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Case ReportOpen Access
Neuromyelitis Optica with Hypothalamic Involvement: A Case Report
Samart K 
,
Phanthumchinda K
,
Phanthumchinda K 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 of NMO.
Keywords: Neuromyelitis optica, Hypersomnia, Confabulation, Hypothalamic, Brain stem
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 of NMO.
Keywords: Neuromyelitis optica, Hypersomnia, Confabulation, Hypothalamic, Brain stem
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