Clinical and Experimental Neuroimmunology最新文献

Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating immune-mediated disease characterized by bilateral and confluent lesions in white matter (WM), with an acute onset. This condition may arise due to a myriad of etiological factors, encompassing mainly vaccines and viral infections. This case report describes a 39-y-old patient who presented with a sudden onset of fever, confusion, and reduced level of consciousness, associated with paraparesis in the lower limbs and urinary retention, 2 d before admission to the neurological emergency department. The work-up included analysis of the cerebrospinal fluid (CSF), which showed 1.6 cells/mm³ and elevated proteins (91 g/dL); in addition to magnetic resonance imaging (MRI) of the brain and the spinal cord, in which hyperintense ovoid lesions with asymmetrical and bilateral distribution in the WM and basal ganglia were observed in the T2 and FLAIR. Later, chikungunya virus was detected in a molecular viral panel in the CSF. The patient exhibited an improvement radiologically, and in his condition following pulse with methylprednisolone and intravenous immunoglobulin therapy, and 40 mg of prednisone was prescribed for management during outpatient follow-up. This study highlights arbovirus infections as a possible cause of acute neurological conditions, involving both the brain and the spinal cord. Furthermore, the findings observed in the report were compared with those described in the literature, including other arboviruses. In conclusion, it was observed that the majority of patients responded to treatment with corticosteroids or immunoglobulins, with some neurological deficits eventually persisting. Therefore, more studies are needed to better investigate therapeutic options.

Autoimmune nodopathy (AN) is characterized by the presence of autoantibodies targeting molecules essential for saltatory conduction in myelinated nerves. Clinical manifestations of AN show similarities with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including progressive and symmetrical sensorimotor deficits with electrophysiological demyelinating features in nerve conduction studies. Although no common autoantibodies have yet been identified in CIDP, AN is characterized by autoantibodies that primarily target specific molecular complex structures represented by the Ranvier node and paranode. Furthermore, these autoantibodies, such as neurofascin-155 (NF155), contactin-1 (CNTN1), contactin-related protein 1 (Caspr1), and the CNTN1/Caspr1 complex, are illustrative examples of such autoantibodies. They can disrupt the septal-like junction of paranodes without triggering cellular immune responses. AN manifests uniquely with symptoms such as ataxia, tremors, and markedly high cerebrospinal fluid (CSF) protein levels, often accompanied by nerve root and cranial nerve hypertrophy. Notably, this condition is resistant to immunotherapies typically effective against CIDP, including intravenous immunoglobulin therapy (IVIg). Current evidence suggests that B-cell depletion therapies, such as rituximab, could benefit AN treatment. Since it has been suggested that existing treatments for CIDP may be effective in cases of autoantibody positivity of subclasses other than IgG4, CIDP that is resistant to conventional therapy requires novel therapeutic strategies that take into account the possibility of IgG4 autoantibodies.