title: [Construction and clinical evaluation of novel methods for detecting autoinomic dysfunction].
abstract: The autonomic nervous system innervates every organ in the body. Since autonomic disturbances affect patient survival, an understanding of and recognition of these disturbances are important. We adopted several new methods to evaluate autonomic function accurately for detecting the onset of small-fiber neuropathy, such as laser-Doppler flowmetry, ultrasonography, 123I-MIBG scintigraphy, electrogastrography, cystometry, glucose-tolerance test, near-infrared spectrophotoscopy, blood pressure tests, and evaluation of sweating. After these examinations, we applied potential effective treatments to improve the survival and daily activity of these patients. We also evaluated the effect of liver transplantation on autonomic dysfunction of familial amyloidotic polyneuropathy (FAP) patients using our tools. Liver transplantation not only prevents the progression of autonomic dysfunction in these patients but also improves some autonomic symptoms in the early stage after the operation. As all domino-liver transplantation-induced amyloid neuropathies, including our cases, were of the sensory type rather than with autonomic involvement, assessment of the pain threshold by preferential stimulation of A delta fibers is a particularly useful tool for diagnosing the onset of small-fiber neuropathies in these patients in addition to autonomic testing. Analyses and comparison of patients with FAP and domino-liver-transplanted patients with autonomic, sensory and motor dysfunction may give a clue to elucidate the pathogenesis and treatment of neuropathy in FAP. As autonomic disturbances play an important role in the symptomatology of small-fiber neuropathy, liver-transplanted FAP, and domino-liver-transplanted patients, further studies of autonomic dysfunction in these patients may lead to the pathogenesis of the disease.
authors: Kumamoto Amyloidosis Unit, Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto 860-8556, Japan. konen@fc.kuh.kumamoto-u.ac.jp
authors: Konen
authors: K
authors: Obayashi
mesh_terms: D028227:Amyloid Neuropathies, Familial / Q000175:diagnosis* / Q000503:physiopathology*; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis* / Q000503:physiopathology; D003943:Diagnostic Techniques, Neurological / Q000295:instrumentation; D006801:Humans; D016031:Liver Transplantation; D014666:Vasomotor System / Q000503:physiopathology