Huntington's, Parkinson's & Dementia (Alzheimer's & Vascular) Flashcards
Parksinson’s disease
- Aetiology
- S+Sx
- Describe gait
- Parkinson’s Plus
- Motor fluctuations
- Investigations
- Histological hallmark
- Management + SEs
- Complications + Management
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
-
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)
-
Carbidopa, Benserazide, Sinemet, madopar
-
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
-
MAO-_B_ Inhibitor
- Selegiline, rasagilline
- MonoAmine Oxidase B inhibitor
- Delay 1
-
COMT inhibitors
- Entacapone
- Catechol-O-Methyltransferase Peri. brkdown of levodopa
-
Amantidine (anti-viral)
- Early, or late bradykinesia
-
Antimuscarinics
Procyclidine, orphenadrine, benztropine, trihexyphenidyl - 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
Huntington’s disease
- Pathophysiology
- Presentation
- Diagnosis
- Management
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
Dementia
- Definition
- Aetiology
- Screening tools + initial investigations
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
Alzheimer’s disease
- Pathophysiology
- Presentation
- Diagnosis
- Management
Pathophysiology (sporadic, 5%AD)
- Widespread Cortical atrophy
- type A Beta-Amyloid plaques
- Tau Neurofibrillary tangles
- 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
-
Acetylcholinesterases Inhibitors
Donepezil, Galantamine, Rivastigmine - Memantine (NMDA antagonist)
Vascular dementia
- Pathophysiology
- Clinical presentation
- Investigations
- Management
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
Lewy Body Dementia
- Presentation
- Investigations + Dx
- Management
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
Define tremor
Differentials (sheet)
Involuntary trembling or quiverin
Define Athetosis
Outline some causes
Slow involuntary repetitive writing movements
- Lesions to Corpus Striatum [affects basal ganglia]
Huntington’s disease - Cerebral palsy
Define Chorea
Outline some causes
Ceaseless occurrence of rapid, jerky, dyskinetic involuntary movements
- Hungtington’s disease
- Sydenham’s chorea [rheumatic fever]
Define Ballismus
Outline some causes
Violent jerky movements of limbs
- Subthalamic nuclei issue (lacunar stroke)
Basal Ganglia structures
Draw
Balal ganglia (direct & indirect pathway)
Lesions for Hungington’s & Parkinson’s disease
Draw
Peripheral neuropathy
Aetiology + Key info
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)