Results 181 to 190 of about 9,571 (221)

MRI in neuronal ceroid lipofuscinosis

Neurological Sciences, 2000
Magnetic resonance imaging in neuronal ceroid lipofuscinosis (NCL) demonstrates cerebral and cerebellar atrophy, T2-hyperintensity of the lobar white matter and thinning of the cerebral cortex. The association of these findings, although non specific, can be observed in all the different forms of NCL, narrows the differential diagnosis of the infantile
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Neuronal ceroid‐lipofuscinosis

Neurology, 1980
We studied the ultrastructural alterations of the dense bodies of peripheral blood lymphocytes in one case of neuronal ceroid-lipofuscinosis. These cytoplasmic vesicles were characterized by vacuolar transformation and "fingerprint" content. When we tilted the specimens, the "fingerprint" arrangement was shown to be the same as the arrangement of the ...
A P, Aguas, J F, Nunes, A D, Ferreira, J P, Vital   +3 more
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Adult Type of Neuronal Ceroid Lipofuscinosis

Developmental Neuroscience, 1991
Adult neuronal ceroid lipofuscinosis (NCL), also called Kufs' disease, is clinically distinct from the other NCLs. It is a rare condition which is difficult to diagnose. More than 50% of the reported cases of Kufs' disease are not adult NCL and correspond very likely to a heterogeneous spectrum of lipidoses.
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Neuronal ceroid-lipofuscinosis and metachromasia

Acta Neuropathologica, 1980
Two cases of neuronal ceroid-lipofuscinosis are described. They presented in the white matter granular material which stained metachromatically with toluidine blue in paraffin sections. The origin of this material is discussed.
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Neuronal ceroid-lipofuscinosis in Borderdale sheep

New Zealand Veterinary Journal, 2002
To describe the gross and histological lesions of a neurological disease in Borderdale sheep characterised clinically by blindness and circling, as a basis to its classification.Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and electron microscopy of variously stained sections.Lesions were those of a lysosomal ...
R D, Jolly, D G, Arthur, G W, Kay, D N, Palmer   +3 more
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Neuronal Ceroid Lipofuscinosis With Hypergonadotropic Hypogonadism

Journal of Child Neurology, 1986
A case of adolescent-onset neuronal ceroid lipofuscinosis presenting with chorea and evidencing pyramidal and cerebellar dysfunction as well as hypergonadotropic hypogonadism is reported. In this patient, primary ovarian failure may be due to accumulation of ceroid in the ovaries. ( J Child Neurol 1986; 1:142-144)
R, Nass, C, Petito, E, Stoner, M, New
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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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Adult neuronal ceroid-lipofuscinosis.

Clinical neuropathology, 1989
Among the different clinical forms of neuronal ceroid-lipofuscinosis (NCL), the adult type is the least frequent, most sporadic and most difficult one to diagnose. Clinical symptomatology differs from the classical childhood NCL forms in that ocular symptoms are absent while changes of behavior, dementia and seizures dominate the clinical picture ...
H H, Goebel, H, Braak
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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, J Peña, G Luzardo, O Méndez, H Velásquez   +4 more
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