Kufs disease

Kufs dis·ease

(kūfs),

Kufs disease

(kŭfs, ko͝ofs)
n.
A type of neuronal ceroid lipofuscinosis with onset usually during adulthood.

Kufs disease

(kufs)
[H. Kufs, Ger. psychiatrist, 1871–1955]
An adult-onset neuronal ceroid lipofuscinosis. The onset of symptoms is usually between 21 and 26 years and always before 40. The disease is characterized by the development of dementia, myoclonic jerks, blindness, and retinitis pigmentosa.

Kufs,

Hugo Friedrich, German neuropathologist, 1871-1955.
Kufs disease - cerebral sphingolipidosis, adult type.
References in periodicals archive ?
The are four other main variants of NCLs, including congenital NCL, infantile NCL (INCL or Santavuori-Haltia disease), late infantile NCL (LINCL or Jansky-Bielschowsky disease) and adult NCL (ANCL, Kufs disease or Parry's disease). 
Adult-onset NCL (CLN4) follows either an autosomal recessive (Kufs disease) or an autosomal dominant (Parry disease) pattern of inheritance and is likely to be linked to different, as yet unknown, gene loci.
The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative diseases that include infantile NCL (INCL; OMIM 256730, where the defective gene is CLN1), classical late infantile NCL (LINCL; OMIM 204500, where the defective gene is CLN2), two variant late infantile NCLs (OMIM 256731, where the defective gene is CLN5; and OMIM 601780, where the defective gene is CLN6), juvenile NCL (JNCL; OMIM 204200; defective gene, CLN3), a juvenile onset epilepsy with progressive mental retardation (EPMR; OMIM 600143; defective gene, CLN8), and adult NCL (Kufs disease; OMIM 204300; defective gene, CLN4) [reviewed in Ref.