TY - JOUR
T1 - Neurogenic arthropathy and recurring fractures with subclinical inherited neuropathy
AU - Dyck, Peter James
AU - Stevens, John Clarke
AU - O’Brien, Peter C.
AU - Oviatt, Karen F.
AU - Lais, Alfred C.
AU - Coventry, Mark B.
AU - Beabout, John W.
PY - 1983/3
Y1 - 1983/3
N2 - Some patients with radiologic findings of neurogenic arthropathy or multiple fractures do not exhibit overt neurologic signs, Results of nerve conduction velocity, computer-assisted sensory examination, periosteal nociception, and morphometric and graded teased-fiber evaluation of cutaneous nerves allowed us to recognize a mild neuropathic abnormality. Neurogenic arthropathy and subclinical neuropathy were also found in relatives. In three kinships, the underlying disorder was probably hereditary sensory neuropathy type 1 and in several others, it was recessively inherited sensory neuropathy. These arthropathies were often painful, and overt loss of superficial and deep pain sensation was not a prominent or necessary condition. An interplay of multiple factors including insensitivity, trauma, obesity, activity, abuse, personality, mental subnormality, and metabolic joint and bone disease are probably involved in the development of the bony lesions and thus provide further evidence that environmental factors affect expression of human mutant genes for inherited ncuropathy.
AB - Some patients with radiologic findings of neurogenic arthropathy or multiple fractures do not exhibit overt neurologic signs, Results of nerve conduction velocity, computer-assisted sensory examination, periosteal nociception, and morphometric and graded teased-fiber evaluation of cutaneous nerves allowed us to recognize a mild neuropathic abnormality. Neurogenic arthropathy and subclinical neuropathy were also found in relatives. In three kinships, the underlying disorder was probably hereditary sensory neuropathy type 1 and in several others, it was recessively inherited sensory neuropathy. These arthropathies were often painful, and overt loss of superficial and deep pain sensation was not a prominent or necessary condition. An interplay of multiple factors including insensitivity, trauma, obesity, activity, abuse, personality, mental subnormality, and metabolic joint and bone disease are probably involved in the development of the bony lesions and thus provide further evidence that environmental factors affect expression of human mutant genes for inherited ncuropathy.
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U2 - 10.1212/wnl.33.3.357
DO - 10.1212/wnl.33.3.357
M3 - Article
C2 - 6681882
AN - SCOPUS:0020675007
SN - 0028-3878
VL - 33
SP - 357
EP - 367
JO - Neurology
JF - Neurology
IS - 3
ER -