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

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

To clarify the relationship between epileptic attacks and cognitive dysfunction, we examined the serial findings of 123I-IMP single photon emission computed tomography (SPECT) in relation to the intelligence quotient (IQ), assessed by Wechsler Intelligence Scale of Children-Revised, in two female patients with focal cortical dysplasia (FCD) over a 10-year period. The age of patient 1 at the initial assessment was 2 years, and the age of patient 2 was 9 months. They developed complex partial epilepsy in infancy, and were treated with antiepileptic drugs, which remained effective until 11 years of age, when their epileptic attacks recurred. Patient 1, a 14-year-old girl with FCD of the left parietal lobe suffered from dyscalculia, right-left disorientation, and finger agnosia even when she was free of epileptic attacks. Following the recurrence of seizures which occurred every night, she became unable to understand what was said to her. A hypoperfusion area detected by 123I-IMP SPECT was restricted to the left parietal lobe during the seizure-free period, but spread to the temporo-parietal lobes following the recurrence. Her verbal IQ declined from 94 (at 9 years of age) to 63 (at 11 years and 8 months). After her seizures were controlled again (at 14 years and 4 months), the 123I-IMP SPECT findings improved. Patient 2, a 12-year-old girl with FCD of the left frontal lobe, showed cognitive dysfunction. Her verbal IQ declined from 91 (at 7 years and 5 months) to 76 (at 11 years and 8 months) following a recurrence of epileptic attacks. 123I-IMP SPECT showed hypoperfusion in the left frontal lobe, where the accumulation count ratio (left/right ratio) declined from 0.86 (at 3 years) to 0.64 (at 11 years). These findings suggest that epileptic attacks are related to cognitive dysfunction in FCD patients. This cognitive dysfunction appears to correlate with the appearance of hypoperfusion areas, as detected by 123I-IMP SPECT.
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PMID:[Longitudinal study of cognitive function in two patients with focal cortical dysplasia]. 1100 34

Acute measles encephalitis is a typical parainfectious encephalitis in which the brain is diffusely affected clinically and neuropathologically. We present here a 5-year-old case of measles encephalitis with intractable seizures, bitemporal MRI abnormalities, and neurological sequelae of auditory agnosia and aphasia. Pathophysiology of the focal MRI lesions and of expressive aphasia due to auditory agnosia was discussed. We stressed that the status convulsions and its ischemic effect played an important role on the development of the focal lesions rather than focal encephalitis or ADEM. In the course of auditory agnosia and acquired aphasia, the patient transiently expressed some meaningful words. The significance of this episode was discussed based on the sequential changes of symptoms and neuroimaging findings.
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PMID:[A study of measles encephalitis with focal changes on MRI, auditory agnosia and expressive aphasia]. 1114 65

We have observed that patients who suffer from hemiplegia after a cerebral stroke, tend to remove their clothes although it is not necessary to change them while they are in hospital. Not only does this activity make it difficult to manage the ward and carry out rehabilitation, but it also often becomes problematic for home care once the patient has been discharged from hospital. However, there have been no previous reports on this activity. In this study, we examined the characteristics pertaining to clothes removal in hemiplegic patients under home care. The subjects were chronic-stage, cerebral stroke hemiplegic patients hospitalized in the rehabilitation ward of this hospital since the first initial seizure. Once it was established that environmental factors, such as room temperature, were not the primary reason for the patients to try to remove their clothes, the patients were closely monitored and the circumstances under which they tried to remove their clothes (location, time and type of clothes removed) were recorded to examine the relationship among age, sex, side affected by paralysis, higher cortical function, motor paralysis and ADL. Thirty-five percent of the patients, mostly women, usually tried to remove their clothes and the tendency was for them to incompletely remove their tops without reason while they were confined to bed or sitting on the bed. This activity was also prevalent among patients with accompanying diminished intellectual function, left hemiplegia, and left unilateral spatial agnosia. The acquired level of ADL by FIM in the group in which this activity was observed was low, except regarding meals. Thus, it was inferred that in stroke hemiplegic patients being taken care of at home the removal of clothes was related to a diminished intellectual function or ADL, which suggested the importance of family guidance based on observations of the patient's behavior in the ward. Targeting a greater number of subjects, we would like to conduct further studies on home-care measures to deal with this activity.
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PMID:[Characteristics of hemiplegic outpatients with stroke who try to remove their clothes unnecessarily]. 1178 1

A case of a girl affected by Landau-Kleffner syndrome (acquired aphasia with convulsive disorder) is reported. The girl showed epileptic seizures and EEG abnormalities. At the age of 7 years 10 months they were followed by onset of aphasic symptoms accompanied with behavioural disturbances. By the age of 9 years she developed a severe verbal auditory agnosia and loss of spoken language expression, but was able to use reading and writing to communicate. This unusual dissociation suggests that spoken or written language can be affected selectively. The girl was followed up to the age of 14 years 6 months and her language recovered well by this age. (Fig. 2, Ref. 42.)
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PMID:Landau-Kleffner syndrome: a case of a dissociation between spoken and written language. 1188 67

Landau-Kleffner syndrome is characterized by a complex group of symptoms including deterioration in language skills, seizures, and abnormal electroencephalography findings. A six-year-old male patient had aphasia for three years and generalized tonic-clonic epileptic seizures for two years. Pure-tone audiometry and auditory brain-stem response audiometry findings were normal. He had verbal auditory agnosia rather than true aphasia. Cranial computed tomography and magnetic resonance imaging studies did not show any abnormal findings. Single-photon emission computed tomography showed hypoperfusion in the right hemispheric temporal lobe. Despite treatment with corticosteroids and intravenous immunoglobulins, multiple exacerbations were interspersed in a four-year follow-up period.
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PMID:[Landau-Kleffner syndrome (acquired epileptic aphasia)]. 1252 74

Landau-Kleffner syndrome is marked by an acquired aphasia in children who have had normal language and motor development. A 3.5-year-old girl was referred to our clinic with stuttering. She was diagnosed as having benign myoclonic epilepsy of infancy at 3.5 months of age and treated with valproic acid. Her electroencephalogram (EEG) returned to normal at the end of the first year. The therapy was stopped after a 2-year seizure-free period. She started to stutter prominently 3 months after the discontinuation of antiepilepsy drugs. She had no verbal agnosia. Her EEG revealed multiple spike and wave discharges. She was diagnosed as having Landau-Kleffner syndrome. Her previous epilepsy history had contributed to her having obtained an EEG in the early period. We suggest that if a child with normal language function starts to stutter, Landau-Kleffner syndrome must be considered in the differential diagnosis.
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PMID:Landau-Kleffner syndrome beginning with stuttering: case report. 1254 39

In this paper we describe a case of severe visual agnosia in a child with an electrophysiological pattern of continuous spike-wave discharges in slow sleep (CSWS) in the occipito-temporal regions. The neuropsychological spectrum related to this phenomenon is discussed. Published paediatric reports associate visual agnosia (i.e. an inability to recognize objects without impairment of visual acuity) mainly with symptomatic occipito-temporal aetiology (e.g. cortical dysplasia, vascular insults) and other neurological symptoms (e.g. autism). We describe a detailed 2 year electrophysiological and neuropsychological follow-up of an 8-year-old boy with sporadic seizures, occipito-temporal CSWS and visual agnosia. The growth and neurological development of the child had been considered as normal, neurological examination did not reveal any focal signs, visual acuity was intact and MRI was normal. First EEG and six consecutive 24 h video EEG recordings during the follow-up of 22 months showed continuous spike-and-wave activity covering over 85% of the non-REM sleep. According to structured neuropsychological tests (Wechsler Intelligence Scale for Children--Third Edition, A Developmental Neuropsychological Assessment (NEPSY), Test of Visual-Perceptual Skills, Corsi block, Hooper Visual Organization Test) the boy had normal verbal intelligence but major deficits in visual perception, especially in object recognition, impaired shape discrimination and detection, and poor copying skills. Attention and executive functions were intact. There were no difficulties in short- or long-term memory. Verbal cues and naming the objects improved visual memory. Tracing the objects with a finger or by moving the head improved object recognition. Currently the boy attends a special school with a rehabilitation plan including neuropsychological and occupational therapies. This case adds a new facet to the spectrum of neuropsychological deficits in children with CSWS. Sleep EEG should be included in the etiological studies of children with specific neuropsychological problems and detailed neuropsychological assessment is needed for diagnostic and rehabilitation purposes.
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PMID:Visual agnosia in a child with non-lesional occipito-temporal CSWS. 1276 57

Pyridoxine-dependent seizure is a rare autosomal recessive disorder that usually presents with neonatal intractable seizures. This syndrome results from an inborn abnormality of the enzyme glutamic acid decarboxylase, which results in reduced pyridazine-dependent synthesis of the inhibitory neurotransmitter gamma amino butyric acid. The full range of symptomatology is unknown; but can be associated with autism, breath holding and severe mental retardation, bilious vomiting, transient visual agnosia, severe articulatory apraxia motor dyspraxia, microcephaly and intrauterine seizures. Parenteral pyridine injection test is a highly effective and reproducible test in confirming the diagnosis. Pyridoxine should be administered as a diagnostic test in all cases of convulsive disorders of infancy in which no other diagnosis is evident. Epileptic seizure discharges subside within 2-6 minutes after the intravenous injection of 50-100 mg of pyridaoxine. Once the diagnosis is confirmed, maintenance therapy should be continued indefinitely and doses increased with age or intercurrent illnesses. The maintenance dose of Bg needed is still not clear. There is a relatively wide range for the daily B6 dose necessary to control the seizure i.e., 10-200 mg/day.
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PMID:Pyridoxine-dependent seizures: a review. 1288 19

Landau-Kleffner syndrome (LKS) and the syndrome of continuous spikes and waves during slow wave sleep (CSWS) are two points on the spectrum of functional childhood epileptic encephalopathies. They are characterized by a severe paroxysmal EEG disturbance that may permanently alter the critical synaptogenesis by strengthening synaptic contacts that should have been naturally "pruned." The much more common benign epilepsy with centrotemporal spikes is also related to LKS and CSWS by a common pathophysiology. Although prognosis in LKS and CSWS for seizure control is good, cognitive function declines and permanent neuropsychologic dysfunction is seen in many cases. This permanent damage is most evident in those patients who had early-onset EEG abnormality and a prolonged active phase of continuous spike-and-wave discharges during sleep. If the active phase of paroxysmal activity persists for over 2 to 3 years, even successful treatment does not resolve neuropsychologic sequelae. In LKS, the paroxysmal activity permanently affects the posterior temporal area and results in auditory agnosia and language deficits; in CSWS, the frontal lobes are more involved and other cognitive disturbances predominate. Aggressive treatment should include high-dose antiepileptic drugs, corticosteroids, and surgery in specific cases.
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PMID:Epileptic encephalopathy of late childhood: Landau-Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep. 1473 35


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