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Genetic neurodegenerative disorder characterized by a
loss of sensations, especially in the lower extremities, leading to
perforating skin ulceration and bone destruction as a result of abnormal
functioning of the autonomic nervous system.
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Acrodystrophic Neuropathy; Burning Feet Syndrome;
Denny-Brown Syndrome I; Hereditary Sensory Neuropathy type I (HSN I);
Hereditary Sensory Radicular Neuropathy; Hick Syndrome; Lumbosacral
Syringomyelia; Mutilating Acropathy; Sensory Radicular Neuropathy, type I;
Smith-Thévenard Syndrome; Thévenard Disease II; Thévenard
Syndrome; Ulcerative and Mutilating Acropathy.
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First reported by Auguste Nélaton, a French physician
and surgeon, in 1852.
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Autosomal dominant, chromosome 9q22.
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Shorter lifespan of ganglion cells in sacral and
lumbar dorsal root ganglia.
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Ganglia biopsy showing loss of neuronal cells with
sometimes amyloid deposits, mainly around the capillaries. At the beginning,
Meissner corpuscles count in skin biopsies are normal. With disease
progression, loss of these sensory corpuscles occurs. Sometimes associated
with brain atrophy.
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Onset is between 15 and 40 years of age. Loss of
sensitivity in the feet, with painless ulcers, occurs first, followed by
shooting pain and lightning pain in the lower legs. Ulcers require years to
heal. There is thermal sensory loss of the lower extremities, often
extending to the thigh, sometimes thermal loss in the hands, and restless
legs and burning sensations in the feet. Shortly thereafter, the patient
complains of bilateral neurosensory deafness. Normal sweating. The other
cranial nerves are not involved. Normal ocular motility, normal pupils and
pupillary reactions, no nystagmus. Disappearance of ankle jerks and knee
jerks. Secondary Charcot-type neurotrophic arthropathy and mutilating
acropathy. Upper extremities seldom affected. No motor involvement. Deafness
is a common feature of the syndrome.
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Precautions before Anesthesia
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Usually patients present with
painless cutaneous ulcers for orthopedic procedures. For medicolegal reasons, it is
advisable to obtain a neurologic evaluation before and after the procedure.
Search for signs of autonomic dysfunction before induction.
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Anesthetic Considerations
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General anesthesia is often preferred,
even though regional anesthesia cannot be ...