Intentional motor phantom limb syndrome.
OBJECTIVE
To investigate the clinical and anatomic correlates of a previously unreported form of chronic supernumerary phantom limb, which developed only in association with motor intent directed at a hemiplegic-anesthetic upper limb.


METHODS
We explored the phenomenology of the phantom illusion in the light of motor control models. Hemodynamic correlates of supernumerary phantom limb were studied with an fMRI sensorimotor paradigm consisting of finger-thumb opposition movements.


RESULTS
The kinesthetic-proprioceptive illusion of a third arm was triggered by any attempt to move the paretic limb, by bimanual actions, and by motor imagery involving the nonfunctional limb. The responsible lesion destroyed the posterior part of the posterior limb of the internal capsule on the opposite side, damaging corticospinal and thalamocortical tracts. Comparison between fMRI signals performed during virtual movement of the phantom hand vs imaginary movement of the paretic hand showed increased activation in thalamus and caudate nucleus in the first condition.


CONCLUSIONS
A preserved sense of agency provided by intact premotor processes translating intention into action may lead to the vivid feeling of movement in a paralyzed limb, similar to kinesthetic illusions in amputees. The interruption of thalamic afferences may explain the persistence and stability of the phantom by preventing any correction of the mismatch between expected and effective movement. The increased blood oxygen level-dependent (BOLD) signal in the basal ganglia-thalamus-cortex pathway during movement of the supernumerary hand may reflect an abnormal closed-loop functioning of the thalamocortical system underlying the phantom phenomenon.
Staub
F
F
Neurology Department, Centre Hospitalier Universitaire Vaudois, Rue du Bugnon 46, 1011 Lausanne, Switzerland. Fabienne.Staub@chuv.ch
Bogousslavsky
J
J
Maeder
P
P
Maeder-Ingvar
M
M
Fornari
E
E
Ghika
J
J
Vingerhoets
F
F
Assal
G
G
D000368:Aged; D001132:Arm / Q000503:physiopathology*; D001921:Brain / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007088:Illusions; D009069:Movement Disorders / Q000175:diagnosis* / Q000503:physiopathology*; D010591:Phantom Limb / Q000175:diagnosis* / Q000503:physiopathology*; D013577:Syndrome