Rehabilitation of an adult with disabilities due to congenital sensory neuropathy.
A 37-year-old woman presented with congenital loss of all peripheral sensory modalities, sparing only her face, paraspinal area and proximal limbs. She was otherwise neurologically normal. Her sensory neuropathy was complicated by autoamputation of phalanges, pathologic fractures, failed total hip replacement, burns, pressure ulcers, and a painless peptic ulcer. An unusual cluster of functional deficits occurred when she was unable to substitute vision for peripheral sensibility. In the sudden darkness of a power failure she would fall to the floor; we advised the installation of battery-operated safety lights activated by a drop in line voltage. Voice-activated lights overcame difficulty in finding light switches in darkened rooms. She was unable to drive at night until she had a light placed under the dashboard of her car so she could see the foot pedals. She was unable to wash her hair, as shampoo irritation made her close her eyes and lose balance; swimming goggles and a nonfogging mirror permitted independence in her hair care. The solution of these functional difficulties provides a fresh perspective on the rehabilitation of patients with severe sensory impairments, especially with a cooperative family.
Nance
P W
PW
Kirby
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RL
D000328:Adult; D002056:Burns / Q000517:prevention & control; D005260:Female; D005598:Fractures, Spontaneous / Q000517:prevention & control; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000235:genetics / Q000534:rehabilitation*; D006801:Humans; D000699:Pain Insensitivity, Congenital / Q000235:genetics; D011434:Proprioception; D012656:Self-Help Devices