Gregory K. Call

Vasoconstriction is not recognized as a cause of cerebrovascular disease except in the vasospasm seen following subarachnoid hemorrhage and possibly in migraine. However, we found four patients to have transient, fully reversible vasoconstriction and dilatation prominently involving arteries around the circle of Willis. All four patients were evaluated for severe headaches and fluctuating or recurring motor or sensory deficits. No cause for the clinical syndromes and angiographic abnormalities was found. Similar patients are reported in the literature under various nosologies. This newly recognized clinical-angiographic syndrome should be differentiated from other known causes of vessel constriction and dilatation; the precipitants of reversible vasoconstriction may then be better defined.

Duplex ultrasound (US) scans of 110 carotid arteries ipsilateral to hemispheric strokes were compared with scans of 90 asymptomatic vessels in the same patients to determine the relative prevalence of stenotic lesions. In addition, scans of paired carotid arteries in patients with stroke involving only one cerebral hemisphere were compared to determine whether the incriminated side demonstrated a greater degree of stenosis than the asymptomatic side. The duplex US findings demonstrated a positive correlation between stenosis and hemispheric stroke. However, only 20% of carotid arteries ipsilateral to hemispheric stroke showed a reduction in diameter greater than 70%, compared with 5% of asymptomatic vessels. A minimal difference was demonstrated between the symptomatic and asymptomatic groups with respect to lesser degrees of stenosis. In paired carotid arteries, the degree of stenosis of the symptomatic vessel exceeded that of the asymptomatic vessel in only 43% of cases. These results suggest that the prevalence of severe carotid stenosis in stroke patients has been previously overestimated. The findings also emphasize the need for further investigation of other plaque-related risk factors that may enhance stroke prevention through improved selection of surgical or medical therapy. Factors currently under investigation include plaque ulceration, intraplaque hemorrhage, plaque echogenicity, and the effects of sequential stenoses.

Neurologic disorders are uncommon but alarming complications of cardiac transplantation. Of 29 patients from the Utah Cardiac Transplant Program (UCTP) who had lumbar puncture because of change in neurologic function, or to assess fever of uncertain etiology, CSF pleocytosis was present in 14 patients, 4 of whom had an active infectious process involving the nervous system. In 10 other patients, CSF pleocytosis with negative cultures appeared following treatment with OKT3 monoclonal antibody. The most prominent clinical signs of this aseptic meningitis syndrome are fever and transient cognitive dysfunction.

Carbamyl phosphate synthetase-I (CPS-I) catalyzes the first reaction required for the conversion of ammonia to urea through the urea cycle. Severe CPS-I deficiency causes marked hyperammonemia with encephalopathy in infancy and usually results in death within the first few months of life. We describe a 33-year-old woman whose CPS-I activity is less than 5% of normal. She has had mild, intermittent symptoms throughout life but has never experienced severe encephalopathy. Although mildly retarded, she has no major neurological deficits. Therapy with a low-protein diet, lactulose, and sodium benzoate has prevented recurrence of hyperammonemia and symptoms. Cranial computed tomographic scans demonstrate prominent lucency of cerebral white matter, and cerebral evoked potential recordings indicate slowed central conduction. These findings suggest that the metabolic disturbances in this patient may have adversely affected central myelin formation or maintenance. This woman represents, to our knowledge, the oldest reported patient with CPS-I deficiency, and the case illustrates the need to consider urea cycle disorders in the differential diagnosis of intermittent neurological symptoms regardless of the patient's age.