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Can I read Disease-specific electrophysiological findings in adult ceroid-lipofuscinosis (Kufs disease) on EtoBox?
Disease-specific electrophysiological findings in adult ceroid-lipofuscinosis (Kufs disease) by William W. Dawson; Donald Armstrong; Melvin Greer; Tom M. Maida; Don A. Samuelson is a Medicine article available to read on EtoBox.
What is Disease-specific electrophysiological findings in adult ceroid-lipofuscinosis (Kufs disease) about?
Two adults with mild dementia and a history of memory loss and disequilibrium were seen in the eye clinic following complaints of acuity loss in the 20/30-20/70 (Snellen) range. Results from the fundus examination of one patient were entirely normal; the other showed minimal vascular attenuation and optic atrophy. Electrophysiology was remarkable: (1) Photopic ERG b-waves were reduced, delayed, and showed pronounced oscillations. ( 2) EOG 'light-rise' potentials were absent or very small. (3) Binocular pattern-VER signals showed addition of the monocular signal. Scotopic ERG signals were normal. Brain biopsy and microscopy showed intercellular, autofluorescent ceroid deposits which provided a clear diagnosis of Kufs disease. Histology of model animal retinal cells show ceroid deposits in cell classes implicated by the human retinal signals. The cluster of electrophysiological results point toward early changes in the pigment epithelium and inner plexfform layer cells as a means of noninvasive diagnosis.
Who reads Disease-specific electrophysiological findings in adult ceroid-lipofuscinosis (Kufs disease)?
It is typically read by researchers, students, and practitioners in Medicine.
- Author
- William W. Dawson; Donald Armstrong; Melvin Greer; Tom M. Maida; Don A. Samuelson
- Publisher
- Springer-Verlag; Kluwer Academic Publishers; Springer Science and Business Media LLC (ISSN 0012-4486)
- Published
- 1985
- Language
- EN
- Field
- Medicine (Health Sciences)