Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 59, Num. 1, 2011, pp. 130-131

Neurology India, Vol. 59, No. 1, January-February, 2011, pp. 130-131

Letter to Editor

Episodic hyperhydrosis with corpus callosum agenesis: A rare case of Shapiro syndrome

Babarao Darku¹, Pramila Kalra², Chandrajit Prasad³, Ravi Yadav¹

¹ Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India
² Department of Endocrinology, M S Ramaiah Medical College and Hospital, New BEL Road, Bangalore, India
³ Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India
Correspondence Address: Babarao Darku, Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India, docravi20@yahoo.com

Date of Submission: 28-Oct-2010
Date of Decision: 30-Oct-2010
Date of Acceptance: 06-Nov-2010

Code Number: ni11036

PMID: 21339684
DOI: 10.4103/0028-3886.76868

Sir,

A 25-year-old woman presented with recurrent episodic profuse sweating associated with cold intolerance and chills since childhood. Each of such episodes had lasted for 5-10 minutes. There was no history of any aura, cardiac and abdominal complaints. Thirst and appetite were normal and menstrual cycles were regular. Examination showed normal blood pressure, normal higher mental function and cranial nerves. Motor and sensory system were also normal. Axillary temperature recorded during the episodes dropped to 32°C and 34°C. Complete blood picture and blood biochemistry were normal. Autonomic function testing, which included heart rate variability (HRV) parameters and cardiac autonomic function, was normal. Thyroid functions and 5-hydroxy indole acetic acid level were found to be normal. Nerve conduction study and sympathetic skin response (SSR), video-electroencephalographic (VEEG) recording during the attacks were also normal. Magnetic resonance imaging showed agenesis of corpus callosum [Figure - 1] and [Figure - 2]. Patient was managed with tab clonidine 0.1 mg twice a day. On follow-up at 3 months, she reported complete remission from the episodes of hyperhydrosis.

Shapiro syndrome, a rare disorder originally described by Shapiro and Plum in 1967, is characterized by episodic hypothermia and hyperhydrosis associated with agenesis of the corpus callosum. ^[1] Proposed hypotheses to explain the clinical features of this syndrome include changes in the set point of the hypothalamic thermostat, ^[2] increased norepinephrine (NE) release and decreased plasma NE clearance. ^[3] Patient can have associated hypothalamic dysfunction in the form of central hypothyroidism ^[4] and thirst abnormalities. Magnetic resonance imaging of the brain in this syndrome is classical and shows agenesis of corpus callosum. ^[5]

To the best of our knowledge, this is probably the first case report of this syndrome from India. We emphasize the importance of recognition of Shapiro syndrome in the evaluation of episodic hyperhydrosis. Treatment with clonidine is very effective in control of these episodes.

References

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