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At least 19 recordsLinked to original sources
PubMed1992

[Landau-Kleffner syndrome].

The Landau-Kleffner syndrome is characterized by the combination of acquired aphasia and epilepsy. Disturbances appear at an age between 2 and 7 years. The prognosis of epilepsy is favourable, but the outcome of aphasia is different. A 15-year old boy is described as one who is totally unable to communicate through speech even 11 years after onset of aphasia. Diagnostics, aetiology, pathophysiology and new therapeutic possibilities of the syndrome are discussed.

Adolescent↗
PubMedDate not supplied

Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: a PET study.

Three right-handed male children (aged 5, 6, and 11 years) with signs, symptoms and/or history of the syndrome of acquired aphasia-epilepsy (Landau-Kleffner syndrome) were studied during drug-induced, electroencephalographically (EEG)-monitored sleep by positron-emission tomography (PET) and the [18F]fluorodeoxyglucose (FDG) method. Our data demonstrate that in Landau-Kleffner syndrome, cerebral glucose utilization is not normal during sleep. The metabolic pattern varied between the children but the metabolic disturbances always predominated over the temporal lobes. They were right-sided, left-sided, or bilateral. In the two first patients, EEG recordings showed continuous spike-and-wave discharges during sleep and a right-greater-than-left asymmetry was observed in temporal areas. In patient 1, the asymmetry was associated with a relative increase of glucose utilization of the right posterior temporal region. In patient 2, the glucose utilization was relatively decreased in the left anterotemporal and left perisylvian regions. In patient 3, the sleep EEG showed no discharge and no significant asymmetry was observed; however, glucose utilization of both temporal lobes was decreased. Lower metabolic rates in subcortical structures than in cortex were also noted in the three children. This metabolic pattern may be related to the maturation of the central nervous system (CNS).

Aphasia↗
PubMed1992

[Acquired epileptic aphasia--Landau-Kleffner syndrome].

Authors report about a girl with Landau-Kleffner syndrome. After the history of normal developmental milestones at the age of five-and-half year the patient had absence seizures, and marked receptiv and expressiv aphasia developed gradually. She was found to have Landau-Kleffner syndrome. This entity initially can be regarded as deafness or psychiatric reaction. The seizure activity usually has a favorable outcome, the aphasia can fluctuate for years. The improvement is more likely in promptly treated cases.

Aphasia↗
PubMed1992

[Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome].

A case of unusual Landau-Kleffner syndrome was reported. She was an 8 year-old girl and showed non-verbal agnosia, diffuse EEG abnormalities and convulsions. Her responses to both verbal and non-verbal sounds remained inconsistent and unstable. When a continuous spike-wave complexes on EEG was detected, she paid no attention to any sound in spite of her fair consciousness. Auditory brainstem response and magnetic resonance imaging of her brain were normal. Auditory agnosia was correlated well with EEG abnormalities, and valproic acid and clonazepam were effective for EEG improvement. After the EEG improvement, clinical responses to sounds recovered well; firstly she could pay attention to sounds and then she could distinguish between verbal and non-verbal sounds. Finally, she could speak a few words after the learning letters.

Agnosia↗
PubMed1990

Acquired childhood aphasia with convulsive disorder (Landau-Kleffner syndrome). A case report.

The Landau-Kleffner syndrome is a rare form of acquired childhood aphasia associated with convulsive disorder. These children are typically misdiagnosed. A 15-year-old girl whose aphasia was not diagnosed until the age of 13 is described. Pitfalls in diagnosis are discussed, particularly with regard to interpretation of the results of audiometric testing. Attention is drawn to the existence of this rare form of aphasia and to the lack of appropriate educational facilities for aphasic children in general.

Adolescent↗
PubMed1992

Is cerebral arteritis the cause of the Landau-Kleffner syndrome? Four cases in childhood with angiographic study.

Four children with Landau-Kleffner syndrome were studied over a six year period. They presented with acquired aphasia, epilepsy, and focal or generalized EEG discharges which were exacerbated during sleep. In addition, cerebral angiography demonstrated isolated arteritis of some branches of the carotid arteries in all cases. Computed tomographic and magnetic resonance images were normal. Nicardipine in a dose of 1 to 2 mg/kg/day, added to conventional anticonvulsant drugs provided effective supplementary control of seizures, of paroxysmal EEG discharges, and of language and behavioural disturbances, even several years after the onset of the disorder and in patients whose response to other medications, including steroids, had been poor. Interruption of nicardipine administration was followed by relapse of the language disorder. Repeat angiography was performed in all four patients and showed recanalization of obstructed vessels in two cases. Focal cerebral vasculitis may be the pathogenesis of the Landau-Kleffner syndrome and calcium channel blockers such as nicardipine may be effective and specific therapy.

Adolescent↗
PubMedDate not supplied

Landau-Kleffner syndrome: a clinical and EEG study of five cases.

In five children with normal initial psychomotor development, a Landau-Kleffner syndrome appeared at age 3-7 years. No neuroanatomic lesions were noted. Aphasia and hyperkinesia were isolated in three patients and associated with global regression of higher cortical functions in one patient. Massive intellectual deterioration and psychotic behavior were associated with transient aphasia in one patient. The epilepsy (focal motor and generalized tonic-clonic seizures, subclinical EEG focal seizures during sleep, and atypical absences) always regressed spontaneously or with antiepileptic drug (AED) treatment. The EEG in waking patients showed focal and generalized spike-wave discharges on a normal background rhythm. Discharge topography and pattern changed frequently. During sleep, discharges always increased. At some time during syndrome development, all patients had bilateral spike-waves for greater than 85% of the sleep period, while at other times the discharges were discontinuous or continuous but focal or unilaterally hemispheric. Discharge topography and abundance changed from night to night. The abnormal EEG and the impaired higher functions developed and regressed together, but not with strict temporal correlation. Our own experience suggests that the Landau-Kleffner syndrome and epilepsy with continuous spike-wave activity in slow-wave sleep cannot be clearly differentiated. They may be different points on the spectrum of a single syndrome.

Aphasia↗
PubMed1992

The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature.

We present six patients with acquired aphasia with convulsive disorder (Landau-Kleffner syndrome) and distill the main clinical features from a review of the recent literature. Our series showed that the clinical picture can vary at onset, as well as during the course of the illness, and that the long-term outcome of the aphasia is quite unpredictable, despite the fact that epilepsy and electroencephalographic abnormalities usually regress or disappear with the years. We also call attention to the electroencephalographic phenomenon of electrical status epilepticus during slow sleep, and we suggest that the course of the aphasia may well be linked to the appearance and disappearance of electrical status epilepticus during slow sleep. Therefore, we recommend a sleep electroencephalogram in all children with Landau-Kleffner syndrome. Finally, our findings did not demonstrate the beneficial effect of treatment with anticonvulsants on the aphasia, but recent studies have shown that treatment with corticosteroids, whether combined with anticonvulsants, is effective.

Aphasia↗
PubMedDate not supplied

Landau-Kleffner syndrome: a pharmacologic study of five cases.

Five children with Landau-Kleffner syndrome (epilepsy, acquired aphasia, and continuous spike-wave discharges during sleep), were treated with antiepileptic drugs (AEDs), sleep-modifying drugs, and corticosteroids. The pharmacologic profiles differed from those observed in focal epilepsies, resembling instead those of certain generalized epilepsies, such as West or Lennox-Gastaut syndromes. Phenobarbital (PB), carbamazepine (CBZ), and phenytoin (PHT) were ineffective or worsened the EEG and neuropsychological symptoms, whereas valproate (VPA), ethosuximide (ESM), and benzodiazepines were partially or transiently efficacious. Dextroamphetamine produced a dramatic but transient improvement in waking and sleep EEG in one of two children; aphasia did not change. Corticosteroid treatment resulted in improved speech, suppression of seizures, and normalization of the EEG in three of three children. Our own experience and data from the literature suggest that corticosteroids should be given in high doses as soon as the diagnosis is firmly established and should be continued in maintenance dose for several months or years to avoid escape. Early diagnosis, before mutism or global deterioration develops, appears to be essential for effective therapy with minimal neuropsychological sequelae.

Adrenal Cortex Hormones↗
PubMed1992

Regional cerebral perfusion in Landau-Kleffner syndrome and related childhood aphasias.

Assessment of cerebral perfusion may elucidate pathogenesis of Landau-Kleffner syndrome (LKS). We obtained 99mTc-HMPAO SPECT studies in five children with LKS and in three children with syndromes of verbal-auditory agnosia. In LKS, perfusion showed temporoparietal asymmetry (9.56% +/- 3.44%) (n = 4) or bilateral parietal abnormality (n = 1). SPECT in non-LKS patients was normal (n = 1), showed (n = 1) totihemispheral hypoperfusion accompanying structural abnormality or (n = 1) a pattern resembling but distinct from LKS. Seizures in LKS patients had never occurred (n = 1), were controlled satisfactorily (n = 2), or poorly (n = 2). Maximum EEG abnormality was left centrotemoral-occipital (n = 1), left frontocentral (n = 1), bitemporal/left central (n = 1), and left central/parasagittal (n = 1). Asymmetric temporoparietal perfusion appears characteristic of LKS, differing from findings in other childhood linguistic disturbances. This abnormality occurs across a spectrum of seizure expression, diverging from the distribution of EEG abnormalities. The SPECT abnormalities parallel PET-defined LKS metabolic abnormalities, and may indicate central pathogenetic features of the disorder.

Aphasia↗
PubMed1990

[Disorders of speech and hearing in the Landau-Kleffner syndrome].

The authors demonstrate a child with Landau-Kleffner's syndrome, i.e. the syndrome of so-called acquired aphasia of child age associated with paroxysmal EEG activity, which was described in 1957 by the above mentioned authors. It is characterized by loss of speech (receptive and expressive function), EEG changes, behavioral disorders, psychomotor disorders and epileptic seizures. In the discussion the authors advocate the term "so-called epileptic aphasia", as at the onset of the disease acoustic agnosia is not involved but rather sensory aphasia with subsequent impairment of the expressive speech component and epileptic EEG activity.

Aphasia↗
PubMed1992

A role of autoimmunity in the etiopathogenesis of Landau-Kleffner syndrome?

Four children, 3 boys and 1 girl, with the syndrome of acquired aphasia Landau-Kleffner were followed up during the last 10 years. In 3 children an episodic form of the disease with a good response to corticosteroid treatment and with a favourable prognosis was observed. In the other 1 no improvement of speech function was observed and also epileptic fits were reduced only with difficulty. A positive autoimmune reaction to central (MOZAN) and peripheral (LISAN) myelin was observed repeatedly during the attacks of clinical worsening. On the contrary, during the periods of improvement of language disturbances during the treatment with corticosteroids this hypersensitivity to central as well as peripheral myelin disappeared. Possible changes in myelinization and the role of autoimmune reactions in the etiopathogenesis of the disease are discussed.

Adolescent↗
PubMed1990

[Landau-Kleffner syndrome].

In 1957, Landau and Kleffner reported six cases of a "syndrome of acquired aphasia with convulsive disorder". Since this first report more than 130 cases with acquired language disabilities with EEG-abnormalities have been described. Comprehension of language is primarily affected, spike discharges are predominantly found in temporal regions. Facultative symptoms are epileptic seizures and psychiatric disorders. The paper gives a review of the literature describing the clinical manifestations, the therapeutic procedure, the prognosis and the etiological factors of this syndrome, which is even listed in the last version of the ICD-10 and the International Classification of Epilepsies and Epileptic Syndromes.

Adolescent↗
PubMed1992

Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome).

We report a girl 3 years and 6 months old with onset of aphasia at age 3 years and 3 months. There was no evidence of brain damage and there were no seizures. The neuropsychological evaluation showed that the girl tended to be right-handed, that aphasia was global and that other higher cortical functions seemed to be preserved. Isolated spikes and spikes-and-wave were recorded during wake over the right temporal region with rare independent contralateral abnormalities. During polysomnography (PSG), the physiological patterns of sleep were preserved and right temporal epileptiform discharges were significantly increased in all sleep stages. Maximal activation was obtained at sleep onset and during rapid eye movement (REM) sleep periods, when focal abnormalities became continuous and spread contralaterally. Repeat PSGs showed that the activation profile retained this particular trait, although subclinical discharges tended to increase during slow wave sleep (SWS). This pattern of subclinical temporal status epilepticus during REM sleep differs from the characteristic activation profile found in the syndrome of continuous spikes-and-waves during SWS. However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up.

Anticonvulsants↗
PubMed1990

[Epilepsy-acquired aphasia syndrome with psychosis. Report of a case ].

We report the case of a boy whose development was normal until the age of three when regression with loss of speech occurred. Other anomalies included eating and sleep disorders, sterotyped behavior disorders, suggesting infantile psychosis. The electroencephalogram evidenced paroxysmal anomalies, particularly during sleep, with no clinical seizures. The diagnosis of epilepsia-acquired aphasia syndrome (Landau-Kleffner syndrome) was made. The psychotic disorders were not considered as a differential diagnosis but rather as intertwined with the elements of the syndrome. The relationship between acquired aphasia and psychosis are discussed.

Aphasia↗
PubMed1992

Epileptic electroencephalographic abnormalities and developmental dysphasias: a study of 32 patients.

The relationships between severe developmental dysphasias and epilepsy were analysed in 32 patients with congenital dysphasias. The mean age was 8 years 2 months; 19 of 32 had never had seizures; 9 had had occasional seizures; 4 were epileptic. Twenty-two of 32 had normal repeated standard EEGs, but 10 (2 of which never had seizures) showed epileptic interictal discharges. During prolonged EEG after sleep deprivation, epileptic abnormalities were observed in 13 of the 32 cases (4 of which never had seizures). The overall night sleep recordings showed epileptic abnormalities in 30 of the 32 cases (17 of which had never had seizures). The epileptic interictal abnormalities varied considerably in intensity and aspect in the same patient from one examination to another. Developmentally aphasic children show a higher incidence of abnormal EEG than expected, particularly during overall night recordings. In most cases, the physiopathology of the language disturbance might be identical to that in Landau-Kleffner syndrome.

Adolescent↗