Diagnostic Clues in Multiple System Atrophy: A Case Report and Literature Review

Abstract

Multiple system atrophy (MSA) is an adult-onset, sporadic, progressive neurodegenerative disease. According to the consensus criteria, patients with MSA are clinically classified into cerebellar (MSA-C) and parkinsonian (MSA-P) subtype. Besides the poor response to levodopa, and the additional presence of pyramidal or cerebellar signs (ataxia) or autonomic failure as major diagnostic criteria, certain other clinical features known as ‘‘red flags’’ or warning signs may raise the clinical suspicion of MSA. Case report: A 67-year-old woman was admitted to our hospital due to inability to walk. Neurological examination revealed severe Parkinsonism with poor response to levodopa therapy, ataxia. Brain MR imaging showed severe atrophy of the cerebellum, pons, minimal cerebrum, and revealed cross section at the pons in the axial section. Mini-Mental State Examination (MMSE) was normal, Median SEP within normal limits but left Tibial SEP was longer than right side (right: 41, left: 44.2). Although sympathetic skin response of upper extremities was normal, the sympathetic skin responses of lower extremities were absent. RR interval study and a graded head-up tilt test were consistent with autonomic dysfunction. We report a patient with MSA because in clinical practice a case who fullfits all criteria is really rare.