Cerebral vascular syndrome of connective tissue dysplasia as a cause of subarachnoid hemorrhage in young patients

Druk I.V.; Rozhdestvenskiĭ A.S.; Smyalovsky D.V.; Smialovskiĭ V.; Galtsova L.G.; Loginova E.N.; Korsakov M.V.

doi:https://doi.org/10.17116/jnevro201811812215

Druk I.V.

Omsk State Medical University, Omsk, Russia

Rozhdestvenskiĭ A.S.

Kafedra nevrologii i neĭrokhirurgii s kursom meditsinskoĭ genetiki Omskoĭ gosudarstvennoĭ meditsinskoĭ akademii

Smyalovsky D.V.

Dmitrovskiy City Hospital, Dmitrov, Russia

Smialovskiĭ V.

Kafedra nevrologii poslediplomnogo obrazovaniia Omskoĭ gosudarstvennoĭ meditsinskoĭ akademii, Omsk

Galtsova L.G.

Clinical Diagnostic Center, Omsk, Russia

Loginova E.N.

Omsk State Medical University, Omsk, Russia

Korsakov M.V.

Regional Clinical Hospital, Omsk, Russia

Cerebral vascular syndrome of connective tissue dysplasia as a cause of subarachnoid hemorrhage in young patients

Authors:

Druk I.V., Rozhdestvenskiĭ A.S., Smyalovsky D.V., Smialovskiĭ V., Galtsova L.G., Loginova E.N., Korsakov M.V.

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Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2018;118(12‑2): 15‑22

DOI: 10.17116/jnevro201811812215

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To cite this article:

Druk IV, Rozhdestvenskiĭ AS, Smyalovsky DV, Smialovskiĭ V, Galtsova LG, Loginova EN, Korsakov MV. Cerebral vascular syndrome of connective tissue dysplasia as a cause of subarachnoid hemorrhage in young patients. S.S. Korsakov Journal of Neurology and Psychiatry. 2018;118(12‑2):15‑22. (In Russ.)
https://doi.org/10.17116/jnevro201811812215

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова. Спецвыпуски (S.S. Korsakov Journal of Neurology and Psychiatry (special issues))

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Abstract:

Objective. To clarify the role of connective tissue dysplasia (CTD) in the development and course of intracranial arterial aneurysm (IAA) and arteriovenous malformation (AVM) in young patients. Material and methods. The first stage of the study was a prospective 7-year follow-up of 549 patients with CDT signs, aged from 18 to 45 years, mean 23.51±8.67 years. The first stage included a comparative analysis of clinical characteristics of patients with DST with asymptomatic pathology of cerebral vessels and patients with DST without this pathology. At the second stage, there was a comparative study in 2 groups of patients with symptomatic pathology of cerebral vessels (AAA and/or AVM): with CTD (n=58) and without CTD (n=135). Results. Symptomatic AAA and/or AVM were identified in 10.56% of young people with CTD including 37 patients with subarachnoid hemorrhages (SAH). The age of clinical manifestations was 25.55±8.13 years. Expressed manifestations of CTD were more frequent in the group of patients with cerebral vascular pathology compared to patients without CTD (p=0.008). The majority of patients had CTD manifestations of 3 or more systems, less than 2 systems were not involved, 12 patients had small abnormalities and/or malformations of the heart and other vessels. Hypertension, pathology of the vertebral arteries, skin, spine, veins, fully open Willis circle were independent risk factors for symptomatic pathology of cerebral vessels in patients with CTD. AVM and AAA in young patients with CTD compared with patients without CTD were characterized by the manifestation at an earlier age (25.55±8.13 years and 36.10±10.58 years, respectively; p=0.000), a more frequent combination with a fully open Willis circle (56.90 and 25.19%, respectively; df=1; p=0.000), more frequent multiple pathologies (15.52 and 4.44%, respectively, p=0.008), EAA in the posterior vascular bed (15.52 and 3.70%, respectively, p=0.004), SAH (63.79 and 35.56%, respectively, p=0.000) with lesser effects of general population risk factors. Conclusion. EAA and AVM in patients with CTD are likely to be considered in the context of vascular syndrome of connective tissue dysmorphogenesis, and CTD as a factor of adverse prognosis of IAA and AVM.

Keywords:

subarachnoid bleeding

arteriovenous malformation

intracranial arterial aneurysm

connective tissue dysplasia

Authors:

Druk I.V.

Omsk State Medical University, Omsk, Russia

Rozhdestvenskiĭ A.S.

Kafedra nevrologii i neĭrokhirurgii s kursom meditsinskoĭ genetiki Omskoĭ gosudarstvennoĭ meditsinskoĭ akademii

Smyalovsky D.V.

Dmitrovskiy City Hospital, Dmitrov, Russia

Smialovskiĭ V.

Kafedra nevrologii poslediplomnogo obrazovaniia Omskoĭ gosudarstvennoĭ meditsinskoĭ akademii, Omsk

Galtsova L.G.

Clinical Diagnostic Center, Omsk, Russia

Loginova E.N.

Omsk State Medical University, Omsk, Russia

Korsakov M.V.

Regional Clinical Hospital, Omsk, Russia

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