MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome

Please always quote using this URN: urn:nbn:de:bvb:20-opus-165543
  • Background Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) are present in a subset of aquaporin-4 (AQP4)-IgG-negative patients with optic neuritis (ON) and/or myelitis. Little is known so far about brainstem involvement in MOG-IgG-positive patients. Objective To investigate the frequency, clinical and paraclinical features, course, outcome, and prognostic implications of brainstem involvement in MOG-IgG-positive ON and/or myelitis. Methods Retrospective case study. Results Among 50 patients with MOG-IgG-positive ON and/orBackground Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) are present in a subset of aquaporin-4 (AQP4)-IgG-negative patients with optic neuritis (ON) and/or myelitis. Little is known so far about brainstem involvement in MOG-IgG-positive patients. Objective To investigate the frequency, clinical and paraclinical features, course, outcome, and prognostic implications of brainstem involvement in MOG-IgG-positive ON and/or myelitis. Methods Retrospective case study. Results Among 50 patients with MOG-IgG-positive ON and/or myelitis, 15 (30 %) with a history of brainstem encephalitis were identified. All were negative for AQP4-IgG. Symptoms included respiratory insufficiency, intractable nausea and vomiting (INV), dysarthria, dysphagia, impaired cough reflex, oculomotor nerve palsy and diplopia, nystagmus, internuclear ophthalmoplegia (INO), facial nerve paresis, trigeminal hypesthesia/dysesthesia, vertigo, hearing loss, balance difficulties, and gait and limb ataxia; brainstem involvement was asymptomatic in three cases. Brainstem inflammation was already present at or very shortly after disease onset in 7/15 (47 %) patients. 16/21 (76.2 %) brainstem attacks were accompanied by acute myelitis and/or ON. Lesions were located in the pons (11/13), medulla oblongata (8/14), mesencephalon (cerebral peduncles; 2/14), and cerebellar peduncles (5/14), were adjacent to the fourth ventricle in 2/12, and periaqueductal in 1/12; some had concomitant diencephalic (2/13) or cerebellar lesions (1/14). MRI or laboratory signs of blood-brain barrier damage were present in 5/12. Cerebrospinal fluid pleocytosis was found in 11/14 cases, with neutrophils in 7/11 (3-34 % of all CSF white blood cells), and oligoclonal bands in 4/14. Attacks were preceded by acute infection or vaccination in 5/15 (33.3 %). A history of teratoma was noted in one case. The disease followed a relapsing course in 13/15 (87 %); the brainstem was involved more than once in 6. Immunosuppression was not always effective in preventing relapses. Interferon-beta was followed by new attacks in two patients. While one patient died from central hypoventilation, partial or complete recovery was achieved in the remainder following treatment with high-dose steroids and/or plasma exchange. Brainstem involvement was associated with a more aggressive general disease course (higher relapse rate, more myelitis attacks, more frequently supratentorial brain lesions, worse EDSS at last follow-up). Conclusions Brainstem involvement is present in around one third of MOG-IgG-positive patients with ON and/or myelitis. Clinical manifestations are diverse and may include symptoms typically seen in AQP4-IgG-positive neuromyelitis optica, such as INV and respiratory insufficiency, or in multiple sclerosis, such as INO. As MOG-IgG-positive brainstem encephalitis may take a serious or even fatal course, particular attention should be paid to signs or symptoms of additional brainstem involvement in patients presenting with MOG-IgG-positive ON and/or myelitis.show moreshow less

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Author: Sven Jarius, Ingo Kleiter, Klemens Ruprecht, Nasrin Asgari, Kalliopi Pitarokoili, Nadja Borisow, Martin W. Hümmert, Corinna Trebst, Florence Pache, Alexander Winkelmann, Lena-Alexandra Beume, Marius Ringelstein, Oliver Stich, Orhan Aktas, Mirjam Korporal-Kuhnke, Alexander Schwarz, Carsten Lukas, Jürgen Haas, Kai Fechner, Mathias Buttmann, Judith Bellmann-Strobl, Hanna Zimmermann, Alexander U. Brandt, Diego Franciotta, Kathrin Schanda, Friedemann Paul, Markus Reindl, Brigitte Wildemann
URN:urn:nbn:de:bvb:20-opus-165543
Document Type:Journal article
Faculties:Medizinische Fakultät / Neurologische Klinik und Poliklinik
Language:English
Parent Title (English):Journal of Neuroinflammation
Year of Completion:2016
Volume:13
Issue:281
Pagenumber:1-23
Source:Journal of Neuroinflammation (2016) 13:281
DOI:https://doi.org/10.1186/s12974-016-0719-z
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Tag:Aquaporin-4 antibodies (AQP4-Ig, NMO-IgG)G; Ataxia; Brainstem encephalitis; Cerebellitis; Diplopia Internuclear ophthalmoplegia (INO); Facial nerve palsy; Hearing loss; Intractable nausea and vomiting; Longitudinally extensive transverse myelitis (LETM); MOG-IgG; Myelin oligodendrocyte glycoprotein (MOG) antibodies; Myelitis; Neuromyelitis optica spectrum disorders (NMOSD); Optic neuritis; Respiratory insufficiency; Rhombencephalitis
Release Date:2020/05/19
Contributing Corporation:Optica Study Group (NEMOS)
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International