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Previous case reports and studies have shown that anti-myelin-associated glycoprotein (MAG) antibody can be detected in patients with polyneuropathy without any detectable M-protein. Nevertheless, the frequency of and related factors have not yet been adequately investigated. The objectives of this study are to examine the prevalence of anti-MAG antibody in patients with demyelinating neuropathy without M-protein and to determine their clinical characteristics. From January, 2004, to September, 2016, consecutive patients with chronic demyelinating neuropathy were recruited. Anti-MAG antibody presence was tested at the first evaluation. We determined the prevalence of anti-MAG antibody without M-protein among included patients and evaluated the clinical characteristics. A total of 44 patients were included in the present study (12 women; median age at first visit 60 years [interquartile range 47-67 years]; median duration between onset and first visit 9 months [3-26 months]). M-protein was found in eight patients (18%) at the first evaluation. Anti-MAG antibody was present in 2 of remaining 36 (5.6 [95% confidence interval 0-13.0] %) patients without M-protein. Patients with anti-MAG antibody exhibited slowly progressive and distal dominant neuropathy with elevated serum IgM levels and refractory to immunotherapy. There were no differences in clinical features between patients having anti-MAG antibody without M-protein, and those with M-protein. One patient with the anti-MAG antibody showed a delayed appearance of M-protein during a 4-year follow-up after diagnosis. The prevalence of the anti-MAG antibody in chronic demyelinating neuropathy without any detectable M-protein was 5.6%. Anti-MAG antibody may be detectable earlier than M-protein.

Although typical corticobasal syndrome (CBS) presents with asymmetric upper limb symptoms, the prevalence and clinical characteristics of patients with symptoms beginning in other sites are unknown. From January 1997 through April 2016, consecutive patients with CBS who fulfilled the modified Cambridge criteria were recruited. Their medical records were reviewed to determine the body part, where the initial symptoms developed and the clinical characteristics. A total of 24 patients [13 female participants, median age at onset: 64 (IQR 60-74) years, and median duration between onset and evaluation: 38 (17-53) months] met the criteria. The initial symptom involved the unilateral upper limb in 14 cases (58%), unilateral lower limb in five (21%), gait in four (17%), and visual field in one (4%). Over a median of 59 (IQR 40-68) months of follow-up, the duration between the onset and the time for need of assistance in walking was significantly shorter in the patients with lower limb (p = 0.018 with log-rank test) or gait (p = 0.025) onset than in those with upper limb onset. About a half of the CBS patients initially complained of symptoms other than the upper limb. The most common area of origin of the initial symptom after the upper limb was the lower limb followed by gait. Such patients need assistance in walking earlier than those with upper limb-onset CBS. Patients with lower limb- or gait-onset CBS are not rare and may have unfavorable outcome.

This study aimed to quantitatively analyze fasciculation potentials (FPs) and to investigate their relationship with muscle strength in amyotrophic lateral sclerosis (ALS). Fifty-one patients with sporadic ALS or progressive muscular atrophy (25 men, 26 women, mean age of 68 years) underwent needle EMG. We determined the duration, phase number, and amplitude of FPs from three muscles (upper trapezius, biceps brachii, and tibialis anterior) and examined their relations with muscle strength. In total, 878 FPs were analyzed. FP duration displayed a significant negative relation with the strength of all three muscles; the weaker muscles showed longer durations of FPs than the muscles with normal strength. The amplitude and phase number were not related with muscle strength, but there were significant correlations between the duration and amplitude of FPs in the trapezius and tibialis anterior muscles. The longer duration of FPs in muscles with weak strength suggests that the morphological changes of FPs were caused by temporal dispersion through progressively degenerating and/or immature reinnervating motor branches, and were observed uniformly in different muscles along with disease progression.

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy. Although its pathogenic mechanism has been revealed and various therapeutic trials have been performed, a proportion of patients experience the severe sequelae associated with GBS. In this paper, we investigated whether the amount of the neuron-specific protein, ubiquitin carboxy-terminal hydrolase-L1 (UCH-L1), in the cerebrospinal fluid of patients with GBS was correlated with the clinical course of the disease. UCH-L1 protein levels were greater in patients with GBS than in controls. The patients with GBS whose UCH-L1 protein levels were higher than those of the controls presented with more severe symptoms at peak. UCH-L1 protein levels tended to become elevated as the total protein levels were increased; however, elevated UCH-L1 without an increase in total protein might be correlated with severe disease course (bedridden or ventilator supported). These results suggest that UCH-L1 could be a biomarker associated with the severity of the disease at the acute phase of GBS.