Neuro-1. A rare case of Devic's syndrome in a 60 year-old female.A 60-year-old African-American female presented with left upper and lower extremity weakness and paraesthesias. There were no visual changes on admission, but she had recently suffered bilateral optic neuritis, which had resolved with corticosteroid therapy. Neurological examination was significant for hyper-reflexic deep tendon reflexes globally and diminished strength, predominantly affecting the left side, but excluding the face. The patient's symptoms and examination worsened over the first 24 hours of admission, including development of severe ataxia, but without bulbar bulbar /bul·bar/ (bul´ber) 1. pertaining to a bulb. 2. pertaining to or involving the medulla oblongata. bul·bar adj. 1. Resembling or relating to a bulb. findings. MRI 1. (application) MRI - Magnetic Resonance Imaging. 2. MRI - Measurement Requirements and Interface. of the brain and spinal cord revealed inflammation of the entire cervical spinal cord. Despite treatment with high dose intravenous methylprednisolone methylprednisolone /meth·yl·pred·nis·o·lone/ (-pred-nis´ah-lon) a synthetic glucocorticoid derived from progesterone, used in replacement therapy for adrenocortical insufficiency and as an antiinflammatory and immunosuppressant; also , the patient's symptoms did not improve. Devic's syndrome is a rare, autoimmune, demyelinating, central nervous system disorder, often difficult to distinguish from other neurologic syndromes. The criteria for diagnosis include optic neuritis, transverse myelitis, and no evidence of clinical disease outside of the optic nerve and spinal cord. Diagnosis is usually made with a MRI demonstrating optic nerve and spinal cord inflammation and cerebrospinal fluid pleocytosis pleocytosis /pleo·cy·to·sis/ (ple?o-si-to´sis) presence of a greater than normal number of cells in cerebrospinal fluid. ple·o·cy·to·sis n. . Most experts consider Devic's disease to be a more severe variant of multiple sclerosis; however, this continues to be a point of controversy. The mean age of onset The age of onset is a medical term referring to the age at which an individual acquires, develops, or first experiences a condition or symptoms of a disease or disorder. Diseases are often categorized by their ages of onset as congenital, infantile, juvenile, or adult. is the fourth decade. While the exact incidence of Devic's syndrome is unknown, only 71 cases were identified over 43 years in one Mayo Clinic study. There is currently no consensus on treatment and the 5-year survival can be as low as 68%. Published therapies include corticosteroids, intravenous immunoglobulin, and other immunosuppressants immunosuppressants, n.pl the agents that lower or reduce immune response; useful in organ transplant surgery to prevent organ rejection. Corticosteroid hormones given in large amounts; cytotoxic drugs, including antimetabolites and alkylating agents; . Devic's syndrome is a rare, autoimmune, demyelinating, central nervous system disorder that primarily affects younger patients. The etiology remains uncertain, and treatment is often unsuccessful. Diagnosis requires a high index of clinical suspicion coupled with evidence of acute optic neuritis and transverse myelitis. Walter N. Ingram, MD, Benjamin G. Romick, MD, William N. Hannah, MD. Keesler Air Force Base Keesler Air Force Base is a United States Air Force base located in Biloxi, Mississippi. The base is home of the 81st Training Wing, and the base is responsible for training airmen who have just completed basic training as well as additional training they will need for upcoming Medical Center, Keesler Air Force Base, MS. |
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