Abstract
IntroductionMultiple system atrophy is a late, adult-onset α-synucleinopathy with no data on the effect of pregnancy on the disease course. Early stage multiple system atrophy can be difficult to distinguish from Parkinson's disease.Case presentationWe describe the case of an Irish woman with parkinsonism starting at age 31, initially diagnosed as having dopa-responsive, idiopathic Parkinson's disease, who successfully delivered a full-term child at age 35. Her pregnancy was complicated by severe orthostatic hypotension and motor fluctuations. Two years post-partum, she underwent bilateral subthalamic nuclei deep brain stimulation for intractable motor fluctuations and disabling dyskinesia. After this treatment course she experienced deterioration of motor symptoms and death eight years after disease onset. Post-mortem neuropathological examination revealed striatonigral degeneration and α-synuclein-positive glial cytoplasmic inclusions in brain stem nuclei, basal ganglia and white matter tracts, consistent with a neuropathological diagnosis of multiple system atrophy.ConclusionsMultiple system atrophy can affect women of child-bearing age and pregnancy may be associated with marked disease progression.
Highlights
Multiple system atrophy is a late, adult-onset a-synucleinopathy with no data on the effect of pregnancy on the disease course
Multiple system atrophy can affect women of child-bearing age and pregnancy may be associated with marked disease progression
Multiple system atrophy (MSA) is an a-synucleinopathy characterized by akinetic-rigid parkinsonism, autonomic failure, urogenital dysfunction, cerebellar signs, and pyramidal signs in varying combinations [1]
Summary
Multiple system atrophy (MSA) is an a-synucleinopathy characterized by akinetic-rigid parkinsonism, autonomic failure, urogenital dysfunction, cerebellar signs, and pyramidal signs in varying combinations [1]. Case presentation A 31-year-old Irish woman who resided in the midwestern USA developed progressive loss of left hand dexterity and rest tremor Eight months later, she experienced episodes of pre-syncope triggered by going up or down stairs or by a hot environment. Results of routine blood examination, ceruloplasmin, and a cerebral magnetic resonance imaging (MRI) scan were normal She was diagnosed as having early-onset idiopathic Parkinson’s disease, based upon gradual onset asymmetric parkinsonism with rest tremor. She had excellent response to levodopa/carbidopa with mild levodopa-induced dyskinesias at doses that provided optimal motor benefit, 900 mg levodopa/day Five months after she started taking levodopa, her UPDRS III score decreased from 20.5 to 11 points and she became pregnant with her first child. Pathological features combined with her clinical presentation led to a diagnosis of MSA-P
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