Biomaterial Database

Paroxysmal kinesigenic choreoathetosis. 1987

S Harel, and U Yurgenson, and M Kutai

A healthy intelligent 13.5-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. He had had unilateral tonic spasms for 2 months. In a stressful situation, especially after a period of immobility, a sudden voluntary movement provoked a dystonic-choreoathetoic posturing of the left arm and leg. Attacks were brought on, for instance, by standing up quickly from a sitting position. They lasted only 5-10 s, without loss of consciousness and occurred up to ten times daily. Between the attacks there were no abnormalities. Family history was non-contributory. Neurological examination and laboratory findings, including repeated EEGs, were all negative. The child responded very well to phenytoin treatment and has now been completely free of dystonic attacks for a period of 9 months. The importance of early recognition of this syndrome and the differential diagnosis with reflex epilepsy and other forms of dystonic choreoathetosis is discussed.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D010672	Phenytoin	An anticonvulsant that is used to treat a wide variety of seizures. It is also an anti-arrhythmic and a muscle relaxant. The mechanism of therapeutic action is not clear, although several cellular actions have been described including effects on ion channels, active transport, and general membrane stabilization. The mechanism of its muscle relaxant effect appears to involve a reduction in the sensitivity of muscle spindles to stretch. Phenytoin has been proposed for several other therapeutic uses, but its use has been limited by its many adverse effects and interactions with other drugs.	Diphenylhydantoin,Fenitoin,Phenhydan,5,5-Diphenylhydantoin,5,5-diphenylimidazolidine-2,4-dione,Antisacer,Difenin,Dihydan,Dilantin,Epamin,Epanutin,Hydantol,Phenytoin Sodium,Sodium Diphenylhydantoinate,Diphenylhydantoinate, Sodium
D002819	Chorea	Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as CHOREATIC DISORDERS. Chorea is also a frequent manifestation of BASAL GANGLIA DISEASES.	Choreatic Disorders,Choreiform Movement,Chorea Disorders,Chorea Syndromes,Chorea, Benign Hereditary,Chorea, Chronic Progressive,Chorea, Rheumatic,Chorea, Senile,Chorea, Sydenham,Choreatic Syndromes,Choreic Movement,Dyskinesias, Paroxysmal,Hereditary Chorea,Hereditary Progressive Chorea Without Dementia,Paroxysmal Dyskinesias,Rheumatic Chorea,Senile Chorea,St. Vitus's Dance,Sydenham Chorea,Sydenham's Chorea,Benign Hereditary Chorea,Benign Hereditary Choreas,Chorea Disorder,Chorea Syndrome,Chorea, Hereditary,Chorea, Sydenham's,Choreas,Choreas, Benign Hereditary,Choreas, Chronic Progressive,Choreas, Hereditary,Choreas, Rheumatic,Choreas, Senile,Choreas, Sydenham,Choreatic Disorder,Choreatic Syndrome,Choreic Movements,Choreiform Movements,Chronic Progressive Chorea,Chronic Progressive Choreas,Disorder, Chorea,Disorder, Choreatic,Disorders, Chorea,Disorders, Choreatic,Dyskinesia, Paroxysmal,Hereditary Chorea, Benign,Hereditary Choreas,Hereditary Choreas, Benign,Movement, Choreic,Movement, Choreiform,Movements, Choreic,Movements, Choreiform,Paroxysmal Dyskinesia,Progressive Chorea, Chronic,Progressive Choreas, Chronic,Rheumatic Choreas,Senile Choreas,St. Vitus Dance,St. Vitus's Dances,St. Vituss Dance,Sydenham Choreas,Sydenhams Chorea,Syndrome, Chorea,Syndrome, Choreatic,Syndromes, Chorea,Syndromes, Choreatic
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001264	Athetosis	A dyskinesia characterized by an inability to maintain the fingers, toes, tongue, or other body parts in a stable position, resulting in continuous slow, sinusoidal, and flowing involuntary movements. This condition is frequently accompanied by CHOREA, where it is referred to as choreoathetosis. Athetosis may occur as a manifestation of BASAL GANGLIA DISEASES or DRUG TOXICITY. (From Adams et al., Principles of Neurology, 6th ed, p76)	Athetoid Movements,Hammond Disease,Hammond's Disease,Athetoid Movement,Athetoses,Disease, Hammond,Disease, Hammond's,Diseases, Hammond,Diseases, Hammond's,Hammond Diseases,Hammond's Diseases,Hammonds Disease,Movement, Athetoid,Movements, Athetoid