We describe a patient who has been complaining of brief jerk-like, rhythmic
, involuntary movements involving the second digit of the left hand for the
last three months. These involuntary jerks produced an adduction movement
of the second digit and were unaffected by peripheral sensory stimuli. In a
ddition, the patient experienced loss of dexterity in the left hand. On exa
mination the patient showed hypotrophy of the first dorsal interosseous (FD
I) muscle of the left hand and a dissociated sensory loss involving the C8-
T1 dermatomes. Magnetic resonance imaging of the brain and spinal cord reve
aled a tonsilar herniation characteristic of the Chiari I malformation asso
ciated with a syrinx extending from C4 to D5 that did not communicate with
the fourth ventricle. The electrophysiological evaluation indicated the pre
sence of a focal myoclonus of spinal origin that is likely to be caused by
the syrinx.