Congenital insensitivity to pain: a 20 year follow up

The exact nosological status of "congenital insensitivity to pain" remains in doubt. Possible pathological correlates of this clinical syndrome include sensory neuropathy, central lesions at the level of the reticular formation or dorsal horn of the spinal cord, or a central indifference t...

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Bibliographic Details
Published in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1994-08, Vol.57 (8), p.973-974
Main Authors: Larner, A J, Moss, J, Rossi, M L, Anderson, M
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Biopsy
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Diagnosis, Differential
Diagnostic Errors
Electromyography
Evoked Potentials
Follow-Up Studies
Hereditary Sensory and Autonomic Neuropathies - diagnosis
Hereditary Sensory and Autonomic Neuropathies - genetics
Hereditary Sensory and Autonomic Neuropathies - pathology
Hereditary Sensory and Autonomic Neuropathies - physiopathology
Humans
Male
Medical sciences
Neural Conduction - physiology
Neurology
Pain Insensitivity, Congenital - diagnosis
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Summary:The exact nosological status of "congenital insensitivity to pain" remains in doubt. Possible pathological correlates of this clinical syndrome include sensory neuropathy, central lesions at the level of the reticular formation or dorsal horn of the spinal cord, or a central indifference to, or asymbolia for, pain. The reassessment of two members of a kindred previously reported more than 20 years ago as having congenital insensitivity to pain indicated that they in fact had an inherited sensory and autonomic neuropathy. Prolonged follow up and morphometric analysis of sequential nerve biopsies may be necessary to definitively establish this diagnosis.
ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.57.8.973