Svensk definition
En grupp ärftliga sjukdomar, kännetecknade av nedbrytning av dorsala nervrötter och autonoma ganglieceller, samt kliniskt av känselbortfall och bristande autonoma funktioner. Det finns fem typer. Typ I uppvisar autosomalt dominant ärftlighet och distal känselpåverkan. Typ II är autosomal, med både distalt och proximalt känselbortfall. Typ III är familiär dysautonomi. Typ IV har drag av smärtokänslighet, värmeintolerans och psykisk störning. Typ V kännetecknas av selektivt bortfall av smärtförnimmelse, men med bibehållen berörings- och vibrationskänsla.
Engelsk definition
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)
Svenska synonymer
Ärflig sensorisk och autonom neuropati typ V — Ärftlig smärtokänslighet — Vittangisjukan — Norrbottnisk ärftlig smärtokänslighet — Ärflig sensorisk neuropati typ I — Familjär dysautonomi typ II — Ärflig sensorisk neuropati — Neurogen akroosteolys — Ärflig sensorisk och autonom neuropati typ II
Engelska synonymer
HSAN (Hereditary Sensory Autonomic Neuropathy) — HSANs (Hereditary Sensory Autonomic Neuropathy) — Neuropathies, Hereditary Sensory and Autonomic — HSAN — Sensory and Autonomic Neuropathies, Hereditary — Hereditary Sensory and Autonomic Neuropathy Type V — Hereditary Sensory Autonomic Neuropathy, Type 5 — Neuropathy, Hereditary Sensory And Autonomic, Type V — HSAN5 — HSAN 5 — Hereditary Sensory and Autonomic Neuropathy, Type 5 — HSAN Type V — HSAN V — Hereditary Sensory Neuropathy Type I — Hereditary Sensory and Autonomic Neuropathy Type I — Hereditary Sensory Autonomic Neuropathy, Type 1 — Neuropathy Hereditary Sensory and Autonomic Type 1 — Hereditary Sensory Neuropathy Type 1 — HSAN 1 — Neuropathy Hereditary Sensory Radicular, Autosomal Dominant — Neuropathy, Hereditary Sensory And Autonomic, Type I — Neuropathy, Hereditary Sensory, Type I — Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant — HSAN I — Hereditary Sensory Neuropathy Type Ia — Hereditary Sensory and Autonomic Neuropathy Type 1 — HSAN Type I — Type I, HSAN — HSN Type I — Type I, HSN — HSAN Type IV — Type IV, HSAN — Pain Insensitivity with Anhidrosis, Congenital — Congenital Insensitivity to Pain with Anhidrosis — Hereditary Sensory Autonomic Neuropathy, Type 4 — HSAN 4 — Insensitivity to Pain, Congenital, with Anhidrosis — Hereditary Sensory and Autonomic Neuropathy 4 — Neuropathy, Congenital Sensory, with Anhidrosis — Familial Dysautonomia, Type 2 — HSAN IV — Familial Dysautonomia, Type II — Hereditary Sensory And Autonomic Neuropathy IV — Hereditary Sensory and Autonomic Neuropathy, Type 4 — Hereditary Sensory and Autonomic Neuropathy Type IV — Insensitivity to Pain with Anhidrosis, Congenital — Sensory Neuropathy, Hereditary — Hereditary Sensory Neuropathies — Hereditary Sensory Neuropathy — Neuropathies, Hereditary Sensory — Neuropathy, Hereditary Sensory — Sensory Neuropathies, Hereditary — Neurogenic Acroosteolysis — Acroosteolysis, Neurogenic — Acroosteolyses, Neurogenic — Neurogenic Acroosteolyses — Neuropathy, Congenital Sensory — Congenital Sensory Neuropathies — Congenital Sensory Neuropathy — Neuropathies, Congenital Sensory — Sensory Neuropathies, Congenital — Sensory Neuropathy, Congenital — Hereditary Sensory and Autonomic Neuropathy Type II — HSN Type II — HSN Type IIs — Hereditary Sensory Autonomic Neuropathy, Type 2 — Giaccai Type Acroosteolysis — Hereditary Sensory Radicular Neuropathy, Recessive Form — HSAN2 — Neuropathy, Hereditary Sensory Radicular, Autosomal Recessive — Acroosteolysis, Giaccai Type — Neuropathy, Progressive Sensory, Of Children — HSANII — HSAN Type II — Hereditary Sensory and Autonomic Neuropathy Type 2 — Hereditary Sensory Radicular Neuropathy