Anti-NF155 IgG4 antibody-positive CIDP shows distinctive clinicopathological features, indicating that the IgG4 antibody is directly associated with the pathogenic mechanisms of anti-NF155 IgG4 antibody-positive CIDP. Muscle Nerve 57: 498-502, 2018.

ObjectiveTo investigate the relationship between antibody reactivities against glycolipid complexes and clinical features in Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome with ophthalmoplegia (GBS-OP).MethodsUsing glycoarray, antibodies against 10 glycolipid antigens (GM1, GM2, GM4, GD1a, GD1b, GQ1b, galactocerebroside, lactosylceramide, GA1, and sulfatide) and 45 glycolipid complexes consisting 2 of the glycolipids were examined in the sera of 63 patients with GBS-OP, 37 patients with MFS, and 27 patients with BBE.ResultsAntibodies to antigens containing GQ1b were identified in 73% of patients with GBS-OP (46/63), 86.5% of patients with MFS (32/37), and 74.1% of patients with BBE (20/27), and GD1b-related antibodies were identified in 49.2% of patients with GBS-OP (31/63), 29.7% of patients with MFS (11/37), and 11.1% of patients with BBE (3/27). Comparing clinical features between patients with GBS-OP with and without both antibodies, the proportion of patients requiring artificial ventilation and presenting moderate or severe muscle weakness was higher in the positive group than in the negative group (p = 0.017 and p = 0.046, respectively).ConclusionsAntibodies binding to antigens containing GD1b and to those containing GQ1b may be involved in the development of limb weakness and respiratory failure in anti-GQ1b antibody–related diseases.

ObjectiveWe examined the clinical and serologic features of Guillain-Barré syndrome (GBS)-related diseases (GBSRDs), including GBS, Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE), after influenza virus infection (GBSRD-I) to reveal potential underlying autoimmune mechanisms.MethodsWe retrospectively investigated the presence of antiglycolipid antibodies against 11 glycolipids and the clinical features of 63 patients with GBSRD-I. Autoantibody profiles and clinical features were compared with those of 82 patients with GBSRDs after Campylobacter jejuni infection (GBSRD-C).ResultsThe anti-GQ1b seropositivity rate was significantly higher, whereas the GM1 and GD1a seropositivity rates were significantly lower in GBSRD-I compared with GBSRD-C. Anti-GQ1b and anti-GT1a were the most frequently detected antiglycolipid antibodies in GBSRD-I (both 15/63, 24%). Consequently, FS was more frequent in GBSRD-I than GBSRD-C (22% vs 9%, p < 0.05). In addition, as for GBS, cranial nerve deficits, sensory disturbances, and ataxia were more frequent in the cases after influenza infection (GBS-I) than in those after C. jejuni infection (GBS-C) (46% vs 15%, 75% vs 46%, and 29% vs 4%, respectively; all p < 0.01). Nerve conduction studies revealed acute inflammatory demyelinating polyneuropathy (AIDP) in 60% of patients with GBS-I but only 25% of patients with GBS-C (p < 0.01).ConclusionsAnti-GQ1b antibodies are the most frequently detected antibodies in GBSRD-I. Compared with GBS-C, GBS-I is characterized by AIDP predominance and frequent presence of cranial nerve involvement and ataxia.

No abstract

We report the first patient with pathologically proven leukoencephalopathy associated with hypophosphatemia. A 61-year-old woman had repetitive episodes of decreased consciousness with pontine and pallidal lesions and extensive leukoencephalopathy on MRI, later found to be associated with hypophosphatemia. Although hypophosphatemia has been linked to central pontine and extrapontine myelinolysis (osmotic myelinolysis), lesions in the deep white matter have not been reported. Brain biopsy performed during the first diagnosis process revealed nonspecific demyelination with gliosis, a finding similar to that of chronic osmotic myelinolysis. After normalization of phosphate levels, her consciousness completely improved and MRI abnormalities partly resolved. We should consider that leukoencephalopathy can be associated with hypophosphatemia, which is often treatable.