Cases reported "Landau-Kleffner Syndrome"

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1/46. 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. ( info)

2/46. 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. ( info)

3/46. magnetoencephalography in presurgical evaluation of children with the landau-kleffner syndrome.

    PURPOSE: Our aim was (a) to localize the primary epileptogenic cortex for possible multiple subpial transsection in four children with the Landau-Kleffner syndrome (LKS), and (b) to evaluate the impact of magnetoencephalography (MEG) in the localizing process. methods: We used EEG to detect the overall epileptiform activity and MEG for selective recording of fissural spikes. The cortical generators of MEG spikes were modeled with dipoles, and their activation order was determined. The voltage distribution, consistent with the earliest MEG sources, was then identified during the course of the patient's EEG spikes to determine the relative timing between stereotypic EEG and MEG spikes and to distinguish the earliest (primary) source area among the secondary ones. RESULTS: In all patients, the earliest spike activity originated in the intrasylvian cortex, spreading in one subject to the contralateral sylvian cortex within 20 ms. Secondary spikes occurred within 10-60 ms in ipsilateral perisylvian, temporooccipital, and parietooccipital areas. A single intrasylvian pacemaker initiated all epileptic activity in two patients, whereas the other two had independent left- and right-hemisphere circuits or focal spikes. MEG source dynamics predicted the results of the methohexital suppression test in two patients and was confirmed by surgery outcome in one patient, in whom all epileptic activity ceased after a small transsection of the sylvian pacemaker. CONCLUSIONS: (a) The intrasylvian cortex is a likely pacemaker of epileptic discharges in LKS, and (b) MEG provides useful presurgical information of the cortical spike dynamics in LKS patients. ( info)

4/46. Acquired aphasia in acute disseminated encephalomyelitis.

    A 12-year-old boy developed a convulsion, hemiparesis, and acquired aphasia with paroxysmal electroencephalogram (EEG) abnormalities consisting of repetitive spikes and waves in the left centro-parietal region. T2-weighted magnetic resonance imaging disclosed high intensity lesions in the left pre-Sylvian and right frontal areas. He was diagnosed as having acute disseminated encephalomyelitis, and thus the oral administration of phenytoin and steroid pulse therapy were begun. With these treatments, his hemiparesis disappeared and the aphasia also improved gradually. magnetic resonance imaging examination revealed the disappearance of the previously noted abnormalities, and the EEG abnormalities disappeared as well. This patient is a rare case of acute disseminated encephalomyelitis presenting an acquired aphasia. A focal lesion of acute disseminated encephalomyelitis may be responsible for the acquired aphasia. The distinction from landau-kleffner syndrome is also discussed. ( info)

5/46. Outcome of multiple subpial transections for autistic epileptiform regression.

    Treatment options for atypical forms of landau-kleffner syndrome (LKS) are not well delineated. Many patients with typical LKS fail to respond to antiepileptic drug treatment, but some benefit from multiple subpial transections (MSTs). The authors report seven patients with autism or autistic epileptiform regression who responded in varying degrees to MSTs after failed medical management. These patients derived from an original cohort of 36 children (29 males, seven females, ranging from 2 years, 3 months to 11 years, 3 months, mean age = 5 years, 8 months) with a history of language delay or regression, as well as varying degrees of social and behavioral abnormalities, who were evaluated with video-electroencephalogram (EEG) monitoring over a 2-year period. Fifteen patients had clinical seizures (11 of the 19 children with autistic epileptiform regression and four of 12 autistic children). epilepsy was refractory to medication in seven. Surgical treatment variously involved MSTs of the left neocortex in temporal, parietal, and frontal regions, often including regions within the classic perisylvian language areas. One patient also had a left temporal lobectomy. In all seven patients, seizure control or EEG improved after MSTs. Language, social, and overall behavior improved to a moderate degree, although improvements were temporary in most. Autistic epileptiform regression resembles LKS in that both may respond to MST. MST is used to treat epilepsy in eloquent regions. The responsiveness of autistic epileptiform regression to MST buttresses the argument that autistic epileptiform regression is a form of focal epilepsy. ( info)

6/46. Autistic regression with rolandic spikes.

    OBJECTIVE: This study was designed to further define the electroencephalographic abnormalities seen in the landau-kleffner syndrome variants and the associated clinical features. BACKGROUND: landau-kleffner syndrome is rare, but its putative variants are more common. METHOD: We report two patients with centro-temporal spikes, autistic epileptiform regression, and variably prominent oro-motor symptoms. RESULTS: The epileptic aphasia pattern found among patients with prominent Rolandic spikes may more frequently involve expressive language than is seen in the typical landau-kleffner syndrome, where verbal auditory agnosia is the rule. CONCLUSIONS: This clinical difference likely reflects the location of the epileptiform activity (centrotemporal as opposed to anterior or mid-temporal) on buccal-lingual function, vocalization, and language production. ( info)

7/46. landau-kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment.

    landau-kleffner syndrome (LKS) is a rare childhood disorder characterized by acquired aphasia with seizures and electroencephalogram (EEG) abnormalities. Tc-99m-HMPAO SPECT was performed in three right handed children with LKS. A relative decrease in perfusion was found in the left temporal cortex of all three patients and also in the left frontoparietal cortex of one patient with hyperkinetic behavior. Degree of regional cerebral perfusion impairment did not correlate with the severity of clinical and EEG abnormalities. Asymmetrical temporoparietal perfusion appears characteristic of LKS. SPECT findings in LKS were evaluated as useful in elucidating the pathogenic features of the disorder in the brain. ( info)

8/46. Childhood epilepsy with neuropsychological regression and continuous spike waves during sleep: epilepsy surgery in a young adult.

    We describe the case of a man with a history of complex partial seizures and severe language, cognitive and behavioural regression during early childhood (3.5 years), who underwent epilepsy surgery at the age of 25 years. His early epilepsy had clinical and electroencephalogram features of the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia (landau-kleffner syndrome), which we considered initially to be of idiopathic origin. seizures recurred at 19 years and presurgical investigations at 25 years showed a lateral frontal epileptic focus with spread to Broca's area and the frontal orbital regions. Histopathology revealed a focal cortical dysplasia, not visible on magnetic resonance imaging. The prolonged but reversible early regression and the residual neuropsychological disorders during adulthood were probably the result of an active left frontal epilepsy, which interfered with language and behaviour during development. Our findings raise the question of the role of focal cortical dysplasia as an aetiology in the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia. ( info)

9/46. 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. ( info)

10/46. 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. ( info)
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