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*Only for medical professionals to read and refer to this case is not easy! A case show of young teachers jointly produced by the "Medical Circle" and the Department of Neurology of Xiangya Hospital-"Refreshing Youth!" 》Has been put on the doctor station.
Among them, Mr.
Xie Yuanyuan brought us a lecture named "The Gloves of Thanos".
The content is wonderful, let us learn together! Case review: A 36-year-old man, married, was admitted to the hospital in September 2020 due to "paroxysmal right upper limb stiffness for 7 months and aggravation in April"
.
Starting from February 2020, the patient has been observed by his family members to have paroxysmal stiffness of the right upper limb without obvious inducement.
Each attack lasts for 1-2 seconds to half an hour, with repeated attacks.
It is obvious when forced, lying down, and tense, on the right side.
It is relieved when lying down and relaxed, and disappears when sleeping at night
.
As of May 2020, the patient's symptoms gradually worsened, and the stiffness occurred frequently, lasting for a few minutes to about 1 hour, and developing to the right neck and right chest and back, accompanied by profuse upper body sweating
.
A head MRI examination in a local hospital showed "a few ischemic foci on both cerebral hemispheres" and was given oral treatment with "clonazepam 1 mg bid + propranolol 1 mg bid", but the control was not good
.
In September 2020, I went to the Department of Neurology, Xiangya Hospital of Central South University.
The outpatient department was admitted to the ward with "dystonia" for treatment
.
Since the onset of the disease, the patient has had no obvious dizziness, headache, unconsciousness, dysphagia, numbness and weakness of the limbs, and no urinary and urinary disorders.
The weight of the patient has lost 15kg in the past 7 months
.
▌ [Past history and personal history] Past history: No special
.
Personal history: 4 years of working in the production of rubber leather (operating machines but not touching rubber); smoking for 10+ years, 20 cigarettes/day, never quitting; drinking for 10+ years, occasionally drinking white wine, having quit drinking for half a year; denying drug exposure History and travel history
.
There is nothing special about marriage and childbirth history and family history
.
▌ [Physical examination] The vital signs are stable, and the heart, lung and abdomen are checked (-)
.
Specialist physical examination: clear speech, normal memory, comprehension, calculation, and orientation, no obvious abnormalities in cranial nerve examination, level 5 muscle strength of the limbs, rough measurement of superficial and deep sensation of both limbs are basically normal, right upper limb muscles Increased tension, tendon reflexes (++), pathological signs (-), meningeal irritation signs (-), and normal commotion
.
▌ [Auxiliary examination after admission] The three major routines, blood biochemistry, and coagulation function are normal; metabolism-related indicators: thyroid function, HCY (homocysteine), and glycosylated hemoglobin are normal; vitamin determination complete set: VE 4.
93ug/ml, normal low Value; tumor and paraneoplastic related indicators: new C12, neurogenic antigen antibody spectrum is normal; four items before blood transfusion, inflammation indicators are normal; ECG and chest CT are not special
.
Head MRI plain scan+enhanced+DWI+MRA+SWI: no obvious abnormalities were seen
.
Electromyography: The H reflex of the bilateral tibial nerves was not elicited, and there were no obvious abnormalities in the electromyography and peripheral nerve electrograms examined; the skin sympathetic reflex of the right lower limb was abnormal (no actual clinical significance)
.
24-hour video EEG: No abnormal EEG
.
▌ Sort the characteristics of the case as follows: 36-year-old male, the course of illness is more than 7 months, chronically worsening; with "paroxysmal right upper limb stiffness" as the main symptom; right upper limb muscle tone is increased, and there are no positive signs of the nervous system; no obvious positive Inspection basis
.
So far, it seems that no effective prompts have been received.
Simple right upper limb dyskinesia or problems in any link of the entire motor pathway may have an impact.
So where should the positioning and qualitative diagnosis emphasized by our neurology department go? Positioning, qualitative diagnosis? ▌ [Location Diagnosis] First of all, for patients with movement disorders, we most often consider damage to the pyramidal tract or extrapyramidal damage
.
(1) Cerebral cortex-left frontal lobe epileptic motor area? What we think of most often is "epilepsy"? Then this patient, with recurrent dysfunction of the right upper limb, seems to have the four major characteristics of epileptic seizures: "seizure, transient, repetitive, and stereotyped", but this patient can have such seizures.
It is induced by a special position and disappears during sleep, which is not in line with the characteristics of epileptic seizures
.
And the patient's EEG showed no obvious abnormalities, so the possibility of considering epilepsy is not great
.
(B) Extrapyramidal system-left basal ganglia? Is it considered as dystonia? According to the latest guidelines for dystonia, its main manifestations are: continuous or intermittent muscle contraction; seizures are intermittently indeterminate and the pattern is constant; usually when the mental tension is aggravated and relieved during rest
.
Therefore, based on the patient's performance, we consider dystonia, which is located in the extrapyramidal system
.
▌ [Qualitative diagnosis] The patient’s current clinical manifestations meet the criteria for dystonia, then we must qualitatively diagnose the cause
.
Considering that the patient is a middle-aged male with a middle-aged onset and no clear family history.
The possibility of hereditary diseases is small.
First of all, we need to consider acquired causes, including infection, drug use, poisoning, blood vessels, tumors, inflammation, mentality, etc.
Aspect (see Figure 1)
.
Figure 1: Diagnosis of the etiology of dystonia Through the review of the above etiology, the most likely cause of this patient is inflammation and infection.
The method of diagnosis is lumbar puncture
.
After completing the lumbar puncture, the results showed that: the pressure of cerebrospinal fluid was 110mmH2O; the routine, biochemical, three major staining, new C12, OB, and paraneoplastic antibodies of cerebrospinal fluid were all normal; synchronous blood glucose + E4A and serum OB were all normal
.
However, the "autoimmune antibody (+)" in the patient's cerebrospinal fluid and the autoimmune brain antibody in the serum are also positive
.
So so far, the patient’s diagnosis is clearly "autoimmune encephalitis (D2R antibody positive)"
.
▌ [Treatment plan] The patient was given plasma exchange (PE) twice during the hospitalization; the patient's symptoms improved significantly after the second PE, but there were still intermittent episodes; hormone shock and sequential declining treatment were given
.
▌ [Follow-up management] The patient will be followed up in the outpatient clinic at the beginning of December 2020; during the same period, oral prednisone 30mg qd; the patient has no obvious seizures; it is recommended to review the D2R antibody titer 3 months after starting the hormone
.
Knowledge review In 2012, there have been studies showing that in a type of encephalitis dominated by movement disorders and mental disorders, D2R antibodies were detected positive, but D1R antibodies and D3R antibodies were not found from the beginning to the end.
Positive, its main manifestations are: ① Dyskinesia: Parkinson’s syndrome, dystonia and chorea are characteristic manifestations; ② Psychiatric symptoms, emotional instability, inattention, confusion, etc.
(see Figure 2): Picture 2: The main manifestation of autoimmune encephalitis (D2R antibody positive) Dopamine receptor 2 (Dopamine-2-Receptor, D2R) is one of the five dopamine receptors and is a G protein-coupled receptor (GPCR).
Consists of seven transmembrane domains, an extracellular nitrogen (N) terminal domain, three extracellular loops, three intracellular loops and an intracellular carbon-terminal domain, which inhibit adenylate cyclase
.
After the combination of D2R antibody and D2R, it can down-regulate the cell surface expression, resulting in motor and psychiatric symptoms; in addition to motor symptoms and psychiatric symptoms, patients with D2R-positive autoimmune encephalitis can also have the following manifestations: ①MRI: normal, or It is manifested as inflammatory changes confined to the basal ganglia and brainstem area; ②EEG: normal, or non-specific slowdown consistent with intracranial lesions; no epilepsy characteristics; ③Cerebrospinal fluid: increased cell count may occur
.
The literature shows that these patients with autoimmune sports and psychiatric diseases usually develop after infection, and early immunosuppression or immunomodulatory treatment is effective, indicating that an immune-mediated process has occurred in the disease; use large doses of methyl strong in the early stage of the disease The empirical treatment of pineal, oral prednisolone sustained-release agent and intravenous immunoglobulin has a positive effect on the relief of patients’ symptoms
.
Among them, Mr.
Xie Yuanyuan brought us a lecture named "The Gloves of Thanos".
The content is wonderful, let us learn together! Case review: A 36-year-old man, married, was admitted to the hospital in September 2020 due to "paroxysmal right upper limb stiffness for 7 months and aggravation in April"
.
Starting from February 2020, the patient has been observed by his family members to have paroxysmal stiffness of the right upper limb without obvious inducement.
Each attack lasts for 1-2 seconds to half an hour, with repeated attacks.
It is obvious when forced, lying down, and tense, on the right side.
It is relieved when lying down and relaxed, and disappears when sleeping at night
.
As of May 2020, the patient's symptoms gradually worsened, and the stiffness occurred frequently, lasting for a few minutes to about 1 hour, and developing to the right neck and right chest and back, accompanied by profuse upper body sweating
.
A head MRI examination in a local hospital showed "a few ischemic foci on both cerebral hemispheres" and was given oral treatment with "clonazepam 1 mg bid + propranolol 1 mg bid", but the control was not good
.
In September 2020, I went to the Department of Neurology, Xiangya Hospital of Central South University.
The outpatient department was admitted to the ward with "dystonia" for treatment
.
Since the onset of the disease, the patient has had no obvious dizziness, headache, unconsciousness, dysphagia, numbness and weakness of the limbs, and no urinary and urinary disorders.
The weight of the patient has lost 15kg in the past 7 months
.
▌ [Past history and personal history] Past history: No special
.
Personal history: 4 years of working in the production of rubber leather (operating machines but not touching rubber); smoking for 10+ years, 20 cigarettes/day, never quitting; drinking for 10+ years, occasionally drinking white wine, having quit drinking for half a year; denying drug exposure History and travel history
.
There is nothing special about marriage and childbirth history and family history
.
▌ [Physical examination] The vital signs are stable, and the heart, lung and abdomen are checked (-)
.
Specialist physical examination: clear speech, normal memory, comprehension, calculation, and orientation, no obvious abnormalities in cranial nerve examination, level 5 muscle strength of the limbs, rough measurement of superficial and deep sensation of both limbs are basically normal, right upper limb muscles Increased tension, tendon reflexes (++), pathological signs (-), meningeal irritation signs (-), and normal commotion
.
▌ [Auxiliary examination after admission] The three major routines, blood biochemistry, and coagulation function are normal; metabolism-related indicators: thyroid function, HCY (homocysteine), and glycosylated hemoglobin are normal; vitamin determination complete set: VE 4.
93ug/ml, normal low Value; tumor and paraneoplastic related indicators: new C12, neurogenic antigen antibody spectrum is normal; four items before blood transfusion, inflammation indicators are normal; ECG and chest CT are not special
.
Head MRI plain scan+enhanced+DWI+MRA+SWI: no obvious abnormalities were seen
.
Electromyography: The H reflex of the bilateral tibial nerves was not elicited, and there were no obvious abnormalities in the electromyography and peripheral nerve electrograms examined; the skin sympathetic reflex of the right lower limb was abnormal (no actual clinical significance)
.
24-hour video EEG: No abnormal EEG
.
▌ Sort the characteristics of the case as follows: 36-year-old male, the course of illness is more than 7 months, chronically worsening; with "paroxysmal right upper limb stiffness" as the main symptom; right upper limb muscle tone is increased, and there are no positive signs of the nervous system; no obvious positive Inspection basis
.
So far, it seems that no effective prompts have been received.
Simple right upper limb dyskinesia or problems in any link of the entire motor pathway may have an impact.
So where should the positioning and qualitative diagnosis emphasized by our neurology department go? Positioning, qualitative diagnosis? ▌ [Location Diagnosis] First of all, for patients with movement disorders, we most often consider damage to the pyramidal tract or extrapyramidal damage
.
(1) Cerebral cortex-left frontal lobe epileptic motor area? What we think of most often is "epilepsy"? Then this patient, with recurrent dysfunction of the right upper limb, seems to have the four major characteristics of epileptic seizures: "seizure, transient, repetitive, and stereotyped", but this patient can have such seizures.
It is induced by a special position and disappears during sleep, which is not in line with the characteristics of epileptic seizures
.
And the patient's EEG showed no obvious abnormalities, so the possibility of considering epilepsy is not great
.
(B) Extrapyramidal system-left basal ganglia? Is it considered as dystonia? According to the latest guidelines for dystonia, its main manifestations are: continuous or intermittent muscle contraction; seizures are intermittently indeterminate and the pattern is constant; usually when the mental tension is aggravated and relieved during rest
.
Therefore, based on the patient's performance, we consider dystonia, which is located in the extrapyramidal system
.
▌ [Qualitative diagnosis] The patient’s current clinical manifestations meet the criteria for dystonia, then we must qualitatively diagnose the cause
.
Considering that the patient is a middle-aged male with a middle-aged onset and no clear family history.
The possibility of hereditary diseases is small.
First of all, we need to consider acquired causes, including infection, drug use, poisoning, blood vessels, tumors, inflammation, mentality, etc.
Aspect (see Figure 1)
.
Figure 1: Diagnosis of the etiology of dystonia Through the review of the above etiology, the most likely cause of this patient is inflammation and infection.
The method of diagnosis is lumbar puncture
.
After completing the lumbar puncture, the results showed that: the pressure of cerebrospinal fluid was 110mmH2O; the routine, biochemical, three major staining, new C12, OB, and paraneoplastic antibodies of cerebrospinal fluid were all normal; synchronous blood glucose + E4A and serum OB were all normal
.
However, the "autoimmune antibody (+)" in the patient's cerebrospinal fluid and the autoimmune brain antibody in the serum are also positive
.
So so far, the patient’s diagnosis is clearly "autoimmune encephalitis (D2R antibody positive)"
.
▌ [Treatment plan] The patient was given plasma exchange (PE) twice during the hospitalization; the patient's symptoms improved significantly after the second PE, but there were still intermittent episodes; hormone shock and sequential declining treatment were given
.
▌ [Follow-up management] The patient will be followed up in the outpatient clinic at the beginning of December 2020; during the same period, oral prednisone 30mg qd; the patient has no obvious seizures; it is recommended to review the D2R antibody titer 3 months after starting the hormone
.
Knowledge review In 2012, there have been studies showing that in a type of encephalitis dominated by movement disorders and mental disorders, D2R antibodies were detected positive, but D1R antibodies and D3R antibodies were not found from the beginning to the end.
Positive, its main manifestations are: ① Dyskinesia: Parkinson’s syndrome, dystonia and chorea are characteristic manifestations; ② Psychiatric symptoms, emotional instability, inattention, confusion, etc.
(see Figure 2): Picture 2: The main manifestation of autoimmune encephalitis (D2R antibody positive) Dopamine receptor 2 (Dopamine-2-Receptor, D2R) is one of the five dopamine receptors and is a G protein-coupled receptor (GPCR).
Consists of seven transmembrane domains, an extracellular nitrogen (N) terminal domain, three extracellular loops, three intracellular loops and an intracellular carbon-terminal domain, which inhibit adenylate cyclase
.
After the combination of D2R antibody and D2R, it can down-regulate the cell surface expression, resulting in motor and psychiatric symptoms; in addition to motor symptoms and psychiatric symptoms, patients with D2R-positive autoimmune encephalitis can also have the following manifestations: ①MRI: normal, or It is manifested as inflammatory changes confined to the basal ganglia and brainstem area; ②EEG: normal, or non-specific slowdown consistent with intracranial lesions; no epilepsy characteristics; ③Cerebrospinal fluid: increased cell count may occur
.
The literature shows that these patients with autoimmune sports and psychiatric diseases usually develop after infection, and early immunosuppression or immunomodulatory treatment is effective, indicating that an immune-mediated process has occurred in the disease; use large doses of methyl strong in the early stage of the disease The empirical treatment of pineal, oral prednisolone sustained-release agent and intravenous immunoglobulin has a positive effect on the relief of patients’ symptoms
.
