Table 2.

Classification of Hereditary Sensory and Autonomic Neuropathy (HSAN)

Type	Gene	Prominent Phenotype
HSAN with autosomal dominant inheritance
HSAN-I	HPTLC1 or HPTLC2 or ATL1	Neuropathic pain; loss of pain/temperature sensation ulcerative mutilations; ± distal muscle atrophy
HSAN-I with dementia	DNMT1	Neuropathic pain; loss of all sensory modalities; ulcerative mutilations; dementia; hearing loss
HSAN with autosomal recessive inheritance
HSAN-II	WNK1 or FAM134B or KIF1A	Loss of sensory functions; mutilating ulcers
HSAN-III	IKBKAP	Congenital sensory loss; absence of tongue fungiform Papillae; hyperhidrosis
HSAN-IV	NTRK1	Congenital sensory loss; anhidrosis; fever; skin lesions; joint deformities
HSAN-V	NGFB	Congenital sensory loss (pain); bone fracture; joint deformities