Hypertrophic dura mater, motor neuron disease, hypotension syndrome

The 3-minute film reading column is updated every two weeks, and selected wonderful cases in the past two weeks for everyone to learn and discuss
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Medical pulse nerve has a fast-paced breakfast case study five working days a week, 3 short and powerful small cases every day, and 3 knowledge points every day
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The collision of everyone's thoughts has produced countless sparks of wisdom, often wandering in the group, and getting new harvests every day
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Case provided: Zhao Fengzhu, Liu Dequan, Zhang Wensheng, Zhuo Xiuwei, Wang Jiemin, Wei Shuyan, Li Hongjin, Wang Shuzhen, Li Huiming, Liang Xinming, Shen Yongguo, Huang Botao, etc.
Authors: Zhao Wei, Zhao Lianhua Case 1 Female, 52 years old, left Sideways numbness for 10 days
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Answer: Cerebral infarction caused by hypereosinophilic syndrome (HES)
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Supplementary medical history: bone marrow cytology examination showed: blood: eosinophils accounted for: 32.
0%, the shape and staining were generally normal; bone marrow: eosinophils accounted for 20% of the classification, no obvious change in morphology
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Eosinophilia can be divided into primary and secondary diseases, primary is a monoclonal expansion disease, and secondary often occurs in, for example, drugs, parasitic infections, autoimmune diseases, or malignancies.

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The most common imaging manifestations of HES-related cerebral infarction are that the infarcts are distributed in the inner and outer border areas, the watershed area and the blood flow distribution area outside the watershed
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The mechanism may be related to cardiogenic embolism or coagulation dysfunction, and hypoperfusion is also an important factor in HES-induced cerebral infarction
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MRI is nonspecific, and cardioembolism, Trousseau syndrome, and systemic hypoperfusion should be considered
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The first-line treatment for HES is glucocorticoid, whose mechanism of action is to effectively inhibit the production of eosinophils and reduce the level of eosinophils in peripheral blood; at the same time, inhibit the release of inflammatory mediators from eosinophils and reduce their effect on the arterial intima.
damage
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At the same time, antiplatelet and statin drugs should be given to patients without contraindications
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 The case was a 228-year-old female with slurred speech and limb weakness for more than 4 years, which worsened for 4 days
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Answer: Behcet's disease
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A recurrent inflammatory disease involving multiple systems, including the triad of oral ulcers, genital ulcers, and uveitis, typical MRI findings of neurobehcet's disease: acute-phase lesions are mainly in the basal ganglia, brain stem, diencephalon, and endometrium Capsules, etc.
, are sheet-like long T1, long T2 signal, prominent edema, chronic phase lesions can shrink or disappear completely
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This imaging change indicates that the acute phase lesions are mainly venous congestion and tissue edema, rather than arterial occlusion causing cerebral infarction
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 Case 371-year-old female, headache for 3 months, worsening and loss of appetite for 3 days
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Answer: Hypertrophic pachymeningitis (HP) is a group of diseases characterized by focal or diffuse dura mater hypertrophy, with chronic headache, multiple groups of cranial nerve damage, and focal brain damage as the main clinical manifestations.
The general term for cerebral MR shows thickened and enhanced dura mater
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Can be divided into secondary and idiopathic
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Secondary is common in (1) various infections: bacteria, viruses, fungi, Mycobacterium tuberculosis, etc.
; (2) chronic inflammatory diseases related to autoimmunity; (3) metastasis and infiltration of malignant tumors; (4) trauma , drugs, etc.

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Proliferative dura mater of unknown etiology, called idiopathic Hypertrophic Pachymeningitis, can cause diffuse or focal damage to the entire intracranial dura mater, usually affecting the falx and tentorium , parasellar and cavernous sinus
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 Case 449-year-old female, headache for 3 days, aggravated with vomiting for 15 hours
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Auxiliary examination: blood routine: N 93.
80%, WBC (white blood cell count) 10.
90×109/L
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FDP 9.
5ug/mL, D-dimer 2.
73ug/mL
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Answer: intracranial venous thrombosis (cerebral venous thrombosis, CVT) cerebral angiography showed intracranial venous thrombosis, urokinase 1 million units of intravenous thrombolysis
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After thrombolysis, he developed numbness and weakness of the right upper extremity, and a re-examination of the head CT showed cerebral hemorrhage and intraventricular hemorrhage
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Subsequently, the patient was transferred to the hospital for intravenous thrombectomy and stent implantation.
After long-term anticoagulation, the patient was followed up for 1 month without remaining symptoms of neurological deficit
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Figure 4A-B head CT showed cerebral hemorrhage and hemorrhage after thrombolysis (A), and head CT after 2 months showed disappearance of high signal in venous sinuses (B) CVT accounts for about 0.
5%~1 of all cerebrovascular diseases %
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Imaging features: CT plain scan: the direct sign is a high-density "cord sign" consistent with the location of the venous sinus, and the superior sagittal sinus thrombus is a high-density "delta sign" in the coronal view
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Simple cortical venous thrombosis is a cord-like density of increased density on the surface of the brain
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Enhanced CT: It can show the filling defect of contrast medium in the venous sinus cavity during thrombus
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In the coronal view, the thrombus in the superior sagittal sinus can present a typical "empty triangle sign"
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MRI: In the acute phase, T1, etc.
, and T2 low signal can be seen, in the subacute phase, both T1 and T2 are high signal, and in the chronic phase, both T1 and T2 are low signal
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DSA can show occluded venous sinuses without visualization or filling defects, delayed visualization of cerebral venous sinuses, prolonged capillary phase, dilated collateral draining veins, increased visualization of scalp veins around affected veins, and reversal of venous flow direction
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 Case 5 is a female, 52 years old, unable to take care of herself for 2 years
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The patient gradually appeared unable to cook 2 years ago without obvious incentives, accompanied by a gradual decline in basic daily living abilities, memory, calculation and other abilities
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Now it has gradually developed to be unable to recognize the time and location, unable to calculate, and unable to take care of life completely
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Physical examination: uncooperative deep and superficial sensory examination, normal tendon reflexes of limbs, uncooperative motor examination, bilateral pathological sign (-), and negative meningeal irritation sign
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The location and time cannot be answered.
Calculation: 100-7=?, the memory answer is wrong, and the MMSE score (11 points)
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Review after 2 years: Answer: Posterior cortical atrophy (PCA) is a neurological disorder characterized by optic-spatial disturbance, visual agnosia, and apraxia, with parietal occipital cortical atrophy and progressive cognitive impairment.
Systemic degenerative diseases
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The age of onset of PCA is earlier than that of typical AD, and it occurs more than 50 to 65 years old.
Patients usually develop early visual and posterior cortical non-visual symptoms, including Balint's syndrome, visual agnosia, apraxia and environmental disorientation, and Gerstmann's syndrome.
Symptoms of visual cortical abnormalities are more pronounced than memory, language, and other cognitive abnormalities
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Neuroimaging manifestations (1) MRI: the posterior cortical atrophy is dominated by the posterior cortical atrophy, with significant atrophy in the occipital and parietal cortex, followed by the temporal cortex; in Alzheimer's disease, the middle temporal cortex, Especially hippocampal atrophy
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 Case 643 years old, female, right lower extremity weakness for 1 year, vesicular tremor on the left tongue edge, normal hearing in both ears, muscle strength of both upper extremities 5-grade, right lower extremity muscle strength 4, left lower extremity muscle strength 5-grade, limbs The muscle tone was slightly increased, the tendon reflexes of both lower extremities were slightly increased, the finger-nose test was normal, the Achilles-knee test was normal, the pathological signs of the left lower extremity were positive and suspicious, the meningeal irritation sign was negative, and the pain sensation was normal
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Three items of anemia: B12 (vitamin B12) 154.
00pg/ml↓ Answer: Motor neuron disease (primary lateral sclerosis)
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Corticospinal tract hyperintensity can predict motor neuron disease.
This patient showed bilateral symmetric flair sequence hyperintensity in the cerebral peduncle; localized symmetrical punctate flair sequence hyperintensity in the hindlimbs of the internal capsule; bilateral precentral gyrus flair sequence white matter hyperintensity All lesions showed white matter hyperintensity along the vertebral tract, and the symmetrical "reverse comma" in the hindlimb of the internal capsule was the most typical
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It should be noted that a variety of diseases can cause symmetrical damage to the bilateral corticospinal tracts
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Such as Krabbe disease, vitamin B12 deficiency, Leigh syndrome, hepatic encephalopathy
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 Case A 753-year-old female with progressive slurred speech and tongue muscle atrophy for 6 months
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Physical examination cooperation, dysarthria can only vocalize, cannot understand, dysphagia, bilateral nasolabial folds are symmetrical, tongue extension is centered, tongue muscle vibration, tongue muscle contraction, normal hearing in both ears, normal muscle tone and muscle strength of limbs , The tendon reflexes of the limbs were active, the finger-nose test was normal, the Achilles-knee test was normal, and the bilateral pathological signs were (+)
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Answer: Motor neuron disease (ALS)
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The patient's tongue muscle was significantly atrophied, the surface of the tongue was curled like undulating waves, and the teeth marks on the edge of the tongue muscle were obvious; the thenar muscle was atrophied, and the palm was flat.
Considering motor neuron disease, oral riluzole and edaravone were administered intravenously.
After half a year, the patient swallowed Difficulties aggravated, unable to speak and eat at all, and a gastrostomy was performed
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Motor neuron disease (MND) is a series of chronic progressive neurodegenerative diseases characterized by damage to the upper and lower motor neurons
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Usually insidious onset and slow progression
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The clinical manifestation is a different combination of upper and lower motor neuron damage, characterized by muscle weakness and atrophy, bulbar palsy, and pyramidal tract signs
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Sensory systems and sphincter function are usually spared
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The damage is limited to the lower motor neurons, showing weakness and muscle atrophy without pyramidal tract signs, which is progressive muscular atrophy (PMA)
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Individual damage to the medullary motor nucleus and manifested as laryngeal muscle and tongue muscle weakness, atrophy, for progressive bulbar palsy
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Primary lateral sclerosis (PLS) occurs when only the pyramidal tract is involved and presents with weakness and pyramidal tract signs
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If both upper and lower motor neurons are damaged, it is called amyotrophic lateral sclerosis (ALS)
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Of these, ALS is the most common
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 Case 8, a 57-year-old male, was found to have muscle atrophy in his limbs for 10 years
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Answer: Distal muscle atrophy is predominant in patients with peroneal muscle atrophy (CMT), chronic disease course, and MRI shows thickened nerve roots
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CMT is the most common hereditary peripheral nerve disease clinically, with onset more than 20 years old, and symptoms progress slowly.
Common clinical symptoms and signs include: difficulty walking or running, high arched feet, foot drop, cross-threshold gait, Distal limb muscle weakness, muscle atrophy, inflexibility of hands, paresthesia in distal limbs, weakened or disappeared tendon reflexes,
etc.

Peripheral neuropathy, such as Refsum disease and chronic inflammatory demyelinating polyneuropathy, also show thickened nerve roots
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 Case 9 was a 37-year-old female with head and neck pain for 1 month
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Answer: Hypotension Syndrome
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Imaging manifestations: ①Diffuse thickening and enhancement of the dura mater.
On T2W, the dura mater was diffusely and uniformly thickened and showed slightly high signal intensity.
The thickened dura mater after enhancement showed diffuse and uniform enhancement, and the pia mater on the surface of the brainstem and sulcus was not enhanced.

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② Downward shift of brain structure, manifested as lower position of midbrain, smaller height of chiasmal cistern, lower optic chiasm and smaller distance between pituitary and optic chiasm, lower hypothalamus and curled up on dorsum of saddle, and enlarged pons Transposition and narrowing of the anterior pons and tonsil downshift, etc.
, are collectively referred to as brain ptosis
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③ Subdural effusion or blood accumulation, subdural effusion manifests as crescent-shaped slightly longer T1 and long T2 signals under the inner plate of the skull, high signal on FLAIR, usually bilaterally symmetrical; subdural accumulation in the early onset With less fluid, FLAIR sequence has a great advantage in detecting a small amount of subdural effusion, and the performance of subdural hemorrhage varies with the period of bleeding
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④Other manifestations, the ventricle is smaller, the pituitary gland is enlarged, the choroid plexus is obviously enhanced, and the dural venous sinus is enlarged
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Case 1060 years old, female, was admitted to the hospital with general pain for more than 1 month
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The patient started to have systemic pain before 1 month, and then neck and bilateral back pain mainly occurred
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Later, gibberish, inaccurate answers, unable to recognize familiar relatives, reluctance to speak or communicate with others, symptoms vary from time to time
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Answer: Autoimmune glial fibrillary acidic protein astrocytosis (GFAP-A)
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Autoimmune astrocytopathies mainly include neuromyelitis optica spectrum disorders (NMOSD) and glial fibrillary acidic protein astrocytopathies (GFAP-A)
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GFAP-A is a group of neurological syndromes involving the meninges, brain, spinal cord and optic nerve, and its specific diagnostic marker is GFAP antibody
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The disease is more common in women over the age of 40
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More acute onset or subacute onset, manifested as progressive exacerbation or relapsing remission
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Clinical manifestations include fever, headache, involuntary movements, myelitis, optic nerve abnormalities, ataxia, psychomood disorders, epilepsy, autonomic dysfunction, and other signs and symptoms of meningo-encephalitis
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MRI shows multiple lesions, involving the white matter, basal ganglia, hypothalamus, brainstem, cerebellum, meninges, ventricles and even the skull.
Enhancement", which can be seen in about 40% to 50% of patients, can easily disappear after treatment
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 Case 11, a 45-year-old male, presented with a "3-day memory loss"
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Blood routine: hemoglobin 91g/L
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Biochemical AST 311.
9U/L, ALT 259.
8U/L
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The first eight items of blood transfusion: hepatitis B big three yang
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Abdominal CT: liver cirrhosis, slightly enlarged spleen, and massive fluid accumulation in the abdominal cavity
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Answer: chronic hepatic encephalopathy, chronic liver failure
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On T1WI, bilateral symmetrical hyperintensity in the globus pallidus and some internal capsule structures can be seen, and there are also caudate nucleus, substantia nigra, midbrain tegmental, and pituitary in the hyperintensity sites
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The worse the liver function, the higher the signal strength
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Spiral CT scan showed normal density of lesions such as globus pallidus
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The abnormal signal of MRI is mainly caused by the deposition of the paramagnetic substance manganese, and the exact mechanism of manganese deposition is not clear
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 Case 12 was a 37-year-old female with left limb weakness for 2 months, which was aggravated and accompanied by involuntary movements of the left arm for 1 day
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Diagnosed with myositis 5 years ago, he was cured after steroid pulse therapy
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Answer: Lupus encephalopathy
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Supplementary medical history and examination (cerebrospinal fluid): cerebrospinal fluid leukocytes 15.
0/ul↑, biochemical (cerebrospinal fluid): cerebrospinal fluid total protein 1.
77g/L↑, immunity (blood): anti-ribonucleoprotein/Smith antigen antibody (nRNP/Sm) positive++++ +↑, anti-Smith antigen antibody (SM) positive ++↑, anti-cardiolipin antibody weakly positive ↑, pathology department antinuclear antibody weakly positive, dilution 1:100, double-stranded DNA antibody negative, dilution 1:10
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Both blood and cerebrospinal fluid were negative for autoimmune antibodies
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Paraneoplastic antibodies were negative
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In addition to lupus encephalopathy, MELAS and autoimmune encephalitis should also be considered
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 Case 1338, a female, had episodic loss of consciousness with limb convulsions for 1 day
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Answer: Posterior reversible encephalopathy syndrome (PRES) combined with HELLP syndrome
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Supplementary information: The patient had an intrauterine pregnancy of 30+3 weeks, blood pressure at the onset of 200/110mmHg, epigastric pain 1 day before, accompanied by nausea and vomiting.
Blood routine: PLT 88×109/L; liver function: ALT 211U/ L, AST 364U/L, total bilirubin 33.
13umol/L, indirect bilirubin 30.
25umol/L, LDH 1070U/L Urine was dark brown, urine routine: protein 3+
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PRES usually has acute or subacute onset, and manifests as headache, seizures, disturbance of consciousness, visual disturbance, and focal neurological deficits
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Among them, headache, disturbance of consciousness and seizures are more common.
The typical imaging features of PRES in the acute phase are vasogenic edema dominated by parieto-occipital subcortical white matter, T1 low signal, T2/Flair high signal, DWI or other low signal , ADC high signal
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In addition to the parieto-occipital lobe, lesions often involve the frontal lobe, temporal lobe, and cerebellum
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HELLP syndrome, characterized by hemolysis (H), elevated liver enzymes (EL), and low platelets (LP), is a serious complication of hypertensive disorders of pregnancy
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 Case 14 was a 17-year-old female with intermittent convulsions and poor right limb movement for more than 12 years
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On physical examination, the patient's left frontal face was sunken with hair loss
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Answer: Linear scleroderma with intracranial lesions
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 Case 15 female, 2 years old, with intermittent right limb convulsions for 10 months, and developed well
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Answer: Linear scleroderma with intracranial lesions
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Linear scleroderma with central nervous system involvement is rare.
Common symptoms include seizures, migraine, mental and behavioral abnormalities, trigeminal neuralgia, Rasmussen encephalitis, and ocular symptoms.
Brain MRI shows brain parenchyma calcification and abnormal white matter signal.
, vascular malformations and vasculitis, lesions can occur in distant parts of the skin lesions
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 Case 16 female, 14 years old, headache with blurred vision for 2 days, convulsions 7 times
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Health in the past
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Physical examination: hairy on the body surface
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Answer: MELAS
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Cerebrospinal fluid lactate: 5.
7, MRS: Inverted lactate doublet was seen in the right occipital
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MRI of MELAS syndrome shows signal changes in the posterior cortex and does not conform to typical large vessel distribution; other features include brain atrophy, signal changes and calcifications in the basal ganglia, and leukodystrophy
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T2 and FLAIR hyperintensities are commonly seen in the acute phase, with limited diffusion on DWI imaging, and increased signal on DWI and ADC imaging in both the subacute and chronic phases
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These changes in cortical signalling are generally transient, and most patients have residual focal cortical atrophy
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 Case 17 male, 4 months, intermittent convulsions for 2 months, repeated status epilepticus
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Answer: Sturge-Weber syndrome type 1
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The patient had significant port wine stains and glaucoma on the left side of the face since childhood
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Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous syndrome
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The clinical manifestations are facial hemangioma (port wine stain), often accompanied by symptoms such as epilepsy, glaucoma, intellectual disability, hemiplegia and migraine
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The main pathological manifestations of SWS are hemangioma involving the pia mater, facial trigeminal innervation area and choroid of the eye; the affected brain tissue often has local atrophy and calcification due to local vascular malformation and vascular stasis
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