Neurology Asia 2018; 23(2) : 163 – 175 Piloerection as the sole symptom of epilepsy: A case report and review of literature *1Ji-Qing Qiu PhD, *2Yu Cui MD, 3Li-Chao Sun MD, 1Bin Qi PhD, 1Xiao-Bo Zhu PhD, 1Zhan-Peng Zhu PhD *JQ Qiu and Y Cui contributed equally to this work and are co-first authors Departments of 1Neurosurgery, 2Otolaryngology and 3Emergency Medicine, The First Hospital of Jilin University, Changchun, Jilin, China Abstract Piloerection is an involuntary erection of body hairs that usually has physiological correlates such as cold or a strong emotional experience. Piloerection may also be a rare manifestation of seizure. Here, we report a case of 54-year-old man who experienced pilomotor seizures from temporal lobe epilepsy. The patient presented with sudden piloerection and no loss of consciousness many times a day. Magnetic resonance imaging of the brain showed three lesions in the right hemisphere, with the largest lesion in the right temporal lobe. A video-EEG showed an ictal discharge in the delta range with right temporal onset. Digital subtraction angiography excluded arteriovenous malformation. The lesion in the right temporal lobe was resected. Immunohistochemistry confirmed a cerebral cavernous malformation. There was no further seizure. A review of the published literature revealed that ictal piloerection as a lone manifestation is rare. Most cases of pilomotor seizure originate in the temporal lobe. Close to four fifth of the cases has a structural lesion. EEG was able to confirm the diagnosis of ictal piloerection in the majority of cases. Keywords: Piloerection, seizure, EEG INTRODUCTION piloerection; each episode lasting 10-20 seconds. Subsequently, the condition worsened, with Piloerection is a neurovegetative phenomenon the increased frequency of the attack up to 10 associated with fever, cold, and strong emotions, 1 episodes per day, lasting up to 30-40 seconds such as fear. Accordingly, piloerection is typically each time. During these episodes, the patient did accompanied by autonomic reactions including not experience other motor or sensory symptoms; tachycardia, tachypnea, vasoconstriction, there was no confusion or loss of awareness. The shivering, and heightened alertness.2 Piloerection 3-9 episodes usually occurred in the daytime, most can also a rare manifestation of seizure. The often when under stress. prevalence of pilomotor seizures in temporal lobe The patient’s past medical and family history epilepsy is estimated at 1.2%.10 Piloerection as 3,5,11 was unremarkable. His gestational development the lone symptom of seizure is said to be rare. and birth history was also normal. He had no We report here the case of a man that presented past illness that may give rise to development with piloerection as the only symptom of seizure of epilepsy, such as head injury, febrile seizures, from temporal lobe cavernoma. We also reviewed encephalitis, meningitis, or cerebrovascular published literature on pilomotor seizures. disease. The patient’s physical, mental, and CASE REPORT neurologic examinations, routine blood tests, A 54-year-old right-handed man was admitted and electrocardiogram were normal. MRI of the to our unit with a 2-year history of unexplained brain showed three lesions in the right hemisphere; recurrent bouts of visible piloerection involving the largest lesion, with a volume of 3.4 cm × 4.6 the whole body. The episodes were isolated cm × 3.7 cm, was in the right temporal lobe. The without other accompanied symptom. Initially, other two lesions were in the right frontal lobe the patient had 2-3 episodes per day of the said and the right insular cortex (Figure 1a-d). The Address correspondence to: Dr Zhan-Peng Zhu, Department of Neurosurgery, The First Hospital of Jilin University, 71 Xinmin Street, Changchun, Jilin 130021, P.R. China. E-mail: [email protected] 163 Neurology Asia June 2018 MRI brain was supportive of cavernous angioma. and severity of the seizures. After 3 months, Digital subtraction angiography was normal and frequency and severity of the seizures increased did not show any arteriovenous malformation again and could not be controlled despite (Figure 1e,f). the use of other antiepileptic drugs (AEDs) As there has been previous reports of lesion (carbamazepine, benzodiazepines, phenytoin, in the temporal lobe causing ictal piloerection12, gabapentin, phenobarbital, levetiracetam, and a diagnosis of epilepsy was suspected. A video- valproic acid alone and in combination). With only EEG was performed showing an ictal discharge mild reduction in the frequency of the seizures, in the delta range with right temporal onset. This the patient was recommended to have surgery. event lasted 27s (Figure 2). Corresponding to the The lesion in the right temporal lobe was rhythmic wave burst, the patient had piloerection resected (Figure 3, 4a). There was no neurological over his whole body. Thus, the EEG confirmed deficits postsurgery. The patient was maintained that the events were focal seizures. on oxcarbazepine 450mg/day, and has remained The patient was thus diagnosed to have temporal seizure free for 4 months during the last follow lobe epilepsy from cavernous malformation. He up. Histopathological examination of the resected was treated with oral oxcarbazepine up to 600 tissue confirmed the diagnosis of cerebral mg/daily. This initially decreased the frequency cavernous malformation (Figure 4b). Figure 1. Preoperative neuroimaging: Axial (a), sagittal (c), and coronal (d) magnetic resonance T2- weighted images revealed a 3.4 cm × 4.6 cm × 3.7 cm lesion in the right medial temporal lobe. Axial T2- fluid attenuated inversion recovery imaging revealed lesions in the right frontal lobe and right insular cortex (b). The lesions showed hypererintense center surrounded by hypointense ring suggestive of cavernoma. Figure 2. Ictal EEG at the onset of piloerection showing Digital subtraction angiography (e, f) excluded irregular slow wave delta activity on the right arteriovenous malformation. temporal region (a-c). 164 Figure 3. Postoperative neuroimaging: CT scan following resection of the right temporal lobe (a, b). DISCUSSION on humans implicate the hypothalamus, limbic system, orbital cortex, and the premotor area of the Piloerection is usually characterized by frontal lobe. In cats and/or monkeys, electrical or involuntary erection of body hairs in response pharmacological stimulation of the hypothalamus, to psychophysiological triggers, including a strong 13 amygdaloid nuclei, and cingulated gyrus elicited emotional experience or cold. As mentioned piloerection, bilateral hypothalectomy abolished above, piloerection can also rarely be a symptom piloerection, and removal of the premotor area of seizure11, particularly from temporal lobe 14 exaggerated piloerection. In humans, piloerection epilepsy. was also associated with changes in brain A comprehensive literature search of the potentials in the premotor area.7,16 PubMed and Web of Science databases from Piloerection occurs as a sympathetic reflex inception to August 2017 using the key words in response to cold, shock, stress, or fear. In ‘piloerection’, ‘goosebump’, ‘pilomotor’ and pilomotor seizures, piloerection may thus be ‘seizure’ by two independent reviewers was also the initial symptom of a seizure or secondarily performed. The searches identified 36 cases in induced during the seizure in response to psychic which piloerection was reported as a manifestation symptoms such as fear. Ictal piloerection is often of seizure (Table 1). Together with our own case, associated with autonomic symptoms involving 26 patients were men, and 10 patients were women the cardiovascular, cutaneous, gastrointestinal, (the gender of one patient was not mentioned), genital, pupillary, respiratory, and urinary systems, with Male : Female ration of 2.6 : 1, suggesting a implying the involvement of the autonomic and male predominance. All the patients were adults, limbic system. age ranged from 23 to 75 years. As for the clinical pattern of spread of In three patients (cases 25, 34, 36)3,5,11, the piloerection, 20/36 (56%) had a focal or piloerection was the lone seizure manifestation. somatotopical pattern, whereas in 16/36 (44%) This suggests that piloerection as a lone ictal cases, the piloerection was bilateral in distribution. manifestation is uncommon. In the majority As physiological piloerection is usually bilateral of patients (25 patients), consciousness was in distribution, a focal or somatotopical pattern preserved, implying that they were experiencing may thus help in the clinical diagnosis of ictal focal-aware seizures.15 piloerection. The precise localization of pilomotor seizure Including the current case, based on CT/MRI, is unknown. Animal studies and case reports Figure 4. Postoperative imaging showing the lesion (a) and cavernous malformation (Hemotoxylin and Eosin staining; 10×)(b). 165 Neurology Asia June 2018 166 Table 1: Review of previously reported cases of piloerection seizures. Etiology or Distribution of associated Case Author/year Age Sex Aura Symptoms Conscious Imaging EEG Surgery Outcome piloerection neurological disease Slow wave Focal cerebral 1 Landau et al. 29 M Rt side of the Feeling of — Y — — — burst infection of 195332 face and neck→ strangeness, streptococcic Rt arm and sadness, fear, septicemia forearm→ Rt and unreality leg→ trunk Sharp waves — — — A sensation like Chilly N — 2 Mulder et al. 25 F — arising from a “quivering” 195433 the lt sylvian in the heart; fissure (after swallowing metrazol)