Abstract
Hereditary Sensory Autonomic Neuropathies (HSAN) are rare genetically group of disorders, affecting sensory and autonomic nervous system. HSAN are divided into five groups on basis of mode of inheritance, clinical symptoms and age at onset. Here we describe two children belonged to one family. Eight years old son, has suffered from multiple clinical features including, complete anesthesia to pain and temperature, multiple painless ulcers, erosive lesions of distal phalanges in toes, mutilating arthritis, osteonecrosis in tarsal bones and osteomyelitis of tibia, which were slowly progressive, since he was 2 years old. Twenty three years old daughter had similar clinical findings, but with greater severity and because of intractable and infective ulcers, below knee amputation had been performed for her. Now she is using prosthesis for right amputated leg. In electro diagnostic tests, all Sensory Nerve Action Potentials (SNAPs) were unobtainable, but no evidence of motor nerves involvement was noted in our study. All of above results are compatible with HSAN type II. There is no cure or definite treatment for this disease. The aim of this article is presentation of these rare disorders and their clinical and electro physiologic findings and finally emphasizing in rehabilitation treatments for prevention of progress and disease complications.