1/15. Prosodic preservation in landau-kleffner syndrome: a case report.landau-kleffner syndrome (LKS) is a rare acquired condition of auditory verbal agnosia and convulsive disorder in children. It has been proposed that there is a functional relationship between electrical disturbance and the speech defect. Prosody or the melody of language, as described by Monrad-Krohn (1947), is one aspect of non-verbal communication which is distributed bilaterally in the brain. Prosodic parameters of expression and perception in one 7.5-year-old child were tested to see if they were preserved as a means of communication. The child was observed during video-electroencephalogram (EEG) monitoring over a 48-hour period. All utterances were recorded and subject to analysis for the salience and variation of acoustic correlates of prosody. Prosodic comprehension was measured using specific perceptual tasks previously presented to normal children between the ages of 5.5 and 8.5. Despite being unable to meaningfully use or perceive phonemes, the child was able to use variations in fundamental frequency, duration and intensity of utterances, to convey both emotional and propositional intent. Similarly, the child was able to discriminate prosodic contours of a male adult voice to an age equivalent to 5.5 to 6.5 years. This argues in favour of the notion for educating such children not only through the visual channel but also through the auditory channel.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
2/15. language disorders in landau-kleffner syndrome.In the present long-term study, we analyzed language disorders in four patients with landau-kleffner syndrome. Their common first symptoms were disability in understanding spoken words, followed by inarticulation and a decreased amount of speech. All patients showed auditory verbal agnosia to some degree at some stage of their illness. However, one patient showed typical sensory aphasia as the first symptom, and another patient showed nonverbal auditory agnosia followed by pure word deafness. Thus, patients with landau-kleffner syndrome show sequential and sometimes hierarchical language disorders beginning with sensory aphasia, followed by auditory agnosia, and finally word deafness during their disease process. During long-term follow-up (20 to 30 years), all patients showed marked recovery in language without any intellectual handicap, but with some disability in spoken language, auditory verbal perception, and a discrepancy between Wechsler Verbal and Performance IQ scores.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
3/15. The spectrum of acquired cognitive disturbances in children with partial epilepsy and continuous spike-waves during sleep. A 4-year follow-up case study with prolonged reversible learning arrest and dysfluency.We report a longitudinal study (7-11 years) of a previously normal boy (MR) who presented from the age of 5 years with rare partial motor seizures and atypical 'absences'. The history revealed a stagnation in development and speech difficulties a few months before onset of his epilepsy. The first waking electroencephalogram (EEG) showed rare generalized discharges during hyperventilation. magnetic resonance imaging revealed an arachnoid cyst in the frontotemporal region. Although his epilepsy never became severe, he experienced important learning difficulties. Subsequent EEGs became increasingly active with left focal epileptic activity and continuous spike-waves during sleep (CSWS) present from the first sleep EEG. The first neuropsychological evaluation (7 years) showed a speech dysfluency, word finding and naming problems, inattention and low intelligence quotient. carbamazepine was changed to clobazam and later ethosuximide was added with a rapid improvement (within 1 month) in linguistic and cognitive performances as well as in behaviour. Furthermore, the patient showed considerable progress in acquisition over the next months whereas learning to read had previously been very difficult. The epileptic activity gradually disappeared and he was able to follow regular school at an age-appropriate level. This case adds a new facet to the already recognized more obvious acquired neuropsychological disturbances known to occur in some partial childhood epilepsy syndromes with CSWS (aphasia, dementia). It manifested as prolonged insidious stagnation in learning and subtle language disability. This study documents rapid specific language improvement with change in anti-epileptic drugs and a restored immediate and long-term learning capacity, suggesting a direct but 'hidden' role of epilepsy.- - - - - - - - - - ranking = 2keywords = speech (Clic here for more details about this article) |
4/15. Effects of high-dose intravenous corticosteroid therapy in landau-kleffner syndrome.Two children with landau-kleffner syndrome were successfully treated with antiepileptic drugs and a high-dose intravenous corticosteroid. A combination of valproate and a benzodiazepine (clonazepam or diazepam) ameliorated epileptic seizures and electroencephalographic spikes and waves, but speech disturbances persisted. Both patients were treated with an intravenous infusion of high-dose methylprednisolone sodium succinate (20 mg/kg daily) for 3 consecutive days. This infusion was repeated three times with a 4-day interval between treatments, which resulted in a rapid improvement in speech ability. After intravenous therapy, prednisolone was given orally (2 mg/kg daily for 1 month, then gradually withdrawn), which maintained the clinical improvement in speech.- - - - - - - - - - ranking = 3keywords = speech (Clic here for more details about this article) |
5/15. Successful use of intravenous immunoglobulin as initial monotherapy in landau-kleffner syndrome.PURPOSE: There is a need for new and more effective therapies for landau-kleffner syndrome. In this article we present the first case in which a patient with landau-kleffner syndrome was given intravenous immunoglobulin (IVIG) as his first and only therapy and responded to it. methods: This previously healthy, left-handed boy presented at 31 months of age with a 3-month history of auditory agnosia, behavioral abnormalities, and progressive, eventually complete loss of speech. electroencephalography (EEG) showed frequent and, in sleep, continuous right central and temporal spike slow wave discharges. Metabolic workup, magnetic resonance imaging, and auditory evoked potentials were normal. cerebrospinal fluid IgG index was high (18%). The patient was treated with IVIG, as his initial and only therapy, receiving 500 mg/kg/day over four consecutive days. RESULTS: On the third day of IVIG, the patient started using single words, and on the fourth, two-word sentences. Two weeks later his speech and behavior returned to normal. At the end of 4 days of IVIG therapy, EEG was within normal limits. Two months later, however, he had a severe relapse clinically and by EEG. He promptly responded to another course of IVIG. A subsequent cerebrospinal fluid IgG index showed normalization (6%). Three months later he had essentially normal speech and behavior. CONCLUSIONS: Repeated, immediate, and remarkable clinical and EEG responses of this patient suggest that IVIG was helpful as first-line therapy in the treatment of landau-kleffner syndrome. It also supports the hypothesis that immunological mechanisms contributed to his symptoms.- - - - - - - - - - ranking = 3keywords = speech (Clic here for more details about this article) |
6/15. Transient opercular syndrome: a manifestation of uncontrolled epileptic activity.Newly appearing oral motor apraxia should alert physicians to the possibility of an organic lesion in the operculum, the perisylvanian cortex. A 6 1/2-year-old boy who is being followed for complex partial seizures with secondary generalization failed to respond to carbamazepine and then to the addition of sodium valproate. The further addition of lamotrigine stopped the seizures but the patient presented with a new onset of opercular syndrome, manifested by severe oral motor apraxia with difficulties in chewing and swallowing and speech. MRI study of the brain revealed no abnormalities. The opercular syndrome resolved completely when the lamotrigine was replaced with phenobarbital, and did not recur during the follow-up period of 2 years. The EEG, however, continued to be abnormal. We conclude that opercular syndrome may be a manifestation of abnormal localized electrical activity at the operculum, even in the absence of an organic lesion on imaging studies, and it may be a marker for an epilepsy which is not easily controlled.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
7/15. Accessibility of spoken, written, and sign language in landau-kleffner syndrome: a linguistic and functional MRI study.landau-kleffner syndrome (LKS) is an acquired aphasia which begins in childhood and is thought to arise from an epileptic disorder within the auditory speech cortex. Although the epilepsy usually subsides at puberty, a severe communication impairment often persists. Here we report on a detailed study of a 26-year old, left-handed male, with onset of LKS at age 5 years, who is aphasic for English but who learned British sign language (BSL) at age 13. We have investigated his skills in different language modalities, recorded EEGs during wakefulness, sleep, and under conditions of auditory stimulation, measured brain stem auditory-evoked potentials (BAEP), and performed functional MRI (fMRI) during a range of linguistic tasks. Our investigation demonstrated severe restrictions in comprehension and production of spoken English as well as lip-reading, while reading was comparatively less impaired. BSL was by far the most efficient mode of communication. All EEG recordings were normal, while BAEP showed minor abnormalities. fMRI revealed: 1) powerful and extensive bilateral (R > L) activation of auditory cortices in response to heard speech, much stronger than when listening to music; 2) very little response to silent lip-reading; 3) strong activation in the temporo-parieto-occipital association cortex, exclusively in the right hemisphere (RH), when viewing BSL signs. Analysis of these findings provides novel insights into the disturbance of the auditory speech cortex which underlies LKS and its diagnostic evaluation by fMRI, and underpins a strategy of restoring communication abilities in LKS through a natural sign language of the deaf (with Video)- - - - - - - - - - ranking = 3keywords = speech (Clic here for more details about this article) |
8/15. Landau Kleffner syndrome.An eight-year-old male child presenting with history of generalized convulsions, gradual loss of speech and generalized EEG (electroencephalography) abnormalities was diagnosed as Landau Kleffner Syndrome. He initially developed generalized convulsions which later changed to partial seizures during the course of illness. He was started on sodium valproate and continued with the drug (30 mg/kg/day) on which, he showed improvement in speech, behavior, hyperkinesis and frequency of convulsions during the follow-up.- - - - - - - - - - ranking = 2keywords = speech (Clic here for more details about this article) |
9/15. sign language in childhood epileptic aphasia (landau-kleffner syndrome).Acquired epileptic aphasia (AEA, or landau-kleffner syndrome) is a unique condition in which children can lose oral language (OL) comprehension and expression for a prolonged period. These children can benefit from visual forms of language, mainly sign language (SL), but the quality of SL has never been analyzed. The case is reported here of a boy with AEA who lost speech comprehension and expression from 3 years 6 months to 7 years and was educated in SL from the age of 6 years. His SL was evaluated at the age of 13 years and 6 months and compared with a control child with congenital sensorineural deafness. It was found that: (1) our patient achieved the same proficiency in SL as the control child with deafness; (2) SL learning did not compete with, but perhaps even hastened, the recovery of OL. Intact ability to learn a new linguistic code such as SL suggests that higher-order language areas were preserved and received input from a separate visual route, as shown by neuropsychological and functional imaging research in deaf and hearing signers.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
10/15. landau-kleffner syndrome (acquired epileptic aphasia) in a Nigerian child.epilepsy is one of the commonest disorders presenting the neurological clinics in developing countries. There are attendant complications especially in untreated or poorly treated cases. In this article, we report an uncommon complication known as landau-kleffner syndrome in a Nigerian girl. The syndrome, typically seen in children consists of an acquired aphasia frequently associated with clinical seizures and paroxysmal electroencephalographic (EEG) accompaniment.There had been no adequate neuropathlogical correlations to explain the symptomatology; thus, various forms of pathogenesis have been ascribed to the syndrome. The clinical seizure is easily controlled by an antiepileptic; but speech recovery is variable, and this is usually poor in cases presenting one or two years after onset.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
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