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Term:
hereditary sensory neuropathy (DOID:0050548)
Annotations: Rat: (29) Mouse: (29) Human: (33) Chinchilla: (29) Bonobo: (29) Dog: (30) Squirrel: (29) Pig: (29)
Parent Terms Term With Siblings Child Terms
46,XY Partial Gonadal Dysgenesis, with Minifascicular Neuropathy  
Agenesis of Corpus Callosum +   
agenesis of the corpus callosum with peripheral neuropathy  
Aicardi-Goutieres syndrome +   
alcoholic neuropathy  
Alexander Disease  
Ataxia and Polyneuropathy, Adult-Onset  
Athabaskan brainstem dysgenesis syndrome  
autonomic peripheral neuropathy 
axonal neuropathy +   
brachial plexus neuropathy +   
Canavan disease  
Central Nervous System Cysts +   
Central Nervous System Vascular Malformations +   
CEREBELLAR ATAXIA, NEUROPATHY, AND VESTIBULAR AREFLEXIA SYNDROME  
cerebellar hypoplasia +   
Cerebrocortical Degeneration of Infancy 
chromosome 17p13.3 duplication syndrome  
chronic polyneuropathy +  
Cockayne syndrome +   
complex cortical dysplasia with other brain malformations +   
Congenital Muscular Dystrophy with Severe Central Nervous System Atrophy and Absence of Large Myelinated Fibers 
cranial nerve disease +   
Dandy-Walker syndrome +   
demyelinating polyneuropathy +   
diabetic neuropathy +   
Drachtman Weinblatt Sitarz Syndrome 
Dystonia Musculorum Deformans +   
ENCEPHALOPATHY, PROGRESSIVE, EARLY-ONSET, WITH BRAIN ATROPHY AND THIN CORPUS CALLOSUM  
erythromelalgia  
Familial Amyloid Polyneuropathies +   
familial encephalopathy with neuroserpin inclusion bodies  
familial episodic pain syndrome +   
Gerstmann-Straussler-Scheinker syndrome  
Gilles de la Tourette syndrome +   
HEART AND BRAIN MALFORMATION SYNDROME  
Hereditary Central Nervous System Demyelinating Diseases +   
Hereditary Optic Atrophies +   
hereditary sensory neuropathy +   
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)
Huntington's disease +   
Huntington's disease-like 2  
Huntington's Disease-Like Syndrome  
idiopathic progressive polyneuropathy 
inflammatory and toxic neuropathy +   
ischemic neuropathy 
Kennedy's disease  
Lafora disease  
Laryngeal Abductor Paralysis with Cerebellar Ataxia and Motor Neuropathy 
Leukoencephalomyelopathy  
Leukoencephalopathy with Dystonia and Motor Neuropathy  
Lipodystrophy with Congenital Cataracts and Neurodegeneration  
Median-Ulnar Nerve Communications 
Mental Retardation, X-Linked +   
Microcephaly, Growth Deficiency, Seizures, and Brain Malformations  
Microphthalmia, Syndromic 10 
Microphthalmia, Syndromic 3  
Microphthalmia, Syndromic 6  
mitochondrial DNA depletion syndrome 6  
mononeuropathy +   
motor peripheral neuropathy +   
myotonia congenita +   
myotonic dystrophy type 1 +   
neural tube defect +   
neuritis +   
neuroacanthocytosis +   
Neurodevelopmental Disorder and Language Delay with or Without Structural Brain Abnormalities  
Neurodevelopmental Disorder with Epilepsy, Cataracts, Feeding Difficulties, and Delayed Brain Myelination  
neurodevelopmental disorder with midbrain and hindbrain malformations  
neurofibromatosis +   
neurogenic scapuloperoneal syndrome Kaeser type  
neuromuscular disease +   
neuronal ceroid lipofuscinosis +   
NFIA-related disorder  
Opticocochleodentate Degeneration 
pantothenate kinase-associated neurodegeneration +   
paraneoplastic polyneuropathy 
PCWH syndrome  
Peripheral Neuropathy, Ataxia, Focal Necrotizing Encephalopathy, and Spongy Degeneration of Brain 
PERIPHERAL NEUROPATHY, AUTOSOMAL RECESSIVE, WITH OR WITHOUT IMPAIRED INTELLECTUAL DEVELOPMENT  
PHARC syndrome  
POEMS syndrome 
Polyendocrine-Polyneuropathy Syndrome  
polyneuropathy in collagen vascular disease 
polyradiculoneuropathy +   
Premature Atherosclerosis with Deafness, Nephropathy, Diabetes Mellitus, Photomyoclonus, and Degenerative Neurologic Disease 
primary cerebellar degeneration +   
PROGRESSIVE PSYCHOMOTOR DETERIORATION 
Ribose 5-Phosphate Isomerase Deficiency  
Schisis Association 
Sensorineural Deafness with Peripheral Neuropathy and Arterial Disease 
sensory peripheral neuropathy  
septooptic dysplasia +   
Severe Infantile Axonal Neuropathy 
spinal muscular atrophy with lower extremity predominance +   
Spinal Muscular Atrophy, Lower Extremity-Predominant, 2B, Autosomal Dominant  
Spongiform Encephalopathy with Neuropsychiatric Features  
survival motor neuron spinal muscular atrophy +   
Tangier disease  
Thiamine Metabolism Dysfunction Syndrome 4 (Bilateral Striatal Degeneration and Progressive Polyneuropathy Type)  
tuberous sclerosis +   
Unilateral Aplasia of Extensor Muscles of Fingers, with Generalized Polyneuropathy 
Unverricht-Lundborg syndrome +   
Wilson disease +   

Synonyms
Exact Synonyms: Congenital Sensory Neuropathy ;   Giaccai Type Acroosteolysis ;   HEREDITARY SENSORY AND AUTONOMIC NEUROPATHY ;   HSAN ;   HSAN (hereditary sensory autonomic neuropathy) ;   congenital sensory neuropathies ;   hereditary sensory and autonomic neuropathies ;   hereditary sensory neuropathies ;   hereditary sensory radicular neuropathy ;   hereditary sensory radicular neuropathy, autosomal dominant ;   hereditary sensory radicular neuropathy, autosomal recessive ;   hereditary sensory radicular neuropathy, recessive form
Narrow Synonyms: AMS ;   acral mutilation syndrome
Primary IDs: MESH:D009477
Alternate IDs: OMIA:001514 ;   RDO:0000851
Xrefs: OMIM:PS162400
Definition Sources: MESH:D009477, http://omim.org/entry/616488?search=616488&highlight=616488

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.