The Full Wiki

More info on Batten mayou

Batten mayou: Wikis

Advertisements

Note: Many of our articles have direct quotes from sources you can cite, within the Wikipedia article! This article doesn't yet, but we're working on it! See more info or our list of citable articles.

Encyclopedia

From Wikipedia, the free encyclopedia

Maculocerebral familial degeneration or Batten Mayou syndrome is an autosomal recessive condition which commences in the first decade of life. Defective vision with a central scotoma is accompanied by a weak intellect which is followed by spasticity and convulsions.

Ophthalmoscopic findings

The discs are pale and vessels small. Macula shows yellowish grey spots and granular pigmentation. Similar changes present in other parts of the retina are referred to as "salt and pepper" appearance.

Other features

1.Lymphocytes in peripheral blood are vacuolated(30%) 2. Intracellular deposition of autofluorescent material-persumably lipofuschin occurs throughout the body.

Diagnosis & Treatment

There is no method of prenatal diagnosis Currently, there is no satisfactory treatment.

Outcome

Invariably fatal.


Advertisements






Got something to say? Make a comment.
Your name
Your email address
Message