The MRI findings in Alexander disease

JVIM 37(2): 670-675

May 22, 2023

Diagnostic features of type II fibrinoid leukodystrophy (Alexander disease) in a juvenile Beagle dog

Case Description: A 3-month-old female Beagle presented with progressive caudotentorial encephalopathy. MRI showed diffuse, bilateral lesions involving the white matter of the cerebellum, brainstem, spinal cord, and forebrain. Histopathological analysis of the brain was consistent with fibrinoid leukodystrophy, also known as Alexander disease.

Alexander Disease: Alexander disease (AD) is an uncommon central nervous system disorder that predominantly affects the white matter tracts. It is caused by a de novo mutation in the GFAP gene coding for glial fibrillary acidic protein. Diagnosis is based on clinical signs, typical MRI findings, and genetic testing.

AD in Dogs: Only a few cases of histopathologically confirmed AD are reported in dogs. This case provides a comprehensive MRI description of AD in a dog using a high field magnet and could be classified as a type II AD. MRI patterns are of high diagnostic value in people with leukoencephalopathies.

MRI findings: MRI of the brain using a 1.5 Tesla scanner showed diffuse, bilateral, T2-weighted and T2-weighted-FLAIR hyperintense, T1-weighted hypointense, noncontrast-enhancing lesions involving the white matter of the cerebellum, brainstem, spinal cord, and forebrain to a lesser extent. There was cerebellar enlargement.

(A) T2W parasagittal image. (B) T2W transverse image at the level of the middle cerebellar peduncles. (C) T2 FLAIR transverse image at the level of the middle cerebellar peduncles. (D) T1W transverse image at the level of the middle cerebellar peduncles. T2W and T2W FLAIR hyperintense signal involving the middle cerebellar peduncles (indicated by arrows) and increased size of the cerebellum.

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