Batten disease

Batten disease
Bat·ten disease 'bat-ən- n a fatal lipofuscinosis that is inherited as an autosomal recessive trait, has an onset between five and eight or nine years of age, and is marked by early symptoms which progress to blindness, paralysis, and dementia
Batten Frederick Eustace (1865-1918)
British neurologist and pediatrician. In London, Batten held posts at the Hospital for Sick Children and at the National Hospital for the Paralysed and Epileptic. His research centered on the histology of disorders of the nervous system. In 1902 he published the first description of a fatal form of lipofuscinosis that is often known as Batten disease. Additional descriptions of the disease were separately published by the German neurologists Heinrich Vogt in 1905 and Walter Spielmeyer in 1908. Batten is also remembered for his 1903 description of a benign form of congenital muscular dystrophy. The author of more than 100 medical papers, he published a book on acute poliomyelitis in 1916.

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Bat·ten disease (batґən) [Frederick Eustace Batten, English ophthalmologist, 1865–1918] see under disease.

Medical dictionary. 2011.

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  • Batten disease — Infobox Disease Name = Batten disease Caption = DiseasesDB = 31534 ICD10 = ICD10|E|75|4|e|70 ICD9 = ICD9|330.1 ICDO = OMIM = 204200 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D009472 Batten disease is a rare, fatal, autosomal… …   Wikipedia

  • batten disease — n. (Medicine) congenital disorder that attacks between the ages of 5 and 10 and causes the infected child to unexpectedly lose vision …   English contemporary dictionary

  • Batten-Mayou disease — Bat·ten Ma·you disease (batґən ma yooґ) [F.E. Batten; Marmaduke Stephen Mayou, English ophthalmologist, 1876–1934] Batten disease; see under disease …   Medical dictionary

  • Batten (disambiguation) — A batten is a piece of construction material.Batten may also refer to:* Batten (car), a British car produced in the late 1930s * Batten (theater), a pole suspended above a stage, from which lights, props or curtains may be hung * Mount Batten, a… …   Wikipedia

  • Batten's disease — /batˈənz diz ēzˈ/ noun A severe progressive genetic disorder which causes blindness, deafness, loss of muscle control and early death ORIGIN: FE Batten (1865–1918), British doctor …   Useful english dictionary

  • Disease — Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the… …   Medical dictionary

  • Batten's disease — one of a group of rare hereditary disorders (known as the neuronal ceroid lipofuscinoses) that also includes Tay Sachs disease. Fatty substances accumulate in the cells of the nervous system, causing progressive dementia, epilepsy, spasticity,… …   Medical dictionary

  • Batten's disease — one of a group of rare hereditary disorders (known as the neuronal ceroid lipofuscinoses) that also includes Tay Sachs disease. Fatty substances accumulate in the cells of the nervous system, causing progressive dementia, epilepsy, spasticity,… …   The new mediacal dictionary

  • Batten — Frederick E., British ophthalmologist, 1865–1918. See B. Mayou disease, B. disease …   Medical dictionary

  • Enfermedad de Batten — Herencia mendeliana autosómica recesiva: dos mutaciones de línea germinal (una de cada uno de los padres) para desarrollar la enfermedad; igualmente transmitida por hombres y mujeres …   Wikipedia Español

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