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

Clinical and Neuropathological data on sixteen cases of progressive myoclonic encephalopathy are reported. This neurological syndrome appears after an average duration of thirty two months of haemodialysis and leads to death in four and a half months, and is characterized by myoclonus, speech disorder, epileptic seizures, and mental-status changes. At first, clinical signs and symptoms are related to haemodialysis, later they become permanent. An early diagnosis is based on EEG which is the only useful laboratory test, demonstrating bisynchronous slow-wave bursts. The caracteristic histopathologic findings are neuronal depopulation, lipofuscin accumulation, and appearance of Neurofibrillary degeneration, especially in Motor cortex, red nucleus and dentato-olivary systems. It seems to be justified to attribute P.M.D.E. to aluminium chronic poisonning; the source of the aluminium intoxication is not aluminium containing phosphate-binding gels but intravenously administreted tape-water. The intracellular binding of aluminium is shown from a histochemical study employing fluorescent stain Morin.
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PMID:[Progressive myoclonic encephalopathy in dialysis patients. Clinical, electroencephalographic and neuropathological study. Pathogenetic discussion]. 10 55

Of 965 children with neurological disorders seen in the Child Neurology Clinic of the University of Nigeria Teaching Hospital, Enugu over a 3-year period (1985-87), 80 (8.3%; 41 boys and 39 girls) had speech problems. The most common speech disorder was dyslalia. Twenty-six (32.5%) of the 80 children were mentally retarded. Besides speech problems, some of the children had other neurological disorders such as hyperactivity, recurrent seizures, microcephaly and deafness. Varying degrees of improvement in speech were observed in only eight girls and four boys, amounting to 24% of the 50 who were followed up for a period ranging from 3 months to 3 years (mean follow-up period 12.26 months). Eight of those who improved (66.6%) did so within the first 18 months of follow-up. There was a disturbingly high rate of default from follow-up, 30 of the patients (37.5%) failing to keep appointments at the clinic after the first assessment. Also, there was a long delay between onset of symptoms and presentation to hospital (mean delay 45.3 months). With the recent acquisition of the services of a speech therapist by the hospital, it is hoped that the prognosis for children with speech problems will be considerably improved.
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PMID:The pattern and prognosis of speech disorders among children in Enugu, Nigeria. 171 91

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

This report describes our experience with CT scanning in a patient with idiopathic familial basal ganglia calcifications. The clinical symptoms were characterized by late onset of progressive mental deterioration, seizures, involuntary movements and speech disorder. An extensive deep cerebral calcification was clearly visualized with CT as was midbrain and cerebellar calcification. Our communication is the first which documents CT profile of this rare disorder.
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PMID:Computed tomographic demonstration of idiopathic familial basal ganglia calcification. 685 2

We describe a family of 9 affected individuals in three generations with nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, and centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment. The speech disorder was prominent, but differed from that of Landau-Kleffner syndrome and of epilepsy with continuous spike and wave during slow-wave sleep. The electroclinical features of this new syndrome of autosomal dominant rolandic epilepsy resemble those of benign rolandic epilepsy, a common inherited epilepsy of childhood. This family shows clinical anticipation of the seizure disorder, the oral and speech dyspraxia, and cognitive dysfunction, suggesting that the genetic mechanism could be expansion of an unstable triplet repeat. Molecular studies on this syndrome, where the inheritance pattern is clear, could also be relevant to identifying a gene for benign rolandic epilepsy where anticipation does not occur and the mode of inheritance is uncertain.
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PMID:Autosomal dominant rolandic epilepsy and speech dyspraxia: a new syndrome with anticipation. 757 60

Alteration of speech is a rare but distressing complication of orthotopic liver transplantation (OLT). We describe a characteristic speech disorder identified in a large series of consecutive patients undergoing OLT. Between 1988 and 1993, 525 adults underwent OLT. For all recipients with neurologic complications, we reviewed clinical findings, imaging and electrophysiologic test results, and perioperative laboratory data. Five patients (ages 23-52; UNOS status 3-4) exhibited a characteristic pattern of stuttering dysarthria, leading to complete loss of speech production, occasionally with elements of aphasia. In four of the five patients, right-sided focal seizures were subsequently noted. All cases presented within the first 10 postoperative days and improved with 1 month of cessation of cyclosporin (CyA), although halting, monotonous speech was evident to some degree in all five for up to 1 year. There was no correlation between onset of symptoms and CyA levels. None of the patients has clinical or radiologic findings suggestive of central pontine myelinolysis or akinetic mutism. EEGs and Spect scan results were consistent with dysfunction in the left frontotemporoparietal regions of the brain. A characteristic speech disorder, which may be described as cortical dysarthria or speech apraxia, occurs in approximately 1% of adults undergoing OLT. Prompt recognition of this syndrome and temporary cessation of CyA therapy may favorable affect the course.
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PMID:Loss of speech after orthotopic liver transplantation. 762 86

Acquired stuttering is an uncommon speech disorder. Supplementary motor area (SMA) lesions have been reported to be directly or indirectly related to acquired stuttering and various types of motor dysfunction. We report on a patient who presented with both acquired stuttering and long-lasting gait disturbance after SMA seizure.
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PMID:Stuttering and gait disturbance after supplementary motor area seizure. 1537 7

KBG syndrome comprises a distinct facial phenotype, macrodontia, short stature, and skeletal anomalies. So far, it has been reported in 29 individuals. Recently, diagnostic criteria were outlined. Here, we describe eight new patients whose clinical and radiological findings fit the diagnostic criteria of KBG syndrome. While most patients were sporadic in occurrence, in two families the disorder was transmitted from mildly affected mothers to their affected children. The phenotype of KBG syndrome has been reviewed based on published and present patients. EEG anomalies with or without seizures, mixed hearing loss, palatal anomalies with secondary speech disorder, distinct age-related behavior, and cryptorchidism are possible additional characteristics. Less common manisfestations were posterior fossa malformations, eye defects, and congenital heart defects.
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PMID:KBG syndrome in a cohort of Italian patients. 1552 20

Clinical and neurophysiological analysis of a case of a 7 year old patient with typical benign partial seizures with rolandic spikes and speech disorder, differing from those in Landau-Kleffner syndrome and in typical benign partial epilepsy of childhood presenting as speech dyspraxia. Two independent foci (in the premotor cortex of the left front lobe (dominant hemisphere) and in the temporal lobe of the right hemisphere were found. Significant clinical improvement and electrographical positive effect in EEG were achiered after prednisolone and sodium valproate treatment.
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PMID:[Bifocal atypical rolandic epilepsy with speech dyspraxia]. 1584 64

Retigabine has anticonvulsant properties that appear to be primarily mediated by opening neuronal voltage-gated potassium channels. This action has been shown in neuronal KCNQ2/3 and KCNQ3/5 potassium channels. In addition to this unique action, retigabine also potentiates GABA-evoked currents in cortical neurons at high concentrations. When used as adjunctive therapy in patients with partial seizures, retigabine 600-1200 mg/day (200-400 mg three times daily) was associated with significant linear dose-dependent reductions in monthly seizure frequency compared with placebo in a large 16-week randomised phase II trial. Median monthly seizure frequency decreased from baseline by up to 35% among patients in the retigabine treatment arms compared with 13% in the placebo group. Retigabine 1200 mg/day was also significantly more effective than retigabine 600 mg/day. Responder rates, defined as the proportion of patients with > or = 50% reduction in seizure frequency, were significantly higher among patients in the retigabine 900 and 1200 mg/day groups than in those who received placebo. CNS-related adverse events were the most commonly reported treatment-emergent adverse events associated with retigabine in clinical trials. Across all three retigabine groups in the large phase II trial, somnolence (20.3%), dizziness (14.6%), confusion (12.3%) and speech disorder (11.3%) were the most frequent CNS-related adverse events.
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PMID:Retigabine: in partial seizures. 1680 Jul 18


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