Nocturnal hypermotor seizures, suggesting frontal lobe epilepsy, can originate in the insula

PURPOSE: To report three patients with drug-resistant nocturnal hypermotor seizures (NHSs), no detectable brain lesion, and clinically defined nocturnal frontal lobe epilepsy (NFLE) or autosomal dominant NLFE (ADNFLE), whose intracerebral EEG ictal onset primarily involved the insula, rather than the mesial or orbital frontal cortex. METHODS: Fourteen to 15 intracerebral electrodes were implanted in each patient, primarily sampling the frontal lobes with 80 to 91 recording leads covering the most likely side of seizure onset, and two to six leads placed within the ipsilateral insula. Electrical stimulation was used to test the epileptic threshold of frontal and insular brain regions at the various recording sites. RESULTS: In all three patients, a low-voltage fast activity was recorded within the anterosuperior aspect of the insula at ictal onset, either in isolation, or extending to the nearby frontal operculum in the ADNFLE patient. The role of the insula was further supported in all three patients either by the presence of high-amplitude spikes that clearly predominated over that region (n = 2) or by triggering the patient's typical aura or seizure when applying an electrical stimulation at that site, selectively (n = 2). CONCLUSIONS: The anterosuperior portion of the insula might play a pivotal role in generating nocturnal hypermotor seizures in some patients with nonlesional drug-resistant epilepsy suggesting NFLE or ADNFLE. Whether these patients are amenable to successful surgery remain an open issue.     

Sleep-related hypermotor seizures in aspartylglucosaminuria: A case report

From the age of 24 years a young man with a definitive diagnosis of aspartylglucosaminuria (AGU) presented short-lasting sleep-related paroxysmal events characterized by sudden awakening with a frightened look, hyperventilation, and complex bilateral motor activity. Nocturnal video-polysomnography recorded several events consistent with a diagnosis of hypermotor epileptic seizures. This pattern of sleep-related epileptic seizures has been reported in rare familial cases, more often in cryptogenic and symptomatic patients in the spectrum of nocturnal frontal lobe epilepsy. Epilepsy and sleep-related nonepileptic problems are common in patients with AGU, but no case of hyperkinetic nocturnal frontal lobe seizures has been reported to date. Differential diagnosis of abnormal paroxysmal motor events in sleep is frequently a challenge for the clinician: Video-polysomnographic recordings might serve to identify the possible epileptic origin of some of the excessive motor activities during sleep referred in patients with AGU.     

Partial epilepsy presenting as focal atonic seizure: A case report

A case of atonic seizures localized to the frontal lobe by video-EEG monitoring is reported. The patient is a 38-year-old female with intractable atonic seizures characterized by abrupt onset of facial grimacing and a slow head drop. The onset of atonic seizures was about 6 years before presentation. Video-EEG monitoring showed that her atonic seizures were emanating from the right frontal head region. A high voltage spike and slow wave discharge invariably coincided with the onset of atonic seizures in the patient, similar to the interruption of tonic muscular activity time-locked to a spike on the EEG described in epileptic negative myoclonus; a syndrome associated with epileptic activity in the premotor cortex.Since routine MRI imaging in this patient was normal, diffusion tensor imaging (DTI) was applied to analyze the white matter integrity of the normal-appearing white matter in the frontal lobes of the patient. We compared the fractional anisotropy, parallel diffusivity and perpendicular diffusivity of normal-appearing white matter in the right versus left frontal lobe. Our results showed no significant difference between the two sides. Possible reasons for the normal DTI findings are discussed.     

Tardive seizure: a case report

Electroconvulsive therapy remains the best option for treatment-resistant depressive episodes. A rare, but potentially dangerous, complication is tardive seizures, which occur after the patient has already stopped convulsing from the electroconvulsive therapy and has recovered full consciousness. We have decided to report this case, which many psychiatrists and psychiatry residents may not be familiar with, to heighten the awareness of the condition because it has ramifications in terms of safe management of the patients concerned.     

Solitary Langerhans cell histiocytosis orbital lesion: case report and review of the literature

Solitary eosinophilic granuloma is a rather benign and localized form of Langerhans's cell histiocytosis. Definitive diagnosis is made by histopathology including immunohistochemical detection of S-100, HLA-DR and CD1a antigens. We report the case of a twenty-five year old boy presented with headache and orbit's pain. A CT scan showed a left supero-lateral orbital mass with evidence of bone erosion. The different options of treatment are discussed and the literature is reviewed.     

Stroop performance in focal lesion patients: dissociation of processes and frontal lobe lesion location

There were three primary objectives: to examine the usefulness of the Stroop interference effect as a measure of frontal lobe function; to investigate the possibility of distinct lesion effects for word reading or color naming; and to specifically determine the brain regions necessary for the performance of the incongruent condition. Fifty-one patients with single focal brain lesions in frontal and non-frontal regions and 26 normal control subjects (CTL) were administered the word reading, color naming and incongruent conditions of the Stroop task. Only frontal lesions produced significant impairment. Patients with posterior lesions were not significantly deficient in any condition. Damage to the left dorsolateral frontal lobe resulted in increased errors and slowness in response speed for color naming. Contrary to Perret (Neuropsychology, 1974; 12: 323-330), lesions of the left frontal lobe did not result in a selective interference deficit on the Stroop incongruent condition. Rather, bilateral superior medial frontal damage was associated with increased errors and slowness in response time for the incongruent condition. This result is interpreted as failure of maintenance of consistent activation of the intended response in the incongruent Stroop condition. The results and conclusion are compatible with the prevalent theories of both the Stroop effect and the role of the superior medial frontal regions. The role of the anterior cingulate cortex on performance of the Stroop task is likely related to task and patient context.     

KCNT1基因突变导致的婴儿癫痫伴游走性局灶性发作一例

目的探讨KCNT1基因突变所致婴儿癫痫伴游走性局灶性发作(EIMFS)的临床特征、基因诊断、治疗及预后。方法对1例诊断为EIMFS患儿的临床表现、脑电图(EEG)特点及基因测序结果进行分析。结果患儿男性,2月20d,频繁抽搐发作,表现为局灶性发作,伴癫痫持续状态,EEG放电部位不固定,抗癫痫药物治疗效果欠佳,起病后出现发育滞后,头颅MRI及血、尿遗传代谢筛查无明显异常,基因测序结果发现患儿存在KCNT1基因突变,该突变为杂合错义突变(c.1283G> A,p.Arg428Gln),患儿父母该位点未发现突变,即患儿为新生突变。结论 KCNT1是EIMFS主要的相关基因,对于临床病因不明且抗癫痫药物治疗效果欠佳的早发性癫痫脑病患儿需考虑基因检测,协助临床诊断及预后评估。     

Sinus pericranii in the frontal region: a case report

Sinus pericranii is a rare vascular anomaly. A case of sinus pericranii at the nasion with an orbital extension is presented. The drainage was into the superior sagittal sinus. The pathogenesis is discussed and the literature is reviewed.     

Peri-ictal atrioventricular conduction block in a patient with a lesion in the left insula: Case report and review of the literature

Seizure-induced syncope is uncommon and is usually due to peri-ictal bradycardia and asystole. In contrast, peri-ictal atrioventricular conduction block (AV block) has been reported only rarely. Here, we review the literature on peri-ictal AV block and describe a patient with recurrent episodes of epigastric auras that were occasionally followed by a sensation of dizziness and syncope because of transient third-degree AV block. After installation of a cardiac pacemaker, epigastric auras persisted, whereas the sensation of dizziness and syncope stopped. MRI revealed a lesion in the left insula, leading to the notion that recurrent syncope was most likely due to ictal activity in central autonomic networks.     

Absence of aphasia in a dextral with a left hemisphere lesion: A case report

Abstract

Right-handed patients with aphasia due to a right hemisphere lesion, or sinistral aphasics with a left hemisphere lesion, are referred to as crossed aphasics (Bramwell 1899). Since left damaged sinistrals with aphasia are the rule (Brown and Hecaen 1976), it is the dextral with a right hemisphere lesion that has received most of the attention. When we reviewed the topic of crossed aphasia in dextrals some years ago in relation to a case report (Brown and Wilson 1973). only seven other cases could be found that satisfied the minimal criteria of a clearcut aphasia dextrality and a negative family history ofleft handedness, but since this paper there have been many additional observations (e.g. Assal, Perentes and Dervaz 1981, Donoso, Santander and Pavez 1984, Pillon, Desi and Lhermitte 1979, Wechsler 1976; see Joanette, Puel, Nespoulos, Rascol and Lecours [1982] and Castro-Caldas and Confraria [1984] for review).     

Rud syndrome with focal cortical dysplasia: a case report

We report a female patient with ichthyosis, epilepsy, mental retardation, hypergonadotrophic hypogonadism, polyneuropathy, and cranial dysmorphisms. This clinical picture may satisfy the main diagnostic criteria that characterize Rud syndrome (RS), a rare neurocutaneous disease. The patient underwent extensive clinical evaluation, neurophysiological studies (wakefulness and sleep EEG, EMG), dermatological and endocrinological evaluation and neuroimaging study (3 Tesla brain MRI). Interestingly, brain MRI unveiled a malformation of cortical development, never reported previously in RS. Although seizure semiology and EEG features could not provide clear cut information suggesting a focal onset, the role of this MRI finding in the genesis of the epileptic seizures cannot be ruled out. The finding of a focal cortical dysplasia in RS might be related to genetic abnormalities affecting the development of both epidermis and neural structures with the same embryological origin.     

Amnesic syndrome with a unilateral thalamic lesion: a case report

Summary A 40-year-old man suffered an attack of transient global amnesia. The anterograde amnesia receded after 2 days, but a deficit remained in the retrieval stage of memory, which impaired the recall of names and of the information necessary to give details of particular events. Computed tomography revealed ischaemic infarction in the left anterolateral thalamus.

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Zusammenfassung Bei einem 40jährigen Mann trat eine amnestische Episode auf. Die anterograde Amnesie dauerte nur 2 Tage, aber es blieb eine spezifische Gedächtnisstörung, die das Wiederfinden von verbalem Material, besonders von Namen, betraf, darüber hinaus aber auch den Zugang zu jenen spezifischen Informationen und Details behinderte, die der einzelnen Erinnerung ihren individuellen Charakter geben. Das CT zeigte einen ischämischen Infarkt im linken anterolateralen Thalamus.

     

A simple febrile seizure with focal onset

Simple febrile seizures last for less than 10 minutes and resolve spontaneously, in the context of a febrile illness, without focal features or recurrence during the subsequent 24 hours. We report the case of fortuitous video‐EEG recording of a FS, clinically classified as “simple”, which demonstrated a focal, temporal onset. This clinical finding is in agreement with animal model studies demonstrating focal onset. [Published with video sequences]