The term ‘paroxysmal autonomic instability with dystonia’ was first given for a group of symptoms comprising diaphoresis, intermittent agitation, hypertension, hyperthermia, tachycardia, tachypnea, and extensor posturing. This usually occurs as a complication of severe brain injury and is infrequently reported in cases of cerebral infection. We report a 1-year-old child with intracranial tuberculoma with symptoms of paroxysmal autonomic instability with dystonia. Pertinent literature is reviewed emphasizing pathophysiology and available treatment options.

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