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July 2008

Neuromyelitis Optica With Coexisting Autoimmune Disorders

Arch Neurol. 2008;65(7):992-996. doi:10.1001/archneur.65.7.995-a

We were greatly interested in the article by Pittock et al1 titled “Neuromyelitis Optica and Non–Organ-Specific Autoimmunity.” We describe a patient with neuromyelitis optica (NMO) and NMO-IgG seropositivity with coexisting organ-specific (Hashimoto thyroiditis) and tissue-specific (alopecia areata) autoimmune disorders. Our patient, a 30-year-old Greek woman, was admitted to the hospital 3 years ago because she had subacute spastic tetraparesis with a C4 sensory level. Her history was remarkable for polycystic ovarian syndrome, hyperprolactinemia, hypothyroidism due to Hashimoto thyroiditis, and alopecia areata. Eight days later, she developed bilateral optic neuritis. Brain magnetic resonance imaging results were normal. Spinal magnetic resonance imaging showed cavitationlike intramedullary necrotic lesions from C2 to C7 (Figure, A-C). She had elevated serum prolactin (4.21 × 107 μg/L [to convert to picomoles per liter, multiply by 43.478]) and free testosterone (1.71 ng/dL [to convert to nanomoles per liter, multiply by 0.0347]) levels, a decreased total thyroxine level (5.1 μg/dL [to convert to nanomoles per liter, multiply by 12.871]), and high titers of antithyroid antibodies. She was found to have HLA-DR15. Eight months later, she developed acute spastic paraplegia, a T5 sensory level, and urinary incontinence. Thoracic magnetic resonance imaging revealed necrotic intramedullary lesions from T3 to T5 (Figure, D). Neither steroid treatment nor plasma exchanges (5 exchanges of 3 L each) led to improvement. Six months later, she developed spastic tetraplegia and bilateral optic neuritis with no resolution until now. Oligoclonal bands were absent in all of the cerebrospinal fluid samples. Lymphocytosis and increased protein levels were present only during relapses.

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