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The progression pattern of neurological disability among patients with anti-myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) was evaluated. Neurological disability was evaluated annually for 408 person-years in 50 patients. More than 30% of the patients had clinical relapses in the first 5 years. Disability progression independent of relapse activity (PIRA) was not seen, whereas a stepwise disability progression was observed after clinical attacks in some instances. Disability worsening was more frequent after relapses than after the onset episode (p 0.01). Similar to patients with anti-aquaporin-4 antibodies, attack-related stepwise disability progression without PIRA is typical in MOGAD, suggesting the importance of relapse prevention.

Predisposing factors before the onset of neuromyelitis optica spectrum disorders (NMOSD) have not been systematically evaluated by now. We investigated the detailed pre-onset history in consecutive NMOSD patients. Thirteen of the enrolled 53 NMOSD patients (24.5%) had accompanying autoimmune diseases, such as Sjogren's syndrome. History of malignancy was seen in 8 of the 53 patients (15.1%). Recent history of non-neurological clinical episodes, such as systemic allergic reaction, systemic infection, surgical operation, or traumatic injury, was seen in 23 of the 53 patients (43.4%). NMOSD patients are likely to have pre-onset history of other autoimmune diseases, malignancy, or recent non-neurological systemic conditions, which may predispose or trigger the onset of NMOSD.

Multiple sclerosis (MS) is a demyelinating neurological disease with unknown causes. In this study, we comprehensively studied blood cell counts in the early phase of MS and compared their values with eventual prognostic variables. We found that the blood monocyte count in the early phase of MS was robustly associated with the clinical severity of MS (rho = 0.64; p = 0.0002) but that the counts of the other blood cells were not associated with severity. This correlation between monocyte count and severity was not observed in neuromyelitis optica. In conclusion, blood monocytes could be a candidate for the prognostic prediction of MS.

We compared the retinal thickness in the unaffected eyes among the following subtypes of unilateral optic neuritis (ON): multiple sclerosis (MS-ON), neuromyelitis optica spectrum disorder with anti-AQP4 autoantibody (AQP4-ON), patients with serum anti-MOG antibody (MOG-ON), and idiopathic ON. In the chronic phase, macular GCC and circum-papillary RNFL in the unaffected eyes were both atrophied in MS-ON and AQP4-ON, but were not atrophied in the others. Titers of anti-AQP4-Ab was suggested to be associated with such latent neurodegenerative process in AQP4-ON. Long-term follow up of OCT is recommended even in the unaffected side in MS-ON and AQP4-ON.

We evaluated the brain volume and rate of atrophy in 85 Japanese patients with multiple sclerosis (MS). The mean brain volume was smaller and the rate of atrophy appeared to be more rapid in MS patients than in normal subjects. The brain atrophy seemed to exist from the early stage of MS and was prominent in the grey matter. Patients with progressive MS showed severer atrophy. We show the existence of pathological brain atrophy in Japanese MS patients for the first time, although the rate of atrophy may be much slower than in Caucasian patients.

We compared the clinical features of optic neuritis (ON) that are frequently observed in various central nervous system demyelinating diseases, including multiple sclerosis (MS), anti-aquaporin 4 (AQP4) antibody- and anti-myelin oligodendrocyte glycoprotein (MOG) autoantibody-related diseases. Almost all the AQP4-ON patients were female, whereas half of the MOG-ON patients were male. The ON-onset age was younger in MS-ON and was older in AQP4-ON. The ON-lesion detected using optic MRI in the acute phase was longer in MOG-ON and showed severe swelling and twisting. The worst visual acuity was similar between the diseases; however, the final visual acuity was significantly worse in AQP4-ON.

The visual acuity prognoses of patients with neuromyelitis optica (NMO) are worse than those with optic neuritis (ON) caused by other diseases. Predicting the prognoses of ON at the time of onset is important for selecting treatments for NMO patients.Twenty-three consecutive anti-aquaporin-4 autoantibody-positive NMO patients who presented with ON and had contrast-enhanced optic MRIs in the acute phase of their first ON episode were examined. Optical coherence tomographies (OCTs) were also examined for 22 of them. The visual acuity at the final follow-up, as assessed with the logMAR scale more than three years after ON onset, served as the outcome measure. These variables were also collected from 12 patients with serum anti-myelin oligodendrocyte glycoprotein antibody (anti-MOG-Ab).The strongest predictor of visual prognosis was the axial ON lesion length in the acute phase (R=0.747, p0.0001), which was not observed in patients with anti-MOG-Ab. Specifically, the ON lesion length within the intra-orbit and canalicular segments exhibited the strongest correlation with visual prognosis (R=0.783, p0.0001). The ON onset age was also correlated with visual prognosis (R=0.435, p=0.0338). OCT data in the chronic phase also showed a correlation with visual prognosis, but they were much weaker than the ON lesion length in the acute phase.The ON lesion length in the acute phase was an important predictor of the visual prognoses of NMO patients.

This study aimed to determine the seasonal impact on the clinical onset of inflammatory neurological diseases of the central nervous system by analyzing the onset month with information on clinical manifestations in Japanese patients. As a result, patients with anti-aquaporin-4 antibodies (AQP4-IgG)-positive neuromyelitis optica spectrum disorders (NMOSD) showed spring-summer predominance of the clinical onset. Conversely, patients with anti-myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease showed autumn-winter predominance of the clinical onset. Both seasonal variations were irrespective of the clinical manifestation. Environmental factors with seasonal variation influence the development of neurological conditions related to AQP4-IgG and MOG-IgG.

We aimed in this study to elucidate the impact of serum AQP4-IgG titer before starting treatments to the clinical manifestation of neuromyelitis optica spectrum disorders (NMOSD). Serum titer at the onset, measured using live cell-based assay method, did not correlate to the subsequent relapse rate or neurological prognosis. Patients with optic neuritis as the first attack showed significantly higher serum titer than patients with acute myelitis or area postrema syndrome, although the titer did not correlate to the visual prognosis. The result implies that the pathological mechanism of optic neuritis and acute myelitis could be different in NMOSD.

Chloride imbalance between the serum and the cerebrospinal fluid (CSF) has been recently shown to exist in the acute phase of neuromyelitis optica (NMO). In this report, we studied the relation between the quotient of chloride (QCl) and the severity of optic neuritis (ON) in NMO patients. There was a positive correlation (R = 0.67; p

Depression and chronic fatigue are frequently present in multiple sclerosis (MS); however, the prevalence rates have not been investigated in neuromyelitis optica (NMO). Thirty-nine consecutive NMO and 75 MS patients were compared using self-rating questionnaires for depressive states, daily activity, and fatigue, as well as serum carnitine levels. A subgroup of patients with low carnitine levels were re-evaluated regarding depression and fatigue after levocarnitine treatment. Depression and fatigue were equally prevalent in MS and NMO and were strongly correlated with one another. Measurement of the serum carnitine levels and the administration of levocarnitine did not appear to be beneficial.

We collected serum-cerebrospinal fluid (CSF)-paired samples during the acute phase of clinical episodes in MS and NMO patients, together with paired samples from non-MS/NMO patients, to compare the quotients of proteins and electrolytes among them. In NMO, the quotient of chloride was significantly higher in the acute phase of optic neuritis and brain lesions, but it was significantly lower in the acute phase of myelitis. Such findings were not observed in MS or in non-MS/NMO cases. With the coexistence of serum anti-AQP4-Ab, chloride imbalance between serum and CSF could be associated with the clinical episodes in NMO.