Hereditary sensory autonomic neuropathy Type IV.
Hereditary sensory autonomic neuropathy Type IV is an autosomal recessive disorder due to lack of maturation of small myelinated and unmyelinated fibers of peripheral nerves, which convey sensation of pain and temperature, therefore, resulting in self mutilation. There is anhidrosis due to lack of innervation of normal sweat glands resulting in recurrent episodes of hyperpyrexia. The clinical presentation of two children with this rare disease is described.
Dua
Tarun
T
University College of Medical Sciences, New Delhi, India. tdua@sify.com
Sharma
Jyoti
J
Singhal
Tanu
T
Arya
Ved Bhushan
VB
D002648:Child; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis*; D006358:Hot Temperature / Q000009:adverse effects; D006801:Humans; D007223:Infant; D008297:Male; D019957:Motor Skills Disorders / Q000235:genetics; D010147:Pain Measurement; D012652:Self Mutilation / Q000235:genetics