PCNSL presents most commonly in the sixth decade with focal neurological deficit, behavioral symptoms and cognitive decline. High grade DLBCL is the commonest histological subtype. Steroids should ideally be withheld until biopsy as they may confound the diagnosis. Most immunocompetent patients respond well to high dose MTX-based chemotherapy with/without radiation. High IELSG scores correlate with worse prognosis in patients with PCNSL.

The leukodystrophies are familial disorders with onset usually in infancy or childhood. The clinical features consist of motor dysfunction with varying degree of cognitive decline. Magnetic Resonance Imaging (MRI) has helped to identify and characterize these disorders. In some leukodystrophies, biochemical enzymatic and genetic defects have been identified. The commonest leukodystrophy seen in India is Megalencephalic Leukodystrophy with subcortical cysts. The essential features consist of large head, mild pyramidal and cerebellar dysfunction, and occasional seizures. MRI studies show extensive white matter changes with temporal cysts. It is common in the Agarwal community in India. An identical mutation in exon 2 of the MLC 1 gene has been identified in this community suggesting a founder effect.

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache (OH), low cerebrospinal fluid (CSF) pressure, and diffuse pachymeningeal gadolinium enhancement (DPME). We present here the case studies of two patients. One patient demonstrated a CSF leak in the mid-thoracic region, and recovered completely with conservative treatment. The other patient in whom leak could not be demonstrated, developed dementia, rapidly worsening encephalopathy, and became comatose, necessitating urgent epidural blood patch (EBP) with 25 cc of autologous blood, after which immediate and complete symptomatic relief was obtained. A second EBP was required a few days later and also provided complete and sustained clinical benefit, without subsequent recurrence. Both patients had OH and showed bilateral subdural fluid collections, DPME and “sagging” of brain on MRI. A high index of suspicion, recognizing the orthostatic nature of headache, and typical findings on contrast enhanced MRI should point to the diagnosis of SIH. EBP can be effective treatment in patients unresponsive to conservative measures.

Mitoxantrone, as an initial therapy, decreases clinical exacerbations and disability progression, and has a reasonable safety profile in Indian patients with MS and NMO.

No abstract