A rare fatal autosomal recessive neurodegenerative disorder that begins in childhood, it is caused by mutation of the CLN3 gene.
Early symptoms of the disorder usually appear around ages 4-10, with gradual onset of vision problems, or seizures. Early signs may be subtle personality and behavior changes, slow learning or regression, repetitive speech or echolalia, clumsiness, or stumbling. There may be slowing head growth in the infantile form, poor circulation in lower extremities (legs and feet), decreased body fat and muscle mass, curvature of the spine, hyperventilation and/or breath-holding spells, teeth grinding, and constipation.
Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight, speech and motor skills. Eventually, children with Batten disease become blind, bedridden, and demented. Batten Disease is a life limiting disease, life expectancy varies depending on the type or variation.
It normally affects very young children of ages like 4-6, but has been known to affect older people as well, Kufs affects adults and symptoms normally start in the 30's.
The prognosis is about 5-7 years and there is currently no cure.
Early symptoms of the disorder usually appear around ages 4-10, with gradual onset of vision problems, or seizures. Early signs may be subtle personality and behavior changes, slow learning or regression, repetitive speech or echolalia, clumsiness, or stumbling. There may be slowing head growth in the infantile form, poor circulation in lower extremities (legs and feet), decreased body fat and muscle mass, curvature of the spine, hyperventilation and/or breath-holding spells, teeth grinding, and constipation.
Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight, speech and motor skills. Eventually, children with Batten disease become blind, bedridden, and demented. Batten Disease is a life limiting disease, life expectancy varies depending on the type or variation.
It normally affects very young children of ages like 4-6, but has been known to affect older people as well, Kufs affects adults and symptoms normally start in the 30's.
The prognosis is about 5-7 years and there is currently no cure.
Person 1: My son was just diagnosed with Batten Disease, the doctors say he has 5-7 years left and his condition will rapidly deteriorate.
Person 2: I'm so sorry, I can't imagine how horrible it would be for your child to have that disease.
Person 2: I'm so sorry, I can't imagine how horrible it would be for your child to have that disease.
by Ze Anonymous October 24, 2009
Get the Batten Diseasemug. by Hhjj June 30, 2014
Get the daniel battenmug. Nautical: secure a boat's hatch-tarpaulins, esp. when rough weather is expected.
Figurative: prepare for a difficult time or crisis, trouble is coming.
Figurative: prepare for a difficult time or crisis, trouble is coming.
by uvu November 19, 2007
Get the batten down the hatchesmug. by Spaghetti And Pete-balls April 23, 2020
Get the Pete Slam Battenmug. a) (original meaning) : among fishermen in North America: on a vessel, especially vessels dedicated to winter time lobster and crab fishing, to close all entrances to the lower decks and crew cabins when you are in a storm
b) (my new and improved meaning): in Atlantic Canada and New England, a term meaning, in cities such as New York, Boston, Montreal, Halifax, to insulate the doors and windows of one's house for the winter months.
b) (my new and improved meaning): in Atlantic Canada and New England, a term meaning, in cities such as New York, Boston, Montreal, Halifax, to insulate the doors and windows of one's house for the winter months.
if you want to properly hibernate, be prepared to batten the hatches definition b), even if it costs a small fortune.
by Sexydimma December 9, 2014
Get the batten the hatchesmug. I Battened that girl last night.
by M@felock October 31, 2008
Get the Battenedmug. Harry’s loves a good sesh, but the one thing he loves more than a general sesh…. Is a sesh that lasts for days and days😏
Harry will teach you lessons that no one else can. He’s always there for you when you need him and he’ll forever have your back…. Even if you piss him off. You can’t count on Harry Batten
Harry will teach you lessons that no one else can. He’s always there for you when you need him and he’ll forever have your back…. Even if you piss him off. You can’t count on Harry Batten
by Sesh ‘Ed Luke November 23, 2021
Get the Harry Battenmug.