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From the Department of Neurology (Drs. Koike, Misu, Sugiura, Iijima, Mori, Yamamoto, Hattori, and Sobue), Nagoya University Graduate School of Medicine, Department of Neurology (Dr. Mukai), Nagoya National Hospital, Department of Laboratory Medicine (Dr. Ando), Kumamoto University School of Medicine, and Third Department of Medicine (Dr. Ikeda), Shinshu University School of Medicine, Matsumoto, Japan.
Address correspondence and reprint requests to Dr. G. Sobue, Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan; e-mail: sobueg{at}med.nagoya-u.ac.jp
Background: Late-onset type I familial amyloid polyneuropathy (FAP TTR Met30) cases unrelated to endemic foci in Japan show clinical features setting them apart from early-onset cases in endemic foci.
Objective: To compare pathologic features between the early- and late-onset types.
Methods: Pathologic findings in FAP TTR Met30 with onset before age 50 in relation to endemic foci (11 cases) were compared with those in 11 later-onset cases unrelated to endemic foci.
Results: Sural nerve biopsy specimens showed predominantly small-fiber loss in early-onset cases; variable fiber size distribution, axonal sprouting, and relatively preserved unmyelinated fibers characterized late-onset cases. Autopsy cases representing both groups showed amyloid deposition throughout the length of nerves and in sympathetic and sensory ganglia, but amounts were greater in early-onset cases. Amyloid deposition and neuronal cell loss were greater in sympathetic than dorsal root ganglia in early-onset cases; the opposite was true in late-onset cases. Size assessment of remaining neurons in these ganglia suggested predominant loss of small neurons in early-onset cases but loss of neurons of all sizes in late-onset cases. Transthyretin-positive, Congo red-negative amorphous material was more conspicuous in nerves from late- than early-onset cases. In extraneural sites, amyloid was more conspicuous in thyroid and kidney from early-onset cases and in heart and hypophysis from late-onset cases. In early-onset cases, cardiac amyloid deposition was prominent in the atrium and subendocardium but was conspicuous throughout the myocardium in late-onset cases.
Conclusion: The pathology of early- and late-onset FAP TTR Met30 correlated well with differences in clinical findings.
Received October 8, 2003. Accepted in final form February 23, 2004.
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