Video 1
Tremor in Roussy-Lévy syndrome. Tremor is shown at rest in the right hand; however, it is more proximal than typically seen in Parkinson’s disease. With arms outstretched, there is a mild to moderate postural tremor on the right. Unlike essential tremor, it has a jerky quality and the predominant movement is wrist pronation-supination rather than flexion-extension. In the wingbeat position, a jerky tremor of mild to moderate amplitude is seen on the right. Mild kinetic tremor (right greater than left) is seen on the finger-nose maneuver; the tremor is jerky and there is no intentional component. During spiral drawing, moderate amplitude tremor is seen on the right and mild tremor on the left. During dot approximation, mild tremor is seen on the right and trace on the left. Gait is wide-based, and the patient was unable to tandem walk without needing to touch the wall with his left hand.
Figure 1
A: Nerve conduction study consistent with primarily demyelinating neuropathy. Nerve conduction velocity in peroneal, median and ulnar nerves < 38 m/sec; characteristic of CMT1A neuropathy.