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Case Reports
. 2001 Jan 23;56(2):254-6.
doi: 10.1212/wnl.56.2.254.

Basal forebrain malformation with hyperhidrosis and hypothermia: variant of Shapiro's syndrome

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Case Reports

Basal forebrain malformation with hyperhidrosis and hypothermia: variant of Shapiro's syndrome

C J Klein et al. Neurology. .

Abstract

A 62-year-old woman presented with episodic sweating and shivering with reduced core temperature. Brain MRI demonstrated a basal forebrain malformation. Physiologic testing included EEG, SPECT, heat challenge, and autonomic testing. Glycopyrrolate aborted spells and raised core temperature. Hypothalamic dysregulation is likely the primary pathophysiology in the setting of other forebrain anomalies. These findings expand the structural abnormalities and treatment options within the temperature dysregulating conditions of Shapiro's syndrome and "diencephalic epilepsy."

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