Motivation: A cervical artery dissection is a frightening event. I met a middle age woman last year, who described sitting in a bench listening to a street musician playing guitar then feeling a twinge in her neck and then not being able to move her left side. She had a carotid dissection leading to a large stroke - chances are that she will never be able to move her left side. For other patients with stroke, we often feel that perhaps if the patient had not smoked and snacked on hamburgers, we could have prevented the stroke. With dissections, we do not know the cause most of the time. One of the more common maneuvers in rounds though is to see if the patient is flexible and can bend her fingers backwards without breaking - attempts to infer underlying connective tissue disorders. But, how commonly are connective tissue diseases associated with cervical artery dissection?
Paper: Brandt, T., Orberk, E., Weber, R. et. al. "Pathogenesis of cervical artery dissections: Association with connective tissue abnormlities." Neurology (2001); 57: 24
Methods: Prospective study of patients with non-traumatic spontaneous symptomatic cervical artery dissection. Dissections were confirmed by MRI of neck, CTA, and angiography. Patients also received thorough clinical evaluation and had skin biopsy. Ten controls with strokes from other mechanisms were also evaluated and biopsied.
Results:
Cohort: Total of 65 patients with dissection were recruited into the study - 36 with single vessel dissection (29 with internal carotid artery and 7 with vertebral arter), 22 with multi-vessel dissection, and 7 with recurrent dissection. Of the 65 patients, 52 presented with cerebral ischemia while the remaining had neck and cranial nerve palsies.
Family Inheritance: Six patients (9%) had first degree relative with cervical artery dissection.
Connective Tissue Disease: Three patients (5%) had systemic signs of connective tissue disorder such as hyperextensible skin, marfanoid appearance, and hypertrophic pseudo-molluscoid scars.
Vascular Disease: In total, six patients (9%) had other vascular anomalies: 2 with intracranial aneurysm, 3 with aortic dissection, and 1 with renal artery dissection.
Biopsy: On electron microscopy, ultrastructural aberrations of the connective tissue disease was found in 36 patients (55%). None of the controls had similar abnormalities. These abnormalities were typically in the collagen fibrils and elastic fibers.
Discussion: This exploratory study demonstrates, I think, that there probably exists new classes of connective tissue disorders affecting vascular structures that we do not at present know. Interestingly, only 5% had other clinical signs of connective tissue disease yet more than half had microscopic evidence. One of the other cautionary points from this paper is that a significant percentage (9%) had concurrent vascular anomalies such as dissection or aneurysms. When treating a patient with newly diagnosed dissection, we should probably be extra vigilant for other anomalies as well. This study, of course, suffers from the small number of controls, and it is unclear how many apparently normal people in a larger study would have microscopic abnormalities without every having any clinical symptoms.
Paper: Brandt, T., Orberk, E., Weber, R. et. al. "Pathogenesis of cervical artery dissections: Association with connective tissue abnormlities." Neurology (2001); 57: 24
Methods: Prospective study of patients with non-traumatic spontaneous symptomatic cervical artery dissection. Dissections were confirmed by MRI of neck, CTA, and angiography. Patients also received thorough clinical evaluation and had skin biopsy. Ten controls with strokes from other mechanisms were also evaluated and biopsied.
Results:
Cohort: Total of 65 patients with dissection were recruited into the study - 36 with single vessel dissection (29 with internal carotid artery and 7 with vertebral arter), 22 with multi-vessel dissection, and 7 with recurrent dissection. Of the 65 patients, 52 presented with cerebral ischemia while the remaining had neck and cranial nerve palsies.
Family Inheritance: Six patients (9%) had first degree relative with cervical artery dissection.
Connective Tissue Disease: Three patients (5%) had systemic signs of connective tissue disorder such as hyperextensible skin, marfanoid appearance, and hypertrophic pseudo-molluscoid scars.
Vascular Disease: In total, six patients (9%) had other vascular anomalies: 2 with intracranial aneurysm, 3 with aortic dissection, and 1 with renal artery dissection.
Biopsy: On electron microscopy, ultrastructural aberrations of the connective tissue disease was found in 36 patients (55%). None of the controls had similar abnormalities. These abnormalities were typically in the collagen fibrils and elastic fibers.
Discussion: This exploratory study demonstrates, I think, that there probably exists new classes of connective tissue disorders affecting vascular structures that we do not at present know. Interestingly, only 5% had other clinical signs of connective tissue disease yet more than half had microscopic evidence. One of the other cautionary points from this paper is that a significant percentage (9%) had concurrent vascular anomalies such as dissection or aneurysms. When treating a patient with newly diagnosed dissection, we should probably be extra vigilant for other anomalies as well. This study, of course, suffers from the small number of controls, and it is unclear how many apparently normal people in a larger study would have microscopic abnormalities without every having any clinical symptoms.
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