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Pilomotor seizures: an autonomic semiology of limbic encephalitis?

Rodrigo Rocamora1, Juan L Becerra2, Pilar Fossas3

  • 1Epilepsy Monitoring Unit, Department of Neurology, Hospital del Mar, Barcelona, Spain.

Seizure
|June 4, 2014
PubMed
Abstract

Insights

Ictal piloerection, often overlooked, is linked to limbic encephalitis in refractory temporal lobe epilepsy (TLE). This study highlights autoimmune causes for pilomotor seizures, emphasizing comprehensive diagnostics for long-standing TLE.

Area of Science:

  • Neurology
  • Epileptology
  • Immunology

Background:

  • Ictal piloerection is an uncommon seizure manifestation, frequently attributed to temporal lobe epilepsy (TLE), but with reported frontal or hypothalamic origins.
  • Etiologies for piloerection include mesial temporal sclerosis, tumors, and post-traumatic or cryptogenic causes.

Observation:

  • This study retrospectively analyzed 766 patients with refractory epilepsy undergoing video-EEG monitoring (VEEGM).
  • Five patients (0.65% prevalence) exhibited ictal piloerection as their primary seizure semiology, all diagnosed with TLE.

Findings:

  • The identified etiology in all pilopilomotor seizure cases was limbic encephalitis, with specific antibody associations (LGI1, anti-Hu, Ma2).
  • Three patients presented with hippocampal sclerosis, a common finding in TLE.

Implications:

  • This research identifies a significant, previously under-recognized association between pilomotor seizures and autoimmune limbic encephalitis.
  • It underscores the importance of investigating autoimmune etiologies in patients with refractory TLE, even with typical MRI findings of hippocampal atrophy.
Keywords:
AutoimmuneAutonomicLimbic encephalitisPilomotorSeizures

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