Jansky-Bielschowsky disease

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any disorder due to abnormal storage of lipofuscins.
neuronal ceroid-lipofuscinosis any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are janský-bielschowsky disease, vogt-spielmeyer disease, and kufs' disease. Formerly known as amaurotic familial idiocy.

Jan·sky-Bi·els·chow·sky dis·ease

(yahn'skē by'els-chov'skē),
cerebral sphingolipidosis, early juvenile type.

Jansky-Bielschowsky disease

(jăn′skē-bē′ĕl-shō′skē, -bēl-shôv′-, yän′-)
A type of neuronal ceroid lipofuscinosis with onset usually between ages two and four.

Jansky-Bielschowsky disease

[yahn′skē byelschov′ske]
the late infantile form of neuronal ceroid lipofuscinosis, occurring between 2 and 4 years of age and characterized by abnormal accumulation of lipofuscin. It begins as myoclonic seizures and progresses to neurological and retinal degeneration and death, usually by the age of 8 to 12 years.


Max, German neuropathologist, 1869-1940.
Bielschowsky-Dollinger syndrome - Synonym(s): Dollinger-Bielschowsky syndrome
Bielschowsky head tilt test
Bielschowsky-Jansky disease - Synonym(s): Jansky-Bielschowsky disease
Bielschowsky method
Bielschowsky stain - a method of treating tissues with silver nitrate to demonstrate reticular fibers, neurofibrils, axons, and dendrites.
Bielschowsky syndrome - Synonym(s): Dollinger-Bielschowsky syndrome
Dollinger-Bielschowsky syndrome - see under Dollinger
Jansky-Bielschowsky disease - see under Jansky


Jan, Czech physician, 1873-1921.
Bielschowsky-Jansky disease - Synonym(s): Jansky-Bielschowsky disease
Jansky classification - the classification of human blood groups now designated O, A, B, and AB.
Jansky-Bielschowsky disease - cerebral sphingolipidosis, early juvenile type. Synonym(s): Bielschowsky-Jansky disease