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Devic's Syndrome
SOURCE: Multiple Websites
Welcome to Devic's support. Support and
information for patients with Devic's / NMO, their caregivers
and friends. We also welcome all researchers and clinicians.
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What is Devic's Syndrome?
Devic's syndrome (also known as Devic's disease and Neuromyelitis Optica) is a
rare, chronic,
inflammation and
demyelinating disease of the
central nervous system
(CNS) which resembles Multiple Sclerosis in several ways and is considered a special
form of multiple sclerosis (MS) with a severe and rapid course.
Devic's
disease is characterized by attacks of acute
optic neuritis
(ON), usually in both eyes (bilateral or on
both sides of the body or head).
At the same time or within a few days, weeks or occasionally
months, the ON is followed by severe
transverse myelopathy (TM) - acute inflammation of the
spinal cord. However, in about 20% of cases, TM can precede the
ON.
In Devic's syndrome, the fatty sheath that protects these
nerves is lost. Individuals may experience vision impairment and
various degrees of paralysis, as well as incontinence. The
disorder is closely linked with MS and lupus, but usually
appears before any symptoms of MS are noted. If an isolated
disease episode affecting the spinal cord and optic nerve occurs
after an infection or common cold, it is considered a
post-infectious acute demyelinated encephalomyelitis (ADE)
rather than Devic's syndrome.
Optic neuritis in Devic's is often
retrobulbar (not visible on back of the eye through an
opthalmoscope). Recovery from attacks of Devic's disease is
typically poorer than
remissions from
Relapsing Remitting Multiple Sclerosis but the relapses are
usually less frequent than is typical in MS.
The symptoms
of Devic's disease include marked loss of vision in both eyes
(optic neuritis) followed by
numbness,muscle weakness,
spasticity,
incoordination,
ataxia,
urinary, bowel, sexual and autonomic dysfunction in parts of the
trunk and limbs served by nerves exiting the spine below the
spinal
lesions.
Neurologists
argue as to whether Devic's syndrome is a completely different
disease to MS or whether it a variant of it. Many authors
consider Devic's syndrome to be a variant of post-viral
Acute Disseminated Encephalomyelitis
(ADEM) perhaps related to the varicella zoster virus.
Other believe that, like MS, Devic's disease is an
autoimmune condition.
The most obvious difference between the two is that Devic's
typically attacks the optic nerve chiasma, optic tract, and
spinal cord - usually bilaterally - whereas MS lesions can be
anywhere in the CNS
white matter albeit with a preference for the
optic nerve,
brainstem,
corpus callosum and periventricular regions.
Is there any treatment?
There is currently no standard treatment for Devic's
syndrome. Generally, treatment is symptomatic and
supportive. Corticosteroids may be prescribed. Treatment for
ADE may include corticosteroids, intravenous immunoglobulin,
and intravenous methylprednisolone
What is the prognosis?
Devic's syndrome is fatal in many patients. Some ADE
patients achieve complete or nearly complete recovery while
others may have residual deficits. Some severe cases of ADE
may be fatal.
What research is being done?
The NINDS supports an extensive research program of
basic studies to increase understanding of how the nervous
system works. A major goal of this research is to develop
methods for repairing damaged nerves and restoring full use
and strength to injured areas.
More stats:
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Very high morbidity.
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Sixty per cent of all Devic's NMO patients will indeed have
brain lesions.
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50% permanently blind in at least one eye within five years.
(Often bilaterally)
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50% paralyzed in at least one limb within the same time frame.
(Often more.)
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33% mortality within the first five years.
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80-90% of cases are relapsing.
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The majority are women.
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No standard treatment protocol. Unlike MS immunomodulation does
not work for the NMO patient, intense immunosuppresion is the
key.
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Principle distinguishing features between
Devic's Syndrome and
Multiple Sclerosis
Feature
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Devic's Syndrome
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Multiple Sclerosis
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Clinical
involvement beyond the spinal cord and optic nerves
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Rarely
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Usually
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Attacks
are bilateral
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Usually
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Rarely
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Rarely
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In over
90% of cases
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White
matter lesions on brain
MRI
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Rarely
and usually resolving
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Usually
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In 20% of
cases
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Rarely
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Swelling
and signal change on MRI
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Often
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Less
Often than Devic's
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Tissue
destruction and cavitation
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More than
MS
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Less than
Devic's
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Protein
content in CSF
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Higher
than MS
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Lower
than Devic's
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Neuromyelitis Optica (NMO)
Neuromyelitis optica is an uncommon, idiopathic,
demyelinating syndrome of the central nervous system that
preferentially affects the optic nerves and spinal cord. It
frequently is misdiagnosed as severe multiple sclerosis, but
usually is readily distinguished from multiple sclerosis in
fully developed cases because of its severity, typical
magnetic resonance imaging (MRI) findings (normal brain MRI
longitudinally extensive lesions on spinal cord MRI), and
cerebrospinal fluid analysis (polymorphonuclear pleocytosis
and absence of oligoclonal banding). A serum auto-antibody
marker, NMO-IgG, is highly specific for the disorder.
Most patients have relapsing disease, and natural history
studies confirm early and severe disability. We treat acute
myelitis and optic neuritis exacerbations with parenteral
corticosteroids and use rescue plasmapheresis for severe,
refractory attacks. Immunomodulatory drugs used for typical
multiple sclerosis seem ineffective for relapse prevention. We
recommend systemic immunosuppression, usually with
azathioprine and oral cortico-steroids, for most patients.
Fulminant disease and breakthrough disease may respond to
other forms of humoral immunotherapy such as rituximab.
NMO is a disorder of the central nervous system which has
many features in common with multiple sclerosis. It is also
known as Devic's disease. Traditionally, it has been defined
as a syndrome where patients had a combination of transverse
myelitis and optic neuritis, often with relatively severe
symptoms in both the optic nerves and the spinal cord.
Other forms of Multiple Sclerosis are:
Devic's Syndrome
The following forms are the four Internationally recognized forms of
MS
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