TY  - JOUR
A1  - Jarius, Sven
A1  - Kleiter, Ingo
A1  - Ruprecht, Klemens
A1  - Asgari, Nasrin
A1  - Pitarokoili, Kalliopi
A1  - Borisow, Nadja
A1  - Hümmert, Martin W.
A1  - Trebst, Corinna
A1  - Pache, Florence
A1  - Winkelmann, Alexander
A1  - Beume, Lena-Alexandra
A1  - Ringelstein, Marius
A1  - Stich, Oliver
A1  - Aktas, Orhan
A1  - Korporal-Kuhnke, Mirjam
A1  - Schwarz, Alexander
A1  - Lukas, Carsten
A1  - Haas, Jürgen
A1  - Fechner, Kai
A1  - Buttmann, Mathias
A1  - Bellmann-Strobl, Judith
A1  - Zimmermann, Hanna
A1  - Brandt, Alexander U.
A1  - Franciotta, Diego
A1  - Schanda, Kathrin
A1  - Paul, Friedemann
A1  - Reindl, Markus
A1  - Wildemann, Brigitte
T1  - MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome
JF  - Journal of Neuroinflammation
N2  - 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.
KW  - Myelin oligodendrocyte glycoprotein (MOG) antibodies
KW  - MOG-IgG
KW  - Neuromyelitis optica spectrum disorders (NMOSD)
KW  - Brainstem encephalitis
KW  - Rhombencephalitis
KW  - Optic neuritis
KW  - Myelitis
KW  - Longitudinally extensive transverse myelitis (LETM)
KW  - Cerebellitis
KW  - Ataxia
KW  - Respiratory insufficiency
KW  - Intractable nausea and vomiting
KW  - Facial nerve palsy
KW  - Diplopia Internuclear ophthalmoplegia (INO)
KW  - Hearing loss
KW  - Aquaporin-4 antibodies (AQP4-Ig, NMO-IgG)G
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-165543
VL  - 13
IS  - 281
ER  -