It is important to note that the current belief that olivopontocerebellar degeneration, Shy-Drager syndrome and striatonigral degeneration are different manifestations of the same underlying disease, namely multiple systemic atrophy (MSA), is recent, and as such many older publications will describe these as separate entities 2,3.
For a discussion of epidemiology and pathology, please refer to: multiple systemic atrophy (MSA).
Olivopontocerebellar degeneration presents predominantly with cerebellar and brainstem symptoms and signs. Typically ataxia and bulbar dysfunction are pronounced. Given the predominance of cerebellar symptoms, it is classified under the MSA-C subtype of MSA.
There is disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle).
- 1. Grossman RI, Yousem DM. Neuroradiology, the requisites. Mosby Inc. (2003) ISBN:032300508X. Read it at Google Books - Find it at Amazon
- 2. Matsusue E, Fujii S, Kanasaki Y et-al. Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 2009;30 (9): 1725-30. doi:10.3174/ajnr.A1662 - Pubmed citation
- 3. Kornienko VN, Pronin IN. Diagnostic Neuroradiology. Springer Verlag. (2008) ISBN:3540756523. Read it at Google Books - Find it at Amazon
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Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.
neurodegenerative MRI brain (an approach)
- measurements and ratios
- midbrain to pons area ratio (for PSP)
- Magnetic Resonance Parkinsonism Index (MRPI) (for PSP)
- frontal horn width to intercaudate distance ratio (FH/CC) (for Huntington disease)
- intercaudate distance to inner table width ratio (CC/IT) (for Huntington disease)
- scoring systems
- measurements and ratios
- typical/classical Alzheimer disease
- variant (e.g. posterior cortical atrophy)
- chronic traumatic encephalopathy (CTE)
- corticobasal degeneration
- frontotemporal lobar degeneration (FTLD) (not all are tau)
- Pick disease
- progressive supranuclear palsy (PSP)
- Alzheimer disease
- cerebral amyloid angiopathy (CAA)
- transthyretine-associated cerebral amyloidosis
- neuronal intranuclear hyaline inclusion disease (NIHID)
- spinocerebellar ataxias
- Huntington disease
- hereditary spastic paraplegia
- amyotrophic lateral sclerosis (ALS)
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
- prion diseases (not always included as neurodegenerative)