Overview Of Neuromyelitis Optica (Devic Syndrome)

Devic syndrome is a very rare, autoimmune disease that affects the optics nerves and the spinal cord, within the central nervous system. The immune system’s antibodies attacks and destroys the myelin sheath (layer around the CNS neurons). There is no genuine statistics that are linked to this disease, so it is very difficult to determine how many individuals have presented with Devic symptoms.

Types

Devic syndrome is very unpredictable, in how the symptoms appear, severity, and the progression of the disease. Some victims will have reoccurring episodic periods, which basically means that they  suffer from the relapsing form. This type has been known to affect more females than males at a rate of 4:1.

Some victims will have one severe attack, which may last from 1-2 months, without relapsing. This type of Devic syndrome has been noted, as affecting men and women alike, with neither being a bigger target.

Symptoms

  • Optic Neuritis (inflammation of the optic nerve), with sudden onset of symptoms
  • Transverse Myelitis (inflammation of the spinal cord, which targets the myelin sheath) causes numbness, tingling, pain, and weakness in the arms and legs. Bladder and bowel complications have also been noted.
  • Neck Stiffness
  • Partial-Full Paralysis
  • Headache
  • Fever
  • Decreased Appetite
  • Unilateral Vision Changes (occurs suddenly)

There have been rare cases where the brainstem has also been affected. Uncontrollable vomiting and hiccups are two main symptoms that can appear in these cases.

Etiology

Devic syndrome is not linked to multiple sclerosis, as once thought. Fifty percent of Devic syndrome victims have been diagnosed with other autoimmune diseases. HIV, varicella, syphilis, and Epstein Barr virus has also been linked to Devic.

Genetic Factor

Human leukocyte antigen (HLA) is linked to Devic syndrome. NMO-IgG (found in 70% of cases)causes toxins to build up in the brain including glutamate, which is a neurotransmitter. Glutamate suppresses myelin production, which in turn makes the neurons vulnerable to external factors.

Treatment

There is currently no cure for Devic syndrome, but the symptoms will be treated with prescription medications.

  • Corticosteroids (decrease inflammation)
  • Opiates (decrease pain)
  • Muscle Relaxants
  • Physical and Occupation Therapy
  • Speech Therapy
  • Plasmapheresis (separates plasma and antibodies from the blood, then replaces it with saline, albumin, or  specifically prepared donor plasma.

Diagnosis

  • Lumbar Puncture
  • Magnetic resonance Imaging
  • Genetic Screening

Prognosis

It is difficult to determine the life expectancy of Devic syndrome victims. Reoccurring relapses will continue to damage and destroy the myelin, this will lead to paralysis and respiratory compromising complications are inevitable, which will dead to death.