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Diseases

Genetic and Rare Diseases Information Center (GARD)

Shapiro syndrome


Other Names for this Disease
  • Shapiro's syndrome
  • Recurrent spontaneous hypothermia with hypoplasia of the corpus callosum
  • Spontaneous periodic hypothermia syndrome
  • Spontaneous recurrent hypothermia syndrome
  • Spontaneous periodic hypothermia
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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 neurological disease characterized by recurrent episodes of excessive sweating and hypothermia along with agenesis of the corpus callosum.[1][2][3] 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, suggested possible mechanisms include hypothalamic dysfunction, neurochemical abnormalities, inflammatory processes, and epileptic activity.[1] Treatment aims to address the symptoms present in each individual and may include re-warming with a warm blanket and the use of medications with varying success.[1][3]
Last updated: 6/6/2016

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 (hyperhidrosis), and agenesis of the corpus callosum.[2][3] A variant form occurs without agenesis of the corpus callosum.[2] Additional symptoms may include nausea and vomiting; a sense of weakness, incoordination and gait unsteadiness; drowsiness, mild bradycardia; and rarely, excessive amounts of urine (polyuria) and excessive thirst (polydipsia).[1][4] Episodes of hypothermia and hyperhidrosis may last for hours to weeks and recur for hours to years.[1] 

Last updated: 6/6/2016

How might Shapiro syndrome be treated?

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 levels of success: oxcarbazepine, carbamazepine, clonidine, cyproheptadine, glycopyrrolate, bromocriptine, chlorpromazine, beta blockers, or sympathectomy.[1][4] It is recommended that treatment options be discussed with a health care professional. Only a patient's personal health care provider can determine the appropriate course of treatment.
Last updated: 6/6/2016

References
Other Names for this Disease
  • Shapiro's syndrome
  • Recurrent spontaneous hypothermia with hypoplasia of the corpus callosum
  • Spontaneous periodic hypothermia syndrome
  • Spontaneous recurrent hypothermia syndrome
  • Spontaneous periodic hypothermia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.