gray matter lesions in mitochondrial disorders

[Guest guest]

, I only found bits and pieces on PubMed and various other web

sources, so I have pasted the relevant parts together rather than

paste whole abstracts. Probably not much more than you found. Hope

this helps.

Barbara

Gray matter refers to areas of the brain and spinal cord that

contain mostly neuronal cell bodies. It is called gray matter

because these areas appear dusky gray when examined in dead bodies.

The name is used in conjunction with white matter which refers to

areas of the brain and spinal cord that contain mostly myelinated

axons. In the spinal cord, the gray matter is situated in the

central part of the cord. When the spinal cord is viewed in cross-

section, the gray matter appears to be butterfly-shaped area. The

wings of the butterfly that are towards the back (dorsal or

posterior) are called the dorsal horns. The wings that are towards

the front (ventral or anterior) are called the ventral horns.

Mitochondrial disease is classically associated with deep gray-

matter lesions. One form of mitochondrial disease, Leigh's disease,

affects mainly the deep gray matter. Lesions are uncommon in the

white matter and cerebral cortex. Characteristically, there are

symmetrical lesions in the brain stem and the diencephalon with

frequent involvement of the globus pallidus, spinal cord, optic

nerve and cerebellum. The lesions are gray brown in color.

The lesions in gray matter associated with mitochondrial disease are

described as nonspecific spongiform lesions of gray matter.

Nonspecific simply means this type of lesion may be seen in a

variety of disorders and is not specific to mitochondrial disease.

There are reports of white matter lesions in mitochondrial disease.

One site listed MELAS under disorders that affect both white matter

and gray matter.

In cerebral gray matter, ATP (adenosine triphosphate, which serves

as fuel) concentration is closely maintained despite rapid, large

increases in turnover and low substrate reserves. Brain ATP is

stable early in seizures, a state of high energy demand, and in mild

hypoxia, a state of substrate deficiency. Like other tissues with

high and variable ATP turnover, cerebral gray matter has high

phosphocreatine concentration and both cytosolic and mitochondrial

creatine kinase isoenzymes.

http://www.healingtherapies.info/creatineNeuroProtection.htm

Animal studies suggest that creatine supplementation may aid

recovery in patients with spinal cord injury and may have a

protective effect on injured spinal cord gray matter.

[Guest guest]

I really haven't read much about Leighs except for the general

articles. There was one in an older UMDF newsletter

Barbara

> Barbara,

> thank you so much for the research, I did indeed find similar

articles. One

> thing I wonder about is the articles I read describe mostly LS

having grey

> matter spinal findings. When I have read about my defect (

complex II/III )

> it says it is associated with a leighs like syndrome seen in

adults. Oddly my

> son had a MRS and daughter and they have cell death or neuronal

loss in the

> basal ganglia, putamen and caudate heads bilaterally. This

particular pattern

> I read about in LS.

> Do you know anything about Leigh like syndrome?

> The other thing i read about is that huntigtons is associated with

a II/III

> defect..and has a similar MRS pattern..

>

>

>

>

[Guest guest]

I really haven't read much about Leighs except for the general

articles. There was one in an older UMDF newsletter

Barbara

> Barbara,

> thank you so much for the research, I did indeed find similar

articles. One

> thing I wonder about is the articles I read describe mostly LS

having grey

> matter spinal findings. When I have read about my defect (

complex II/III )

> it says it is associated with a leighs like syndrome seen in

adults. Oddly my

> son had a MRS and daughter and they have cell death or neuronal

loss in the

> basal ganglia, putamen and caudate heads bilaterally. This

particular pattern

> I read about in LS.

> Do you know anything about Leigh like syndrome?

> The other thing i read about is that huntigtons is associated with

a II/III

> defect..and has a similar MRS pattern..

>

>

>

>

[Guest guest]

I really haven't read much about Leighs except for the general

articles. There was one in an older UMDF newsletter

Barbara

> Barbara,

> thank you so much for the research, I did indeed find similar

articles. One

> thing I wonder about is the articles I read describe mostly LS

having grey

> matter spinal findings. When I have read about my defect (

complex II/III )

> it says it is associated with a leighs like syndrome seen in

adults. Oddly my

> son had a MRS and daughter and they have cell death or neuronal

loss in the

> basal ganglia, putamen and caudate heads bilaterally. This

particular pattern

> I read about in LS.

> Do you know anything about Leigh like syndrome?

> The other thing i read about is that huntigtons is associated with

a II/III

> defect..and has a similar MRS pattern..

>

>

>

>