Paroxysmal hypothermia: causes

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Paroxysmal hypothermia: causes. This is associated with agenesis of the corpus callosum. Fully referenced neurology checklist.

Major causes: Shapiro syndrome

  • This is associated with agenesis of the corpus callosum
  • It may be linked with genetic mutations: SCN, KCNQ, KCNA, CHRNA, GABRB
  • Hypoglycaemia has been reported
  • Holoprosencephaly has been reported

Major causes: others

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Vascular causes

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Infective causes

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Iatrogenic causes

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Spontaneous or idiopathic: possible mechanisms

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Other causes

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References

  1. Goh Y, Lee XY, Chin AXY, Jing M, Makmur A, Quek AML. Shapiro syndrome: a cause of episodic hyperhidrosis, hypothermia and altered mental status. QJM 2023 (Online ahead of print).
  2. Ren L, Gang X, Yang S, Sun M, Wang G. A new perspective of hypothalamic disease: Shapiro's syndrome. Front Neurol 2022; 13:911332.
  3. Lewis C, Freeman N, Gupta N. Hypoglycemia as a manifestation of Shapiro syndrome. Cureus 2022; 14:e23120. 
  4. Evers Smith CM, Creed E. Case report of dysmorphic physical findings in male with Shapiro syndrome. Cureus 2022; 14:e26064.
  5. Hemelsoet DM, De Bleecker JL. Post-traumatic spontaneous recurrent hypothermia: a variant of Shapiro's syndrome. Eur J Neurol 2007; 14:224-227. 
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