Paroxysmal Movement Disorders!
Dyskinesias!!!
- 4 groups, overlap
- prevalence: 1/150 000
- myoclonus, dystonia, chorea, athetosis, tics
2) First report of paroxysmal dyskinesia
- 1892 by Shuzo Kure, Japanese psychiatrist (1865-1932)
- referred to as atypical Thomsen’s disease (Myotonia congenital)
-precipitated by sudden voluntary movement
-most common paroxysmal movement disorders
-multiple attacks, frequency up to 100/day
-attacks are brief, seconds
-antiepileptic drug responsiveness
- precipitated by alcohol, fatigue, caffeine, strong emotion
- onset childhood
- multiple attacks, frequency 2-3/day
- duration: minutes to hours
- avoid triggers, benzo responsive
- NO antiepileptics responsiveness
6) Exercise-induced
- precipitated by a prolonged or sustained exercise
- onset childhood
- attacks: 2-5 min (up to 2 hours), stop within 10 min after stopping exercise
- most common presentation dystonia
- relationship: Young-onset PD, GLUT1 def, DYT 9& 18
- restrict exercise
7) Exercise-induced
8) Exercise-induced
- is this a real paroxysmal movement disorder?
- attacks occur during Non-REM sleep
- many attacks < 1 min, can be indistinguishable from frontal lobe epilepsy
- dystonic posturing, ballistic or choreic movements, without ictal EEG abnormalities
- ADCY-5 mutation!
10) Summary
- Kinesigenic: ⬆️⬆️⬆️attacks/day; seconds; response AED- NON-Kinesigenic: ⬆️attacks/day; minutes; non-response AED
- Exercise-induced: exercise
