctly diagnose the pathology. Fifty-five patients were considered for endoscopic CSF diversion procedures; an ETV was performed in 52 patients and AS in 2. An ETV could not be performed in 3 patients for technical reasons. A VP shunt was inserted in 32 patients, with 25 undergoing shunt placement at the same time as the ETV and 7 at a later date. Significant bleeding was encountered in 3 patients during the tumor biopsy and in 1 patient during the ETV. The ETV failed in 1 patient during the follow-up, and a repeat ETV was required. Conclusions Endoscopic biopsy sampling and a concurrent CSF diversion procedure through a single bur hole and trajectory can be considered for intraventricular tumors. The overall success rates of 83% for the biopsy procedure and 86% for the ETV indicate that the procedures are beneficial in the majority of cases. A concordance rate of 75% was found in patients who underwent an initial biopsy procedure and a subsequent microsurgical approach for tumor excision.

We report the clinical, electrophysiological, radiological and morphological features in a series of 12 patients of histopathologically confirmed cases (infantile, juvenile and adult onset) of neuronal ceroid lipofuscinosis (NCL) observed from 1979 to 1998 at National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore (South India). The commonest type of NCL was juvenile (n = 8, 67%) while infantile and adult forms were two each (n = 2, 16.8%). The age at presentation ranged from 2 to 45 years (mean--12.6, 14.3 years; median--7 years; M:F ratio of 2:1). Four patients (33%) had positive family history and five patients had history of consanguineous parentage (41.6%). The commonest presenting symptoms were regression of milestones (83.3%) and/or seizures, myoclonus (83.8%) followed by involuntary choreiform movements (50%), visual loss (41.6%), ataxia (33.3%) and abnormal behaviour (16.6%). Neuro-ophthalmological abnormalities like optic atrophy (50%), macular degeneration (33.3%) and retinitis pigmentosa (8.3%) were seen in two thirds. Nerve conduction studies (n = 4) revealed abnormalities in two, suggestive of sensorimotor neuropathy. Scalp EEG (n = 9) showed slowing of background activity (BGA) of varying degrees with paroxysmal bursts of seizure discharges in majority. Cranial CT scan (n = 4) revealed varying degrees of diffuse atrophy. Diagnostic brain biopsy was carried out in 11 and brain was examined at autopsy in 1 case. Histological examination revealed characteristic PAS and Luxol Fast Blue (LFB) positive, autofluorescent (AF) intracellular ceroid material, both in neurons and astrocytes in the grey matter. Electron microscopy (n = 5) revealed curvilinear (n = 4), lamellar (n = 2) and electron dense (n = 2) inclusions in neurons, astrocytes and vascular endothelial cells. To conclude, this neurodegenerative disease had varied but characteristic clinical presentations and required histopathological confirmation of diagnosis.

Infection with human immunodeficiency virus (HIV) clade C is the most common HIV infection worldwide, yet its impact on the nervous system remains largely unknown. Autopsy studies from regions affected by this virus are scarce, and HIV dementia has only rarely been reported from these countries. Most patients who develop neurologic complications die of opportunistic infections. We thus conducted a neuropathologic study from a single institution in India to characterize the HIV-infected cells in the inflammatory infiltrates in a total of 15 cases (5 patients each who died of either CNS toxoplasmosis, tuberculosis, or cryptococcal meningitis). Nearly, all patients had HIV-infected cells in the brain, although these cells were most abundant in patients with toxoplasma encephalitis. Interestingly, none of the patients had any multinucleated giant cells. HIV-infected cells were found in the parenchyma, perivascular regions, and choroid plexus and found infiltrating the parenchyma from the meninges, suggesting multiple portals of entry into the brain. These findings suggest the possibility that patients, even if successfully treated for an opportunistic inflection, may be at high risk of developing HIV encephalitis and subsequent dementia.

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Dysferlinopathy was the second most common identifiable cause (21%) of LGMD next to sarcoglycanopathies (27%).