Hereditary sensory and autonomic neuropathy is a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by sensory and/or autonomic abnormalities.  Hereditary sensory and autonomic neuropathy type 5 (HSAN5) patients manifest loss of pain perception and impaired temperature sensitivity, in the absence of any other major neurological anomalies.  Occasionally, the following features are observed; acral ulcers, increased susceptibility to Staphylococcus aureus infections and accidental injury to joints due to decreased sensation.  The signs and symptoms of HSAN5 appear usually at birth or during infancy.  Diagnosis is based upon clinical features as well as the degree of both sensory and autonomic dysfunction; HSAN5 patients have little reduction of C-fibers, the histamine test may be normal.

HSAN 5 follows an autosomal recessive pattern of inheritance.  Mutations in the NGF gene, encoding the nerve growth factor beta subunit (NGFβ) on chromosome 1p13.1, are the cause of HSAN 5.  NGFβ protein is important in the development and survival of neurons, including sensory neurons.