[Transforming growth factor beta in patients with cervical artery dissection]. / Transformiruyushchii faktor rosta beta u bol'nykh s dissektsiei vnutrennikh sonnykh i pozvonochnykh arterii.

OBJECTIVE: To evaluate transforming growth factor beta (TGF-ß) in patients with cervical artery dissection (CeAD). MATERIAL AND METHODS: TGF-ß was studied by enzyme immunoassay in 74 of 336 patients with CeAD observed at the Research Center of Neurology (Moscow) from 2000 to 2021. The average patient's age at the time of TGF-ß study was 41.6±9.8 years; the proportion of women was 51%. TGF-ß was studied in the first month of the disease (n=9), for 2-3 months (n=12) and at a later period (mean - 4.3±5.03 years) (n=53). The control group consisted of 20 healthy volunteers, matched for age and sex. Dissection occurred in internal carotid artery (ICA) (n=42), vertebral artery (VA) (n=29), ICA+VA (n=3) and involved 1 artery (n=58) or 2-3 arteries (n=16). Clinical manifestations included ischemic stroke (IS) (n=49), isolated cervical-cephalic headache (n=23), lower cranial nerve palsy (n=2). Pathological CeAD tortuosity was detected by angiography in 13 patients, and a dissecting aneurysm in 15 patients. RESULTS: TGF-ß1 and TGF-ß2 were elevated in patients with CeAD patients compared with the control: TGF-ß1 - 4990 [3950; 7900] pg/ml vs. 3645 [3230; 4250] pg/ml, p=0.001; TGF-ß2 - 6120 [4680; 7900] pg/ml vs. 3155 [2605; 4605] pg/ml, p=0.001. The highest TGF-ß1 and TGF-ß2 levels were noted at 2-3 months of the disease. There was no correlation between the TGF-ß level and various clinical and angiographic parameters. CONCLUSION: Increased TGF-ß level confirms that CeAD patients have connective tissue disorder that underlies the arterial wall weakness. A higher TGF-ß level at 2-3 months of CeAD seems to be connected with an active reparative process in arterial wall after dissection. TGF-ß can be used as a biomarker of connective tissue dysplasia in patients with CeAD.

[Cervical artery dissection in women: relationships with pregnancy and postpartum period]. / Dissektsiya vnutrennei sonnoi i pozvonochnoi arterii u zhenshchin: vzaimootnoshenie s beremennost'yu i poslerodovym periodom.

OBJECTIVE: To study the frequency of CeAD that developed during pregnancy or in post partum period among all CeADs in women; to study the course of pregnancy in women with prior CeAD. MATERIAL AND METHODS: 162 women (mean age 37.1±4.1 years) with CeAD we examined at the Research Center of Neurology (Moscow), 98% women were studied during last 15 years. 140 women were of childbearing age (≤45 years, mean age - 35±2.8 years). All patients were interviewed whether or not CeAD occurred during pregnancy or in post partum period (CeADPPP). Obstetric history before and after CeAD was studied in 57 women of childbearing age (average age - 35.9±7.3 years at CeAD development). RESULTS: CeADPPP developed in 6 out of 162 all female patients (3.7%) or of 140 childbearing age patients (4.3%). It occurred 2-6 months (4 patients) and 10 days after delivery (1 patient), or on the 25th week of pregnancy (1 patient). CeADPPP patients were younger than patients with CeAD out of pregnancy or postpartum period (29.8±8 years vs 35.1±6.7 years, p>0.05). CeADPPP in comparison with CeAD outside these periods more often involved internal carotid artery (ICA) (50% vs 35%, p=0.666), more often occurred in 2-3 arteries (50% vs 31%, p=0.386) and more often was accompanied by dissecting aneurysm development (50% vs 8%, p=0.013). After CeAD, 18 out of 57 patients in whom obstetric history was studied, including 3 patients with postpartum dissection had 29 pregnancies. The pregnancy outcomes were as follows: childbirth (17 pregnancies, 59%), fetal loss (8 pregnancies, 27%) and medical abortion (4 pregnancies, 14%). Delivery occurred on average 4.5±2.061 years after CeAD in women aged 33.0±4.25 years (cesarean section - 15 patients). Fetal loss occurred at 7.4±3.5 weeks of pregnancy in women aged 37.6±3.13 years on average 2.7±1.4 years after CeAD. Fetal loss frequency after CeAD was higher than before it (27% vs 7%, p=0.016). There were no CeAD recurrences during pregnancy and postpartum period in women who had previously undergone CeAD. CONCLUSION: CeADPPP frequency among all dissections in women is 3.7-4.3%. The risk of CeAD recurrence during pregnancy or the postpartum period after prior CeAD is very low. The risk of fetal loss during 2.7±1.4 years after CeAD is higher than before it (27% vs 7%). Hormonal and growth factors associated with pregnancy and the postpartum period is suggested to contribute to cervical artery wall damage. It is possible that the prolonged action of some of these factors may disrupt the placental vessels formation, predisposing to miscarriage.

[Internal carotid artery dissection in patients with Turner's syndrome]. / Dissektsiya vnutrennei sonnoi arterii u bol'nykh s sindromom Shereshevskogo­Ternera.

Cervical artery dissection (CeAD) due to arterial wall weakness (dysplasia) is one of the most common causes of ischemic stroke (IS) at a young age. A rare and little known cause of CeAD is Turner's syndrome (TS)-is an inherited disease caused by completely or partially missing X chromosome. In this paper, we describe 2 female patients, aged 27 and 33 years, with genetically confirmed TS (karyotype 45X0) and internal carotid artery dissection(ICAD).TS frequency among our 304 patients with CeAD was 0.07%. Both patients had short stature, received hormone replacement therapy from the age of 14 and had arterial hypertension. In addition, the first patient suffered from hypothyroidism, osteoporosis and survived a nephrectomy for hydronephrosis. ICAD in first patient manifested by IS. MRI of the neck arteries, MRA and CTA revealed intramural hematoma, hemodynamically significant stenosis, which regressed in 4 months. In the second patient, dissection was manifested by local symptoms (Horner's syndrome, cervicocephalic pain on the dissection side). MRA and CTA revealed a precranial dissected aneurysm of the left ICA (on the side of local symptoms), fusiform expansion of the right ICA, and pathological tortuosity of both ICA. The paper discusses the cause of vasculopathy in TS. It is assumed that connective tissue damage is associated with a deficiency of biglycan - extracellular matrix protein, which interacts with collagen and elastin to strengthen the arterial wall. Biglycan gene is linked to X-chromosome which is completely or partlially missing in TS resulting in a biglycan deficiency. The role of sex hormone deficiency as a cause of arterial wall weakness is unlikely.

[Aneurysms and pathological tortuosity of the internal carotid and vertebral arteries in patients with dissection of these vessels: a results of long-term study]. / Anevrizmy i patologicheskaya izvitost' vnutrennikh sonnykh i pozvonochnykh arterii u bol'nykh s dissektsiei etikh sosudov: rezul'taty dolgosrochnogo issledovaniya.

OBJECTIVE: To study the frequency, angiographic and clinical features of aneurysms and tortuosity (T) in patients with internal carotid artery (ICA) and vertebral artery (VA) dissection. MATERIAL AND METHODS: Three hundred and twenty-seven patients (average age - 37.8±9.1 years, women - 57%) with ICA/VA dissection verified by neuroimaging were studied. Repeated neuroimaging in 2.4±3.3 years was performed in 254 patients. In one case, tortuous ICA fragment resected at the surgery complicated by dissection was histologically studied. RESULTS: ICA/VA aneurysms were found in 46 (14%) patients. At repeated neuroimaging aneurysms did not change (38%), increased (11%) or decreased in size (8%), were not detected (38%) or were detected for the first time (5%). Patients with aneurysms compared with those without aneurysms more often had multiple dissections (44% vs. 20%, p=0.001) and T (35% vs. 13%, p=0.001), but less frequently the artery lumen occlusion in the acute period (15% vs. 40%, p=0.001). T was found in 53 (16%) patients. Patients with T compared with patients without T were older (40.6±8.1 vs. 37.3±9.3 years, p=0.039), more often had aneurysms (30% vs. 11%, p=0.001) and recanalization of occlusion observed in the acute period (89% vs. 54%, p=0.006). Dissection more often occurred in tortuous than in non-tortuous artery (79% vs 21%, p=0.001). During 4.8±3.6 years of follow-up, TIA developed inone patient (2%) with an aneurysm. Histological examination of tortuous ICA fragment, which also contained a small aneurysm, revealed dysplastic changes. CONCLUSION: The association between aneurism and T in patients with ICA/VA dissection suggests their common basis - the arterial wall weakness due to dysplasia. Age-related changes are also important for T development. T is a risk factor for ICA/VA dissection. Aneurysms formed after ICA/VA dissection have a benign course.