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PILOERECTION AS A RARE MANIFESTATION IN EPILEPSY PATIENTSJulio Cesar Hernandez M.D.
Introduction Subjective: Rare ictal manifestation
overlooked in epilepsy patients Objective: Evidence of piloerection is
seldom available due to limitations of video resolution and the impracticality of continuous exposure of involved body areas in the EMU
Ayman M and Abou-Khalil B. Epileptic Disord 2012; 14 (1): 76-9
Reported in patients with temporal lobe epilepsy
420 patients with temporal lobe farmacorresistant epilepsy, 1.2% had piloerection (Stefan et al., 2002)
12 of 14 patients had temporal lobe epilepsy (Loddenkemper et al., 2004)
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883
Ayman M and Abou-Khalil B. Epileptic Disord 2012; 14 (1): 76-9
Rarely associated with frontal lobe epilepsy (Seo et al ., 2003)
Classified as a subtype of autonomic seizure and rarely as primarily ictal manifestation
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883
3500 patients who underwent vEEG monitoring at CCF between 1994 and 2001
The patient population: 75% with focal epilepsy (temporal: 48%; extratemporal: 19%; non-classifiable focal: 8%), 5% with generalised epilepsy, 1% with multifocal epilepsy, and 19% with non-epileptic seizures
Results 14 right-handed patients with ictal piloerection Ictal piloerection was documented in 9 cases
by observation and video recordings and 5 cases by history
12 cases (85%), the EZ was located in the temporal lobe either by EEG, neuroimaging or by seizure freedom after temporal lobectomy
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883
7 patients (50%), the ictal EEG onset was located in the RH and one patient independently from LH and RH
9 patients (64%) experienced bilateral ictal piloerection and 5 patients had unilateral (or initially unilateral) ictal piloerection
5/9 patients (56%) with bilateral piloerection had right TLE. 4/5patients (80%) with unilateral (or initially unilateral) ictal piloerection had the ictal onset in the hemisphere ipsilateral to the side of piloerection
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883
Associated clinical findingsPreceeding symptoms
Following symptoms
Autonomic findings
Fear (3 patients), nausea (3 patients), loss of consciousness (3 patients), olfactory auras, gustatoryauras, cephalic auras, loneliness, and automatisms
Automatisms (5 patients),loss of consciousness (2 patients), nausea,unilateral tonic seizures, cephalic auras, bilateral somatosensory aura, andperioral or nasal paresthesias
Shortness of breathand hyperventilation (6 patients), tachycardia (4patients), ictal cold (3 patients), diaphoresis (3), urinary urge (3 patients), pallor,Salivation, and hyperlacrimationn=14
9 patients were aware of the piloerection during and after the seizure, whereas 5 patients did not notice the ‘‘goosebumps’’Loddenkemper et al. J Neurol Neurosurg Psychiatry
2004;75:879–883
Literature review of 18 patients with documented unilateral (or unilateral onset of) ictal piloerection
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883
Left hemispheric predominance(?)
MRI Lesion in 11/14 cases: 4 patients hippocampal atrophy 2 had hippocampal atrophy associated
with FP encephalomalacya in one and multiple bilateral tubers due to tuberous sclerosis in another
8 had other lesionsLoddenkemper et al. J Neurol Neurosurg Psychiatry
2004;75:879–883
Generator Insula and medial prefrontal cortex, central nucleus of the
amygdala, preoptic region, hypothalamus, midbrain periaquaeductal grey matter, the pontine parabrachial region, the nucleus of the solitary tract, and the intermediate reticular zone of the medulla
Electrical or pharmacological stimulation in humans and animals at multiple sites: insula, hippocampus, amygdala, hypothalamus, midbrain reticular core, and medial prefrontal cortices
Others: parahypocampal formation and anterior cingulateLoddenkemper et al. J Neurol Neurosurg Psychiatry
2004;75:879–883
A. Interictal B. Ictal
Masnou et al. Epileptic Disorders. Volume 8, Number 3, 232-7, September 2006
F7, T1, Sp1 F7, T1, Sp1
Conclusions: Piloerection as ictal phenomena
Rare ictal manifestation Rare independently, frequently associated to
other ictal manifestations Predominantly in TLE (specially insula) Bilateral piloerection localize EZ in RH (?), LH
predominance (?), unilateral piloerection localize EZ ipsilateral to the side of the piloerection (84% of cases)
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