Abstract

Progressive Migraine in Patients with Ehlers-Danlos Syndrome (Hypermobility Type)

Author(s): Ali Al Kaissi, Mohammad Shboul, Lamia Ben Jemaa, Franz Grill, Suzan Salih-Rasool, Susanne Gerit Kircher

Objective: Frequent and intolerable bouts of migraine originating from the neck and radiated to the head creating a constellation of symptomcomplex. Ranging from vision problems and cognitive impairment to episodes of syncope and possibly stroke.
Materials and Methods: Three female patients (aged 20, 25, and 33 years old, respectively). Tall stature (above 97 th percentile), long and thin limbs overwhelmed through generalized ligamentous hyperlaxity, were the paramount clinical features. A history of frequent migraine attacks and early onset joint pain since late childhood, were the reasons behind frequent hospitalization. Clinical and radiological phenotypic characterization have been organized as well as tomographic studies to further understand the etiology behind.
Results: All patients manifested the clinical phenotype compatible with the diagnosis of Ehlers- Danlos syndrome –hypermobile type (EDS-ht). Molecular genetics showed no COL3A1 mutation, though family gathering information was strongly suggestive of (EDS-ht). We encountered variable cranial, vertebral and vascular abnormalities. The first patient´s 3D reformatted CT scan of the cranio-cervical junction showed sub-clinical basilar invagination (bulging of the odontoid into the brain stem of 17-mm above Chamberlain´s line). The contrast- enhanced computed tomography angiography of the cervical and cerebral arteries for the second patient, revealed anomalies of cervical vasculature (kinking/tortuosity and stenosis in several segments of the right and left common carotid arteries associated with stenosis in the lower third of the left common carotid artery and the left subclavian artery). Lateral skull radiograph of the third patient showed elements of osteoarthritis and derangement of the temporomandibular joint (TMJ). We confirmed our findings via 3D reformatted CT scan, which showed narrowing of the joint space in connection with dysplastic disc of the (TMJ).
Conclusion: Patients with a complex multisystem disorder, serious situations are to be expected. It is mandatory to determine the etiology. Pitfalls and speculations in diagnosis are extremely hazardous element, though they are commonly practiced through many medical disciplines. The profound clinical analysis and family history are fundamental tools tin solving the complicated clinical status of chronic illnesses. Congenital vascular and bony abnormalities may cause progressive headaches and might lead to severe neurological deficits. PDF