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QJM 2008 101(1):61-62; doi:10.1093/qjmed/hcm106
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© The Author 2008. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Shapiro syndrome

Dr Chetan Shenoy

Guthrie/Robert Packer Hospital, Sayre, Pennsylvania, USA.

email: chetan_shenoy{at}yahoo.com

This previously healthy 34-year-old man presented with lethargy, confusion, episodic chills and profuse sweating of two months duration, associated with a 9 kg weight loss. Physical examination revealed delirium, significant diaphoresis, hypothermia with a core temperature of 89.9°F (32.1°C) and signs of dehydration. Laboratory testing revealed electrolyte disturbances consistent with dehydration. The leukocyte count was normal and the drug screen was negative. A central cause of the hypothermia was suspected and magnetic resonance imaging of the brain was obtained, which revealed the complete absence of the corpus callosum (Figures 1, 3 and 4), leading to a diagnosis of Shapiro syndrome.


Figure 1
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  		Figure 1. Sagittal view of MRI head of our patient showing the absence of corpus callosum.

 

Figure 3
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  		Figure 3. Axial view of MRI head of our patient again showing the absence of corpus callosum.

 

Figure 4
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  		Figure 4. Coronal view of MRI head of our patient showing the lateral ventricles forming a ‘Viking helmet’ or ‘moose head’ appearance due to the absence of corpus callosum.

 
Originally described by Shapiro and Plum in 1967, Shapiro syndrome is a rare syndrome of episodic hypothermia and hyperhidrosis associated with agenesis of the corpus callosum. Only about 30 cases of the syndrome have been described in the English literature so far. The exact pathophysiologic mechanism for this syndrome is not understood. Postulated mechanisms include ‘resetting’ of the hypothalamic thermostat to a lower level due to degenerative processes, neurochemical (norepinephrine, melatonin) dysfunction and inflammatory processes leading to a possible epileptic focus or focus of periodic dysfunction. Clonidine has been described to help in remission of symptoms in a few recent reports and is proposed to act by regulating the hypothalamic dysfunction.

Our patient was treated with 100 µg of clonidine every 8 h and vigorous intravenous fluids. His mental status and electrolyte disturbances resolved over the next three days and he had no further episodes of hypothermia or hyperhidrosis during the hospital stay. Although very rare, it is important to recognize the Shapiro syndrome.


Figure 2
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  		Figure 2. Normal sagittal view of MRI head.

 


Figure 5
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  		Figure 5. Normal coronal view of MRI head.

 

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This Article
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