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

C. J. Klein; Michael H. Silber; J. R. Halliwill; S. A. Schreiner; G. A. Suarez; P. A. Low

doi:10.1212/WNL.56.2.254

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

C. J. Klein, Michael H. Silber, J. R. Halliwill, S. A. Schreiner, G. A. Suarez, P. A. Low

Neurology

Research output: Contribution to journal › Article › peer-review

25 Scopus citations

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".

Original language	English (US)
Pages (from-to)	254-256
Number of pages	3
Journal	Neurology
Volume	56
Issue number	2
DOIs	https://doi.org/10.1212/WNL.56.2.254
State	Published - Jan 23 2001

ASJC Scopus subject areas

Clinical Neurology

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title = "Basal forebrain malformation with hyperhidrosis and hypothermia: Variant of Shapiro's syndrome",

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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AU - Schreiner, S. A.

AU - Suarez, G. A.

AU - Low, P. A.

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