Multiple system atrophy (MSA-C)

Case contributed by Keshaw Kumar
Diagnosis almost certain

Presentation

Difficulty in walking (ataxia)

Patient Data

Age: 40 years
Gender: Male

Brain

mri

There is moderate diffuse cerebellar atrophy with prominent cerebellar folia and associated with mild thinning of pons, medulla and middle cerebellar peduncle.

T2W hyperintensity in the pons, which appears as a cross on axial images, suggestive of hot cross bun sign.

Rest of the brain is unremarkable.

Hot cross bun sign

mri

The hot bun cross sign refers to T2W hyperintensity in the pons, which appears as a cross on axial images. It is due to selective degeneration of transverse pontocerebellar tracts and median pontine raphe nuclei. Most of the time it is seen in MSA-C but may also be seen in other neurodegenerative disease.

Case Discussion

Multiple system atrophy (MSA) is a sporadic neurodegenerative disease (synucleinopathy). In MSA-C, there is a predominant cerebellar symptom. Its diagnosis is usually made by history, clinical examination, and typical imaging appearance. Unfortunately, no definite treatment is available currently.

Co-author: Dr. Souvik Dubey sir, DM.

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