NeuroTeach 17 - Polyminimyoclonus

 Polyminimyoclonus!!!

“intermittent, low-amplitude, arrhythmic movements of the hands, with amplitudes just sufficient to produce visible movements of the joints.”

American child neurologist Alfred J. Spiro (1930–2019)

2) Features

- Mostly noticed when maintaining a posture (outstretched hands) or during action (initial phase of the movement)

3) Pathophysiology

Motor cortex - execution of fractionated muscle activation Anterior horn cells - generators of complex fasciculation Possible role of brainstem, cerebellum, and basal ganglia

4) How to clinically differentiate peripheral from central origin?

EEG & EMG Peripheral: denervation Central: parkinsonism or epilepsy

5) Polyminimyoclonus: Hirayama disease

6) Polyminimyoclonus: Hirayama disease

7) Polyminimyoclonus: Spinal muscular atrophy type-3 (Kugelberg–Welander disease)

8) Polyminimyoclonus: Spinal muscular atrophy

9) Polyminimyoclonus: Chronic Aluminium Toxicity

10) Polyminimyoclonus: Hirayama disease

11) Polyminimyoclonus: Progressive myoclonic ataxia

12) Polyminimyoclonus

13) Polyminimyoclonus

14) Polyminimyoclonus: Myoclonus Renal Failure Syndrome

15) Polyminimyoclonus: Congenital myoclonus syndrome

16) Polyminimyoclonus: Renal failure syndrome

17) Polyminimyoclonus: Hallermann-Streiff syndrome

18) Polyminimyoclonus

19) Polyminimyoclonus: Hirayama disease

20) Polyminimyoclonus

21) Polyminimyoclonus

22) Polyminimyoclonus: Morvan syndrome

23) Polyminimyoclonus

24) Polyminimyoclonus: Myoclonus-dystonia syndrome