Objective: Parkinsonism is characterized by bradykinesia, rigidity, rest tremor, and postural instability. The most common cause of parkinsonism is Parkinson’s disease (PD) and, rarely, Parkinson-plus syndromes. Here we report an unusual case of concurrent parkinsonism pathologies.

Background: A 79 year old woman with a history of hypertension and prior alcohol abuse presented to the movement disorders clinic with a five-year progression of fine motor skill loss, early shuffling and freezing of gait, imbalance, falls, insomnia, agitation, and rigidity. Of note, her symptoms began a few weeks after suspected postoperative hypoxic brain injury. Her rigidity was treated with levodopa for two years, without clear benefit, and she was tapered off after developing dyskinesias.

Methods: She was evaluated with neurologic examination, laboratory studies, brain and cervical spine MRI, FDG PET scan, neuropsychological testing, and neuropathology at autopsy (six years after symptom onset).

Results: Five years after symptom onset, her neurologic exam revealed severe, asymmetric dystonic posturing, spontaneous foot levitation, and bilateral contractures preventing rapid alternating movements. There was no tremor, myoclonus, or chorea. Additionally, impaired vertical eye movements were observed. Several months later, she developed worsening muscle spasms and confusion. Brain MRI showed nonspecific volume loss and white matter disease consistent with small vessel ischemia. FDG PET scan showed increased uptake in the cerebellum and bilateral motor strips. Neuropsychological testing revealed severe memory and visuospatial deficits, with moderate deficits in the executive and language domains. Neuropathology demonstrated severe Lewy body disease, neuron loss, gliosis in the substantia nigra pars compacta, and predominant synucleinopathy, consistent with PD. In addition, there was frontotemporal lobar degeneration, neocortical balloon neurons, astrocytic plaques, and tauopathy, consistent with corticobasal degeneration (CBD).

Conclusions: Although her initial presentation was consistent with parkinsonism, five years into her symptomatic course, she developed asymmetric dystonia, muscle spasms, confusion, and restricted voluntary eye movements in the vertical plane. Neuropathologic examination was consistent with concomitant PD and CBD, demonstrating a rare coexistence of these two parkinsonism pathologies.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinicopathological-characteristics-of-co-existant-parkinsons-disease-and-corticobasal-degeneration-a-case-study/