Neurodegenerative Conditions Flashcards

1
Q

Name some common signs of Parkinson’s disease on examination

A
  • Triad of bradykinesia with decrement, rigidity (cogwheel, worsened with synkinesis) and resting tremor (pill-rolling)
  • Gait: shuffling, slow turning, reduced arm swing, poor initiation of movement
  • Hypomimia
  • Postural instability: pull test
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2
Q

Name some extra-motor signs/symptoms of Parkinson’s disease

A
  • Sleep disturbance: REM
  • Anosmia
  • Urinary incontinence, nocturia
  • Constipation
  • Postural hypotension
  • Dementia
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3
Q

What are the Parkinsonian disorders? Name some other causes of Parkinsonism

A
Parkinson's disease
Multiple system atrophy
Progressive supranuclear palsy 
Corticobasilar degeneration
Lewy body dementia
Also:
-Infection: HIV, syphilis, CJD
-Vascular Parkinson's
-Metabolic: Wilson's 
-Iatrogenic: antipsychotics, antiepileptics, antiemetics
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4
Q

Describe the key characteristics of multiple system atrophy.

A
  • Alpha synucleinopathy
  • Prominent autonomic symptoms
  • Cerebellar ataxia
  • Parkinsonism
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5
Q

Describe the key characteristics of progressive supranuclear palsy

A
  • Parkinsonism, symmetrical onset
  • Vertical gaze palsy (intentional)
  • Pseudobulbar palsy
  • Tendency to early falls
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6
Q

Describe the key characteristics of corticobasilar degeneration

A
  • Parkinsonism
  • Unilateral
  • Alien hand
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7
Q

Describe the key characteristics of lewy body dementia

A
  • Dementia onset prior to Parkinsonism
  • Visual hallucinations (Lilliputian)
  • Fluctuating course
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8
Q

Describe the treatment for Parkinson’s disease

A

Conservative:

  • MDT: OT and PT important
  • Manage symptoms eg. laxatives, citalopram
  • Inform DVLA, carers etc

Medical:

  • 1st line: Levo-Dopa best for movement (w carbidopa) eg. Sinemet
  • Also: Ropinirole, Selegeline
  • Adjuncts/2nd: Entacapone, apomorphine SC, amantadine

Surgical:

  • DBS
  • Surgery to BG
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9
Q

What are some complications of Parkinson’s treatment?

A
  • Dyskinesias
  • On-off phenomena
  • End of dose wearing off
  • N+V
  • Psychosis
  • Dry mouth
  • Insomnia
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10
Q

What investigations should be done for someone presenting with Parkinsonism?

A

Depends on the age/presentation eg. typical features need no Ix
Atypical:
-Bloods: HIV, syphilis, Cu and caeruloplasmin
-Imaging: MRI 1st, consider DaT scan if drug-induced/vascular

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11
Q

If asked to examine a patient who has Parkinsonism, what will you specifically look for?

A
  • Face: hypomimia
  • Oculomotor: vertical gaze palsy
  • Upper limb: rigidity, tremor, decrement (finger tap) with synkinesis. Check for normal power and reflexes
  • Gait and pull test

+ full neuro, cognitive assessment (eg MoCA), review drug chart

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12
Q

Name the different forms of dementia in order of frequency

A

Alzheimer’s
Vascular
Lewy body
Frontotemporal

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13
Q

Briefly describe the characteristic features of Alzheimer’s

A
  • Progressive permanent global cognitive decline
  • F >M
  • Amnesia, nominal aphasia, Agnosia, Disorientation, Apraxia
  • Progress into behavioural and psychiatric symptoms
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14
Q

Briefly describe the characteristic features of vascular dementia

A
  • Sudden step-wise deterioration in cognitive function
  • May affect any area
  • In vasculopaths eg. HTN, T2DM
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15
Q

Briefly describe the characteristic features of lewy body dementia

A
  • Fluctuating cognition
  • Parkinsonism
  • Lilliputian hallucinations
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16
Q

Briefly describe the characteristic features of frontotemporal dementia

A
  • Personality change
  • Behavioural problems eg. disinhibition, hypersexuality
  • Poor executive functioning
17
Q

Describe the treatments for Alzheimer’s disease

A

Conservative:

  • MDT at old age psych/memory clinic
  • DVLA
  • Psychological interventions
  • Social needs

Medical:

  • 1st line- AChE i: donepezil, rivastigmine, etc
  • 2nd line- Memantine
18
Q

What are the treatment options for non-Alzheimer’s dementia?

A

Vascular- manage RFs
LBD- AChEi, galantamine
Frontotemporal- psychological

19
Q

Name some causes of dementia

A
Vascular
Infection/inflamm: syphilis, HIV, CJD 
Trauma
Autoimmune
Metabolic: Wilson's, hypothyroidism, B12 deficiency, alcohol
Neoplastic
Congenital: Down's syndrome
Degenerative: Alzheimer's, LBD, Parkinson's
20
Q

Describe the key features of Huntington’s disease

A
  • AD condition caused by mutation in the Huntingtin gene -> CAG repeats
  • Presents in middle age
  • Characterised by chorea and athetosis w decline of cognitive function (usually executive)
21
Q

How is Huntington’s diagnosed?

A

Clinical diagnosis.

  • Order CAG repeat genetic testing to confirm
  • Bloods to rule out other pathology if unclear eg. HIV, syphilis, FBC, B12 and folate
  • Imaging if unclear eg. MRI
22
Q

Define multiple sclerosis

A

Demyelinating inflammatory condition of the central nervous system, with at least 2 lesions separated by space and time

23
Q

Describe the epidemiology of MS

A

2 peaks
Young females (20s-30s). Northern Euro descent
Elderly males

24
Q

Describe the symptoms and signs of MS

A

S+Ss can be basically any manifestation of central NS lesion eg:

  • Sensory abnormality- parasthesia, anaesthesia
  • Motor dysfunction- spastic paraparesis
  • Autonomic dysfunction- incontinence, constipation
  • Visual changes- commonly optic neuritis (painful monocular loss), internuclear ophthalmoplegia
  • Cerebellar signs

Classic other features:

  • Lhermitte’s sign: shooting pain when flexing neck
  • Uhthoff’s: Worsening of sensory symptoms in heat
25
Describe the pathogenesis of MS
``` Unknown Strong familial disposition ? viral trigger Inflammation and axonal degeneration eg. relapsing remitting -> inflammatory episodes, eventually degeneration ```
26
What are the types of MS?
Relapsing-remitting 80% -> can become secondary progressive (one episode is called Clinically Isolated Syndrome- CIS- until second episode) Primary progressive 20%
27
What is the approach to diagnosis of MS?
- History and examination support Dx (2 episodes divided by space + time) * ***Has to occur for >48 hours, no intercurrent infection - Bloods: FBC, CRP, ESR, TFTs, HbA1c, U+Es, B12 + folate, antibodies (ANCAs, ENA, dsDNA), BBVs (HIV, syphilis) - LP: oligoclonal bands - Imaging: MRI brain and spine w gadolinium enhancement + FLAIR
28
Describe the treatment for MS
Conservative: - MDT at special MS centre with OT and PT - Exercise, diet, sleep Medical: -Neuropathic pain: gabapentin, antidepressants, etc -Spasticity: Baclofen -Acute relapse w functional impairment: 1g methylpred IV infusion, plasma exchange (RRMS) immunomodulators: interferon beta, glatiramer, dimethyl fumarate, teriflunomide -2nd line: fingolimod, biologics (PPMS) ocrelizumab
29
Describe the presentation of Wernicke's
Triad of: - Delirium - Ataxia - Ophthalmoplegia, nystagmus
30
How is Wernicke's treated?
Parenteral thiamine | -Treatment dose: 2-3 pairs, TDS, 3-5 days
31
Describe the types of MND
ALS (amyotrophic lateral sclerosis): most common. UMN and LMN PLS (primary lateral sclerosis): UMN only Progressive muscular atrophy: LMN only
32
Describe the presentation of ALS
Progressive unremitting motor dysfunction - Often starts in limbs or bulbar muscles, asymmetrical - Spreads contiguously - May have UMN or LMN signs - Painful muscle spasms * Bulbar symptoms: coughing/choking on food, slurred nasal speech,
33
Describe the diagnostic process for suspected ALS
- History suggestive: eg. weakness - Examination shows UMN or LMN lesions - Bloods: FBC, U+Es, LFTs, CK, TFTs, cortisol, B12, antibodies (VGCC, AChR) - Imaging: MRI head and spine - EMG, NCS
34
Describe the treatment of ALS
MDT approach -Conservative: OT, PT, SALT. Psych. Dietician -Medical: Riluzole. NIPPV when resp dysfunction Monitoring: -FVC monitoring