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

Certain behaviors that occur during a complex partial seizure (CPS) are useful in lateralizing the side of seizure onset. In 5 (5.3%) of 94 consecutive patients with partial epilepsy, we observed ictal unilateral arm and hand paresis during 27 of 34 CPS. In all these seizures, this behavior occurred contralateral to an epileptogenic temporal lobe, as determined by video-EEG monitoring and surgical outcome. In 5 of the 27 seizures, an observer demonstrated that the paretic arm and hand were flaccid. None of these patients had postictal (Todd's) paralysis. In most of the seizures, the arm ipsilateral to seizure onset had simultaneous purposeful movements or automatisms, sometimes with awkward posturing. Ictal unilateral paresis is distinctly different from ictal dystonia or postictal paralysis and consistently lateralizes seizure onset to the contralateral temporal lobe. Recognition of this particular ictal behavior and comparison to other simultaneous behaviors can aid in the lateralization and possibly localization of the epileptogenic zone.
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PMID:Ictal contralateral paresis in complex partial seizures. 755 83

This is the first reported case of dystonia with a partial deletion of the long arm (q) of chromosome 18. Neurologic findings in the 18q- syndrome include mental retardation, seizures, nystagmus, incoordination, tremor, and chorea. A 36-year-old woman with an 18q terminal deletion [karyotype 46,XX,del(18)(q22.2)] had hypothyroidism, diabetes mellitus, borderline intelligence, short stature, short neck, sensorineural hearing loss, and sensorimotor axonal neuropathy. Parents' karyotypes were normal. She had had incoordination and writing difficulty since childhood. Posturing and tremor of the head began at age 16, followed by arm tremors. She had jaw deviation and tremor, neck tremor with retrocollis, involuntary pronation of the right arm, coarse postural and severe action tremor, and tight pen grip with dystonic wrist extension on writing. The 18q- syndrome should be added to the list of genetic causes of secondary dystonia. A karyotype analysis should be considered in secondary dystonias, particularly when there are associated features such as short stature and endocrinopathies.
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PMID:Dystonia in a patient with deletion of 18q. 756 32

We evaluated head turning in 239 complex partial seizures (CPS) with or without generalization in 32 patients with unilateral temporal lobe epilepsy (TLE). Head turns occurred in 187 seizures of 31 patients, more than once in 71 seizures. The first head turn was ipsilateral to the focus in 162 seizures (87%), with a mean latency of 22 s, as compared with 83 s for contralateral first head turns. Concomitant dystonic posturing of the arm occurred with 71% of all ipsilateral head turns and with 16% of all contralateral head turns. The mean difference in absolute latency between the first head turn and concomitant dystonic posturing was 6 s. Examination of all instances of concomitant head turning and dystonic posturing (160) showed them to be contralateral to each other in 154 (96%). Furthermore, the dystonia was contralateral and head turning was ipsilateral to the focus in 149 (93%). Forty-one seizures secondarily generalized, with transitional tonic head deviation contralateral to the focus in 35. Early head turning suggests an ipsilateral temporal seizures focus, particularly when associated with contralateral dystonic posturing. Similar mechanisms may account for both. Tonic head deviation preceding secondary generalization probably has a different mechanism.
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PMID:Association of ipsilateral head turning and dystonia in temporal lobe seizures. 758 49

We report on a 12-year-old Chinese child with type C Niemann-Pick disease, who presented primarily with neurologic symptoms. He started to develop ataxia and dysarthria at the age of six years. Dementia, dysphagia, dystonia and seizures, in that sequence, followed within a couple of years. He was anarthric and bedridden five years after onset. Supranuclear vertical gaze palsy was found at the beginning of the illness. However, no hepatosplenomegaly or other physical abnormality was noted. Bone marrow aspirates revealed foamy storage cells and sea-blue histiocytes. However, sphingomyelinase activity in the cultured skin fibroblast was normal. The characteristic clinical presentations and typical pathologic and histochemical findings meet the diagnostic criteria of type C Niemann-Pick disease. We report the first Chinese case of type C Niemann-Pick disease and review 73 cases reported previously.
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PMID:Type C Niemann-Pick disease: report of a Chinese case. 790 66

We describe a distinctive epilepsy syndrome in six families, which is the first partial epilepsy syndrome to follow single gene inheritance. The predominant seizure pattern had frontal lobe seizure semiology with clusters of brief motor attacks occurring in sleep. Onset was usually in childhood, often persisting through adult life. Misdiagnosis as night terrors, nightmares, hysteria, or paroxysmal nocturnal dystonia was common, and the inheritance pattern was often not appreciated. This autosomal dominant epilepsy syndrome is ideal for identification of partial epilepsy genes.
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PMID:Autosomal dominant frontal epilepsy misdiagnosed as sleep disorder. 860 95

This review discusses several aspects of epilepsy and sleep. The level of wakefulness is controlled by transmitters such as acetylcholine, norepinephrine, serotonin, and histamine. These neurotransmitters are involved in modulatory neurotransmission of the ascending brain stem systems, which play an important role in controlling the sleep-wake cycle. Experimental evidence suggests that rapid eye movement sleep atonia is induced by increased endogenous acetylcholine release. Regarding sleep factors, recent data suggest that prolactin may stimulate rapid eye movement sleep and that growth hormone-releasing factor is involved in non-rapid eye movement sleep regulation. The neuropharmacologic features of several animal models of epilepsy have been investigated. Epileptiform discharges of genetic absence seizures in rats have been found to be suppressed by cholinergic drugs. The beta-carboline abecarnil has a strong antiepileptic effect on spike-wave discharges in rats that generate spontaneous spike-wave discharges, and it may be useful as an antiepileptic drug. Sleep epilepsy has been studied in a model of amygdala-kindled kittens. During the postkindling development, multifocal epilepsy with convulsions occurred that were distributed throughout the sleep-wake cycle; this finding agrees with the clinical literature. A typical feature of juvenile myoclonic epilepsy is the occurrence of seizures in a strict relationship to the sleep-wake cycle. There is now some neurophysiologic evidence that this syndrome also causes disturbance of sleep stability with increased arousal reactions. Nocturnal paroxysmal dystonia is not a homogeneous entity. Several clinical reports indicate that the short-lasting variant is most likely a form of frontal lobe epilepsy, but the nature of the longer-lasting variants is still obscure.
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PMID:Epilepsy and sleep. 791 13

Acute catatonic syndrome is a condition that can be caused by a variety of metabolic, neurological, psychiatric, and toxic conditions, including neuroleptic malignant syndrome. Although ictal catatonia as a manifestation of non-convulsive status epilepticus has been described, reference to the occurrence of seizures in patients with acute catatonic syndrome is anecdotal. Twenty nine patients with acute catatonic syndrome were reviewed to identify patients with seizures after the onset of acute catatonic syndrome. Patients were divided into four diagnostic groups: affective (15), schizophrenic (eight), toxic (two), and organic (four). Seizures occurred in four patients (13.8%): two patients with dystonic seizures had viral encephalitis and schizophrenic disorder respectively; one patient with complex partial seizures had viral encephalitis and one patient with absence status had neuroleptic malignant syndrome. The results indicate the value of EEG in detection of epileptic activity in patients with acute catatonic syndrome, both at onset and in the course of such disturbance, particularly to provide a differential diagnosis between pseudo-seizures and neuroleptic-induced acute dystonia.
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PMID:Epileptic seizures in patients with acute catatonic syndrome. 796 25

Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10% of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70% were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85%). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.
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PMID:[Neurologic complications by cocaine abuse]. 799 Jun 84

Dentatorubropallidoluysian atrophy (DRPLA) has been described chiefly in Japan and appears to be rare in Europe. It is of autosomal dominant inheritance. We report the first British family with DRPLA, which contains four affected individuals in two generations. The diagnosis was made at autopsy in one case. The age of onset of symptoms ranged from 15 to 38 years, and clinical features included ataxia, dementia, chorea, and dystonia; three patients had generalized seizures. The three living patients resemble those with early Huntington's disease clinically. Three main phenotypes of DRPLA have been proposed: an ataxo-choreoathetoid type, a pseudo-Huntington type, and a myoclonic epilepsy type. The variation in clinical presentation in our family demonstrates the difficulty in applying such classifications to this and other dominantly inherited disorders with phenotypic variation. DRPLA is likely to be confused with Huntington's disease in European families.
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PMID:Autosomal-dominant dentatorubropallidoluysian atrophy in the United Kingdom. 804 69

Different paroxysmal movements occur during sleep. They correspond either to epileptic seizures of sleep, or to parasomnia. Recently, other nocturnal motor phenomena have been described in the literature as nocturnal or hypnogenic paroxysmal dystonia (NPD), paroxysmal arousal, episodic nocturnal wanderings, etc. The NPD are involuntary nocturnal movements characterized by the association of dystonic postures, tonic movements of the four limbs and the body axis, automatisms, affective mimicry, and vocalization. In certain patients, the EEG records show abnormalities characteristic of epilepsy; in others, the EEG appears normal. A large proportion of the patients present epileptic seizures as antecedents. Typical generalized tonic-clonic seizures can follow an NPD. The NPD are improved with anti-epileptics. The considerable similarity of the clinical and paraclinical signs and of the effects of anti-epileptic treatments do not seem to justify the individualization of different subgroups as a function of the EEG patterns: the NPD are always the result of focal epilepsy, and never of a pathology of movement or of parasomnia. Numerous arguments based on the symptoms and the EEG suggest that these seizures involve the mesial frontal regions.
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PMID:Nocturnal paroxysmal dystonia: a clinical form of focal epilepsy. 809 Jan 54


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