leukodystrophy, Globoid cell
Definition:

Globoid cell leukodystrophy, or Krabbe disease, is a rare, fatal degenerative disease that destroys the myelin content of the nervous system.


Diagnosis:

Globoid cell leukodystrophy can be diagnosed through nerve demyelination, destruction of brain cells and a characteristic grouping of multinucleated globoid cells.


Treatment:

There is no exact cure for the disease, but bone marrow transplant has shown potential effects that could lead to recovery, although this has only been tried on mild cases.


Symptoms and Signs:

Persons affected with Globoid cell leukodystrophy begin to experience symptoms at age three. They start to suffer from consistent irritabitlity, limb stiffness, eating difficulties, vomiting, as well as slow mental and motor development. Others meanwhile undergo lethargy, spasticity, optic atrophy, deafness and eventual paralysis.


Causes:

Globoid cell leukodystrophy is an autosomal recessive genetic disorder.


:

leukodystrophygloboidcell



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