Journal of pediatric neurosciences
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Sjogren-Larsson syndrome is an autosomal recessive disorder characterized by defective activity of fatty aldehyde dehydrogenase. It presents as a triad of congenital ichthyosis, spastic diplegia, and mental retardation. The pathology behind this syndrome is the failure of degradation of fatty aldehydes. This case is presented for its rarity.
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Hydrocephalus is a most common complication of tubercular meningitis (TBM). Relieving hydrocephalus by ventriculoperitoneal shunt (VPS) placement has been considered beneficial in patient in Palur Grades II or III. The role of VPS placement in those of Grades III and IV is controversial, and general tendency is to avoid its use. Some authors have suggested that patient in Grades III and IV should receive a shunt only if their condition improves with a trial placement of external ventricular drain (EVD). However, recent studies suggest that VPS may be undertaken without the trial of an EVD. Our study prospectively evaluates the role of direct VPS placement in patient in Grades III and IV TBM with hydrocephalus (TBMH). ⋯ This study demonstrates that direct VPS surgery could improve the outcome of Grades III and IV TBMH. Despite poor grade at admission, 80% patients in Grade III and 20% patients in Grade IV had a good outcome at 3 months follow-up. Direct VPS placement is a safe and effective option even in a patient in Grades III and IV grade TBMH with a low complication rate.