Seizure (2005) 14, 288—291
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CASE REPORT
Pilomotor seizures in mesial temporal lobe epilepsy: A case confirmed by intracranial EEG Naotaka Usui *, Yasukazu Kajita, Satoshi Maesawa, Otone Endo, Shigenori Takebayashi, Jun Yoshida Department of Neurosurgery, Nagoya University School of Medicine, Japan KEYWORDS Pilomotor seizure; Mesial temporal lobe epilepsy; Intracranial EEG
Summary We report the first case in which intracranial EEG was used to confirm pilomotor seizures of mesial temporal origin. A 41-year-old man who had pilomotor seizures in the left arm in addition to complex partial seizures was admitted for presurgical evaluation. Intracranial EEG revealed ictal discharges in the left mesial temporal area during left pilomotor seizures. Moreover, electrical stimulation of the left mesial temporal area induced a habitual pilomotor seizure. After anteromesial temporal lobectomy, the patient became seizure-free. The localizing and lateralizing value of pilomotor seizures are discussed. # 2005 BEA Trading Ltd. Published by Elsevier Ltd. All rights reserved.
Introduction
Case presentation
Pilomotor seizures are relatively infrequent ictal autonomic phenomena that may start either unilaterally or bilaterally. Although temporal limbic components that interconnect with the hypothalamus and brainstem have been considered to be the sites most commonly related to pilomotor seizures,1—10 direct demonstration of the temporal lobe origin by intracranial EEG has not been documented. There is only one report of intracranial EEG recording in a case of pilomotor seizures of cingulate origin.11 We report a patient with unilateral pilomotor seizures who was monitored with invasive video EEG, and discuss the localizing and lateralizing significance of these seizures.
History and noninvasive presurgical evaluation
* Corresponding author at: National Epilepsy Center, Shizuoka Medical Institute for Neurological Disorders, 886 Urushiyama, Shizuoka 420-8688, Japan. Tel.: +81 54 245 5446; fax: +81 54 247 9781. E-mail address:
[email protected] (N. Usui).
A 41-year-old right-handed man was admitted for presurgical evaluation of a seizure disorder. He had had three febrile convulsions during early childhood, and started having occasional ‘goose bumps’ in the left arm when he was 24 years old. At 25 years of age, he had the first episode of loss of consciousness. Antiepileptic medication was then started. Auras were characterized by a funny feeling in the head, followed by piloerection in the left arm. The duration of the pilomotor seizures was about 1 min. He sometimes complained of epigastric sensation with or without piloerection. Thereafter he would become unresponsive, stare, and both arms would become tonic. He also sometimes lost consciousness without a preceding aura. Seizures occurred four to five times per month. Several
1059-1311/$ — see front matter # 2005 BEA Trading Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.seizure.2005.03.002
Pilomotor seizures in MTLE
medications including carbamazepine, phenytoin, primidone, clonazepam, zonisamide, and clobazam failed to adequately control his seizures. Noninvasive video-EEG monitoring with sphenoidal electrodes was done 4 years earlier and four pilomotor seizures were recorded. Sharp waves were seen in the left sphenoidal electrode interictally. Ictal EEG during pilomotor seizures revealed rhythmic spiking also at the left sphenoidal electrode. Brain MRI including coronal fluid-attenuated inversion-recovery (FLAIR) sequence was normal. [18F]fluoro-2-deoxyglucose-positron emission tomography revealed hypometabolism in the left frontotemporal regions.
Invasive presurgical evaluation and epilepsy surgery The patient underwent a left fronto-temporal craniotomy and subdural electrodes were implanted over the left temporal and frontal regions (Fig. 1). A 2 6 grid was placed in the left orbitofrontal area (electrodes A and B), a 3 6 grid in the left perisylvian area (electrodes C—E), and three 1 6 strips in the left mesial-basal temporal area (electrodes F—H). In addition, a depth electrode was intended to target the left hippocampus (electrode I); unfortunately, it was in fact inserted into the white matter just superior to the left hippocampus. Placement of subdural and depth electrodes was confirmed by MRI. Interictally, spikes were seen most frequently in the left mesial temporal area and less frequently in the left orbitofrontal area. Three complex partial seizures (CPSs) out of sleep and six simple partial seizures (SPSs) were recorded during the 5-day
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monitoring period. Four of six SPSs were pilomotor seizures characterized by ‘goose bumps’ in the left arm. Ictal EEG (Fig. 2) revealed rhythmic spiking in the left mesial temporal area (G1, 2, and H1, 2 electrodes) during pilomotor seizures involving the left arm. The EEG onset preceded the clinical onset by 3—14 s. Moreover, electrical stimulation of the electrode on the mesial temporal area (H1 electrode with D6 electrode, biphasic square pulses, 50 Hz, pulse duration: 0.3 ms, intensity: 6 m, duration: 2 s) induced afterdischarges in the mesial temporal area. The patient reported the habitual piloerection 27 s after the stimulation. Three CPSs also originated from the left mesial temporal area. Left anteromesial temporal lobectomy was subsequently performed, and histological examination revealed mesial temporal sclerosis. The postoperative course was uneventful, and the patient has remained seizure free for 10 months.
Discussion Pilomotor seizures are infrequently reported. Piloerection is sometimes accompanied by other ictal manifestations such as sweating, chills and epigastric sensation, and may not be noticed. Tyndel et al.1 reported a patient with pilomotor seizures. In their patient, piloerection was preceded by an unusual smell, and accompanied by feelings of anxiety, flushing and cold sweat. They commented that pilomotor seizures might be more common than was apparent. The results of animal experiments suggest that the hypothalamus,12 limbic system,13 and frontal
Figure 1 Localization of the intracranial electrodes (left, anteroposterior view; right, lateral view). Subdural strips were placed over the left orbitofrontal area, left perisylvian area, and left basal temporal area. In addition, a depth electrode was targeted to the left hippocampus. Unfortunately, it was in fact inserted into the white matter just superior to the left hippocampus.
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Figure 2 Ictal EEG during a pilomotor seizure. An ictal EEG shows rhythmic spiking in the mesial temporal area (G1, 2, and H1, 2 electrodes). Three seconds after the EEG onset, the patient complained of ‘goose bumps’ in his left arm. (A and B: orbitofrontal cortex, and F—H: left basal temporal cortex).
lobe are involved in the regulation of piloerection. Studies in patients with epilepsy suggest the temporal lobe as the most frequent source of pilomotor seizures, based on scalp EEG findings1—7,9 and location of epileptogenic lesions.1—5,7,10 However, the seizure focus has not been confirmed by intracranial EEG. In addition to the temporal lobe and insula,8 Seo et al.11 recently reported a case of pilomotor seizures of cingulate origin examined by intracranial EEG. Intracranial EEG is still the method that can identify the seizure focus most precisely. In our patient, EEG showed left sphenoidal sharp waves interictally, and ictal discharges during pilomotor seizures also revealed rhythmic spiking at the left sphenoidal electrode. No hippocampal abnormality was evident on MRI, including FLAIR sequence. The question then was which region (mesial temporal or orbitofrontal) was the real focus of the seizure, since epileptogenicity in either area may generate sphenoidal discharges on EEG. We therefore decided to perform invasive video-EEG monitoring before surgical resection. Except piloerection, the whole clinical picture including semiology, EEG and neu-
roimaging was not suggestive of a cingulate origin, we therefore did not place an electrode strip to target the cingulate gyrus. Ictal discharges were confined to the mesial temporal area and never spread to the other areas covered by the electrodes. To our knowledge, this is the first reported case in which intracranial EEG demonstrated the mesial temporal area as the source of pilomotor seizures. We were also able to induce a habitual pilomotor seizure by electrical stimulation of the mesial temporal area. Afterdischarges were confined to the mesial temporal area during piloerection. Although the mechanisms of piloerection may be more complex and not confined to a single cortical region, the mesial temporal lobe is considered to be a symptomatogenic zone of pilomotor seizures, in addition to the cingulate gyrus. Piloerection occurred on the side ipsilateral to the epileptic focus in our patient. This finding agrees with some previous reports.2,7,13 Yu et al.7 reported pilomotor seizures in two patients who had piloerection spreading in a pattern like the ‘Jacksonian march’, confined ipsilaterally to the epileptogenic
Pilomotor seizures in MTLE lesion. On the contrary, Roze et al.9 reported a patient with pilomotor seizures in the right hemibody while EEG revealed left fronto-temporal discharges. Stefan et al.5 reported the left hemispheric predominance of focal abnormality associated with autonomic auras including goose bumps and/or cold shivers. Further studies are necessary to clarify the lateralizing value of pilomotor seizures.
Conclusion Pilomotor seizures are considered to be a useful localizing sign. A mesial temporal origin of pilomotor seizures was confirmed by ictal recording with intracranial EEG and electrical stimulation in our patient, and this area is considered to be a symptomatogenic zone for piloerection, in addition to the cingulate gyrus.
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