Spinal cord vascular malformations, intracranial tumors, Wernicke's encephalopathy

The 3-minute reading column is updated every two weeks, and highlights the wonderful cases of the past two weeks for everyone to learn and discuss.

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.

The collision of everyone's thoughts has produced countless sparks of wisdom.
They often wander in the group and gain new gains every day.

Summary: Zhao Wei, Zhao Lianhua Cases provided: Shao Mingtao, Li Huiming, Zhang Kun, Liu Xiang, Wang Jie, Ou Kaiyun, Zhu Haibing, Wang Xudong, Cheng Wei, He Zhongyan, Yu Xiaogang, Xie Guangdi, Lu Huadong, Liu Bo, Xie Hongrong, Li Yutao, Xu Dandan, Zhang Leguo, Liu Dequan, Liang Xinming, Shen Yongguo, Huang Botao Case 1 female, 41 years old.

Sudden weakness of both lower limbs for 6 hours.


Answer: Spinal vascular malformation and bleeding.

The results of angiography and surgery are as follows.

Spinal cord hemorrhage is acute and rare in clinical practice.
Vascular malformations are the main cause of spinal cord hemorrhage.

Mainly manifested as severe chest, waist and back pain and paraplegia, sensory disturbance and sphincter dysfunction.

Spinal cord hemorrhage and acute myelitis, spinal cord infarction are mutually distinguished.

Before the onset of myelitis, there may be triggers such as respiratory or digestive tract infections, nerve root irritation symptoms are not obvious, and MRI examinations can show abnormal high signals across T2WI.

Spinal cord infarction can be acute or subacute.
It usually occurs in middle-aged and elderly people.
It is more common with anterior spinal artery infarction, obvious dyskinesia, slight or absent sensory disturbance.
MRI examination T2WI shows high signal in the blood supply area of ​​the anterior spinal artery, and more posterior cords.
Not tired.

Case 2 is a 41-year-old female patient with intermittent weakness of both lower limbs for 2 months and laborious urination for 1 month.

Answer: Spinal dural arteriovenous fistula (SDAVF).

SDAVF is the most common spinal vascular malformation, mostly involving the thoracic and lumbar segments, accounting for 70% of spinal vascular malformations.

The disease is more common in middle-aged and elderly men, with slow onset and progressive exacerbation, and a few with acute onset, making clinical diagnosis difficult.

The clinical manifestations are abnormal gait, lower limb weakness, sensory disturbance, pain, with or without sphincter dysfunction.

Atypical symptoms can be misdiagnosed as peripheral neuropathy, such as unilateral weakness of the lower limbs.

It is misdiagnosed as diabetic peripheral neuropathy when it is manifested as bipedal sock-like paresthesia.

It was misdiagnosed as degenerative spinal disease when it showed radiation pain in the lower limbs.

Spinal cord MRI: T1 showed swollen spinal cord with low-intensity shadow with diffuse enhancement; T2 showed multi-segment high-intensity in the central part of the spinal cord (large white radish-like); mostly circular low-intensity on the dorsal side of the spinal cord (indicating dilated, twisted Veins, like small black sesame seeds).

Case 3 The patient is 81 years old and male.

Sudden low back pain accompanied by dysuria for 18 days, numbness and weakness of both lower limbs for 1 day.

Answer: Spinal vascular malformation-spinal dural arteriovenous fistula.

The analysis is the same as above.

Case 4 is a 70-year-old male.
The patient presented with symptoms of bacteremia when he presented to the emergency department.

Analysis of cerebrospinal fluid showed elevated protein levels and increased lymphocytes.

Answer: spinal syphilis.

Sagittal T2 and STIR images have high signal intensity, involving the posterior cord of cervical spinal cord at C5-C6 segment.

The enhanced scan showed no enhancement of the lesion and no hemorrhage.

Slight pia mater strengthens all over the front and back surfaces of the spinal cord.

Axial T2 image shows symmetrical hyperintensity on both sides of the posterior column of the spinal cord.

Posterior column disease is a late manifestation of neurosyphilis, leading to demyelination of the posterior column of the spinal cord.

The most common manifestations of T2-STIR images are symmetrical high signal and bilateral high signal.

Generally enhanced without reinforcement.

The main differential diagnoses to be considered include: multiple sclerosis: generally asymmetry, mild.

Transverse myelitis: usually not limited to the posterior column.

HIV vacuolar myelopathy: requires a history of HIV infection.

Tumor: Not limited to the posterior column, the spinal cord is thickened, and there is enhancement.

Ischemia: Acute attack, DWI diffusion is limited.

In the analysis of cerebrospinal fluid, Treponema pallidum specific antibody was positive, and there was no evidence of other microbial infection.

Case 5 The patient is 9 years old and male.

Mental retardation and developmental delay.

Answer: Mucopolysaccharidosis.

MRI showed moderate hydrocephalus, and the fourth ventricle, cerebral aqueduct, third ventricle, and lateral ventricle were significantly enlarged.

Brain atrophy accompanied by enlarged sulci and cistern.

There are obvious perivascular spaces, accompanied by cribriform signs of deep white matter around the ventricles.

Mucopolysaccharidosis is an inherited metabolic disorder caused by the accumulation of mucopolysaccharides in the body caused by enzyme deficiency.

Typical clinical manifestations include malformations of skeletal development, short stature, joint stiffness, hearing loss, hernia, corneal opacity, glaucoma, hepatosplenomegaly, heart and respiratory diseases.

Head MRI findings: hydrocephalus and brain atrophy, multiple cystic and linear abnormal signal shadows in the white matter around the lateral ventricle, and the gray matter of the brain is unclear.

Bone manifestations include: tongue protrusion on the anterior edge of the vertebral body, spinal stenosis (mostly located in the C1/C2 segment, due to dysplasia of the dentate and C1 posterior arch, and then compression of the cervical spinal cord junction, cervical myelopathy or spinal cord Cavitation), atlantoaxial dislocation or subluxation, kyphosis, scoliosis, cervical deformity.

Madelung's deformity, the wrist bones are small and the number is reduced, the proximal metacarpal bones are bullet-shaped, and the fourth metacarpal bone is short.

Case 6 is a 31-year-old female patient with hearing loss and intermittent convulsions 3 years ago.

Answer: Mitochondrial encephalomyopathy with hyperlactic acidemia and stroke-like episode syndrome (MELAS).

The clinical manifestations are multi-system involvement, mainly involving the central nervous system, muscles, auditory nerves, pancreas, heart, and kidneys.
Stroke-like seizures and epilepsy are the most common clinical manifestations, followed by muscle weakness, headache, mental retardation, and short stature.

Imaging features: The lesions are more likely to occur in the cortex and subcortical white matter areas, and the deep white matter is less affected.

It can be single or multiple, mostly asymmetrically distributed, inconsistent with the cerebral artery blood supply area, and it is variable and migratory; enhanced scan lesions are not enhanced or slightly enhanced, or line-like enhancements distributed along the gyri of the diseased area ; Often accompanied by basal ganglia calcification, mainly located in the globus pallidus, followed by the thalamus, dentate nucleus and diencephalon; brain atrophy and ventricular enlargement are more common and not specific, but the degree of atrophy is more obvious, which is not consistent with age.

Perfusion showed increased cerebral blood perfusion in the acute and subacute phases; double peaks of Lac, decreased NAA peaks, and increased Cho peaks appeared in the MRS lesion area.

Case 7 is a 56-year-old male patient with onset of epilepsy.

Answer: Astrocytoma (WHO classification II).

It is the most common tumor of the nervous system, accounting for about 5.
6% of intracranial tumors and 25% to 30% of adult gliomas.

The peak incidence is between 20 and 40 years old, and it can also be seen in children.
It is rare in the elderly, and it usually occurs in the frontotemporal lobes and brainstem.

The main clinical symptoms are epilepsy and increased intracranial pressure.

On MR, T1WI is low signal, and T2WI is high signal, with no enhancement or mild patchy enhancement.

If there is enhancement, it indicates that the lesion is progressing to a higher level.

Case 8 is a 13-year-old male patient with numb right upper limb and awkward movements for more than 3 months.

Answer: germ cell tumor.

Germ cell tumors can occur in the midline (pineal or suprasellar area), mediastinum or sacrococcygeal area and other parts; the prominent clinical manifestations are endocrine disorders, manifested as upper vision disorders and precocious puberty, and may be accompanied by hypothalamic function Disorders such as diabetes insipidus, polydipsia, lethargy, and obesity; germ cell tumors have specific locations and high calcification rates, which are the main basis for diagnosis.

The patient's head CT is as follows: Case 9 is a female, 31 years old, with headache for more than half a month, and worsened by 1 week, and was admitted to the hospital on March 25, 2020.

The patient had a headache half a month ago, and woke up during sleep, lasting several minutes, once a day.

The headache symptoms worsened 1 week ago, with periodic attacks (headache lasts for several minutes and relieved for several minutes), with an interval ranging from half an hour to 1 hour, and the duration and frequency of attacks gradually increase, about 7-8 times per day, basically bedridden State (except eating and two stools), the headache is obvious from sitting to standing, the headache is relieved when sleeping, and the pain is more awake.

Three days ago, the patient had blurred vision, which was obvious when looking at distant objects, and the visual impairment gradually worsened.

During the course of the illness, the patient felt feverish, self-tested body temperature was not high, and no nausea and vomiting.

Re-examination of cranial MRI on April 7, 2020: Lumbar puncture, cerebrospinal fluid pressure 450mm.

Cerebrospinal fluid routine: nucleated cells: 23×10^6/L, lymphocytes 96%.

Cerebrospinal fluid biochemistry: protein 0.
27g/L, chlorine 123mmol/L, glucose: 3.
48mmol/L.
Cerebrospinal fluid smear: Ink staining sees Cryptococcus neoformans.

Cerebrospinal fluid cryptococcal capsule antigen is positive.

Answer: Cryptococcal meningitis.

Colloid pseudocysts formed by the fusion of enlarged VR gaps, diameter> 5mm, single or multiple, can be clustered into clusters of cysts.

The distribution of the lesions is consistent with the expansion of the VR gap.
It is often located in the basal ganglia area.
It is symmetrically distributed.
It is the mucus produced by the cryptococcal capsule.
The gelatinous substance is filled and expanded to form a small cavity containing a large number of cryptococcal bacteria, which is soapy.
Bubbly, with certain characteristics.

It has a round shape with clear borders.
T1WI shows low or slightly low signal, T2WI shows slightly higher or high signal, enhancement can be enhanced (enhancement of chronic inflammatory response considered to be an immune intact host), and diffusion can be limited.

 Case 10, a 52-year-old male patient, was admitted to the hospital due to "hallucinations and gibberish for 3 days, with double vision for 2 days".

Answer: Wernicke's encephalopathy.

Areas that are susceptible to damage caused by thiamine (vitamin B1) deficiency include the thalamus, papillary body, peri-aqueduct and paraventricular areas, locus coeruleus, cranial nerve nuclei, and reticular structures.

The clinical manifestations include the following three symptoms: ophthalmoplegia, ataxia and encephalopathy symptoms, changes in mental or intellectual status.

 Case 11, a 26-year-old female patient, was admitted to the hospital with “repeated limb twitches for 11 years, unfavorable speech, and unresponsiveness for 4 days”.

The patient had epileptic seizures for the first time when he was 15 years old.
It was manifested by foaming at the mouth, tilted head to the right, twitching of the limbs, and loss of consciousness.
It lasted for a few seconds and then relieved on its own.
It recurred after a period of time, often before each seizure.
He was diagnosed as "epilepsy" due to fever and headache.
He was treated with Debakin and stopped after half a year of self-administration.

The patients were 19 years old, 21 years old, 25 years old, and in November this year.
The above-mentioned epileptic seizures occurred.
The family members of the patients complained that the duration of each seizure was less than 1 minute, the seizures were frequent, and the intermittent period gradually shortened.

The patient developed speech problems and unresponsiveness 4 days ago.

2018.
11.
3 NMR is as follows: 2018.
12.
27 NMR is as follows: 2019.
1.
19 NMR is as follows: Answer: MELAS.

Typical imaging findings: CT: calcification of the basal ganglia is partly seen.

MRI: T2WI, FLAIR, DWI sequence corresponding to high signal; ADC can be high signal, low signal or mixed signal, suggesting the presence of cytotoxic edema and vascular edema; Magnetic resonance spectroscopy (MRS) suggests the presence of lactic acid in the brain .

The lesions are mostly distributed in the cortex and subcortical white matter, and the deep cortex is not involved; the changes of the lesions in the acute to subacute period may fluctuate significantly and migrate to completely disappear; brain atrophy after repeated attacks.

Case 1261-year-old male suffers from epilepsy.

The head MRI in 2014 is as follows: The head MRI in 2020 is as follows: Answer: Cerebral amyloid angiopathy-related inflammation (CAA-RI).

The onset is mostly acute or subacute.
The most common manifestations are cognitive and behavioral changes, which can be manifested as varying degrees of dementia, hallucinations, personality changes, consciousness disturbances and even coma, followed by hemiplegia, hemisensory disturbance, aphasia, and shared Focal neurological impairment such as dysfunction.

In addition, there are many patients with symptoms such as epilepsy and headache.
Compared with CAA alone, the incidence of intracranial hemorrhage is lower.

The most common manifestations on MRI are obvious enhancement of the pia mater and high-intensity foci of subcortical white matter on T2WI or FLAIR.
The lesions are often asymmetric and can be scattered or fused into slices, with less gray matter involved.

Case 13 is a 59-year-old female with headache, nausea and vomiting for 2 days.

Answer: Reversible posterior encephalopathy syndrome (PRES).

PRES is essentially a reversible vasogenic edema, accompanied by acute neurological symptoms, such as seizures, encephalopathy, headaches, visual disturbances, etc.
The common causes are hypertension, renal failure, use of cytotoxic drugs, and autoimmune diseases , Preeclampsia or eclampsia.

PRES often causes endothelial damage due to rapid blood pressure fluctuations or direct effects of cytokines on the blood vessel wall, causing damage to the blood-brain barrier, and then brain edema.

The disease is usually reversible, including imaging manifestations and clinical symptoms, and the prognosis is mostly good.

The reexamination head MRI is as follows: Case 14 See the picture to identify the disease.
Answer: Diffuse large B-cell lymphoma.

The initial symptoms of the disease are mostly limited manifestations, such as limb numbness, hemiplegia, aphasia, and ataxia.

Increased intracranial pressure is also common.

Mental symptoms can be seen when the tumor invades the corpus callosum, and arousal disorder can occur when the tumor invades the thalamus.

The predominant sites are the deep frontal and parietal lobe, basal ganglia, periventricular and corpus callosum, hypothalamus, and cerebellum.
They can also be involved.
They often infiltrate the subependymal tissue and spread to the meninges through the cerebrospinal fluid.

Some domestic scholars believe that "fist-like" or "brain-like" enhancement is only seen in lymphoma cases.

 Case 15 is a 29-year-old female patient who was admitted to the hospital mainly because of progressive decline in visual acuity in the right eye for 5-6 days.

Answer: Mog-related neuromyelitis optica.

At the first onset, 35.
4% of MOG antibody-positive patients had supratentorial lesions and 14.
6% had subtentorial lesions.

The first-onset supratentorial MRI lesions include periventricular white matter lesions, corpus callosum lesions (some may have fusion), frontal, parietal, temporal and deep occipital white matter lesions, cortical or subcortical (including insula) lesions, thalamus and basal nerves Section lesions.