Clinical and angiographic features and outcome in 25 patients with spontaneous dissections of the vertebral arteries are described. Most patients were in their fourth or fifth decade of life, and women predominated. Forty-eight percent of the patients were hypertensive. Angiographic evidence of fibromuscular dysplasia was noted in one only. Brainstem ischemic symptoms (usually a lateral medullary syndrome) and ipsilateral occipital headache and neck pain (often preceding but sometimes associated with or following the brainstem ischemic event) were the most common clinical findings. The angiographic features in decreasing order of frequency were luminal stenosis (often irregular and tapered), aneurysm, occlusion, and intimal flap. On follow-up, most of the patients (88%) made complete or very good recoveries. Angiographic abnormalities either subsided or improved in 76%. Multivessel dissection (involvement of both vertebral arteries or one or both vertebral arteries and one or both internal carotid arteries) was noted in about two-thirds of the patients. This tendency of vertebral artery dissections to involve multiple cervicocephalic vessels concurrently, if not simultaneously, implies that four-vessel angiography should be attempted if a vertebral artery dissection is visualized. It also raises the possibility of an underlying arteriopathy that predisposes the vessel to dissection.

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily the renal and internal carotid arteries and less often the vertebral, iliac, subclavian, and visceral arteries. Although its pathogenesis is not completely understood, humoral, mechanical, and genetic factors as well as mural ischemia may play a role. The natural history is relatively benign, with progression occurring in only a minority of the patients. Typical clinical manifestations are renovascular hypertension, stroke, subarachnoid hemorrhage, abdominal angina, or claudication of the legs or arms. In patients with symptoms, percutaneous transluminal angioplasty has emerged as the treatment of choice in most involved vascular beds.

The relationship between the number of cortical tubers observed by magnetic resonance imaging (MRI) and the severity of cerebral dysfunction of tuberous sclerosis patients has been examined in a meta-analysis of the published literature. The literature review has identified five independent studies for examining the association. These studies consistently reveal that the cortical tuber count detected on MRI scans is increased among those with more severe cerebral disease. Severity of the cerebral dysfunction is measured by the seizure status and its control and by the developmental status and the level of mental retardation. Meta-analysis demonstrates that within a study population, the MRI-detected cortical tuber count is six times more likely to be above the median count for tuberous sclerosis patients with severe cerebral dysfunction (poor seizure control or moderate-severe retardation or both) than more mildly affected tuberous sclerosis patients. Similarly, across studies, moderately to severely affected patients are five times more likely to have greater than seven MRI-detected cortical tubers than those more mildly affected. These associations are both statistically significant and strong. The cortical tuber count is a biomarker that reasonably predicts the severity of cerebral dysfunction of tuberous sclerosis. Cortical tubers of tuberous sclerosis form in the early gestational period. The embryologic disruption determining the clinical severity of the cortical dysfunction of tuberous sclerosis is set in the early gestational period.

Traumatic dissections of the extracranial internal carotid artery (ICA) in 18 patients aged 19 to 55 years were studied. All had suffered blunt head or neck injury of marked or moderate severity; motor-vehicle accidents were the leading cause of the injury. Delayed focal cerebral ischemic symptoms were the most common presenting symptoms. Less commonly noted was focal unilateral headache associated with oculosympathetic paresis or bruit. Following a head injury, the abrupt onset of focal cerebral symptoms after a lucid interval should raise the suspicion of arterial injury, particularly when computerized tomography fails to show abnormalities that would explain the evolving neurological deficits on the basis of direct trauma to the brain. Unilateral headaches, oculosympathetic palsy, and bruits also help in establishing the diagnosis. Focal cerebral ischemic symptoms may develop months or years after the initial trauma. These delayed symptoms are caused by embolization from a thrombus within a residual dissecting aneurysm. Common angiographic findings, in decreasing order of frequency, are: aneurysm, stenosis of the lumen, occlusion, intimal flap, distal branch occlusion (embolization), and slow ICA-to-middle cerebral artery flow. Although two patients died as the result of massive cerebral infarction and edema and some were left with severe neurological deficits, most made a good recovery. Residual dissecting aneurysms and occlusion seem to occur more frequently with traumatic dissections than with spontaneous dissections of the extracranial ICA.

The records of five patients with primary melanoma of the spinal cord were reviewed. The tumor most frequently presented as an intramedullary middle or lower thoracic cord lesion. The average duration of symptoms before pathological diagnosis was 29 months, and the average survival after laminectomy and radiation therapy was 6 years 7 months. The findings in this series, when compared with those in the literature, suggest that primary spinal melanoma is a more indolent malignancy than previously reported or than melanoma metastatic to the central nervous system.