Two women (patient 1, 77 years old, and patient 2, 63 years old) with stron
g clinical evidence for corticobasal degeneration (CBD) are presented. Pati
ent 2 was in an early stage of the disease with only a mild disability of h
er left hand. In addition to the clinical characteristics, both patients pr
esented the typical cortical reflex myoclonus. Magnetic resonance imaging s
tudies for both patients revealed nearly identical hyperintense lesions som
atotopic from the left-hand primary motor cortex (M1), extending to the mid
line and possibly supplementary motor area (SMA) in patient 2. To our knowl
edge, this has not been previously described in patients with CBD. These le
sions may play a role in the etiology and the development of CBD with invol
vement of the M1 and may correspond to the underlying pathology of demyelin
ation or gliosis.