Dissociation of neuropathology from severity of dementia in late-onset Alzheimer disease.

BACKGROUND: Little is known about Alzheimer disease at advanced ages, although its incidence continues to increase at least through the ninth decade of life. OBJECTIVE: To examine the effects of age on the relationship between clinical dementia severity and neuropathologic hallmarks in a large sample spanning the full age range. METHODS: The authors assessed 81 subjects during life for dementia severity, and examined their brains. They analyzed plaque and tangle burden, as well as the activities of the cholinergic marker enzymes acetylcholinesterase (AChE) and choline acetyltransferase (ChAT), in relation to age at death and the clinical severity of dementia. RESULTS: Dementia severity was strongly related to plaque and tangle burden in relatively young patients (aged < 75 years), but this correlation diminished with age and disappeared in the ninth decade of life. Among the oldest patients studied, there was no difference in plaque and tangle load between the mild and severe dementia cases. This interaction (p < 0.0001 for plaque density) was not observed for the cholinergic markers ChAT and AChE. CONCLUSION: The nature or expression of Alzheimer disease may be different in severely demented older patients, who have equal cholinergic deficits but significantly lower plaque and tangle burden. If confirmed in a prospective study, these findings have diagnostic and therapeutic implications.

Cutaneous Waldenström's macroglobulinemia: report of a case and overview of the spectrum of cutaneous disease.

Specific infiltration of the skin is a rare complication of Waldenström's macroglobulinemia. Cutaneous disease may also develop as a direct result of paraproteinemia and paraprotein specific antiepidermal autoimmune disease. We report a patient with Waldenström's macroglobulinemia in whom cutaneous infiltration developed with a clonal population of lymphoplasmacytoid B cells, summarize previously reported cases, and review the spectrum of cutaneous disease associated with this hematologic malignancy.

Relationships of sex hormone levels to dependence in activities of daily living in the frail elderly.

OBJECTIVES: We undertook this nursing home study in order to determine the relationships between dependency in activities of daily living (ADL) and blood levels of estrone, testosterone, androstenedione, and dehydroepiandrosterone (DHEA). Little is known about this issue. METHODS: cross-sectional study of 370 nursing home residents. Hormone levels in blood specimens drawn in 1997 and 1998 were correlated with degree of ADL dependency recorded in medical charts. RESULTS: Because of multiple comparisons associations were deemed significant for P-values < or =0.017 for males and < or =0.0125 for females. In males, the following were inversely related: testosterone levels with dependency in transferring and eating; estrone with eating and a summary ADL index; and androstenedione with toileting and a summary ADL index (in all cases, r=-0.4; P=0.007-0.015). Inverse trends existed between testosterone levels and dependency in mobility and a summary ADL index; and androstenedione and eating (in all cases r=-0.3; P=0.030-0.055). Among females the following were directly related: estrone levels with dependence in mobility, toileting, transferring, and a summary ADL index; and DHEA with transferring and a summary ADL index (r=0.2-0.3, P=0.0001-0.01). Trends existed between estrone and eating, and DHEA and toileting (r=0.1-0.2, P=0.04). CONCLUSION: In male residents, higher sex hormone levels are associated with better ADL performance. Among females the opposite is true. While further studies are needed to elucidate these relationships, our results and recent findings of others suggest sex hormone actions in older women differ from those in younger populations. A possible stress-related mechanism is also presented.

Elevated serum total and LDL cholesterol in very old patients with Alzheimer's disease.

The relationships of serum lipids with Alzheimer's disease (AD) and other dementias in very old patients are not clear. All residents of an academic nursing home were studied clinically for dementia and for serum lipids. All those autopsied over a 7.7-year period had apolipoprotein E (apoE) genotyping and detailed neuropathological examination. Those with pathologically defined criteria for AD (n = 84) were compared to all others who also had clinical dementia but did not show AD changes (n = 22). In contrast to most other reports of serum lipids in very old patients with AD, total cholesterol (TC) and low density lipoprotein cholesterol levels were each significantly higher for those with AD. The lipid-AD associations were progressively stronger with increasing pathological certainty of AD diagnosis. These relationships remained significant after adjustment for apoE genotype and for other known risk factors. The lipid-AD associations in a very old cohort, and prior evidence that elevated TC in middle life is a risk factor for later dementia, prompt consideration of factors associated with lipid metabolism in the development of Alzheimer's dementia.

Restraint reduction reduces serious injuries among nursing home residents.

OBJECTIVES: To describe how removing physical restraints affected injuries in nursing home settings. DESIGN: A 2-year prospective study of an educational intervention for physical restraint reduction. SETTING: Sixteen diverse nursing homes with 2075 beds in California, Michigan, New York, and North Carolina. PARTICIPANTS: Study A: 859 residents who were physically restrained at the onset of the intervention on October 1, 1991. Study B: all residents who occupied the 2075 beds in the 16 facilities 3 months before the intervention and 3 months after its completion. INTERVENTION: Educational program for nursing home staff followed by quarterly site consultations to participating nursing homes. MAIN OUTCOME MEASURES: Rate of physical restraint use and injuries. RESULTS: Study A: Serious injuries declined significantly among the 859 residents restrained initially when restraint orders were discontinued (X2 = 6.2, P = .013). Study B: During the intervention period, physical restraint use among the 2075 residents decreased from 41% to 4%, a 90% reduction. The decrease in the percentage of injuries of moderate to serious severity was significant (i.e., 7.5% vs 4.4%, P2-tail = .0004) as was the rate of moderate and serious injuries combined (Rate Ratio = 1.580, P2-tail = .0033). CONCLUSIONS: A substantial decrease in restraint use occurred without an increase in serious injuries. Although minor injuries and falls increased, restraint-free care is safe when a comprehensive assessment is done and restraint alternatives are used.

Arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens: treatment with isotretinoin.

A patient with arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens who showed a dramatic response to isotretinoin is described. This, to our knowledge, is the first report documenting effective treatment of this condition, whose nosologic position with respect to other spondyloarthropathies associated with cutaneous disease is considered.

Dermatofibrosarcoma protuberans in an infant with a history of choroid plexus papilloma.

We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor.

Treatment of lichen planus and lichen nitidus with itraconazole: reports of six cases.

Six patients with either lichen planus or lichen nitidus were treated with itraconazole. Two of four patients with lichen planus experienced complete clearing, and the remainder showed a partial response. Itraconazole, like griseofulvin, may prove to be an alternative treatment worth considering in patients with lichen planus or lichen nitidus who have failed other therapies.

The relationship between apolipoprotein E, dementia, and vascular illness.

The purpose of this study was to concurrently assess the relationship of Apolipoprotein E (APOE) with both dementias and vascular illnesses in the very old. Nine hundred and fifty nine subjects (mean age 85 years) in a long-term care facility were genotyped and cognitively tested with the Mini Mental State Exam. All subjects were studied for the relationship of APOE with atherosclerotic heart disease, hypertension, or stroke without concomitant dementia. Four hundred fifty individuals met criteria for inclusion into one of the following groups: Alzheimer's disease (n = 318), vascular dementia (n = 49), or not demented controls (n = 83) and were investigated for the relationship between APOE and these diagnostic categories. APOE epsilon4 was not associated with atherosclerotic heart disease, hypertension, or stroke without concomitant dementia. The APOE epsilon3 allele was more common in men with atherosclerotic heart disease. In contrast, the APOE epsilon4 allele was more common in patients with Alzheimer's disease (22%) and vascular dementia (26%) than in not demented controls (7%). APOE epsilon4 is associated with dementias in the very old, whereas its relationship with either peripheral or central nervous system vascular disease without dementia is not as robust.

Assessing life expectancies of older nursing home residents.

OBJECTIVE: Care of nursing home (NH) residents is often based on the usual survival of the home's residents. In order to improve our understanding of this population, and, thus, ultimately facilitate individualization of their care, we developed a mathematical model that predicts their survival. SETTING: The Jewish Home and Hospital (JHH), a nursing home. PARTICIPANTS: 1145 older residents who were at the JHH from January 1, 1986, through July 1, 1986. MEASUREMENTS: Information abstracted from medical records and JHH computerized data: clinical, demographic, and dependencies in activities of daily living (ADLs). MAIN OUTCOME MEASURE: survival from July 1, 1986. DESIGN: Retrospective cohort study via medical chart review. The study period covered admission to JHH through January 17, 1996. Accelerated failure time (AFT) models generated the life expectancy model derived from 50% of the study group and were validated on the remaining sample. We computed predicted AFT and proportional hazards (PH) life expectancies. RESULTS: Significant, independent predictors of decreased survival were male gender, increased age, increase in summary ADL index, and impairment of cardiac, respiratory, neurological, and endocrine/metabolic systems. The interaction between gender and respiratory system impairment was significant. The Spearman correlation coefficients between the observed survivals and those predicted by the Phase I model are 0.49 for Phase I residents and 0.42 for Phase II residents. Our sample life table includes NH residents with different risk profiles and their associated survival estimates as well as interquartile ranges. AFT and PH survivals were similar. CONCLUSION: This first comprehensive model that predicts survival of NH residents can help formulate public health policies and identify appropriate NH residents for clinical trials. The model is a promising step toward improving the health care of NH residents.

Symmetrical erosions in a neonate: a case of neonatal sucking blisters.

Neonatal sucking blisters appears as unilateral or bilaterally symmetrical bullae or erosions involving the distal upper extremities at birth. They represent minor self-inflicted trauma, presumably the result of a vigorous intrauterine sucking reflex. Recognizing this benign self-limited condition avoids unnecessary tests and alleviates anxiety on the part of the infant's family and physicians.

Lichen sclerosus following the lines of Blaschko.

A case of lichen sclerosus that developed in a pattern corresponding to the lines of Blaschko is described. This pattern of extragenital lichen sclerosus has not, to our knowledge, previously been reported and could result from an epidermal clone with altered androgen sensitivity supporting a hormonal pathogenesis for this disease.

Cutaneous heparin necrosis in a patient with heterozygous protein S deficiency.

A patient with heterozygous protein S deficiency experienced cutaneous necrosis following subcutaneous heparin administration. Deficiencies of both protein C and protein S, known risk factors for the more frequently encountered coumarin necrosis, may predispose patients to this complication of heparin therapy as well. The putative association of protein S deficiency with cutaneous heparin necrosis could not be proven, however, since attempts to reproduce the heparin necrosis were unsuccessful.

Taking charge. The role of nursing administrators in removing restraints.

The leadership and commitment of nursing administrators play a pivotal role in minimizing the use of restraints and maintaining a restraint-free environment. This article describes the role of nursing administrators in reducing the use of physical restraints as part of a 2-year, national nursing home restraint-reduction project. It reviews important information about restraint-free care the benefits of restraint-free care, and strategies to reduce the use of restraints in nursing homes, much of which is applicable to settings other than nursing homes.