This document discusses piloerection as a rare manifestation in epilepsy patients. The key points are: 1) Piloerection is a seldom reported ictal phenomenon that is often overlooked due to limitations in video resolution and exposure of body areas. 2) It most commonly occurs in patients with temporal lobe epilepsy, with 85% of cases in one study having an epileptogenic zone located in the temporal lobe. 3) Bilateral piloerection may localize the epileptogenic zone to the right hemisphere, while unilateral piloerection often localizes to the hemisphere ipsilateral to the side affected.

1. PILOERECTION AS A
RARE MANIFESTATION
IN EPILEPSY PATIENTS
Julio Cesar Hernandez M.D.

2. 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

3.  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

4.  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

5.  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

6. 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.  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

8. Associated clinical findings
Preceeding symptoms Following symptoms Autonomic findings
Fear (3 patients),
nausea (3 patients), loss
of consciousness (3
patients), olfactory
auras, gustatory
auras, cephalic auras,
loneliness, and
automatisms
Automatisms (5
patients),
loss of consciousness (2
patients), nausea,
unilateral tonic seizures,
cephalic auras, bilateral
somatosensory aura,
and
perioral or nasal
paresthesias
Shortness of breath
and hyperventilation (6
patients), tachycardia (4
patients), ictal cold (3
patients), diaphoresis
(3), urinary urge (3
patients), pallor,
Salivation, and
hyperlacrimation
n=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

9. 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

10. Left hemispheric
predominance(?)

11. 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 lesions
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883

12. 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 cingulate
Loddenkemper et al. J Neurol Neurosurg Psychiatry 2004;75:879–883

13. A. Interictal B. Ictal
Masnou et al. Epileptic Disorders. Volume 8, Number 3, 232-7, September
F7, T1, Sp1 F7, T1, Sp1

14. 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)

15. Nicaragua: The hot heart of Central
America