$Unique_ID{BRK00988}
$Pretitle{Ill Defined Symptoms and Rare Diseases}
$Title{What is Leigh's Disease?}
$Subject{Leigh's diagnosis}
$Volume{N-1}
$Log{}

Copyright (c) 1991   Tribune Media Services, Inc.


What is Leigh's Disease?


------------------------------------------------------------------------------

QUESTION:  Please will you tell us about Leigh's Disease?  All the information
we have obtained from our pediatrician and metabolic doctor is very sketchy.
The patient is 4 years old.  Thanks for any information.

------------------------------------------------------------------------------

ANSWER:  Little wonder your doctors have had little to tell you, for
information about this rare disease is both scarce and not very promising.
Also called "Subacute necrotizing encephalopathy" it was first described by
Dr. D. Leigh in 1951, and is named for him.  It affects infants of both sexes
equally, and its cause is unknown, although it probably is inherited in some
fashion, under the influence of several genes.  Various portions of the brain
gradually die, seemingly without reason, and with their destruction, body and
muscle functions are also gradually lost, until a terminal stupor or coma
occurs.  The evaluation depends upon the physical examination, for there are
no specific tests that prove the diagnosis, except that computerized axial
tomography (CT scan) can show changes occurring in the shape of the brain.  No
known treatment exist except supportive care, and unfortunately death occurs
within a few years.  A few children have lived to their mid teens, but they
too fail to develop normally or thrive.

----------------

The material contained here is "FOR INFORMATION ONLY" and should not replace 
the counsel and advice of your personal physician.  Promptly consulting your 
doctor is the best path to a quick and successful resolution of any medical 
problem.
