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MSA-OPCD syndrome in a 35 years old Filipino presenting as progressive ataxia: A case report.


 Dayrit, Greg David V,
 Fugoso, Leonardo,
 Santiago, Jonas

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Publication Information

Publication Type
The Philippine Journal of Neurology
2003 Jul
Publication Date


BACKGROUND: Olivopontocerebellar degeneration (OPCD) is part of the subtypes of multisystem atrophy (MSA) characterized by neuronal degeneration of the cerebellar cortex, the inferior olives, and the pons. The symptoms associated with this disorder are wide-ranging: progressive ataxia, difficulties with balance, slurring of speech, difficulty walking, tremor, abnormal movements, abnormal eye movements, sexual dysfunction, bowel or bladder problems, stiffness or rigidity, spasticity, and difficulty swallowing. At present, there is no cure for OPCD. The disorder is slowly progressive with death usually occurring 10 years after onset of symptoms
OBJECTIVES: To present a rare, interesting case of MSA-OPCD syndrome in a 35 year-old Filipina with progressive ataxia and PET findings of hypometabolisrn in the cerebellum and brainstem
CASE SUMMARY: A 35 years old Filipina female presented with a 2-year history of progressive gait difficulty, frequent tripping, slurred speech, non-specific bladder complaints and vertigo. On examination, she had slight postural hypotension, slight difficulty with arithmetic, scanning speech, tremor worse on the left and impaired saccades, Rombergs sign with tilting to the left, and prominent dysmetria. There were no significant cognitive impairment, evidence of pyramidal tract involvement, or family history. Cranial magnetic resonance imaging showed pancerebellar and brainstem atrophy with enlarged fourth ventricle. Positron emission tomography (PET) imaging revealed reduced metabolism in the brainstem and cerebellum. Neuroscan polygraphy revealed parkinsonian rest tremors of 5-7 Hz in the arms and legs. She was placed on trial levodopa and physical therapy
CONCLUSION: The MSA-OPCD syndrome is a progressive neurodegenerative disease that is uniformly fatal. Mean survival is 6 years with a considerable range. Factors portending a faster progression and shorter survival include older age of onset and the presence of more than one clinical feature. Care is directed towards treatment of symptoms. (Author)

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