Characterizing the Role of Animal Exposures in Cryptosporidiosis and Shiga Toxin-producing Escherichia coli Infections: South Dakota, 2012.

Cryptosporidium spp. and Shiga toxin-producing Escherichia coli strains (STEC) are important causes of human illness. Incidence rates of these illnesses are high in South Dakota compared to the USA as a whole. Direct animal contact has been identified as a possible route of exposure for these illnesses. Ruminant animals may carry STEC subclinically, while young ruminants are common sources of zoonotic strains of Cryptosporidium. South Dakota patients with either STEC or cryptosporidiosis during 2012 were interviewed regarding seven categories of animal exposure: (i) petting zoo/fair attendance, (ii) animal event/rodeo attendance, (iii) feed/pet store visits, (iv) farm visits, (v) employment or residence at a farm, (vi) residence with pets and (vii) visiting other households with pets. Of the 50 STEC cases, 78.0% reported animal exposure prior to illness onset, with 23.3% having lived or worked on a farm. Farm visitors in particular had high degrees of animal contact and infrequently practiced personal protective measures. Of the 115 cryptosporidiosis cases, 87.8% reported animal exposures, with 45.6% having lived or worked on a farm and 29.0% having visited a farm prior to illness. Cases with farm exposures reported a high degree of direct animal contact and inconsistent use of personal protective measures. Cryptosporidiosis patients were significantly more likely than STEC patients to have lived or worked on a farm prior to their illness and were older on average. Patients with these illnesses had high rates of animal contact prior to illness. Animal contact on farms emerged as an important exposure route. Educational messages about personal protective measures should be directed at these individuals.

Effects of aging on visual evoked responses.

The effects of aging of visual evoked responses (VER) and critical frequency of photic driving (CFPD) was studied in 74 volunteers aged 18 to 79. The amplitude of VER to pattern reversal stimulation did not vary with sex or age. The latencies of the first major negative and of the first major positive defection of the VER were significantly delayed (P less than .001) with advancing age. This increase of latency probably reflects a slowing of conduction velocity in the optic nerve or optic pathways or both. CFPD, defined as the highest frequency of photic driving response expressed in flashes per second, showed an inverse correlation, decreasing with age in older subjects. Critical frequency of photic driving is the electrophysiological counterpart of critical flicker fusion, which is known to decrease with advancing age. These data support the concept that aging influences the functions of specialized sensory systems.

Visual electroencephalographic computer analysis (VECA). A new electrophysiologic test for the diagnosis of optic nerve lesions.

The function of optic pathways was studied by visual electroencephalographic computer analysis (VECA). Pattern and flash stimuli were used. The VECA profile was abnormal in two types of lesions: pathology involving the eye, and pathology involving the optic nerve. When ocular pathology is excluded, an abnormal profile indicates optic nerve dysfunction. Of the multiple sclerosis patients tested, 77 percent had an abnormal VECA profile. The test was always abnormal in patients with optic neuritis. Delayed visual evoked responses occurred in 18 of 29 multiple sclerosis patients judged to be clinically without visual deficits. VECA is reliable and sensitive for detecting clinical and subclinical optic nerve pathology.

Inheritance of reading epilepsy.

Primary reading epilepsy was diagnosed by special electroencephalographic (EEG) studies in an adolescent youth after his first grand mal seizure. Similar studies of members in three generations of his family showed primary reading epilepsy in his first cousin and in two of his siblings. The disorder in the siblings had not been noticed by parents or teachers and probably would have remained undetected without the use of special techniques. This pedigree buttresses other reported evidence for autosomal dominant transmission of reading epilepsy. The centrencephalic EEG trait also is present in some members of this family. The possible inplications of this association are discussed.

Dystrophia myotonica in a 47, XXY male.

Classical clinical manifestations of dystrophia myotonica were apparently unaltered by aneuploidy in a 47, XXY male. His extremely eunuchoid habitus may represent a contribution by the point mutation to his Klinefelter's syndrome. Association of the two disorders may be due to factors other than chance. The danger of overlooking Klinefelter's syndrome in males with dystrophia myotonica can be avoided by cytologic studies.

Diagonal earlobe creases and ischemic stroke: Preliminary report.

Diagonal earlobe creases are associated with coronary arterial disease, cardiac morbidity and mortality, and all-cause mortality. We studied the influence of earlobe creases on the incidence of ischemic stroke as well as the relationship between earlobe creases and gender, coronary arterial disease, hypertension, diabetes mellirus, and hypercholesterolemia. Data were obtained prospectively from the medical records of 116 patients with ischemic stroke and 232 age- and gender-matched patients without ischemic stroke. All patients were seen in neurological consultation and were examined for the presence of earlobe creases. In the total group of 348 patients, earlobe creases were significantly related to advancing age. In those with ischemic stroke, earlobe creases were significantly related to coronary arterial disease, diabetes mellitus, and nonlacunar ischemic stroke. The cumulative incidences of coronary arterial disease, hypertension, diabetes mellitus, and hypercholesterolemia were also significantly higher in those patients with earlobe creases who also had ischemic stroke. When controlled for coronary arterial disease, earlobe creases, in those with ischemic stroke, continued to be significantly related to nonlacunar ischemic stroke.

Frequency of XYY males in Wisconsin state correctional institutions.

Karotyping of 3011 males at five Wisconsin state correctional institutions revealed that 1% had a chromosome abnormality. The frequency of occurrence of the XYY complement was about 5 times that for newborn males. Approximately the same rate was found among 2556 males in the three penal institutions for adults. The frequency of XYY at the institution for juvenile offenders was about ten times background. The relatively low frequency of XYY (0.38%) found at the mental-penal institution may have been due to previous sampling done there. Of the 16 XYY males discovered, only two were below the 85th percentile for height. A relatively low frequency of XYY was found among black males. Our data contradict the notion that a high rate of XYY among adult males in penal settings may be due to a disproportionately large number of tall men in prisons.

Structure and Barr body formation of an Xp + chromosome with two inactivation centers.

A patients with seizures, Von Willebrand disease, and symptoms of Turner syndrome was a chromosomal mosaic. In blood culture (1974), 56% of the cells were 45, X 33% 46, XXp+ and 11% 47,XXp + Xp +; in the skin, no cells with 47 chromosomes were found. Presumably the Xp + chromosome arose through a break in the Q-banded dark region next to the centromere on Xp to which an Xq had been attached. The abnormal X was late-labeling and formed a larger than normal Barr body. Of the chromatin-positive fibroblasts, 18.2% showed bipartite Barr bodies, which agrees with the hypothesis that the X inactivation center lies on the proximal part of the Xq. On the basis of the structure and behavior of the bipartite bodies in the present patient, as compared to those formed by other chromosomes with two presumed inactivation centers, we propose that the dark region next to the centromere of Xp remains active in the inactive X. In cells with 45,X and 46,XY, this region has the same relative size, whereas it is significantly shorter in the active X of three females, including the present patient, with one abnormal X. We propose that this region on the active X reveals different states of activity, as reflected in its length, depending on how many other X chromosomes are in the cell.

Essential tremor, nystagmus and duodenal ulceration. A "new" dominantly inherited condition.

The familial occurrence of essential tremor combined with (congenital) nystagmus, duodenal ulceration and a narcolepsy-like sleep disturbance caused by an autosomal dominant gene with high penetrance and fairly uniform expressivity is reported in a family of Swedish-Finnish ancestry. Twelve of 17 affected family members had essential tremor which began between 30-40 years of age and which could be controlled temporarily by alcohol; this resulted in alcoholism in several affected individuals. The most severly affected persons showed cerebellar signs which may reflect a possible pathogenetic relationship of the syndrome to the genetic cerebellar atrophies. Nystagmus, observed in 12 of 17 affected family members (eight of whom were also affected with tremor) usually was congenital and accompanied by refractive errors. Duodenal ulcers occurred almost exclusively in individuals with the neurological syndrome, and preceded its onset in some cases. The ulcer disease therefore seems to be a component manifestation of the syndrome and is interpreted as a pleiotropic effect of the gene which also causes the nystagmus, tremor and sleep disturbance.