Recurrent hypothermia with hyperhidrosis in two siblings: familial Shapiro syndrome variant
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A 21-year-old woman was referred for recurrent (up to 2 times per month) episodes of generalized hyperhidrosis and hypothermia with a core temperature fluctuating between 32 and 35°C, lasting from 2–3 h. During these attacks, the woman sweated profusely and felt faint; her skin was pale and cool and sinus bradycardia (<50 bpm) was observed. At admission, blood chemistry and cell count were unremarkable. Ictal EEGs, ECG Holter, and echocardiography were unremarkable. Brain MRI revealed the total absence of the corpus callosum (Fig. 1b). The patient had normal mental status and did not display facial dysmorphisms. No neurologic or physical abnormalities were noted. Endocrine evaluation of hypothalamic-pituitary axis, thyroid, adrenals, and gonads was unremarkable. Electroencephalograms and lack of response to anticonvulsants (levetiracetam 1,000 mg twice/day) excluded the epileptic origin of the attacks.
KeywordsClonidine Corpus Callosum Levetiracetam Facial Dysmorphisms Physical Abnormality
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© Springer-Verlag 2011