Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology

ABSTRACT

Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

KEYWORDS:

• Corticobasal syndrome
• corticobasal degeneration
• α-synuclein
• tau
• dementia with Lewy body disease
• pathology

Acknowledgments

We thank the patient, Mr. Lynwood Blote, and his family for their invaluable contribution to neurodegeneration research. We have included the patient’s full name as per his family’s request.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This work was supported by grant number P50AG023501 from NIH National Institute on Aging, the Hellman Family Foundation, and the Consortium on Frontotemporal Dementia Research.