PT  - JOURNAL ARTICLE
AU  - D J Spalton
AU  - D S Taylor
AU  - M D Sanders
TI  - Juvenile Batten's disease: an ophthalmological assessment of 26 patients.
AID  - 10.1136/bjo.64.10.726
DP  - 1980 Oct 01
TA  - British Journal of Ophthalmology
PG  - 726--732
VI  - 64
IP  - 10
4099  - http://bjo.bmj.com/content/64/10/726.short
4100  - http://bjo.bmj.com/content/64/10/726.full
SO  - Br J Ophthalmol1980 Oct 01; 64
AB  - Twenty-six children with juvenile Batten's disease are reviewed. On clinical and histological evidence they appear to represent a specific disease entity, which though rare is a substantial cause of blindness in children aged 5--15 years. Children present with rapid progressive visual loss at age 6--7 years, early mental deterioration, and fits about 2--4 years later, and this is the stage at which the diagnosis is usually made. Macular degeneration appears to be a consistent early feature, and peripheral retinal changes become more marked as the disease progresses. Phototoxicity may possibly play a part in the retinal degeneration.