Pantothenate kinase associated neurodegeneration (PKAN) is an uncommon degenerative disease of the basal ganglia caused by mutations in the PANK2 gene. We describe a 19-years-old man with clinically and radiologically diagnosed case of PKAN, who presented with generalized tonic clonic seizures 2 years preceding other classical extrapyramidal features of the disease. PKAN presenting with seizure has not, to our knowledge, been reported previously.

The myotonic dystrophies (DMs) are autosomal dominant disorders, subdivided by their genetic defect into DM1and DM2 and characterized by gradually progressive muscle weakness, myotonia, cataracts, endocrine disturbances and functional abnormalities of the cardiorespiratory and gastrointestinal tract. We report the unusual coexistence of spinal cord meningioma in a patient of myotonic dystrophy. As per our knowledge, this is the second case of a meningioma reported with myotonic dystrophy in the literature.

Background: Tuberculous meningitis (TBM) remains to be one of the most fatal central nervous system infections. The exact pathogenesis of TBM at cellular level remains unclear. In this study, we assessed the cytokine levels in the serum and cerebrospinal fluid (CSF) of TBM patients and determined their correlation with the disease activity. Methods: The levels of tumor necrosis factor-α (TNF-α) and interleukin-1β (IL-1β) were measured by enzyme linked immunosorbant assay (ELISA) in both serum and CSF of 38 patients at baseline, and in 17 of these patients at 1 and 6 month of follow-up. Clinical examination and imaging was performed at baseline and on follow-ups. Results: There was a remarkable rise in the levels of serum and CSF TNF-α and IL-1β in TBM patients as compared to age and sex matched controls (p<0.05). A significant correlation was found between cytokine levels and stages of TBM (p< 0.05). TNF-α levels in both serum and CSF and IL-1β levels in serum were found to be significantly higher in those patients who died than those who survived and had better outcome. TNF-α was higher in patients who developed tuberculoma on follow-up than those who did not (p<0.05). The cytokine levels progressively declined over time but remained detectable till 6 months in most patients. Conclusions: The higher levels of TNF-α and IL-1β were associated with poor outcome in TBM. The higher cytokine levels in patients developing tuberculoma on antituberculous therapy and steroids suggests that these patients may benefit from immunomodulation agents like anti-TNF-α antibody.
Tuberculosis, Meningeal ; Cytokines