1/37. Chronological progression of a language deficit appearing to be postictally reversible in a patient with symptomatic localization-related epilepsy.A language deficit occurring interictally, with chronological progression, and postictally in a patient with symptomatic localization-related epilepsy, which began at 1.6 years of age, is reported. The patient was a 30-year-old right-handed man whose seizures seemed to originate from the left frontal lobe and to involve the left temporal lobe. The deficit in oral language consisted mainly of features of motor aphasia, including delayed initiation of speech with great effort, echolalic and palilalic tendencies, and word-finding difficulty, but he also showed features of sensory aphasia. Written language had agraphia observed in sensory aphasia, including well-formed letters, paraphasias, neologisms, and paragrammatism. Postictally, the language deficit appeared to be superficially reversible, and evolved from mutism through non-fluent jargon to the interictal level of language. Analysis of the patient's diaries from 10 to 26 years of age disclosed chronologically progressive deterioration of language with paragrammatism, showing an increase of grammatical errors, neologismus, literal and verbal paraphasias and misconstruction of sentences. The results suggest that localization-related epilepsy of presumably left frontal lobe origin causes not only a postictal language deficit but also a slowly progressive deficit of language function.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
2/37. 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 = 1.0971011256026keywords = speech, perception (Clic here for more details about this article) |
3/37. Automatisms with preserved responsiveness and ictal aphasia: contradictory lateralising signs during a dominant temporal lobe seizure.The 25-year-old right-handed woman suffering from temporal lobe epilepsy (TLE) was referred to our centre for presurgical evaluation. MRI showed a right-sided hippocampal sclerosis. During video-EEG-recorded seizures, abdominal aura was followed by oral automatisms, during which she was completely reactive to external stimuli, although she was unable to speak. Ictal EEG showed right temporal seizure pattern, without contralateral propagation. She had abnormal speech postictally. speech-activated functional transcranial Doppler sonography revealed right-sided speech dominance. She has become seizure free after a right-sided amygdalo-hippocampectomy. In our patient, contradictory clinical ictal lateralising signs (automatisms with preserved responsiveness vs. ictal and postictal dysphasia) occurred during right-, speech-dominant-sided seizures. This is the first report when automatisms with preserved consciousness occurred during a seizure originating and involving the speech-dominant hemisphere.- - - - - - - - - - ranking = 4keywords = speech (Clic here for more details about this article) |
4/37. Transient inability to distinguish between faces: electrophysiologic studies.It is not known with certainty at which level of face processing by the cortex the distinction between a familiar and an unfamiliar face is made. Subdural electrodes were implanted under the fusiform gyrus of the right temporal lobe in a patient who developed an unusual inability to distinguish differences between faces as part of the epileptic aura ("all faces looked the same"). A cortical region located posterior to the epileptic focus was identified that exhibited a maximum evoked response to the presentation of facial images (N165), but not to objects, scenes, or character strings. evoked potentials elicited by a variety of visual images indicated that any perturbation away from novel whole-face stimuli produced submaximal responses from this region of the right temporal lobe. Electrical stimulation of this region resulted in an impairment of face discrimination. It was found that presentation of familiar faces (grandmother, treating physician) produced a different response from that observed for novel faces. These observations demonstrate that within 165 msec of face presentation, and before the conscious precept of face familiarity has formed, this cortical region has already begun to distinguish between a familiar and an unfamiliar face.- - - - - - - - - - ranking = 2.1742432083959keywords = discrimination (Clic here for more details about this article) |
5/37. speech delay due to a prelinguistic regression of epileptic origin.A 2-year-old boy presented with an early form of benign partial epilepsy with centro-temporal spikes (BCERS) and a severe speech delay. family video analysis revealed an early regression of babbling and stagnation since the age of 12 months. Complete recovery occurred with anti-epileptic treatment. The deficit corresponded to a transient speech apraxia attributed to an epileptic disconnection of networks coordinating speech articulation. This observation is, to the best of our knowledge, the first demonstration that delayed emergence of language can be due to an epileptic dysfunction interfering with prelinguistic skills and therefore mimicking a developmental delay.- - - - - - - - - - ranking = 3keywords = speech (Clic here for more details about this article) |
6/37. Myxopapillary ependymoma of the temporal lobe--report of a rare case of temporal lobe epilepsy.Myxopapillary ependymomas are a benign variant of ependymomas, occurring almost exclusively in the cauda equina region. We report an extremely rare case of myxopapillary ependymoma located in the left anterior temporal lobe. A 22-year-old man is presented with intractable seizures of 2 years duration with no focal neurologic deficits. Imaging of the brain revealed a well-circumscribed heterogeneous mass in the left anterior temporal pole with no connection to the ventricles. Imaging of the spine was normal. The patient underwent surgical removal of the tumor and at follow-up 4 months after surgery, there was improvement in his memory and speech along with complete cessation of seizures. Microscopic examination revealed the tumor to be a myxopapillary ependymoma, further confirmed by histochemical and immunohistochemical stains. To the best of our knowledge, this is the first documentation of myxopapillary ependymoma at this location and consequently, the first case to clinically present as intractable temporal lobe epilepsy.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
7/37. Contribution of neuropsychology to epilepsy surgery.Surgical treatment is being used with increasing frequency for patients with intractable epilepsy. Operative success depends to a large degree on the results of a comprehensive pre-operative patient evaluation the main purpose of which is to delineate the epileptogenic lesion. The pre-operative assessment includes video EEG monitoring, structural and functional (fMRI) neuroimaging and neuropsychological evaluation. The likelihood of the success of surgery is increased when all test results point to a single epileptogenic focus. The unique contribution of neuropsychology, which includes standard neuropsychological assessment and intracarotid sodium amytal (Wada) testing, is its capability to predict lateralization and often localization of a brain lesion based on cognitive function and dysfunction, which can be demonstrated also in the absence of a structural lesion. The Wada test is used to determine the cerebral speech dominance, to predict postsurgical amnesia and is found to be useful in predicting laterality of seizure focus in candidates for temporal lobectomy.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
8/37. Ictal kissing and religious speech in a patient with right temporal lobe epilepsy.We report the case of a 25-year-old female patient with intractable complex partial seizures characterized by repetition of certain religious statements and a rather compulsive kissing behavior. Presurgical evaluation revealed a right-sided, mesial temporal focus and hippocampal sclerosis on MRI. After selective amygdalohippocampectomy, she has only occasional auras. Her rare and peculiar ictal manifestations are discussed in the context of semiology and pathogenesis. [Published with videosequences].- - - - - - - - - - ranking = 4keywords = speech (Clic here for more details about this article) |
9/37. Emotion recognition from facial expressions in a temporal lobe epileptic patient with ictal fear.Ictal fear (IF) is an affective aura observed in patients with temporal lobe epilepsy. It has been suggested that the amygdala, a region implicated in emotion processing, is involved in generating IF. Several studies have reported that the patients with IF have disturbances in emotional experience, but there has been no testing of the emotional recognition in those patients. In this report, emotion recognition from facial expressions was investigated in a patient with IF. The patient suffered from IF due to temporal lobe epilepsy, and underwent hippocampectomy surgery which completely suppressed IF. We examined the patient before and after surgery. Before surgery, the patient tended to attach enhanced fear, sadness, and anger to various facial expressions. After surgery, such biases disappeared. As an underlying mechanism of the pre-surgical skewed perception of emotional stimuli, the abnormal epileptogenic circuits involving a hypersensitive amygdala possibly due to the kindling mechanism were suggested.- - - - - - - - - - ranking = 0.09710112560265keywords = perception (Clic here for more details about this article) |
10/37. speech-induced aphasic seizures in epilepsy caused by LGI1 mutation.PURPOSE: patients with autosomal dominant lateral temporal lobe epilepsy (ADTLE) may have seizures precipitated by sound or speech. We have examined a patient with speech-induced seizures caused by an LGI1 mutation (C46R). methods: A clinical study and a video-EEG recording using interrogative speech as the activation procedure was performed in a 23-year-old man. RESULTS: He had experienced short episodes of sensory aphasia in situations in which he was suddenly verbally addressed. Voices became distorted, and he could not comprehend despite hearing words. The day after a late party, his girlfriend unexpectedly spoke to him. Her speech became unintelligible to him. He did not reply and had a generalized tonic-clonic (GTC) seizure. During an EEG, he was suddenly asked for the names of his siblings. He answered, but lost understanding of the further conversation and described how syllables floated together with an echoing character. With a versive movement to the right, another GTC occurred. In the EEG, rhythmic 6-Hz activity built up in the frontotemporal areas starting on the left side with bilateral and posterior spreading. Postictal slowing was symmetrical, and no aphasia was noted on awakening. CONCLUSIONS: To our knowledge, this is the first video-EEG recorded seizure in LGI1-caused ADTLE. This peculiar seizure semiology and precipitating effect of speech may serve as a marker for identifying further individuals with this particular phenotype and genotype and may indicate that the LGI1 gene may have a physiologic function connected to the human capacity for speech and language.- - - - - - - - - - ranking = 6keywords = speech (Clic here for more details about this article) |
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