1/38. Autosomal dominant partial epilepsy with auditory features: description of a new family.PURPOSE: To report the clinical and genetic study of a new family with autosomal dominant partial epilepsy with auditory features (ADPEAF). methods: All the living affected members underwent a full clinical, neurophysiological, and magnetic resonance imaging (MRI) study. Genetic analysis was performed by typing their dna with seven microsatellite markers previously found to cosegregate with ADPEAF on chromosome 10q24. RESULTS: The three living affected members had a childhood onset of rare and drug-responsive tonic-clonic seizures constantly preceded by a humming sensation. Routine and sleep electroencephalograms revealed rare and inconstant focal abnormalities over both temporal regions. MRI detected atrophy with increased T2 signal in the subcortical lateral portion of the right temporal lobe in one case. Analysis of 10q24 polymorphic alleles showed the same haplotype in all three affected members but different alleles in unaffected individuals. CONCLUSIONS: ADPEAF is a distinct condition with homogeneous clinical features. Genetic findings are consistent with linkage of ADPEAF to chromosome 10q24.- - - - - - - - - - ranking = 1keywords = family, member (Clic here for more details about this article) |
2/38. Supporting evidence of a gene for partial epilepsy on 10q.A four-generation family with nine individuals with temporal partial epilepsy was studied. Detailed epilepsy history was investigated by structured interview. All putatively affected family members underwent a standardized electroencephalographic examination. The phenotype in the family was characterized by a short acoustic aura followed by rapid secondary generalization. To examine if the trait is linked to a region on 10q (interval D10S185-D10S1671), which has been reported in two other epilepsy families with similar phenotypes, linkage analysis was performed using nine markers covering the previously reported region. A maximum two-point lod score of 2.1 at a recombination fraction of zero was obtained. All living affected individuals shared the same haplotype, while three unaffected at-risk adults did not. This result presents supporting evidence of a gene for partial epilepsy on 10q.- - - - - - - - - - ranking = 0.59504689994038keywords = family, member (Clic here for more details about this article) |
3/38. Familial temporal lobe epilepsy with aphasic seizures and linkage to chromosome 10q22-q24.PURPOSE: To describe the phenotypic expression of a new family with familial lateral temporal lobe epilepsy with aphasic seizures, and to compare the findings with the clinical features of previously reported families linked to chromosome 10q22-q24. methods: medical records were collected from 12 living affected members. The patients underwent a personal interview and a clinical neurologic examination. Results from interictal scalp EEGs and neuroimaging examinations were obtained. RESULTS: The cardinal ictal symptom was a brief sensory aphasia in eight of the patients. In four, this was accompanied by auditory symptoms, usually in the form of monotonous unformed sounds. Simple partial seizures with psychic or somatosensory seizures also were present. Visual ictal symptoms and complex partial seizures were absent. All patients had generalized tonic-clonic seizures. magnetic resonance imaging (MRI) or computed tomography (CT) did not reveal morphologic correlates. Improvement with age seemed to occur in many patients. Significant linkage to chromosome 10q22-q24 was established by testing 17 polymorphic microsatellite markers. CONCLUSIONS: The epilepsy of this family appears to represent a variety of autosomal dominant lateral temporal lobe epilepsy. Aphasic seizures and a peculiar seizure-precipitating effect of the activation of speech (initiation or perception) may serve as markers for identifying further families with this phenotype.- - - - - - - - - - ranking = 0.3987617249851keywords = family, member (Clic here for more details about this article) |
4/38. exercise-induced temporal lobe epilepsy.Although precipitation of seizures by exercise has been described, the reproducible induction of temporal lobe seizures by exercise is unusual. The authors report two patients with left temporal lobe seizures induced by exercise. In one patient the family history suggested autosomal-dominant inheritance. Prolonged hyperventilation, simple movements, and visualization of a competitive game did not produce epileptiform discharges on the interictal EEG.- - - - - - - - - - ranking = 0.19628517495529keywords = family (Clic here for more details about this article) |
5/38. epilepsy: a neurological complication of thalidomide embryopathy.The possibility of epilepsy resulting from maternal thalidomide ingestion was investigated by obtaining a telephoned neurological history from all suspect families in the United Kingdom in which the specific diagnosis was uncertain. The annual incidence in the first seven years of life was found to be five times the figure for the general population, and the prevalence of active epilepsy is significantly increased in the teenage thalidomide population. That this increased incidence and prevalence of epilepsy is not a chance observation is supported by published clinical and experimental evidence of central nervous system abnormalities in thalidomide embryopathy, in addition to the known neurological effects of the drug in the adult.- - - - - - - - - - ranking = 0.0035170447989384keywords = life (Clic here for more details about this article) |
6/38. Selective emotional detachment from family after right temporal lobectomy.Behavioral changes, such as mood disorders, anxiety, psychosis, and nonepileptic seizures often occur after temporal lobectomy. We report a man who selectively lost emotional attachments to family members after right temporal lobectomy. However, emotional responsiveness to strangers was normal or increased.- - - - - - - - - - ranking = 0.98761724985096keywords = family, member (Clic here for more details about this article) |
7/38. Benign Familial Neonatal seizures.Benign Familial Neonatal seizures (BFNS) occur in normal newborns without perinatal neurological damage or metabolic abnormalities in the setting of a positive family history for neonatal seizures. This autosomal dominant disorder has an excellent prognosis, in contrast to most other causes of neonatal convulsions. This paper points out the need to include BFNS in the differential diagnosis of neonatal seizures and to specifically seek a family history to avoid an unnecessarily extensive diagnostic evaluation and poor prognostication. We present a family with one atypical and three classic cases. Further study of BFNS may reveal more definitive basic science information leading to the inclusion of variant forms into the currently narrow clinical definition.- - - - - - - - - - ranking = 0.58885552486586keywords = family (Clic here for more details about this article) |
8/38. anomia for people's names, a restricted form of transient epileptic amnesia.A 37-year-old man consulted after two episodes of transient anomia for people's names over a period of 6 months. The first episode lasted about 10 min and was restricted to an inability to remember his 2-year-old son's first name. The second, was limited to an inability to recall his daughter's first name for 5 min with clear abnormal experiential quality. Witnessed descriptions of the attacks confirmed the absence of any other cognitive impairment or motor automatisms. The neurological examination was normal except for hyposmia. Inter-ictal cognitive evaluation was normal apart from the anomia for people's names or retrieval of names of familiar people in his childhood on definition and on famous faces naming test. A wake electroencephalograph showed left temporal epileptiform abnormalities, following hyperpnea. On magnetic resonance imaging, quantitative analysis revealed a mildly decreased volume of the left hippocampus. The diagnosis of transient epileptic amnesia (tea) was considered and the patient did not recur for 6 months under lamotriginum. Thus anomia for people's names may be the sole clinical manifestation of tea. Such a clinical presentation may easily be overlooked. Treatment may prevent further recurrence and the installation of more important and permanent autobiographical memory impairment. Our observation may suggest an isolated system not only for people's knowledge, but for people's naming. It is consistent with the notion of proper name as pure referring expression.- - - - - - - - - - ranking = 0.0061913750745207keywords = member (Clic here for more details about this article) |
9/38. No evidence for a seriously increased malignancy risk in LGI1-caused epilepsy.The leucine-rich glioma Inactivated-1 (LGI1) gene is supposed to be a tumor suppressor gene involved in glial tumors. Mutations in this gene were recently found to cause autosomal dominant lateral temporal lobe epilepsy (ADLTE). We have now analysed the comorbidity in a large Norwegian ADLTE family. No evidence was found that LGI1 is a high-penetrance tumor suppressor gene associated with a serious risk for malignancies in ADLTE families.- - - - - - - - - - ranking = 0.19628517495529keywords = family (Clic here for more details about this article) |
10/38. A case of "double" depression under outpatient treatment conditions.During his professional practice the author encountered a case of coexistence of "major depression" with psychic depressive attacks (dysthymic attacks) of so-called temporal epilepsy. Apart from major depression of medium intensity, other manifestations developed. These were independent of the time of the day, suddenly occurring within several seconds, developing without any cause, attacks of very strong dejection, sadness, breakdown, feeling of lacking sense and hopelessness of life with slight lessening of consciousness and strong groundless fear. Detailed psychiatric examinations, observations of the patient during such attacks and EEG records confirmed the diagnosis of dysthymic attacks of temporal epilepsy. The author treated the patient with sertraline starting at a low dose and increasing up to 100 mg daily - administered orally once daily in the morning, clonazepam in oral doses 1 mg in the morning, 1 mg at lunchtime, 2 mg in the evening, and carbamazepine 200 mg tablets from low doses to 400 mg administered once daily in the evening. Complete remission of major depression and complete regression of dysthymic attacks of "temporal epilepsy" were obtained.- - - - - - - - - - ranking = 0.0035170447989384keywords = life (Clic here for more details about this article) |
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