The Association among Polycystic Kidney Disease, Brain Aneurysms and Cerebral Cavernous Malformations

Caucasian familial moyamoya syndrome with rare multisystemic malformations.

Moyamoya disease is an idiopathic progressive steno-occlusive disorder of the intracranial arteries located at the base of the brain. It is associated with the development of compensatory extensive network of fine collaterals. Moyamoya disease is considered syndromic when certain genetic or acquired disorders such as polycystic kidney disease, neurofibromatosis, or meningitis are also present. ...

Neurovascular manifestations of heritable connective tissue disorders. A review.

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 m...

Skeletal malformations and polycystic kidney disease.

Increased number of white matter lesions in patients with familial cerebral cavernous malformations.

BACKGROUND AND PURPOSE Familial cerebral cavernous malformations, an autosomal dominant disorder, result in excess morbidity and mortality in affected patients. The disorder is most prevalent in the Southwest United States, where the affected families are most often carriers of the CCM1-KRIT1 Common Hispanic Mutation. The brain and spinal cord parenchyma in these individuals is usually affected...

Coincident Giant Cavernous Angioma and Large Middle Cerebral Artery Aneurysm

Cavernous angiomas although relatively common lesions rarely reach a large size. They have a well documented association with AVMs, capillary telangiectases and venous angiomas but are not specifically associated with intracerebral aneurysms. We present a case of what we believe to be the 4th largest reported giant cavernous angioma to present in adulthood. This cavernous angioma also happened ...