Diogenes syndrome

Diogenes syndrome also known as dermatitis passivata, severe self-neglect syndrome, aged recluse or social breakdown of the elderly, is characterized by extreme self-neglect, poor personal and domestic hygiene and social withdrawal with abnormal collecting pattern, tendency to hoard excessively (syllogomania), and refusal of help, which may be precipitated by stressful event ¹. Diogenes syndrome is a rarely reported syndrome of extreme self-neglect, social withdrawal and domestic squalor ². Diogenes syndrome is named after the Greek Philosopher “Diogenes of Sinope” (4th century BCE) who taught about cynicism philosophy (minimizing needs and a natural way of life) ³. He kept his need for clothing and food to a minimum by begging. He used to follow some ideas like “life according to nature,” “self-sufficiency,” “freedom from emotion,” “lack of shame,” “outspokenness,” and “contempt for social organization” ³. Diogenes syndrome has been described in different psychiatric literature and very few cases associated with dermatological presentation have been reported.

The term Diogenes syndrome was coined in 1975 by Clark et al. ⁴ to describe elderly patients with severe self-neglect, poor personal and domestic hygiene. Diogenes syndrome usually affects elderly persons and there is no sex predilection. Diogenes syndrome affects men and women equally and may be seen in all socioeconomic groups. Diogenes syndrome usually follows a distinct sociodemographic profile where it is found that persons are usually single, aged, having average or above average intelligence, and also having good income ⁵. Most live alone in physical isolation ². Hoarding of rubbish may or may not be present ⁶.

Nutritional deficiencies of iron, folate, vitamin B12, vitamin C, vitamin D and serum proteins may be seen. Mental illness, withdrawal and denial of need in old age may be triggered by various stressful situations ⁷.

Diogenes syndrome has been classified as primary or pure which is not associated with mental illness and secondary or symptomatic. Secondary Diogenes syndrome is associated with underlying mental illness like schizophrenia, depression, and dementia ⁵. Alcohol abuse has been identified as a cofactor ⁸.

Certain characteristics of the Diogenes syndrome have been recognized; these include social withdrawal, filthy home, neglected self care, squalor syndrome, collection of useless objects or hoarding, shameless attitude, and stubborn refusal of help ⁵. At least 4 of them are almost permanent symptoms: patients do not ask for any help although they possesses nothing; unusually fond of objects (hoarding of rubbish, or nothing in the house); unusual behavior with other people (misanthropy) and extreme self-neglect ⁹. Hoarding may be absent in some cases ¹⁰. A question has been raised whether Diogenes syndrome is due to self-neglect or maltreatment of the elderly but the latter has been identified as the most probable cause resulting in this pitiful condition ⁵. Though principally affecting the elderly, young persons have been diagnosed with this condition. Such persons usually have above average intelligence and it is now clear that some stressful event precipitates the disease in predisposed individuals. Multiple deficiency states have been associated with Diogenes syndrome including iron, folate, vitamin B12, vitamin C, calcium and vitamin D, serum proteins and albumin, water, and potassium ⁴.

Skin lesions are mainly due to uncleanliness which may result in various infestations and infections. These are ignored by the patient. Dirt, dust, bacterial, fungal, and parasitic debris conglomerate to form thick crusts and scales over various parts of the body ⁸.

Diogenes syndrome causes

Although exact causes of Diogenes syndrome is not still well known, it has been hypothesized that Diogenes syndrome may occur as a result of a response given to the various stress factors such as death of spouse or a significant medical illness among people with subclinical personality disorder ¹¹. These patients may have psychiatric disorders like paranoid disorders, mood affection, or temporofrontal dementia ¹. However, it was indicated that an exaggerated sense of self disorganized lifestyle or indifference depending on the aggravation can occur together with older ages ¹². In some studies, it was reported that there were no mental illness in patients affected by Diogenes syndrome; on the contrary they have average or high intellectual level ¹³. However, differences among the cases in cognitive and socio-economic levels were reported ¹⁴. At least in 50% of the cases a kind of psychiatric disorder was described ¹⁵. Therefore, these cases can be classified as secondary Diogenes Syndrome ¹⁴.

Dementia and Diogenes syndrome

New-onset Diogenes syndrome in older age may be due to dementia, for instance. Most patients showing self-neglect are diagnosed with dementia within 1 or 2 years of presentation ¹⁶. In fact, patients with dementia invariably develop progressive inability to take care of themselves. It has been long observed that individuals with dementia develop inability to assess critically what is of value, and that can result in the accumulation of trash and objects ¹⁷. In the Eastern Baltimore study, dementia was present in 15% of those with moderate and severe social breakdown syndrome, twice as many as in the general population of the same age group ¹⁸. In the diagnostic consensus study of Neary et al ¹⁹, decline in personal hygiene is one of the supportive features of frontotemporal dementia. Lebert underlines the frequent presence of Diogenes syndrome (36%) in frontotemporal dementia ²⁰: different neuropsychological modifications in frontotemporal dementia can contribute to symptoms of Diogenes syndrome. Apathy, for example, can reduce the inclination to wash oneself and the alteration of executive functions can explain the simplification of complex tasks, such as maintenance of washing ²⁰. A possible link with frontal lobe dementia has been questioned due to the younger age of onset of this type of dementing disease ²¹. The prognosis is poor, with 5-year mortality rate of 46%, possibly due to physical complications ²². Cognitive dysfunction may be the trigger or the one of the consequences, for example nutritional intake is poor and many have a very restricted intake, often limited to certain types of food. A special problem is capacity, a term that, improperly, is often used interchangeably with competence. Competence is the quality or state of being functionally adequate or having sufficient knowledge, strength, and skill. Mental capacity is a functional term that may be defined as the mental (or cognitive) ability to understand the nature, and effects of one’s acts. Capacity may have several dimensions, including decisional ability, personal care, and self-care. There may be a variety of dimensions of capacity in which an older adult may be able to make some decisions but not others. Competence can fluctuate, according to some experts ²³. It is possible that individuals can retain some significant decisionmaking power even with advancing dementia, albeit usually not regarding health care. The diagnosis of dementia is not itself a criterion for incapacity ²⁴. In addition, cultural considerations related to lifestyle and environmental patterns should be explored thoroughly during the capacity evaluation: forensic assessments can help to sort out dangerousness on the basis of dementia versus an eccentric lifestyle taken to the extreme.

Diogenes syndrome symptoms

Diogenes syndrome causes lack of hygiene due to self-neglect, poor nutrition which causes anemia and vitamin deficiencies, and due to take or refuse medical care it may lead aggravation of diseases such as congestive heart failure, bronchopneumonia, diabetes mellitus, ischemic heart disease, cerebrovascular disease, and different malignancies ¹⁴. Nutritional deficiencies of iron, folate, vitamin B12, vitamin C, vitamin D and serum proteins may be seen ¹. Skin lesions are mainly due to uncleanliness which may result in various infestations and infections. Dirt, dust, bacterial, fungal, and parasitic debris conglomerate to form thick crusts and scales over various parts of the body ⁸.

Cessation of normal skin cleansing seen in geriatric or self-neglected patients can cause accumulation of keratinous crusts on the skin. In the extreme end of this spectrum is a condition known as Diogenes syndrome ¹. The cutaneous manifestations of neglect (dermatitis passivata) arise from accretion of keratin and dirt and resemble a carapace. Lesions are commonly found on the upper central chest, back and groin ²⁵.

Diogenes syndrome may be confused with abuse or neglect of the elders depending on malnutrition and poor living conditions, especially vitamin K and C deficiencies, coagulopathies due to alcoholic liver disease and common bruising and severe hemorrhage as a result of minor traumas. Because of the social isolation and living alone, disappearance of these cases may go unnoticed by those who live around and in most of the cases they found dead by chance and when they found there can be seen in various stages of decay on corpses. Organ and tissue losses that occur depending on decay or due to changes in the postmortem examination and autopsy, there may be identification and investigation difficulties in determining the cause of death.

Diogenes syndrome diagnosis

The assessment of Diogenes syndrome must begin with gathering a comprehensive history, which should include a thorough history of behavioral disturbances. A complete physical examination and blood screening is essential: this should include iron, folate, vitamin B12, calcium, serum proteins, albumin, and potassium. Liver function tests, renal function, and thyroid status will serve as baseline tests. Neuroimaging studies are also necessary to rule out underlying medical causes. Such an evaluation would include neuropsychological and personality assessment along with consideration of the psychosocial factors which might be maintaining the self-neglect behavior.

Diogenes syndrome treatment

Management is a difficult issue: patients’ continued refusal of help gives rise to complex ethical and medicolegal issues ²⁶. Specifically, intervention usually does not occur at the request of the individuals themselves. The diversity of associated mental and physical health problems lends support to the argument that squalor may be treated best as a state associated with, or a consequence of, a range of physical and mental disorders which requires careful assessment and treatment, rather than as a rare syndrome due to reclusiveness or an eccentric personality ²⁷. There are no clear guidelines, pharmacological or nonpharmacological, on how best to manage people with Diogenes syndrome, and there are no controlled trials or even case series in this area  ²⁶. However, management involves not only treatment of the underlying disease, but also an understanding of available service agencies. Day care and community care are the main lines of management rather than hospital admission. A safe environment should be provided, while respecting the patient’s wishes as much as possible. While Diogenes syndrome can be treated with medical support, death may also occur as a result of these diseases ²⁸. Atypical antipsychotic agents have been used when paranoid symptoms are present. Herran and Vazquez-Barquero ²⁹ reported the case of a 77-year-old woman fulfilling criteria for dementia with symptoms of Diogenes syndrome: treatment with risperidone improved the behavioral symptoms. Galvez-Andres el al ³⁰ described the significant improvements seen in frontotemporal dementia patients with Diogenes syndrome after the start of treatment with quetiapine and sodium valproate. The use of selective serotonin reuptake inhibitors to manage the compulsive hoarding behaviors has been reported ³¹. Noncompliance, with treatment and follow-up are common in patients with Diogenes syndrome; thus the outcomes of the syndrome are poor despite efforts and care ³². Gentle persuasion initially, and finally use of the mental health act is probably the best approach, as the patients are otherwise a risk to themselves and others.

References

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