PEDIATRICS Vol. 44 No. 4 October 1969, pp. 570-583
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Zeman, W.
Right arrow Articles by Dyken, P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Zeman, W.
Right arrow Articles by Dyken, P.

NEURONAL CEROID-LIPOFUSCINOSIS (BATTEN'S DISEASE): RELATIONSHIP TO AMAUROTIC FAMILY IDIOCY?

Wolfgang Zeman M.D.1 and Paul Dyken M.D.1

1 Division of Neuropathology and the Section of Child Neurology, Indiana University Medical Center, Indianapolis

From the conditions generically classified as amaurotic familial idiocy, two distinctly different groups have emerged. One is characterized by grossly abnormal profiles for cerebral sphingolipids and consists of GM1-gangliosidosis and GM2-gangliosidosis or Tay-Sachs disease. The other group is composed of cases with normal sphingolipid profiles, but with neuronal accumulation of lipopigments of the ceroid/lipofuscin type, designated as neuronal ceroid-lipofuscinosis (NCL) or Batten's disease. Clinical data on 26 patients with this disorder reveal the course and symptomatology to be variable, but pathomorphologic and biochemical findings are reasonably constant. The course of the disorder is directly influenced by the presence or absence of intractable seizures.

Submitted on November 22, 1968
Accepted on June 1, 1969




This article has been cited by other articles:


Home page
 NeurologyHome page
R.-M. Boustany, J. W. Britton, J. E. Parisi, and B. M. Keegan
A 23-year-old man with seizures and visual deficit
Neurology, January 1, 2008; 70(1): 73 - 78.
[Full Text] [PDF]

Home page
 J Child NeurolHome page
R. Koul, A. Al-Futaisi, A. Ganesh, and S. Rangnath Bushnarmuth
Late-Infantile Neuronal Ceroid Lipofuscinosis (CLN2/Jansky-Bielschowsky Type) in Oman
J Child Neurol, May 1, 2007; 22(5): 555 - 559.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
E. de los Reyes, P. R. Dyken, P. Phillips, M. Brodsky, S. Bates, C. Glasier, and R. E. Mrak
Profound Infantile Neuroretinal Dysfunction in a Heterozygote for the CLN3 Genetic Defect
J Child Neurol, January 1, 2004; 19(1): 42 - 46.
[Abstract] [PDF]

Home page
 Hum Mol GenetHome page
J. Isosomppi, J. Vesa, A. Jalanko, and L. Peltonen
Lysosomal localization of the neuronal ceroid lipofuscinosis CLN5 protein
Hum. Mol. Genet., April 15, 2002; 11(8): 885 - 891.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
K. E. Wisniewski, N. Zhong, and M. Philippart
Pheno/genotypic correlations of neuronal ceroid lipofuscinoses
Neurology, August 28, 2001; 57(4): 576 - 581.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
Y. Kurachi, A. Oka, M. Mizuguchi, Y. Ohkoshi, M. Sasaki, M. Itoh, M. Hayashi, Y. Goto, and S. Takashima
Rapid immunologic diagnosis of classic late infantile neuronal ceroid lipofuscinosis
Neurology, April 25, 2000; 54(8): 1676 - 1680.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
H. H. Goebel
Topical Review: The Neuronal Ceroid-Lipofuscinoses
J Child Neurol, November 1, 1995; 10(6): 424 - 437.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
N. Fueki, K. Iinuma, A. Kojima, K. Yanai, K. Haginoya, K. Tada, T. Ido, and M. Ito
Reduced Regional Cerebral Metabolic Rate for Glucose at the Terminal Stage in a Case of Late Infantile Neuronal Ceroid Lipofuscinosis
J Child Neurol, April 1, 1990; 5(2): 98 - 100.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
P. R. Dyken
Review Article: The Neuronal Ceroid Lipofuscinoses
J Child Neurol, July 1, 1989; 4(3): 165 - 174.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
K.E. Wisniewski, R.E. Madrid, M. Dambska, I. Rapin, R. Pullarkat, and S. Sklower
Spino-Cerebellar Degeneration With Polyneuropathy Associated With Ceroid Lipofuscinosis in One Family
J Child Neurol, January 1, 1988; 3(1): 33 - 41.
[Abstract] [PDF]