• Internal medicine · Mar 2023

    Case Reports

    Dentatorubropallidoluysian Atrophy with Prominent Autonomic Dysfunction.

    • Ayako Shioya, Hiroshi Takuma, Norio Ohkoshi, Kuniyoshi Hirano, Tomohiko Ishihara, Kazuhiro Ishii, and Akira Tamaoka.
    • Department of Neurology, Mito Kyodo General Hospital, Tsukuba University Hospital Mito Area Medical Education Center, Japan.
    • Intern. Med. 2023 Mar 15; 62 (6): 889892889-892.

    AbstractWe herein report a 45-year-old man with dentatorubropallidoluysian atrophy (DRPLA) who presented with mild dementia, ataxia, and involuntary movement and developed constipation, dysuria, and orthostatic hypotension. Thermography revealed an abnormal thermal response of the skin to cold stimulation. Skin temperature reflects the skin blood flow and is regulated by the sympathetic nervous system. Thermography is currently used to study diseases associated with vasomotor dysfunction of the skin. The thermography results suggested the possibility of autonomic dysfunction. Although little is known regarding autonomic dysfunction in DRPLA, this report demonstrates the importance of autonomic dysfunction in DRPLA.

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