Huntington's, Parkinson's & Dementia (Alzheimer's & Vascular) Flashcards

1
Q

Parksinson’s disease

  • Aetiology
  • S+Sx
    • Describe gait
  • Parkinson’s Plus
  • Motor fluctuations
  • Investigations
    • Histological hallmark
  • Management + SEs
  • Complications + Management
A

Parkinson’s disease

Loss of dopaminergic neurones in Substantia Nigra Pars Compacta - unknown aetiology

S+Sx - TRAP

  • Hypomemia (mask face)
  • Greasy + Dry subhorrhoic dermatitis
  • Excess salivation
  • T remor 5Hz resting, pill-rolling
  • R igidity lead pipe + cogwheel
  • A /bradykinesia slow
  • P ostural instability
    • Extrapyramidal posture (simian/ gunslinger)
    • Gait
      • Suffling gait
      • Festination (hurry)
      • Retropulsion (falling back)
      • No arm swing
      • Clockface turn

Parkinson’s Plus - CRAM’D

  • C ortico-basal
    • Alien hand, apraxia
  • R ichardson- Olszewski PSP
    Progressive Supranuclear Palsy
    • Vertical gaze
  • A utonomic Shy Drager
    • Orthostatic hypotension
  • M ulti-system atrophy MSA
    • Cerebellar, pyramidal, autonmic
  • D ementia Lewy Body
    • Visual hallucinations

Motor fluctuations

  • End-of-dose (meds wearing off)
  • On-off (dyskinesia to immobility)
    (late, high L-Dopa dose)
  • Dyskinesia
    (anytime: start, peak or end of dose)

Investigations

  • Clinical Dx (Parkinson’s disease society): Bradykinesia + T,RorP
  • Dopamine Transporter Scan (DaT)
    is a Positron Emission Tomography (PET) with contrast
    • Loss of dopamineric neurones in Lentiform nucleus
  • Histological hallmark: Lewy Bodies

Management

  • Counselling, physio, occupational, speech & language therapists
  1. L-dopa + Peripheral decarboxylase inhibitor
    • ​Carbidopa, Benserazide, ​Sinemet, madopar
      • Sinemet, Madopar
      • Duodopa via PEG tube
    • Best, works for 5yrs
    • SE: rare N+V, Dyskinesia (titrate)
  2. Dopamine receptor agonist
    • Pramipexole, ropinirole, totigotine
    • Ergot derived (SE: Fibrosis);
      Bromocroptine, Cabergoline, Pergolide(CV)
      CXR, Echo, ESR and Creatinine prior
    • Delay 1, reduces ‘off’ effect
    • SEs: Impulse control, daytime sleepiness
  3. MAO-_B_ Inhibitor
    • Selegiline, rasagilline
    • MonoAmine Oxidase B inhibitor
    • Delay 1
  4. COMT inhibitors
    • ​Entacapone
    • Catechol-O-Methyltransferase Peri. brkdown of levodopa
  5. Amantidine (anti-viral)
    • Early, or late bradykinesia
  6. Antimuscarinics
    Procyclidine, orphenadrine, benztropine, trihexyphenidyl
  7. Sudden severe off episode: Apomorphine

Complications

  • Depression + Anxiety: TCA Nortryptiline
  • Dementia: Cholinesterase inhibitors
  • Compulsive behaviour with Dopamine agonists: adjust
  • Hallucinations + Psychosis
  • Parkinson’s crisis Acute Akinesia
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2
Q

Huntington’s disease

  • Pathophysiology
  • Presentation
  • Diagnosis
  • Management
A

Rare autosomal dominant

  • Abnormal CAG repeat sequence
    Huntingtin gene Chromosome 4p16.3
  • No GABA: Chorea
    Normal dopamine: Movements precise

Presentation

  • 4/5th decade
  • Early
    • Personality, memory
    • Slow Sacchadic eye movements
  • Chorea, dystonia + ballismus
  • Dementia, depression, anxiety, aggression
  • Late: Spasticity + clonus

Diagnosis

  • Genetic testing
  • MRI/ CT: Striatum/ caudate atrophy

Management

  • Chorea: Tetrabenazine (dopamine-depleting agent)
  • Psychosis/ depressions are per
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3
Q

Dementia

  • Definition
  • Aetiology
  • Screening tools + initial investigations
A

Chronic failure of cognitive function

Aetiology

  • 50% Alzheimer’s disease
  • 25% Vascular disease
  • ​15% Dementia with Lewy Bodies
  • Frontotemporal dementia
    • Pick’s disease (protein tangles)
  • Parkinson’s disease
  • Normal pressure hydrocephalus

Screening tools

  • Mini-mental state examination
  • Reversible causes
    • TFTs Hypothyroidism
    • FBC, U+Es, Glucose
    • Vitamin B12 + folate
    • Neuroimaging
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4
Q

Alzheimer’s disease

  • Pathophysiology
  • Presentation
  • Diagnosis
  • Management
A

Pathophysiology (sporadic, 5%AD)

  1. Widespread Cortical atrophy
  2. type A Beta-Amyloid plaques
  3. Tau Neurofibrillary tangles
  4. LOW Acetylcholine​

Presentation

  • 1st ST memory loss ⇒
  • Language (word finding, reading, writing)
  • Personality (apathy, depression, emotional)

Diagnosis

  • Differentiate Vascular Dementia
  • *Single-P**hoton Emission Computed Tomography (SPECT)
  • Histology: 40% Lewy bodies

Management

  1. Acetylcholinesterases Inhibitors
    Donepezil, Galantamine, Rivastigmine
  2. Memantine (NMDA antagonist)
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5
Q

Vascular dementia

  • Pathophysiology
  • Clinical presentation
  • Investigations
  • Management
A

Pathophysiology

  • Strokes
    • Summative effect of lots of tiny strokes causing stepwise deterioration
    • Occasionally a large basal ganglia stroke can cause permanent confusion state
  • Biswanger’s disease - chronic ischaemia damaging white matter at hemisphere junction

Clinical presentation

  • History of several minor/ major strokes
  • Stepwise deterioration
  • History of hypertension, vascular problems, AF
  • Focal signs (brisk reflexes & upbringing plantar responce)

Investigations

  • CT/ MRI multiple areas of infarction
  • Atrial fibrilation ECF
  • Differentiate:
    Single-Photon Emission Computed Tomography (SPECT)

Management is treating cause/ risk factor

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6
Q

Lewy Body Dementia

  • Presentation
  • Investigations + Dx
  • Management
A

Presentation

  • Fluctuating Dementia (visuospatial, excutive)
    • Visual hallucinations (seeing people)
    • Attention + concentration
    • Parkinsonism (after dementia)
  • REM sleep behaviour disorder
  • SEVERE Neuroleptic sensitivity (severe parkinsonism)

Investigations + Dx

  • Single-Photon Emission Computed Tomography (SPECT)
    • LOW dopamine uptake in Basal Ganglia
  • ​​​Histology: Lewy Bodies

Management

  • Acetylcholinesterases Inhibitors
    Donepezil, Galantamine, Rivastigmine
  • Parkinsonism can be treated as per parkinson’s but psychiatric conditions may worsen (Levodopa)
  • Neuroleptic sensitivity: NO antipsychotics
    Atypicals better than typicals
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7
Q

Define tremor

Differentials (sheet)

A

Involuntary trembling or quiverin

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8
Q

Define Athetosis

Outline some causes

A

Slow involuntary repetitive writing movements

  • Lesions to Corpus Striatum [affects basal ganglia]
    Huntington’s disease
  • Cerebral palsy
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9
Q

Define Chorea

Outline some causes

A

Ceaseless occurrence of rapid, jerky, dyskinetic involuntary movements

  • Hungtington’s disease
  • Sydenham’s chorea [rheumatic fever]
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10
Q

Define Ballismus

Outline some causes

A

Violent jerky movements of limbs

  • Subthalamic nuclei issue (lacunar stroke)
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11
Q

Basal Ganglia structures

Draw

A
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12
Q

Balal ganglia (direct & indirect pathway)

Lesions for Hungington’s & Parkinson’s disease

Draw

A
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13
Q

Peripheral neuropathy

Aetiology + Key info

A

ADCDE+3

  • Alcohol
  • B12 + folate
  • CKD
  • Diabetes
    +Drugs
  • Every vasculitis
  • +Cancer
  • +Lyme disease (tick bite erythema migrans, cardioneuro)
  • +Charcot-Marie Tooth (hereditary sensorimotor, foot drop)
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