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*Only for medical professionals to read and refer to the Neurological Archives: layer by layer analysis to improve clinical diagnosis and treatment thinking.
Anti-N-methyl-D-aspartate receptor (anti-NMDA receptor) encephalitis is an immune-mediated disease characterized by complex neuropsychiatric syndrome and positive anti-NMDA receptor antibodies in the cerebrospinal fluid.
The disease was first reported by Dalmau et al.
in 2007, and its annual incidence rate was 1.
5/1000,000.
The disease is more common in women, women: men=8:2.
The median age of onset was 21 years old, and the age range was 1-85 years old.
Figure 1: With the development of the disease, patients will have different symptoms at different times (quoted from Reference 1) ①Symptoms of viral infection before 0-1 weeks; ②Psychiatric symptoms group of 1-2 weeks: delusions, hallucinations, mania, easy Irritability, speech changes, confusion, nervousness, insomnia, and epilepsy; ③weeks to months Nervous system complications: dyskinesia, autonomic dysfunction, central hypopnea, epilepsy; ④months to years long-term functional impairment : Executive dysfunction, impulse disinhibition, sleep disturbance.
The clinical features of primary anti-NMDA receptor encephalitis 1.
It is more common in children and young people, and more women than men; 2.
Acute onset, which usually reaches a peak in 2 weeks to several weeks; 3.
There may be prodromal symptoms such as fever and headache; 4.
Mainly manifested as abnormal mental behavior, seizures, cognitive impairment, speech disorder/silence, movement disorder and consciousness disorder.
Autonomic nerve dysfunction can be manifested as sinus tachycardia, bradycardia, increased salivation, central hypopnea, hypotension, central fever, etc.
; 5.
Focal neurological deficits.
There are certain differences between children and adults: epilepsy, abnormal movement, insomnia, and irritability are more common in children; abnormal mental behavior is more common in adults.
In the course of the disease, adults show more frequent memory impairment and hypoventilation than children, and fewer focal neurological deficits and language or movement disorders.
The relationship between the secondary anti-NMDA receptor encephalitis and ovarian teratoma, the domestic professor Guan Hongzhi conducted a single-center retrospective study on anti-NMDA receptor encephalitis.
The study included 220 patients with anti-NMDA receptor encephalitis, 19.
5% had potential tumors, 100% of female tumor patients were teratomas, and only 1 male tumor patient was lung cancer.
Figure 2: The number distribution of female patients with or without ovarian teratoma at different ages (quoted from Reference 2) The pathogenesis of ovarian teratoma-related anti-NMDA receptor encephalitis is still unclear.
The NMDA receptor usually consists of NR1 (binding glycine) and NR2 (binding glutamate) subunits.
Research by Dalmau et al.
found that in the 5 examined tumors, there were nerve tissues that highly expressed the NR2 subunit and had an immune reaction with the patient's antibody.
Although the mechanism that triggers the immune response is still unclear, scholars speculate that the ectopic expression of the NR2 subunit of the nervous tissue contained in the teratoma helps to destroy the immune tolerance.
Figure 3: Imaging manifestations of ovarian teratoma: a cystic mass with fat or watery density, round or round, with clear boundaries, uniform cyst wall thickness, and calcification or ossification.
Among them, fat is a characteristic sign.
(Quoted from Reference 3) Figure 4: Intraoperative findings of ovarian teratoma (quoted from Reference 3) Figure 5: Pathological manifestations of ovarian teratoma: Bone tissue (asterisk), found in mature ovarian teratoma, Adipose tissue (filled asterisks), mature cartilage tissue (open asterisks), tracheocilial columnar epithelium (right arrow), dermis and its accessory tissues (filled triangles) (quoted from Reference 3) Figure 6: Pathology of the patient’s tumor Mature nerve tissue (asterisk) was found in the slices (quoted from Reference 3) MRI of the brain with three anti-NMDA receptor encephalitis.
35.
9% of patients with anti-NMDA receptor encephalitis showed positive MRI lesions.
It can be manifested as scattered cortex and subcortical spots of FLAIR and T2WI hyperintensity; some patients can see limbic system lesions, which can also extend beyond the limbic system.
A small number of patients also have imaging features of inflammatory demyelinating diseases of the central nervous system.
Figure 7: (A) White matter punctate FLAIR hyperintensity in the left frontal cortex; (B) focal FLAIR lesion on the right frontal lobe; (C) bilateral posterior ventricular white matter FLAIR hyperintensity; (D) left FLAIR hyperintensity in the lateral parietal cortex; (E) bilateral medial temporal lobe including the hippocampus FLAIR hyperintensity; (F) T1WI showed obvious brain atrophy (decreased brain volume) (quoted from Reference 4) four diagnostic criteria (1) the following 6 One or more of the main symptoms: ① abnormal mental behavior or cognitive impairment; ② speech disorder; ③ epileptic seizures; ④ dyskinesia/involuntary movement; ⑤ disturbance of consciousness; ⑥ autonomic dysfunction or central hypopnea .
(2) Anti-NMDA receptor antibody positive: the cerebrospinal fluid is positive (CBA method is recommended) (3) Exclusive five treatment process and prognosis Figure 8: Anti-NMDA receptor encephalitis treatment process (quoted from Reference 5) anti-NMDA receptor Patients with encephalitis should be removed as soon as possible once the ovarian teratoma is found.
However, the long-term recurrence rate of mature cystic teratoma after surgical resection is 4.
2%.
Young people (<30 years) or large cysts (diameter ≥8cm) or bilateral cysts or large central nervous system component expression rates are at higher risk of recurrence.
For young female patients (≥12 years old) who have not found a tumor, it is recommended to have a pelvic ultrasound examination every 6-12 months for 4 years after the onset of disease.
If autoimmune encephalitis is associated with malignant tumors, it is necessary to maintain immunotherapy during the anti-tumor treatment, and the first-line treatment is the main one.
80% of patients with anti-NMDA receptor encephalitis recovered well.
Early receiving immunotherapy and non-critically ill patients have a better prognosis.
The average treatment period in the intensive care unit is 1-2 months, and the fatality rate is 2.
9%-9.
5%.
A few patients need more than 2 years to fully recover. Reference materials: [1]An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models.
Lancet Neurol 2019 11;1811(11).
[2]Anti-NMDAR encephalitis A single-center, longitudinal study in China.
Neurol Neuroimmunol Neuroinflamm 2020 01; 71(1).
[3]Ovarian teratoma-associated anti-NMDAR encephalitis: a single-institute series of six patients from China.
Arch Gynecol Obstet 2020 Nov 20.
[4]Clinical and Magnetic Resonance Imaging Outcome Predictors in Pediatric Anti-N-Methyl-D-Aspartate Receptor Encephalitis.
Ann Neurol 2020 07; 881 (1).
[5] Expert consensus on Chinese autoimmune encephalitis.
Chinese Journal of Neurology 2017 2 (50): 91-98 .
Anti-N-methyl-D-aspartate receptor (anti-NMDA receptor) encephalitis is an immune-mediated disease characterized by complex neuropsychiatric syndrome and positive anti-NMDA receptor antibodies in the cerebrospinal fluid.
The disease was first reported by Dalmau et al.
in 2007, and its annual incidence rate was 1.
5/1000,000.
The disease is more common in women, women: men=8:2.
The median age of onset was 21 years old, and the age range was 1-85 years old.
Figure 1: With the development of the disease, patients will have different symptoms at different times (quoted from Reference 1) ①Symptoms of viral infection before 0-1 weeks; ②Psychiatric symptoms group of 1-2 weeks: delusions, hallucinations, mania, easy Irritability, speech changes, confusion, nervousness, insomnia, and epilepsy; ③weeks to months Nervous system complications: dyskinesia, autonomic dysfunction, central hypopnea, epilepsy; ④months to years long-term functional impairment : Executive dysfunction, impulse disinhibition, sleep disturbance.
The clinical features of primary anti-NMDA receptor encephalitis 1.
It is more common in children and young people, and more women than men; 2.
Acute onset, which usually reaches a peak in 2 weeks to several weeks; 3.
There may be prodromal symptoms such as fever and headache; 4.
Mainly manifested as abnormal mental behavior, seizures, cognitive impairment, speech disorder/silence, movement disorder and consciousness disorder.
Autonomic nerve dysfunction can be manifested as sinus tachycardia, bradycardia, increased salivation, central hypopnea, hypotension, central fever, etc.
; 5.
Focal neurological deficits.
There are certain differences between children and adults: epilepsy, abnormal movement, insomnia, and irritability are more common in children; abnormal mental behavior is more common in adults.
In the course of the disease, adults show more frequent memory impairment and hypoventilation than children, and fewer focal neurological deficits and language or movement disorders.
The relationship between the secondary anti-NMDA receptor encephalitis and ovarian teratoma, the domestic professor Guan Hongzhi conducted a single-center retrospective study on anti-NMDA receptor encephalitis.
The study included 220 patients with anti-NMDA receptor encephalitis, 19.
5% had potential tumors, 100% of female tumor patients were teratomas, and only 1 male tumor patient was lung cancer.
Figure 2: The number distribution of female patients with or without ovarian teratoma at different ages (quoted from Reference 2) The pathogenesis of ovarian teratoma-related anti-NMDA receptor encephalitis is still unclear.
The NMDA receptor usually consists of NR1 (binding glycine) and NR2 (binding glutamate) subunits.
Research by Dalmau et al.
found that in the 5 examined tumors, there were nerve tissues that highly expressed the NR2 subunit and had an immune reaction with the patient's antibody.
Although the mechanism that triggers the immune response is still unclear, scholars speculate that the ectopic expression of the NR2 subunit of the nervous tissue contained in the teratoma helps to destroy the immune tolerance.
Figure 3: Imaging manifestations of ovarian teratoma: a cystic mass with fat or watery density, round or round, with clear boundaries, uniform cyst wall thickness, and calcification or ossification.
Among them, fat is a characteristic sign.
(Quoted from Reference 3) Figure 4: Intraoperative findings of ovarian teratoma (quoted from Reference 3) Figure 5: Pathological manifestations of ovarian teratoma: Bone tissue (asterisk), found in mature ovarian teratoma, Adipose tissue (filled asterisks), mature cartilage tissue (open asterisks), tracheocilial columnar epithelium (right arrow), dermis and its accessory tissues (filled triangles) (quoted from Reference 3) Figure 6: Pathology of the patient’s tumor Mature nerve tissue (asterisk) was found in the slices (quoted from Reference 3) MRI of the brain with three anti-NMDA receptor encephalitis.
35.
9% of patients with anti-NMDA receptor encephalitis showed positive MRI lesions.
It can be manifested as scattered cortex and subcortical spots of FLAIR and T2WI hyperintensity; some patients can see limbic system lesions, which can also extend beyond the limbic system.
A small number of patients also have imaging features of inflammatory demyelinating diseases of the central nervous system.
Figure 7: (A) White matter punctate FLAIR hyperintensity in the left frontal cortex; (B) focal FLAIR lesion on the right frontal lobe; (C) bilateral posterior ventricular white matter FLAIR hyperintensity; (D) left FLAIR hyperintensity in the lateral parietal cortex; (E) bilateral medial temporal lobe including the hippocampus FLAIR hyperintensity; (F) T1WI showed obvious brain atrophy (decreased brain volume) (quoted from Reference 4) four diagnostic criteria (1) the following 6 One or more of the main symptoms: ① abnormal mental behavior or cognitive impairment; ② speech disorder; ③ epileptic seizures; ④ dyskinesia/involuntary movement; ⑤ disturbance of consciousness; ⑥ autonomic dysfunction or central hypopnea .
(2) Anti-NMDA receptor antibody positive: the cerebrospinal fluid is positive (CBA method is recommended) (3) Exclusive five treatment process and prognosis Figure 8: Anti-NMDA receptor encephalitis treatment process (quoted from Reference 5) anti-NMDA receptor Patients with encephalitis should be removed as soon as possible once the ovarian teratoma is found.
However, the long-term recurrence rate of mature cystic teratoma after surgical resection is 4.
2%.
Young people (<30 years) or large cysts (diameter ≥8cm) or bilateral cysts or large central nervous system component expression rates are at higher risk of recurrence.
For young female patients (≥12 years old) who have not found a tumor, it is recommended to have a pelvic ultrasound examination every 6-12 months for 4 years after the onset of disease.
If autoimmune encephalitis is associated with malignant tumors, it is necessary to maintain immunotherapy during the anti-tumor treatment, and the first-line treatment is the main one.
80% of patients with anti-NMDA receptor encephalitis recovered well.
Early receiving immunotherapy and non-critically ill patients have a better prognosis.
The average treatment period in the intensive care unit is 1-2 months, and the fatality rate is 2.
9%-9.
5%.
A few patients need more than 2 years to fully recover. Reference materials: [1]An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models.
Lancet Neurol 2019 11;1811(11).
[2]Anti-NMDAR encephalitis A single-center, longitudinal study in China.
Neurol Neuroimmunol Neuroinflamm 2020 01; 71(1).
[3]Ovarian teratoma-associated anti-NMDAR encephalitis: a single-institute series of six patients from China.
Arch Gynecol Obstet 2020 Nov 20.
[4]Clinical and Magnetic Resonance Imaging Outcome Predictors in Pediatric Anti-N-Methyl-D-Aspartate Receptor Encephalitis.
Ann Neurol 2020 07; 881 (1).
[5] Expert consensus on Chinese autoimmune encephalitis.
Chinese Journal of Neurology 2017 2 (50): 91-98 .
