Bullous pemphigoid and antecedent neurological diseases: An association with dementia.

BACKGROUND: Bullous pemphigoid is the most common subepidermal immunobullous disorder. Studies have reported the association between bullous pemphigoid and various neurological diseases. AIMS: The aim of this study was to evaluate whether bullous pemphigoid is associated with pre-existent neurological diseases and whether specific diseases exhibit this association. METHODS: All dermatology inpatients from January 2010 to May 2015 were analyzed. Bullous pemphigoid cases were identified based on clinical features and consistent histopathologic and direct immunofluorescence findings. Patients with other autoimmune bullous skin disorders were excluded. An equal number of inpatients with other skin conditions were selected randomly as age- and sex- matched controls. RESULTS: Out of 3015 inpatients, 103 cases of bullous pemphigoid and 103 age- and sex-matched controls were included. Seventy six patients with bullous pemphigoid had a history of at least one neurological disease. After adjusting for age, gender, race, functional status and neuro-psychiatric medications, patients with bullous pemphigoid were found to be approximately thrice as likely to have a history of at least one neurological disease than were controls (odds ratio: 2.88; 95% confidence interval: 1.32-6.26; P = 0.008). Amongst the pre-existing neurological diseases, only dementia was statistically more prevalent in bullous pemphigoid cases compared to controls (adjusted odds ratio: 2.61; 95% confidence interval: 1.19-5.75; P = 0.017). Parkinson disease and psychiatric disorders demonstrated a higher adjusted risk among bullous pemphigoid patients but the difference was not statistically significant. LIMITATIONS: The limitations were potential referral and selection bias, as the patients were inpatients. There is a possible misclassification as the diagnosis of neurological diseases was performed using medical records. The duration from the diagnosis of neurological diseases to bullous pemphigoid could not be accurately determined as it was a retrospective review of records and most neurological diseases have a prolonged course. CONCLUSIONS: Pre-existent neurological disease, specifically dementia, was found to be associated with bullous pemphigoid.

[Current status of dementia in admitions to national leprosariums nationwide].

The purpose of this study was to get an overview of the following aspects of people admitted to 13 national leprosariums in Japan: the prevalence of dementia, medical and nursing systems, and facilities and equipment. Subjects included 1733 people admitted to wards for patients or disabled people in these leprosariums. Subjects were examined for cognitive function using Nishimura's behavioral rating scale for the mental states of the elderly (NMS), and for the prevalence of behavioral and psychological symptoms of dementia (BPSD). We also investigated medical and nursing systems, facilities and equipment, and status of nursing education. The results showed that, 288 subjects (16.6%) had a diagnosis of dementia. According to the NMS, 47.5% of the subjects were categorized as mild to severe dementia, while cognitive dysfunction was observed in 63.5% if borderline cases were included. Non-specialist physicians managed 30.8% of the subjects in 4 institutions, and there were no certified nurses specialized in dementia management. Results from this study suggest that there is need for the placement of dementia specialists', improvement of specialized medical services, and human resource development of specialized nurses in leprosariums where many elderly people live.

The prevalence of dementia and depression in Taiwanese institutionalized leprosy patients, and the effectiveness evaluation of reminiscence therapy--a longitudinal, single-blind, randomized control study.

OBJECTIVE: This study investigated the prevalence of depression and dementia in long-term institutionalized older leprosy patients in Taiwan. We then examined the effectiveness of reminiscence group therapy on depressive symptoms and cognitive function in this population. METHODS: We recruited 129 long-term institutionalized older leprosy patients in Taiwan and used the Geriatric Depression Scale-Short Form (GDS-SF), the mini mental state examination (MMSE), and the Clinical Dementia Rating (CDR) scale for outcome measurement. We then conducted a single-blind, randomized, longitudinal quasi-experimental analysis comparing the pre-test and post-test results of two equivalent groups at a 24-week intervention interval. The intervention in the experimental group was three sessions of reminiscence group therapy per week, whereas that in the control group was weekly individual supportive interviews. RESULTS: The prevalence of dementia was 45.7-50.4% according to a CDR score ≥ 0.5 and an MMSE score < 25, and the prevalence of depression was 25% based on a GDS-SF score ≥ 7. According to the Wilcoxon signed-rank test, the GDS-SF scores in the experimental group decreased significantly (p = 0.02) after intervention as compared with that in the control group (p = 0.22), whereas the MMSE scores in both groups remained steady. CONCLUSIONS: The prevalence of dementia and depression in long-term institutionalized older leprosy patients in Taiwan is high. Reminiscence group therapy has been confirmed effective on depression in this population, but its effectiveness on cognitive function requires further verification.

[Increased incidence of senile dementia in the Hansen's disease sanatoriums of Japan].

In 1973, Narita reported that the rate of demented patients above 65 years was about 2% at the sanatorium--Tama Zenshoen, which was much lower than that of the average aged people (4-5%). The reason for that was thought to be due to the acquirement of strong mind under loneliness and difficulties faced by the patients through social enforcement to live in isolation. From that report on the low percentage of dementia among the Japanese Hansen patients reemed to be widely accepted. Fifteen years later in 1988, Narita reported that the rate of demented patients above 65 years was 3.2% in males and 3.6% in females indicating still a low rate at Tama Zenshoen. In 1994, however, Narita found unexpectedly that the percentage of dementia among patients above 80 at Tama Zenshoen was higher than the average aged population. Therefore in this report, we tried to analyze the reason for this difference. In addition to Tama Zenshoen patients, we also investigated those of another Hansen disease sanatorium namely Nagashima Aiseien which has similar structural organization as Tama Zenshoen. A comparison of dementia between these two was carried out, taking patients above 65 years as the study group (Used GBS scale based on Karasawa's method). In Tama Zenshoen, the rate of patients with dementia was 18.6% in males and 31% in females. Above 80 years those were 54.4% in males and 75.9% in females. The reason for the above high rate of dementia may be probably due to the passage of long years (40 to 50 years) after the enforcement to live in isolation causing the weakening of the spirit. The patients might have got some mental support if they had offspring, but they had no way to have any under the forced contraception. On the contrary, in Nagashima Aiseisen, the percentage of patients with dementia was low, namely 3.6% in males and 9.1% in females. In those patients above 80 years, it was 2.7% in males and 6.8% in females, those were almost similar to the average normal population (6%). Such a big difference was quite surprising. The reason behind this difference, may be due to the construction of the houses for the disabled persons. Both Nagashima Aiseien and Tama Zenshoen, have small individual rooms. In Tama Zenshoen, patients are able to visit their close neighbor, only after changing their shoes or with the aid of wheel chairs, but in Nagashima Aiseien, the corridors connecting quarters allow the patients to move easily to his neighbors with bare feet or even with crawl. Therefore, in Nagashima Aiseien, the number of patients who have social contacts with their neighbors is much greater in number which make them mentally active, thus eliminating the major factors affecting dementia. We can conclude that such communication between friends and co-mates make them alert and keep the spirit in the aged.

Neuropathological analysis of dementia in a Japanese leprosarium.

In a neuropathological study of consecutive autopsies, prevalence and cause of dementia in a Japanese leprosarium were investigated, where more than 95% of inpatients with a mean age of 70 years are now free from active leprosy. In 10 years (1983-1992), clinically overt dementia at death was 35/136 (25.7%) in the age group over 65 years (mean age 79.4). Autopsy was performed in 85 cases (mean age 81 years), and clinically overt dementia was seen in 25 subjects (29.4%). Neuropathologically, Alzheimer's disease (AD) was seen in 9 cases (10.6%), vascular dementia (VD) in 9 cases (10.6%), mixed type in 3 cases (3.5%) and unclassified in 4 cases (4.7%). In the age group of 65-84 years, AD was 5/58 (8.6%), VD was 4/58 (6.9%), mixed type was 2/58 (3.4%), and unclassified was 1/58 (1.7%). Compared with previous Japanese general population-based data, where VD was more frequent than AD, the rate of dementia in our leprosarium was high, and pathologically confirmed AD was as common as VD. Recently, a prophylactic effect of the antileprosy and anti-inflammatory drug DDS (dapsone, 4,4'-diaminodiphenyl sulfone) has been suggested. Lepromatous patients take more DDS (51.9%) than tuberculoid patients (11.5%), however, as the dementia rate of tuberculoid leprosy (17.9%) in those 65-84 years old is similar to lepromatous leprosy (15.9%) in our study, we do not support their viewpoint.