Elderly-Onset Multiple System Atrophy with Lewy Body Pathology: A Case Report

Area of Science:

• Neurology
• Pathology

Background:

• Multiple System Atrophy (MSA) is a rare, sporadic, and fatal neurodegenerative disorder characterized by autonomic dysfunction, parkinsonism, and cerebellar ataxia.
• Parkinson's disease (PD) is a progressive neurodegenerative disorder primarily affecting dopamine-producing neurons in the substantia nigra.

Observation:

• An 81-year-old woman presented with progressive dysarthria and gait disturbance, later developing orthostatic hypotension, sleep apnea, tremor, and rigidity, consistent with MSA.
• Despite dysphagia and choking, she declined artificial feeding or tracheostomy.
• She died suddenly at age 91, with autopsy revealing neuropathological features of both MSA and PD.

Findings:

• Autopsy confirmed co-existing MSA and PD pathology.
• Peripheral autonomic ganglia showed pre- and postganglionic synucleinopathy, a potential factor in sudden death.
• The patient survived 10 years post-onset, longer than expected given poor prognostic factors like old age, early autonomic dysfunction, falls, and co-existing PD pathology.

Implications:

• This case underscores the complex interplay between MSA and PD pathologies.
• Peripheral autonomic synucleinopathy may contribute to sudden mortality in MSA.
• Multidisciplinary care and preserved cognition may facilitate longer survival in MSA patients, even with complicating factors.