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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report six children with acquired aphasia of unknown etiology. The clinical picture was clearly different from that seen in the usual childhood aphasias and resemble other cases initially reported as "syndrome of acquired aphasia with convulsive disorder". All had associated paroxysmal EEG abnormalities, and 5 have had clinical seizures. The language disorder has improved or remained stationary and no other neurological signs have developed. Our review of the literature and the study of our personal cases show no uniform clinical picture in these children. Three different clinical patterns seem to emerge. The first group show rapid onset and recovery of aphasia, frequent fluctuations in the severity of the language deficit typical of so-called epileptic aphasia. These children appear to have a better prognosis. The second group show worsening of the aphasic deficit after repeated seizures or episodes of aphasia. In the third group progressive deficit in language comprehension (auditory agnosia) with a variable degree of recovery and rare or no clinical seizures. The possible significance of the EEG abnormalities has been discussed and the importance of the aphasia on general behavior and the problems of differential diagnosis have been stressed.
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PMID:Acquired aphasia in childhood with seizure disorder: a heterogeneous syndrome. 57 97

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.
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PMID:Is cerebral arteritis the cause of the Landau-Kleffner syndrome? Four cases in childhood with angiographic study. 137 38

Four children treated for seizures between 1980 and 1986 were diagnosed as having Landau-Kleffner syndrome (acquired aphasia with convulsive disorder), following the onset of aphasia. They received early and prolonged ACTH or corticosteroid therapy, with high initial doses. In all four cases the EEG promptly became normal, with subsequent long-lasting remission of the aphasia and improvement of seizure control. Three to six years after discontinuation of hormone therapy the children are off medication and free from seizures and language disability.
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PMID:Effect of early corticosteroid therapy for Landau-Kleffner syndrome. 170 23

Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (Landau-Kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.
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PMID:Acquired aphasia, dementia, and behavior disorder with epilepsy and continuous spike and waves during sleep in a child. 171 72

Eighty-two school-age children with severe and persistent specific speech and language disorders were studied. 71 had specific developmental language disorders, three had structural malformations (cleft palate) and eight had disorders acquired after a period of normal language development, including five with Landau-Kleffner syndrome. The sex ratio was 3.8 boys to one girl. Nearly half had a family history of speech-language disorder, with one in 5.2 affected siblings. Aetiological factors were found in 26 per cent: 11 per cent prenatal, 3 per cent perinatal and 12 per cent postnatal. 21 per cent had had a seizure and 7 per cent had had seizures after the age of eight. 29 per cent were left-handed, 90 per cent were clumsy and 22 per cent first walked after 18 months. The complex origins of specific speech and language disorders are discussed.
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PMID:Causes and associations of severe and persistent specific speech and language disorders in children. 172 Jul 49

The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. An encephalitic etiology has often been postulated but never proved. We report two patients with this syndrome who were treated surgically. Despite careful search, no pathologic evidence of encephalitis was found. One patient, with the typical course, had no seizures but striking positive correlation between epileptic discharge and language disorder; the second, after classic onset, developed intractable temporal lobe epilepsy, a previously unreported outcome of this syndrome. EEG discharges are generalized, bilateral, multifocal, or with shifting predominance but mainly temporal in 85% of reported cases, and unilateral, also predominantly temporal, in 15%. Language areas are preferentially involved. This syndrome has certain biologic features that resemble the benign epilepsies of childhood and may be the result of the unusual localization of the epileptic abnormality.
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PMID:The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis. 244 19

A healthy 7-year-old boy developed a language disorder associated with clinical seizures and a paroxysmal EEG. Computed tomography and magnetic resonance imaging revealed a small cysticercus deep in the left Sylvian fissure. We postulate a cause and effect relationship between the parasitic cyst and the clinical manifestations supported by the strategic location of the cyst and the critical age at which this lesion developed. This case provides evidence that the syndrome of acquired epileptic aphasia may be explained in terms of an unilateral structural brain lesion.
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PMID:Acquired epileptic aphasia (the Landau-Kleffner syndrome) due to neurocysticercosis. 247 38

Twenty children (15 males and 5 females) suffering from a particular type of myoclonic epilepsy were submitted to a longitudinal study. All children were neurologically normal. Familial antecedents existed for epilepsy in 25% of the cases (5/20) and for febrile convulsions in 15% (3/20). The first fit appeared with fever at the mean age of 6 months in all cases but one of clonic type. Frequent similar febrile or afebrile clonic seizures recurred in all subjects before the age of 12 months. At this time the EEG was normal in 14 cases and brief discharges of generalized spike-waves during ILS or during sleep were present in 6 cases only. Later, frequent non-febrile clonic unilateral or generalized fits, frequent atypical 'absences' often accompanied by jerks, high photosensitivity and non-epileptic erratic myoclonias appear. Nevertheless, atomic and/or tonic seizures did not appear. The evolution is characterized by the persistence of fits and the appearance of severe language disorder and light cerebellar and pyramidal signs. The authors present their results and discuss the nosological problems of this severe infant myoclonic epilepsy.
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PMID:[Severe infant myoclonic epilepsy (author's transl)]. 680 12

A boy aged 5 had displayed from the age of 3 a fluctuating deficit in understanding spoken language. Audiometric testing proved his hearing to be normal. After 18 months, the parents noticed minor attacks of 'absence'. An EEG showed a normal background pattern and frequent spikes and spike waves complexes; CT-scanning of the brain revealed no abnormalities. On the basis of the acquired language disorder, the seizures and the features of the nocturnal EEG, the diagnosis the syndrome of Landau-Kleffner was made. After treatment with ethosuximide a temporary improvement of the aphasia occurred. A permanent improvement however was realized by treatment with prednisone. The boy could attend a primary school. The syndrome runs a fluctuating course; the patient may recover, but he may also undergo general mental deterioration. Antiepileptic agents only may have a brief favorable effect on the aphasia. In case of failure corticosteroids or even subpial cortical transsection are indicated.
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PMID:[A special case of 'deafness'; Landau-Kleffner syndrome]. 954 68

Previous studies of children with Landau-Kleffner syndrome and related language-epilepsy syndromes have focused on the relationship of seizure control to language recovery. We examined the effect of premorbid language skills and behavior, as well as some characteristics of clinical seizures and electroencephalograms, on language recovery in a retrospective study of 67 children with the severe receptive and expressive language disorder, verbal auditory agnosia. Fifty-eight percent of these children had seizures, 76% were autistic, and 24% had a history of language regression after showing previously normal language skills. The duration of language loss was not influenced by the persistence of clinical seizures. Premorbid language and behavior were more predictive of language recovery in these children. Most children with normal early language (acquired verbal auditory agnosia) had onset of language loss after age 3 years, in contrast to those with abnormal early language. Children with acquired verbal auditory agnosia were more likely to show fluctuations in language skills than those in other groups. Autistic children were more likely to begin having seizures before age 3 years, and had a longer duration of language loss and lower educational placement at time of last follow-up than those with normal behavior. This study emphasizes the importance of assessing premorbid language and behavior in predicting recovery of language skills in children with language-epilepsy syndromes.
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PMID:The influence of premorbid language skills and behavior on language recovery in children with verbal auditory agnosia. 1064 9


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