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A case of concentric sclerosis to share
.
Not much to say, let's take a look at this case
.
Author: Wang Sa (physician, Xianyang, Yan'an University Hospital), Kang Juan, Liu Xuedong this article is the author's permission NMT Medical publish, please do not reprint without authorization
.
Case profile The patient Xu Moumou, female, 36 years old, working at a gas station, consciously caught a cold and fever 15 days ago (2021.
09.
20), with a body temperature of up to 37.
3℃, and self-administered "999 Ganmaoling" did not improve
.
The next day (2021.
09.
21) his family members found that his speech was slurred, his pronunciation was illegible, he was still able to communicate, he felt that his right limb was weak, his right upper limb was laboriously lifted, his right lower limb was soft when walking, there was no dragging temporarily, and his walking was not stable.
Dizziness, no nausea, vomiting, can take care of himself, that is, he went to Zhenping County Hospital, Ankang City, Shaanxi Province
.
A CT scan of the brain showed that there were low-density lesions in the frontal lobe, basal ganglia, occipital lobe, and right temporal lobe on both sides, and the brain tissue was full and swollen (the original report was not seen, read the film), and no special treatment was given
.
Later, he went to the Ankang Central Hospital and underwent cranial MRI+MRA+DWI to show: 1.
Bilateral frontal, parietal lobe, lateral ventricle, basal ganglia, hippocampus, left thalamus, left occipital lobe and right cerebellar hemisphere, left edge of brainstem, corpus callosum, multiple foci, consider multiple sclerosis, cerebral infarction to be discharged ;2.
Inflammation of the ethmoid sinuses on both sides and hypertrophy of the left inferior turbinate
.
Ankang Central Hospital’s telephone feedback: Lumbar puncture has been performed, CSF pressure is normal; CSF routine and biochemistry are normal; CSF AQP4 is negative, CSF and serum IgG oligoclonal bands are negative; CSF IgG increased by 84.
6mg/L (Normal value 10-40), serum IgG is normal
.
Six days after the onset of the disease (2021.
09.
26), the above symptoms were aggravated in the morning, and the right limbs were unable to move, speech was vague, unable to communicate normally, unable to fully understand speech, commanded movements were partially completed, coughing and dysphagia without drinking water , Constipation, urine is basically normal, according to the treatment of "multiple sclerosis", give "methylprednisolone" pulse therapy (1000mg for 4 days, 500mg 1 day), give "clopidogrel bisulfate, aspirin enteric-coated tablets, citicophos Choline sodium, esomeprazole, potassium chloride sustained-release tablets were taken orally, and the patient's symptoms did not improve significantly
.
On October 4, 2021, the patient went to the emergency department of our hospital for complete related examinations
.
Brain MRI+MRA showed: 1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum, and left occipital lobe have multiple abnormal signals.
Considering demyelinating disease, concentric circles are more likely to sclerosis; 2.
There was no obvious abnormality in brain MRA
.
Brain + chest CT: 1.
1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum pressure and flaky low-density shadows around the posterior horn of the lateral ventricle; 2.
Chest CT scan showed no clear lesions
.
There were no abnormalities in the ultrasound of the blood vessels of the heart, abdomen, and neck
.
Given complete related laboratory tests, blood routine showed white blood cells 14.
81X109/L; liver function showed albumin 35.
8g/L; procalcitonin quantitative was 0.
091ng/ml
.
The patient's right lower limb weakness and speech disorder further aggravated, and the emergency department continued to receive "Methylprednisolone" 500mg/day
.
On October 5, 2021, the emergency department was admitted to our department as "Multiple Sclerosis Waiting"
.
Since the onset of the patient's onset, the appetite is fair, the mental rest is slightly poor, the mood is unstable, crying, constipation, constipation, normal urine, and no significant changes in weight
.
Past history and family history are nothing special
.
Personal history: working at a gas station, nothing more than special
.
01 Admission to the hospital for physical examination and general medical examination: stable vital signs, positive signs in specialist examination (less cooperation): right handedness, sensorimotor aphasia, right limb muscle strength level 0, decreased muscle tone, active tendon reflexes, patelloclonus Positive ankle clonus, right pathological sign (+), left limb muscle strength, muscle tone, and tendon reflex are normal, pathological sign (-), feeling uncooperative on physical examination
.
02Auxiliary examination before admission ➤2021.
09.
21 cranial CT: low-density lesions can be seen in the frontal lobe, basal ganglia, occipital lobe, and right temporal lobe on both sides, and the brain tissue is full and swollen (original report not seen, read film)
.
➤2021.
09.
22 cranial CT: changes in the left occipital lobe consider cerebral infarction
.
➤2021.
09.
24 cranial MRI+MRA+DWI shows: 1.
Bilateral frontal, parietal lobe, lateral ventricle, basal ganglia, hippocampus, left thalamus, left occipital lobe and right cerebellar hemisphere, left edge of brainstem, corpus callosum, multiple foci, consider multiple sclerosis, cerebral infarction to be discharged , Please combine with clinical; 2.
Inflammation of the ethmoid sinuses on both sides and hypertrophy of the left inferior turbinate
.
➤2021.
10.
02 cranial CT: bilateral frontal lobe, parietal lobe, basal ganglia, right temporal lobe, left occipital lobe, multiple low-density lesions
.
➤2021.
10.
04 cranial MRI+MRA: 1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum, and left occipital lobe have multiple abnormal signals.
Consider demyelinating diseases.
Concentric sclerosis is more likely.
Please combine it with clinical practice
.
2.
There is no obvious abnormality in the brain MRA (partial artifacts)
.
Slide to view imaging data ↓↓↓↓↓↓↓↓↓03 After admission, auxiliary examination of lumbar puncture: normal intracranial pressure, cerebrospinal fluid cytology, routine, biochemical, immune, paraneoplastic, OB have no obvious abnormalities; laboratory tests: 1.
Hypothyroidism suggests changes in hypothyroidism (high TSH); 2.
High blood sugar (hormone-related impaired glucose tolerance); 3.
Routine blood tests suggest mild iron deficiency anemia; 4.
Routine urine shows red and white blood cells and protein +, consider this Period related
.
3 head MRI+Flair+DWI+MRA+enhanced+SWI+MRS: Bilateral frontal and parietal lobe, semioval center, basal ganglia, thalamus, corpus callosum, and left occipital lobe have multiple abnormal signals, showing long T1, T2 Signal shadow, Flair+DWI can see high signal; no significant enhancement is found on enhancement; no bleeding is seen on SWI; MRS indicates possible demyelination, considering demyelinating disease, concentric circle sclerosis is more likely; no obvious abnormality in brain MRA; head CT showed bilateral frontal lobes, semi-oval center, basal ganglia, right temporal lobe, left occipital lobe, low-density shadows and full brain tissue edema
.
MRI of cervical thoracic spine: 1.
6/7 of the cervical intervertebral discs were slightly herniated to the right in the center
.
If you see abnormal signals in the brain, please combine with cranial MR for further observation
.
2.
There was no obvious abnormality on plain MRI scan of the thoracic spine
.
➤2021.
10.
08 enhanced MRI + MRS + enhanced scan: bilateral frontal and parietal lobes, basal ganglia, corpus callosum pressure and left occipital lobe have multiple abnormal signals, the NAA value of the area of interest is reduced, and demyelinating diseases, concentric sclerosis should be considered more The possibility is high, please combine clinical and laboratory examinations
.
Location: bilateral frontal and parietal lobes, semi-oval center, basal ganglia, thalamus, corpus callosum, and left occipital lobe
.
Qualitative: Immunization? Diagnosis: 1.
Concentric sclerosis (multiple sclerosis variant) 2.
Subacute hypothyroidism 3.
Mild iron-deficiency anemia treatment was given to the patient with methylprednisolone hormone shock therapy.
After the treatment, the patient’s body was examined, and the patient’s cerebral cortical function was advanced.
Basically normal, the right limb muscle strength was 0 at the time of admission.
After hormone treatment, the right upper limb muscle strength was 0 and the right lower limb muscle strength was 3.
After treatment, it improved, which is the evolution of paralysis
.
The family members complained that the patient was unable to express when he was admitted to the hospital, part of the language was incomprehensible, and it was an incomplete sensorimotor aphasia.
After treatment, the patient still had mixed aphasia, and the degree was improved
.
Urine and stool barrier-free; right hemianopia is not completely determined; the optic disc of the fundus is normal, ignoring papillary edema, and the intracranial pressure is not high; the right pathological signs are positive, the right tendon reflex is active, and the left knee tendon reflex is active
.
Follow-up 1 month later, the patient's right limb muscle strength was level 4, his speech was basically clear, and the intracranial lesions were reduced
.
Case thinking Balo's concentric sclerosis (Balo's concentric sclerosis, BCS) is a rare CNS demyelinating disease that consists of a concentric layered pattern composed of alternating rings of different strengths
.
It is considered to be a variant of multiple sclerosis, but it is still controversial
.
The previous diagnosis mainly relied on biopsy, and the current diagnosis is mainly based on the diagnosis of typical head MRI
.
BCS was first reported by Marburg in 1906, when it was called polyaxial encephalitis
.
In 1928, Balo first reported its pathological confirmation of demyelinating properties, named Balo concentric sclerosis.
The etiology is unclear, and it may be related to viral infection
.
Pathology: Mainly occurs in the white matter of the supratentorial brain, retaining cortical u fibers.
The lamellar pattern of the disease may reflect the demyelination zone and the relative preservation zone of myelin, with very little loss of axons
.
Astrocyte proliferation and sleeve-like infiltration of perivascular inflammatory cells can be seen
.
Pathogenesis: It is related to the immune response induced by viral infection
.
Clinical manifestations: Acute or subacute onset, with three forms of self-limiting, remission-relapse mutation, and rapid progression.
It usually occurs in young adults, 20-50 years old, abroad: male>female, domestic: female>male, 70% are Asians, with China, the Philippines or Japan the most
.
Clinical manifestations can have a variety of brain symptoms and signs, including mental disorders, headaches, aphasia, hemiplegia, cognitive or behavioral abnormalities, and seizures, etc.
.
According to the diagnostic criteria of Sekijima in 1994: 1.
Young and middle-aged, more common in women (consistent); 2.
Acute or subacute onset (consistent); 3.
The most common clinical manifestations are headache, aphasia, hemiplegia, cognition or behavior Abnormalities and epileptic seizures; (the patient has aphasia, hemiplegia, and behavioral abnormalities); 4.
MRI of the head shows concentric circles or onion-like white matter lesions (consistent); 5.
Pathological biopsy: for atypical people, and special needs Myelin staining
.
Treatment: Methylprednisolone is the main shock, plasma exchange, immunoglobulin therapy, natalizumab, cyclophosphamide, rituximab, etc.
may be effective in reducing the recurrence of BCS (this patient uses methylprednisolone)
.
Prognosis: BCS is often considered to be a rapidly fatal disease.
After regular hormone shock treatment, the recurrence rate is very low, mostly in a single course, and the prognosis is generally good
.
Differential diagnosis: Acute hemorrhagic leukoencephalitis (AHLE): AHLE is a very rare demyelinating disease, accompanied by severe encephalopathy and multifocal central nervous system symptoms.
It progresses rapidly and often dies within 1 week after the onset.
The appearance is similar on MRI, but with bleeding
.
This patient's SWI confirmed that there was no intracranial hemorrhage, so it was not consistent
.
The imaging of tumor-like demyelinating disease, primary central system lymphoma and inflammatory granuloma is inconsistent with this patient.
Generally, there is enhancement in magnetic resonance enhancement.
This patient has no obvious enhancement, but there are reports of concentric sclerosis In the initial report, it is not necessary to enhance it, but it can be enhanced in the later stage.
At present, the patient has been treated for more than 20 days, and there is no obvious enhancement on magnetic resonance.
Therefore, the evidence for these three diseases is temporarily insufficient, and pathological biopsy is required for diagnosis
.
.
Not much to say, let's take a look at this case
.
Author: Wang Sa (physician, Xianyang, Yan'an University Hospital), Kang Juan, Liu Xuedong this article is the author's permission NMT Medical publish, please do not reprint without authorization
.
Case profile The patient Xu Moumou, female, 36 years old, working at a gas station, consciously caught a cold and fever 15 days ago (2021.
09.
20), with a body temperature of up to 37.
3℃, and self-administered "999 Ganmaoling" did not improve
.
The next day (2021.
09.
21) his family members found that his speech was slurred, his pronunciation was illegible, he was still able to communicate, he felt that his right limb was weak, his right upper limb was laboriously lifted, his right lower limb was soft when walking, there was no dragging temporarily, and his walking was not stable.
Dizziness, no nausea, vomiting, can take care of himself, that is, he went to Zhenping County Hospital, Ankang City, Shaanxi Province
.
A CT scan of the brain showed that there were low-density lesions in the frontal lobe, basal ganglia, occipital lobe, and right temporal lobe on both sides, and the brain tissue was full and swollen (the original report was not seen, read the film), and no special treatment was given
.
Later, he went to the Ankang Central Hospital and underwent cranial MRI+MRA+DWI to show: 1.
Bilateral frontal, parietal lobe, lateral ventricle, basal ganglia, hippocampus, left thalamus, left occipital lobe and right cerebellar hemisphere, left edge of brainstem, corpus callosum, multiple foci, consider multiple sclerosis, cerebral infarction to be discharged ;2.
Inflammation of the ethmoid sinuses on both sides and hypertrophy of the left inferior turbinate
.
Ankang Central Hospital’s telephone feedback: Lumbar puncture has been performed, CSF pressure is normal; CSF routine and biochemistry are normal; CSF AQP4 is negative, CSF and serum IgG oligoclonal bands are negative; CSF IgG increased by 84.
6mg/L (Normal value 10-40), serum IgG is normal
.
Six days after the onset of the disease (2021.
09.
26), the above symptoms were aggravated in the morning, and the right limbs were unable to move, speech was vague, unable to communicate normally, unable to fully understand speech, commanded movements were partially completed, coughing and dysphagia without drinking water , Constipation, urine is basically normal, according to the treatment of "multiple sclerosis", give "methylprednisolone" pulse therapy (1000mg for 4 days, 500mg 1 day), give "clopidogrel bisulfate, aspirin enteric-coated tablets, citicophos Choline sodium, esomeprazole, potassium chloride sustained-release tablets were taken orally, and the patient's symptoms did not improve significantly
.
On October 4, 2021, the patient went to the emergency department of our hospital for complete related examinations
.
Brain MRI+MRA showed: 1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum, and left occipital lobe have multiple abnormal signals.
Considering demyelinating disease, concentric circles are more likely to sclerosis; 2.
There was no obvious abnormality in brain MRA
.
Brain + chest CT: 1.
1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum pressure and flaky low-density shadows around the posterior horn of the lateral ventricle; 2.
Chest CT scan showed no clear lesions
.
There were no abnormalities in the ultrasound of the blood vessels of the heart, abdomen, and neck
.
Given complete related laboratory tests, blood routine showed white blood cells 14.
81X109/L; liver function showed albumin 35.
8g/L; procalcitonin quantitative was 0.
091ng/ml
.
The patient's right lower limb weakness and speech disorder further aggravated, and the emergency department continued to receive "Methylprednisolone" 500mg/day
.
On October 5, 2021, the emergency department was admitted to our department as "Multiple Sclerosis Waiting"
.
Since the onset of the patient's onset, the appetite is fair, the mental rest is slightly poor, the mood is unstable, crying, constipation, constipation, normal urine, and no significant changes in weight
.
Past history and family history are nothing special
.
Personal history: working at a gas station, nothing more than special
.
01 Admission to the hospital for physical examination and general medical examination: stable vital signs, positive signs in specialist examination (less cooperation): right handedness, sensorimotor aphasia, right limb muscle strength level 0, decreased muscle tone, active tendon reflexes, patelloclonus Positive ankle clonus, right pathological sign (+), left limb muscle strength, muscle tone, and tendon reflex are normal, pathological sign (-), feeling uncooperative on physical examination
.
02Auxiliary examination before admission ➤2021.
09.
21 cranial CT: low-density lesions can be seen in the frontal lobe, basal ganglia, occipital lobe, and right temporal lobe on both sides, and the brain tissue is full and swollen (original report not seen, read film)
.
➤2021.
09.
22 cranial CT: changes in the left occipital lobe consider cerebral infarction
.
➤2021.
09.
24 cranial MRI+MRA+DWI shows: 1.
Bilateral frontal, parietal lobe, lateral ventricle, basal ganglia, hippocampus, left thalamus, left occipital lobe and right cerebellar hemisphere, left edge of brainstem, corpus callosum, multiple foci, consider multiple sclerosis, cerebral infarction to be discharged , Please combine with clinical; 2.
Inflammation of the ethmoid sinuses on both sides and hypertrophy of the left inferior turbinate
.
➤2021.
10.
02 cranial CT: bilateral frontal lobe, parietal lobe, basal ganglia, right temporal lobe, left occipital lobe, multiple low-density lesions
.
➤2021.
10.
04 cranial MRI+MRA: 1.
Bilateral frontal and parietal lobes, basal ganglia, corpus callosum, and left occipital lobe have multiple abnormal signals.
Consider demyelinating diseases.
Concentric sclerosis is more likely.
Please combine it with clinical practice
.
2.
There is no obvious abnormality in the brain MRA (partial artifacts)
.
Slide to view imaging data ↓↓↓↓↓↓↓↓↓03 After admission, auxiliary examination of lumbar puncture: normal intracranial pressure, cerebrospinal fluid cytology, routine, biochemical, immune, paraneoplastic, OB have no obvious abnormalities; laboratory tests: 1.
Hypothyroidism suggests changes in hypothyroidism (high TSH); 2.
High blood sugar (hormone-related impaired glucose tolerance); 3.
Routine blood tests suggest mild iron deficiency anemia; 4.
Routine urine shows red and white blood cells and protein +, consider this Period related
.
3 head MRI+Flair+DWI+MRA+enhanced+SWI+MRS: Bilateral frontal and parietal lobe, semioval center, basal ganglia, thalamus, corpus callosum, and left occipital lobe have multiple abnormal signals, showing long T1, T2 Signal shadow, Flair+DWI can see high signal; no significant enhancement is found on enhancement; no bleeding is seen on SWI; MRS indicates possible demyelination, considering demyelinating disease, concentric circle sclerosis is more likely; no obvious abnormality in brain MRA; head CT showed bilateral frontal lobes, semi-oval center, basal ganglia, right temporal lobe, left occipital lobe, low-density shadows and full brain tissue edema
.
MRI of cervical thoracic spine: 1.
6/7 of the cervical intervertebral discs were slightly herniated to the right in the center
.
If you see abnormal signals in the brain, please combine with cranial MR for further observation
.
2.
There was no obvious abnormality on plain MRI scan of the thoracic spine
.
➤2021.
10.
08 enhanced MRI + MRS + enhanced scan: bilateral frontal and parietal lobes, basal ganglia, corpus callosum pressure and left occipital lobe have multiple abnormal signals, the NAA value of the area of interest is reduced, and demyelinating diseases, concentric sclerosis should be considered more The possibility is high, please combine clinical and laboratory examinations
.
Location: bilateral frontal and parietal lobes, semi-oval center, basal ganglia, thalamus, corpus callosum, and left occipital lobe
.
Qualitative: Immunization? Diagnosis: 1.
Concentric sclerosis (multiple sclerosis variant) 2.
Subacute hypothyroidism 3.
Mild iron-deficiency anemia treatment was given to the patient with methylprednisolone hormone shock therapy.
After the treatment, the patient’s body was examined, and the patient’s cerebral cortical function was advanced.
Basically normal, the right limb muscle strength was 0 at the time of admission.
After hormone treatment, the right upper limb muscle strength was 0 and the right lower limb muscle strength was 3.
After treatment, it improved, which is the evolution of paralysis
.
The family members complained that the patient was unable to express when he was admitted to the hospital, part of the language was incomprehensible, and it was an incomplete sensorimotor aphasia.
After treatment, the patient still had mixed aphasia, and the degree was improved
.
Urine and stool barrier-free; right hemianopia is not completely determined; the optic disc of the fundus is normal, ignoring papillary edema, and the intracranial pressure is not high; the right pathological signs are positive, the right tendon reflex is active, and the left knee tendon reflex is active
.
Follow-up 1 month later, the patient's right limb muscle strength was level 4, his speech was basically clear, and the intracranial lesions were reduced
.
Case thinking Balo's concentric sclerosis (Balo's concentric sclerosis, BCS) is a rare CNS demyelinating disease that consists of a concentric layered pattern composed of alternating rings of different strengths
.
It is considered to be a variant of multiple sclerosis, but it is still controversial
.
The previous diagnosis mainly relied on biopsy, and the current diagnosis is mainly based on the diagnosis of typical head MRI
.
BCS was first reported by Marburg in 1906, when it was called polyaxial encephalitis
.
In 1928, Balo first reported its pathological confirmation of demyelinating properties, named Balo concentric sclerosis.
The etiology is unclear, and it may be related to viral infection
.
Pathology: Mainly occurs in the white matter of the supratentorial brain, retaining cortical u fibers.
The lamellar pattern of the disease may reflect the demyelination zone and the relative preservation zone of myelin, with very little loss of axons
.
Astrocyte proliferation and sleeve-like infiltration of perivascular inflammatory cells can be seen
.
Pathogenesis: It is related to the immune response induced by viral infection
.
Clinical manifestations: Acute or subacute onset, with three forms of self-limiting, remission-relapse mutation, and rapid progression.
It usually occurs in young adults, 20-50 years old, abroad: male>female, domestic: female>male, 70% are Asians, with China, the Philippines or Japan the most
.
Clinical manifestations can have a variety of brain symptoms and signs, including mental disorders, headaches, aphasia, hemiplegia, cognitive or behavioral abnormalities, and seizures, etc.
.
According to the diagnostic criteria of Sekijima in 1994: 1.
Young and middle-aged, more common in women (consistent); 2.
Acute or subacute onset (consistent); 3.
The most common clinical manifestations are headache, aphasia, hemiplegia, cognition or behavior Abnormalities and epileptic seizures; (the patient has aphasia, hemiplegia, and behavioral abnormalities); 4.
MRI of the head shows concentric circles or onion-like white matter lesions (consistent); 5.
Pathological biopsy: for atypical people, and special needs Myelin staining
.
Treatment: Methylprednisolone is the main shock, plasma exchange, immunoglobulin therapy, natalizumab, cyclophosphamide, rituximab, etc.
may be effective in reducing the recurrence of BCS (this patient uses methylprednisolone)
.
Prognosis: BCS is often considered to be a rapidly fatal disease.
After regular hormone shock treatment, the recurrence rate is very low, mostly in a single course, and the prognosis is generally good
.
Differential diagnosis: Acute hemorrhagic leukoencephalitis (AHLE): AHLE is a very rare demyelinating disease, accompanied by severe encephalopathy and multifocal central nervous system symptoms.
It progresses rapidly and often dies within 1 week after the onset.
The appearance is similar on MRI, but with bleeding
.
This patient's SWI confirmed that there was no intracranial hemorrhage, so it was not consistent
.
The imaging of tumor-like demyelinating disease, primary central system lymphoma and inflammatory granuloma is inconsistent with this patient.
Generally, there is enhancement in magnetic resonance enhancement.
This patient has no obvious enhancement, but there are reports of concentric sclerosis In the initial report, it is not necessary to enhance it, but it can be enhanced in the later stage.
At present, the patient has been treated for more than 20 days, and there is no obvious enhancement on magnetic resonance.
Therefore, the evidence for these three diseases is temporarily insufficient, and pathological biopsy is required for diagnosis
.
