Case Study 3

Reason for Clinical Encounter

Karen was referred to the Neurological Rehabilitation Outreach Team (NROT) at Doncaster after
experiencing cognitive and physical deterioration due to her diagnosis of Huntingdon's disease (HD).
According to the referral sent by Karen's GP and Social Services, it has been suggested that Karen
was no longer capable of taking care of herself. There was an increased risk of Karen neglecting
herself and being at risk of harm, such as increased risk of falls, malnutrition, and other related
issues. The referral noted that Karen's cognitive and physical difficulties may be exacerbated by her
consumption of large amounts of alcohol.

The NROT Team provides an integrated rehabilitation for adults in their homes and outpatient clinics
with a neurological diagnosis in the Doncaster area. The team is made up of a multi-disciplinary
team. The aim is to provide a seamless service across the rehabilitation pathway.

Background to the Case

A review of the notes suggests that Karen was initially referred to neurorehabilitation services in July
2010. The referral was triggered due to issues regarding excessive alcohol consumption, difficulties
managing her finances, and falls. The notes suggest that Karen was referred onto alcohol
rehabilitation service. However, no further support was put in place, due to her lack of insight into
the difficulties her alcohol misuse caused her she declined input from that service.

Karen was referred to neuropsychology in neurorehabilitation services again, and she was reviewed
by the consultant neuropsychologist in 2016. It was noted that she presented with some moderate
cognitive and memory deficits consistent with her HD diagnosis. However, she was still able to self-
care and showed no signs of self-neglect in her personal hygiene.

In 2019, Karen’s cousin Mary visited Karen at the time of her sister’s funeral. The house was in an
awful state, with faeces on the floor; the kitchen was unkempt, and there were empty bottles of
alcohol (brandy) around the house. Mary, concerned for her cousin, contacted Karen’s GP, who
suggested a call to Social Services. Karen was re-referred to and assessed by the Outreach team.

The Social Services team for the adult eastern region intervened and set up a system of four daily
calls to support her. However, Karen persuaded the team that she only required one daily call, so the
frequency was reduced. She had also expressed her willingness to consider Extra Care housing, but
during a follow-up visit, she rejected the option.

Issues around finances started to emerge. There were incidents where Karen accused her support
workers of stealing money and taking her bank card. She had also accused her neighbours of
stealing money from her home. These incidents were reported to the police. Mary visited Karen
and found numerous sums of cash stashed around her home. She also found her bank book and card
by the reclining chair. The investigation was closed due to a lack of evidence.

Mary also found money hidden in various places around her house. This totalled approximately
£2500. Mary suggested that Karen should go and stay with her in Leeds. However, during the
weekend visit, Karen became agitated and accused Mary of stealing £35,000. Mary tried to support
Karen and rally support from the family, but this agitated Karen further. Mary eventually rang the
police and reported the incident to them. Mary felt unsafe supporting Karen, so she was driven back
to her home address in Doncaster after a few days. The police investigation revealed that no money
had been stolen.

In 2019, upon returning back home to live in Doncaster, she was reviewed again by the NROT team.
Additional carer calls were placed back in to support Karen. However, the carers withdrew after a
few days due to Karen’s aggressive behaviour towards them and accusing them of stealing her
money. Due to the frequency of these allegations and increased aggressive behaviour, Karen’s
capacity to manage her property and finances was assessed by a Neuropsychologist. A lasting power
of attorney was subsequently drawn up. Karen agreed that she needed assistance to manage her
property, and household bills and ensure she was on the best tariff for her usage. However, she was
assessed to still retain access to her money.

During the recent referral, Clare, the trustee for property and finances, was made aware that Karen
had attempted to withdraw money from the bank. However, she had used her “stolen bankbook,”
and the branch revoked her access. Prior to this, Clare had also recorded concerns regarding large
cash withdrawals. Clare reported that when she had tried to review and put in a suitable budget to
support Karen with her finances. This often resulted in Karen taking more money out of her account.

Clare reported that Karen would often spend approximately £130 at Tesco once a week from her
weekly budget of around £200. According to reports from the carers, the money was spent on
Brandy, as empty bottles were regularly found. Karen's food was delivered and provided by Wiltshire
Farm Foods. The carers also reported that Karen was not eating regularly and they had discovered a
foul smell emanating from her fridge, which was not working and was full of food.

A multi-agency team meeting involving Karen’s GP, Social services and the MDT from NROT revealed
that there had been multiple safeguarding alerts raised from a variety of sources. These have
included the ambulance service, the police and concerns raised by people in the local community.
There had also been numerous Hospital admissions as a result of falls.

Details from her previous inpatient admission were found and presented (October 2022). Karen was
admitted following a fall. She was found to be intoxicated at the scene, and she was transferred to
the hospital under the Mental Capacity Act. During her admission, she remained sober, and after a
few weeks, her cognition improved to the point where she was assessed to have regained her
capacity, and she was subsequently discharged. However, upon arriving home, she blockaded the
door, obtained alcohol and refused to allow care staff to come and support her. This situation was
reported when the carers came to complete their call.

The frequency of Karen’s challenging behaviour, including both verbal and physical aggression to
staff who attempted to visit her, and her level of self-neglect increased. It was agreed that a mental
capacity assessment needed to be completed to explore if Karen had the mental capacity to
understand and consent to care and residency.

Rationale for the Work Undertaken

HD is a progressive neurodegenerative disorder. Symptoms usually begin around the fourth

decade of life and people typically live for 15 to 20 years after diagnosis. The condition is caused by
a single gene mutation of the cytosine-adenine-guanine (CAG) trinucleotide on chromosome 4
(Roos, 2010). HD is usually associated with changes in multiple brain regions. These mainly include
the fronto-subcortical tracts (Paulsen et al, 2010., Aylward et al, 2012) including the striatum
(caudate and putamen) and the basal ganglia.
HD is commonly referred to as a movement disorder due to the physical changes that manifest in
the people affected. These include rapid involuntary movements in the face, limbs and torso.
These are referred to as chorea movements. As well as motor slowing, and limb rigidity
(bradykinesia). The movement disorder is not the most debilitating aspect of the condition. The
cognitive and behavioural changes are the aspects that have the most significant effect on a
person’s quality of life (QoL) and independence (Hamilton et al, 2003, Marderet al 2000, Mayeux et
al 1986 Rothlind et al 1993).

A formal diagnosis of HD is usually made in the presence of unequivocal motor presentation.

However, cognitive and behavioural symptoms are often present prior to a formal motor diagnosis.
This is referred to as the prodromal stage of HD (prHD) (Paulsen, et al., 2010b). The prHD maybe
seen up to 15 years before the onset of the motor manifestation. This was an important factor to
remember and consider, as during the assessment of Karen, it was noted that she was in the early
stages of the condition.

A study completed by Duff et al., (2010a) identified 575 cases (40% of participants in the study) of
HD carriers who, before diagnosis, met the criteria for mild cognitive impairment (MCI) in at least
one cognitive domain. The cognitive domains that demonstrated the most significant decline
included processing speed, executive function, working memory, visuomotor control, and time
estimation and production (Zakzais, 1998., Paulsen et al 2013b). These deficits are usually seen in
people who experience changes in brain structures referred to above.

Executive functioning deficits include planning, attention and concentration, organisation and
sequencing and cognitive flexibility (Craufurd and Snowden, 2014; Lawrence et al., 1996; Watkin et
al., 2000). Memory deficits are reported both by people living with HD and by the people around
them. This deficit can also be easily identified on standard memory assessments (Craufurd and
Snowden, 2014). However, their deficits are unique as they tend to experience difficulties with
motor skill learning and the attainment of repetitive routines. These deficits are typical of striatal
function, and therefore, they do not present as amnesic, which is more associated with medial
temporal lobe atrophy. Therefore, people with HD benefit from prompts and cues to retain and
recall information following a delay.

Difficulties managing emotions have also been noted to take place before the symptoms of the
condition become more apparent. Moreover, it has also been noted that people with HD may
struggle on tests of social cognition/theory of mind (Craufurd and Snowden, 2014). However,
whether this is secondary to or independent of executive dysfunction is unclear.

Depression, apathy and irritability are the most common mood-related symptoms that are
presented in people living with HD (Craufurd and Snowden, 2014). Lack of motivation and drive are
early indicators and become more persistent as the condition progresses. In addition, irritability and
loss of temper control deteriorate during the initial stages of the disease. However, this decreases
during the later stages of the condition. It is thought that this is a result of apathy. Unfortunately,
when poor temper control and irritability become present; this usually results in tension within the
family and relationships become strained.

People with HD retain the ability and knowledge of how they would complete tasks. Therefore, they
can verbally describe the processes they would follow. However, in practice, their performance
becomes ineffective and error-prone. This is largely due to poor planning and organisation and
failure to self-monitor and make appropriate adjustments. An additional difficulty most people
experience is a loss of motivation and drive, which usually results in people with HD neglecting
domestic and occupational tasks. Therefore, they are likelier to stay in bed or sit and watch TV
without external prompts. Cognitive and behavioural changes also make it difficult for people with
HD to remain in paid employment.

Deficits within the domain of executive functioning impact functional abilities such as multitasking,
which can also impact simple tasks such as walking and talking.

The Mental Capacity act (2005) for England and Wales provides a legal framework concerned with
decision making. It applies to everyone over the age of 16 years. The Act provides a framework of
how capacity can be assessed and how someone can be deemed as lacking capacity to make specific
decisions. (Assessing mental capacity handbook). There are five principles of the act that will
mentioned but discussed in great detail as it is not the scope of this report. These include:

1. Presumption of capacity
2. Supported decision making
3. The rights to make “unwise” decision
4. The principle of best interests
5. Considering the least restrictive alternatives.

The MCA code of practice in 2005 laid out the “two stage” test of capacity. I am aware that there is
a draft MCA code of practice (2022) but I will not refer to this in this report. I will continue with the
2005 framework as this was used during this clinical encounter.

Stage one, the diagnostic test, requires the assessor to describe the impairment or disturbance in
the functioning or the mind or brain. As Karen has a diagnosis of HD, she met the criteria for the
MCA assessment to be completed. There was evidence of a disturbance in the mind and brain.

Stage two, the functional test, provides the framework to consider a person to lack capacity, if they
are unable to do any one of the following:

 Understand information relevant to the decision.

 Retain relevant information long enough to make a decision.
 Use or weigh up relevant information in the decision-making process.
 Communicate their decision.

The decisions we make can range from very simple everyday decisions to very complex decisions
that can have a significant effect on a person’s life. In this report, the focus on the latter as the
decision being made was regarding Karen’s capacity to consent to care and her residency.

However, central to being able to demonstrate a person has capacity is a range of cognitive abilities.
These include memory, attention and concentration, executive functioning, speed of information
processing, language, (comprehension and expression), visuo-spatial perception, general intelligence
and orientation. However, it is widely accepted that the first three cognitive domains have the
greatest influence on the ability to make decisions (Dreer et al., 2008; Dymek et al., 2001; Marson et
al., 1997) (Chap 10 Assessing MCA Book).
It was recognised that individuals with HD may experience cognitive impairments that can affect the
accuracy of information obtained during a clinical interview. To mitigate this, it was determined that
the assessment process should not rely solely on the clinical interview but also incorporate feedback
from the system around Karen and cognitive assessments.

Work Undertaken

Karen underwent several assessments over a six-month period. During the first assessment, it was
observed that Karen was unable to identify any negative aspects of staying at her current residence.
However, with repeated prompts and cues, she eventually acknowledged that she was unable to
access the upstairs area of her property and was also unable to take showers or baths. As a result,
she had to rely on sink washes. Additionally, it was noted during the assessment that Karen was not
able to maintain her property, and several empty bottles of brandy were found lying around the
house.

The Addenbrookes cognitive screen (ACE-III) was also completed during the visit.

Total score 48/100

Attention 12/18 6
Memory 11/26 15
Fluency 1/14 13
Language 15/26 9
Visuospatial 9/16 7

Impairments were noted within all cognitive domains suggesting significant cognitive impairment.
Scores below 88 and 82 out of 100 are suspicious of dementia. Further analysis of the subtest
scores suggested deficits were primarily within memory and executive functioning domains. The
results suggest profound difficulties with learning new information and word retrieval (fluency).

It was also noted that her language ability was not deemed low on the ACE III. However, it was noted
that she relied on heavily practised conversations during the clinical assessments. For example, she
deflected questions by talking about her past experiences, her career as a teacher, her marriage and
how this broke down, her current care package and being under trusteeship for her property and
finances.

A joint follow-up visit with a social worker following further allegations of money being stolen by the
support workers. Upon searching the house, £2230 was found and counted in front of the
individual. However, she believed that there was only £700 in total. The risks of keeping such a large
sum of money were discussed, but the individual did not seem to fully understand the potential
consequences. During the visit it was also observed that Karen had placed frozen food in the
refrigerator, assuming that the freezer was not functioning properly. Upon further investigation, it
was discovered that the freezer only required defrosting. However, Karen was not willing to accept
this conclusion.

During a subsequent visit, there were growing concerns about excessive alcohol consumption as the
number of brandy bottles found had increased. This heightened concerns about the impact on
Karen's cognitive and physical health. Additionally, during this visit, it was observed that one of the
fridges had stopped working, and the food inside had become rancid. Karen could not detect this,
even when it was pointed out to her. Support was requested from the financial attorney to assist
with finances and remove the fridge.

During a subsequent visit to Karen's house, a capacity assessment was conducted to determine if she
had the ability to choose her place of residence. Upon arrival, it was observed that Karen was still in
bed even though it was late morning. Her living space was found to be disorganised and unkempt,
with an unidentified liquid spillage in the kitchen that had been hastily covered up with blue paper
towels. Additionally, refuse was discovered in the middle of her living room, resulting in an
unpleasant odour that required immediate attention. All windows were promptly opened to
mitigate the odour. Sufficient time was given to Karen to awaken and prepare for the assessment.
Karen was determined to lack the capacity to make a decision as she was unable to retain all the
necessary information, despite it being written down. Additionally, she was unable to weigh up the
information presented to her. Karen did not feel that she needed any support despite the
environmental factors present during the assessment. The only reason she gave for wanting to stay
home was that she had lived there for a long time. When we informed Karen of the assessment
results, she became verbally and physically aggressive, which led us to leave the premises.

On the third visit Karen appeared brighter in mood and happy for the visit. Karen was able to
comprehend the reason for our visit. Moreover, she was able to recall that Gill, Assessor had visited
her earlier in the week and recalled that she would be leaving her home to stay in a care home for a
period of assessment. Karen was not happy with this and explained that she wanted to stay at her
home. There was no evidence that Karen had drunk alcohol the day before. Suggesting that she was
cognitively at her most able.

Attempts were made to continue with the assessment, however, she seemed agitated by this and
changed the conversation to her money being stolen by support workers. Time was taken to discuss
how the money had been found and had been accounted for. However, Karen reported that SWs
were moving her money to confuse her and make her look incapable of living independently.

Attempts were made to continue with the assessment; however, Karen deflected these questions
and changed the conversations to talk about her past (parents, previous marriages, etc). It was
noted that she repeated information with “stock phrases” to give the impression that she could live
independently as she had done in the past. Tried to discuss how she could be supported to stay in
her home, i.e., to have more than one call a day, to have additional financial support to manage her
finances, etc. However, she became agitated, annoyed and verbally aggressive at this point.

Again, she was reminded that the aim of the assessment was not just to highlight the difficulties she
has been experiencing but also to discuss if she understood how she would be able to overcome
these. Moreover, if she could demonstrate that she was able to weigh up the pros and cons, she
would be able to demonstrate that she had the capacity to make her decision about her care needs.

After conducting capacity assessments, it was found that Karen was lacking capacity when it came to
making decisions related to her health and welfare. A best interest meeting was organised by social
services, which included all members of Karen's MDT, her GP, and Mary. The best interest process
was followed, and all parties agreed that Karen should move into a care home. The decision was
made with the objective of mitigating the impact of alcohol on her cognitive abilities and to explore
her functional abilities. Moreover, it allowed sufficient time for her to receive medical treatment and
care in a stable and supported environment. It was agreed that her cognitive abilities and capacity
would subsequently re-evaluated.
After a month of staying at the care home, Karen's cognitive abilities were re-evaluated using the
ACE-III test. Although her overall score had improved, it still fell within the range that could indicate
the possibility of dementia.

Total score 71/100

Attention 15/18 3 83.3
Memory 17/26 9 65.4
Fluency 4/14 10 28.6
Language 20/26 6 76.9
Visuospatial 12/16 3 75

It was observed that the initial assessment had evaluated both the impact of alcohol and the
cognitive decline associated with HD. During Karen's stay at the care home, she did not have access
to any alcohol and was eating a balanced meal three times a day. Therefore, the assessment results
showed a decline in cognitive abilities associated with HD. As described earlier, these are primarily
in domain of memory and executive functioning. So were there underlying neurocognitive effects of
HD?

After another month, a further appointment was arranged to re-assess her capacity at the care
home with the Assessment Officer from Social Services. Karen looked physically well; her hair was
brushed, and her nails were done. Physically, she seemed much healthier; her legs were less swollen
from oedema, and it appeared that she was no longer retaining fluid. In addition to this, she also
appeared to be more cognitively aware, so it was agreed that the previous capacity assessment
would be repeated.

Karen reported that she was unhappy with her stay in the facility. She mentioned that she did not
like the other residents as they were noisy and disruptive. She also reported that she was unable to
sleep and found the meals to be cold. Additionally, on one occasion, she found that there was no
toilet paper in the bathroom when she needed it..

Karen did not have many positive things to say about staying at the care home. However, she
reported many benefits of moving back to her home, such as maintaining a sense of normality, being
able to cook and eat the food she enjoyed, not having to share a remote control, and being able to
watch people walk by and talk to her acquaintances in the local area. Karen was reminded that she
had not engaged with any of the stated tasks or activities for a number of years. However, she was
not able to accept this.

Karen was asked if she could share any potential downsides of moving back home. She was
reminded that identifying negatives would not prevent her from returning home. Instead, it would
indicate that she was aware of the potential drawbacks and has considered them in making an
informed decision. However, even with multiple prompts, she was not able to provide any
negatives, except for the fact that she could not access a shower and had to rely on cloth washes.
Additionally, she is unable to use the upstairs of her house and is not able to have a lift installed due
to the shape and structure of the staircase.

During the assessment Karen realised she was unable to provide rational responses to the questions.
She therefore switched to talk about the breakdown of her marriage and how this was emotionally
difficult for her and led her to drink alcohol in excess. She then also moved the conversation to not
being happy that she was being supported to manage her finances and her property. When directed
back to the MCA assessment, Karen became visibly agitated and annoyed and started to become
verbally and physically aggressive.

The clinical impression during the assessment was that Karen was finding it difficult to weigh up the
pros and cons of staying at her home versus living in a care home. She was only fixed on returning
home only and unable to review and assess how her physical health and general wellbeing had
changed positively since moving into the care home.

. Due to agitation, difficult to determine her capacity on this visit. Agreed that a repeat of the
cognitive screen would be completed to explore any changes in her presentation.

Cognitive screen completed by Assistant and found that impairments were within the domains of
memory and verbal fluency. Fits with impairments associated with HD.

A further MCA assessment appointment was booked and completed jointly with a Mental Capacity
assessor from social services. The capacity question was to explore whether Karen had capacity ot
understand and consent to her care and residency. At first, Karen appeared to agree that she had
experienced some difficulties that had resulted in her moving into a care home. However, she felt
these were unrelated to her decisions or actions. Therefore, time was taken to explore this in more
detail. The initial part of the assessment focused on the risks if she was discharged home. However,
Karen was not able to accept these. Therefore, to support Karen, the focus shifted to focus on
difficulties she had experienced that resulted in her moving into a care home for a period of
assessment.

Again, Karen was not accepting of these difficulties and suggested that she would be able to keep
herself safe if she was discharged back home. She was therefore asked how she would be able to
provide some suggestions on what she would change or put in place that would enable her to be
safe and reduce or remove the risks mentioned. However, she was unable to do this. She defaulted
to using stock phases and conversations about her past experiences and memories to avoid the
questions or provide any additional information.

Again, to support Karen, it was explained that the capacity assessment aimed to assess if she
understood and was aware of the difficulties. Moreover, that if she could explain how she could
overcome these challenges or accept that she could not overcome them but that she was aware of
the risks then she would be deemed to have capacity. However, she was unable to provide any
additional information.

Karen was deemed not to have capacity to make a decision about her discharge destination. Why?

Summary formulation and next step

Might be were worth doing a draft based on 5Ps and a biopsychosocial consideration of her case. We
could look at something more complex once you have done this.

Generally there’s a lot left to do but your getting there

Please look at my amendments change what you can. May develop the detail around the
assessment. If this is an MCA you need to say much more about the evidence that she lacks capacity.
The absence of alcohol at you reassessment highlights the need to explore links between the
neurocognitive factors and the way they affect her ability to make this decision.