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Sandhoff Disease
Definition: An autosomal recessive neurodegenerative disorder characterized by an accumulation of G(M2) GANGLIOSIDE in neurons and other tissues. It is caused by mutation in the common beta subunit of HEXOSAMINIDASE A and HEXOSAMINIDASE A. Thus this disease is also known as the O variant since both hexosaminidase A and B are missing. Clinically, it is indistinguishable from TAY-SACHS DISEASE.

Synonym(s): G(M2) Gangliosidosis, Type II / Gangliosidosis G(M2), Type II / Hexosaminidase A and B Deficiency Disease / Adult Sandhoff Disease / Deficiency Disease, Hexosaminidase A and B / GM2 Gangliosidosis, Type II / Gangliosidosis GM2, Type II / Infantile Sandhoff Disease / Juvenile Sandhoff Disease / Sandhoff Disease, Adult / Sandhoff Disease, Infantile / Sandhoff Disease, Juvenile / Sandhoff's Disease / Sandhoffs Disease
See also:
beta-N-Acetylhexosaminidase  G(M2) Ganglioside   

MeSH 2010 © U.S. National Library of Medicine®.
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  More information from the Genetics Home Reference Website
What is Sandhoff disease?
Sandhoff disease is a rare inherited disorder that causes progressive destruction of nerve cells in the brain and spinal cord (the central nervous system).
The most common and severe form of Sandhoff disease begins in infancy. Infants with this disorder typically appear normal until the age of 3 to 6 months, when development slows and muscles used for movement weaken. Affected infants lose motor skills such as turning over, sitting, and crawling. They also develop an exaggerated startle reaction to loud noises. As the disease progresses, children with Sandhoff d ...

How common is Sandhoff disease?
Sandhoff disease is a rare disorder; its frequency varies among populations. This condition appears to be more common in the Creole population of northern Argentina; the Metis Indians in Saskatchewan, Canada; and people from Lebanon.


More information from the Genetics Home Reference Website
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  Web resources for "Sandhoff Disease"
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view the robot description of the site Sandhoff Disease Symptoms, Diagnosis, Treatments and Causes - RightDiagnosis.comwww.wrongdiagnosis.com  
view the HON description of the site HONcode - Sandhoff disease - Genetics Home Referenceghr.nlm.nih.gov 2
view the robot description of the site Sandhoff Disease Information Page: National Institute of Neurological Disorders and Stroke (NINDS)www.ninds.nih.gov 4
view the HON description of the site HONcode - Orphanet: Sandhoff diseasewww.orpha.net 2
view the robot description of the site rare-diseases — National Organization for Rare Disorderswww.rarediseases.org  
 
 

See also: - beta-N-Acetylhexosaminidase

Broader term(s): - Gangliosidoses, GM2 - Gangliosidoses - Sphingolipidoses - Brain Diseases - Central Nervous System Diseases - Nervous System Diseases
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  Medical image(s) for "Sandhoff Disease"
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Broader term(s): - Brain Diseases, Metabolic - Brain Diseases - Nervous System Diseases
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  Medical News for "Sandhoff Disease"
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Broader term(s): - Nervous System Diseases

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Broader term(s): - Gangliosidoses, GM2 - Gangliosidoses - Sphingolipidoses - Brain Diseases - Central Nervous System Diseases - Nervous System Diseases

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  Broader term(s):
      Diseases
          Nutritional and Metabolic Diseases
              Metabolic Diseases
                  Metabolism, Inborn Errors
                      Lysosomal Storage Diseases
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
                      Lipid Metabolism, Inborn Errors
                          Lipidoses
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses 
      Diseases
          Nutritional and Metabolic Diseases
              Metabolic Diseases
                  Metabolism, Inborn Errors
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
                  Lipid Metabolism Disorders
                      Lipidoses
                          Sphingolipidoses
                              Gangliosidoses
                                  Gangliosidoses, GM2 
                  Brain Diseases, Metabolic
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses 
      Diseases
          Nutritional and Metabolic Diseases
              Metabolic Diseases
                  Brain Diseases, Metabolic
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
      Diseases
          Congenital, Hereditary, and Neonatal Diseases and Abnormalities
              Genetic Diseases, Inborn
                  Metabolism, Inborn Errors
                      Lysosomal Storage Diseases
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
                      Lipid Metabolism, Inborn Errors
                          Lipidoses
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses 
      Diseases
          Congenital, Hereditary, and Neonatal Diseases and Abnormalities
              Genetic Diseases, Inborn
                  Metabolism, Inborn Errors
                      Brain Diseases, Metabolic, Inborn
                          Lysosomal Storage Diseases, Nervous System
                              Sphingolipidoses
                                  Gangliosidoses
                                      Gangliosidoses, GM2 
      Diseases
          Nervous System Diseases
              Central Nervous System Diseases
                  Brain Diseases
                      Brain Diseases, Metabolic
                          Brain Diseases, Metabolic, Inborn
                              Lysosomal Storage Diseases, Nervous System
                                  Sphingolipidoses
                                      Gangliosidoses 
                                          Gangliosidoses, GM2 

MeSH 2010 © U.S. National Library of Medicine®.

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