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*Only for medical professionals to read for reference Unilateral cortical encephalitis + epilepsy? Don't forget the cause! In recent years, the role of myelin oligodendrocyte glycoprotein (Myelin Oligodendrocyte Glycoprotein, MOG) antibody in central nervous system inflammatory demyelinating diseases has received extensive attention
.
MOG antibody disease has various clinical phenotypes, including optic neuritis, myelitis, encephalitis, aseptic meningitis, acute disseminated encephalomyelitis, etc.
8 major clinical phenotypes in one article)
.
MOG antibody-related cortical encephalitis (Cerebral Cortical Encephalitis, CCE) was first described by Ogawa et al.
in 2017 [1].
As a new clinical phenotype, how should it be diagnosed? Let's take a look together
.
Case data: A 23-year-old male patient was admitted to the hospital with “fever, acute aphasia, and weakness of the right limb”
.
The patient had headache symptoms 10 days ago
.
Brain MRI showed: T2 Flair high signal with swelling in the left frontal cortex, T2 Flair high signal near the brain sulcus, pial enhancement; T2WI showed no obvious abnormality; DWI showed high cortical signal but subarachnoid space remained, ADC slightly low signal Prompt that the diffusion is limited (see the figure below)
.
Figure 1: The patient's brain MRI routine EEG shows the slow wave of the left cerebral hemisphere
.
He was suspected of having infectious encephalitis, and began to receive empirical anti-infective treatment, and was given phenytoin for epilepsy
.
Lumbar puncture showed that the pressure of the cerebrospinal fluid was 190mmH2O, the white blood cell count was 85/ul (64% of lymphocytes), the cerebrospinal fluid protein was increased, and the cerebrospinal fluid/blood sugar ratio was normal
.
Infectious pathogens, autoimmune encephalitis and paraneoplastic encephalitis antibodies were negative
.
On the third day of admission, the ambulatory electroencephalogram recorded a seizure originating from the left cerebral hemisphere.
The clinical manifestations were aphasia, head shifting to the right, and right arm twitching, which increased the anti-epileptic effect of levetiracetam
.
On the 4th day of admission, the patient presented with refractory intracranial hypertension
.
Lumbar puncture showed that the pressure of the cerebrospinal fluid was increased (310mmH2O), the white blood cell count was 599/ul (the percentage of lymphocytes was 34%, the percentage of centrifugal cells was 49%), the protein of cerebrospinal fluid was increased, and the oligoclonal zone (-)
.
On the 10th day of admission, the lumbar puncture was rechecked due to severe headache, the pressure of cerebrospinal fluid was 390mmH2O, the white blood cell count was 180/ul (lymphocyte percentage 71%), and the protein of cerebrospinal fluid increased
.
Considering that autoimmune encephalitis may be given intravenous injection of methylprednisolone 1g, the patient's headache symptoms disappeared on the 15th day of admission, no epilepsy occurred and there was no neurological deficit
.
Four months later, re-examination of the brain MRI showed that the initial abnormal signal subsided, and only weak T2 Flair high signal was seen in the left frontal cortex and sulcus
.
However, imaging examination revealed new high-intensity lesions in the pons and subcortex (see figure below)
.
Figure 2: The result of CBA detection of serum MOG-IgG in the newly-emerging hyperintensity lesions in the pons and subcortex was positive
.
Retrospective testing of the patient's initial serum and cerebrospinal fluid MOG-IgG is still positive
.
Discussion on MOG antibody-related CCE reports are rare, and it is considered to be a new clinical phenotype of MOG antibody disease
.
MOG antibody-related encephalitis often involves supratentorial white matter, pericortical gray matter, cerebellum, midbrain, medulla oblongata, and corpus callosum, while CCE is indeed very rare [2]
.
Budhram et al.
systematically reviewed 20 cases of MOG-related CCE.
The most common symptom was seizures (85%), followed by headache (70%), fever (65%) and cortical symptoms (55%) [3]
.
The vast majority of patients have at least the above two symptoms
.
In these patients, CCE is often confined to one side, and bilateral involvement is rare
.
In addition, 30% of patients have T2Flair hyperintensity near the sulci and pia mater enhancement
.
One side of CCE with seizures is considered to be a specific clinical phenotype of MOG antibody disease
.
▌ There has been controversy over whether anti-MOG antibodies are pathogenic antibodies or bystanders in CCE
.
In the past, there have been few studies on pathological biopsy of MOG antibody-related CCE
.
Brain biopsy showed lymphocyte infiltration in the subarachnoid space and brain parenchyma and lymphocyte infiltration around blood vessels
.
However, no axonal, astrocyte damage and typical demyelinating lesions were seen [4]
.
In addition, despite extensive cortical involvement, there was no increase in CSF myelin basic protein
.
Therefore, some researchers believe that anti-MOG antibodies are just bystanders in CCE, and the antibodies against neurons that may coexist are the real disease-causing antibodies [5]
.
When unilateral authentication ▌ skin T2Flair high signal appears diagnosis requires epilepsy induced abnormal brain MRI, Rasmussen encephalitis, Creutzfeldt-Jakob disease (CJD) were identified and the like
.
Epilepsy-induced cranial MRI abnormalities are usually located in the subcortex, subcortical white matter, corpus callosum, basal ganglia, and hippocampus, and DWI diffusion is obviously limited
.
In patients with MOG antibody-related CCE, DWI diffusion limitation is often not obvious or manifests as mild diffusion limitation
.
Rasmussen encephalitis is characterized by refractory epilepsy, progressive hemiplegia, and cognitive decline
.
In the chronic stage of the disease, one side of the cerebral cortex is atrophy, and the effect of hormone therapy is not obvious
.
Sporadic CJD is a fatal degenerative disease of the nervous system.
The patient presents with rapidly progressive dementia.
The typical DWI manifestations are cortical silk sign and double basal ganglia hyperintensity
.
References: [1] Ogawa R, Nakashima I, Takahashi T, Kaneko K, Akaishi T et al (2017) MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy.
Neurol Neuroimmunol Neuroinflamm 16; 4 (2): e322.
doi:10.
1212/NXI.
0000000000000322.
[2]Tao R,Qin C,Chen M,Yu HH,Wu LJ,Bu BT,Tian DS (2020) Unilateral cerebral cortical encephalitis with epilepsy: a possible special phenotype of MOG antibody -associated disorders.
Int J Neurosci 130(11):1161-1165.
doi:10.
1080/00207454.
2020.
1720676.
[3] Budhram A, Mirian A, Le C, Hosseini-Moghaddam SM, Sharma M, Nicolle MW et al (2019 ) Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES):characterization of a distinct clinico-radiographic syndrome.
J Neurol 266(10):2481-2487.
doi:10.
1007/s00415-019-09440- 8.
[4]Ikeda T,Yamada K,Ogawa R,Takai Y,Kaneko K et al (2018) The pathological features of MOG antibody-positive cerebral cortical encephalitis as a new spectrum associated with MOG antibodies:A case report.
J Neurol Sci 15;392:113-115.
doi:10.
1016/j.
jns.
2018.
06.
028.
[5]Zhou L,ZhangBao J,Li H,Li X,Huang Y et al (2017) Cerebral cortical encephalitis followed by recurrent CNS demyelination in a patient with concomitant anti-MOG and anti-NMDA receptor antibodies.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
.
MOG antibody disease has various clinical phenotypes, including optic neuritis, myelitis, encephalitis, aseptic meningitis, acute disseminated encephalomyelitis, etc.
8 major clinical phenotypes in one article)
.
MOG antibody-related cortical encephalitis (Cerebral Cortical Encephalitis, CCE) was first described by Ogawa et al.
in 2017 [1].
As a new clinical phenotype, how should it be diagnosed? Let's take a look together
.
Case data: A 23-year-old male patient was admitted to the hospital with “fever, acute aphasia, and weakness of the right limb”
.
The patient had headache symptoms 10 days ago
.
Brain MRI showed: T2 Flair high signal with swelling in the left frontal cortex, T2 Flair high signal near the brain sulcus, pial enhancement; T2WI showed no obvious abnormality; DWI showed high cortical signal but subarachnoid space remained, ADC slightly low signal Prompt that the diffusion is limited (see the figure below)
.
Figure 1: The patient's brain MRI routine EEG shows the slow wave of the left cerebral hemisphere
.
He was suspected of having infectious encephalitis, and began to receive empirical anti-infective treatment, and was given phenytoin for epilepsy
.
Lumbar puncture showed that the pressure of the cerebrospinal fluid was 190mmH2O, the white blood cell count was 85/ul (64% of lymphocytes), the cerebrospinal fluid protein was increased, and the cerebrospinal fluid/blood sugar ratio was normal
.
Infectious pathogens, autoimmune encephalitis and paraneoplastic encephalitis antibodies were negative
.
On the third day of admission, the ambulatory electroencephalogram recorded a seizure originating from the left cerebral hemisphere.
The clinical manifestations were aphasia, head shifting to the right, and right arm twitching, which increased the anti-epileptic effect of levetiracetam
.
On the 4th day of admission, the patient presented with refractory intracranial hypertension
.
Lumbar puncture showed that the pressure of the cerebrospinal fluid was increased (310mmH2O), the white blood cell count was 599/ul (the percentage of lymphocytes was 34%, the percentage of centrifugal cells was 49%), the protein of cerebrospinal fluid was increased, and the oligoclonal zone (-)
.
On the 10th day of admission, the lumbar puncture was rechecked due to severe headache, the pressure of cerebrospinal fluid was 390mmH2O, the white blood cell count was 180/ul (lymphocyte percentage 71%), and the protein of cerebrospinal fluid increased
.
Considering that autoimmune encephalitis may be given intravenous injection of methylprednisolone 1g, the patient's headache symptoms disappeared on the 15th day of admission, no epilepsy occurred and there was no neurological deficit
.
Four months later, re-examination of the brain MRI showed that the initial abnormal signal subsided, and only weak T2 Flair high signal was seen in the left frontal cortex and sulcus
.
However, imaging examination revealed new high-intensity lesions in the pons and subcortex (see figure below)
.
Figure 2: The result of CBA detection of serum MOG-IgG in the newly-emerging hyperintensity lesions in the pons and subcortex was positive
.
Retrospective testing of the patient's initial serum and cerebrospinal fluid MOG-IgG is still positive
.
Discussion on MOG antibody-related CCE reports are rare, and it is considered to be a new clinical phenotype of MOG antibody disease
.
MOG antibody-related encephalitis often involves supratentorial white matter, pericortical gray matter, cerebellum, midbrain, medulla oblongata, and corpus callosum, while CCE is indeed very rare [2]
.
Budhram et al.
systematically reviewed 20 cases of MOG-related CCE.
The most common symptom was seizures (85%), followed by headache (70%), fever (65%) and cortical symptoms (55%) [3]
.
The vast majority of patients have at least the above two symptoms
.
In these patients, CCE is often confined to one side, and bilateral involvement is rare
.
In addition, 30% of patients have T2Flair hyperintensity near the sulci and pia mater enhancement
.
One side of CCE with seizures is considered to be a specific clinical phenotype of MOG antibody disease
.
▌ There has been controversy over whether anti-MOG antibodies are pathogenic antibodies or bystanders in CCE
.
In the past, there have been few studies on pathological biopsy of MOG antibody-related CCE
.
Brain biopsy showed lymphocyte infiltration in the subarachnoid space and brain parenchyma and lymphocyte infiltration around blood vessels
.
However, no axonal, astrocyte damage and typical demyelinating lesions were seen [4]
.
In addition, despite extensive cortical involvement, there was no increase in CSF myelin basic protein
.
Therefore, some researchers believe that anti-MOG antibodies are just bystanders in CCE, and the antibodies against neurons that may coexist are the real disease-causing antibodies [5]
.
When unilateral authentication ▌ skin T2Flair high signal appears diagnosis requires epilepsy induced abnormal brain MRI, Rasmussen encephalitis, Creutzfeldt-Jakob disease (CJD) were identified and the like
.
Epilepsy-induced cranial MRI abnormalities are usually located in the subcortex, subcortical white matter, corpus callosum, basal ganglia, and hippocampus, and DWI diffusion is obviously limited
.
In patients with MOG antibody-related CCE, DWI diffusion limitation is often not obvious or manifests as mild diffusion limitation
.
Rasmussen encephalitis is characterized by refractory epilepsy, progressive hemiplegia, and cognitive decline
.
In the chronic stage of the disease, one side of the cerebral cortex is atrophy, and the effect of hormone therapy is not obvious
.
Sporadic CJD is a fatal degenerative disease of the nervous system.
The patient presents with rapidly progressive dementia.
The typical DWI manifestations are cortical silk sign and double basal ganglia hyperintensity
.
References: [1] Ogawa R, Nakashima I, Takahashi T, Kaneko K, Akaishi T et al (2017) MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy.
Neurol Neuroimmunol Neuroinflamm 16; 4 (2): e322.
doi:10.
1212/NXI.
0000000000000322.
[2]Tao R,Qin C,Chen M,Yu HH,Wu LJ,Bu BT,Tian DS (2020) Unilateral cerebral cortical encephalitis with epilepsy: a possible special phenotype of MOG antibody -associated disorders.
Int J Neurosci 130(11):1161-1165.
doi:10.
1080/00207454.
2020.
1720676.
[3] Budhram A, Mirian A, Le C, Hosseini-Moghaddam SM, Sharma M, Nicolle MW et al (2019 ) Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES):characterization of a distinct clinico-radiographic syndrome.
J Neurol 266(10):2481-2487.
doi:10.
1007/s00415-019-09440- 8.
[4]Ikeda T,Yamada K,Ogawa R,Takai Y,Kaneko K et al (2018) The pathological features of MOG antibody-positive cerebral cortical encephalitis as a new spectrum associated with MOG antibodies:A case report.
J Neurol Sci 15;392:113-115.
doi:10.
1016/j.
jns.
2018.
06.
028.
[5]Zhou L,ZhangBao J,Li H,Li X,Huang Y et al (2017) Cerebral cortical encephalitis followed by recurrent CNS demyelination in a patient with concomitant anti-MOG and anti-NMDA receptor antibodies.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
Mult Scler Relat Disord 18:90-92.
doi:10.
1016/j.
msard.
2017.
09.
023.
