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8. CMS > Lecture day 3 > Flashcards

Lecture day 3 Flashcards

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Introductory Sommelier Exam (CMS) flashcards
1
Q

What are the common categories for neurology pathologies?

A 
Cognition
Headache 
Seizure and syncope
Vision
Hearing and balance
Smell and taste
Motor
Sensation
Sphincter
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2
Q

What area of the brain does Alzheimer’s disease affect most?

A

Hippocampal

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3
Q

When is the common onset for Alzheimer’s disease?

A

mid-70s to early-80s

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4
Q

What is a common misdiagnosis for young people with Alzheimer’s?

A

Anxiety or depression

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5
Q

What are common pathological hallmarks of frontotemporal lobar degeneration?

A
  1. FTLD-tau
  2. TDP-43
  3. FTLD-FUS
  • Misfolded proteins may act as prion-like strains that seed network based disease spread
  • Progressive change in behaviour or language
  • Neurodegenerative of frontal or anterior temporal lobes
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6
Q

What are the different types of frontotemporal dementia?

A
  1. Behavioural variant (50%)

2. Language variant (50%): semantic or non-fluent

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7
Q

What are key features of behavioural frontotemporal dementia?

A
  • Early behavioural disinhibition
  • Early loss of empathy and emotional reactivity
    Perseverative, stereotyped or compulsive behaviours
  • Hyper-orality and dietary change
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8
Q

What are key features of semantic dementia?

A
  • Impaired single word and object knowledge
  • Surface dyslexia
  • Spared speech production and repetition
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9
Q

What are key features of non-fluent aphasia dementia?

A
  • Motor speech deficit, effortful hesitant speech
  • Agrammatism
  • Preserved single word knowledge
  • Uses nouns more than words
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10
Q

How does Lewy body dementia often present?

A
  1. Visual hallucinations (often animals and children)
  2. Parkinsonism
    - Drugs for this make people worse; become delirious
  3. Cognitive fluctuations
    - If you give them antipsychotics, the Parkinsonian symptoms will get worse
  4. Dysautonomia
  5. REM sleep disorders
  6. Neuroleptic sensitivity
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11
Q

How does alcohol affect neuronal function?

A
  1. Malnutrition
  2. Alcohol neurotoxicity
  3. Recurrent TBI
  4. Metabolic disturbances
  5. Seizures
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12
Q

How does HIV affect neuronal function?

A

HIV related neurocognitive disorder refers to a spectrum of disorders from asymptomatic neurocognitive impairment to HIV associated dementia

  • Widespread use of HAART has resulted in a marked decrease in HIV related dementia
  • All patients presenting with dementia should have an HIV test
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13
Q

Why do people with epilepsy have cognitive impairment?

A

Due to chronic epilepsy

Due to AED’s which reduce neuronal firing and slow people down

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14
Q

What do prion diseases lead to?

A

Can transform into a beta-sheet, which cannot be removed

Causes rapid dementia, which will lead to death in four months

Often mid-50s

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15
Q

What disease can be confused with Alzheimer’s but is curable?

A

Sleep apnoea

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16
Q

What genetic factors are involved in dementias?

A
  1. C9ORF72
  2. Amyloid precursor protein (Ch21q)
  3. Presenilin 1 (Ch14q): 50% of early-onset AD
  4. Progranulin (posterior AD)
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17
Q

What pharmacologicals can be used in AD?

A

Anticholinesterase inhibitors: delay the progression by a few months

Memantine

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18
Q

What non-pharmalogical steps can be disease-modifying?

A
  • Cognitive stimulation activities
  • Keep active and exercise
  • Treat other medical conditions
  • Manage anxiety, frustrations and depression
  • Reduce unnecessary medication
  • Support in community
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19
Q

What are types of focal injury?

A
  1. Vascular injury
  2. Axonal injury
  3. Contrusions
  4. Lacerations
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20
Q

What are types of diffuse injury?

A
  1. Diffuse axonal injury

2. Diffuse vascular injury

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21
Q

What are the categories of sleep disorders?

A
  1. Insomnias
  2. Hypersomnias
  3. Parasomnias
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22
Q

What are the types of insomnias?

A
  1. Sleep initiation insomnia
  2. Sleep maintenance insomnia
  3. Sleep termination insomnia
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23
Q

What are the types of hypersomnias?

A
  1. Narcolepsy, idiopathic hypersomnia
  2. CDB, PLMD
  3. Drugs, psychiatric illness
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24
Q

What are the types of parasomnias?

A
  • Non-REM parasomnias:
    1. Sleeptalking
    2. Sleep walking
    3. Sleep terrors
    4. Bruxism
  • REM parasomnias:
    1. Rem sleep behavioural disorder, sleep paralysis, hypnagogic hallucinations
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25
Q

What is fatigue versus sleepiness?

A

Fatigue – “I feel exhausted after a 5 minute walk”, “If I concentrate too long I need to rest”

Sleepiness – do they actually feel like they want to sleep?

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26
Q

What would you use to quantify sleepiness?

A

Epworth score

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27
Q

What are causes of sleepiness?

A
  1. Obstructive sleep apnoea
  2. Restless les syndrome
  3. Behaviourally Induced Insufficient Sleep Syndrome
  4. Drugs
  5. Psychiatric disorders
  6. Central hypersomnias
  7. Circadian rhythm disorders
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28
Q

what is restless leg syndrome?

A
  • Very common sensorimotor disorder
  • Mainly characterised by urge to move legs or sensory
    symptoms in legs
  • May also affect other body parts: arms, abdomen, face
  • Four essential criteria:
    1. Urge to move legs, usually with unpleasant sensations
    2. Urge/sensations worsen with inactivity
    3. Urge/sensations partially or transiently relieved by movement
    4. Urge/sensations present or worse in evening/night
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29
Q

What is a central hypersomnia?

A

Narcolepsy

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30
Q

What are the main symptoms of narcolepsy?

A
  1. Excessive daytime sleepiness
  2. Hypnogogic hallucinations
  3. Sleep paralysis
  4. Cataplexy = Sudden temporary loss of muscle tone, usually precipitated by strong emotion, esp laughter and joking
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31
Q

What do you know about the excessive daytime sleepiness in narcolepsy?

A
  • Discrete sleep attacks or persistent sleepiness
  • Inappropriate times or locations
  • Brief sleeps usually refreshing
  • Total sleep time per 24 hours is normal
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32
Q

What do you know about the hypnogogic hallucinations in narcolepsy?

A
  • Predominantly visual, but may also be auditory
  • Generally lasts for less than ten minutes
  • Occasionally more bizarre, especially if associated with sleep paralysis
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33
Q

What is a brain finding in people with narcolepsy?

A

Loss of hypocretin-producing cells in lateral hypothalamus

34
Q

What factors influence the circadian rhythm?

A

Light, melatonin, genetics, exercise, temperature, feeding, aging

35
Q

What do you know about the epidemiology of MS?

A
  • More women than men
  • Onset around 30
  • More people further away from he equator
36
Q

What are risk factors for developing MS?

A
  1. Genetics
  2. Infectious agents (EBV)
  3. Low vitamin D
  4. Cigarette smoking (linked to onset and progression)
  5. Obesity in early life
37
Q

What are main symptoms of MS?

A
  1. Optic neuritis
    - Reduced vision - central and colour
    - Pain on eye movement
    - RAPD, swollen optic disk
    - In normal optic neuritis, symptoms get better and go away
  2. Lhermitte’s sign (When you put your head down on your chin, you get pain in the neck)
38
Q

What are other symptoms of MS?

A

Clumsiness, fatigue, anxiety, sleep disorders, sleep disorders, impaired speech and swallowing, tremor

39
Q

What investigations would you do for MS?

A

MRI
Blood tests to rule out other conditions
Lumbar puncture
Visual evoked potentials

40
Q

What are the differentials for MS?

A

Acute disseminated encephalomyelopathy

Idiopathic transverse myelitis

Neuromyelitis optica

Neurosarcoidosis

Migraine

Tumours

41
Q

What is the pathological hallmark of MS?

A

Demyelation within the central nervous system due to innate and adaptive immune systems

42
Q

How would you manage MS?

A
  1. General measures
    1. No smoking
    2. Healthy diet
    3. Positivity
    4. Vitamin D
  2. Disease-modifying drugs
    1. Siponimod
    2. Ozanimod
    3. Ofatumumab
    4. Ponesimod
  3. Relapse management
  4. Symptom control
43
Q

What are good prognostic factors in MS?

A

Young age of onset
Female
Onset optic neuritis
First remission greater than 2 years

44
Q

What are adverse prognostic factors in MS?

A 
Older age of onset
Male
Symptom of onset motor
Relapse frequency 3 or more in first five years 
Incomplete recovery after relapse
45
Q

How would you manage relapses?

A

ONLY treat relapses that cause distress

Steroids: for mild-mod oral for 5 days; for severe IV 3 days
–> methyl-prednisolone

46
Q

What would you give for severe relapses when steroids do not work?

A

Plasma exchange

47
Q

What is the most helpful investigation for MS?

A

MRI brain and spine

48
Q

What is a tremor?

A

Involuntary, rhythmic, oscillatory movement of a body part

49
Q

What are key facts about an essential tremor?

A
  1. Isolated tremor syndrome of bilateral upper limb action tremor
  2. At least 3 year duration
  3. With or without tremor in other locations like head, voice, or lower limbs
  4. Absence of other neurological signs such as dystonia, ataxia or parkinsonism
50
Q

What often improves essential tremors?

A

Alcohol

Involvement of cerebello-thalamo-cortical pathways - stimulation of the VIM improves the tremor

51
Q

What is the course of essential tremor?

A

Slowly progressive

52
Q

How would you treat essential tremors?

A
  • If mild, no treatment required
  • If moderate-severe:
    • Propranolol
    • Primidone
    • Combined propranolol or primidone
    • Emerging Treatments: Gabapentin and Topiramate
53
Q

What is dystonic tremor syndrome?

A

Dystonic tremor syndromes are tremor syndromes combining tremor and dystonia as the leading neurological signs

54
Q

What are key facts about dystonic tremor syndrome?

A

Position-dependence or task-specificity

Antagonistic gestures may lead to a reduction in the tremor amplitude

Irregular amplitude and variable frequencies (mostly below 7 Hz), “Jerky” tremor

Topographically, they present as head or arm tremor, but may also occur as voice, jaw or facial tremor and in some case tremor may precede dystonia (DD: essential tremor).

55
Q

How would you treat dystonic tremor syndrome?

A

anti-cholinergic (Trihexyphenidyl), tetrabenazine, Beta blockers

56
Q

What causes cortical tremors?

A

Drugs (fluoxetine, clozapine, gabapentin etc)

57
Q

What are key facts about functional tremors?

A

Also called psychogenic tremor

Characterised by distractibility, frequency entrainment or antagonistic muscle co-activation

58
Q

What are the main features of PD?

A
  1. Bradykinesia
  2. Rigidity
  3. Resting tremor
  4. Postural instability
  5. Gait abnormalities
59
Q

What kind of lesions do MRI scans pick up well?

A

Inflammatory lesions

60
Q

What disease can a PET scan detect?

A

Dementia

61
Q

How does CSF flow?

A

CSF is produced by the CP

Flows from lateral ventricle to third ventricle via the interventricular foramen
Then to 4th via cerebral aqueduct
In 4th ventricle:
- continue within the ventricular system and flow through the spinal canal or,
- flows into the subarachnoid space via the foramen of Magendie or via the foramina of Luschka

Reabsorbed into the systemic circulation via the arachnoid villi into the dural-venous sinuses

62
Q

What are contraindications for lumbar punctures?

A
  1. Platelet count under 40
  2. Local skin infection
  3. Local developmental abnormalities
  4. Raised ICP
63
Q

What are the most common nerve root disorders?

A

Cervical radiculopathy

Lumbrosacral radiculopathy

64
Q

How does a cervical radiculopathy often present?

A

Neck and upper limb pain

Sensory disturbance and weakness in arm, hand an finger

65
Q

How does a lumbrosacral radiculopathy often present?

A

Lower back pain: sciatica type symptoms and weakness in lower limb

66
Q

What is the most common anterior horn cell disorder?

A

Motor neuron disease

67
Q

What is the most common neuromuscular disorder?

A

Myasthenia gravis

68
Q

What are the most common muscle disorders?

A

Inflammatory myopathies

69
Q

What are groups often develop myasthenia gravis?

A

Mainly in young women and old men

70
Q

What muscles and system does MG affect?

A
  1. Ocular muscles: eyelids
  2. Facial muscles
  3. Speech, swallowing (bulbar)
  4. Limbs (generalised)
  5. Does not affect bladder, bowel or heart
71
Q

What are suggestive clinical signs of MG?

A
  • Weakness without muscle pain
  • Usually improvement with rest
  • Gradual or rapid onset
  • May come and go initially, sometimes over months
  • May follow illness, stress or pregnancy
  • Weakness becomes worse the more a certain muscle / muscle group is used
72
Q

What is the hallmark of MG?

A

Fatiguable, painless muscle weakness that improves with rest

73
Q

What do blood tests for MG show?

A

Blood tests show positive anti-acetylcholine receptor (AChR) antibodies

MuSK and LRP4 antibodies much less common

74
Q

How would you manage MG?

A
  1. Pyridostigmine: Reduces the breakdown of ACh between nerve and muscle
  2. Immune treatments: corticosteroids
  3. Crisis options: ICU and plasma exchange with iV immunoglobulins
75
Q

What are acquired muscle disorders?

A

Inflammatory muscle disease:

  1. Polymyositis
  2. Dermatomyositis
  3. Inclusion body myositis
  4. Necrotising myopathy
76
Q

How do you diagnose muscle disorders?

A

Needle biopsy

77
Q

What are the five most common muscle diseases?

A
  1. Myotonic dystrophy (30%)
  2. Dystrophinopathies (20%)
  3. Facioscapulohumeral dystrophy
  4. Limb girdle
  5. Spinal muscular atrophy
78
Q

What are key factors about myotonic dystrophy 1?

A

Distal muscle wasting and weakness, early cataracts

Cardiac problems

Distal muscle weakness in DM1, proximal in DMD2

79
Q

What are key factors of Duchenne?

A 
Delayed walking
Waddling gait
Gower's manoeuvre 
Calf pseudo hypertrophy 
Loss of ambulation by 12 years

Give corticosteroids in young boys

80
Q

What are key factors of Becker muscular dystrophy?

A

Walking until after 16, then wheelchair bound

Give corticosteroids in young boys

81
Q

What are key facts about facioscapulohumeral?

A

Early involvement of facial and scapular stabiliser muscles

Facial weakness often asymptomatic

Asymmetrical in limbs

Activities become more and more difficult, disease spreads (but no cardio-resp involvement)

8. CMS (21 decks)

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