2. Psych [2] Flashcards

1
Q

Describe 4 clinical features that PTSD may present with.

A

Hyperarousal; hypervigilance, sleep problems, irritability, difficulty concentrating, exaggerated startle response

Avoidance, of people and place related to event

Re-experience e.g. flashbacks, nightmares, intrusive memories

Emotional numbing

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2
Q

Complex PTSD is defined as PTSD but has 3 additional groups of symptoms. What are these?

A

Negative / unstable sense of self / self concept

Dysregulation of affect

Disturbance in relationships

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3
Q

What are the options for treatment of PTSD?

A

In mild cases, watching and waiting can be useful, with advice about sleep hygiene etc, and regular follow ups.

CBT

EMDR (eye movement desensitization and reprocessing); patient is asked to think about the trauma whilst attending to other sensory stimulus e.g. flashing lights

SSRI or venlafaxine can also be used

Severe / refractory; may be able to add in antipsychotic e.g. risperidone if severe hyperarousal / psychotic features and no response to other treatments.

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4
Q

Risk factors for PTSD can be split into person-factors and event factors. Give some of each.

A

Person:
Hx of previous trauma
Hx of previous mental health diagnosis
Lower socioeconomic status
Female
Younger age
Certain professions
Multiple other major stressors at time of event
Low social support
Refugees and asylum seekers

Event:
Increased severity of event
Longer duration of event/s e.g. years of child abuse or torture
Intentional > accidental
Physical injury included in traumatic event

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5
Q

What is the prognosis of PTSD?

A

50% self resolve in 3 months.

1/3 have moderate / severe symptoms lasting for years.

Can present straight after the event, but 15% may present years later.

Patients with PTSD are more likely to have other medical problems e.g. drug and alcohol abuse, GI symptoms, cardiorespiratory symptoms etc.

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6
Q

ASD involves impaired social interaction, social communication and behaviours. Give some clinical examples of how each of these areas may present in an individual with ASD.

A

Communication:
Delay / regression in language development.
Repetitive use of words or phrases.
Lack of eye contact / non-verbal communication.
Difficulty with imaginative play etc.

Interaction:
Cannot understand non-verbal social cues.
Avoids physical contact.
Doesn’t make eye contact.
Difficulty making friendships.

Behaviours:
Interest in objects / patterns / numbers over people.
Rigid routine and repetitive behaviours and anxiety and distress when this is disrupted.
Stimming / self stimulating e.g. hand flapping, rocking
Deep, intense interests.
Restricted food habits.

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7
Q

ASD is a highly heritable condition, with a heritability of around 80%. Substantial proportion of this risk arises from sporadic mutations, and the same genetic variants can increase risk of neurodevelopmental disorders. What other conditions are linked to the genes that have been linked to ASD?

A

Intellectual disability
Epilepsy
ADHD
Schizophrenia

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8
Q

3 areas that ASD affects:

A

Social communication

Social interaction

Behaviour

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9
Q

Describe briefly the heritability and risk factors for ADHD.

A

Highest heritability of all psychiatric disorders, ~80%.

First degree relative of someone with ADHD has 20% chance of having it also.

Prenatal, perinatal and postnatal environmental factors also increase risk: maternal smoking, alcohol and heroin use during pregnancy, very low birth weight, fetal hypoxia, perinatal birth injury, prolonged emotional neglect during infancy.

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10
Q

ADHD symptoms can be split into 2 groups, and individuals may display mainly one or the other, or both. What are the two groups and give examples.

A

Hyperactivity / Impulsivity:
Can’t sit still, fidgets, restless.
Talks excessively.
Difficulty taking turns.
Poor risk perception, reckless.

Inattention:
Difficulty focusing on tasks especially those that are mundane, doesn’t finish tasks, careless mistakes etc.

Easily distracted, seems like isn’t listening, frequently ‘daydreaming’.

Difficulty with organisation, e.g. day to day tasks, forgetful.

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11
Q

Give some differentials for attention deficit in adults.

A

ADHD
Secondary to substance use
Intellectual disability
Secondary to other psychiatric disorder e.g. schizophrenia, depression
Brain injury
Neurodegeneration e.g. depression

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12
Q

First line management of ADHD is always environmental modification and parenting skill support. Give some examples of these.

A

School age: seating plan arrangements, support of a teaching assistant.

Use of headphones to reduce distractions.

Regular movement breaks during tasks.

Reinforcing verbal instructions in writing.

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13
Q

In ADHD, if despite optimal environmental modifications the child is still experiencing significant impairment, medication can be offered. Outline the medication options and the order of which they may be tried.

A

Methylphenidate; CNS stimulant. Comes in short acting form of Ritalin and other modified release forms e.g. Concerta XL, Xaggitin XL etc. FIRST LINE IN CHILDREN.

Lisdexamfetamine 2nd line if 6 week trial of methyphenidate at adequate dose not working / side effects.

Atomoxetine and Guanfacine = 3rd line.

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14
Q

Describe when a diagnosis of ADHD should be considered.

A

At least 6 (5 in adults) symptoms of hyperactivity/impulsivity or inattention symptoms are present.
Present for >6 months and interfering with educational or occupational performance.
Symptoms started before age 12.
Present in 2 settings or more.
Cannot be explained by another disorder.

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15
Q

Name 3 drugs used to treat ADHD.

A

Methylphenidate
Lisdexamfetamine
Atomoxetine
Guanfacine

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16
Q

Describe the mechanism of lisdexamfetamine and prescribing notes.

A

It’s a pro-drug that is metabolised to dexamfetamine by an enzyme in red blood cells. This limits the rate at which dexamfetamine is generated, limiting its potential for abuse.

However, some patients benefit from the faster on-off times that dexamfetamine straight provides. Can try this as a second option after trial of lisdexamfetamine.

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17
Q

What associations with methylphenidate might a parent come to you with concerns about, and how is this monitored?

A

Growth suppression with prolonged use.

Regular weight and height monitoring is always indicated, every 6 months, and drug holidays can be used to allow for catch-up growth.

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18
Q

What is the MOA of methylphenidate? And give some side effects.

A

CNS stimulant; primarily acts as a dopamine and noradrenaline reuptake inhibitor.

Growth suppression, abdo pain, nausea, dyspepsia.

Stimulants are sympathomimetic, and can potentially suppress appetite.

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19
Q

What investigation must be done prior to starting ADHD medication and why?

A

Baseline ECG, as all of these drugs are potentially cardiotoxic.

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20
Q

What is recommended as first line treatment for bulimia nervosa, and what does it involve?

A

BN-GSH; bulimia nervosa guided self help.
Helps to:
Understand their eating disorder
Develop coping strategies for binge-purge cycles
Develop healthier attitudes towards food and body image
Monitor their own symptoms

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21
Q

When should CBT be considered for bulimia nervosa?

A

If BN-GSH has been trialled for 4 weeks with no improvement.

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22
Q

Bulimia nervosa is an eating disorder characterised by:

A

Binge eating episodes followed by purgative behaviours including intentional vomiting, use of laxative / diuretics and excessive exercise.

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23
Q

Describe the DSM-5’s diagnostic criteria for bulimia nervosa (6 bullet points).

A

Recurrent episodes of binge eating.

Lack of control during episodes.

Recurrent inappropriate compensatory mechanisms to prevent weight gain.

Episodes occur at least once a week for 3 months.

Self evaluation is unduly influenced by body shape.

Does not occur exclusively during periods of anorexia nervosa.

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24
Q

Give some complications of anorexia nervosa.

A

Cardiac arrhythmias due to electrolyte imbalance, hypotension, bradycardia.

Osteoporosis due to chronic malnutrition, amenorrhoea.

Gastroparesis and constipation.

Anaemia and leukopenia.

Lanugo hair growth and xerosis (dry skin).

Death due to multiorgan failure.

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25
Give 3 points described in the DSM-5 for the diagnosis of anorexia nervosa (3).
1. Restriction of eating leading to significantly low body weight in context of age, sex, developmental stage etc. 2. Intense fear of gaining weight. 3. Disturbance in body perception, unaware of own body weight or shape, undue weight on body image when considering sense of self.
26
What management options are recommended by NICE for a) adults b) children and teenagers for anorexia nervosa?
Adults: Eating disorder focused CBT MANTRA; Maudsley Anorexia Nervosa Treatment for Adults SSCM; supportive specialist clinical management These are all long term, 20-40 sessions, focusing on regaining weight, involving carers, helping to found healthy eating behaviours, Children: anorexia based family therapy AN is the most common cause for admission to psychiatric wards in child and adolescent services. Up to 10% of patients will die because of the disorder. Medical risk / psychiatric risk e.g. suicide may require admission to medical unit or psychiatric unit respectively.
27
Outline the 'cycle' observed in obsessive compulsive disorder.
1. Obsession 2. Anxiety 3. Compulsion 4. Temporary relief
28
Some milder OCD cases can be managed with education and self help resources. What options are available for more severe cases?
CBT and ERP (exposure and response prevention) SSRI Clomipramine (TCA)
29
What does a Section 2 order of the Mental Health Act involve?
Compulsory admission for assessment, following a MHA assessment. Lasts up to 28 days. Cannot be renewed, can only end in discharge or by putting a Section 3 order in place.
30
What does a Section 3 order of the Mental Health Act involve?
Compulsory admission for treatment. Lasts up to 6 months, but the responsible clinician can arrange to extend if required. Detention under Section 3 requires a MHA assessment, can be from a Section 2 admission to a Section 3, or can be admitted from the community if the patient is well known to mental health services.
31
What does a Section 4 order of the Mental Health Act involve?
Detention for 72 hours when necessary / urgent scenario and where other processes do not have time to be put in place. It requires a AMHP and only one doctor. It is followed by a MHA assessment. Often changed to a Section 2 on arrival to hospital.
32
What does Section 136 of the Mental Health Act involve?
Used by the police to remove a person that appears to have a mental health disorder from a public space to a place of safety to allow a MHA assessment to be carried out. Lasts up to 24 hours.
33
What do Section 5(2) and 5(4) orders of the MHA refer to respectively?
5(2); patient already in hospital voluntarily, used in an emergency to detain patients. Requires only 1 doctor, lasts 72 hours. 5(4); patient already in hospital voluntarily, used in an emergency to detain patients. Requires only 1 nurse to hold the patient for up to 6 hours. Both are followed by a MHA assessment.
34
What does Section 135 of the Mental Health Act involve?
Allows the police to break into a home to remove the person at risk to a place of safety for assessment.
35
What does Section 17a of the Mental Health Act refer to?
Also known as CTO / Supervised Community Treatment. Allows recall of the patient from the community to the hospital for treatment if not complying with the conditions of the order within the community e.g. taking medication.
36
How should an SSRI be stopped?
Gradually reduced over a 4 week period (not necessary with fluoxetine)
37
SSRIs in pregnancy:
Weight up benefit and risk First trimester small increased risk of congenital heart defects Third trimester can result in PPHN Paroxetine has increased risk of congenital malformations esp in first trimester
38
Which two types of drugs if coprescribed with SSRIs can cause serotonin syndrome?
Triptans MAOIs
39
Describe the review method of a patient who has just been started on SSRIs.
Reviewed by a doctor after 2 weeks. If <25 or high risk of suicide then 1 week. Continue on treatment 6 months after secession of symptoms as this reduces risk of relapse.
40
How do MAOIs work and give 2 examples that are used in Parkinson's.
Block action of monoamine oxidase-B enzymes, helping to increase levels of circulating dopamine (the B enzymes are more specific to dopamine, other monoamine oxidases breakdown serotonin and adrenaline as well). Selegiline and Rasagiline
41
Give 5 indications for MAOI use.
Atypical depression (hypersomnia, overeating and anxiety) Anxiety disorders Parkinson's disaese Migraine prophylaxis Tuberculosis
42
The combination of MAOIs and ?-rich foods can precipitate ?
Tyramine rich foods can precipitate a hypertensive crisis in combination with MAOIs. E.g. mature cheese, yeast extract, broad beans, soya beans, pickled / smoked fish Throbbing headache is an early warning sign of a hypertensive crisis; check BP of anyone on an MAOI who feels unwell.
43
Give some examples of drugs that may precipitate a hypertensive crisis if taken alongside MAOIs.
Adrenaline / noradrenaline Amphetamines Cocaine l-DOPA , dopamine Ephedrine (decongestant) Local anaesthetic with adrenaline
44
Give 3 contraindications to a MAOI.
Mania Phaeochromocytoma Cerebrovascular disease
45
As well as a hypertensive crisis, what other 2 side effects are important to be aware of in patients taking an MAOI?
Anticholinergic side effects Postural hypotension
46
Order the following drugs in terms of duration of action (short, medium, long): chlordiazepoxide, midazolam, nitrazepam, diazepam, lorazepam.
SHORT Midazolam Lorazepam MEDIUM Nitrazepam LONG Chlordiazepoxide Diazepam
47
What is the mechanism of action of benzodiazepines?
Potentiates GABA receptors, which is the main inhibitory neurotransmitter, increases frequency of chloride channels. Allows more chloride ions into the cell which hyperpolarises the postsynaptic membrane and reduces neuronal excitability.
48
Discuss some indications for benzodiazepines (5 minimum).
Insomnia (short acting, short term only) Anxiety (short term only) Alcohol withdrawal (chlordiazepoxide) Akathisia Acute psychosis / mania (for sedation) + epilepsy prophylaxis, seizures, muscle spasm and anaesthetic premedication
49
Discuss important side effects of benzodiazepines and when to be cautious in prescribing.
Dependence risk is high (especially in prolonged use and shorter acting drugs) Drowsiness, ataxia and reduced motor coordination (warn about driving / operating heavy machinery) Caution in older adults; drowsiness / confusion can precipitate falls or delirium Chronic respiratory disease caution; may precipitate respiratory depression
50
Benzodiazepines can be fatal in overdose but only usually when taken in combination with other sedatives. What drug is available to reverse the effect of benzodiazepines and what should you be cautious of?
Flumazenil Can lower seizure threshold and cause acute benzodiazepine withdrawal especially with history of chronic use. Should only be done in hospital settings.
51
Discuss the differences in pharmacodynamics between chlordiazepoxide and diazepam.
Both are long acting benzos. Time to peak effect is significantly shorter for chlordiazepoxide, so it acts faster. Both half lives are 100h. Both can be taken orally, but only diazepam can be PR, IV, (IM last line).
52
Compare 2 short-acting benzodiazpines.
Midazolam and Lorazepam. Mid has onset of 5-10 mins whilst lorazepam takes 1-4 hours. Half lives are 2 and 15 hours respectively.
53
If patients withdraw too quickly from benzos they may experience benzo withdrawal symptoms, which may occur up to 3 weeks after stopping a long acting drug. Give some features.
Insomnia Irritability Anxiety Tremor Loss of appetite Tinnitus Perspiration Perceptual disturbances Seizures
54
Barbiturates also act on the GABA neurotransmitter; what are their MOA?
Barbiturates increase the duration of chloride channel opening. Compared to benzos, where the frequency of chloride channels is increased.
55
It's important to look for a potential physical cause when considering a psychiatric anxiety diagnosis. Give some conditions and medications that may trigger a secondary anxiety.
Hyperthyroidism Cardiac disease Phaeochromocytoma Cushing's Salbutamol Theophylline Caffeine Steroids Antidepressants Alcohol
56
Physical symptoms of anxiety are caused by overactivity of the sympathetic nervous system. Give 6 physical symptoms of anxiety, and a medication that is often used to try and combat these.
57
Describe some emotional / cognitive symptoms of GAD.
Excessive worrying and being unable to control the worrying. Restlessness and difficulty relaxing. Easily tired. Difficulty concentratin.
58
What tool can be used to assess severity of GAD, and what do the scores indicate?
GAD-7 (GAD Questionnaire) 5-9 = mild anxiety 10-14 = moderate anxiety 15-21 = severe anxiety Mild can be managed with active monitoring, self help strategies, sleep, diet, exercise and alcohol / caffeine / drug abstinence. Mod-severe requires CBT and or medication.
59
What is first line for GAD that requires drug treatment?
SSRI If unsuccessful, try a different SSRI or SNRI. 3rd line may be pregabalin. Warn of risk of suicidal thoughts and self harm. Weekly follow up required.
60
Give the definition of a phobia.
Extreme fear of certain situations or things, causing symptoms of anxiety or panic. . Acrophobia = fear of heights Glossophobia = fear of public speaking Trypanophobia = fear of needles Graded exposure therapy can be effective.
61
Give 3 features of CBT.
Time limited 12-24 sessions. Goal orientated, focuses on present problems and not how they developed or unconscious factors. Collaborate therapeutic relationship.
62
Discuss general differences between counselling, psychodynamic psychotherapy and CBT.
Counselling = unstructured, allowing patients to generate own solutions to problems. Psychodynamic psychotherapy = aims to allow patient to recognise unconscious processes in themselves that are causing problematic symptoms. CBT = aims to help patient to identify and change the links between how they think, feel, sense and behave. Often tackles cognitive distortions. SEE NOTION TABLE FOR DIFFERENT TYPES OF THERAPY AND THEIR INDICATIONS.
63
Discuss epidemiological differences between EUPD and APD.
EUPD Younger age and females Link to childhood sexual abuse 40x increase in suicide rate Comorbid = depression, substance abuse, bulimia and anxiety APD 25-44 year old MEN School dropout, conduct disorders Very high prevalence in prisons Comorbidity = SUBSTANCE ABUSE
64
Which psychiatric illness has it's risk multiplied 2 fold during pregnancy?
OCD
65
Describe the classical time frame of 'baby blues' and the associated symptoms.
Within first 10 days post delivery, peak symptoms at day 3-5, and should resolve in 2 weeks. Teary, mild depression and emotional lability, anxiety, irritability.
66
Describe the typical onset and end period of postnatal depression.
Within 3 months of delivery, with peak time to onset being 3-4 weeks. Not considered postnatal if >1 year post delivery onset. 1/3 have symptoms antenatally. 10-15% are affected in the West. Episodes resolve within 3-6 months usually, but 20% are still depressed at 1 year post delivery.
67
What feature of postnatal depression requires urgent psychiatric assessment, and which version is the most worrying?
Infanticidal thoughts These are often distressing (ego-dystonic), which is more reassuring. If they are reported to not distress the mother, and may involve active planning (ego-syntonic), this is worrying.
68
Discuss considerations in treating postnatal depression.
Relationship counselling, health visitors, mother and baby groups are important and useful in mild. Infant's wellbeing and emotional development is important to consider too. Most antidepressants are transmitted in breast milk in tiny quantities, and do not harm the child, but have a mentally ill parent can, so this must be considered.
69
The postpartum period is extremely high risk for the development of a psychotic episode. 25-50% risk of recurrence in future pregnancies. When is this most likely to occur, what would an episode typically look like and what must be done?
50% of cases experience onset of symptoms within postnatal days 1-3, most within 2 weeks of delivery. Insomnia, restlessness, fluctuating psychotic symptoms. Mood symptoms either end of the spectrum can be prominent. Most require admission - assessing for risk of infanticide and suicide is crucial. Mother and baby unit admission is always preferred.
70
Which phase of the menstrual cycle does PMDD present with symptoms?
Luteal phase, day 15-28 Mood and cognitive symptoms that are severe enough to cause functional impairment. Treatment includes contraception that prevents ovulation, CBT, SSRIs (continuous or days 15-28 only).
71
Can you breastfeed if on opiate substitute therapy?
Yes Breastfeeding when on opioid substitute therapy may actually reduce the intensity and length of neonatal abstinence syndrome and has been associated with improved outcomes.
72
Which SSRI should be avoided in breast feeding if possible, and which one is recommended by SIGN and why?
Fluoxetine due to a long half life (avoid) Paroxetine due to low milk / plasma ratio
73
What scale exists to screen for depression in postpartum women?
Edinburgh Postnatal Depression Scale 10 items, with a max score of 30. Score over 13 indicates 'depressive illness of varying severity'. Sensitivity and specificity is 90%.
74
List different types of dementia, with their % prevalence if you can.
Alzheimer's 62% Vascular (17%) Lewy body dementias (DLB 4%), PD 2%) Frontotemporal dementias (2%, BUT 20% of EOS) CJD / prion disease Huntington's
75
Describe key pathological changes in Alzheimer's dementia.
Beta-amyloid protein deposition between neurons. Neurofibrillary tangles of hyperphosphorylated tau proteins inside neurons (impaired function of tau which is meant to stabilise microtubules and promote tubulin assembly).
76
Which drugs are indicated first line for Alzheimer's disease and describe the MOA. What is used second line?
Cholinesterase inhibitors; these work as there is cholinergic neuron degeneration in the nucleus basalis of Meynert, causing acetylcholine deficiency. These are used in mild-moderate dementia Donepezil Rivastigmine Galantamine Second line = NMDAr antagonist Memantine; for moderate to severe AD MOA is thought to be reducing excitotoxic damage by reducing influx of calcium.
77
Give a relative contraindication to the first line drug in Alzheimer's, and one common side effect.
Donepezil; Relatively contraindicated in bradycardia. Insomnia is a SE.
78
Discuss the genetic factors implicated in early-onset AD (3).
3 genes, Autosomal Dominant trait Chr 21; APP (amyloid precursor protein) Chr 14; Presenelin 1 Chr 1; Presenelin 2
79
Discuss the genetic factors implicated in late-onset AD (1).
ApoE 4 e4 allele - encodes cholesterol transport protein. 2 other risk factors include caucasian ethnicity and Down syndrome.
80
State some non-genetic risk factors for Alzheimer and vascular dementia.
Both: Hypertension Hypercholesterolaemia Smoking Diabetes Previous MI Obesity Late onset depression Air pollution Vascular: AF Stroke history Alzheimer: Low educational attainment Head injury
81
Which abnormal protein is associated with DLB and Parkinson's disease dementias?
Alpha synuclein, forms inclusion bodies
82
Which abnormal protein is associated with CJD and Huntington's respectively?
CJD = prion Huntington's = huntington protein
83
Describe the macroscopic and microscopic findings in CJD.
Macroscopic: Spongiform changes throughout cortex and the subcortical nuclei. Microscopic: Extracellular prions especially in the cerebellum
84
Describe the macroscopic and microscopic findings in Huntington's.
Macroscopic: Atrophy of basal ganglia and frontal lobes Microscopic: Intracellular aggregates of Huntington and ubiquitin.
85
What are Lewy bodies, and where are they likely to be seen in a) DLB and b) Parkinson Disease Dementia?
DLB: cortex. Atrophy of frontal, parietal and occipital lobes. PD: brainstem nuclei. Atrophy of substantia nigra.
86
Describe the 3 main subtypes of vascular dementia.
1. Stroke-related; multi or single infarct dementia. 2. Subcortical; small vessel disease. 3. Mixed; VD + Alzheimer's disease present.
87
Describe the classical presentation of vascular dementia.
Years or months of history of a sudden or stepwise deterioration of cognitive function. Symptoms and speed of deterioration vary but can include: Focal neurology e.g. visual disturbance, motor, sensory symptoms. Difficulty with attention and concentration Seizure Memory, gait, speech and emotional disturbance
88
What is CADASIL?
Cerebral AD arteriopathy with subcortical infarcts and leukencephalopathy. NOTCH 3 gene.
89
What disorder often precedes the onset of Lewy Body Dementia by several years?
REM sleep behaviour disorder.
90
DLB and Parkinson disease dementia are now viewed under the same umbrella of Lewy Body Dementias. They have the same pathogenesis, and as the diseases progress are very similar. How do we differentiate between DLB and Parkinson disease dementia?
If dementia occurs at the same time or within a year of onset of parkinsonism = DLB. If dementia occurs >1 year after established PD = Parkinson disease dementia.
91
Give 3 key features that would differentiate a Lewy body dementia over an Alzheimer's diagnosis.
Hallucinations Early parkinsonism Hx of REM sleep behaviour disorder
92
When is dementia classed as early onset?
<65
93
Which criteria does NICE recommend for diagnosis of vascular dementia, and describe it.
NINDS-AIREN 1. Presence of cognitive decline that interferes with activities of daily living NOT due to secondary effects of the cerebrovascular event; done by clinical examination and brain imaging. 2. Cerebrovascular disease, defined by neurological signs and or brain imaging (MRI). 3. Relationship between [1] and [2] inferred by onset of dementia within 3 MONTHS FOLLOWING RECOGNISED STROKE, abrupt deterioration in cognitive function and fluctuating, stepwise progression of deficits.
94
Give 4 common features of FTD.
<65 at age of onset Insidious onset Relatively preserved memory and visuospatial skills Personality change and social conduct disorder
95
State 3 recognised types of FTD and discuss the main features.
Pick's disease (postmortem dx?); PERSONALITY CHANGE and IMPAIRED SOCIAL CONDUCT. Knife blade atrophy and Pick bodies present. Chronic progressive aphasia (CPA); non fluent speech, comprehension is usually preserved. Semantic dementia; Recent memory is better than older memory, as opposed to AD. Fluent, progressive aphasia, speech is fluent but has little meaning.
96
What is the inheritance pattern of Huntington's, and what is the pathophysiology?
AD with complete penetrance. Excessive CAG repeats in huntington gene. Length is inversely correlated to age of onset of the disease. This abnormal protein causes neuronal death particularly in the basal ganglia.
97
How does CJD present, including what the common first symptom to arise is?
Rapidly progressive dementia over 6-8 months associated with cerebellar ataxia and myoclonic jerks. First symptoms are often visual, and so may present at TIA clinic or optician, due to occipital lobe involvement. EEG = sharp wave complexes MRI = hyperintense signals in the basal ganglia and thalamus.
98
Discuss the protein pathology in CJD.
Prion proteins induce amyloid folds and form beta pleated sheets that are resistant to proteases.
99
Most cases of CJD are sporadic, accounting for 85%. The average age of onset is 65. What is difference about the presentation and onset of new variant CJD?
Average age is 25. Psychological symptoms including anxiety, withdrawal and dysphonia (hoarse voice). Chr 20 implicated. Median survival is 13 months.
100
A prion is an infectious protein, and a number of diseases exist due to transmission of the protein. Kuru is an example of a known prion disease; how is this transmitted?
Prion transmission via cannibalism of neural tissue (highland tribes of New Guinea).
101
Why are benzos relatively contraindicated in most patients with dementia?
They are particularly vulnerable to the adverse effects of sedation, falls and delirium.
102
Which type of dementia can have a catastrophic reaction in 50% of patients to antipsychotic drugs, and what might this look like?
Dementia with Lewy bodies. Can precipitate potentially irreversible parkinsonism, impaired consciousness and severe autonomic symptoms. 2-3x increase in mortality.
103
Which type of dementia are acetylcholinesterase inhibitors indicated?
Alzheimer's No others, apart from if comorbid AD present.
104
Which virus can cause dementia, what changes might be seen on a CT and give 2 symptoms that might be present.
HIV; symptoms can be caused by the virus itself, due to direct damage to the brain, in addition to the neurocomplications of HIV infection. Subcortical (and also cortical) changes are seen on CT. Diffuse multifocal destruction of white matter and subcortical structures. Behavioural changes and motor impairment.
105
Using the PINCH ME mnemonic, write down 8 potential causes of delirium.
Pain Infection Nutrition Constipation Hydration Medication Environment
106
Discuss features of delirium.
Acute onset Impairment of conscious level, acute change from baseline Fluctuating course Abnormal attention Impaired cognition Hallucinations, visual Paranoid delusions Agitated Short term memory loss over long term
107
Discuss the contents of the 4AT assessment tool, and what the scores indicate.
Alertness AMT 4; name, DOB, location, current year Attention (months in reverse order) Acute change or fluctuating course
108
Give 5 predisposing factors for delirium.
Dementia / cognitive impairment Hip fracture Frailty / multimorbidity Polypharmacy
109
Delirium is a medical emergency unless prior ceiling-of-care discussions have taken place. How should it be managed initially, and what would indicate progression to pharmacological intervention?
Orientation e.g. clocks, calendars, photos Glasses, hearing aids Same nursing staff Family members present If patients condition is severely distressing them, or they are risk to themselves or others, or it is preventing them from getting important medical treatment then you can progress to pharmacological management.
110
Give options for pharmacological intervention for symptoms (not causes) of delirium.
Haloperidol low dose first line. Olanzapine if contraindicated / ineffective. Contraindications to haloperidol include prolonged QT and parkinsonism / dystonia. Avoid benzodiazepines, unless the underlying cause is alcohol withdrawal in which these would combat the underlying cause. Management is challenging in Parkinson's, as antipsychotics worsen symptoms; careful reduction in Parkinson's meds OR quetiapine / clozapine preferred if urgent treatment is required.
111
What is the average length of a delirium episode?
7 days
112
Give some factors that worsen the prognosis of a delirium.
Prolonged episode of delirium Pre-existing dementia / cognitive impairment Frailty / older age Hypoxic illness e.g. pneumonia Hypoactive delirium Visual impairment
113
Assessment tools for dementia in a non-specialist setting:
6CIT - 6 item cognitive impairment tool 10-CS - 10 point cognitive screener ?MMSE can be used for cognitive impairment, but not endorsed by NICE. Score <24/30 = possible dementia * If dementia is suspected in a patient with learning disabilities, refer for specialist assessment.
114
What assessment tool is often used in specialist memory services?
Addenbrooke's Cognitive Examination III
115
Which domains are tested in the ACEIII?
Attention Memory Language Visuospatial function Verbal fluency
116
A patient comes into the GP with their wife with a history of memory problems. The GP does a 6CIT, and thinks there is possible dementia. What bloods / investigations should be sent for before referral to the specialist memory clinic? What will be done there?
All the bloods! for a reversible cause. Including B12/Folate, FBC, U&E, lfts, crp esr , calcium, hba1c, MSU urine culture? CXR lung cancer? MRI brain and ACEIII in clinic
117
Modifiable risk factors for dementia:
Exercise Healthy weight Controlled BP Controlled sugars Cognitive stimulation e.g. work
118
What is the function of Wernicke's area?
Forms the speech before sending it to Broca's
119
What type of aphasia is Broca's?
Expressive
120
Is comprehension impaired or normal in Wernicke's aphasia?
Impaired
121
Is comprehension impaired or normal in Broca's aphasia?
Normal
122
Where is the lesion most likely to be in Wernicke's aphasia?
Superior temporal gyrus Inferior division of left MCA
123
Where is the lesion most likely to be in Broca's aphasia?
Inferior frontal gyrus Superior division of left MCA
124
Where is the lesion most likely to be in conduction aphasia?
Arcuate fasciculus Connection between Wernicke's and Broca's areas
125
Scores in a 6CIT:
0-7 normal 8-9 mild cognitive impairment, probably refer 10-28 = significant cognitive impairment, refer
126
Scores in a 10-CS:
8 or more = normal 6/7 = possible cognitive impairment 5 or under = probable cognitive impairment
127
Features of conduction aphasia:
Speech fluent Repetition poor, aware of mistakes
128
Features of global aphasia:
Large lesion affecting all 3 areas Severe receptive and expressive aphasia