Nmo ppt
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Neuromyelitis optica (NMO), also known as Devic's disease, is a demyelinating disorder that causes optic neuritis and transverse myelitis. It was once considered a variant of multiple sclerosis, but is now recognized as a distinct condition associated with antibodies against aquaporin-4. NMO predominantly affects women and has a more severe prognosis than MS, with incomplete recovery after episodes and higher mortality. Treatment involves steroids for acute attacks and B-cell depleting therapies such as rituximab to reduce relapse risk.
Nmo ppt
- 1. NEUROMYELITIS OPTICA (NMO) (DEVIC DISEASE) Dr. Pawan Kumar Barolia MBBS MD Fellowship in intensive care Pediatrics
- 2. Neuromyelitis optica (NMO) (Devic disease) It is a demyelinating disorder characterized by monophasic or polyphasic episodes of optic neuritis and/or transverse myelitis It was once thought that NMO was a variant of MS Most authorities believe that NMO is a distinct disorder. Epidemiology Nmo has an age of onset of 11 yr. In 1 study, in monophasic patients, the range of age of onset was 1- 54 yr; In polyphasic patients, the range was 6-72 yr NMO is more common in women and girls than in men and boys; 65% of monophasic and 80-85% of polyphasic NMO patients are female. It is also more common in asians than in blacks or whites Appears to have a higher mortality rate in individuals of african descent than in others
- 3. Pathogenesis NMO is associated with IgG antibodies to the aquaporin-4 water channel It is not clear why the attack on and depletion of spinal cord and optic nerve aquaporin-4 results in disruption of the myelin sheath in these parts of the CNS, Pathologic examination of autopsy specimens reveals both anti–aquaporin-4 IgG deposits and B cells in the spinal cord and optic nerves of NMO patients Although most cases of NMO are idiopathic only occasional familial cases have been reported there have been reports of postinfectious NMO HIV, syphilis, chlamydia, varicella, cytomegalovirus, and Epstein-Barr virus have been associated with subsequent development of NMO.
- 4. Clinical Manifestations Either optic neuritis or transverse myelitis or both Visual field defects and color desaturation are common. The symptoms and signs of transverse myelitis depend on the spinal level and completeness of the inflammatory changes NMO differs from MS in that other parts of the nervous system are generally not involved Recovery of visual and spinal cord function is generally not as complete after each episode Optic neuritis is more frequently bilateral in NMO than in MS NMO is more frequently fatal than MS.
- 5. Laboratory Findings CSF in patients with NMO often has 50 or more WBC/mL Unlike MS, it is devoid of oligoclonal bands Serum positivity for anti–aquaporin-4 antibodies (so-called NMO antibodies) has a sensitivity of 73% and a specificity of 91% for NMO Neuroimaging studies may show small, asymptomatic lesions in the white matter of the brainstem or hemispheres, but not the large (>3 mm), oval lesions in the periventricular white matter seen in MS. MRI lesions are most commonly demonstrated in the optic nerves and spinal cord, and in the diencephalons
- 6. Diagnosis Clinical criteria for the diagnosis of NMO include finding at least 2 of the following normal brain MRI, spinal cord widening and cavitation involving at least 3 spinal segments, decreased serum/CSF albumin ratio with normal IgG synthesis rate the absence of oligoclonal bands, acute episode(s) of spinal cord and/or optic nerve involvement separated by months or years without any other systemic or neurologic features
- 7. Differential Diagnosis MS; ADEM rheumatologic etiologies of transverse myelitis and/or optic neuritis including SLE, Behcet disease, and neurosarcoidosis (usually accompanied by other nonneurologic manifestations); idiopathic transverse myelitis, tropical spastic paraparesis, viral encephalomyelitis (none of which have NMO antibodies in the serum or CSF); metabolic and idiopathic causes of isolated optic neuritis or other acute monocular or binocular visual
- 8. Complications left with fixed neurologic deficits affecting visual acuity, visual fields, color vision motor and sensory function, balance, and bowel/bladder function.
- 9. Treatment Initial episodes and relapses may be treated acutely with methylprednisolone, 20-30 mg/kg/day (maximum 1,000 mg/day) for 3-5 days, followed by a slow prednisone taper It is not yet known whether injectable DMT (interferon- beta1α or interferon-beta1β; glatiramer acetate) reduces relapse frequency in NMO. However, agents like rituximab that reduce B cell number and function have shown promise in this regard in early studies.
- 10. Prognosis The prognosis in NMO is generally poor for patients with NMO In 1 study, approximately 20% remained functionally blind (i.e., 20/200 vision or worse) in at least 1 eye 31% had permanent monoplegia or paraplegia. Five-year survival of the patients with paraplegia is approximately 90%.