Subacute necrotizing encephalomyelopathy (Leigh disease): CT study.

Abstract:

:Leigh disease, or subacute necrotizing encephalomyelopathy (SNE), is a familial, degenerative disorder characterized by lesions of the gray and white matter in the brain and spinal cord. Low attenuation in the putamina on computed tomography (CT) scans is considered to be characteristic of the disease. The authors used CT to study five patients, in three of whom the disease was confirmed histologically. In one of the patients with documented SNE, there was extensive gray matter lucency with normal basal ganglia. CT scans obtained in a second patient showed diffuse, diseased white matter with focal cortical extension and bilateral caudate involvement. In the other three cases, CT scans revealed the usual changes that occur in the basal ganglia. The appearance of SNE on CT scans thus reflects the variable and widespread distribution of the disease. The absence of radiologically detectable abnormalities in the basal ganglia should not deter one from the diagnosis of Leigh disease given in the appropriate clinical context.

journal_name

Radiology

journal_title

Radiology

authors

Paltiel HJ,O'Gorman AM,Meagher-Villemure K,Rosenblatt B,Silver K,Watters GV

doi

10.1148/radiology.162.1.3786750

subject

Has Abstract

pub_date

1987-01-01 00:00:00

pages

115-8

issue

1 Pt 1

eissn

0033-8419

issn

1527-1315

journal_volume

162

pub_type

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