Historical Background
- 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).
- 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.