Diagnostics and tactics of management of patients with signs of connective tissue dysplasia, headache and disorder of sensitivity. Literature review


DOI: https://dx.doi.org/10.18565/therapy.2020.6.124-130

Ivanova D.S., Viktorova I.A., Kochimov R.S, Adyrbayev A.M.

1) Omsk State Medical University of the Ministry of Healthcare of Russia; 2) Clinical medical and Surgical Center of the Ministry of Healthcare of the Omsk Region, Omsk
The purpose of current review is to identify diagnostic criteria that explain the causes of headache complaints, neurological deficits and impaired sensitivity of unknown origin in patients with connective tissue dysplasia (CTD) for a diagnostic search in the right direction and adequate patient care. Headache of unknown genesis in combination with neurological symptoms is a frequent reason for seeking medical help in patients of working age. At the same time, undetected signs of CTD in this category of patients can cause late diagnosis and further inadequate care. One of the backgrounds for this symptom-complex is hypermobile type of Ehlers – Danlos syndrome associated with Chiari malformation of type 0 or 1. Its typical clinical signs include excessive joint mobility, arthralgia, dorsalgia, hyper-extensibility of skin, dislocations or subluxations of joints, headache, fatigue, dizziness, myalgia, weakness and numbness in the limbs, sensory disturbances like a hood or half-jacket, problems with balance, memory and sleep. With the help of magnetic resonance imaging (MRI), a displacement of the metencephalon structures into the foramen magnum with frequent formation of hernial medullary longitudinal cavities manifested by syringomyelia is detected. Diagnosis is carried out by collecting of special complaints, also by means of physical methods and MRI. The curation tactics is expectant, conservative, with the help of regime measures, patient’s informing and symptomatic treatment. In some cases, manual therapy could be used. In severe progressive course, the defect is surgically corrected. Understanding the correlation between hypermobility type of Ehlers–Danlos syndrome, Chiari malformation and syringomyelia will lead to better diagnostics performance and adequate care for patients with CTD and headache.
Keywords: Chiari malformation, Ehlers–Danlos syndrome, joint hypermobility, connective tissue dysplasia, headache
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About the Autors

Daria S. Ivanova, PhD, associate professor of the Department of polyclinic therapy and internal diseases of Omsk State Medical University of the Ministry of Healthcare of Russia. Address: 644099, Omsk, 12 Lenin Str. Tel.: +7 (913) 962-83-86. E-mail: darja.ordinator@mail.ru. ORCID: 0000-0002-4145-7969
Inna A. Viktorova, MD, professor, head of the Department of outpatient therapy and internal diseases of Omsk State Medical University of the Ministry of Healthcare of Russia. Address: 644099, Omsk, 12 Lenin Str. Tel.: +7 (3812) 95-72-77, +7 (906) 990-09-07. E-mail: vic-inna@mail.ru. ORCID: 0000-0001-8728-2722
Roman S. Kochimov, 5th year student of the medical faculty of Omsk State Medical University of the Ministry of Healthcare of Russia. Address: 644099, Omsk, 12 Lenin Str. Tel.: +7 (960) 984-34-67.
Albert M. Adyrbaev, PhD, head of the day hospital of Clinical Medical and Surgical Center of the Ministry of Healthcare of the Omsk region (polyclinic, day hospital). Address: 644007, Omsk, 105 Bulatov Str. Tel.: +7 (3812) 95-72-77.
E-mail: ovpomsk@mail.ru


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