Hemangioma Syndrome, Uremic Encephalopathy, LGI-1 Encephalitis 3 Minute Reading·34 Issue

The 3-minute reading column is updated once every two weeks, and highlights the wonderful cases of the past two weeks for everyone to learn and discuss
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There are thunderous breakfast case studies for medical pulse nerves five working days a week, 3 short and succinct 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.
They often wander in the group and gain new gains every day
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Authors: Zhao Wei, Zhao Lianhua Cases provided: Liu Bo, Chen Donghui, Xu Haoyou, Liu Zhihong, Li Jinsong, Huang Zhiqiang, Zhao Zihan, Lu Fang, Zhou Ming, Zhou Xiaoyu, Liu Liquan, Zhou Shanshan, Liu Buyun, Shi Zhenghao, Xie Jianguo, Zhao Qiong, He Zhongyan, Wang Shang, Li Huiming, Shen Yongguo, Liang Xinming, Huang Botao Case 1 is a male patient, 82 years old, with sudden unconsciousness with intermittent convulsions for half a day
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Recheck the following figure after 8 days
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Answer: Thrombosis of the left transverse sinus and sigmoid sinus
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Cerebral venous thrombosis (CVT) can cause headaches, focal neurological deficits, and seizures
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Transverse sinus thrombosis usually leads to hemorrhagic infarction of the temporal parietal lobe (due to Labbé vein occlusion) and headache.
Involvement of the left side can cause aphasia and seizures
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Isolated sigmoid sinus involvement is rare, but it can cause pain in the mastoid process and rarely cause neuropathy at the base of the skull
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CT sinus or deep vein high density (compact triangle sign or strip sign) can be seen in 1/3 of patients
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In stroke patients, headaches and seizures are more likely to be CVT rather than arterial ischemic stroke (up to 40%).
Neuroimaging does not match the infarction of a single arterial blood supply and the obvious bleeding component supports CVT
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 Case 2 is a male patient, 83 years old, who has been dizzy repeatedly for more than 2 months
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Answer: Sturge-Weber syndrome (SWS)
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The main characteristics of SWS: Facial skin capillary hemangioma is located in the area where the first or second branch of the trigeminal nerve is distributed, often unilateral, about 10% are bilateral
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Meningeal uveoid hemangioma consists of dilated veins located under the arachnoid membrane, often involving the occipital and temporal lobes of the brain
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Clinical symptoms: 1.
Skin changes: Red wine-colored flat vascular nevus can be seen at birth, distributed along the first branch of the trigeminal nerve, and can also spread to the second and third branches.
In severe cases, it can spread to the opposite side, neck and trunk
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2.
Nervous system symptoms: epilepsy, mental disorders, spastic hemiplegia, hemiplegia, hypoalgesia, behavioral disorders and migraine in some cases
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3.
Ocular symptoms: 30% of patients are accompanied by glaucoma and exophthalmos.
Damage to the occipital lobe can lead to contralateral hemianopia, congenital iris defect and lens opacity
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 Case 3, a 35-year-old female patient with a normal body, had sudden left limb weakness for 4 days
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Answer: Acute cerebral infarction, dissection of the C1 segment of the right internal carotid artery
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1% to 2% of ischemic stroke are caused by carotid artery dissection (CAD), but in young patients, the incidence of ischemic stroke caused by CAD is as high as 25%
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Typical manifestations of CAD include neck pain, headache, Horner syndrome, and secondary ischemia
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The typical sign of carotid artery dissection is a crescent-shaped hyperintensity on the upper canal wall hematoma on cervical MRI T1-weighted image
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DSA is beaded on the performance stenosis or occlusion (flame syndrome), vascular smooth tapered or irregular (rat tail sign, line-like symptoms), pseudoaneurysm, free intimal flap like
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 Case 4, a 45-year-old female, was admitted to the hospital due to "loss of memory for 6 months"
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Answer: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with subcortical infarcts and leukoencephalopathy
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The main clinical manifestations of CADASIL include migraine, subcortical ischemic events, cognitive decline and affective disorders
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Image features include white matter hyperintensity (WMH), lacunar infarction, and microhemorrhage (CMB)
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WMH initially affects the center of the semi-oval, and then gradually expands to the bilateral temporal poles, outer capsule, and corpus callosum as the disease progresses
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The characteristic temporal pole WMH (O'Sulliva sign) and the lateral high signal of the external capsule ("person" sign) are the imaging signs for the diagnosis of CADASIL
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 Case 5, a 55-year-old male, was admitted to the hospital with "twice seizures within a month"
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Answer: LGI-1 encephalitis
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The most common clinical manifestations of anti-LGI1 encephalitis are facial-brachial dystonia (FBDS) and hyponatremia.
In addition, seizures, cognitive impairment and insomnia are also common
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The diagnostic criteria of LGI1 encephalitis: (1) Acute or subacute onset and progressive exacerbation
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(2) It is clinically consistent with limbic encephalitis, or manifested as FBDS
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(3) The number of white blood cells in the cerebrospinal fluid is normal or shows mild lymphocytic inflammation
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(4) Head MRI: Bilateral or unilateral medial temporal lobe abnormal signal, or no obvious abnormality
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(5) Abnormal EEG
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(6) Anti-LGI1 antibody positive in serum and/or cerebrospinal fluid
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 Case 6 was a 74-year-old male with a history of diabetes.
He was treated with “cerebral infarction” at the local hospital due to “numbness and weakness on his left side for a week”.
He had a convulsive seizure one day before admission
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Answer: cortical vein thrombosis
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The imaging manifestations of cortical vein thrombosis lack specificity, and can manifest as cerebral infarction, cerebral hemorrhage, cerebral edema and subarachnoid hemorrhage
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When the extent of the lesion is relatively small, it can be confined to the cortex and manifests as an isolated lesion
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Plain scan CT can show high signal, FLAIR high signal on MRI, low signal on T2*\SWI, and high signal on T1WI
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 Case 7 The patient is 57 years old, male, convulsed limbs with unconsciousness for 3 hours
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He has maintained hemodialysis treatment for diabetic nephropathy and uremia in the past
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Answer: uremic encephalopathy
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Uremic encephalopathy can be manifested including various neurological symptoms, such as movement disorders (tremor, myoclonus), cognitive impairment, and changes in mental status
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It should be noted that the lentiform fork sign (LFS) can be present in metabolic acidosis caused by any cause, including end-stage renal disease, methanol poisoning, and dialysis imbalance syndrome
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 Case 8 The patient is 30 years old, male, and has utterly unclear speech for more than 5 years
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Answer: Hepatolenticular degeneration
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Supplementary information: Ceruloplasmin <0.
1 g/L
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Abdominal ultrasound: diffuse liver disease
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Patients with typical hepatolenticular degeneration usually refer to patients with liver involvement and/or extrapyramidal symptoms (dyskinesias, dystonia, and psychiatric symptoms), while the corneal KF ring is positive, and serum ceruloplasmin is significantly reduced (< 200mg/L) and 24h urine copper content increased (>100μg)
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Typical imaging manifestations CT can show bilateral lenticular nucleus symmetry low-density shadow
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MRI showed low signal and high signal on T1-weighted images of the putamen (especially putamen), caudate nucleus, midbrain and pons, thalamus, cerebellum, and frontal cortex, or putamen and caudate nucleus on T2-weighted images The image shows high and low confounding signals, and there may be different degrees of brain atrophy
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 Case 9 is a 57-year-old female patient with repeated headaches for 2 months
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Answer: Multinodular and Vacuolating Neuronal Tumors (MVNT)
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MVNT is a kind of benign superficial neuron tumor, mostly manifested as epileptic seizures
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MRI showed small clusters of subcortical, cystic, nodular lesions, T2/FLAIR showed high signal (like many bubbles) (T1 may have a slightly low signal)
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The lesions are mostly located in the deep cortical zone and the white matter under the superficial skin layer, and most of them have no obvious enhancement, but may be slightly enhanced, without diffusion limitation and magnetic sensitivity effects
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The lesions rarely progress or increase in size, mostly without mass effect and peritumoral edema
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Differential diagnosis: Dysplastic neuroepithelial tumor (DNET).
DNET (below) has a similar appearance, but is usually located in the cortex (rather than under the cortex) and usually has a bright FLAIR ring
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Case 10 is a 56-year-old female patient.
Two days after vaccination, her right lower limb suddenly became weak
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Answer: Lymphoma of the central nervous system
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Typical performance: ➤ Location: 80% are located in the supratentorial cerebral hemisphere, periventricular white matter, deep gray matter nucleus, corpus callosum, and subependymal area
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Atypical parts: posterior fossa, brainstem, cranial nerves, cavernous sinus, sellar area, pineal area, spinal cord ➤CT: slightly higher density, obvious enhancement
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➤Low signal such as TI, high signal such as T2, high signal such as DWI, low signal of ADC ➤Low perfusion such as ASL, high perfusion after hormone treatment ➤MRS NAA↓ Cho↑, tall Lip peak, visible Lac peak ➤Significantly strengthened (hand fist Sign, sharp angle sign, fissure sign) Case 11, a 29-year-old female patient with dizziness and weakness of both lower limbs for 6 months
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Answer: Adult Alexander's disease (AxD)
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Alexander disease is a rare autosomal dominant leukoencephalopathy caused by mutations in the glial fibrillary acidic protein (GFAP) gene.
According to the clinical symptom group and its prognosis, it can be divided into type I and type II.
Among them, type I is mostly early onset, manifesting as epilepsy, giant skull, developmental delay, etc.
, the disease is exacerbated by paroxysmal, progressing faster, and the survival period is short; type II is mostly late onset, manifesting as symptoms of the back of the brain, such as dizziness, Autonomic nerve dysfunction, abnormal eye movements, bulbar palsy, etc.
, progress slowly, generally without obvious developmental abnormalities, and have a relatively long survival period
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The typical imaging changes are manifested by head MRI as the medulla oblongata, the upper cervical spinal cord atrophy is "tadpole-like", and there may be demyelinating changes in the white matter of the brain
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When it is found that the patient has brainstem symptoms and obvious bulbar atrophy, the possibility of AxD Ⅱ should be considered
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 Case 12, a 1-year-old male; convulsions for 1 day
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Answer: Nodular sclerosis
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The typical clinical triad is: epilepsy, mental retardation and facial sebaceous adenoma, namely "Vogt triad"
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CT shows nodules or calcifications under the ependyma and around the ventricle, with round or irregular high-density, and multiple lesions on both sides; enhanced scan nodules are clearer, calcification without enhancement; sometimes multiple occurrences in the cortex or white matter Small nodular calcification, its density is lower than that of ventricle wall calcification, and the boundary is not clear; if it occurs in the cerebellum, it may be extensive nodular calcification; blocking the cerebrospinal fluid channel may cause hydrocephalus; some of the ventricle enlargement and brain atrophy; a few Cases may be combined with subependymal giant cell astrocytoma
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The subependymal nodules usually showed equal or slightly high signal on T1WI, high signal on T2WI and FLAIR, and low signal on calcification; enhanced scanning nodule enhancement
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 Case 13 is a 13-year-old female with left upper limb weakness for one month
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Answer: germ cell tumor
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The imaging features of germ cell tumors in the basal ganglia: the tumor is large in size, but the space-occupying effect is not obvious, the edema around the tumor is light, and the cerebral cortex in the ipsilateral sylvian fissure is mostly accompanied by atrophy
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The enhanced scan showed irregular rosette-like enhancement or spot-like enhancement
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