Milton Alter

Creutzfeldt‐Jakob disease Possible medical risk factors

To explore possible risk factors in the past medical history of patients with Creutzfeldt-Jakob disease (CJD), we conducted a case-control study among 26 cases and 40 matched controls. Statistically significant odds ratios were obtained for intraocular pressure testing; injury to or surgery on the head, face, or neck; and trauma to other parts of the body. The odds ratios were nearly significant for head trauma and procedures requiring sutures. These data suggest that the CJD agent may be acquired by inoculation through injury or during surgery, and perhaps on certain absorbable sutures of animal origin. The tonometer used for glaucoma testing may also be a vehicle of transmission.

Stroke in the Lehigh Valley: seasonal variation in incidence rates.

We investigated the seasonal pattern of stroke using the Lehigh Valley Stroke Register. This register includes all patients hospitalized with stroke or transient ischemic attack (TIA) from among the 600,000 Lehigh Valley residents. Meterological data were obtained from the National Oceanic and Atmospheric Administration. The study, which uses 18 months of data, included 1,944 cases. Using single harmonic regression analysis, the seasonal pattern of TIA and infarction, but not hemorrhage, fit a sine-cosine wavefunction with a 12-month period (R2 = 41% and 36%, respectively). For infarction, the strongest seasonal pattern was exhibited for women of all ages and for both sexes in the age groups 65-74 and 75-84, but only the sine component was significant. The peak months for TIA were June-August, while the peak months for infarcts were February-April. Correlations between ambient temperature and each type of stroke were computed. A significant positive correlation for TIA was found (r = 0.57, p = 0.01). After adjusting for a 2-month lag between the low for infarction and the peak for temperature, a significant negative correlation was found (r = -0.64, p = 0.01). No significant correlation was found for hemorrhage. Possible reasons for the opposite relations of TIA and infarct are discussed.

Stroke in the Lehigh Valley Racial/ethnic differences

We investigated black/white differences in stroke rate (standardized morbidity), severity, and subtype, and the relative frequencies of 5 primary risk factors (hypertension, diabetes, myocardial infarction, other heart diseases, and transient ischemic attack [TIA]) using the Lehigh Valley Stroke Register. Blacks had a statistically significant higher, age-adjusted rate of stroke than whites. We found no differences in stroke severity using our measures but blacks had a statistically higher proportion of lacunar stroke, while whites had a higher proportion of embolic stroke. There were no differences in proportions of thrombotic stroke or intracerebral hemorrhage. The relative frequencies of hypertension, myocardial infarction, other heart diseases, and diabetes were higher for blacks, while the relative frequency of TIA was higher for whites. These observations are consistent with other reports that blacks have a higher frequency of stroke and tend to have more small-vessel cerebrovascular pathology than whites.

Multiple sclerosis and childhood infections

Migration from an area where MS is common to an area where it is rare (and vice versa) affects the risk of MS, provided migration occurs in childhood. A childhood infection might explain this effect. Therefore, the age pattern of infectious diseases in different regions was examined. A higher proportion of children showed positive titers to many viral diseases early in life in areas where MS is rare compared with those where MS is common. Also, mortality rates from a variety of infectious diseases correlated negatively with the MS mortality. Thus, infection early in life may “protect” against MS, and conversely, later infection, when the immune system has partially matured, may increase risk. MS may be an age-dependent, host-immune response to childhood infection.

Stroke in the Lehigh Valley Risk factors for recurrent stroke

Age-specific risk of recurrent stroke for various risk factors, calculated independently, was estimated using the first year of data from the Lehigh Valley Stroke Register. The register is based on a population of more than one-half million. Among the risk factors examined, the highest overall risk of recurrent stroke, 41.4, occurred with a history of at least one transient ischemic attack (TIA). After myocardial infarction (MI), the relative risk of a recurrent stroke was 8.0, while with all other heart diseases combined it was 8.4. With diabetes, the relative risk of a recurrent stroke was 5.6; with hypertension, it was 4.5. The relative risk increased with age after TIA and MI, but not for other heart disease, diabetes, and hypertension, except in the 85 + year-old age group.

Cerebellar ataxia, congenital cataracts, and retarded somatic and mental maturation Report of cases of Marinesco‐Sjögren syndrome

A SYNDROME CHARACTERIZED by cerebellar ataxia, congenital cataracts, and retarded physical and mental maturation was first described in 1931 by Marinesco, Draganesco, and Vasiliu in 4 Rumanian siblings. Almost twenty years passed before additional cases were recognized. After an intensive search in Sweden, Sjiigren was able to locate 14 affected individuals in 6 families. His genetic analysis suggested that the disorder was hereditary and transmitted by a single autosomal recessive gene. Richards? in 1950, presented 2 cases which he thought represented examples of this condition. Garland and Moorhouse reviewed the clinical features of this syndrome in 19S3 and presented 2 new cases. More recent descriptions of this condition have been presented by Franceschetti and associates,6 MacGillivray,B Dogulu and Mutlu, Dureaux and coworkers,* and Amyot,@ bringing the total of cases reported to date to 31. We have encountered 4 related Negroes who show the characteristic features of this rare syndrome. To our knowledge this condition has not yet been reported in the United States, nor has it ever been described in Negroes. These considerations have prompted us to report our observations.

Stroke in the Lehigh Valley: Combined risk factors for recurrent ischemic stroke

We used the Lehigh Valley Stroke Register and a logistic regression model for the odds ratio to study the relative contribution of several factors, considered jointly, to the risk of recurrent ischemic stroke. The factors were hypertension (HT), transient ischemic attack (TIA), myocardial infarction (MI), other heart diseases (OHD), diabetes mellitus (DM), age, and sex. Among these factors MI, OHD, and TIA constituted significantly greater risk than HTN, DM, age, or sex for ischemic stroke recurrence.

Standardized Incidence Ratios of Stroke: A Worldwide Review

Comparison of stroke incidence in various parts of the world is difficult because differences in diagnostic criteria, medical facilities and age-sex distributions are often not taken into account. We calculated age-sex standardized incidence ratios (SIR) of stroke in 37 regions based on recent reports. The USA (1976) was taken as the standard population. In western countries SIRs varied from 0.8 to 2.1. In China and Japan, SIRs varied from 0.7 to 3.6. While the lowest and highest SIRs differed by a factor of 5, only 6 of the 37 studies yielded a SIR of more than 2.0 or below 0.75. Of these, 4 were from China and Japan. The highest ratios were based on a very intensive door-to-door survey and probably should not be compared to less intensive studies. We conclude that world-wide variation in age-sex adjusted stroke rates is relatively small.

Stroke in the Lehigh Valley: Incidence Based on a Community-Wide Hospital Register

Since July 1982 a population-based study of stroke has been carried out in the Lehigh Valley, a region in Pennsylvania and New Jersey, USA, with 580,000 people. During the first year, the annual incidence rates of stroke and transient ischemic attack were 167 and 49 per 100,000 population, respectively. Cerebral thrombosis, embolus, cerebral hemorrhage and subarachnoid hemorrhage accounted for 76, 13, 8 and 3% of the strokes, respectively. The overall incidence rate and distribution of stroke by type agree well with other population-based studies. The large size of the population in the Lehigh Valley means that information on the epidemiology of stroke, as well as the effects of various therapies on stroke frequency and outcome, can be collected in a shorter period of time than in most other communities studied to date. Finally, an assessment of concordance in diagnosis between attending physicians and a neurologist using standardized criteria was possible for the entire community.

Clinical features of the Guillain-Barré syndrome.

In a country-wide search for patients with Guillain-Barré syndrome (GBS) in Israel, 89 patients were found between 1969 and 1972 who met standardized diagnostic criteria. These cases, derived from a well defined population, were analyzed to provide a more accurate picture of the full range of clinical signs than is available from selected case series in the literature. Preceding illnesses, presenting symptoms, maximal neurological deficit, reflex changes, sensory deficit, cranial nerve, sphincter, respiratory, autonomic disturbances and spinal fluid changes were determined. Mortality was 5.6% which is lower than in many series. Alternatives to account for the apparent benignity of GBS in Israel were offered.