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LETTER TO THE EDITOR
Year : 2017  |  Volume : 20  |  Issue : 2  |  Page : 169-172
 

Piloerection: A rare ictal phenomenon – Case report and review of literature


1 Department of Neurophysiology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Neurology, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication8-May-2017

Correspondence Address:
Samhita Panda
Department of Neurophysiology, Sir Ganga Ram Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.AIAN_427_16

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How to cite this article:
Panda S, Agarwal C, Sharma A. Piloerection: A rare ictal phenomenon – Case report and review of literature. Ann Indian Acad Neurol 2017;20:169-72

How to cite this URL:
Panda S, Agarwal C, Sharma A. Piloerection: A rare ictal phenomenon – Case report and review of literature. Ann Indian Acad Neurol [serial online] 2017 [cited 2020 May 31];20:169-72. Available from: http://www.annalsofian.org/text.asp?2017/20/2/169/205769


Sir,

We report the case of a 28-year-old man who presented with spells of unusual nature for 8 months. These events were characterized by frequent brief episodes of fearfulness and sensation of panic associated with goose bumps or piloerection over the left upper limb with unusual cold sensation going down his spine, palpitations, brief breathing difficulty and brief change in voice. He was completely aware and communicative during the episodes which lacked other autonomic or motor symptoms, lasted for 30 s-1 min and occurred 10–20 times a day. Although mainly spontaneous, there was increased frequency during periods of mental stress. An additional history of loss of consciousness with probable tonic-clonic limb movements was elicited at the very onset of this illness. However, no specific antiepileptic drug had been given. There was no febrile seizure, head trauma, encephalitis, meningitis, or cerebrovascular disease in the past. Family history was not contributory. The patient had attributed these symptoms to certain significant recent stressful life events and his inability to deal them. He initially consulted a psychiatrist and was treated as a case of panic disorder with anxiolytics and beta-blockers with marginal improvement. After 8 months, one of the authors considered the possibility of partial seizures with autonomic features though psychogenic nonepileptic events could not be ruled out completely. Long-term video-electroencephalography (EEG) monitoring showed intermittent theta (5–6 Hz) activity and frequent spike wave discharges over the left frontotemporal region. Ten habitual events characterized by piloerection over his left forearm with the sensation of cold running down his spine were recorded while conscious and responsive. No orofacial or limb automatism, speech arrest or behavioral arrest was noted. Ictal EEG showed ictal onset over left frontotemporal region preceding the clinical event by 10–15 s [Figure 1]a and [Figure 1]b. Magnetic resonance imaging brain showed features suggesting left mesial temporal sclerosis. The patient was initiated on antiepileptic drugs (lamotrigine and clobazam with gradual dose escalation) and behavioral counseling with 80% seizure reduction at 6 months follow-up. The patient's family refused surgery due to personal reasons.
Figure 1: (a and b) Ictal electroencephalography shows rhythmic spike wave discharges followed by 5–6 Hz theta activity over left temporal region

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The interaction between seizures and autonomic nervous system is very complex. Abnormal neuronal electrical activity arising from autonomic centers can result in autonomic symptoms anytime in the peri-ictal period. Episodic autonomic dysfunction is sometimes difficult to diagnose as seizures. Piloerection which may be accompanied by cold shiver is a very uncommon ictal sign of visceral epilepsies and may be isolated or occur in combination with other autonomic signs or complex partial seizures.[1],[2] About 106 cases of pilomotor seizures have been reported in literature.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] Out of these, 31 had isolated pilomotor phenomenon without any other aura or dialeptic features. The majority of patients reported in literature were noted to have ictal onset over the temporal region.[1],[2],[6],[7],[10] Piloerection is mostly unilateral or initially unilateral with later contralateral spread, and at times it can be bilateral at onset. Although majority had left temporal localization, one-third (35 cases) had right temporal localization.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] On the other hand, bilateral piloerection had no definite lateralizing value.[1] Varied etiology of pilomotor seizures includes tumors, encephalitis, stroke, neurodegenerative disease, hippocampal sclerosis, or autoimmune process. Although hippocampal sclerosis is a more frequent cause,[1],[2],[7] a few recent reports have recorded pilomotor seizures in autoimmune nonparaneoplastic limbic encephalitis especially in relation to voltage-gated potassium channel antibodies.[6],[10]

In temporal lobe epilepsy, piloerection, and other autonomic phenomena are proposed to reflect seizure spread to the insula.[1] An interesting observation is the high frequency of ictal pilomotor events with multiple daily to weekly events up to 20–30/h.[1],[3],[5],[6],[7],[8],[9],[10],[11],[12],[13] The exact pathophysiology of this high frequency of neuronal hypersynchronization is unclear. The presence of dysplastic cells, inflammation or involvement of limbic circuit including amygdala, mesial temporal and probably orbitofrontal structures may trigger increased seizure frequency due to high epileptogenicity index. We postulate that a reverberating circuit between limbic regions and centers of autonomic control may lead to a vicious cycle wherein pilomotor seizures and panic/anxiety or behavioral changes may be perpetuating each other. Further research using stereoelectroencephalography looking at the epileptogenic network involved in pilomotor seizures may answer this question. The peculiar features of ictal piloerection may masquerade as other psychiatric or cardiac disorders [Table 1]. The conglomerate of symptoms of gooseflesh, tachycardia, palpitations and sweating in themselves can easily be confused as neurovegetative dysfunction due to possible nonepileptic events.[5],[6],[7],[8],[10],[11] As a consequence of the ictal phenomena and high frequency of recurrent events, personality and behavioral traits may develop simultaneously, further confusing the diagnosis. Eleven patients have been reported in literature to have initial misdiagnosis, 5 as due to psychiatric cause,[5],[6] one as cardiac and one as migraine. This leads to a significant delay in diagnosis with patients undergoing elaborate investigative tests and treatment for other disorders. The label of a psychiatric disorder may negatively impact the quality of life of the patient.

Therefore, it is of paramount importance to suspect an epileptic etiology when encountering stereotyped, short-lasting, episodic events, howsoever bizarre, and uncommon in symptomatology.
Table 1: Literature review of 11 patients with ictal piloerection and delay in diagnosis

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Loddenkemper T, Kellinghaus C, Gandjour J, Nair DR, Najm IM, Bingaman W, et al. Localising and lateralising value of ictal piloerection. J Neurol Neurosurg Psychiatry 2004;75:879-83.  Back to cited text no. 1
    
2.
Stefan H, Pauli E, Kerling F, Schwarz A, Koebnick C. Autonomic auras: Left hemispheric predominance of epileptic generators of cold shivers and goose bumps? Epilepsia 2002;43:41-5.  Back to cited text no. 2
    
3.
Roze E, Oubary P, Chédru F. Status-like recurrent pilomotor seizures: Case report and review of the literature. J Neurol Neurosurg Psychiatry 2000;68:647-9.  Back to cited text no. 3
    
4.
Seo DW, Lee HS, Hong SB, Hong SC, Lee EK. Pilomotor seizures in frontal lobe epilepsy: Case report. Seizure 2003;12:241-4.  Back to cited text no. 4
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5.
Cutts J, Lee G, Berarducci M, Thomas C, Dempsey PK, Kadish SP. Goosebumps. Lancet 2002;360:690.  Back to cited text no. 5
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6.
Rocamora R, Becerra JL, Fossas P, Gomez M, Vivanco-Hidalgo RM, Mauri JA, et al. Pilomotor seizures: An autonomic semiology of limbic encephalitis? Seizure 2014;23:670-3.  Back to cited text no. 6
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7.
Dove GH, Buchhalter JR, Cascino GD. Acute repetitive pilomotor seizures (goose bumps) in a patient with right mesial temporal sclerosis. Clin Neurophysiol 2004;115:2511-3.  Back to cited text no. 7
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8.
Puligheddu M, Bortolato M, Barberini L, Genugu F, Gioi G, Balestrieri A, et al. Dissociation between neurovegetative signs and subjective symptoms in a case of idiopathic pilomotor seizures. Cogn Behav Neurol 2010;23:274-7.  Back to cited text no. 8
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9.
Mittal S, Oni-Orisan A, Stenz J, Shah AK. Temporal neocortical origin of pilomotor seizures in association with an infiltrating glioma: A case confirmed by intracranial electroencephalography monitoring. J Neurosurg 2010;113:388-93.  Back to cited text no. 9
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10.
Lam EM, Worrell GA, Laughlin RS. Semiology of the rare seizure subtype piloerection. Arch Neurol 2010;67:1524-7.  Back to cited text no. 10
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11.
Haykal MA, Abou-Khalil B. Pilomotor seizures: A video case report. Epileptic Disord 2012;14:76-9.  Back to cited text no. 11
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12.
Kurita T, Sakurai K, Takeda Y, Kusumi I. Pilomotor seizures in temporal lobe epilepsy: A case report with sequential changes in magnetic resonance imaging. Epilepsy Behav Case Rep 2013;1:142-5.  Back to cited text no. 12
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13.
Asha MJ, Tansey RJ, Gan YC. 'Goose bumps' as presenting feature of intraventricular glioblastoma multiforme. Br J Neurosurg 2014;28:276-7.  Back to cited text no. 13
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