Aminoff Suffering Syndrome: a New Pathological Entity in End-Stage Dementia.

©2006 by MEDIMOND S.r.l. G716C0586 55

Aminoff Suffering Syndrome (ASS): A New Pathological Entity in End-stage Dementia

B.Z. Aminoff

Geriatric Division, The Chaim Sheba Medical Center, Tel Hashomer 52621;
Human Suffering and Satisfaction Research Center, POB 56, El-Ad 48900, Israel.


The Aminoff Suffering Syndrome (ASS) defined by us may be the key criterion for enrolling end-stage dementia (ESD) patients for palliative and hospice treatment, and for the development of new alternative setting approaches, such as “Relief of Suffering Units” for end-stage and dying dementia patients.

Key words: End-stage dementia, Aminoff suffering syndrome, irreversible condition, dying.

Patient suffering is a pathological syndrome traditionally viewed as a state encompassing psychological distress, spiritual concerns and various aspects of physical pain. There is insufficient clinical evidence of suffering in dying dementia patients and key criteria for irreversible medical conditions that may lead to inappropriate evaluation and insufficient palliative treatment. The purpose of our study was to evaluate the Aminoff Suffering Syndrome in terminal dementia patients using the (MSSE) scale over time, from admission to a geriatric ward and during a 6-month followup period. The MSSE scale is a novel objective tool developed by us (1, 2) and has been presented at world and regional congresses in Berlin (1999), Jerusalem (2000), Vancouver (2001), Stockholm (2002), Tokyo (2003), Las Vegas (2004), Rio de Janeiro (2005), Madrid (2006), the Committee for Labor, Social Services and Health of the Israeli Knesset (Parliament) (2005). 56 10th International Conference on Alzheimer’s Disease and Related Disorders Materials and Methods We prospectively studied consecutive ESD patients admitted to our center during a 24-month period. Follow-up continued to cessation of the study.
Diagnosis of dementia was made according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-4) revised criteria. Inclusion criteria comprised severe dementia interference in verbal communication (MMSE 0/30),
and complete dependence in activities of daily living and functional movement (Functional Independence Measure [FIM] 18/126). We recruited patients diagnosed as suffering from Alzheimer’s disease, multi-infarct dementia, post-stroke dementia and dementia of unknown origin. The study was authorized by the local Helsinki Ethics Committee, and informed consent was given by families and caregivers. A total number of 158 ESD patients were admitted to our ward from various intensive care departments of the hospital. Eighty-seven patients were in a stable medical condition and most were discharged from the ward to their previous living arrangements, or to other nursing facilities. In the final analysis we included the remaining 71 patients (28 females, 43 males) with extremely advanced dementia who died in our ward during the study period. All patients were evaluated by the MSSE scale during the first week of admission to our geriatric department and during the last week of life. We also recorded other laboratory and clinical data relevant to the patients’ condition.
Statistical Analysis:
Comparisons between the three levels of MSSE (low, intermediate and high) with regard to demographic and clinical variables were performed using analysis of variance (ANOVA), Kruskal Wallis non-parametric test and Fisher’s
exact test, where applicable. The statistical significance level was set to 0.05 and the SPSS for Windows software, version 11.0, was used for the analysis.
The results of our research showed that care in the geriatric department failed to reduce the high level of suffering of ESD patients. The suffering level of an ESD patient increased until demise. Our research concluded that
63% and 30% of dementia patients died with a high and intermediate level of suffering, respectively (3). Only 7% died with a low level of suffering. On day of admission the total score of the MSSE scale was 5.62 ± 2.31, and
increased to 6.89 ± 1.95 on the last day of life, with a significant difference (P < 0.0001) (4). Mean survival time was 57.76 ± 9.73 days for the low MSSE score group (MSSE = 2.24 ± 0.99), 44.70 ± 5.99 days for the median MSSE score group (MSSE = 4.92 ± 0.83) and 27.54 ± 4.16 days for the high MSSE score group (MSSE = 8.06 ± 1.00). Differences between the survival times of these three MSSE score groups were statistically significant (Kaplan-Meier Analysis Log Rank Test, P = 0.0018; Breslow Test, P = 0.0027). The Cox proportional Hazard model of survival revealed a significant interrelation of high MSSE scores and shorter survival (P = 0.013) (5). Discussion: The decision-making process regarding palliative or a hospice-like approach for ESD patients is complicated because data on appropriate criteria to assess the suitability of hospice care are limited, resulting in <2% of patients with advanced dementia being enrolled in hospice care programs. One of the enrollment criteria identified by the American Medicare Hospice Benefit is a life expectancy <6 months. This narrow eligibility criterion ensures that the majority of enrolls will die within 6 months, and excludes a substantial proportion of individuals with advanced dementia who do not survive during that period. Several studies have investigated the 6-month survival of individuals with a primary diagnosis of advanced dementia. The National Hospice Organization eligibility guidelines for patients with dementia are based primarily on the Functional Assessment Staging (FAST) criteria that have been heavily criticized. A study on Dutch patients has also suggested that the Hospice Medicare Guidelines are not valid for predicting survival in late dementia. Palliative care is aimed at relieving suffering and improving the quality-of life of patients with advanced illnesses, and should involve a routine and standardized symptoms assessment. Defining and measuring these aspects is difficult, albeit crucial, as these patients are unable to recognize and communicate verbally with family members, are totally dependent on others in daily activities and experience repeated health problems. Our results regarding the interrelation of high MSSE scores with shorter survival period, irreversible and intractable a ggravation of suffering and medical condition until demise was defined by us as a new pathological entity in ESD – Aminoff Suffering Syndrome. Thus, we humbly suggest that diagnosis of Aminoff Suffering Syndrome could serve as a key criterion for screening in general hospital wards, nursing homes and community - of those ESD patients with high levels of suffering and shorter survival periods. The Aminoff Suffering Syndrome was proposed as the key criterion for enrolling end-stage disease and dying patients to a hospice or “Relief of Suffering Unit”. The only criterion for hospitalization in such a unit would be the high level of suffering, diagnosed by an objective method to estimate the suffering of an ESD patient. The indication for hospitalization in the unit is neither the cognitive, functional and nutritional condition, nor any other medical problem, but intensive care to reduce the level of suffering of the ESD patient. Treatment in the unit does not imply euthanasia. It may comprise intensified nursing and qualified medical staff in order to control the patients’ suffering and that of their families (6). The aim of the “Relief of Suffering Units” is to treat patients with high levels of suffering, to support them until their last breath and allow them to die honorably. Should there be an improvement in the patients’ condition 58 10th International Conference on Alzheimer’s Disease and Related Disorders during hospitalization in the unit, and their level of suffering diminishes, they may be discharged from the unit and transferred to a regular geriatric department, nursing home, or to their own home. The estimated hospitalization period in the “Relief of Suffering Units” is approximately 1 month. These units would be the source for integral medical, nursing, religious,ethical, psychological and sociological research, seeking methods to cope with the horrendous burden of suffering of ESD patients, their families and the nursing staff. On the 5th July, 2005, we presented our research results to the Committee for Labor, Social Services and Health of the Knesset (Israeli Parliament) and our suggested Amendment to the law for the founding of special units to treat dying patients with Aminoff Suffering Syndrome may be a possible solution for the establishment of the new Israeli Law “The Dying Patient”. Conclusion: Aminoff Suffering Syndrome (ASS) in terminal dementia is the new pathological symptomatology and entity that is characterized by a high MSSE scale score, <6 months survival, irreversible and intractable aggravation of suffering and medical condition until demise. References: 1. AMINOFF B.Z., PURITS E., NOY SH., ADUNSKY A. Measuring the suffering of end-stage dementia: reliability and validity of the . Arch Gerontol Geriatr 38:123-130, 2004. 2. AMINOFF B.Z. (MSSE) Scale: first 5 years. Isr Med Assoc J 6:645-646, 2004. 3. AMINOFF B.Z., ADUNSKY A. Dying dementia patients: too much suffering, too little palliation. Am J Alzheimer Dis Other Dementias 19: 243-247, 2004. 4. AMINOFF B.Z., ADUNSKY A. Dying dementia patients: too much suffering, too little palliation. Am J Hosp Palliat Med 22:5, 344-348, 2005 5. AMINOFF B.Z., ADUNSKY A. The last six months of life: Suffering and survival of end-stage dementia patients. Age Ageing 2006 ([in press]). 6. AMINOFF B.Z. Overprotection phenomenon with dying dementia patients. (Editorial). Am J Hosp Palliat Med 22:247-248, 2005.