RESEARCH: A novel grading scale for striatonigral degeneration (multiple system atrophy).

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J Neural Transm 2002 Mar;109(3):307-20

A novel grading scale for striatonigral degeneration (multiple system

atrophy).

Wenning GK, Seppi K, Tison F, Jellinger K.

Department of Neurology, University of Innsbruck School of Medicine,

Innsbruck,

Austria.

SUMMARY: Striatonigral degeneration (SND) is commonly thought to represent

the

neuropathological substrate of L-Dopa unresponsive parkinsonism in patients

with

multiple system atrophy (MSA). Other neuropathological hallmarks of MSA

include

olivopontocerebellar atrophy (OPCA) and preganglionic sympathetic spinal

cord

lesions. Clinicopathological evaluation of MSA patients recruited into

ongoing

natural history studies or neuroprotective intervention trials will require

standardized grading of MSA pathology. Based on 25 autopsy cases of MSA, we

propose a novel SND grading scale which allows semiquantitative assessment

of

lesion severity based on neuronal loss, astrogliosis and presence of

alpha-synuclein positive glial cytoplasmic inclusions (GCIs) in substantia

nigra, putamen, caudate nucleus, and globus pallidus. SND grade I is defined

as

degeneration of the substantia nigra pars compacta (SNC) with relative

preservation of the striatum except for minimal gliosis and GCIs in the

posterior putamen ( " minimal change MSA " ). SND grade II is characterized by

neuronal loss, astrogliosis and presence of GCIs in SNC and

posterior/dorsolateral putamen. Caudate nucleus and external globus pallidus

may

exhibit slight gliosis. Striatal pathology is severe and extends to anterior

ventromedial subregions in SND grade III. There is neuronal loss in caudate

nucleus and globus pallidus. GCIs are more abundant in grade II than grade

III

SNC and putamen. Preliminary clinicopathologic correlation studies suggest

milder parkinsonian disability and better initial L-Dopa responsiveness in

SND

grade I and II cases compared to grade III cases. Prospective

clinicopathologic

studies are required to validate the proposed SND grading scale and may

result

in further subdivisions, particularly of SND grade III.

PMID: 11956953 [PubMed - in process]