caa a22 4500 465767826 CHVBK 20180323111919.0 cr unu---uuuuu 170327e19901001xx s 000 0 eng 10.1007/BF00307638 doi (NATIONALLICENCE)springer-10.1007/BF00307638 An autopsy case of peroneal muscular atrophy with rigidity and tremor [Elektronische Daten] Ultrastructural and systematic morphometrical studies on peripheral nerves [Y. Itoh, S. Yagishita, N. Amano, K. Iwabuchi] Summary: An autopsy case of hereditary peroneal muscular atrophy (PMA) with rigidity and static tremor is presented. The patient developed slowly progressive distal muscular atrophy of the legs at the age of 15 years. By the age of 52 years, PMA became marked associated with pes cavus, and tremor and rigidity of the extremities were noted. Motor and sensory conduction velocities gradually depressed and lost near the end of his life. At autopsy, the major neuropathological abnormalities involved the peripheral nervous systems, and were characterized by axonal atrophy and loss of myelinated fibres. These changes involved both the proximal and distal nerves, being more severely affected in the distal. The pathological changes in other regions of the nervous systems were mainly confined to the spinal cord, dorsal ganglia and spinal nerve roots, and pigmented neurons in the brain stem. Morphometrically, the total fascicular area was much smaller than in control, but the total number of myelinated fibers greatly outnumbered that of control 75 200 to 48 200 at the proximal sciatic nerve and then gradually decreased towards the periphery; however, even in the distal sural nerve, the total number of myelinated fibers exceeded that of control (6820 to 5469). Thus, the density of myelinated fibers were much higher, being 1.5 to 2 times greater, than in control. Its abrupt decline at the distal nerve might account for neurogenic atrophy of the distal musculature. Unmyelinated fibers were slightly increased in density and not atrophic. This case is unique in its clinicopathology and does not belong to any subtypes of PMA including "neuronal plus”. Springer-Verlag, 1990 Peroneal muscular atrophy nationallicence Rigidity and tremor nationallicence Autopsy nationallicence Morphometry nationallicence Electron microscopy nationallicence Itoh Y. Department of Pathology, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut Yagishita S. Department of Neuropsychiatry, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut Amano N. Department of Neuropsychiatry, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut Iwabuchi K. Department of Neuropathology, Psychiatric Research Institute of Tokyo, 2-1-8 Kamikitazawa, Setagaya-Ku, 156, Tokyo, Japan aut Acta Neuropathologica Springer-Verlag 80/6(1990-10-01), 671-679 0001-6322 80:6<671 1990 80 401 https://doi.org/10.1007/BF00307638 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00307638 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Itoh Y. Department of Pathology, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut NATIONALLICENCE 700 1- Yagishita S. Department of Neuropsychiatry, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut NATIONALLICENCE 700 1- Amano N. Department of Neuropsychiatry, Kanagawa Rehabilitation Center, Nanasawa 516, 243-01, Atsugi-shi, Kanagawa-ken, Japan aut NATIONALLICENCE 700 1- Iwabuchi K. Department of Neuropathology, Psychiatric Research Institute of Tokyo, 2-1-8 Kamikitazawa, Setagaya-Ku, 156, Tokyo, Japan aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/6(1990-10-01), 671-679 0001-6322 80:6<671 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer