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Genetic and Rare Diseases Information Center (GARD)

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Shapiro syndrome

Other Names for this Disease
  • Recurrent spontaneous hypothermia with hypoplasia of the corpus callosum
  • Shapiro's syndrome
  • Spontaneous periodic hypothermia
  • Spontaneous periodic hypothermia syndrome
  • Spontaneous recurrent hypothermia syndrome
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Your Question

My father is 63 years old and suffers from Shapiro syndrome. So far no doctor or treatment plan has helped. The condition and length of time of the attacks continue to get worst.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Shapiro syndrome?

Shapiro syndrome is a rare disease affecting about 50 people worldwide that is typically characterized by recurrent episodes of excessive sweating and hypothermia and the agenesis of the corpus callosum. The duration and frequency of the episodes vary from person to person, with some episodes lasting hours to weeks and occurring from hours to years; the reason for the variations in the episodes is not yet known. The cause of the condition is currently unknown; however, a "resetting" of the temperature of the body to a lower level has been suggested. Although different treatment options have been attempted in some patients, the treatments have been unsuccessful or of doubtful efficacy because of the small number of individuals that have been documented as having this condition.[1][2][3]
Last updated: 7/13/2009

What are the signs and symptoms of Shapiro syndrome? Do the symptoms tend to worsen with time?

Shapiro syndrome generally consists of three findings: spontaneous periodic hypothermia, excessive sweating, and agenesis of the corpus callosum. However, there has been a documented case of a 4-year-old girl with Shapiro syndrome who did not have agenesis of the corpus callosum. Additionally, there have been some patients who also produce excessive amounts of urine  (polyuria) and have experienced excessive thirst (polydipsia).[3]  Given that some people with Shapiro syndrome do not respond well to the various treatment options available for the condition, the symptoms may worsen with time for some people.  
Last updated: 7/13/2009

What treatment options have been attempted for Shapiro syndrome?

Evaluating effective treatment options for Shapiro syndrome can be difficult because of the limited number of diagnosed cases, the periodic nature of the disease, and other factors. Nonetheless, the following have been attempted and have resulted in varying responses: anticonvulsants, clonidine, cyproheptadine, glycopyrrolate, bromocriptine, chlorpromazine, or sympathectomy.[3] It is recommended that treatment options be discussed with a health care provider. Only a patient's health care provider can determine the appropriate course of treatment.
Last updated: 7/13/2009