11/15. Acquired aphasia without deafness in childhood--the landau-kleffner syndrome.A young boy presented with loss of speech and behaviour disturbance and was thought to be deaf. He was subsequently found to have the landau-kleffner syndrome (LKS), or acquired aphasia with epilepsy. Children with this disorder commonly present to an audiology or ENT clinic. Early recognition is important to initiate supportive, speech and educational care.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
12/15. Converging evidences on language impairment in landau-kleffner syndrome revealed by behavioral and brain activity measures: a case study.OBJECTIVE: To assess the linguistic abilities of a boy having landau-kleffner syndrome, and relate the focal disturbance of brain activity due to epilepsy to the cognitive and linguistic deficits. methods: Several kinds of assessments were carried out, including epileptic source analysis using electronic source localization methods and PET, neuropsychological assessment of cognitive functions, and assessment of speech perception skills (discrimination of phonetic and stress cues) using ERPs. RESULTS: The source of epileptic activity was localized in the left superior temporal lobe. The neuropsychological assessment showed dissociation between verbal and nonverbal functions, and the performance in former was bellow the normal range. ERPs obtained to the processing of phonetic and stress speech cues indicated that the two cues were processed asymmetrically: the mismatch negativity component (MMN) was obtained for the phoneme difference, but not for the stress pattern difference. CONCLUSIONS: Our data converged as it showed that the patient presented a selective impairment of the language system, and the verbal working memory system appeared to be especially defective. It is suggested that the language deficit is at least partly due to the focal disturbance of those neural networks that underlie the functioning of the working memory system. SIGNIFICANCE: LKS is a childhood language disorder that might serve as a model in studying what happens to the language system if, in the course of development, the essential neural circuits are severely disturbed.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
13/15. Efficacy of very high dose steroid treatment in a case of landau-kleffner syndrome.landau-kleffner syndrome (LKS) is an acquired childhood aphasia associated with paroxysmal bitemporal electroencephalogram (EEG) abnormalities and, sometimes, clinical seizures. We report the case of a female aged 5 years 6 months who presented clinically with apparent hearing loss, deterioration in speech, and seizure activity over 12 days. The female had previous detailed speech/language assessments at 3 to 4 years of age due to articulation delay. LKS was diagnosed on EEG with bitemporal spike and wave activity during sleep. The patient was treated with high dose prednisolone 3mg/kg/day, intensive speech/language therapy, and followed a modified educational program. We recorded a marked regression in receptive and expressive language skills, as well as her speech, language, and cognitive profiles before and during treatment with prednisolone, during an 18-month follow-up period. The patient demonstrated an excellent clinical response highlighting the importance of a multidisciplinary approach to management of LKS.- - - - - - - - - - ranking = 2keywords = speech (Clic here for more details about this article) |
14/15. A chance SPECT study of ictal aphasia during simple partial seizures.We report obtaining an ictal single photon emission computed tomographic (SPECT) scan in a right-handed 51-year-old man who had an astrocytoma in the left cerebral hemisphere and simple partial seizures characterized by aphasia. An epileptic seizure producing loss of speech and right-sided facial twitching occurred by chance during a SPECT scan. During the attack, he was unable to speak, but auditory comprehension and writing were intact. Ictal SPECT showed an area of increased perfusion in the left frontal cortex, with the area of highest perfusion involving the left frontal operculum to the inferior part of the left precentral gyrus. Interictal SPECT showed hypoperfusion in the same area. These SPECT findings suggest that the frontal operculum of the dominant hemisphere is one of the regions that can give rise to epileptic aphasia.- - - - - - - - - - ranking = 0.5keywords = speech (Clic here for more details about this article) |
15/15. landau-kleffner syndrome: consistent response to repeated intravenous gamma-globulin doses: a case report.PURPOSE: Although several treatments have been tried for landau-kleffner syndrome (LKS) too many patients are refractory to known therapies. We report an 8-year-old girl who failed other therapies but who had a consistent response after treatment with intravenous (i.v.) gamma-globulin. methods: We monitored the girl from the age of 6 years, when she presented with a 6-month history of loss of language with normal hearing, normal brain magnetic resonance imaging (MRI), increased cerebrospinal fluid (CSF) IgG index, and an EEG showing almost continuous, predominantly left-sided spike- and slow-wave complexes. She had no clinical seizures and did not respond to consecutive trials of valproate (VPA), clonazepam (CZP), prednisone, and carbamazepine (CBZ). She received three courses of intravenous (i.v.) gamma-globulin; after each course, clinical and electrographic improvement lasted a few months. After each of the initial two courses, clinical improvement lasted 3-4 months but was followed by recurrence of the spikes on the EEG and by speech deterioration. RESULTS: However, her last remission has been continuous for the past 16 months. Her CSF IgG index became normal after the first i.v. gamma-globulin infusion. CONCLUSIONS: Based on our experience with this patient and on other investigators' experience, we believe that further research into immunologic mechanisms and therapies of this syndrome are warranted.- - - - - - - - - - ranking = 0.5keywords = speech (Clic here for more details about this article) |
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