Objective: To describe the clinical phenotype of patients with MSA in a center in Mexico City

Background: Multiple System Atrophy (MSA) is an adult-onset;progressive,neurodegenerative disease characterized by autonomic failure ,Parkinsonism and cerebellar ataxia.It can be divided into parkinsonian (MSA-P) and cerebellar (MSA-C).Onset is in the 6^th decade of life;mean survival from the onset of symptoms is 6-10 years.OSA affects 40% of patients.On MRI we observe atrophy of putamen,middle cerebellar peduncle,pons or cerebellum or “hot-cross-bun sign”in _T2-weighted sequences.On FDG-PET the most common pattern is hypometabolism in posterior putamen and cerebellum.

Methods: We reviewed the database of patients seen at the Movement Disorders Clinic during the last 3 years.Patients diagnosed with probable MSA were included.The following variables were collected:demography,clinical features,subtype and diagnostic tests.

Results: Fourteen patients  were included (8 women).Mean age was 65.1 years (48-83). Mean age of onset of symptoms was 59.9 (42- 74). Current disease duration was 5.4 years (2- 9). The most frequent variant was MSA-P.Five patients had urinary alterations at the time of diagnosis.Cognitive impairment was found on working memory and dysexecutive functioning.Thirteen patients had different combinations of sleep disorders. In all patients, MRI showed decreased cortical volume in the frontal and parietal. Most patients showed signs of cerebellar atrophy,2 bulbar/pontine degeneration,1 pars compact atrophy,and 9 microangiopathy.Thirteen patients had SSEPs with proprioceptive alterations,9 showed generalized cerebellar hypometabolism on FDG-PET and 9 dysautonomic  alterations.Only 6 patients with AMS-P showed a mild response to Levodopa.

Conclusions: Medical Literature describes a male predominance, we found a female predominance.Other populations showed the average onset of disease to be 50 years, in our population it was 65.07. Our most frequent clinical variant was MSA-P.The mean age of survival is 5-9.5 years;n our population the average was 6 years.Most of our patients had OSA and all of our patients with SSEPs presented alterations. These clinical differences as well as the age of onset could be explained by ancestry and it would be worthwhile to carry out additional studies in our population.

References: 1.-Fanciulli, A., & Wenning, G. K. (2015). Multiple-system atrophy. New England Journal of Medicine, 372(3), 249-263.

2.-Wenning GK, Geser F, Krismer F, et al. The natural history of multiple system atrophy: A prospective European cohort study. Lancet Neurol. 2013;12(3):264-274.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-phenotype-in-patients-with-multiple-system-atrophy-in-the-mexican-population/