This invisible "vascular stenosis" can cause cerebral infarction

It is only for medical professionals to read for reference.
Multiple cerebral infarctions caused by small vessel disease.
When mentioning cerebral infarction, we always think of cerebral infarction caused by large vessel stenosis or thromboembolism first, but in fact, multiple cerebral infarction caused by small vessel disease It's not uncommon
.

In this issue of "Watershed Cerebral Infarction Caused by Invisible "Vessel Stenosis"," we invited Dr.
Ling Yifeng from the Department of Neurology of Shanghai Huashan Hospital to lead us to start with the case and understand the multiple cerebral infarctions caused by small vessel disease step by step
.

1.
Case introduction The patient, female, 61 years old, was admitted to the hospital for "repeated syncope with unresponsiveness for 13 days"
.

On June 21, 2018, when accompanied by family members in the hospital, he suddenly fainted without convulsions of the limbs.
After about 5 minutes, he recovered consciousness.
After waking up, he became slow in reaction, apathetic, and memory loss; on June 22, 2018, he fainted again, 2 minutes After recovery, he went to an outside hospital for treatment; June 23, 2018, head MRI showed: bilateral semi-oval center and lateral paraventricular DWI high signal, consider "multiple cerebral embolism"; treated with low molecular weight heparin and atorvastatin Later, he was discharged from the hospital on July 3
.

On the same day, the patient reappeared dizziness, weak limbs, and no loss of consciousness, so he went to our hospital for treatment and was admitted to the hospital for a clear diagnosis
.

The patient complained of low blood pressure for many years, ranging from 90 to 105/55 to 65 mmHg
.

He has a history of subacute thyroiditis, and denies diabetes or other family history
.

On admission to the hospital for physical examination: clear, indifferent expression, unresponsive to correct answers, decreased memory and calculation ability, muscle strength of limbs 5, bilateral sensation symmetry, no pathological signs elicited
.

Second, multiple intracranial infarction - hypoperfusion identify the cause: the main artery stenosis and hypovolemia, hypotension
.

Thromboembolism: Arterial to artery embolism, cardiogenic embolism (atrial fibrillation, valvular disease, patent foramen ovale, atrial myxoma, endocarditis, etc.
)
.

Coagulopathy: tumor, abnormal coagulation factor induced hypercoagulable state
.

Other: all kinds of infections, intravascular lymphoma
.

3.
Condition analysis 1.
Consider hypotension + large vessel stenosis? [Ambulatory blood pressure] 24h average blood pressure is 114/66mmHg, awake average 109/62mmHg, sleep average 129/81mmHg, 24h minimum blood pressure is 83/38mmHg
.

(The lowest blood pressure occurs during sleep at night, and no clinical symptoms are shown
.

) [Head and Neck CTA] There is no occlusion of large blood vessels
.

2.
Consider cardiogenic embolism? 【Holter Cardiogram】There is no obvious abnormal heart rhythm in 24 hours
.

[Foaming test] Negative, does not support the right-to-left shunt of the heart
.

[Esophageal echocardiography] There is no sign of patent foramen ovale
.

3.
Consider the hypercoagulable state? [Cagulation function] Protein S, protein C, antithrombin III antigen and antithrombin III activity, von Willebrand factor, VWF activity, coagulation factor VIII activity, etc.
are not abnormal
.

[Tumor Markers] Within the normal range
.

[Chest B-ultrasound, chest CT] No obvious abnormalities were found
.

4.
Consider small vessel disease? [Head MRI] DWI showed: bilateral hemisphere watershed acute infarction, FLAIR showed multiple white matter hyperintensity, involving bilateral temporal poles
.

[Gene test] NOTCH3 mutation
.

 4.
Bilateral temporal lobe white matter lesions-identification of etiology 1.
CADASIL: autosomal dominant cerebral artery disease with subcortical infarction and leukoencephalopathy; more common, early temporal pole involvement; NOTCH3 gene pathogenic mutation
.

2.
DM1: myotonic muscular dystrophy type 1; CTG repeat amplification>50 times in the DMPK gene is rare; muscular dystrophy manifestations
.

3.
CARASAL: cathepsin A-related arterial disease with stroke and leukoencephalopathy; CTSA gene pathogenic mutations; rare, with refractory hypertension
.

4.
Other: CARASIL, HDLS, ADL, HDLS other case reports
.

V.
Basic introduction to CADASIL 1.
Autosomal dominant cerebral arterial disease with subcortical infarction and leukoencephalopathy: the most common hereditary cerebral small vessel disease is caused by a pathogenic mutation in the NOTCH3 gene
.

2.
Clinical manifestations 3.
Typical imaging characteristics 4.
Pathological features The pathological accumulation of granular osmophilic substance (GOM) on the surface of degenerative vascular smooth muscle cells (SMC), and the increase in fibrosis of arterioles caused by the apoptosis of arteriole smooth muscle cells Thick and narrow
.

 5.
Literature review-clinical features ① The patient has no large arterial stenosis, which is mostly related to hypotension and decreased blood volume
.

②There is cerebral white matter hypoperfusion: MRI perfusion of a similar patient found bilateral deep white matter hypoperfusion: cerebral blood flow decreased, average transit time prolonged, and cerebral blood volume decreased
.

③Abnormal cerebral blood flow regulation: After transcranial Doppler ultrasound, compared with healthy controls, CADASIL patients have a lower increase in cerebral blood flow rate and delayed response time after visual stimulation
.

④ Abnormal blood pressure rhythm: The nocturnal blood pressure drop in CADASIL patients is not obvious, and the autoregulation of blood vessels is impaired
.

In this case, the patient had elevated blood pressure at night
.

⑤Higher systolic blood pressure may be related to new lacunas: higher baseline systolic blood pressure and number of lacunas are independent predictors of new lacunas after 3 years in CADASIL patients.
Patients with baseline systolic blood pressure higher than 140mmHg were excluded.
Still significant
.

New-onset lacunas are significantly related to cognitive decline
.

6.
Summary diagram: CADASIL flow chart.
Want to know how the experts comment on the case? What are the useful lessons learned? Click [Read the original text] or scan the QR code below to download the doctor station App, the famous doctor classroom allows you to watch the original text for free, and watch it now↓↓↓↓