NeuroTeach 18 - Wing-beating tremor

 Wing-beating tremor!!!

“pseudosclerotic, parkinsonism, and dystonic/choreic forms of Wilson’s disease”

American-born British neurologist Samuel Alexander Kinnier Wilson (1878–1937)

2) Pathophysiology

“lesion in the dentatorubrothalamic pathway” - cerebellar outflow pathway Complex phenomenology - dystonic, myoclonic, rhythmic tremor?

3) Definition

“low-frequency, high-amplitude, posture-induced proximal arm tremor, elicited by sustained abduction of the arms, with flexed elbows and palms facing downward” - postural tremor - wing-beating posture

4) Wing-beating tremor – features

- low frequency & high amplitude > inc amplitude w/ inc duration of posture holding - posture-induced - proximal arm tremor

5) Wing-beating tremor: Wilson's disease

6) Wing-beating tremor: Wilson's disease

💡double panda sign

7) Wing-beating tremor: Creutzfeldt-Jakob Disease

💡myoclonus?

8) Wing-beating tremor: Wilson's disease

💡zolpidem-responsive

9) Wing-beating tremor: Wilson's disease

💡 after liver transplantation

10) Wing-beating tremor: Wilson's disease

11) Wing-beating tremor: Wilson's disease

💡dimercaprol-responsive

12) Wing-beating tremor: Wilson's disease

💡Face of a Giant Panda

13) Wing-beating tremor: Wilson's disease

14) Wing-beating tremor: Wilson's disease

15) Wing-beating tremor: Wilson's disease

16) Wing-beating tremor: Wilson's disease

17) Wing-beating tremor: Wilson's disease

18) Wing-beating tremor: Wilson's disease

19) Wing-beating tremor: Wilson's disease

20) Wing-beating tremor: Wilson's disease

21) “We don't always observe true 'wing beating tremor' like Kinnier Wilson described, however when we see it; it can be dramatic”