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| LETTER TO EDITOR |
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| Year : 2011 | Volume
: 59
| Issue : 1 | Page : 130-131 |
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
| Date of Submission | 28-Oct-2010 |
| Date of Decision | 30-Oct-2010 |
| Date of Acceptance | 06-Nov-2010 |
| Date of Web Publication | 18-Feb-2011 |
Correspondence Address:
Babarao Darku
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.76868
How to cite this article:
Darku B, Kalra P, Prasad C, Yadav R. Episodic hyperhydrosis with corpus callosum agenesis: A rare case of Shapiro syndrome. Neurol India 2011;59:130-1 |
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. | Figure 1: (a, b) Axial T1W and T2W images show the absence of corpus callosum and widely separated and dilated posterior horn of lateral ventricle (colpocephaly). (c, d) Axial T1W and T2W images show the absence of corpus callosum with sunray pattern of orientation of sulci and gyri
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 | Figure 2: Coronal T2W images show the absence of corpus callosum with "Viking helmet" appearance of lateral ventricle
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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 | |  |
| 1. | Shapiro WR, Williams GH, Plum F. Spontaneous recurrent hypothermia accompanying agenesis of the corpus callosum. Brain 1969;92:423-36. 
[PUBMED] [FULLTEXT] |
| 2. | Summers GD, Young AC, Little RA, Stoner HB, Forbes WS, Jones RA. Spontaneous periodic hypothermia with lipoma of the corpus callosum. J Neurol Neurosurg Psychiatry 1981;44:1094-9.
[PUBMED] [FULLTEXT] |
| 3. | Sanfield JA, Linares OA, Cahalan DD, Forrester JM, Halter JB, Rosen SG. Altered norepinephrine metabolism in Shapiro's syndrome. Arch Neurol 1989;46:53-7.
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| 4. | Arkader R, Takeuchi CA. Shapiro syndrome with hypothalamic hypothyroidism. Arq Neuropsiquiatr 2008;66:418-9.
[PUBMED] [FULLTEXT] |
| 5. | Tambasco N, Corea F, Bocola V. Subtotal corpus callosum agenesis with recurrent hyperhidrosis-hypothermia (Shapiro syndrome). Neurology 2005;65:124.
[PUBMED] [FULLTEXT] |
[Figure 1], [Figure 2]
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