45 - Corticobasal degeneration

Summary

Historical review

Dutil (1899) from the Salpêtrière may have been describing a case of corticobasal degeneration (CBD) when reporting a patient said to have the so- called “hemiplegic” form of “parkinsonism in extension,” the latter being the description used by Charcot and his pupils to describe cases of parkinsonism who were atypical because of an extended posture rather than the characteristic flexed posture of paralysis agitans which they recognized. The picture of this patient shows a French woman with a hypomimic face and a rather erect posture with the right arm rigidly flexed at the elbow and perhaps is the first picture of a CBD patient (Goetz, 2000). However, the first clear description was given by Rebeiz, Kolodny, and Richardson (1968), who described three cases with a distinct clinical syndrome characterized by an asymmetric onset of slow, clumsy limb movement, accompanied by rigidity, tremor and dystonia, progressing to impairment of gait, dysarthria and dysphagia, disordered eye movement, and increasing disability. Intellectual function was said to be relatively intact. The illness led to death in 6 to 8 years. The pathological findings were distinctive. There was asymmetric frontal and parietal cortical atrophy, with extensive neuronal loss and gliosis. Some of the cortical pyramidal neurons, mainly in the third and fifth layers, had an unusual swollen hyaline appearance; the cytoplasm did not take up usual stains, except for faint eosinophilia, so such neurons were termed achromatic. No classical Pick bodies, neurofibrillary tangles, senile plaques, or Lewy bodies were found.