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Devic's Disease

Synonyms: Neuromyelitis optica, Devic's syndrome
German: Neuromyelitis optica

1 Definition

Devic's disease is an inflammatory disease of the central nervous system, which mainly affects the myelin sheath covering the optic nerve and spinal cord. Devic's disease resembles Multiple sclerosis.

Devic's disease is characterized by attacks of bilateral acute optic neuritis. This maybe followed by severe transverse myelopathy (acute inflammation of the spinal cord), either at the same time along with the attacks of optic neuritis or after a period of days to months. The course of Devic's disease maybe monophasic or relapsing.

  • Monophasic: the patients experience either unilateral or bilateral optic neuritis or a single episode of myelitis.
  • Relapsing: in this course the patients continue to have a discrete exacerbation of optic neuritis and/or myelitis.

2 Epidemiology

Neuromyelitis optica usually affects young adults, but also has been reported in infants. The ratio of men to women may differ according to the course of the disease. Most reports suggest a ratio of approximately 1.4 to 1.8. Neuromyelitis optica (NMO) is more common in African Americans, Japanese and other pacific islanders.

3 Causes

The cause of NMO is not known. Its clinical and pathological features consider it as an autoimmune variant of Multiple sclerosis. A high prevalence of serum auto antibodies suggests that NMO maybe be driven primarily by B cell dysfunction.

4 Clinical features

Devic's syndrome consists of one or more episodes of optic neuritis in combination with myelitis.

  1. a viral prodrome precedes the onset of the disease in 30-50% of the cases. The prodrome consists of headache, fever, fatigue, myalgias and respiratory or gastrointestinal complaints.
  2. Rapid, bilateral and occasionally, complete visual loss.
  3. Acute transverse myelitis, defined as, severe bilateral inflammatory spinal cord injury with neurological dysfunction worsening over several hours to days and involving sensory, motor and sphincter function is typical presentation of NMO. The acute tranverse myelitis may present with the following symptoms:
  • back and girdle pain
  • paraesthesia and weakness in lower limbs
  • Weakness rapidly evolves to paraplegia and or quadriplegia often causing complete sensory loss caudal to the lesion and a flaccid bladder.
  • The acute lesion usually traverses at least three contiguous vertebral segments of the spinal cord and may result in a spinal shock with flaccid weakness, absent deep tendon reflexes and mute plantar responses.
  • Other symptoms include vertigo, facial numbness, nystagmus, headache and postural tremor.

5 Diagnosis

  • MRI of the brain and spinal cord: during acute optic neuritis the MRI may demonstrate a swelling of the affected optic nerve or the chiasm. During acute myelitis the affected region is expanded and swollen. Heterogeneous T2 signal within the lesion may suggest cavitations and necrosis. Typically, the lesions are in the central part of the cord.
  • Cerebrospinal fluid (CSF) analysis: occasionally patients may have a pleocytosis around the time of acute myelitis exacerbation. The CSF leukocyte differential may reveal the presence of neutrophils.
  • Serological tests: auto antibodies such as anti nuclear antibody, anti-double-stranded DNA antibody, extractable nuclear antigen and anti-thyroid antibodies are commonly present at the time of diagnosis.
  • Neurophysiologic tests: visual evoked potentials may occasionally detect sub clinical optic nerve lesions.

6 Treatment

The mainstay of therapy is the treatment of acute attacks, prevention of medical complications and rehabilitation.

  • Intravenous corticosteroids are given during exacerbations of NMO.
  • Plasmapheresis is used as the second-line treatment.
  • Intravenous immune globulin has also been used anecdotally.
  • Recent studies found that Interferon beta-1b is effective in the treatment of NMO.
  • Prevention of complications is important. Acute cervical cord attack may cause respiratory failure. Patients at risk of this complication may require intensive care unit observation and evaluation of respiratory and bulbar status.
  • Medical measures to prevent thromboembolic complications, aspiration pneumonia, decubiti and urinary tract infections are also required.

7 Links

For further information on nmo please visit the worldwide devic's and nmo support group: [1]

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