Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Amyotrophic lateral sclerosis with muscle weakness and dropped head during the course of Parkinson's disease: an autopsy case

Shinji Oda, M.D.1), Terunori Sano, M.D.2), Noriko Nishikawa, M.D., Ph.D.1), Michita Mikasa, M.D., Ph.D.3), Yuji Takahashi, M.D.,Ph.D.1) and Masaki Takao, M.D., Ph.D.2)

1) Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry
2) Department of Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry
3) Department of Neurology, Tokyo Metropolitan Hiroo Hospital

A 64-year-old female developed Parkinson's disease at the age of 52 years. She experienced muscle weakness in the upper right extremities and dropped head at 62 and 63 years, respectively; both symptoms were considered to be associated with Parkinson's disease (PD). The dosage of L-DOPA was increased from 200 mg/day to 900 mg/day; however, her neurological symptoms did not improve. Eventually, she was diagnosed with amyotrophic lateral sclerosis (ALS) at 64 years. She was placed under palliative care, and died of respiratory failure and malnutrition. Neuropathologic findings were consistent with the coexistence of PD and ALS. In fact, there were α-synuclein immunoreactive Lewy bodies (Braak stage 4) as well as TDP-43 immunoreactive deposits in the motor nuclei at the level of brainstem and spinal cord. Therefore, coexisting pathologies must be taken into account in a patient showing multi-system symptoms.
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(CLINICA NEUROL, 61: 373|377, 2021)
key words: amyotrophic lateral sclerosis, Parkinson's disease, Brait-Fahn-Schwartz disease, dropped head, wearing-off phenomenon

(Received: 17-Sep-20)