Neuromyelitis Optica

Definition

Devic Syndrome is a rare neurological disorder that affects both the protective sheet that lines the spinal cord and the optic nerve of the eye. People that have Devic syndrome lose the fatty covering of the spinal cord (myelin) and experience eye pain due to an exaggerated inflammatory response that occurs in the eye. The resulting spinal cord damage is known as transverse myelitis and the resulting eye inflammation is known as optic neuritis. Devic syndrome is a severe neurodegenerative disorder that can lead to blindness, paralysis, and incontinence (loss of bowel or bladder control).

Description

Devic syndrome is an autoimmune disorder that is considered by many scientists to be a form of multiple sclerosis, another neurodegenerative disease that affects the protective coating of the spinal cord called the myelin sheath. In Devic syndrome, the course of the disease is more rapid and severe. Symptoms typically observed in patients that have multiple sclerosis usually appear after symptoms associated with Devic syndrome, distinguishing the two neurodegenerative diseases. Devic syndrome is also known as Devic disease and neuromyelitis optica.

It is still controversial whether Devic syndrome is a variant of multiple sclerosis. It is considered by some scientists to be a variant of a disease caused by exposure to the varicella zoster virus that results in acute disseminated encephalomyelitis (ADEM).

Demographics

Devic syndrome can occur spontaneously, or in conjunction with multiple sclerosis or systemic lupus erythematosus. It affects males and females equally. Devic syndrome is a rare disorder, affecting less than an estimated five persons per million population per year.

Causes and symptoms

Devic syndrome is a chronic and degenerative disorder that usually affects both eyes. The eyes develop diminished sensitivity to bright lights, color vision impairment, and diminished light reflexes. Approximately two-thirds of persons with Devic syndrome experience complete visual loss. The symptoms begin with significant loss of vision that precedes muscle weakness, ataxia (coordination difficulties and unsteady gait, or manner of walking), and numbness. Inflammatory sites of attack are usually the optic nerve chiasma, optic tract, and spinal cord. Usually, the optic neuropathy (damage to the optic nerve) is accompanied by severe transverse myelitis, which involves an acute inflammation of the spinal cord. The optic neuropathy usually happens before the transverse myelitis occurs, but in approximately 20% of patients it occurs in the reverse order.

Persons with Devic syndrome can also experience urinary, gastrointestinal, and sexual dysfunction. This occurs due to degeneration of the nerves that exit the spinal cord and serve the body's trunk and limbs. Patients with Devic syndrome rarely experience clinical signs that involve defects beyond symptoms arising from the spinal cord and optic nerve. There are also characteristic brain MRI scan findings including swelling and signal changes that are typically observed, as well as increased protein content in the cerebral spinal fluid.

Content licensed from:

Author Info: Bryan Richard Cobb PhD, The Gale Group Inc., Gale, Detroit, Gale Encyclopedia of Neurological Disorders, 2005

This feature is for informational purposes only and should not be used to replace the care and information received from your healthcare provider. Please consult a healthcare professional with any health concerns you may have.