- ►nih.gov J Collins, GE Holder, H Herbert, GGW Adams - British Medical Journal, 2006 - bjo.bmj.com Results: Age at onset ranged from 4–8 years. At presentation, two of nine
patients had normal fundi; only two of nine patients had a bull's eye
maculopathy. The electroretinogram (ERG) findings in this series included ... Cited by 7 - Related articles - BL Direct - All 6 versions
I Mantel, MA Brantley Jr, C Bellmann, AG … - Klinische MonatsblÃĪtter fÞr Augenheilkunde, 2004 - ncbi.nlm.nih.gov BACKGROUND: The neuronal ceroid lipofuscinoses (Batten disease) are a
heterogeneous group of autosomal recessively inherited disorders causing
progressive neurological failure, mental deterioration, seizures and visual ... Cited by 8 - Related articles - BL Direct - All 2 versions
HR Adams, J Kwon, FJ Marshall, EA de Blieck … - Journal of child neurology, 2007 - jcn.sagepub.com Neurocognitive deficits are prominent in juvenile neu- ronal ceroid
lipofuscinosis but have not been well quanti- fied. In the past 3 decades, there
have been only a few formal studies of cognitive function in juvenile ... Cited by 3 - Related articles - BL Direct - All 3 versions
A Larsen, K Sainio, L berg, P Santavuori - European Journal of Paediatric Neurology, 2001 - Elsevier Fourteen patients with a confirmed diagnosis of juvenile neuronal ceroid
lipofuscinosis (JNCL) (aged 6–12.5 years at the beginning of the study) were
prospectively followed for 5 years. An electroencephalogram (EEG) was ... Cited by 3 - Related articles - BL Direct - All 3 versions
SA Woodruff, S Fraser, LC Burton, GE Holder, … - Eye, 2004 - nature.com Results EDT was found to be of value in 91% of the children tested and was
considered an essential investigation in 71%. EDT made a new diagnosis in 7% of
patients, changed it in 5%, and confirmed or excluded a diagnosis in 79%. ... Cited by 4 - Related articles - BL Direct - All 3 versions
M Seeliger, K RÞther, E Apfelstedt-Sylla, W … - Der Ophthalmologe: Zeitschrift der Deutschen …, 1997 - ncbi.nlm.nih.gov BACKGROUND: Juvenile neuronal ceroid lipofuscinosis (JNCL) is important to the
ophthalmologist, since eye symptoms are usually the first evidence of the
disease and permit establishment of an early diagnosis. The disorder ... Cited by 4 - Related articles
H Adams, EA de Blieck, JW Mink, FJ Marshall … - Developmental Medicine and Child Neurology, 2006 - Cambridge Univ Press We obtained information about the behavioral, psychiatric, and functional status
of 26 children (13 males, 13 females) with juvenile neuronal ceroid
lipofuscinosis (JNCL; mean age 12y 3mo [SD 3y 4mo]; range 6y 9mo to 18y ... Cited by 4 - Related articles - BL Direct - All 5 versions
L Eksandh, V Ponjavic, PB Munroe, H Eiberg, … - Ophthalmic Genetics, 2000 - informahealthcare.com Acknowledgements: We thank Drs Nicholas Greene, Kit- Yi Leung, Hannah Mitchison,
Ing- Marie Holst, and Susanne Boy for their excellent technical assistance. This
study was supported by grants from Synfrämjandet, Åke Wibergs stiftelse, ... Cited by 12 - Related articles - BL Direct - All 5 versions
JM Kwon, PG Rothberg, AR Leman, JM … - Neuroscience letters, 2005 - Elsevier Juvenile Neuronal Ceroid Lipofuscinosis (JNCL), or Batten disease, is a
childhood neurodegenerative disease that is characterized clinically by
progressive visual loss, seizures, dementia, and motor incoordination. ... Cited by 6 - Related articles - All 3 versions
ML Bäckman, LE berg, ET Aronen, PR … - European Journal of Paediatric Neurology, 2001 - Elsevier Patients with juvenile neuronal ceroid lipofuscinosis (JNCL) often have severe
psychiatric symptoms. These are common in their mid-teens and include such
symptoms as anxiety and affective and psychotic disorders. The older ... Cited by 6 - Related articles - BL Direct - All 3 versions
