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

A 62-year-old woman presented with episodic sweating and shivering with red uced core temperature. Brain MRI demonstrated a basal forebrain malformatio n. Physiologic testing included EEG, SPECT, heat challenge, and autonomic t esting. Glycopyrrolate aborted spells and raised core temperature. Hypothal amic dysregulation is likely the primary pathophysiology in the setting of other forebrain anomalies. These findings expand the structural abnormaliti es and treatment options within the temperature dysregulating conditions of Shapiro's syndrome and "diencephalic epilepsy.