John W. Harbison

Smith‐Lemli‐Opitz Syndrome: Neuropathological and Ophthalmological Observations

The case of a three‐year‐old boy with the Smith‐Lemli‐Opitz syndrome is reported. In addition to the constellation of skeletal and genital anomalies classically described in this syndrome, this patient had spontaneous opsoclonus‐like eye movements, strabismus, lack of visual following responses and of opticokinetic reflexes. At autopsy the cerebellar vermis was found to be absent. There were retinal hemangiomas. Microscopical examinations showed loss of Purkinje cells and extensive neuronal degeneration within dentate nuclei, associated with patchy demyelination of cerebellar peduncles and central white matter. These findings may contribute to the explanation of the pathophysiology of opsoclonus and some of the neuro‐ophthalmological findings.

Papilledema after acute head injury.

Low grade papilledema after acute, severe head injury was identified in 15 (3.5%) of 426 patients. Papilledema was recognized immediately after head injury in 1 patient, during the 1st week in 10 patients, and in the 2nd week or after in 4 patients. Initial computed tomographic scans showed evidence of brain injury in 11 of these patients. The intracranial pressure (ICP) was monitored continuously for 3 or more days in 9 patients; it was mildly elevated (20 to 40 mm Hg) in 7 patients and moderately elevated (40 to 60 mm Hg) in 2 patients. Intracranial hypertension was controllable in each patient. A sudden, severe, but transient increase in ICP best explained the immediate development of papilledema and survival of 1 patient. Sustained but mild to moderately elevated ICP accounted for papilledema appearing in the 1st week. Papilledema in the 2nd week or after occurred from impaired cerebrospinal fluid absorption and consequent communicating hydrocephalus or delayed focal or diffuse cerebral swelling. A lesser degree of head injury in patients with posttraumatic papilledema was suggested by a higher Glasgow coma score, milder and controllable elevations in ICP, and the absence of any fatality in this group. The favorable outcome was significant compared to the mortality of the more severely injured patients (chi square-4.327; P less than 0.04). Papilledema did not occur in 6 patients with sustained, severely elevated ICP (greater than 60 mm Hg) for 3 or more days. Each of these patients died. The severity of the trauma apparently accounts for the failure of papilledema to develop, possibly by arresting axoplasmic production and transport in retinal nerve fibers.

Ticlopidine versus aspirin for the prevention of recurrent stroke. Analysis of patients with minor stroke from the Ticlopidine Aspirin Stroke Study.

Background and Purpose: Ticlopidine has not been formally compared with aspirin in patients with a completed stroke. We therefore performed an analysis on a subgroup of patients from the Ticlopidine Aspirin Stroke Study (TASS) with a recent minor completed stroke as the qualifying ischemic event. Methods: This was a multicenter, double-blind, randomized trial of patients with a recent history of cerebral ischemia. Eligible patients had a qualifying minor stroke within 3 months of study entry. All patients received either 650 mg aspirin twice daily or 250 mg ticlopidine twice daily for up to 5.8 years. The primary study end point was the first occurrence of nonfatal stroke or death from any cause. A secondary end point was the first occurrence of a fatal or nonfatal stroke. Results: Minor stroke was the qualifying ischemic event in 927 patients (463 received ticlopidine and 464 received aspirin). The cumulative event rate at 1 year for nonfatal stroke or death was 63% for patients receiving ticlopidine and 10.8% for patients receiving aspirin, a 42% risk reduction in favor of ticlopidine. For fatal or nonfatal stroke, the cumulative event rate at 1 year was 4.8% for patients receiving ticlopidine and 7.5% for those receiving aspirin, a risk reduction of 36% for ticlopidine relative to aspirin. The overall risk reductions were 22.1% for nonfatal stroke or death and 19.9% for fatal or nonfatal stroke. Adverse reactions were reported in 58% of the ticlopidine patients and 51% of the aspirin patients. Conclusions: The results in this subgroup are consistent with the overall TASS results and show that ticlopidine is somewhat more effective than aspirin for reducing the risk of stroke in patients with a completed minor stroke.

Perceptions of housestaff stress and dysfunction within the academic medical center

Stress, emotional dysfunction, and work impairment are seen as accelerating phenomena in residency training, and have received increased attention in the medical literature. The authors review relevant literature in this area, and note continued deficiencies in programs for prevention and intervention. The present study focuses on the perceptions of key members of the academic hierarchy regarding housestaff stress and impairment. Chairmen, program directors, and chief residents in all specialties were asked to estimate the prevalence of several impairment syndromes, to describe any existing policies or programs to assist residents, and to express their opinions about developing such intervention strategies. The results are compared by respondent group, by resident postgraduate year, and by groupings of surgical and non-surgical programs. Suggestions for further research in this area are then offered.

Clinical Significance of Transient Visual Phenomena in the Elderly

To evaluate the significance of transient visual phenomena in the elderly patient, a retrospective study of 43 patients over 40 years of age (mean, 58) presenting between 1971 and 1982 was conducted. Historical, clinical and diagnostic features were collated and analyzed by computer. The study revealed that 31 patients were diagnosed as migraine and 12 as vertebrobasilar insufficiency based on features identified in this review. Follow-up was obtained in 90% of the patients with a mean follow-up of 2.4 years. In general, prognosis proved to be excellent, with two deaths of cardiac origin and an 81% incidence of remission or symptomatic improvement of the visual events. No retinal or cerebral strokes were observed and TIAs occurred in only 9% of the population. This study suggests that transient visual phenomena in the elderly are benign.

Unilateral proptosis secondary to a retroorbital tumor: fibrous histiocytoma or xanthogranulomatous lesion.

Abstract A patient was first examined because of unilateral decreased vision, proptosis, and generalized headaches secondary to a retro-orbital tumor. An unusual orbital tumor with intracranial in-volvement was found. The pathological diagnosis was fibrous histiocytoma or xanthogranulomatous lesion.

Prevention of Cerebrovascular Ischemic Events with Antiplatelet Drugs: Who Benefits Most?

Antiplatelet agents have been studied extensively in clinical stroke prevention trials for 25 years. Only two have proven effective, aspirin and ticlopidine. Current data suggest that antiplatelets ha