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Spinocerebellar Ataxia

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This is an elderly patient who

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is being evaluated for ataxia.

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On the sagittal T2-weighted scan, you see

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what is likely to represent DISH, that is

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Diffuse Idiopathic Skeletal Hyperostosis,

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with large flowing osteophytes anteriorly

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with relative sparing of the disc spaces.

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However, the finding on the spinal cord

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evaluation is a relative decrease in

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the overall caliber of the spinal cord.

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If we look at the spinal cord on axial scans,

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again, we're a little bit impressed with relative

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decrease in the caliber of the spinal cord

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in its AP width.

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Normally, we say that the spinal cord

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should represent greater than 50%

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of the overall thecal sac diameter,

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and this one is borderline low.

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If you look at the, um, cerebellum on this

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patient, you notice that there appears

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to be somewhat prominent folia of the

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cerebellum, as well as the diminution in

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the caliber of the spinal cord overall.

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Fortunately, the patient had a

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CT scan that accompanied the MRI

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because of this problem with ataxia.

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On the CT scan, we're impressed with the degree

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of sulcal enlargement around the superior vermis

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and folia of the cerebellar hemisphere,

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and we contrast that with what looks like a more

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normal appearance to the supratentorial cerebral

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structures, in that there doesn't appear to be

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that striking amount of atrophy, and certainly,

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the ventricles are not particularly enlarged.

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This patient has spinocerebellar ataxia.

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There are multiple different varieties of spinocerebellar

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ataxia, including olivopontocerebellar

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degeneration, where one has a small pons,

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as you see here, and middle cerebellar peduncles.

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However, there are other varieties that

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do not affect the pons but are purely in

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the cerebellum and in the spinal cord.

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In fact, there are over 15 different varieties

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and they're labeled SCA2, SCA8, SCA15, and many

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of these are congenital in their transmission.

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This person had SCA8,

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spinocerebellar ataxia, showing the

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manifestations of pontine, cerebellar, and spinal

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cord volume loss and the distinction among the

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various SCAs cannot be generally made by imaging.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Neuroradiology

Musculoskeletal (MSK)

Metabolic

MRI

CT

Acquired/Developmental

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