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Updated: Aug 19, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Autonomic Dysfunction in ATTR Amyloidosis]
Minori Kodaira1, Yoshiki Sekijima
1Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine.
None:
In addition to polyneuropathy, patients with hereditary transthyretin amyloidosis (ATTRv) often experience autonomic dysfunction, which reduces quality of life. Compared with patients with ATTRv from endemic areas, those from non-endemic areas rarely develop autonomic dysfunction as an initial symptom. Conversely, autonomic dysfunction can be a diagnostic red flag in patients from non-endemic areas without a family history. Autonomic dysfunction in patients with wild-type transthyretin amyloidosis (ATTRwt) has only recently been reported. However, further knowledge is needed to clarify its pathophysiology.
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