ECTRIMS eLearning

Adult MOG-IgG-positive, benign, unilateral, cerebral cortical encephalitis with epileptic seizure
Author(s): ,
R Ogawa
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
I Nakashima
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
T Takahashi
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
H Ono
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
K Kaneko
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
T Akaishi
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
K Kurosawa
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
Y Takai
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
D.K Sato
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan;Brain Institute, The Pontifical Catholic University of Rio Grande do Sul, Rio de Janeiro, Brazil
,
S Nishiyama
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
T Misu
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
,
K Fujihara
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan;Department of Multiple Sclerosis Therapeutics, Fukushima Medical University, Fukushimashi, Japan
M Aoki
Affiliations:
Department of Neurology, Tohoku University, School of Medicine, Sendaishi, Japan
ECTRIMS Learn. Ogawa R. 09/14/16; 145442; EP1347
Ryo Ogawa
Ryo Ogawa
Contributions
Abstract

Abstract: EP1347

Type: ePoster

Abstract Category: Clinical aspects of MS - MS Variants

Objective: To describe the clinical, radiological and laboratory features of adult patients with encephalitis positive for myelin oligodendrocyte glycoprotein-IgG (MOG-IgG).

Background: MOG is a glycoprotein located on the myelin sheath of the central nervous system (CNS). Recently, anti-MOG antibody had been detected in the serum of various CNS demyelinating diseases including neuromyelitis optica spectrum disorder and multiphasic acute disseminated encephalomyelitis.

Design and methods: We tested for IgG1 subclass of MOG-IgG using a cell-based assay in consecuitive 24 adult patients with encephalitis of unknown etiology seen at Tohoku University Hospital, Japan between 2008 and 2014. The clinical, radiological and laboratory features of MOG-IgG-positive cases were retrospectively analyzed.

Results: Total 4 male case were MOG-IgG positive (age median 37, range 23-39). The major symptom of 4 cases was generalized epileptic seizure. Three patients exhibited emotional behavior, two developed optic neuritis, and one had dysuria in addition. In all cases, brain MRI showed characteristic unilateral cerebral cortical FLAIR-high intensity lesions, which corresponded to hyperperfusion in SPECT study. Spinal cord lesions were not detected in any of 4 cases. Cerebrospinal fluid (CSF) study showed moderate mononuclear pleocytosis and mildly elevated total protein level but normal myelin basic protein concentration. Oligoclonal IgG bands were negative in all. Antibodies against aquaporin-4, glutamete receptor, and voltage-gated potassium channel were negative in both sera and CSF of all the cases.

Conclusion: We have detected MOG-IgG in patients with steroid responsive autoimmune encephalitis with unique clinical features. Although it is unknown whether the MOG-IgG is truly pathogenic, it may have the potential to be a specific marker for the autoimmune encephalitis with good prognosis.

Disclosure:

R. Ogawa: nothing to disclosure

I. Nakashima: nothing to disclosure

T. Takahashi: nothing to disclosure

H. Ono: nothing to disclosure

K. Kaneko: nothing to disclosure

T. Akaishi: nothing to disclosure

K. Kurosawa: nothing to disclosure

Y. Takai: nothing to disclosure

D.K. Sato: nothing to disclosure

S. Nishiyama: nothing to disclosure

T. Misu: nothing to disclosure

K. Fujihara: nothing to disclosure

M. Aoki: nothing to disclosure

Abstract: EP1347

Type: ePoster

Abstract Category: Clinical aspects of MS - MS Variants

Objective: To describe the clinical, radiological and laboratory features of adult patients with encephalitis positive for myelin oligodendrocyte glycoprotein-IgG (MOG-IgG).

Background: MOG is a glycoprotein located on the myelin sheath of the central nervous system (CNS). Recently, anti-MOG antibody had been detected in the serum of various CNS demyelinating diseases including neuromyelitis optica spectrum disorder and multiphasic acute disseminated encephalomyelitis.

Design and methods: We tested for IgG1 subclass of MOG-IgG using a cell-based assay in consecuitive 24 adult patients with encephalitis of unknown etiology seen at Tohoku University Hospital, Japan between 2008 and 2014. The clinical, radiological and laboratory features of MOG-IgG-positive cases were retrospectively analyzed.

Results: Total 4 male case were MOG-IgG positive (age median 37, range 23-39). The major symptom of 4 cases was generalized epileptic seizure. Three patients exhibited emotional behavior, two developed optic neuritis, and one had dysuria in addition. In all cases, brain MRI showed characteristic unilateral cerebral cortical FLAIR-high intensity lesions, which corresponded to hyperperfusion in SPECT study. Spinal cord lesions were not detected in any of 4 cases. Cerebrospinal fluid (CSF) study showed moderate mononuclear pleocytosis and mildly elevated total protein level but normal myelin basic protein concentration. Oligoclonal IgG bands were negative in all. Antibodies against aquaporin-4, glutamete receptor, and voltage-gated potassium channel were negative in both sera and CSF of all the cases.

Conclusion: We have detected MOG-IgG in patients with steroid responsive autoimmune encephalitis with unique clinical features. Although it is unknown whether the MOG-IgG is truly pathogenic, it may have the potential to be a specific marker for the autoimmune encephalitis with good prognosis.

Disclosure:

R. Ogawa: nothing to disclosure

I. Nakashima: nothing to disclosure

T. Takahashi: nothing to disclosure

H. Ono: nothing to disclosure

K. Kaneko: nothing to disclosure

T. Akaishi: nothing to disclosure

K. Kurosawa: nothing to disclosure

Y. Takai: nothing to disclosure

D.K. Sato: nothing to disclosure

S. Nishiyama: nothing to disclosure

T. Misu: nothing to disclosure

K. Fujihara: nothing to disclosure

M. Aoki: nothing to disclosure

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