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9 thoughts on “ Spielmeyer Vogt Disease

  1. Batten disease, also called Spielmeyer-Vogt-Sjogren-Batten disease or juvenile Batten disease, rare and fatal neurodegenerative disease that begins in childhood. The disease is named for British physician Frederick Batten, who in described the cerebral degeneration and macular changes characteristic of the condition.
  2. Also known as Spielmeyer-Vogt-Sjogren-Batten disease, Batten disease is the most common form of a group of disorders called neuronal ceroid lipofuscinoses (or NCLs). Although Batten disease is usually regarded as the juvenile form of NCL, it has now become the term which encompasses all forms of NCL.
  3. Overview. Batten disease is a rare, fatal autosomal recessive neurodegenerative disorder that begins in childhood. Also known as Spielmeyer-Vogt-Sjögren-Batten disease, it is the most common form of a group of disorders called neuronal ceroid lipofuscinosis (or NCLs).
  4. Spielmeyer, Walter, German neurologist, Spielmeyer acute swelling - a form of degeneration of nerve cells in which the cell body and its processes swell and stain palely and diffusely. Spielmeyer-Stock disease - Synonym (s): Batten disease. Spielmeyer-Vogt disease - Synonym (s): Batten disease.
  5. Batten disease. Batten disease. [ bat´en ] Vogt-Spielmeyer disease. more generally, any or all of the group of disorders constituting neuronal ceroid Bat·ten dis·ease. Batten disease. Batten disease.
  6. Spielmeyer-Vogt disease - a congenital progressive disorder of lipid metabolism having an onset at age 5 and characterized by blindness and dementia and early death. juvenile amaurotic idiocy.
  7. Spielmeyer-Vogt disease: A congenital progressive lysosome dysfunction that usually presents itself around the age of five, and is characterized by cerebroretinal degeneration, dementia and early death. Named along with neurologist Heinrich Vogt ().
  8. Spielmeyer-Vogt kendes også under den medicinske betegnelse Neuronal Ceroid Lipofuscinoser og internationalt som Batten Disease. I Danmark forekommer den med en hyppighed på , og i år () er der på nationalt plan omkring 30 diagnosticerede børn og unge.
  9. The neuronal ceroid lipofuscinoses (NCLs) are a group of genetic neurodegenerative disorders of the childhood in which there is excessive accumulation of lipofuscin.. The group consists of 1. type 1: Santavuori-Haltia disease type 2: Jansky-Bielschowsky disease type 3: juvenile, Batten-Spielmeyer-Vogt disease type 4: Kufs disease type 5: Finnish variant late infantile.

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