Stroke, Vol 25, 889-903, Copyright © 1994 by American Heart Association
WI Schievink, VV Michels and DG Piepgras
BACKGROUND: Heritable disorders of connective tissue are recognized in a
small minority of patients with neurovascular diseases. In this report, we
review the neurovascular manifestations of four heritable connective tissue
disorders: Ehlers-Danlos syndrome, Marfan's syndrome, osteogenesis
imperfecta, and pseudoxanthoma elasticum, as well as two other systemic
disorders with potential vascular manifestations: neurofibromatosis and
polycystic kidney disease. SUMMARY OF REVIEW: Typical neurovascular
complications of Ehlers-Danlos syndrome are carotid-cavernous fistulae,
intracranial aneurysms, and cervical artery dissections. Arterial
dissections and intracranial aneurysms cause the majority of neurovascular
symptoms in Marfan's syndrome. Neurovascular disease is uncommon in
osteogenesis imperfecta, although carotid- cavernous fistulae and vertebral
artery dissections have been reported. Neurovascular disease in
pseudoxanthoma elasticum is characterized by intracranial aneurysms and
cerebral ischemia caused by premature arterial occlusive disease.
Intracranial occlusive arterial disease is the most common neurovascular
manifestation of neurofibromatosis, followed by cervical arteriovenous
fistulae and aneurysms and intracranial aneurysms. Intracranial aneurysms
are the hallmark of polycystic kidney disease. CONCLUSIONS: Recognition of
an underlying generalized connective tissue disorder may be of considerable
importance, although marked phenotypic heterogeneity often complicates the
diagnosis of these disorders. Conversely, the association of certain
neurovascular anomalies with generalized connective tissue disorders and
recognition of their basic molecular defect may offer clues to the etiology
and pathogenesis of these neurovascular diseases in general.
ARTICLES
Neurovascular manifestations of heritable connective tissue disorders. A review
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minn. 55905.
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