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

Photosensitivity has proved to be a useful model to study the acute effects of experimental antiepileptic drugs (AEDs). The photosensitivity range is usually diminished or even abolished after administration of a known or experimental AED. An increase in photosensitivity, an unexpected reaction, was found in four photosensitive epileptic patients after oral ingestion of 500, 100, or 50 mg of Org 6370. Moreover, the three patients receiving doses of 100 and 500 mg reported nausea, dizziness, restlessness, and an increase in spontaneous epileptic seizures (myoclonus and in one patient a generalized tonic-clonic convulsion). The side effects coincided with peak Org 6370 serum levels. Our findings indicate that in the photosensitivity model experimental drugs with proven anticonvulsant properties in animals may increase rather than decrease the degree of patient photosensitivity. Photosensitive patients may represent a special subgroup of epileptic patients and therefore need to be classified as such.
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PMID:Preliminary assessment of the efficacy of Org 6370 in photosensitive epileptic patients: paradoxical enhancement of photosensitivity and provocation of myoclonic seizures. 173 47

A 32-year-old woman presented with increasing motor difficulties and memory disturbances. Neurological examination only showed mild cerebellar and extrapyramidal symptoms, whereas neuropsychological evaluation disclosed severe cognitive changes consistent with dementia. Her motor and mental status progressively deteriorated until death, which occurred 5 years after the first admission. One year before death, while she was almost bedridden, symptoms of myoclonic epilepsy first appeared, with frequent generalized seizures and generalized myoclonus, occurring especially upon sensory stimulation or passive joint movements. Pathological examination showed neuronal inclusions typical of Kufs' disease. This case, with primary progressive dementia and late-onset myoclonic epilepsy, differs from previously reported cases. Three special electrophysiological features were abnormal, "giant", evoked potentials; unusually marked photosensitivity; and seizure induction by any sensory stimulation.
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PMID:Kufs' disease presenting as progressive dementia with late-onset generalized seizures: a clinicopathological and electrophysiological study. 173 61

We reviewed 18 EEG studies in four members of a family with the Lafora form of progressive myoclonic epilepsy. Each patient was the product of a consanguinous marriage and presented as a teenager with progressive seizures, myoclonus, dementia, and ataxia, and had biopsy proven disease. The EEG early in Lafora disease has spike-wave activity resembling that seen in a primary generalized epilepsy; the background slowing is more typical of a secondary generalized epilepsy. With disease progression, there is increased epileptiform activity, and a striking change in the spike-wave complexes, with a marked increase in frequency up to 6-12 Hz, and many more short duration polyspike components. Unlike some other forms of secondarily generalized epilepsy, the EEG in Lafora disease is distinguished by an increased frequency of the spike-wave complexes with disease progression.
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PMID:Longitudinal EEG studies in a kindred with Lafora disease. 174 63

Four siblings aged 12-18 years with progressive myoclonus epilepsy demonstrated a subclinical stage at the age of 9-11 years, with visual blackouts and polyspike electroencephalographic (EEG) activity on photic stimulation, an early myoclonic stage at the age of 12-15 years, with increasing segmental, stimulus-sensitive myoclonus, occasional nocturnal buildup myoclonic "cascade" seizures, slowing of EEG alpha-activity, episodic 4-6 Hz bilateral sharp waves and polyspikes with myoclonias on photic stimulation, and a disabling myoclonic stage at the age of 16-18 years, with periodic generalized myoclonias, nocturnal myoclonic "cascade" seizures, ataxia, dysarthria, mental changes, intermittent wheelchair dependency, and continuous EEG slow waves with polyspikes and intense myoclonias on photic stimulation. One of the siblings died at the age of 18 years with no apparent cause of death. Treatment with antiepileptic drugs other than valproate may have contributed but none of the siblings were ever treated with phenytoin. Extensive clinical and laboratory investigations revealed no abnormalities and excluded other known possible causes of progressive myoclonus epilepsy. The diagnosis was consistent with Unverricht-Lundborg disease and rested on typical age of onset, clinical signs, EEG, and evoked response abnormalities. Buildup myoclonic seizures are typical in advanced stages of Unverricht-Lundborg disease. We have labeled these myoclonic "cascade" seizures. A typical seizure was studied with video-EEG and cardiorespiratory monitoring. Characteristics revealed were onset with continuous arrhythmic myoclonic jerks followed by intense rhythmic myoclonus with increasing muscle tone that successively reduced the amplitude of the jerks. The EEG during the whole seizure showed intense polyspike activity. Obstructive apnea was seen at the peak of the seizure. There were no cardiac dysrhythmias. Consciousness was normal or only slightly impaired. Postictal drowsiness was not observed. Myoclonic "cascade" seizures are easily confused with generalized tonic-clonic seizures.
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PMID:Clinical and neurophysiological development of Unverricht-Lundborg disease in four Swedish siblings. 174 64

A 22-year-old woman with progressive myoclonus epilepsy associated with the first and second branchial syndrome is described. Clinical features included generalized convulsive seizure, myoclonus, cerebellar ataxia and intellectual deterioration with micrognathia and malformation of auricles. She was initially suspected of mitochondrial encephalomyopathy, but the analysis of muscle biopsy and mitochondrial enzyme activities was negative. Her micrognathia and malformation of auricles were diagnosed as the first and second branchial syndrome. The case of progressive myoclonus epilepsy associated with this syndrome has never been reported, and the relationship between them remains unknown.
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PMID:[A case of progressive myoclonus epilepsy with the first and second branchial syndrome]. 176 51

Spinal cord seizures are infrequently reported. They have been associated with intravenous dye placement, transverse myelitis and multiple sclerosis, but never with traumatic spinal cord injury (SCI). We report the case of a 48-year-old SCI male with complete C6 quadriplegia, and apparent spinal cord seizures. These seizures were characterised by myoclonus simplex activity involving the upper extremities only. The lower extremities were spared. The patient was conscious throughout the myoclonic activity and an electroencephalogram of the brain obtained during an event revealed no cortical epiliptiform activity. The seizures lasted approximately 30 seconds to a few minutes, and an acute increase in blood pressure and a decrease in pulse generally occurred 30 to 60 seconds prior to the event. Previously reported spinal cord seizures in multiple sclerosis were frequently treated with carbamazepine. In this case successful treatment was with diazepam. Spinal cord seizures may present in those with traumatic SCI. Benzodiazepines may be useful in the treatment of spinal cord seizures.
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PMID:Spinal cord seizures: a possible cause of isolated myoclonic activity in traumatic spinal cord injury: case report. 177 64

The possible associations of myoclonic phenomena, progressive or non progressive encephalopathies and epileptic phenomena are reviewed with special emphasis on childhood. This leads to the following five groups of conditions: (1) myoclonus without encephalopathy and without epilepsy; (2) encephalopathies with non-epileptic myoclonus; (3) progressive encephalopathies with myoclonic seizures or epileptic syndromes (Progressive myoclonus epilepsies); (4) epileptic encephalopathies with myoclonic seizures; (5) myoclonic epilepsies. In the first group, which also includes physiological myoclonus, a more thorough description of "benign sleep myoclonus of newborn" and "benign myoclonus of early infancy" is given. Characteristic of group 2 are "Kinsbourne syndrome" and certain types of "Hyperekplexia" which pose interesting differential diagnosis problems with stimulus-sensitive epilepsies. In group 3, the concept of progressive encephalopathies is stressed, meaning that "Progressive Myoclonus Epilepsies" are always in fact progressive encephalopathies presenting with myoclonic types of seizures or epileptic syndromes among other neurologic and psychologic signs and symptoms. Major and rare causes are reviewed. The term major is applied to typical features or to frequency, whereas rare causes include not only those what are rarely seen, but also some myoclonic variants of diseases which usually have different symptoms. The fourth group refers to severe epilepsies, mainly in infancy and childhood, which lead to mental retardation irrespective of their cause. The assumption is that diffuse and persistent epileptic activity may interfere with normal development of the higher cerebral functions. "West syndrome" and "Lennox-Gastaut syndrome" are the more representative examples and may present with myoclonic type of seizures, but they are not dealt with in detail here. Group 5 comprises true myoclonic epilepsies, differentiating syndromes recognized as idiopathic, such as "benign myoclonic epilepsy of infancy" and "juvenile myoclonic epilepsy", from those which are cryptogenic and carry a more cautious prognosis--i.e.: "cryptogenic myoclonic and myoclono-astatic epilepsies" and "Severe myoclonic epilepsy of infancy". Finally other epileptic syndromes usually not considered as myoclonic epilepsies, but presenting sometimes myoclonic seizures, are mentioned.
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PMID:[Myoclonus and epilepsies in children]. 178 Jun 7

A 20 years old female patient with epileptic seizures (bilateral myoclonus and generalized tonic-clonic seizures (bilateral myoclonus and generalized tonic-clonic seizures) since the age of 13 was treated with valproic acid. Eight hours after 2 of 3 prophylactic antimalarial treatments with mefloquine generalized tonic-clonic seizures occurred. A causal relationship is suggested.
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PMID:[Suspected convulsive side-effect of mefloquine (Lariam)]. 179 56

A retrospective study of 36 confirmed cases of subacute sclerosing panencephalitis (SSPE) was carried out to detect any variation in the clinical and EEG patterns previously described. There were 31 males and 5 females, aged 4 to 24 years. Onset of SSPE at or after 15 years of age was observed in 22.2% of cases. Rapid progression of the disease was observed in 52%. The first symptom was myoclonus in 61.6%, mental regression in 22.2% and generalised seizures in 11.1% of cases. Low positive measles antibody titres in the CSF were found in 36.1% of cases. EEG analysis revealed slow background in 69.2% and periodic complexes in 94.4% of cases. Atypical periodic complexes, focal abnormalities and paroxysms of bisynchronus sharp activity were also observed.
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PMID:Subacute sclerosing panencephalitis. 181 21

The prevalence, onset, and type of seizure disorders, as well as seizure control, were studied in a large cohort of 405 individuals with Down syndrome (age range, 6 months to 45 years). The evaluation of a questionnaire completed by the subjects' parents and of the patients' medical records indicated that 33 (8.1%) of 405 persons with Down syndrome had seizure disorder. With regard to the onset of seizures, a bimodal distribution was noted: 40% of patients began having seizures before the age of 1 year, and another 40% started with seizure activity in the third decade of life. In the younger age group, primarily infantile spasms and tonic-clonic seizures with myoclonus were observed, and the older patients often had partial simplex or partial complex seizures as well as tonic-clonic seizures.
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PMID:Seizure disorders in Down syndrome. 182 77


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