Objective: To present a case of a patient with multiple system atrophy (MSA) parkinsonian subtype (MSA-P) with unusual long disease duration.

Background: MSA is a neurodegenerative disease without any specific pharmacological treatment, that invariably, leads to death in the range of 6 to 9 years from disease onset. It has been reported that a few of these patients can show good l-dopa response for many years and that about 4% of MSA patients may present a long disease duration up to 21 years. The moment in which dysautonomic symptoms appear are a crucial prognostic factor.

Methods: Description of case report of prolonged survival in MSA patient and review of the literature.

Results: This female patient started with rest tremor and bradykinesia at age 39, being diagnosed of Parkinson´s disease (PD). She started on l-dopa with good response. After 10 years she presented motor fluctuations that were difficult to treat pharmacologically due to the presence of wearing-off periods together with dystonic postures of the limbs and orofacial dyskinesias. She was referred to our center to be evaluated for DBS at age 54. A brain MRI disclosed no abnormalities and a l-dopa test showed 40% improvement of UPDRS score. However, she was rejected for surgery since the quality of the on period was judged to be insufficient. In the ensuing years, motor fluctuations, freezing of gait and dystonic postures of the limbs induced by levodopa were aggravated; besides, she developed severe REM sleep behavior disorder symptoms, and dysautonomic features. After 17 years of disease duration, the diagnosis of MSA was suspected, and a second MRI showed a bilateral putaminal rim, severe cerebellar and pontine atrophy and the hot-cross bun sign. An I-123-MIBG showed absence of heart denervation. A laryngoscopy showed vocal cord dystonic contractions. Severe urinary incontinence, orthostatic hypotension and pyramidal signs were also present. At this moment, the patient fulfilled the diagnostic criteria for probable MSA-P; Currently she is alive after 22 years after the onset of symptoms.

Conclusions: Long survival of MSA patients has been previously reported, however this case represents the more prolonged survival in a MSA-P patient reported until know. It has been proposed that the reason explaining the prolonged response to levodopa in this patients could be a predominant presynaptic derangement with relative preservation of the striatal terminals. The existence of such cases must be considered in patients diagnosed of PD presenting atypical signs and symptoms even after many years of disease onset.

References: 1. Petrovic IN, Ling H, Asi Y, Ahmed Z, Kukkle PL, Hazrati LN, Lang AE, Revesz T, Holton JL, Lees AJ. Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch. Mov Disord 2012; 27:1186–90. 2. Berciano J, Valldeoriola F, Ferrer I, Rumià J, Pascual J, Marín C, Rey MJ, Tolosa E. Presynaptic parkinsonism in multiple system atrophy mimicking Parkinson’s disease: a clinicopathological case study. Mov Disord. 2002;17(4):812-6.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/unusual-prolonged-survival-in-multiple-system-atrophy-a-case-report/