Multiple system atrophy (MSA) is defined as an adult onset, sporadic, rapidly progressive,
multisystem, neurodegenerative disease of undetermined etiology, characterized by varying
combinations of parkinsonian, autonomic, cerebellar and pyramidal system signs and symptoms.
A 54 years old male presented with insidious and gradually progressive pancerebellar dysfunction
(gait, appendicular ataxia & dysarthria) with autonomic dysfunction and without cognitive, cranial
nerve, pyramidal & extra pyramidal and sensory impairment. MR imaging of Brain revealed diffuse
cerebellar atrophy along with atrophy of inferior olivary nucleus and pons depicting typical "hot
cross bun sign" which was made out in the region of pons. The idea behind presenting this case as
a case report is, though MSA is a rare neurodegenerative disease, the diagnosis should always be
considered in patients presenting with features of parkinsonism or cerebellar involvement with
autonomic dysfunction in order to prevent the misdiagnosis of this fatal disease.
Key words: MSA-C [multiple system atrophy-cerebellar], autonomic dysfunction, cerebellum, magnetic
resonance imaging of brain(MRI).
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