[Cranial nerve palsies: ganglioside autoantibodies indicating an immunoneuropathy]. / Isolierte Hirnnerven-Läsionen: Gangliosid-Autoantikörper als Hinweis auf Immunneuropathie.

BACKGROUND: Cranial neuropathies are not infrequent and need a broad differential diagnostic approach. Etiologically autoimmune processes have to be considered. METHOD: In five patients with cranial neuropathy (in two cases lesion of the abducens nerve, in one case bilateral facial palsy, in one case abducens, facial and bilateral vestibular lesion, in one case bilateral glossopharyngeal lesion) IgG and IgM autoantibodies directed against most relevant gangliosides were quantitatively analyzed (Bühlmann Laboratories AG, Schönenbuch, Switzerland). All patients underwent lumbar puncture and cranial imaging. RESULTS: Trauma, tumor, elevated intracranial pressure and vascular lesion could be excluded. In one patient varicella infection as a cofactor was probable, in the remaining patients direct infection could be excluded. In one patient a prominent cyto-albumin dissociation of the CSF was found. In all patients autoantibodies directed against gangliosides were detected with positive antibodies against GD1a and GD1b in all patients. Prior to the cranial neuropathy one patient reported an infection of the upper airway and one reported an infection of the gastrointestinal tract. One patient was treated with steroids and one patient with intravenous immunoglobulin, the other patients were been carefully observed. All patients recovered well. CONCLUSION: Autoimmune processes with positive autoantibodies directed against gangliosides can be found in cranial neuropathy. Based on these observations an immunomodulating therapy should be considered.

Hemichorea Associated with CASPR2 Antibody.

This letter was written in response to: Ramdhani RA, Frucht SJ. Isolated chorea associated with LGI1 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8MG7MFC.