Abstract | BACKGROUND: OBJECTIVE: To examine the electrophysiological features of late-onset FAP TTR Met30 unrelated to endemic foci. METHODS: Nerve conduction findings in 44 cases with an onset of more than 50 years of age in a non-endemic area were assessed and compared with findings from 21 earlier-onset cases related to endemic foci. RESULTS: The extent of the reduction of the compound muscle action potential and, especially, the sensory nerve action potential was more profound in the late-onset group even when the decline of these indices with aging in normal control subjects was taken into account. The feature of predominant lower-limb involvement seemed to be more conspicuous in the late-onset group. Electrophysiological indices tended to be aggravated as the duration of neuropathic symptoms increased in the early-onset group, while most of these indices in the lateonset group did not show this correlation. A slowing of conduction velocity and a prolongation of distal latency, which suggests demyelination, were conspicuous in some patients. Pathologically, a predominant loss of small-fibers was not conspicuous in sural nerve biopsy specimens from late-onset patients. Large myelinated fiber density showed a negative correlation with the disease duration in early-onset cases, but not in late-onset cases. CONCLUSIONS: Electrophysiological differences between late- and early-onset cases were present, probably reflecting the different underlying pathogenic mechanisms of neuropathy. The demyelinating feature does not exclude the possibility of this disease.
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Authors | Haruki Koike, Yuichi Kawagashira, Masahiro Iijima, Masahiko Yamamoto, Naoki Hattori, Fumiaki Tanaka, Masaaki Hirayama, Yukio Ando, Shu-ichi Ikeda, Gen Sobue |
Journal | Journal of neurology
(J Neurol)
Vol. 255
Issue 10
Pg. 1526-33
(Oct 2008)
ISSN: 0340-5354 [Print] Germany |
PMID | 18821042
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Amyloid
- Prealbumin
- amyloid prealbumin
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Topics |
- Action Potentials
- Adult
- Age Factors
- Age of Onset
- Aged
- Amyloid
(genetics)
- Amyloid Neuropathies, Familial
(epidemiology, physiopathology)
- Female
- Humans
- Leg
(innervation, physiopathology)
- Male
- Median Nerve
(physiopathology)
- Middle Aged
- Nerve Fibers, Myelinated
(pathology)
- Neural Conduction
- Prealbumin
(genetics)
- Sensory Receptor Cells
(pathology, physiology)
- Sural Nerve
(pathology)
- Tibial Nerve
(physiopathology)
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