eprintid: 22076
rev_number: 12
eprint_status: archive
userid: 1589
dir: disk0/00/02/20/76
datestamp: 2016-11-02 13:02:03
lastmod: 2021-06-15 20:45:26
status_changed: 2016-11-02 13:02:03
type: article
metadata_visibility: show
creators_name: Jarius, Sven
creators_name: Kleiter, Ingo
creators_name: Ruprecht, Klemens
creators_name: Asgari, Nasrin
creators_name: Pitarokoili, Kalliopi
creators_name: Borisow, Nadja
creators_name: Hümmert, Martin W.
creators_name: Trebst, Corinna
creators_name: Pache, Florence
creators_name: Winkelmann, Alexander
creators_name: Beume, Lena-Alexandra
creators_name: Ringelstein, Marius
creators_name: Stich, Oliver
creators_name: Aktas, Orhan
creators_name: Korporal-Kuhnke, Mirjam
creators_name: Schwarz, Alexander
creators_name: Lukas, Carsten
creators_name: Haas, Jürgen
creators_name: Fechner, Kai
creators_name: Buttmann, Mathias
creators_name: Strobl, Judith
creators_name: Zimmermann, Hanna
creators_name: Brandt, Alexander U.
creators_name: Franciotta, Diego
creators_name: Schanda, Kathrin
creators_name: Paul, Friedemann
creators_name: Reindl, Markus
creators_name: Wildemann, Brigitte
title: MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome
subjects: ddc-610
divisions: i-712000
divisions: i-911100
abstract: 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.
date: 2016
publisher: BioMed Central
id_scheme: DOI
ppn_swb: 1653760699
own_urn: urn:nbn:de:bsz:16-heidok-220764
language: eng
bibsort: JARIUSSVENMOGIGGINNM2016
full_text_status: public
publication: Journal of Neuroinflammation
volume: 13
number: 281
place_of_pub: London
pagerange: 1-23
issn: 1742-2094
citation:   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     
document_url: https://archiv.ub.uni-heidelberg.de/volltextserver/22076/1/12974_2016_Article_719.pdf