Dementia 1

Question 1

Define dementia

Answer 1

Dementia is a syndrome caused by a number of brain disorders which cause memory loss, decline in some other aspect of cognition, and difficulties with activities of daily living.

The symptoms fall into three groups:

1. Cognitive impairment: causing difficulties with memory, language, attention, thinking, orientation, calculation, and problem-solving.
2. Psychiatric or behavioural disturbances: changes in personality, emotional control, and social behaviour; depression, agitation, hallucinations, and delusions.
3. Difficulties with activities of daily living, such as driving, shopping, eating, and dressing

Deterioration must represent a progressive decline from a previous higher level of functioning, and consciousness should not be clouded (compare with acute confusional state or delirium).

Question 2

What is the typical memory loss pattern in dementia ?

Answer 2

Memory loss is typically for recent events and long-term memory can be remarkably intact.

Question 3

Define mild cognitive impairment

Answer 3

Is a decline in cognitive function greater than expected, taking account of the subject’s age and education, which is not interfering with activities of daily living. This is often clinically a pre-dementia state.

Question 4

Does dementia affect men or women more ?

Answer 4

In general it affects both equally but alzhimers affects women more commonly

Question 5

What are the 4 main types of dementia ?

Answer 5

1. Alzhimers (most common) dementia
2. Vascular dementia
3. Dementia with lewy bodies (DLB)
4. Frontotemporal dementia

Note you can get mixed patterns of dementia e.g. vascular and alzhimers often patients can be co-morbid for these

You can also get dementia in parkinsons (very similar to DLB, more explained later)

Question 6

How is the general diagnosis of dementia made ?

Answer 6

Dementia is a clinical diagnosis made when acquired cognitive deficits in more than one area of cognition interfere with activities of daily living and represent a decline from a previously higher level of functioning.

A detailed history - if history suggests cognitive impairment then screening MMSE or MoCA test should be used

In individuals with suspected cognitive impairment, the MMSE should be used in the diagnosis of dementia. Initial cognitive testing can be improved by the use of Addenbrooke’s Cognitive Examination as well

Question 7

Describe the different results someone can get from the MMSE

Answer 7

It is scored out of 30

• Score of >27 rules out dementia
• Score of <24 supports dementia

A score of 20 to 24 suggests mild dementia

13 to 20 suggests moderate dementia

< 12 indicates severe dementia.

The test assesses Orientation, Memory, Visuospatial and Language

Question 8

Describe the Addenbrookes Cognitive Examination III and the results which can be obtained for it in the investigation for dementia

Answer 8

Scored out of 100 with 5 domains;

1. Orientation and attention
2. Memory
3. Fluency
4. Language
5. Visuospatial functioning

• Score > 88 excludes dementia
• Score < 83 supports dementia

Question 9

What is the diagnostic criteria (elicited from history) and supported by cognitive impairments tests is used to diagnose dementia ?

Answer 9

There are cognitive or behavioural symptoms which:

1. Affect ability to function in normal activities.
2. Represent a decline from a previous level of function.
3. Cannot be explained by delirium or other major psychiatric disorder e.g. depression
4. Have been established by history-taking from patient and informant, and formal cognitive assessment.

Involve impairment of at least two of the following domains:

1. Ability to acquire and remember new information
2. Judgement, ability to reason or handle complex tasks
3. Visuospatial ability
4. Language functions
5. Personality and behaviour

Question 10

After a diagnosis of dementia or if diagnosis in doubt has been made what can be done to differentiate between the different subtypes of dementia ?

Answer 10

• Imaging - CT, MRI and SPECT scans can all be used to aid diagnose and help differentiate subtype
• Neuropsychological testing should be used in the diagnosis of dementia, especially in patients where dementia is not clinically obvious.

Question 11

What are some of the reversible causes of cognitive impairment which should be assessed before diagnosing with dementia ?

Answer 11

• Delirium
• Alcohol
• Medication
• Depression
• B12 or folate
• Thyroid dysfunction

Question 12

What tests can be used in the assessment of other possible causes of cognitive impairment (i.e. the reversible causes)

Answer 12

• FBC, ESR or CRP, MSU, U&E, LFT, glucose, Ca2+, TFT, B12 and folate
• 4AT screening test for delirium

Question 13

Describe the symptoms of alzhimers dementia

Answer 13

Symptoms in early stages include:

• Memory lapses - particularly short-term memory
• Forgetting names of people and places
• Difficulty finding words for things
• Inability to remember recent events
• Forgetting appointments

As the disease progresses, symptoms include:

• Difficulties with language
• Apraxia - difficulty with the motor planning to perform tasks or movements
• Problems with planning and decision making
• Confusion

In the later stages, symptoms include:

• Wandering, disorientation
• Apathy - lack of interest, enthusiasm or concern
• Psychiatric symptoms - depression, hallucinations, delusions
• Behavioural problems - disinhibition, aggression, agitation
• Altered eating habits
• Incontinence

Question 14

What are the main risk factors for vascular dementia (look out for these in a question)

Answer 14

• History of stroke or transient ischaemic attack (TIA).
• Atrial fibrillation.
• Hypertension.
• Diabetes mellitus.
• Hyperlipidaemia.
• Smoking.
• Obesity.
• Coronary heart disease.
• Family history of stroke or cardiovascular disease

Question 15

Describe the characteristic progression of vascular dementia

Answer 15

VaD is a progressive disease where deteriorations may be sudden or gradual but tend to progress in a stepwise manner.

Question 16

What are the typical presenting features which may suggest vascular dementia ?

Answer 16

• Focal neurological abnormalities: visual disturbances (eg, field defects), sensory or motor symptoms (eg, dysphasia, hemiparesis, visual field defects) or extrapyramidal signs (eg, dystonias (sustained or repetitive muscle contractions result in twisting and repetitive movements) and Parkinsonian features).
• Difficulty with attention and concentration
• Seizures
• Memory disturbance
• Gait disturbance
• Speech disturbance
• Emotional disturbance

Question 17

Describe the typical presentation of dementia with lewy bodies

Answer 17

• Dementia is usually the presenting feature, with memory loss, decline in problem solving ability and spatial awareness difficulties.
• Characteristically there are fluctuating levels of awareness and attention - delirium like
• Signs of mild Parkinsonism (tremor, rigidity, poverty of facial expression, festinating gait). Falls frequently occur. (parkinsonism features CANNOT be present > 1yr before dementia symptoms)
• Visual hallucinations - often complex and well described
• Sleep disorders including rapid eye movement sleep disorder (this is where paralysis in REM sleep is incomplete or absent allowing the person to ‘act out’ his or her dreams, typically acting out dreams that are vivid, intense and violent), restless legs syndrome, nocturnal cramps.
• Fainting spells.

Question 18

What is the characteristic histological feature seen in people with dementia with lewy bodies ?

Answer 18

Alpha-synuclein proteins (Lewy bodies) in the brainstem and neocortex.

Question 19

What are the 3 main clinical syndromes of frontotemporal dementia ?

Answer 19

1. Behavioural variant frontotemporal dementia.
2. Progressive non-fluent aphasia.
3. Semantic dementia.

Question 20

Describe the pathophysiology of frontotemporal dementia and ==> the 3 subtype classifications of frontotemporal dementia

Answer 20

There is extreme atrophy of the frontal and temporal lobes.

There are protein inclusions in neurons and glial cells

The histological classification of FTLD into three different groups of diseases is based on the protein inclusions as follows:

FTLD-TAU. Cellular inclusions contain the protein tau. Includes:

• Corticobasal degeneration (CBD).
• Classic PiD. Pick bodies (tau-positive spherical cytoplasmic inclusions) and Pick cells (ballooned neurons) are typically seen.
• Progressive supranuclear palsy (PSP).

FTLD-TDP. Cellular inclusions contain trans-active-response (TAR) DNA-binding protein 43 (TDP-43).

FTLD-FUS. Cellular inclusions contain fused-in sarcoma (FUS) protein.

Question 21

Describe the typical presenting features of Behavioural variant frontotemporal dementia.

Answer 21

• Loss of inhibition.
• Inappropriate social behaviour.
• Loss of motivation but without depression.
• Loss of empathy and sympathy.
• Change in preferences.
• Repetitive or compulsive behaviours, rituals.
• Loss of control over eating or drinking.
• Difficulties with planning, organisation or decision making.
• Memory and visuospatial skills usually preserved in early stages. Cognitive deficit less apparent than behavioural changes.
• Lack of insight.
• Loss of awareness of personal hygiene, and incontinence as the disease progresses

Question 22

What are the typical features of semantic frontotemporal dementia ?

Answer 22

• Loss of vocabulary with fluency of speech maintained.
• Asking the meaning of familiar words.
• Difficulty finding the right word and having to talk around it or describe it.
• Loss of recognition of familiar faces or objects.
• Memory and visuospatial skills comparatively well preserved

Question 23

What are the typical features of progressive non-fluent aphasia frontotemporal dementia ?

Answer 23

• Slow, hesitant, difficult speech. (fluency of speech not maintained)
• Grammatical errors in speech.
• Impaired understanding of complex sentences, although recognition of individual words preserved.
• Loss of literacy skills
• There may be impairment of orofacial movements such as swallowing, coughing or yawning on command (although still present as a reflex).
• There may be stuttering, impairment of ability to write or read, or impaired repetition ability.

Question 24

Describe the general management of dementia

Answer 24

• Advance planning should be encouraged while patients have the capacity to decide about future needs. - It should include practical and legal advice (i.e Powers of Attorney, driving). (if patient already does not have capacity then follow mental capacity act)
• Postdiagnostic counselling might be required.
• As disease progresses & behavioural and psychological symptoms progress institutional care might be required
• Treatment of dementia is a MDT process
• The management of carers is integral to good care of a person with dementia. Psychosocial interventions have the capacity to reduce carer distress and delay nursing home admission

Question 25

What are some of the non-pharmacological therapies offered to patients with dementia ?

Answer 25

• Cognitive stimulation programmes
• Multisensory stimulation
• Music therapy
• Art therapy
• Dancing
• Massage
• Aromatherapy
• Structured exercise programmes
• Animal-assisted therapy

Question 26

What are the laws on driving and dementia ?

Answer 26

People who have been diagnosed with dementia are legally obliged to inform the DVLA

For HGV or passenger carrying vehicles, a licence would be revoked at this point, but some people may be able to continue driving an ordinary car, subject to medical reports or assessment, and annual review.

Question 27

What is the classic pathological feature seen in lewy body dementia (hint it is the exact same as in parkinsons disease)

Answer 27

Degeneration of the substantia nigra