juvenile myoclonic epilepsy


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ju·ve·nile my·o·clon·ic ep·i·lep·sy

[MIM*606904]
an epilepsy syndrome typically beginning in early adolescence, and characterized by early morning myoclonic jerks that may progress into a generalized tonic-clonic seizure. A genetic disorder: some families have had gene linkage to chromosome-6. The EEG is characterized by generalized polyspike and wave discharges at 4-6 Hz.

juvenile myoclonic epilepsy

Neurology A seizure disorder that comprises ± 4% of seizure disorders Clinical Normal IQ, onset in adolescence, affecting the flexor muscles of the head, neck and shoulders; the attacks tend to occur as clonic-tonic-clonic seizures upon awakening EEG 4-6 Hz multispike and wave pattern; 40% of relatives, especially ♀, have myoclonus Management Valproic acid. See Epilepsy.

ju·ve·nile my·o·clon·ic ep·i·lep·sy

(jū'vĕ-nil mī'ō-klon'ik ep'i-lep'sē)
An inherited epilepsy syndrome typically beginning in early adolescence, and characterized by early morning myoclonic jerks that may progress into a generalized tonic-clonic seizure.

juvenile myoclonic epilepsy

A form of epilepsy typically noticed in teenagers, characterized by early morning jerking movements of the extremities, and during stress or sleep deprivation, generalized tonic/clonic or absence seizures.
Synonym: Janz syndrome.
See also: epilepsy
References in periodicals archive ?
Clinicaland EEG Findings In Juvenile Myoclonic Epilepsy.
Linkage analysis of juvenile myoclonic epilepsy and microsatellite loci spanning 61 cM of human chromosome 6p in 19 nuclear pedigrees provides no evidence for a susceptibility locus in this region.
The majority of participants had juvenile myoclonic epilepsy (JME), although the study also included patients with juvenile absence epilepsy or tonic-clonic seizures upon awakening.
While no relation was found between seizure type and depression in some studies (5, 6), in some other studies, it has been reported that depressive symptoms are observed frequently especially in temporal lobe epilepsy which is accompanied with complex partial seizures and even in juvenile myoclonic epilepsy with genetic origin (7, 8, 9, 10).
Epileptic myoclonus including juvenile myoclonic epilepsy, progressive myoclonic epilepsies (PME), epilepsia partialis continua, Rasmussen's encephalitis, early infantile myoclonic encephalopathy, infantile spasms (also known as West syndrome), Lennox-Gastaut syndrome, benign familial myoclonic epilepsy, and Angelman syndrome; and
Juvenile myoclonic epilepsy was the most common diagnosis in our patients (n=17) and 5 of the main group had at least one episode of absence status epilepticus.
Juvenile myoclonic epilepsy is characterized by brief jerks of the extremities (termed myoclonus), generalized tonic clonic seizures (often upon awakening in the morning), and, occasionally, absence seizures.
MR spectroscopy shows reduced frontal lobe concentrations of N-acetyl aspartate in patients with juvenile myoclonic epilepsy Epilepsia 2000; 41: 290-6.
Juvenile myoclonic epilepsy also responds well to treatment, but the seizures usually return when medication is discontinued.

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