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Devic's syndrome

NMF neil.fournier at sympatico.ca
Thu Feb 5 22:23:46 EST 2004

Dear Karla,

I am not all that familiar with this disorder but I'll tell you what I do

Generally the disorder is associated with a sudden onset of blindness in
either one eye or both, followed by a transverse or ascending myelitsis
(basically inflammation of the spinal cord).  So I am assuming that they
have ruled out vascular peculiarities (often early symptomotology is very
similar to vascular disruption to specific branches of the anterior cerebral
artery).   There is also condition that exists which is form of subacute
necrotic myelopathy, which has the same pathological features of Devic
disease but lack the optic neuritis or inflammation.  So I am also assuming
that its true Devic Disease and not this alternative condition.

Many believe that Devic's disease (also called neuromyelitis optica) may be
a variant form of multiple sclerosis.  However, that connection I believe is
commonly debated among neurologists in that field. This is because of some
major of the differences presented in clinical and pathological features of
Devic's disease compared to MS.  So it seems that in some cases MS and
Devic's stand apart, especially since often after many years of illness
there is a failure to develop conspicuous demyelinative lesions in the brain
stem, cerebellar, or cerebral white matter in patients with Devic's disease.
(wide-spread demyelination is commonly observed in MS patients who have
suffered from the disorder for many years).
(In case you don't know what demyelination means.  Demyelination is due to
an immune response from the body that causes the attacking or depletion of a
fatty-like sheath that wraps around a neuron's axon. (Axons are long "tube"
like protrusion that carry the nerve signal used to communicate signals to
neighboring neurons. This sheath basically allows nerve-impulses to be
rapidly transmitted. Because the immune system is triggered the myelin
becomes slowly "eaten away" along certain parts of the neuron.  This causes
a significant "slowering" of the nerve impulses and disorganization of the
original nerve signal).

Unfortunately since the process isn't purely demyelinative there is often
necrotizing of the nerve tissue (in the optic nerve and spinal cord). This
may lead to cavitations and the permanent destruction of segments of the
spinal cord and even the optic tract.

I do know of a few cases, mainly in children, where they suffered only a
single episode of the neurological illness.  I do not believe they
understand why this may happen.  But it does at least provide hope that the
condition could be transient and self-correcting.

In terms of treatment, I believe standard therapies are given that resemble
that involved with MS.  But treatments are usually not very successful b/c
of the necrotic component to the disease (i.e. the death of cells).
Probably treatments are high-dose corticosteroids (to cause
immunosuppression) and cyclophosphamide.

Now I don't know if this true but if it is similar to MS, then if a woman is
pregnant the symptoms will actually improve (due to the transient
immunosuppression that accompanies pregnancy).  Also heat will exacerbate
the symptoms (at least the demyelinating features of the disorder).

"Karla" <jenkinskarla at aol.com> wrote in message
news:bf7633ac.0402050715.1290452c at posting.google.com...
> Does anyone have personal experience or information on Devic's?
> It is a possible diagnosis for a friend of mine.
> Thanks

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