Supranuclear palsy, subacute combined degeneration, gray matter heterotopia|3 minute reading•26 period

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.

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.

Authors: Zhao Wei, Zhao Lianhua, collation, review, case provided: Zeng Wei, Shao Mingtao, Zhang Kun, Li Huiming, Zhuo Xiuwei, Gao Yunqing, Li Huiming, Shen Yongguo, Liang Xinming, Huang Botao Case 1 male, 66 years old , Progressive mobility difficulties with cognitive decline for 5 years.


Answer: Supranuclear palsy (PSP).

Diagnosis points: hummingbird sign, midbrain atrophy.

Progressive supranuclear palsy is Parkinson’s syndrome with postural instability, vertical supranuclear gaze palsy, pseudobulbar palsy, extrapyramidal symptoms and mild dementia as the main clinical features.

MR showed a hummingbird sign in the mid-sagittal position.

The atrophy of the midbrain tectum (involved in the vertical gaze center) resembles the beak of a hummingbird, and the pons are not often affected.

Case 2 is a 79-year-old female patient with numb limbs and unstable walking for 1 week.
She has a history of gastric CA and subtotal gastrectomy for 8 years.

Answer: Subacute combined degeneration (SCD).

SCD is a degenerative disease of the central and peripheral nervous system caused by obstacles in the intake, absorption, combination, transport or metabolism of vitamin B12, mainly involving the posterior cord, lateral cord and peripheral nerves of the spinal cord.

The most common causes include: pernicious anemia, Crohn's disease, long-term use of proton pump inhibitors, gastrointestinal surgery, atrophic gastritis, laughing gas abuse, malnutrition, etc.

Spinal cord MRI in patients with SCD showed symmetrical T2WI high signal on cervical and thoracic posterior cord or lateral cord.
The sagittal position showed abnormal signal in the vertical direction, and the axial position showed "anti-rabbit ear sign" or "inverted V sign.
"
 Case 3 is a middle-aged male patient with recurrent seizures and disturbance of consciousness for 20 years, and was admitted to the hospital for another 2 days.

Physical examination: response is slightly slower, memory is slightly decreased, and there are no obvious abnormalities.
MRI is as follows: Answer: Dyke-Davidoff-Masson Syndrome (DDMS, Da-Dai-Marco Syndrome) is a rare central nervous system disease and a common cause of cerebral
hemiplegia .
Typical clinical manifestations of DDMS include epilepsy, contralateral hemiplegia or hemiparesis, mental retardation, facial Asymmetry, a small number of patients may have facial capillary malformations.
The
etiology of DDMS can be divided into congenital and acquired.
Congenital usually has no obvious cause.
Symptoms appear gradually after birth.
The most likely cause is intrauterine or birth.
Various brain injuries at the time; acquired DDMS may be related to trauma, infection, ischemic or hemorrhagic state, subventricular germinal layer and intraventricular hemorrhage in preterm infants and other diseases that affect the normal development of the brain.

Typical imaging manifestations are unilateral brain Hemisphere atrophy, ipsilateral ventricle dilatation, large softening of some brain parenchyma, ipsilateral skull hyperplasia, sinus and mastoid sinus enlargement, etc.

 Case 4 male, 82 years old, with progressive cognitive dysfunction for 3 months .

A power seven, antinuclear antibody profile 17, tumor markers, blood, CRP, ESR, blood biochemistry, anemia three normal.

outgoing blood, cerebrospinal fluid from the brain Free antibody negative.

outgoing cerebrospinal fluid, blood paraneoplastic Antibody negative.

Cerebrospinal fluid: protein: 0.
8g/L, sugar, chlorine, and cell count are normal.

Ambulatory EEG: mild abnormality.

Hepatobiliary and urinary system color Doppler ultrasound is normal.

Chest CT scans the right upper lobe of the lung with tiny nodules.

Give strong thyroid after admission .
Long 1000 mgqd was halved for 3 days, and after 2 weeks of gradual application, the patient's cognitive impairment symptoms were significantly better than when he was admitted to the hospital.

Answer: Cerebral amyloid angiopathy-related inflammation (CAA-RI).

See the picture below for SWI.

Cerebral amyloid angiopathy-related inflammation (CAA-RI): is a special subtype of cerebral amyloid angiopathy (CAA), which is characterized by an autoimmune response to the deposition of β amyloid in cerebral blood vessels.

It is manifested as subacute cognitive function changes and vasogenic edema, rather than acute cerebral hemorrhage.

The pathology is characterized by inflammatory cell infiltration around the blood vessels where Aβ is deposited.

The main clinical manifestations of CAA-RI are cognitive decline, seizures, and headache.

Imaging usually shows features of CAA such as multiple lobular microhemorrhages, and patchy or confluent asymmetric white matter hyperintensity (WMHs) (indicating subcortical edema).

Gray matter is less involved, and the enhanced scan shows brain parenchyma and pia mater enhancement.

The disease responds well to immunosuppressive therapy including glucocorticoids.

Case 5, a 9-year-old boy with intermittent fever and headache was admitted to the hospital for 1 month.

The head MRI is as follows: Cerebrospinal fluid: white blood cells 338×10^6/L, mononuclear 71.
3%, multinucleus 28.
7%.

Cerebrospinal fluid biochemistry: glucose: 0.
56mmol/L, protein: 0.
69g/L; cerebrospinal fluid ink staining: Cryptococcus cerebrospinal fluid fungal culture indicates cryptococcal growth; fungal G test: 211.
4pg/ml.

In the past 2 months, I have raised pigeons at home and have been exposed to pigeons.

After half a month of initial treatment, symptoms such as fever and headache were all relieved, and the cerebrospinal fluid reexamination improved.
Later, the child recurred drowsiness, headache and fever.
The head MRI was reexamined with large white matter lesions.
The head MRI is as follows.

After hormonal therapy is started, the symptoms of the child are controlled.

Answer: Cryptococcal Encephalitis-Central Immune Reconstruction Inflammatory Syndrome.

The immune reconstitution inflammatory syndrome (iris) of the central nervous system is relatively rare, and it usually occurs after the treatment of conditional pathogenic bacteria, such as cryptococcus, toxoplasma, and cytomegalovirus infections.

The pathogenic process of pathogenic microorganisms is closely related to the body's immune response.

Invasive microorganisms stimulate the body to produce a sharp inflammatory response and quickly and effectively remove pathogens.
At this time, once the balance between inflammation and anti-inflammatory response is broken, the host will be damaged.

Once the body's immune reconstitution after antimicrobial therapy, especially after the rapid or sudden gain of immunity, it may have adverse consequences, and may even worsen the condition due to immune reconstitution.
This phenomenon is called IRIS.

 Case 6 is a male child who is 4 years old and 10 months old.
He has reduced active speech for 3 years and has had multiple seizures.

Answer: Gray matter heterotopia (GMH).

According to the occurrence location, it is divided into: subependymal type, focal subcutaneous type, diffuse type, cleft brain malformation type, and mixed type.

Main clinical features: seizures (most common), mental retardation, neurological dysfunction.

Typical imaging manifestations: One or more longer T1 and longer T2 signals appear around the lateral ventricle or in the subependymal area, similar to gray matter signals in other parts of the brain.

It can exist alone, or it can be combined with other malformations of the central system such as split brain malformations, corpus callosum dysplasia, giant gyrus malformations, multiple microgyrus malformations, transparent septal defects, etc.

Case 7 is a male patient, 24 years old, with repeated blurred vision for 9 months, accompanied by weakness of both lower limbs for 1 month.

Answer: GFAP astrocytosis.

Supplementary examination: GFAP: (+), AQP4, MOG antibody (-).

GFAP astrocyte disease usually presents acute or subacute onset and is more common in middle-aged and elderly people.
Common manifestations include headache, fever, disturbance of consciousness, seizures (mostly refractory), and psychiatric symptoms.

Head MRI manifests as common lesions distributed on both sides of the midline structure, usually with T1 low signal, T 2 /FLAIR high signal, and DWI is mostly normal; the characteristic imaging manifestations are line-like radial vascular enhancement perpendicular to the ventricle, from The GFAP-enriched lateral ventricle emits and passes through the white matter of the brain, and occasionally the cerebellum can be seen with similar enhancement; the spinal cord can be affected by long segments, similar to NMOSD.