Rinshō shinkeigaku = Clinical neurology
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There are growing experimental and clinical data on the pathophysiological roles of antiganglioside antibodies in Guillain-Barré syndrome (GBS) and Fisher syndrome (FS). Antibodies to a ganglioside complex (GSC) consisting of two different gangliosides are detected in some GBS and FS sera. Recently, anti-GM1/GalNAc-GD1a complex antibodies, anti-GA1/GQ1b antibodies with no reaction against GM1/GQ1b, and anti-GM1/LM1 antibodies have been detected in GBS or FS sera. ⋯ Complement-independent pathophysiology such as blockade of voltage-gated Ca channels, the apoptotic mechanism of neurons, and alteration of microdomains in the nerve cell membrane should also be considered. Complex glycolipid environments in the cell membrane may govern the accessibility and avidity of antiganglioside antibodies for target gangliosides. Thus, the pathogenic effect of antiganglioside antibodies may depend on the local glycolipid environment in the nerve membrane, as well as on the antibody specificity.
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Spontaneous remyelination occurs in many early multiple sclerosis (MS) patients, however its capacity decreases as the disease becomes chronic. Even in those chronic MS patients, an enough number of oligodendrocyte precursor cells (OPCs) are preserved within the demyelinated lesions, suggesting that the differentiation arrest of oligodendroglial cells underlies the remyelination failure in chronic MS. We have previously reported that TIP30, a factor inhibiting nucleocytoplasmic transport within the cell, is responsible for the differentiation arrest in MS lesions. ⋯ Moreover, inflammatory conditions surrounding OPCs may be involved in the efficient remyelination in early MS lesions, alternative stimulatory factor may therefore be mandatory to induce OPC differentiation into oligodendrocytes within the chronic lesion. We have previously reported that targeting FcRγ protein on OPCs may stimulate their differentiation and consequently remyelination in the chronic lesions. A timely collaboration of these two approaches may be required for successful remyelination and neurological recovery in chronic MS patients.
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Case Reports
[Case of Lemierre syndrome associated with infectious cavernous sinus thrombosis and septic meningitis].
A 33-year-old man was admitted to our hospital because of right exophthalmos, diplopia and left neck pain. Neurological examination revealed lateral and inferior disturbance of his right eye movement and the meningeal irritation sign. Cerebrospinal fluid showed elevated polynuclear cells. ⋯ However, after his discharge, he was required re-antibiotics therapy because of septic embolus- induced multiple lung abscesses. Lemierre syndrome is characterized by disseminated abscesses and thrombophlebitis of the internal jugular vein after infection of the oropharynx. Because Lemierre syndrome is potentially life-threatening, early diagnosis and initiation of appropriate therapy are important.
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A 16-year-old male was admitted to our hospital because of fever, altered consciousness and subsequent tonic convulsions of upper and lower extremities. A head CT scan revealed evidence of diffuse brain edema. Novel influenza H1N1 viral RNA was detected in nasopharyngeal specimens by specific PCR examination. ⋯ Extensive disruption of astrocytic projections (clasmatodendrosis), which is indicative of acute encephalopathy, was detected by anti-glial fibrillary acidic protein (GFAP) immunostaining of brain tissue. This is the first autopsy case report of pandemic (H1N1) 2009 influenza virus-associated encephalopathy. The clinical course, laboratory profiles and pathological findings were similar to those of conventional seasonal influenza encephalopathy in children that are reported previously.
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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated polyradiculoneuropathy. The American Academy of Neurology criteria has been used widely in diagnoses, and has generated clinical and pathological information. Recently, the EFNS/PNS criteria revised the concept of conventional "typical CIDP" and "atypical CIDP", with atypical CIDP including five phenotypes: DADS (distal acquired demyelinating symmetric), MADSAM (multifocal acquired demyelinating sensory and motor), focal, pure motor, and pure sensory neuropathy. ⋯ These results suggest that "hybrid therapies", IVIg induction and corticosteroid maintenance, may be effective. A recent study showed that IVIg stabilizes axonal potentials and axonal membranes, and our group showed that juxtaparanodal TAG-1 may be associated with IVIg responsiveness. Although CIDP is a demyelinating disease, the involvement of axon or axon-myelin interactions should be considered.