Hereditary Sensory and Autonomic Neuropathies (HSAN)

A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)
Also Known As:
HSAN; Congenital Insensitivity to Pain with Anhidrosis; Hereditary Sensory Neuropathy; Neuropathy, Hereditary Sensory; Insensitivity to Pain with Anhidrosis, Congenital; Familial Dysautonomia, Type 2; Giaccai Type Acroosteolysis; HSAN (Hereditary Sensory Autonomic Neuropathy); HSAN 1; HSAN 4; Hereditary Sensory Autonomic Neuropathy, Type 1; Hereditary Sensory Autonomic Neuropathy, Type 2; Hereditary Sensory Autonomic Neuropathy, Type 4; Hereditary Sensory Autonomic Neuropathy, Type 5; Hereditary Sensory Neuropathy Type 1; Hereditary Sensory Radicular Neuropathy; Hereditary Sensory Radicular Neuropathy, Recessive Form; Hereditary Sensory and Autonomic Neuropathy 4; Hereditary Sensory and Autonomic Neuropathy Type 2; Insensitivity to Pain, Congenital, with Anhidrosis; Neurogenic Acroosteolysis; Neuropathy Hereditary Sensory Radicular, Autosomal Dominant; Neuropathy Hereditary Sensory and Autonomic Type 1; Neuropathy, Congenital Sensory; Neuropathy, Congenital Sensory, with Anhidrosis; Acroosteolyses, Neurogenic; Acroosteolysis, Giaccai Type; Acroosteolysis, Neurogenic; Congenital Sensory Neuropathies; Congenital Sensory Neuropathy; HSANs (Hereditary Sensory Autonomic Neuropathy); Hereditary Sensory Neuropathies; Neurogenic Acroosteolyses; Neuropathies, Congenital Sensory; Neuropathies, Hereditary Sensory; Sensory Neuropathies, Congenital; Sensory Neuropathies, Hereditary; Sensory Neuropathy, Congenital; Type Acroosteolysis, Giaccai; Type I, HSAN; Type I, HSN; Type IV, HSAN; Type V, HSAN; HSAN Type I; HSAN Type II; HSAN Type IV; HSAN Type V; HSN Type I; HSN Type II; Neuropathies, Hereditary Sensory and Autonomic; Pain Insensitivity with Anhidrosis, Congenital; Sensory Neuropathy, Hereditary; Sensory and Autonomic Neuropathies, Hereditary
Networked: 180 relevant articles (1 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Pain (Aches)
2. Neurogenic Arthropathy
3. Mental Retardation (Idiocy)
4. Hereditary Sensory and Autonomic Neuropathies (HSAN)
5. Hypohidrosis


1. Levy, Jacov: 9 articles (03/2013 - 09/2002)
2. Timmerman, Vincent: 8 articles (05/2013 - 10/2002)
3. Indo, Y: 8 articles (10/2012 - 01/2000)
4. Indo, Yasuhiro: 6 articles (05/2015 - 05/2002)
5. Auer-Grumbach, Michaela: 6 articles (05/2013 - 10/2002)
6. Hornemann, Thorsten: 6 articles (05/2013 - 04/2009)
7. Penno, Anke: 5 articles (12/2011 - 04/2009)
8. Houlden, Henry: 5 articles (02/2011 - 08/2003)
9. Pinsk, Vered: 5 articles (03/2009 - 09/2002)
10. Mardy, S: 5 articles (10/2001 - 01/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hereditary Sensory and Autonomic Neuropathies:
1. pamidronate (APD)FDA LinkGeneric
2. Nerve Growth Factor (NGF)IBA
3. 3-(2-carboxyindol-3-yl)propionic acidIBA
4. Protein-Tyrosine Kinases (Tyrosine Kinase)IBA
5. Nerve Growth Factor Receptor (Nerve Growth Factor Receptor, Low Affinity)IBA
6. Monoclonal AntibodiesIBA
7. protein N-terminal acetyltransferaseIBA
8. Phosphotransferases (Kinase)IBA
9. Serine C-PalmitoyltransferaseIBA
10. tyrosine receptor (receptor, tyrosine)IBA

Therapies and Procedures

1. Amputation
2. Anesthesia
3. General Anesthesia
4. Investigational Therapies (Experimental Therapy)
5. External Fixators