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

The syndome of acquired aphasia with associated seizures in children has been known since the first report in 1957. Since then, 42 cases have been reported. This paper presents a review of these cases and discusses clinical features of three additional children in whom the specific syndrome of auditory verbal agnosia was identified. Presenting features, medical and neurological findings, and results of detailed psychometric and speech and language evaluations are discussed. The syndrome should be suspected in any child who develops loss of previously acquired language, preceded, accompanied, or followed by seizures. Joint diagnostic evaluation and therapeutic planning by neurologists, speech-language pathologists, and teachers are recommended.
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PMID:Acquired auditory verbal agnosia and seizures in childhood. 9 2

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

A case of isolated, pure and paroxystic auto-prosopagnosia in a young man aged 19 years and 10 months, followed until age 21 years and 6 months, is described. He also exhibited petit-mal seizures since the age of 18 years, these two pathological manifestations being chronologically independent. Clinically, were noted a total absence of hetero-prosopagnosia, color-agnosing agnosia for objects or body scheme disorders. The ophtalmologic examination was totally normal. The psychologie study was performed with the aid of the psychodiagnostic tests of Rorschach, Fuchs, Kataguchi and with the "Symbol-Test" of Roemer, and the result obtained, as well as their psychodynamic meanings are detailed. Disappearance, at the age of 21 years and 6 months, of the auto-prosopagnosia and rarefaction of the petit-mal seizures. In a brief survey of the literature, the author underlines the indisputable scarcity of the isolated and paroxystic forms of auto-prosopagnosia as well as their association with epilepsy.
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PMID:[Paroxystic auto-prosopagnosia and epilepsy. Physio-pathological correlation or chance association?]. 82 21

The selective inability to comprehend the spoken word, in the absence of aphasia or defective or defective hearing, is defined as pure word deafness (auditory verbal agnosia). Reported cases of this rare disorder have suggested the site of involvement to be strategically placed, interrupting fibers from left and right primary auditory receptive areas which project to Wernicke's are in the dominant hemisphere. Our patient is a 44-year-old male who suffered from an uncertain illness complicated by fever, jaundice and generalized seizures seven years previously. Following an apparent convulsion, the patient was noted to be unable to understand spoken language without loss of ability to recognize and respond to sounds or marked impairment of speech or reading. The evidence suggested bilateral cerebral hemisphere disease more marked on the right. The abrupt onset without progression is consistent with a vascular or ischemic etiology. Conclusions about the nature of the lesion and areas involved must await further studies and ultimately tissue examination.
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PMID:Pure word deafness. (Auditory verbal agnosia). 84 5

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.
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PMID:Regional cerebral perfusion in Landau-Kleffner syndrome and related childhood aphasias. 138 78

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.
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PMID:[Disorders of speech and hearing in the Landau-Kleffner syndrome]. 169 48

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

We evaluated, at our institute, 70 females with Rett syndrome between 2 1/2 to 34 1/2 years old. This provided an opportunity of observing the natural history of this condition. The evolution of a subacute encephalopathy of very early onset, maximizing in the second year of life, with slow recovery and devastating sequelae, was recognized. The hyperorality, visual auditory and tactile agnosia with aphasia and seizures resembled symptoms described in human Kluver Bucy syndrome. Over interpretation of behavioral abnormalities as seizures was common. Scoliosis was not a necessary concomitant of age. A consistent biochemical or neurophysiological abnormality was not detectable in understanding the cause and pathogenesis of this disease process. Life span appears to be unaffected though life tables have not yet been established.
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PMID:Rett syndrome--natural history in 70 cases. 308 4

Since the initial description by Landau and Kleffner, in 1957, of a syndrome occurring exclusively in children and consisting of aphasia, paroxysmal changes in E.E.G. and, frequently, epileptic seizures, 80 cases have been reported. The acquired speech disorder, sometimes associated with auditory agnosia, differs in symptomatology and development from the usual forms of infantile aphasia. The prognosis, based on the regression or persistence of the aphasia, is favourable in 60% of the cases and unfavourable in 40%. None of the various antiepileptic drugs is consistently effective. The cause of the syndrome remains unknown.
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PMID:[The Landau-Kleffner syndrome. Infantile "acquired" aphasia, paroxysmal electroencephalographic changes and epileptic seizures]. 618 18

A patient with intractable partial seizures was intensively studied before surgical removal of the epileptogenic focus. A subdural electrode array was surgically placed over the left temporoparietal cortex to better localize the epileptogenic focus and localize cortical function. In addition to speech and sensory findings, acalculia, agraphia, right-left confusion, and finger agnosia were transiently produced by electrical stimulation in the perisylvian area. These findings and their relationship to the controversy surrounding Gerstmann's syndrome are discussed.
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PMID:Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation. 653 64


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