PMID- 28601810
OWN - NLM
STAT- MEDLINE
DCOM- 20170911
LR  - 20190127
IS  - 1468-330X (Electronic)
IS  - 0022-3050 (Linking)
VI  - 88
IP  - 9
DP  - 2017 Sep
TI  - Pattern of ocular involvement in myasthenia gravis with MuSK antibodies.
PG  - 761-763
LID - 10.1136/jnnp-2017-315782 [doi]
AB  - BACKGROUND: Myasthenia gravis (MG) with antibodies to the muscle-specific kinase 
      (MuSK) has a characteristic phenotype. Ocular manifestations have not been 
      systematically evaluated. OBJECTIVE: To investigate the features of extrinsic 
      ocular muscle involvement in patients with MuSK-MG. METHODS: We retrospectively 
      evaluated the prevalence, time of onset, clinical pattern and outcome of ocular 
      symptoms in 82 patients with a clinical follow-up >/=2 years. RESULTS: Ocular 
      manifestations were observed in 79 patients (96.4%) and were the presenting 
      symptoms in 48 (58.5%). Intermittent diplopia with subtle ophthalmoparesis was 
      the most common complaint, ptosis was generally symmetrical and conjugated gaze 
      paresis occurred in 35% of the patients. Ocular manifestations responded well to 
      prednisone and partially to symptomatic treatment. A few patients developed 
      chronic symmetrical ophthalmoparesis, associated with persistent weakness in 
      other muscle groups. All patients with ocular presentation progressed to 
      generalised disease, though weakness spread to other muscle groups was 
      considerably delayed in a few cases. CONCLUSIONS: In MG with antibodies to MuSK, 
      ocular manifestations were as frequent as in other disease subtypes. Symmetrical 
      ophthalmoparesis with conjugated gaze limitation was rather common and associated 
      with low functional disability. A proportion of these patients developed chronic 
      eye muscle paresis.
CI  - (c) Article author(s) (or their employer(s) unless otherwise stated in the text of 
      the article) 2017. All rights reserved. No commercial use is permitted unless 
      otherwise expressly granted.
FAU - Evoli, Amelia
AU  - Evoli A
AD  - Institute of Neurology, Catholic University, Fondazione Policlinico A. Gemelli, 
      Rome, Italy.
FAU - Alboini, Paolo E
AU  - Alboini PE
AD  - Institute of Neurology, Catholic University, Fondazione Policlinico A. Gemelli, 
      Rome, Italy.
FAU - Iorio, Raffaele
AU  - Iorio R
AD  - Institute of Neurology, Catholic University, Fondazione Policlinico A. Gemelli, 
      Rome, Italy.
FAU - Damato, Valentina
AU  - Damato V
AD  - Institute of Neurology, Catholic University, Fondazione Policlinico A. Gemelli, 
      Rome, Italy.
FAU - Bartoccioni, Emanuela
AU  - Bartoccioni E
AD  - Institute of General Pathology, Catholic University, Fondazione Policlinico A. 
      Gemelli, Rome, Italy.
LA  - eng
PT  - Journal Article
DEP - 20170610
PL  - England
TA  - J Neurol Neurosurg Psychiatry
JT  - Journal of neurology, neurosurgery, and psychiatry
JID - 2985191R
RN  - 0 (Anti-Inflammatory Agents)
RN  - 0 (Autoantibodies)
RN  - 0 (Receptors, Cholinergic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Anti-Inflammatory Agents/therapeutic use
MH  - *Autoantibodies
MH  - Child
MH  - Child, Preschool
MH  - Diplopia/epidemiology
MH  - Eye
MH  - Female
MH  - Humans
MH  - Male
MH  - Myasthenia Gravis/*diagnosis/*immunology
MH  - Ophthalmoplegia/epidemiology
MH  - Prednisone/therapeutic use
MH  - *Receptor Protein-Tyrosine Kinases
MH  - *Receptors, Cholinergic
MH  - Retrospective Studies
OTO - NOTNLM
OT  - MuSK antibodies
OT  - immunosuppression
OT  - myasthenia gravis
OT  - ocular myasthenia
OT  - ophthalmoparesis
COIS- Competing interests: None declared.
EDAT- 2017/06/12 06:00
MHDA- 2017/09/12 06:00
CRDT- 2017/06/12 06:00
PHST- 2017/02/03 00:00 [received]
PHST- 2017/04/10 00:00 [revised]
PHST- 2017/04/11 00:00 [accepted]
PHST- 2017/06/12 06:00 [pubmed]
PHST- 2017/09/12 06:00 [medline]
PHST- 2017/06/12 06:00 [entrez]
AID - jnnp-2017-315782 [pii]
AID - 10.1136/jnnp-2017-315782 [doi]
PST - ppublish
SO  - J Neurol Neurosurg Psychiatry. 2017 Sep;88(9):761-763. doi: 
      10.1136/jnnp-2017-315782. Epub 2017 Jun 10.