Results 181 to 190 of about 6,644 (221)
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Canine ceroid-lipofuscinosis — A model for human neuronal ceroid-lipofuscinosis and aging

Mechanisms of Ageing and Development, 1973
Abstract A controlled longitudinal morphologic study revealed that the formation and accumulation of an autofluorescent lipopigment, identified as “ceroid” in the isolated state, commences in utero and progresses linearly with time. Only after a distinct loss of neurocytoplasm and its functional organelles is demonstrable, do clinical signs and ...
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Neuronal ceroid‐lipofuscinosis and hydrocephalus in a chihuahua

Journal of Small Animal Practice, 2003
A two‐year‐old, female chihuahua presented with a six‐month history of visual dysfunction. Computed tomography revealed dilation of the lateral ventricles in the central nervous system (CNS). The dog was tentatively diagnosed as having hydrocephalus and a month later was euthanased at the owner's request.
M, Kuwamura   +4 more
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Evoked Potentials in Neuronal Ceroid Lipofuscinosis

European Neurology, 2008
Nerve conduction, EEG, visual evoked potentials, electroretinograms and somatosensory evoked potentials were investigated in 3 children with the Bielschowsky-Jansky-type and in 1 child diagnosed as Spielmeyer-Vogt-type of neuronal ceroid lipofuscinosis. Electroretinograph-ic responses were abolished in all of them.
W, Tackmann, D, Kuhlendahl
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Pigment variant of neuronal ceroid‐lipofuscinosis

American Journal of Medical Genetics, 1995
AbstractA 6‐year‐old girl had progressive ataxia, and visual disturbances resulting in blindness. She died in her sleep at age 22 years. She shared with her sister and paternal relatives bilateral pes cavus deformities and impaired deep‐tendon reflexes which suggested Charcot‐Marie‐Tooth disease.
H H, Goebel   +4 more
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The canine neuronal ceroid-lipofuscinosis: a review.

Schweizer Archiv fur Tierheilkunde, 2014
Die vorliegende Arbeit gibt eine Übersicht und Zusammenfassung über den aktuellen Wissensstand der neuronalen Ceroid-Lipofuszinose (NCL) beim Hund. Die NCL ist eine heterogene Gruppe lysosomaler Speicherkrankheiten bei Mensch und Tier. Aufgrund einer Genmutation kommt es zur Akkumulation von Ceroid-Lipofuszin in Nervenzellen, Zellen der Netzhaut, Haut ...
Karli, Philemon   +5 more
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Morphologic Diagnosis in Neuronal Ceroid Lipofuscinosis

Neuropediatrics, 1997
Morphologic pathology in NCL is marked by two processes, the interaction of which has not yet been completely clarified: 1) degeneration of nerve cells, foremost in the cerebral cortex, resulting in considerable cerebral atrophy in early childhood forms, likely responsible for clinical and neuroradiological findings; 2) widespread accumulation of ...
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Neuronal Ceroid Lipofuscinosis

Ophthalmology, 1984
Guillermo De Venecia, Michael Shapiro
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NEURONAL CEROID LIPOFUSCINOSIS

Journal of Neuropathology and Experimental Neurology, 1998
J Cardozo   +4 more
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Neuronal ceroid lipofuscinosis

Journal of the Neurological Sciences, 1979
Shaila Nadkarni   +3 more
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