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At least 19 recordsLinked to original sources
PubMed2000

Temporal lobe epilepsy with sensory aura: interictal glucose hypometabolism.

Patients with mesial temporal lobe epilepsy (mTLE) exhibit marked depressions of the regional cerebral glucose metabolism (rCMRGlu) in the mesiotemporal region. We hypothesised that patients with temporal lobe epilepsy (TLE) who have a bilateral somatosensory or acoustic ( = temporolateral/SII-) aura can be differentiated from mTLE by rCMRGlu depressions primarily involving temporo-perisylvian locations. We therefore used this ictal semiology as a clinical criterion to define a subgroup of such patients and measured the rCMRGlu in 16 patients with TLE as evident from interictal and ictal EEG-video monitoring. Clinically, they presented with medically refractory complex partial seizures and were subjected to presurgical evaluation. The pattern of the interictal rCMRGlu in the TLE patients was different from that observed in patients with mTLE and showed significant depressions ipsilateral to the epileptic focus in mesial temporal and lateral temporal regions but spared the thalamus. The neocortical metabolic depressions were spatially more extended in right than in left TLE patients. Magnetic resonance images (MRI) were either normal (n = 5) or revealed unilateral or bilateral hippocampal atrophy/sclerosis (n = 7), or temporal or extratemporal focal cortical dysplasia (n = 4). The selected TLE patients presented here comprise a heterogeneous group showing most pronounced metabolic depressions in the lateral temporal cortex. Thus, our data suggest that non-invasive metabolic imaging can assist in identifying the neocortical symptomatogenic zone in putative temporo-perisylvian lobe epilepsy.

Abdomen↗
PubMed2005

LGI1 gene mutation screening in sporadic partial epilepsy with auditory features.

Partial epilepsy with auditory features occasionally segregates in families as an autosomal dominant trait. In some families mutations in the leucine-rich glioma inactivated (LGI1) gene have been identified. Sporadic cases might harbour either denovo or low-penetrant LGI1 mutations, which will substantially alter the family risk for epilepsy. We selected sixteen sporadic patients with cryptogenic temporal lobe epilepsy and partial seizures with auditory features. We compared clinical features of these patients with those of published autosomal dominant family cases. We screened these patients for LGI1 mutations. Comparing the sporadic patients with the published familial cases no difference in either the primary auditory features or in the other associated epileptic manifestations was identified. Sequence analysis of the whole LGI1 gene coding regions in sporadic patients did not reveal changes in the LGI1 gene. The genetic analysis demonstrates that LGI1 is not a major gene for sporadic cases of partial epilepsy with auditory features at least in the Italian population. Screening of sporadic patients for LGI1 mutations appears not useful in genetic counselling of these patients.

Adult↗
PubMed2004

LGI1 mutations in temporal lobe epilepsies.

BACKGROUND AND OBJECTIVES: A number of familial temporal lobe epilepsies (TLE) have been recently recognized. Mutations in LGI1 (leucine-rich, glioma-inactivated 1 gene) have been found in a few families with the syndrome of autosomal dominant partial epilepsy with auditory features (ADPEAF). The authors aimed to determine the spectrum of TLE phenotypes with LGI1 mutations, to study the frequency of mutations in ADPEAF, and to examine the role of LGI1 paralogs in ADPEAF without LGI1 mutations. METHODS: The authors performed a clinical and molecular analysis on 75 pedigrees comprising 54 with a variety of familial epilepsies associated with TLE and 21 sporadic TLE cases. All were studied for mutations in LGI1. ADPEAF families negative for LGI1 mutations were screened for mutations in LGI2, LGI3, and LGI4. RESULTS: Four families had ADPEAF, 22 had mesial TLE, 11 had TLE with febrile seizures, two had TLE with developmental abnormalities, and 15 had various other TLE syndromes. LGI1 mutations were found in two of four ADPEAF families, but in none of the other 50 families nor in the 21 individuals with sporadic TLE. The mutations were novel missense mutations in exons 1 (c.124T-->G; C42G) and 8 (c.1418C-->T; S473L). No mutations in LGI2, LGI3, or LGI4 were found in the other two ADPEAF families. CONCLUSION: In TLE, mutations in LGI1 are specific for ADPEAF but do not occur in all families. ADPEAF is genetically heterogeneous, but mutations in LGI2, LGI3, or LGI4 did not account for families without LGI1 mutations.

Adult↗
PubMed2000

Auras, seizure focus, and psychiatric disorders.

OBJECTIVE: The goal of this study was to assess the types of auras and their associations with the focus of seizures in 144 consecutive adult patients with partial epilepsy refractory to medical treatment. The association of frequency and type of aura with a psychiatric disorder was also studied. BACKGROUND: The nature of the aura, its association with the seizure focus, and its relation with emotions and behavioral dysfunction have received relatively little attention, and remain controversial. METHODS: Patients were admitted to an epilepsy investigation unit for detailed observation of their seizures and type of aura. Patients underwent standard electroencephalographic telemetry, including implanted subdural electrodes, if necessary, to delineate the seizure focus. The auras were classified into nine categories. All patients had an independent clinical psychiatric interview for a Diagnostic and Statistical Manual, Third Edition-Revised (DSM-III-R)-based diagnosis. RESULTS: Of the 144 patients studied, 111 (77.1%) had one or more symptoms during the aura and 33 (22.9%) had no aura. The most frequent aura types were viscerosensory (47 cases [32.6%]), experiential (44 cases [30.6%]), and cephalic (21 cases [14.6%]). A temporal lobe focus was present in 116 (80.5%) patients. Only experiential auras were significantly associated with a temporal lobe seizure focus (33 of 44 cases). A DSM-III-R psychiatric diagnosis was present in 74 (51.4%) patients. Of the 116 patients with a temporal lobe focus, 60 (50.2%) had a DSM-III-R psychiatric diagnosis. Patients with two or more auras were significantly more likely to have a psychiatric diagnosis (70.2%) compared with those with one aura (39.1%) or no aura (48.5%). A significant association between experiential auras and psychiatric morbidity was seen. CONCLUSIONS: Auras occurred in a majority of patients with a treatment-refractory partial epilepsy who were hospitalized for assessment of their seizure focus for possible surgical candidacy. Two or more auras were associated with a psychiatric diagnosis as was the presence of an experiential aura. Experiential auras were most common in temporal lobe epilepsy. Approximately half the patients, irrespective of seizure focus, had an associated psychiatric morbidity.

Adolescent↗
PubMedDate not supplied

[Olfactory seizures and parasellar meningioma].

INTRODUCTION: Partial olfactory seizures are infrequent. They are related to the presence of lesions in the uncinate area of the temporal lobe. Patients describe smells during the ictal phase that are generally unpleasant. We report the cases of two patients with olfactory disorders of a paroxysmal nature caused by a parasellar meningioma. CASE REPORTS: Case 1: a 43 year old male who presented paroxysmal episodes in which he perceived a bitter smell, with dysphoria, linked to the presence of a left side parasellar meningioma. Case 2: a 45 year old female who presented paroxysmal episodes during which she could smell formaldehyde and this was linked to the presence of a left side parasellar meningioma. DISCUSSION: Olfactory seizures are related to structural alterations to the amygdala. The most frequent causes are associated to the presence of mesial temporal sclerosis and, above all, to tumours situated in the temporomesial structures that have diverse aetiologies.

Abdominal Pain↗
PubMedDate not supplied

[Partial benign crises in adolescence].

The aim of this paper is to present the case of teenage patient with partial seizures fulfilling the criteria of benign partial seizures of adolescence. A 16-year-old male patient had two seizures with a sensory-motor "march" that evolved into a secondarily generalized tonic-clonic seizure on the same day. Several weeks before this event he had had several simple partial sensory seizures. The patient had no previous history of seizures and there was no family history of epilepsy. The neurological examination, EEG and magnetic resonance imaging were normal. The patient was treated with antiepileptic monotherapy during two years. The treatment was gradually tapered and withdrawn over the following six months. He has had no recurrences during the five years of follow-up. The early diagnosis of this entity has the significant prognostic and therapeutic repercussions.

Adolescent↗
PubMed2000

Autosomal dominant partial epilepsy with auditory features: defining the phenotype.

The authors previously reported linkage to chromosome 10q22-24 for autosomal dominant partial epilepsy with auditory features. This study describes seizure semiology in the original linkage family in further detail. Auditory hallucinations were most common, but other sensory symptoms (visual, olfactory, vertiginous, and cephalic) were also reported. Autonomic, psychic, and motor symptoms were less common. The clinical semiology points to a lateral temporal seizure origin. Auditory hallucinations, the most striking clinical feature, are useful for identifying new families with this synome.

Epilepsy, Partial, Sensory↗
PubMedDate not supplied

Cerebral hyperperfusion syndrome-a case report and literature review.

Cerebral reperfusion following carotid endarterectomy occasionally causes cerebral hyperperfusion syndrome. This is a rare but important complication and this case report acted as a stimulus for a literature review of this problem. A 60-year-old businessman had a right carotid endarterectomy for a severe stenosis which had caused recurrent attacks of amaurosis fugax. The left internal carotid artery had occluded asymptomatically. The operation and his immediate postoperative recovery were entirely uneventful but he developed right-sided headaches and focal sensory motor seizures. He subsequently recovered. Hemodynamically compromised patients appear to be at greater risk and as the mortality of the operation is reduced and more complex patients are treated, it is likely that this unusual complication will increase in incidence.

Brain↗
PubMed2003

Painful focal sensory seizure arising from the primary somatosensory cortex.

A 31-year-old, right-handed woman had frequent focal painful seizures involving the right hand without any movement. EEG demonstrated an ictal activity arising from the left centroparietal region. No cerebral structural abnormality was seen on MRI. Ictal single photon emission CT showed markedly increased activity in the left perirolandic cortex, which remained active following the ictal symptoms when the EEG seizure pattern had completely disappeared. It is concluded that the painful seizures in the present patient originated from the primary somatosensory cortex. The prolonged increase of regional blood flow in the perirolandic area may reflect the possibility of persistent subclinical epileptogenicity.

Adult↗
PubMed2005

[Supplementary sensory-motor seizures--symptomatology, etiology, and surgical management with illustrative case reports].

In the past decade, owing to the advance of epilepsy surgery, growing knowledge has accumulated on the role of the supplementary motor area, described by Penfield and coworkers in the early fifties, in movement regulation and on the characteristics of seizures involving this area. In the Hungarian neurological literature this topic--despite its neurophysiological and practical clinical importance--has been hardly touched. The authors, based on their own experience obtained from surgeries performed within the framework of the "Co-operative Epilepsy Surgery Program", describe the electrophysiological features of this area, its role in movement regulation and the symptoms of epileptic seizures stemmed from or spread onto this area. Using cases as illustrations, they demonstrate the reasoning and various algorithms of the multidisciplinary examination necessary to explore the seizure onset zone and the pathways of seizure spread. Details of the surgical solution are also described.

Adolescent↗
PubMed2005

Symptoms in focal sensory seizures. Clinical and electroencephalographic features.

PURPOSE: Aura is a brief subjective symptom that may represent the initial manifestation of a partial epileptic seizure with objective signs or constitute the entire epileptic attack (focal sensory seizure (FSS)). We studied the electro-clinical features of FSSs recorded in 28 patients. METHODS: Using long-term surface video-EEG recordings, we examined 28 patients (from a consecutive series of 64) with stereotyped FSSs and complex partial seizures (CPS) preceded in at least one instance by identical subjective manifestations (overall 255 FSSs and 39 CPS were recorded). FSSs were subdivided according to the type of sensation into somatosensory, visual or oculosensory, viscerosensory, experiential, cephalic and diffuse warm sensations. The EEG discharges accompanying FSSs were examined by two of the authors either blinded as to the type and timing of the seizure, or unblinded, i.e. after receiving complete clinical information including timing of the patient's warning. RESULTS: The ictal pattern accompanying FSSs was identified blind in 13 patients and unblind in 8 patients. In seven patients, the ictal discharge remained undetected. In the cases with recognizable ictal abnormalities, two main patterns could be distinguished, static and dynamic. FSSs whose ictal discharge could be recognized by blind EEG examination more frequently consisted of somatosensory and visual or oculosensory manifestations, and the discharge generally involved the centro-parieto-occipital regions. The ictal discharge of viscerosensory and experiential FSSs more easily remained undetected; when identified, it generally involved the fronto-temporal regions. CONCLUSIONS: FSSs are often accompanied by ictal abnormalities recognizable on surface EEG. A thorough knowledge of their EEG accompaniments may be a useful diagnostic aid in patients with partial epilepsy.

Adult↗
PubMed2004

Gaze-evoked brainstem myoclonus.

The clinical and electrophysiological aspects of a case where brainstem reticular reflex myoclonus was related to an enlarging pontine lesion are described. It had the unusual characteristic of being evoked by sustained up gaze and left gaze.

Adult↗
PubMed2006

Non-ketotic hyperglycaemia-related paroxysmal bilateral hand paraesthesia misdiagnosed as diabetic neuropathy.

Non-ketotic hyperglycaemia (NKH)-related partial seizure disorders are not uncommon in clinical practice but still deserve attention as they significantly affect neurologic outcome if unnoticed. The atypical presentation of sensorimotor symptoms can be seen in this setting, with paroxysmal character as the rule. Atypical manifestations could cause confusion and might lead to improper diagnosis and treatment. We report a case of inadequately controlled diabetes mellitus and NKH presenting as paroxysmal paraesthesia of both hands, which was misdiagnosed as diabetic neuropathy.

Aged↗