Brian P. O'Neill

The injury severity score: a method for describing patients with multiple injuries and evaluating emergency care.

A method for comparing death rates of groups of injured persons was developed, using hospital and medical examiner data for more than two thousand persons. The first step was determination of the extent to which injury severity as rated by the Abbreviated Injury Scale correlates with patient survival. Substantial correlation was demonstrated. Controlling for severity of the primary injury made it possible to measure the effect on mortality of additional injuries. Injuries that in themselves would not normally be life-threatening were shown to have a marked effect on mortality when they occurred in combination with other injuries. An Injury Severity Score was developed that correlates well with survival and provides a numerical description of the overall severity of injury for patients with multiple trauma. Results of this investigation indicate that the Injury Severity Score represents an important step in solving the problem of summarizing injury severity, especially in patients with multiple trauma.

Adrenoleukodystrophy Increased plasma content of saturated very long chain fatty acids

With a new method we measured the saturated very long chain fatty acids in the plasma of adrenoleukodystrophy (ALD) hemizygotes, ALD heterozygotes, and controls. ALD hemizygotes showed increased levels of hexacosanoate (C26 fatty acid) which represented 0.081 2 0.0066% (SEM) of total Fatty acids, compared to 0.015 ± 0.0032% in the controls. C25, C24, and C23 fatty acids were also increased, but the C22 and C20 fatty acids were normal. C26 levels were also increased in most ALD heterozygotes, with a mean level 0.057 2 0.0063% of total fatty acids. The technique can be used for diagnosis and carrier identification, and in the evaluation of therapy.With a new method we measured the saturated very long chain fatty acids in the plasma of adrenoleukodystrophy (ALD) hemizygotes, ALD heterozygotes, and controls. ALD hemizygotes showed increased levels of hexacosanoate (C26 fatty acid) which represented 0.081 ± 0.0066% (SEM) of total fatty acids, compared to 0.015 ± 0.0032% in the controls. C25, C24, and C23 fatty acids were also increased, but the C22 and C20 fatty acids were normal. C26 levels were also increased in most ALD heterozygotes, with a mean level 0.057± 0.0063% of total fatty acids. The technique can be used for diagnosis and carrier identification, and in the evaluation of therapy.

Geographic Variations in Mortality from Motor Vehicle Crashes

Using a new technique to study the mortality associated with motor vehicle crashes, we calculated population-based death rates of occupants of motor vehicles during the period 1979 through 1981 and mapped them according to county for the 48 contiguous states of the United States. Mortality was highest in counties of low population density (r = 0.57; P less than 0.0001) and was also inversely correlated with per capita income (r = 0.23; P less than 0.0001). Death rates varied more than 100-fold; for example, Esmeralda County, Nevada, with 0.2 residents per square mile (2.6 km2), had a death rate of 558 per 100,000 population, as compared with Manhattan, New York, with 64,000 residents per square mile and a death rate of 2.5 per 100,000. Differences in road characteristics, travel speeds, seat-belt use, types of vehicles, and availability of emergency care may have been major contributors to these relations.

Automobile head restraints--frequency of neck injury claims in relation to the presence of head restraints.

THE STUDY SAMPLE CONSISTED OF 67,143 INSURANCE CLAIMS; THE MANUAL SEARCH OF THE FILES ASSOCIATED WITH THESE CLAIMS LED TO THE IDENTIFICATION OF 6,833 STRUCK CARS MEETING THE STUDY CRITERIA. FOR THESE CLAIMS THE FOLLOWING ITEMS OF DATA WERE RECORDED FOR EACH FORWARD CAR: MODEL YEAR, MANUFACTURER, SEX OF THE DRIVER, AND WHETHER ANY TYPE OF NECK OR BACK INJURY WAS CLAIMED. IT WAS DETERMINED THAT THERE WAS AN 18 PERCENT REDUCTION IN THE FREQUENCY OF CLAIMED NECK INJURIES TO DRIVERS (BOTH SEXES COMBINED) IN CARS WITH HEAD RESTRAINTS AS STANDARD EQUIPMENT WHEN COMPARED WITH THE FREQUENCY FOR DRIVERS IN CARS WITHOUT HEAD RESTRAINTS AS STANDARD EQUIPMENT. SIMILAR RESULTS BY SEX OF DRIVER ARE ALSO PRESENTED. AN ANALYSIS BY MANUFACTURER REVEALED DIFFERENCES SIMILAR TO THE OVERALL PATTERN, BUT THE NUMBERS OF OBSERVATIONS WHEN DIVIDED INTO THESE VARIOUS SUBGROUPS WERE GENERALLY TOO SMALL TO SUPPORT FIRM CONCLUSIONS. THE RESULTS OF A VISUAL SURVEY OF DRIVERS IN 4,983 MOVING DOMESTIC PASSENGER CARS WITH ADJUSTABLE HEAD RESTRAINTS IN THE LOS ANGELES AND WASHINGTON, D. C. METROPOLITAN AREAS INDICATED THAT IN THE LOS ANGELES AREA, 74 PERCENT OF THE MALE DRIVERS AND 57 PERCENT OF THE FEMALE DRIVERS HAD THE HEAD RESTRAINTS IMPROPERLY POSITIONED WHILE IN THE WASHINGTON, D. C. AREA 93 PERCENT OF THE MALES AND 80 PERCENT OF THE FEMALES HAD THE RESTRAINTS IMPROPERLY POSITIONED. THE STUDY IS CONCLUDED WITH A DISCUSSION OF PREVIOUS RESEARCH ON WHIPLASH INJURIES AND HEAD RESTRAINTS.

Gynecologic Cancer in Patients With Subacute Cerebellar Degeneration Predicted by Anti-Purkinje Cell Antibodies and Limited in Metastatic Volume

Between 1982 and 1989, 19 patients with gynecologic carcinoma, paraneoplastic cerebellar degeneration, and seropositivity for anti-Purkinje cell cytoplasmic antibodies were identified at our institution. Seven of the patients had no clinical, computed tomographic, or magnetic resonance imaging evidence of cancer but had undergone laparotomy solely because anti-Purkinje cell antibodies were found in their serum; all had high-grade adenocarcinoma. Cerebellar symptoms preceded or coincided with the initial cancer diagnosis in 15 patients and preceded the diagnosis of recurrent cancer in 4 patients. The cancers were 14 ovarian, 2 fallopian tube, 2 surface papillary, and 1 poorly differentiated metastatic adenocarcinoma in a periaortic lymph node. Two remarkable surgical observations in patients with high-grade ovarian and tubal cancers were the conspicuous lack of peritoneal implants and the small metastatic volume. A comparison of the 8 patients who had primary stage III cancer with 24 matched control patients without paraneoplastic cerebellar degeneration revealed no difference in primary tumor volume but a significantly smaller volume of metastatic tumor in the seropositive group (P = 0.05). Anti-Purkinje cell antibodies were not detected in 111 neurologically normal patients with advanced ovarian cancer. The small metastatic volume in the face of high-grade and advanced stage malignancy in seropositive patients with paraneoplastic cerebellar degeneration suggests that an immune response to the tumor (presumably cross-reactive with cerebellar cells) may impair the metastatic process. Earlier diagnosis and treatment of cancer, based on prompt serologic testing, may offer an improved neurologic and oncologic prognosis.

Paraneoplastic Cerebellar Degeneration: A Clinical Comparison of Patients With and Without Purkinje Cell Cytoplasmic Antibodies

In a review of 32 patients with paraneoplastic cerebellar degeneration (PCD), 16 (all of whom were women) had Purkinje cell cytoplasmic antibodies (PCAb) and 16 (8 women) did not. Most patients (15 of 16 seropositive and 12 of 16 seronegative patients) had active cancer at the time of neurologic diagnosis. Gynecologic or breast cancers were found in 14 of 16 seropositive and in 2 of 8 seronegative female patients; lung cancer was diagnosed in 7 of 16 seronegative patients but in no seropositive patient. In seropositive patients, the onset of the syndrome was more often abrupt and abnormalities of affect, mentation, ocular motility, and cerebrospinal fluid IgG index were more common than in seronegative patients. Additional paraneoplastic neurologic syndromes were present in five seronegative patients but in no seropositive patient. Clinical impairment was equivalent in both groups; approximately 75% of patients were confined to a wheelchair or bed at last follow-up. Four of 16 seropositive patients died (4 to 18 months after onset of PCD), and 7 of 16 seronegative patients died (7 to 120 months after onset of PCD). Thus, PCAb seem to be a marker for a clinical subset of female patients with gynecologic or breast cancer. The high frequency of other autoimmune paraneoplastic syndromes in patients with seronegative PCD suggests that PCD in both seropositive and seronegative patients may have a common pathogenic basis that involves an as yet unidentified antineuronal autoimmune mechanism.

Autoimmune paraneoplastic cerebellar degeneration: Ultrastructural localization of antibody-binding sites in Purkinje cells

Sera from three of four patients with paraneoplastic cerebellar degeneration (PCD) associated with gynecologic cancer had antibodies that stained the cytoplasm of Purkinje cells in a characteristic discrete and coarsely granular pattern. No such antibodies were found in PCD patients with small cell cancer of the lung, in patients with cerebellar degeneration without cancer, in nonneurologic patients with small cell carcinoma or gynecologic cancer, or in normal subjects. Immunoelectron microscopy revealed that the antibodies of PCD bound to clusters of ribosomes, granular endoplasmic reticulum, and the trans-face of the vesicles of the Golgi complex in Purkinje cells. Immunostaining was localized in orderly arrays of stacked parallel cisternae of the endoplasmic reticulum in the perikaryon and dendritic processes. This pattern suggested that at least one autoantigen of PCD may be a glycoprotein specific for cerebellar tissue that is associated with the endoplasmic reticulum. Some patches of Purkinje plasma membrane also were immunostained.

Intracranial Hypertension in Behçet's Disease: Demonstration of Sinus Occlusion With Use of Digital Subtraction Angiography

We describe two patients with Behçets disease who had symptomatic intracranial hypertension due to cerebral venous sinus thrombosis. The sinus thrombosis was demonstrated by digital subtraction angiography. In those patients with Behçets disease who have unexplained headaches, papilledema, and elevated cerebrospinal fluid pressure, venous digital subtraction angiography is an expedient, accurate, and safe procedure for demonstrating intracranial venous thrombosis.

Paraneoplastic Brachial Plexopathy in a Patient With Hodgkin's Disease

We describe a case of inflammatory brachial plexopathy that occurred in the context of a mild, diffuse sensorimotor peripheral neuropathy associated with Hodgkins disease. Clinical, electrophysiologic, and pathologic studies helped distinguish this disorder from other causes of brachial plexopathy in patients with cancer. Treatment with corticosteroids seemed beneficial in this patient. We suggest that this may be another type of paraneoplastic condition associated with Hodgkins disease.

Adrenoleukodystrophy Clinical and biochemical manifestations in carriers

We studied 21 women from four definite adrenoleukodystrophy(ALD) kinships. Nine women had a spastic paraparesis, including two with peripheral neuropathy. Fifteen women were assigned heterozygote status based on abnormal very-long-chain fatty acid (VLCFA) concentrations in plasma, fibroblasts, or both. These 15 included those with abnormal examinations and all women judged to be carriers by clinical criteria. Some ALD heterozygotes have varying degrees of neurologic disease, usually spastic paraparesis. Carrier detection is possible by determination of VLCFA concentrations in plasma and cultured skin fibroblasts. Any woman at risk in an ALD kinship, and who demonstrates a spastic paraparesis, should be assumed to be a carrier until biochemical testing is obtained. To determine whether a woman is an ALD carrier, measurement of plasma VLCFA levels should be done initially. If this gives normal or equivocal results, VLCFA levels should be measured in cultured skin fibroblasts.We studied 21 women from four definite adrenoleukodystrophy (ALD) kinships . Nine women had a spastic paraparesis, including two with peripheral neuropathy. Fifteen women were assigned heterozygote status based on abnormal very-long-chain fatty acid ( VLCFA ) concentrations in plasma, fibroblasts, or both. These 15 included those with abnormal examinations and all women judged to be carriers by clinical criteria. Some ALD heterozygotes have varying degrees of neurologic disease, usually spastic paraparesis. Carrier detection is possible by determination of VLCFA concentrations in plasma and cultured skin fibroblasts. Any woman at risk in an ALD kinship, and who demonstrates a spastic paraparesis, should be assumed to be a carrier until biochemical testing is obtained. To determine whether a woman is an ALD carrier, measurement of plasma VLCFA levels should be done initially. If this gives normal or equivocal results, VLCFA levels should be measured in cultured skin fibroblasts.