Brief Communication

non-Shiga toxin-associated HUS are not caused by mutations of regulatory proteins of the complement alternative pathway like FH and cofactor protein (CD46), but
occur in the context of an autoimmune disease with the
development of anti-FH antibodies leading to an acquired
FH deficiency. In the present case, we might speculate
that the patient has developed such antibodies, leading to
an acquired FH deficiency causing HUS.11 Unfortunately,

References
1 Coppo P, Bengoufa D, Veyradier A,
Wolf M, Bussel A, Millot GA et al.
Severe ADAMTS13 deficiency in adult
idiopathic thrombotic microangiopathies
defines a subset of patients characterized
by various autoimmune manifestations,
lower platelet count, and mild renal
involvement. Medicine 2004; 83:
23344.
2 Hamasaki K, Mimura T, Kanda H,
Kubo K, Setoguchi K, Satoh T et al.
Systemic lupus erythematosus and
thrombotic thrombocytopenic purpura: a
case report and literature review. Clin
Rheumatol 2003; 22: 3558.
3 Amoura Z, Costedoat-Chalumeau N,
Veyradier A, Wolf M, Ghillani-Dalbin P,
Cacoub P et al. Thrombotic
thrombocytopenic purpura with severe
ADAMTS-13 deficiency in two patients

FH and anti-FH antibodies were not checked to confirm

this hypothesis.
In conclusion, this case highlights the importance of
looking for TM, such as TTP and also HUS, when a patient
treated for lupus nephritis does not improve after intensive therapy and presents with haematological disorders.
Such an association must be recognised very early and
requires specific therapy that was PE in our case.

with primary antiphospholipid

syndrome. Arthritis Rheum 2004; 50:
32604.
Copelovitch L, Kaplan BS. The
thrombotic microangiopathies. Pediatr
Nephrol 2008; 23: 17617.
Veyradier A, Obert B, Houllier A,
Meyer D, Girma JP. Specific von
Willebrand factor-cleaving protease
in thrombotic microangiopathies:
a study of 111 cases. Blood 2001; 98:
176572.
Tostivint I, Mougenot B, Flahault A,
Vigneau C, Costa MA, Haymann JP et al.
Adult haemolytic and uraemic
syndrome: causes and prognostic factors
in the last decade. Nephrol Dial
Transplant 2002; 17: 122834.
Caprioli J, Noris M, Brioschi S,
Pianetti G, Castelletti F, Bettinaglio P
et al. Genetics of HUS: the impact of
MCP, CFH, and IF mutations on clinical

10

11

presentation, response to treatment, and

outcome. Blood 2006; 108: 126779.
George JN. Clinical practice. Thrombotic
thrombocytopenic purpura. N Engl J Med
2006; 354: 192735.
Hunt BJ, Tueger S, Pattison J,
Cavenagh J, DCruz DP.
Microangiopathic haemolytic anaemia
secondary to lupus nephritis: an
important differential diagnosis of
thrombotic thrombocytopenic purpura.
Lupus 2007; 16: 35862.
Zipfel PF. Hemolytic uremic syndrome:
how do factor H mutants mediate
endothelial damage? Trends Immunol
2001; 22: 3458.
Dragon-Durey MA, Loirat C, Cloarec S,
Macher MA, Blouin J, Nivet H et al.
Anti-factor H autoantibodies associated
with atypical hemolytic uremic
syndrome. J Am Soc Nephrol 2005; 16:
55563.

Phenomenology of squalor, hoarding and self-neglect:

an Australian aged care perspective
imj_2634

98..105

S. M. Lee1 and D. LoGiudice2

1

Western Health, Sunshine Hospital and 2Royal Park, Melbourne Health and National Ageing Research Institute, Melbourne, Victoria, Australia

Key words
squalor, hoarding, self-neglect, Diogenes
syndrome.
Correspondence
Sook Meng Lee, The Aged Care Assessment
Service, Sunshine Hospital, Furlong Road, St.
Albans, Vic. 3032, Australia.
Email: sookmeng.lee@wh.org.au

Aged care health professionals in Australia are increasingly referred patients whose
standard of cleanliness and self-care has deteriorated to levels resulting in public health
concern. This paper describes three illustrative case studies of people referred to an
Australian Aged Care Assessment Service who present with Diogenes Syndrome. The
diversity and complexity of these cases reflect variable underlying diagnoses. Symptoms
of self-neglect, hoarding and domestic squalor and combinations thereof may provide a
more useful classification system of the older person who presents in such circumstances
than the frequently used term Diogenes syndrome. Practical guidelines are required for
appropriate assessment and management of these conditions.

Received 31 July 2010; accepted 7 September

2010.
doi:10.1111/j.1445-5994.2011.02634.x

98

2012 The Authors

Internal Medicine Journal 2012 Royal Australasian College of Physicians

Brief Communication

Australian data showed 1 in 1000 person over age 65

lives in squalor1 and a third of them with associated
clutter. With an ageing population, Aged Care physicians
and health professionals of Aged Care Assessment
Services (ACAS) in Australia are increasingly referred
patients whose standard of self-care and cleanliness have
declined to such low levels that there is cause for public
health concern. The responses can be variable depending
on the individual assessors experience, knowledge and
attitude towards the condition, as well as the attitudes
and responses of the person referred.
The symptoms of extreme self-neglect, domestic
squalor, social withdrawal, apathy, tendency to hoard
rubbish and lack of shame2 can occur in varying combinations. Different titles describing this condition are
found in the literature, including Senile Breakdown,3
Diogenes Syndrome,4 Senile Recluse5 and Senile Squalor
Syndrome.6 However, the condition is not restricted to
the elderly7 and can be present in those with psychiatric
disorders, dementia and long-standing alcohol abuse.8
Self-neglect is defined as failure to engage in activities
that a given culture deems necessary to maintain a
socially accepted standard of personal and household
hygiene, and health status.9 These people often exhibit
extreme reluctance to accept help. Domestic squalor
describes living conditions that are filthy and disgusting
and does not make any inference to the person.1 The
passive accumulation of rubbish from failure to remove
household waste is strictly speaking a form of neglect
rather than hoarding.1
In contrast, compulsive hoarding is defined as: (i) the
acquisition of a large number of possessions; (ii) subsequent failure to discard possessions; and (iii) resulting
clutter that precludes the use of living spaces in the
manner for which those spaces were designed.10 The
severity of hoarding is proportional to greater emotional
attachment to the possessions, reliance on possessions for
emotional comfort and an inflated sense of responsibility
of the possessions, causing difficulty in discarding.10
When Clark coined Diogenes Syndrome, he described
a group of people with high intellect and education from
a higher socio-economic background and normal mental
state. These findings have not been replicated and other
conditions, such as dementia, psychiatric illnesses and
alcohol abuse, have been associated with self-neglect,
squalor and hoarding.8 This raises the issue whether the
presence of these symptoms constitutes a syndrome in
their own right.

Funding: None.
Conflict of interest: None.
2012 The Authors
Internal Medicine Journal 2012 Royal Australasian College of Physicians

There is also debate about the suitability of the name

Diogenes to describe the syndrome. Diogenes was a
Greek philosopher whose core values were autonomy
through self-sufficiency and contentment unrelated to
material possessions; these values were not necessarily
upheld by the people living in hoarded environments,
squalor and self-neglect. Although not encouraged by
experts in the field in Australia, the term Diogenes Syndrome has been useful in raising the issue among health
professionals.
Not surprisingly, there is confusion in the application of
diagnostic labels in clinical practice. Community health
professionals are more likely to differentiate self-neglect,
squalor, collecting and hoarding,11 rather than using an all
encompassing term, such as Diogenes Syndrome. To illustrate some of these points, three case studies are described.
Case 1: The manager of a post office referred a 79-yearold woman to the ACAS with concerns about her health
and state of mind. She was described as malodorous,
unwashed and dishevelled. Footage from the security
surveillance video revealed the patient voiding inappropriately in the post office in full view of customers seemingly indifferent to their reactions. She was known to
have chronic schizophrenia and has long been estranged
from her family.
On assessment, she was dyspnoeic on exertion,
unsteady on feet and appeared unwell. She wore multiple layers of dirty clothes despite the warm weather.
The ACAS assessor had to engage with her on the street
before eventually gaining permission to enter her house,
which was filthy with rotting food, human excrement,
rubbish and dirt. The stench was nauseating. The house
was sparsely furnished, there was no fresh food and all
utilities were no longer connected.
On admission to hospital, the patient was dehydrated,
disorientated, doubly incontinent and in atrial fibrillation
with rapid ventricular response. Her investigations
revealed that she had multiple nutritional and vitamin
deficiencies, and significant anaemia. She deteriorated
rapidly during her inpatient stay and eventually died of
hospital-acquired pneumonia.
Underlying condition: chronic schizophrenia
Predominant symptoms: self-neglect and domestic
squalor
Other symptoms: social withdrawal, apparent lack of
shame
Absent: compulsive hoarding
Case 2: A neighbour referred an 87-year-old man who
was frail, forgetful and described as eccentric. He had a
history of bilateral frontal lobe haematomas from a previous road accident and alcohol-related dementia. The
neighbour also reported vermin infestation and hoarding
of junk in the house and garden.
99

Brief Communication

On arrival at the house, the patient was seen wearing

soiled pyjamas, emptying a bucket of urine on the nature
strip. The patients hair was matted and his finger nails
were filthy. He was malodorous and had long and curled
onychogryphotic toenails with black cheesy material
between his toes, highly suggestive of long-standing
fungal infection.
The property contained thousands of bicycles and
machinery, the house was in a state of disrepair with a
narrow passageway hoarded with newspaper, pieces of
wood, bicycles, tins of paint and solvents. The ACAS
assessors had to turn sideways and step over piles of
newspaper to get through. The stove had a thick layer of
blackened grease and spilt food, the exhaust fan dripped
grease and the sink was filthy. The kitchen table and
chairs were not visible because of the amount of material
piled on them. The rest of the house was similarly
hoarded.
This person was facing prosecution by the council for
not responding to an order to clean-up the property.
An application for a guardian and administrator was
successful.
Underlying condition: frontal lobe dysfunction and
alcohol-related dementia
Predominant symptoms: hoarding, self-neglect and
domestic squalor
Cases 3: An elderly couple was referred by their local
doctor. The initial assessment was performed at their
doctors clinic on the couples request. They were articulate, but their embarrassment about the state of their
house had prevented them from seeking help earlier
even though they had difficulty coping. Several meetings
took place at a fast food outlet before they eventually
agreed to allow ACAS assessors into their house. To complicate matters, the wife became unwell and was admitted into hospital.
The house had three bedrooms and each one was full
of hoarded possessions from floor to ceiling. The
hoarded material consisted of books, magazines, board
games, soft toys, sewing material and clothes. The front
door was completely barricaded and the kitchen and
shower could not be identified. They have not been able
to make a cup of tea, cook a meal or wash themselves in
their own house for 20 years. They have been eating at
fast-food outlets and sponging themselves in public
toilets. Both had diabetes, hypertension and obesity and
obstructive sleep apnoea. They did not fulfil the criteria
for Axis I psychiatric disorders and their neuropsychological assessments did not detect any dementia or cognitive impairment.
The husband repeatedly said that he could not understand how the hoarding situation became so extreme. He
was ashamed of the state of his home and agreed to
100

declutter the passageway, one bedroom, kitchen and

bathroom. He also understood that decluttering was necessary for service providers to provide home cleaning
services, meals and hygiene assistance. He was brought
up in an orphanage and did not wish to live in an aged
care facility. He signed a consent form before the intervention proceeded. He was on-site during the cleanup and his approval was sought before any item was
discarded.
However, on the second day of the intervention, he
became distressed and concerned about some missing
items. He accused cleaning staff of stealing and said he had
been raped. He wrote numerous complaint letters and
reported the matter to the police. A case manager from a
Community Aged Care Package was appointed for
ongoing management and she was able to continue the
cleaning process at a much slower rate that was more
acceptable to him. His wife was discharged home when
service providers were able to commence safely and they
were able to utilise living spaces that were not accessible
previously.
Underlying condition: nil noted
Predominant symptom: compulsive hoarding with
strong emotional attachment to his possessions. There
was secondary loss of use living areas required for selfcare resulting in squalor living arrangements and unconventional strategies for food and hygiene.
The three cases highlight the complexity and diversity
of patients who may have been labelled as having
Diogenes Syndrome. The first two cases had underlying
conditions, chronic schizophrenia and frontal lobe
dysfunction, respectively, that can be associated with
self-neglect, squalor and hoarding. In these instances,
the use of the term Diogenes syndrome would distract
from the fact that there were underlying conditions
that may explain their clinical situation and would be
deemed as inappropriate. In contrast, standard assessments of the couple in the third case failed to demonstrate cognitive impairment or any Axis I psychiatric
illnesses.
An advantage of using descriptive terms is to alert
service providers about the situation they would encounter. The degree of squalor and hoarding can be further
defined using instruments, such as the Environmental
Cleanliness and Clutter Scale12 and the Clutter Image
Rating.13 The management of such cases is complex with
high mortality rates for those who are hospitalised and
high recidivism rates in survivors.3 While these issues
may begin as problems for the individuals, they inevitably become problems for the community these individuals live in.
Until further studies into possible mechanism and
aetiology are available, it may be more practical for
2012 The Authors
Internal Medicine Journal 2012 Royal Australasian College of Physicians

clinicians to apply the terms self-neglect, domestic

squalor or hoarding to describe the situation at hand.
There are often several community agencies involved
with the management of these people. A clear under-

References
1 Snowdon J, Shah A, Halliday G. Severe
domestic squalor: a review. Int
Psychogeriatr 2007; 19: 3751.
2 Cybulska E, Rucinski J. Gross
self-neglect in old age. Br J Hosp Med
1986; 36: 215.
3 MacMillan D, Shaw P. Senile
breakdown in standards of personal and
environmental cleanliness. Br Med J
1966; 2: 10327.
4 Clark ANG, Mankikar GD, Gray I. Diogenes syndrome. A clinical study of gross
neglect in old age. Lancet 1975; 1: 3668.
5 Post F. Functional disorders I. In:
Levy R, Post F, eds. The Psychiatry of Late

standing of standardised terminology may lead to a

more considered approach by community stakeholders,
which hopefully will lead to a more successful
outcome.

Life. Oxford: Blackwell Scientific

Publications; 1982; 18081.
Shah AK. Senile Squalor Syndrome:
what to expect and how to treat it.
Geriatr Med 1990; 20: 26.
Snowdon J. Uncleanliness among
persons seen by community health
workers. Hosp Community Psychiatry 1987;
38: 4914.
Halliday G, Banerjee S, Philpot M,
MacDonald A. Community study of
people who live in squalor. Lancet 2000;
355: 88266.
Lauder W. The utility of self-care theory
as a theoretical basis for self-neglect.
J Adv Nurs 2001; 34: 54551.

10 Frost RO, Hartl TL. A

cognitive-behavioural model of
compulsive hoarding. Behav Res Ther
1996; 34: 34150.
11 McDermott S. The devil is in the details:
self-neglect in Australia. J Elder Abuse
Negl 2008; 20: 23150.
12 Halliday G, Snowdon J. The
Environmental Cleanliness and Clutter
Scale (ECCS). Int Psychogeriatr 2009; 21:
104150.
13 Frost RO, Steketee G, Tolin D,
Renaud S. Development and validation
of the clutter image rating. J Psychopathol
Behav Assess 2008; 30: 193203.

I M AG E S I N M E D I C I N E

Abnormal air collection on plain abdominal X-ray

imj_2633

A 69-year-old woman with type 2 diabetes mellitus and

hypertension presented to the emergency department
with progressive right flank and right lower abdominal
pain for 1 week. On examination, her bowel sounds were
decreased. The abdomen was tender to palpation in the
right lower quadrant, with no guarding or rebound. Right
costovertebral angle percussion tenderness was also
evident. Laboratory studies disclosed leukocytosis (41
109/L) in blood and pyuria (66 white blood cells per
high-power field) on urinalysis. A radiograph of the
abdomen revealed a large gas collection in the right
abdomen, which was initially interpreted as colonic gas
(Fig. 1). She was treated for acute pyelonephritis.
However, the abdominal pain worsened the next day.
Computed tomography of the abdomen was then performed, which disclosed emphysematous pyelonephritis
with a large, gas-containing, ruptured abscess (Fig. 2).
She underwent emergent percutaneous drainage of the
abscess. About 500 mL of thick pus with foul smelling gas
was drained. Cultures from blood, urine and pus all
yielded Klebsiella pneumoniae. After subsequent treatment
with parenteral ceftriaxone and continuous abscess
drainage through drainage tubes, she was eventually discharged in a good condition.
The initial clinical manifestations of emphysematous
pyelonephritis are not usually different from those of
2012 The Authors
Internal Medicine Journal 2012 Royal Australasian College of Physicians

101..108

Figure 1 Plain abdominal X-ray showing a large collection of gas in the

right side of the abdomen.

101

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