title: MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome
creator: Jarius, Sven
creator: Kleiter, Ingo
creator: Ruprecht, Klemens
creator: Asgari, Nasrin
creator: Pitarokoili, Kalliopi
creator: Borisow, Nadja
creator: Hümmert, Martin W.
creator: Trebst, Corinna
creator: Pache, Florence
creator: Winkelmann, Alexander
creator: Beume, Lena-Alexandra
creator: Ringelstein, Marius
creator: Stich, Oliver
creator: Aktas, Orhan
creator: Korporal-Kuhnke, Mirjam
creator: Schwarz, Alexander
creator: Lukas, Carsten
creator: Haas, Jürgen
creator: Fechner, Kai
creator: Buttmann, Mathias
creator: Strobl, Judith
creator: Zimmermann, Hanna
creator: Brandt, Alexander U.
creator: Franciotta, Diego
creator: Schanda, Kathrin
creator: Paul, Friedemann
creator: Reindl, Markus
creator: Wildemann, Brigitte
subject: ddc-610
subject: 610 Medical sciences Medicine
description: 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/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.
publisher: BioMed Central
date: 2016
type: Article
type: info:eu-repo/semantics/article
type: NonPeerReviewed
format: application/pdf
identifier: https://archiv.ub.uni-heidelberg.de/volltextserverhttps://archiv.ub.uni-heidelberg.de/volltextserver/22076/1/12974_2016_Article_719.pdf
identifier: DOI:
identifier: urn:nbn:de:bsz:16-heidok-220764
identifier:   Jarius, Sven ; Kleiter, Ingo ; Ruprecht, Klemens ; Asgari, Nasrin ; Pitarokoili, Kalliopi ; Borisow, Nadja ; Hümmert, Martin W. ; Trebst, Corinna ; Pache, Florence ; Winkelmann, Alexander ; Beume, Lena-Alexandra ; Ringelstein, Marius ; Stich, Oliver ; Aktas, Orhan ; Korporal-Kuhnke, Mirjam ; Schwarz, Alexander ; Lukas, Carsten ; Haas, Jürgen ; Fechner, Kai ; Buttmann, Mathias ; Strobl, Judith ; Zimmermann, Hanna ; Brandt, Alexander U. ; Franciotta, Diego ; Schanda, Kathrin ; Paul, Friedemann ; Reindl, Markus ; Wildemann, Brigitte  (2016) MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome.  Journal of Neuroinflammation, 13 (281).  pp. 1-23.  ISSN 1742-2094     
relation: https://archiv.ub.uni-heidelberg.de/volltextserver/22076/
rights: info:eu-repo/semantics/openAccess
rights: Please see front page of the work (Sorry, Dublin Core plugin does not recognise license id)
language: eng