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NEUROLOGY 1998;51:714-721
© 1998 American Academy of Neurology

Denervation of eccrine glands in patients with familial amyloidotic polyneuropathy type I

A. Ohnishi, MD, T. Yamamoto, MD, Y. Murai, MD, Y. Ando, MD, M. Ando, MD, Y. Hoshii, MD and M. Ikeda, PhD

From the Department of Neurology (Drs. Ohnishi, Yamamoto, and Murai), School of Medicine, and Department of Occupational Health Economics (Dr. Ikeda), Institute of Industrial Ecological Sciences, University of Occupational and Environmental Health, Kitakyushu; The First Department of Internal Medicine (Drs. Y. Ando and M. Ando), Kumamoto University School of Medicine, Kumamoto; and Department of Pathology (Dr. Hoshii), Yamaguchi University School of Medicine, Ube, Japan.

Address correspondence and reprint requests to Dr. Akio Ohnishi, Department of Neurology, University of Occupational and Environmental Health, Japan, 1-1, Iseigaoka, Yahata Nishi-ku, Kitakyushu 807-8555, Japan.

Objective: To study the alterations in the structure and innervation of eccrine glands in familial amyloidotic polyneuropathy (FAP) type I with Val 30 Met transthyretin mutation.

Background: Anhidrosis of the distal lower limbs is a prominent feature of FAP type I.

Methods: Qualitative and morphometric study of amyloid deposition, eccrine glands, and their innervation in nine patients with FAP type I (duration of sensory symptoms, 8.4 ± 3.9 years [mean ± SD]; range, 3 to 15 years) and seven control subjects.

Results: On light microscopy, the endoneurium of cutaneous nerve fascicles had no definite amyloid deposition. Amyloid deposition was observed around eccrine glands in seven of nine patients. On electron microscopy, no focal destruction and degeneration of eccrine glands or ducts and of Schwann cell processes with or without nerve terminals or unmyelinated axons were observed in relation to adjacent amyloid deposition. Secretory vacuoles and granules of dark cells were markedly decreased in some secretory coils. Nerve terminals and unmyelinated axons of eccrine glands were considerably fewer in patients than in control subjects, and denervation was prominent in all patients. A few nerve terminals and unmyelinated axons of eccrine glands were present in patients who had experienced sensory symptoms for 3, 5, and 6 years, but were absent in patients with sensory symptoms for more than 7 years.

Conclusions: Eccrine glands are markedly to totally denervated in patients with FAP type I and chronic sensory symptoms. The extent of denervation indicates the severity of autonomic denervation and therefore may suggest the timing of liver transplantation.

Supported in part by a research grant (7B-1-06) for nervous and mental disorders from the Ministry of Health and Welfare, Japan.

Received January 15, 1998. Accepted in final form April 29, 1998.






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