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*Only for medical professionals to read and reference [Review the classics] Lung cancer complicated by reversible posterior leukoencephalopathy syndrome Reversible posterior encephalopathy syndrome (Posterior reversible encephalopathy syndrome, PRES) is of bilateral occipital parietal symmetrical vascular origin Edema is an imaging feature, and its clinical symptoms are mainly headaches, seizures, disturbances of consciousness, visual disturbances and other encephalopathy symptoms.
In recent years, it has gradually become well known to everyone, and the diagnosis depends on imaging.
There are many causes, the most common being hypertensive encephalopathy, pre-eclampsia or eclampsia, and severe kidney disease.
Lung cancer complicated by reversible posterior leukoencephalopathy syndrome is rarely reported, and it is easy to be misdiagnosed as brain metastases.
Director Tang Wei of the Department of Neurology, Xinhua Hospital Affiliated to Dalian University, led everyone to review the classics.
Case introduction: A 76-year-old male, mainly due to paroxysmal dizziness and blackout, went to see a doctor for 2 weeks.
Two weeks before admission, the patient had dizziness, blackout, and blurred vision without obvious inducement.
It can be relieved by itself for 3-5 minutes, and the attacks will be 3-5 times a day.
Diagnosed transient ischemic attack (TIA), once intravenously administered the drug thromboxane, there was no obvious relief.
In the past 2 days, the patient has frequent episodes of amaurosis, 8-10 times a day.
He had a history of lung cancer and brain metastasis for 8 months, and was given radiotherapy to the lungs and head.
During this period, he started to take the targeted drug osimertinib and took it intermittently for three months.
Admission examination: blood pressure 130/80mmHg, heart rate 75 beats/min, clear, blurred vision in both eyes, ignoring double shadows.
The pupils on both sides are equal to the same circle, with a diameter of 3.
5mm, sensitive to light reflection, the muscle strength and muscle tension of the limbs are normal, and the Babinski sign and Chadock sign on both sides are negative.Lung CT showed right lung cancer (upper lobe) (Figure A), head MRI showed bilateral occipital lobes with sheet-like long T1 long T2 abnormal signals (Figure BCD), FLAIR image showed high signal (Figure E), diffusion was not limited ( Figure F).
Diagnosis: Lung cancer complicated with reversible posterior leukoencephalopathy syndrome.
He was treated with mannitol and methylprednisolone for 3 days, and the symptoms disappeared.
Figure A Figure B Figure C Figure D Figure E Figure F Figure A: Lung CT shows the upper lobe of the right lung cancer; Figure BCD shows the head MRI shows the bilateral occipital lobes with sheet-like long T1 long T2 abnormal signals; Figure E shows: FLAIR image The bilateral occipital lobes showed high signal intensity.
Figure F shows that the bilateral occipital lobes are diffusely unrestricted.
Discussion 1PRES typical neuroimaging The typical neuroimaging changes of PRES are located in the white matter of the back of the brain.
Due to the lower density of white matter tissue, it is more prone to edema than the cortex.
Due to the relative lack of sympathetic innervation, the posterior brain tissue is more susceptible to blood pressure fluctuations.
CT images often showed diffuse subcortical edema on both frontal and parietal lobes, and disappearance of the sulci.
MRI showed involvement of any part of the brain dominated by the back of the brain (parieto-occipital lobe).
Probability of occurrence in various parts: occipital lobe, parietal lobe 98%, frontal lobe 68%, inferior temporal lobe, occipital lobe 40%, cerebellum 32%, brainstem 13%, basal ganglia 14%, deep brain white matter 18%, corpus callosum pressure part 10%.
The MRI of the affected head showed abnormal signal of long T1 and long T2 on both sides of the occipital lobe.
The FLAIR image showed high signal.
It is a classic PRES image and supports the diagnosis of PRES.
2 PRES pathogenesis PRES is reversible, subcortical, vasogenic edema, the mechanism is as follows: normal cerebral perfusion pressure is maintained between 50-100mmHg, cerebral blood flow can only be achieved through cerebrovascular pressure response, chemical regulation and autonomic nerve regulation.
In a stable state.
Under pathological conditions, excessive blood pressure fluctuations exceed the self-regulation capacity of blood vessels, or excessive cytokine release in the circulation leads to impaired endothelial function, and autonomic nerves are insufficient to regulate perfusion pressure, which will cause a series of undesirable consequences, leading to cerebrovascular permeability Sexual changes, which are the pathological basis of vasogenic brain edema.
Causes of PRES: common hypertension (mostly malignant hypertension), pregnancy eclampsia, renal insufficiency, etc.
The rapidly progressing hypertension exceeds the upper limit of cerebral blood flow self-regulation, resulting in high blood flow perfusion, leading to blood The destruction of the brain barrier and the exudation of plasma and macromolecules cause vasogenic brain edema.
The blood pressure of the patient is normal, which does not support this theory.
About 50% of PRES patients have a history of autoimmune diseases, and the use of immunosuppressive agents or cytotoxic drugs may also be one of the triggering factors of PRES.
Systemic lupus erythematosus, drug immunosuppressants such as cyclosporine, tacrolimus, sirolimus; chemotherapy drugs such as methotrexate, cisplatin, cytarabine, vincristine, sunitinib, Bevacizumab; immunomodulators such as rituximab; sepsis, hypomagnesemia, hypocalcemia, all of the above factors can cause damage to endothelial function, damage to the blood-brain barrier, and increase vascular permeability , Causing vasogenic brain edema.
Malignant tumors are a common cause of PRES.
Studies have found that a variety of tumors can cause PRES, among which solid tumors such as lung cancer, ovarian cancer, kidney cancer, and gastric cancer account for 71%, blood tumors such as lymphoma and leukemia account for 26%, and primary intracranial tumors such as glioma account for 3% %.
Therefore, some scholars have proposed that PRES is a neurological manifestation of malignant tumors.
The mechanism of tumors leading to PRES may be that a variety of cytokines, cytotoxins, chemotherapeutics, etc.
directly damage the blood-brain barrier, causing vasogenic edema.
This patient has a clear history of lung cancer, and most of the tumors have immune abnormalities.
At the same time, oral targeted drugs can directly damage the vascular endothelium.
The FLAIR image of the patient showed high signal in both occipital lobes, no high signal in DWI, and no low signal in ADC phase, suggesting that the bilateral occipital lobes were diffusely unrestricted, which was consistent with vasogenic cerebral edema.
3PRES clinical manifestations: headache, epilepsy, disturbance of consciousness, and visual disturbance are the most common in PRES.
Headache is the most common clinical manifestation of PRES, which is related to the increase in intracranial pressure caused by cerebral edema, and may be accompanied by nausea and vomiting.
Seizures can be the first symptom of the nervous system.
The seizures have various forms, which can be partial seizures, and usually develop into generalized tonic-clonic seizures.
Almost all patients have visual abnormalities (such as blurred vision, hemianopia, enlarged visual blind zone, cortical Sexual blindness, etc.
).
Visual abnormalities often indicate that the disease involves the occipital visual center that supplies blood to the back of the brain.
This patient has visual abnormalities and paroxysmal amaurosis, which relieves in 3-5 minutes, and is considered to be a seizure, which is consistent with the imaging changes of the bilateral occipital lobes of the head MRI.
4 Differential diagnosis The patient should be differentiated from the following diseases: (1) Radiation encephalopathy: The patient has received radiotherapy and should be differentiated from radiation encephalopathy.
The lesion location is basically the same as the range of radiation, and the lesions are mainly distributed in the temporal lobe, brain stem, and cerebellum.
; The irradiated site of this patient is the left temporal lobe, which is not consistent with the lesion site.
(2) Brain metastases: The patient has a history of brain metastases from lung cancer.
Metastases should be identified, but the lesions are bilaterally symmetrical, and no metastatic lesions are found, which can be excluded.
5 Treatment and prognosis After timely and effective treatment of PRES, the above symptoms and signs improved rapidly, and generally no neurological sequelae were left.
However, some patients may have permanent neurological damage.
Discovering and reversing/eliminating potential predisposing factors as soon as possible can usually relieve the clinical symptoms of patients and improve their prognosis.
In this case, lung cancer complicated by PRES, considering the existence of immune factors, received mannitol and methylprednisolone treatment for 3 days, the symptoms disappeared, the effect was good, and the effect of mannitol alone was not good in the early stage.
Although PRES is mostly a benign disease, its lesions can completely resolve within a few days to a few weeks, but not all patients have a good prognosis.
Increased creatinine levels, high-intensity areas on MRI diffusion-weighted images, and broader brain tissue involvement (especially involving the brain stem) are signs of poor prognosis.
In recent years, it has gradually become well known to everyone, and the diagnosis depends on imaging.
There are many causes, the most common being hypertensive encephalopathy, pre-eclampsia or eclampsia, and severe kidney disease.
Lung cancer complicated by reversible posterior leukoencephalopathy syndrome is rarely reported, and it is easy to be misdiagnosed as brain metastases.
Director Tang Wei of the Department of Neurology, Xinhua Hospital Affiliated to Dalian University, led everyone to review the classics.
Case introduction: A 76-year-old male, mainly due to paroxysmal dizziness and blackout, went to see a doctor for 2 weeks.
Two weeks before admission, the patient had dizziness, blackout, and blurred vision without obvious inducement.
It can be relieved by itself for 3-5 minutes, and the attacks will be 3-5 times a day.
Diagnosed transient ischemic attack (TIA), once intravenously administered the drug thromboxane, there was no obvious relief.
In the past 2 days, the patient has frequent episodes of amaurosis, 8-10 times a day.
He had a history of lung cancer and brain metastasis for 8 months, and was given radiotherapy to the lungs and head.
During this period, he started to take the targeted drug osimertinib and took it intermittently for three months.
Admission examination: blood pressure 130/80mmHg, heart rate 75 beats/min, clear, blurred vision in both eyes, ignoring double shadows.
The pupils on both sides are equal to the same circle, with a diameter of 3.
5mm, sensitive to light reflection, the muscle strength and muscle tension of the limbs are normal, and the Babinski sign and Chadock sign on both sides are negative.Lung CT showed right lung cancer (upper lobe) (Figure A), head MRI showed bilateral occipital lobes with sheet-like long T1 long T2 abnormal signals (Figure BCD), FLAIR image showed high signal (Figure E), diffusion was not limited ( Figure F).
Diagnosis: Lung cancer complicated with reversible posterior leukoencephalopathy syndrome.
He was treated with mannitol and methylprednisolone for 3 days, and the symptoms disappeared.
Figure A Figure B Figure C Figure D Figure E Figure F Figure A: Lung CT shows the upper lobe of the right lung cancer; Figure BCD shows the head MRI shows the bilateral occipital lobes with sheet-like long T1 long T2 abnormal signals; Figure E shows: FLAIR image The bilateral occipital lobes showed high signal intensity.
Figure F shows that the bilateral occipital lobes are diffusely unrestricted.
Discussion 1PRES typical neuroimaging The typical neuroimaging changes of PRES are located in the white matter of the back of the brain.
Due to the lower density of white matter tissue, it is more prone to edema than the cortex.
Due to the relative lack of sympathetic innervation, the posterior brain tissue is more susceptible to blood pressure fluctuations.
CT images often showed diffuse subcortical edema on both frontal and parietal lobes, and disappearance of the sulci.
MRI showed involvement of any part of the brain dominated by the back of the brain (parieto-occipital lobe).
Probability of occurrence in various parts: occipital lobe, parietal lobe 98%, frontal lobe 68%, inferior temporal lobe, occipital lobe 40%, cerebellum 32%, brainstem 13%, basal ganglia 14%, deep brain white matter 18%, corpus callosum pressure part 10%.
The MRI of the affected head showed abnormal signal of long T1 and long T2 on both sides of the occipital lobe.
The FLAIR image showed high signal.
It is a classic PRES image and supports the diagnosis of PRES.
2 PRES pathogenesis PRES is reversible, subcortical, vasogenic edema, the mechanism is as follows: normal cerebral perfusion pressure is maintained between 50-100mmHg, cerebral blood flow can only be achieved through cerebrovascular pressure response, chemical regulation and autonomic nerve regulation.
In a stable state.
Under pathological conditions, excessive blood pressure fluctuations exceed the self-regulation capacity of blood vessels, or excessive cytokine release in the circulation leads to impaired endothelial function, and autonomic nerves are insufficient to regulate perfusion pressure, which will cause a series of undesirable consequences, leading to cerebrovascular permeability Sexual changes, which are the pathological basis of vasogenic brain edema.
Causes of PRES: common hypertension (mostly malignant hypertension), pregnancy eclampsia, renal insufficiency, etc.
The rapidly progressing hypertension exceeds the upper limit of cerebral blood flow self-regulation, resulting in high blood flow perfusion, leading to blood The destruction of the brain barrier and the exudation of plasma and macromolecules cause vasogenic brain edema.
The blood pressure of the patient is normal, which does not support this theory.
About 50% of PRES patients have a history of autoimmune diseases, and the use of immunosuppressive agents or cytotoxic drugs may also be one of the triggering factors of PRES.
Systemic lupus erythematosus, drug immunosuppressants such as cyclosporine, tacrolimus, sirolimus; chemotherapy drugs such as methotrexate, cisplatin, cytarabine, vincristine, sunitinib, Bevacizumab; immunomodulators such as rituximab; sepsis, hypomagnesemia, hypocalcemia, all of the above factors can cause damage to endothelial function, damage to the blood-brain barrier, and increase vascular permeability , Causing vasogenic brain edema.
Malignant tumors are a common cause of PRES.
Studies have found that a variety of tumors can cause PRES, among which solid tumors such as lung cancer, ovarian cancer, kidney cancer, and gastric cancer account for 71%, blood tumors such as lymphoma and leukemia account for 26%, and primary intracranial tumors such as glioma account for 3% %.
Therefore, some scholars have proposed that PRES is a neurological manifestation of malignant tumors.
The mechanism of tumors leading to PRES may be that a variety of cytokines, cytotoxins, chemotherapeutics, etc.
directly damage the blood-brain barrier, causing vasogenic edema.
This patient has a clear history of lung cancer, and most of the tumors have immune abnormalities.
At the same time, oral targeted drugs can directly damage the vascular endothelium.
The FLAIR image of the patient showed high signal in both occipital lobes, no high signal in DWI, and no low signal in ADC phase, suggesting that the bilateral occipital lobes were diffusely unrestricted, which was consistent with vasogenic cerebral edema.
3PRES clinical manifestations: headache, epilepsy, disturbance of consciousness, and visual disturbance are the most common in PRES.
Headache is the most common clinical manifestation of PRES, which is related to the increase in intracranial pressure caused by cerebral edema, and may be accompanied by nausea and vomiting.
Seizures can be the first symptom of the nervous system.
The seizures have various forms, which can be partial seizures, and usually develop into generalized tonic-clonic seizures.
Almost all patients have visual abnormalities (such as blurred vision, hemianopia, enlarged visual blind zone, cortical Sexual blindness, etc.
).
Visual abnormalities often indicate that the disease involves the occipital visual center that supplies blood to the back of the brain.
This patient has visual abnormalities and paroxysmal amaurosis, which relieves in 3-5 minutes, and is considered to be a seizure, which is consistent with the imaging changes of the bilateral occipital lobes of the head MRI.
4 Differential diagnosis The patient should be differentiated from the following diseases: (1) Radiation encephalopathy: The patient has received radiotherapy and should be differentiated from radiation encephalopathy.
The lesion location is basically the same as the range of radiation, and the lesions are mainly distributed in the temporal lobe, brain stem, and cerebellum.
; The irradiated site of this patient is the left temporal lobe, which is not consistent with the lesion site.
(2) Brain metastases: The patient has a history of brain metastases from lung cancer.
Metastases should be identified, but the lesions are bilaterally symmetrical, and no metastatic lesions are found, which can be excluded.
5 Treatment and prognosis After timely and effective treatment of PRES, the above symptoms and signs improved rapidly, and generally no neurological sequelae were left.
However, some patients may have permanent neurological damage.
Discovering and reversing/eliminating potential predisposing factors as soon as possible can usually relieve the clinical symptoms of patients and improve their prognosis.
In this case, lung cancer complicated by PRES, considering the existence of immune factors, received mannitol and methylprednisolone treatment for 3 days, the symptoms disappeared, the effect was good, and the effect of mannitol alone was not good in the early stage.
Although PRES is mostly a benign disease, its lesions can completely resolve within a few days to a few weeks, but not all patients have a good prognosis.
Increased creatinine levels, high-intensity areas on MRI diffusion-weighted images, and broader brain tissue involvement (especially involving the brain stem) are signs of poor prognosis.
