Neuromyelitis optica with hypothalamic involvement: a case report.

Current diagnostic criteria of neuromyelitis optica (NMO) includes presence of acute optic neuritis (ON) and myelitis with at least two of the three supportive criteria, which consist of spinal cord magnetic resonance image (MRI) lesion extending over 3 vertebral segments, brain MRI lesion, which does not meet the diagnostic criteria for multiple sclerosis, and NMO-IgG seropositive status A 34-year-old woman presented with two episodes of acute demyelinating processes in the central nervous system within three years. Firstly, she presented with a 2-week history of neck pain, oscillopsia, vertigo, and weakness. MRI of the brain revealed a high signal change at cervicomedullary junction. She responded to a short course of high-dose corticosteroid. One year after the first presentation, she developed bilateral optic neuritis. High dose corticosteroid therapy was prescribed for this attack After the second episode, she received long-term azathioprine. Two weeks before admission, she developed hypersomnia and confabulation. General physical examination was unremarkable. Neurological examination revealed visual acuity (VA) of 20/200 in both eyes. Optic fundi were normal. MRI of the brain demonstrated hypersignal intensity lesions at the hypothalamus, tuber cinereum, medial aspect of thalami, dorsal midbrain, and occipital periventricular white matter in T2 weighted and FLAIR images. Cerebrospinal fluid (CSF) analysis revealed a white blood cell count of 33 cells/mm3 (100% lymphocytes), protein of 34 mg/dL, CSF sugar of 55 mg/dL, and blood sugar of 100 mg/dL. Oligoclonal band was negative. Two weeks after admission, she developed quadriparesis, pain, and proprioceptive sensory loss below the 6th thoracic level. She also had urinary retention and constipation. MRI of the whole spinal cord showed multilevel hypersignal intensity lesions on T2 weighted and FLAIR images involving medulla, cervicomedullary junction and all segments of the spinal cord. She was diagnosed as NMO. Hypothalamic and brainstem involvement demonstrated in this patient were uncommon but rather pathognomonic for NMO. The authors proposed that the involvement of hypothalamus and brainstem be included in the criteria for diagnosis ofNMO.