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Related Experiment Videos

Segmental hyperhidrosis in two siblings with Chiari type I malformation

L J Stovner1, O Sjaastad

  • 1Department of Neurology, Trondheim University Hospital, Norway.

European Neurology
|January 1, 1995
PubMed
Summary

Two siblings with Chiari type I malformation experienced segmental hyperhidrosis and sensory loss. This suggests a link between the malformation and sympathetic nervous system dysfunction, impacting thermoregulation.

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Area of Science:

  • Neurology
  • Neuroscience
  • Medical Imaging

Background:

  • Chiari type I malformation is a structural defect in the cerebellum.
  • Sympathetic nervous system dysfunction can manifest in various ways, including altered sweating patterns.
  • Dissociated sensory loss involves the loss of specific sensory modalities while others remain intact.

Observation:

  • Two siblings presented with unilateral segmental hyperhidrosis and dissociated sensory loss.
  • Both siblings were diagnosed with Chiari type I malformation via MRI.
  • The male sibling also had a cervical syrinx, but its level did not correlate with the affected sensory areas.

Findings:

  • The female sibling, without a syrinx, exhibited hyperhidrosis and sensory loss in the head, neck, upper trunk, and arm.

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  • Anisocoria (unequal pupil size) was noted, with the larger pupil on the same side as the hyperhidrosis.
  • These symptoms in both siblings suggest a potential link between Chiari malformation and autonomic dysfunction.

    • Implications:

    • The findings suggest that Chiari type I malformation may cause disinhibition of sympathetic neurons.
    • Lesions interrupting descending inhibitory pathways could disrupt normal thermoregulatory sweating.
    • This rare disorder highlights the complex interplay between structural brain abnormalities and autonomic nervous system regulation.