Multiple System Atrophy

The Parkinson's-Reversing Breakthrough

Parkinson Disease Information

Get Instant Access

This diagnosis is particularly difficult to study historically, because the variety of symptoms and different phenotypes have been labeled with a wide vocabulary and nosology. In 1865, Sanders introduced the term "dystaxia or pseudo-paralysis agitans" to describe a patient with severe action tremor and gait impairment without sensory loss (29). Dejerine and Thomas described olivopontocerebellar atrophy in 1900 and emphasized varying mixtures of parkinsonian, cerebellar, and autonomic dysfunction (30). Critchley and Greenfield reviewed cases from the early 20th century, citing such names as Pierre Marie, Murri, Lhermitte, and Wilson, commenting on the changing terminology and noso-graphic confusion related to this disorder (31). In 1925, Ley reported the same type of presentation as Dejerine and Thomas in a 50-yr-old man, and at autopsy, he noted not only olivopontocerebellar lesions, but also atrophic substantia nigra (32,33).

Fig. 6. Photograph from 1925 article by Lhermitte (25) and studied by Ballan and Tisson (26), showing a patient with possible corticobasal degeneration. The picture captures his unusual posturing of the right arm and hand with marked dyspraxia.

In 1960, Shy and Drager described two patients with orthostatic hypotension and parkinsonism or pyramidal/cerebellar features, who showed highly distinctive autopsy features including vacuolation of the autonomic ganglia, shrunken, hyperchromatic cells in the intermediolateral column, and degenerative changes in the pons, substantia nigra, hypothalamus, locus ceruleus, and Purkinje layer of the cerebellum (34). Striatonigral degeneration became widely known after Adams, van Bogaert, and van der Eecken published their series in 1961 (35). The conditions were consolidated in the seminal article by Graham and Oppenheimer (36).

Early reports of multiple system atrophy of the striatonigral phenotype were written by Lewy's student, Fleischhacker, in 1924 and Scherer in 1933, both analyzed by Wenning and colleagues (3739). Of these five subjects, three had severe parkinsonism and two had more prominent cerebellar features. All had neuropathological evidence of putaminal and pallidal degeneration as well as depig-mentation and degeneration of the small cells of the substantia nigra. Those with cerebellar features had additional olivopontocerebellar lesions. Fleischhacker's case had been clinically diagnosed during life as paralysis agitans, but the author argued that both clinically and pathologically, the findings were atypical for Parkinson's disease. Clinically, he emphasized the atypical tremor (coarse and postural rather than resting), an early age of disease onset, and prominent rigidity. Berciano considers that Scherer's contribution was essential in establishing the striatal-nigral lesions that underlie parkinsonism in this phenotype. With his report, he definitively established that parkinsonism in olivopontocerebellar atrophies related to additional degenerative changes outside this system (33).

Was this article helpful?

0 0

Post a comment