Neuromyelitis optica spectrum disorder coinciding with hematological immune disease: a case report

• Published in: Multiple sclerosis and related disorders 2016-09, Vol.9, p.101-103
• Main Authors: Patejdl, R, Wittstock, M, Zettl, U.K, Jost, K, Grossmann, A, Prudlo, J
• Format: Article
• Language: English
• Subjects: Agranulocytosis - complications; Agranulocytosis - diagnostic imaging; Agranulocytosis - therapy; Anemia, Hemolytic - complications; Anemia, Hemolytic - diagnostic imaging; Anemia, Hemolytic - therapy; Autoimmune hemolytic anemia; Autoimmune thrombocytopenia; Cervical Cord - diagnostic imaging; Humans; Longitudinal extensive transverse myelitis; Male; Middle Aged; Neurology; Neuromyelitis optica; Neuromyelitis Optica - complications; Neuromyelitis Optica - diagnostic imaging; Neuromyelitis Optica - therapy; Thrombocytopenia - complications; Thrombocytopenia - diagnostic imaging; Thrombocytopenia - therapy
• ISSN: 2211-0348; 2211-0356
• DOI: 10.1016/j.msard.2016.07.006

Abstract Introduction Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature. Case Report A 38 year old male subsequently developed thrombocytopenia, hemolytic anemia and agranulocytosis over a 23 month period. Three months after an episode of agranulocytosis, he noticed ascending sensory disturbances and progressive weakness of his legs. Within two days, symptoms worsened to give almost complete paraplegia and loss of sensation below a midthoracic level. MRI revealed signal hyperintensity and edema in T2-weighted sequences reaching from the 2nd cervical to the 9th thoracic vertebral body. Two years later, he developed a second episode with lesions in the spinal cord and periventricular areas of brain stem and thalamus. Conclusion The relapsing time course and the topographical pattern of central nervous system lesions restricted to axial brain structures and the spinal cord fulfill the criteria that have recently been defined for AQP4-Ab-negative NMO-spectrum disease. Systematic studies on the association of hematological autoimmune phenomena and spinal cord disease are needed to clarify whether this coincidence is just a casual phenomenon or whether it points to a yet undiscovered but perhaps therapeutically interesting link of immunological mechanisms affecting both organ systems.