1b)

1b). by severe optic neuritis and transverse myelitis 6-O-2-Propyn-1-yl-D-galactose (1,2). NMO and optic-spinal multiple sclerosis are NMO spectrum disorders (NMOSD) and can be distinguished from multiple sclerosis (MS) by the serum autoantibody marker NMO-IgG/anti-aquaporin 4 antibody (AQP4-IgG) (3). The recognition of variable distributions of NMO lesions outside the spinal cord and optic nerves has promoted the concept of NMOSD (4,5). Recently, the specificity of AQP4-IgG for making an NMO diagnosis has facilitated observations that further broadened the clinical Rabbit Polyclonal to BAD and neuroimaging spectrum of NMO. The International Panel for NMO Diagnosis released an updated set of guidelines for diagnosing NMO and NMOSD in 2015. These disorders are now known as NMOSD (6). Initially, NMOSD had been considered moderate or with low numbers of cerebral lesions. NMOSD is usually relatively frequent in Asia (7,8). Characteristic images are described regularly, but progressive and extensive lesions of the brain are not well described in NMOSD. However, there have been some recent reports of progressive white-matter lesions in NMOSD (9-11). The present patient showed optic 6-O-2-Propyn-1-yl-D-galactose neuritis as the initial symptom after 50 years old. Nine years after the initial symptom, the patient showed exacerbation of clinical symptoms without remission, with lesions mainly in the cerebral white matter and brain stem during the total course of 14 years. There was no evidence of spinal cord symptoms. Thus, we suspected that the patient had secondary progressive MS and did not measure AQP4-IgG. Nevertheless, this case was pathologically diagnosed as NMOSD after an autopsy. In NMOSD, the secondary progressive type is usually thought to be extremely rare. Therefore, this case is an extremely useful case diagnosed as NMOSD by a histopathological examination, although this diagnosis was unable to be deduced from the clinical course. Case Report A 57-year-old woman with no specific medical history or a family history of neurological illness visited the Department of Ophthalmology because of decreased visual acuity in her left vision. An ophthalmologist diagnosed her with optic neuritis. She received intravenous methylprednisolone (1,000 mg/day for 2 days) and a retrobulbar injection of dexamethasone (80 mg/day for 5 days). A month after starting the treatment, her symptoms subsided. 6-O-2-Propyn-1-yl-D-galactose At 60 years of age (X+3 years), she presented with symptoms of left hemiparesis, including the face, when she frequented the Department of Neurology. Computed tomography of the head showed a low-density area at the right internal capsule. She was treated as a patient with ischemic stroke. After this episode, the patient again experienced visual deterioration in her left vision, and she was hospitalized in the ophthalmology department. She was prescribed oral steroids for two months and completely recovered. At 63 years old (X+6 years), she presented with hemiparalysis on the right side and facial paralysis around the left side. Magnetic resonance imaging (MRI) of the head revealed multiple lesions in her right superior cerebellar peduncle and middle cerebellar peduncle. A diagnosis of MS was made, and we administered steroid pulse therapy, upon which she almost completely recovered. Data on oligoclonal 6-O-2-Propyn-1-yl-D-galactose bands and myelin basic protein in the cerebrospinal fluid were not available, but her serum was positive for both anti-SS-A antibody 70.4 (<20) and anti-SS-B antibody 26.8 (<20) without sicca syndrome. Mizoribine was administered for 6 months. However, she had to discontinue treatment due to hepatic dysfunction. At 67 years of age (X+10 years), she presented with gait disturbance, nystagmus, and left-sided ataxia. At this time, we found a lesion in the cerebellar peduncle. After steroid pulse therapy, -interferon therapy was initiated. However, even after starting the treatment, her symptoms remained, and she showed gradual deterioration in her activities of daily living (ADL). At 68 years old (X+11 years), she became susceptible to falling and presented with symptoms of incontinence and cognitive impairment. MRI of the head showed new lesions, including in the right parietal subcortical region and lateral geniculate body. Although steroid pulse therapy was performed, walking difficulties remained, and cognitive impairment progressed. At 69 years old (X+12 years), she again showed indicators of decreasing left visual acuity and received steroid pulse therapy. However, she failed to regain visual acuity and was declared completely blind. During that time, she had another fall and suffered a right femoral and thoracolumbar vertebral fracture. During the course, she had been.