The first known description of paroxysmal kinesigenic dyskinesias (PKD) was by Shuzo Kure (1892) detailing a Japanese man with PKD.
Initial reports often regarded PKD as a form of reflex epilepsy.
Currently it considered as one of the three categories of paroxysmal dyskinesias, the other two being paroxysmal non-kinesigenic dyskinesia (PNKD), and paroxysmal exercise-induced dyskinesia (PED) (Jankovic J & Demirkiran M, 2002).
Clinical Features
Lishman et al. (1962) provided a thorough and beautifully written account of PKD's clinical characteristics while describing seven patients.
Attacks usually begin in childhood and occur frequently.
Precipitated by sudden movement, often after a period of rest.
Movement of the legs is more likely to initiate attacks than the arms.
An element of surprise or "startle" is important; tension, anxiety, or self-consciousness increases the likelihood of attacks.
Subjective preparation for movement and slow initiation of action tend to reduce or abort attacks.
Attacks consist of tonic spasm, potentially involving the whole body but more commonly localized to the initiating limb or confined to one side.
In three cases, the movements had a writhing character reminiscent of torsion spasm, with clonic movements being absent.
A sensory aura, when present, began in the initiating limb and spread briefly before the spasm.
The pattern of attack was generally stereotyped, though attacks might be arrested before fully developing.
Consciousness was never lost, although transient clouding was sometimes evident.
Attacks could sometimes be voluntarily induced if the provoking movement was abrupt and forceful.
Kertesz (1967) introduced the term "paroxysmal kinesigenic choreoathetosis," emphasizing:
Provocation by movement.
Short duration of episodes.
Good response to phenytoin.
Familial occurrence, initially speculated to be recessive but later understood as autosomal dominant with reduced expressivity.
