List II - Less Common 'Know of' Conditions Flashcards

1
Q

What is Pick’s disease?

A
  • Type of fronto-temporal dementia - atrophy of frontal and temporal lobes
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2
Q

How can Pick’s disease be distinguished from Alzheimer’s disease?

A
  • Pick’s - not usually associated with memory loss in its early stages
  • Especially known for the aphasia it causes
  • Behaviour problems and personality changes are often a primary first symptom
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3
Q

What are the symptom’s of Pick’s disease?

A
  • Hesitant speech
  • Difficulty articulating
  • Stuttering
  • Ungrammatical speech
  • Difficulty recalling words or names
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4
Q

What are the behaviour problems and personality changes associated with Pick’s disease?

A
  • Unusual behaviour
  • Disinhibition
  • Passivity
  • Hyperactivity
  • Anxiety
  • Slovenly appearance
  • Delusions and paranoia
  • Lack of empathy
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5
Q

What is the underlying cause of Pick’s disease?

A
  • Caused by the build up of tau proteins, called ‘Pick bodies’ in the brain
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6
Q

What are the more common features of frontotemporal lobar dementias?

A
  • Onset before 65
  • Insidious onset
  • Relatively preserved memory and visuospatial skills
  • Personality change and social conduct problems
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7
Q

What is the appearance of Pick’s disease atrophy?

A
  • Focal gyral atrophy with a knife-blade appearance is characteristic of Pick’s disease
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8
Q

What are the macroscopic changes seen in Pick’s disease?

A
  • Atrophy of the frontal and temporal lobes
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9
Q

What are the microscopic changes seen in Pick’s disease?

A
  • Pick bodies - spherical aggregations of tau protein (silver-staining)
  • Gliosis
  • Neurofibrillary tangles
  • Senile plaques
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10
Q

What is the management of Pick’s disease?

A
  • NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia
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11
Q

What is Creutzfeldt-Jakob disease (CJD)?

A
  • Rapidly progressive neurological condition caused by prion proteins
  • These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases

Features

  • Dementia (rapid onset)
  • Myoclonus
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12
Q

What are the investigation findings in CJD?

A
  • CSF is usually normal
  • EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
  • MRI: hyperintense signals in the basal ganglia and thalamus
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13
Q

What are the features of sporadic CJD?

A
  • Acounts for 85% of cases
  • 10-15% of cases are familial
  • Mean age of onset is 65 years
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14
Q

What are the features of new variant CJD?

A
  • Younger patients (average age of onset = 25 years)
  • Psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features
  • ‘Prion protein’ is encoded on chromosome 20 - it’s role is not yet understood
  • Methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD - all patients who have so far died have had this
  • Median survival = 13 months
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15
Q

What other prion diseases exist?

A
  • Kuru
  • Fatal familial insomnia
  • Gerstmann Straussler-Scheinker disease
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16
Q

What is HIV related dementia?

A
  • Before HAART, people with HIV would go on to develop dementia, but this is now uncommon
  • However, many people who have HIV still experience a milder type of cognitive impairment such as problems with their memory and thinking - sometimes called HIV-associated neurocognitive disorder (HAND)
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17
Q

What problems does HAND cause for people with it?

A

People with HAND may experience difficulties with:

  • Concentration
  • Memory
  • Planning
  • Organising
  • Decision making

Symptoms usually stay stable over time rather than progressing to dementia - the person may also have problems with their mood such as depression or irritability

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18
Q

How should people with HIV be monitored in relation to HAND?

A
  • If a person has had a recent diagnosis of HIV they should have their cognitive and emotional wellbeing assessed within 3 months
  • Should then be re-assessed at least once a year
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19
Q

How is learning disability defined?

A
  • IQ <70
  • Loss of adaptive social functioning
  • Onset before the age of 18
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20
Q

What is NOT learning disability?

A
  • People who develop an impairment after the age of 18

* People who suffer brain injury in accidents after the age of 18

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21
Q

What is mild LD?

A
  • IQ 50-69 - language fair, reasonable level of independence
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22
Q

What is moderate LD?

A
  • IQ 35-49 - generally better receptive than expressive language
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23
Q

What is severe LD?

A
  • IQ 20-34 - increased sensory and motor deficits, 50% will have epilepsy
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24
Q

What is profound LD?

A
  • IQ <20 - increased need and vulnerability

* Developmental level about 12 months

25
Q

What are the possible causes of LD?

A
  • Pre-natal
  • Genetic or congenital e.g. Downs syndrome, fragile X
  • Maternal drug or alcohol use foetal alcohol syndrome
  • Perinatal/intrapartum
  • Oxygen deprivation during birth
  • Injury secondary to birth complications
  • Difficulties from premature birth
  • Post natal
  • Infection, injury, environmental conditions
  • Combination
26
Q

Name 3 common causes of LD?

A
  • Down’s syndrome
  • Foetal alcohol syndrome
  • Fragile X
  • Most common inherited cause
  • FMR1 gene mutation - CGG repeats >200
27
Q

Name other causes of LD?

A
  • Cerebral palsy
  • Austism
  • Epilepsy
  • Prader-Willi syndrome - IQ 50-85 which may include learning disability
  • Tuberous sclerosis - up to 50% have LD
28
Q

What are children with Downs syndrome at risk of having?

A
  • Abnormal internal organs - ASD, VSD, oesophageal atresia
  • Cataracts
  • Hypothyroidism
  • Chest infection
  • Transient leukaemia
  • Epilepsy
  • Dementia at an earlier age
29
Q

What is the leading preventable cause of LD?

A
  • Foetal alcohol syndrome - 25-30% of people with FAS have a learning disability
30
Q

What are the features of foetal alcohol syndrome?

A
  • Growth retardation
  • Characteristic facial features
  • CNS abnormalities/dysfunction
31
Q

What are the facial features of a child with foetal alcohol syndrome?

A
  • Low nasal bridge
  • Minor ear abnormalities
  • Indistinct philtrum
  • Micrognathia
  • Epicanthal folds
  • Short palpebral fissures
  • Flat midface and short nose
  • Thin upper lip
32
Q

How common is autism?

A
  • 1/100
33
Q

What is autism characterised by?

A

Difficulties with:

  • Social interaction
  • Verbal and non-verbal communication
  • Restricted and repetitive behaviours
  • 55% co-exists with LD
34
Q

What are the difficulties that can arise from a child with autism?

A
  • Sensory distortion
  • Perceptual distortion
  • Executive function difficulties
  • Central coherence difficulties
  • Inflexibility of thought and action
  • Social interaction difficulties
35
Q

How common is mental health problems in people with LD?

A
  • 24-40% of people with LD will develop mental health problem in their lifetime
    vs
  • 16-25% of total population will develop a mental health problem in their lifetime
36
Q

Why are people with down’s syndrome at greater risk of developing dementia?

A
  • APP gene is on chromosome 21 therefore x 3 = greater risk of developing dementia
37
Q

What is PTSD?

A
  • Post Traumatic Stress Disorder - experience of life threatening or catastrophic event likely to cause pervasive distress in almost anyone
  • Developing in the aftermath
  • Flashbacks, nightmares, intrusive memories
  • Avoidance of activities and situations associated with trauma
  • Hyperarousal/anxiety with hypervigilance and increased startle reaction i.e. jumpy
  • Numbness, emotional blunting, detachment from others
  • Associated depression and suicidal ideation
  • Onset usually 1-6 months
  • Fluctuating course but most recover
38
Q

Who are the high risk groups for PTSD?

A
  • Soldiers
  • Disaster victims
  • Rape victims
  • Torture victims
  • Assault victims
39
Q

What are the predisposing factors for PTSD?

A
  • Personality traits (compulsive, asthenic)
  • Previous history of neurotic illness
  • Genetic (oversensitive amygdala and hippocampus, decreased hippocampal size on MRI)
40
Q

What is the recommended treatment for PTSD according to NICE?

A
  • Trauma focused CBT
  • Repeated graded exposure
  • Testimony based techniques
  • Eye movement desensitisation and reprocessing (EMDR)
  • Antidepressant (paroxetine or mirtazepine)
  • 65% recover in 12/18
41
Q

What is the ICD10 for phobias?

A
  • Group of disorders in which anxiety is evoked in certain well defined situations that are not currently dangerous
  • Results in such situations characteristically avoided or endured with dread
  • Patient may be concerned with individual symptoms such as palpitations, feeling faint
  • Secondary fears may include fear of dying, losing control or going mad
  • Phobic anxiety and depression often co-exist
42
Q

What are the different types of phobias according to ICD10?

A
  • Agoraphobia
  • Social phobias
  • Specific (isolated) phobias
43
Q

What is the ICD10 for agoraphobia?

A
  • Fears of:
  • Leaving home
  • Entering shops
  • Crowds and public places
  • Travelling alone in trains, buses or planes
  • Panic disorder is a frequent feature
44
Q

What is the ICD10 for social phobias?

A
  • Fear of scrutiny by other people leading to avoidance of social situations
  • More pervasive social phobias are due to low self-esteem and fear of criticism
  • May present as a complaint of blushing, hand tremor, nausea, urgency of micturation
  • Symptoms may progress to panic attacks
45
Q

What is the ICD10 for specific (isolated) phobias?

A
  • Restricted to highly specific situations such as proximity to particular animals, heights, thunder, darkness, flying, closed spaces, urinating or defecating in public places, eating certain foods, dentistry, or the sight of blood or injury
  • Triggering situation may be discrete however contact with it can evoke panic as in agoraphobia or social phobia
46
Q

What is personality and how does it develop?

A
  • Refers to a collection of characteristics or traits developed as we have grown up and which make each person unique
  • Governs the way we think, behave and feel
  • Usually develops by late teens, early twenties
47
Q

What is the ICD 10 definition of a personality disorder?

A
  • Severe disturbance in the characterological constitution and behavioural tendencies of the individual, usually involving several areas of the personality, and nearly always associated with considerable personal and social disruption
48
Q

Which personality disorders are in cluster A?

A

Odd, Eccentric

  • Paranoid
  • Schizoid
  • Schizotypal
49
Q

Which personality disorders are in cluster B?

A

Dramatic

  • Dissocial
  • Emotionally unstable
  • Histrionic
  • Antisocial
  • Borderline
  • Narcissistic
50
Q

Which personality disorders are in cluster C?

A

Anxious/fearful

  • Anankastic
  • Anxious
  • Dependent
  • Obsessive-compulsive
  • Avoidant
  • Dependent
51
Q

How common is a personality disorder?

A
  • 50% psychiatric patients
  • 40-70% have been admitted
  • 30-40% seen in outpatients
52
Q

What are the characteristics of emotionally unstable/ borderline personality disorder?

A
  • Marked tendency to be impulsive
  • Unstable mood
  • Intense anger and violent outbursts
  • Outbursts of violence or threatening behaviour
  • Disturbance of self image; chronic feelings of emptiness
  • Intense unstable relationships
  • Suicidal threats and self harm
53
Q

What are the 4 problem areas resulting from EU/borderline PD?

A
  • Difficulty tolerating distress - self harm
  • Problems with interpersonal relationships
  • Problems regulating mood
  • Problems with sense of self
54
Q

How can DBT be used as a psychological intervention for borderline PD?

A
  • DBT - patients learn skills to address each of the problem areas
  • Learn to tolerate distress
  • Learn skills for better relationships
  • Learn mindfulness so can accept in order to change
  • CBT, mentalisation based therapies, psychoanalytical and therapeutic therapies can be useful
55
Q

What are the general principles of managing personality disorders?

A
  • Conduct individual needs assessment
  • Manage crises
  • Manage comorbidity
  • Refer to specialist services
  • Drug treatment not for the disorder itself but to manage any comorbidities
  • Clarity
  • Avoid admission if possible - only use as a last resort to manage risk
56
Q

What are the characteristics of a paranoid personality disorder?

A
  • Suspicious
  • Sensitive
  • Mistrustful
  • Jealous
  • Resentful
  • Bears grudges
  • Self important
57
Q

What are the characteristics of an antisocial personality disorder?

A
  • Callous
  • Transient relationships
  • Irresponsible
  • Impulsive and irritable
  • Lack guilt and remorse
  • Fail to accept responsibility
58
Q

What are the characteristics of an obsessive compulsive personality type?

A
  • Pre-occupied with rules and details
  • Inhibited by perfectionism
  • Overconscientious and scrupulous
  • Excessively concerned with productivity
  • Rigid and stubborn
  • Expects others to submit to his ways
  • Excessively pedantic and bound by convention
  • Excessively doubting and cautious
  • Miserly, hoards money