Neuropediatrics 2000 Datei: NE1565 Seite: 273 8.12.2000 ± 16:31 K C M Y nnnn We report two children, suffering from idiopathic partial epilepsy, who started to present, in the same period of time, with epileptic negative myoclonus (ENM) in one lower limb and fecal incontinence (FI). Polygraphic recordings showed that ENM was associated with paroxysmal activities distributed over the vertex region. Both ENM and FI disappeared when ethosuxi- mide treatment was started. We hypothesize that, in our pa- tients, ENM in one lower limb and FI depended on a transitory impairment, caused by epileptic activity that altered the func- tionality of nearby cortical areas, located in fronto-mesial re- gions, involved in the control of the muscular tone of the lower limbs and of the pelvic floor muscles. n Key words: Epileptic negative myoclonus ± Fecal incontinence ± Ethosuximide ± Childhood partial epilepsy ± Somatotopy Introduction Epileptic negative myoclonus (ENM) is a motor disorder char- acterized by a sudden, brief loss of the muscular tone, associat- ed with a paroxysmal EEG abnormality, that can manifest itself as a brisk interruption of a tonic contraction. Its clinical spec- trum can be extremely wide, varying from overt phenomena, such as frequent drops of the head or of the arms (causing, for instance, dropping of objects from the hands), to a subtle dis- order that can impair the performance of a motor task, so as to cause, over time, a sort of motor neglect [4,13,15]. ENM can be observed in a variety of epileptic conditions, and its incidence is probably underestimated. The diagnosis of ENM relies on the polygraphic demonstration of a brief interruption of the tonic EMG activity, time-locked to an epileptic EEG abnormal- ity. The pathophysiology of ENM has been investigated in sev- eral studies and various hypotheses have been postulated [2, 4,6,9,15]. More recently, some papers have focused on its specific treatment. Particularly, ethosuximide (ETS) has been reported to be effective in improving ENM occurring in partial epilepsies of childhood [3,7,11]. We report two cases of idiopathic childhood partial epilepsy presenting with ENM in the lower limbs and frequent, small losses of feces. In both patients, ENM and fecal incontinence (FI) were relieved by ETS treatment. We discuss the possible relationships between ENM in the lower limbs and FI. Case Report Case 1 This is a 10-year old, neurologically and mentally normal girl, with unremarkable personal and familial history who started to present, at the age of 8 years, with brief simple partial motor seizures involving the left lower limb. EEG showed focal sharps and waves in the right central and vertex regions, increasing during sleep. MRI was normal. Carbamazepine (CBZ) (20 mg/ kg/day) was started, producing a decrement of the seizure fre- quency; to improve the situation, sodium valproate (VPA) (20 mg/kg/day) and clobazam (10 mg/day) were added. Serum levels were 85g/ml for VPA and 9.1 for CBZ. Clobazam was withdrawn after one month because of drowsiness. She con- tinued to present monthly motor partial seizures in the left lower limb. At age 9, the girl started to present frequent falls to the ground. In the same time, her parents started to notice every day that her underwear was stained by loss of feces, in- dicating that she was suffering from a sort of minimal FI. She had never had this disturbance in the past. A polygraphic re- cording demonstrated brief interruption of EMG tonic activity in the left tibialis anterior, time-locked to contralateral spikes in the vertex and right fronto-central regions, consistent with ENM in the left lower limb. At this age, ESM (20 mg/kg/day) was added to the preexistent treatment. ENM and FI disap- peared within 15 days, and also her seizures strikingly im- proved. Three months after the introduction of ETS, CBZ was gradually discontinued. ESM and VPA blood levels were re- spectively 67 g/ml and 65 g/ml. No adverse events were re- ferred. After one year follow-up, she is seizure-free. Case 2 This is a 5-year old male with unremarkable personal and fa- milial history. At the age of 3, after a febrile illness, he started to present with a fluctuating gait disturbance and frequent Short Communication Intermittent Falls and Fecal Incontinence as a Manifestation of Epileptic Negative Myoclonus in Idiopathic Partial Epilepsy of Childhood G. Capovilla 1 , G. Rubboli 2 , Francesca Beccaria 1 , Stefania Meregalli 3 , P. Veggiotti 4 , P. M. Giambelli 3 , S. Meletti 2 , C. A. Tassinari 2 1 Department of Child Neuropsychiatry, C. Poma Hospital, Mantova, Italy 2 Department of Neurological Sciences, Bellaria Hospital, Bologna, Italy 3 Department of Neurology, Niguarda Hospital, Milano, Italy 4 Department of Child Neuropsychiatry, C. Mondino Foundation, Pavia, Italy Neuropediatrics 2000; 31: 273 ± 275  Georg Thieme Verlag Stuttgart · New York ISSN 0174-304X Received: March 30, 2000 Accepted after Revision: August 17, 2000 273