Movement disorders Flashcards
PD drug management:
a) 1st line - 3 common classes (2 unlikely alternatives)
b) Which is preferable in younger patients with lower degrees of motor impairment
c) Which is most effective? (how is it given?)
d) If cannot take oral medication - option?
e) Which drugs are not recommended 1st line now and why?
f) Refractory PD - consider what non-drug therapy?
a) - L-Dopa (given with dopa-decarboxylase inhibitor)
- Non-ergot derived D2 agonist (e.g. ropinirole), or
- MAO-BI (e.g. selegiline, rasagiline)
May also try…
- Amantadine (weak dopamine agonist)
- Anticholinergic (eg. procyclidine)
b) Selegiline/ropinirole
c) L-Dopa:
- given with dopa-decarboxylase inhibitor in a preparation like Sinemet® or Madopar®
d) Duo-Dopa pump:
- Levodopa/Carbidopa infusion via PEG tube
- Steadier levels of dopamine - may reduce motor fluctuations
e) Ergot-derived dopamine agonists (eg. carbergoline, bromocriptine), due to:
- Risk of fibrosis, particularly of heart valves (also pulmonary and retroperitoneal)
f) DBS
L-Dopa
a) Main side effects from long-term use
b) What drugs can be added to reduce these?
c) Other ways of reducing these phenomena
a) - Motor fluctuations: on/off phenomenon, wearing off phenomenon, freezing
- Dyskinesias: peak-dose dyskinesias (usually choreic), diphasic dyskinesia and dystonia
b) - Ropinirole (D2 agonist)
- MAO-BI, or
- COMT inhibitor (e.g. entacapone)
c) Prolonged-release L-dopa preparations, taking 30 minutes before food, smaller and more frequent doses
Peripheral metabolism of L-Dopa into dopamine
a) What 2 enzymes
b) What 2 drug classes inhibit these enzymes (with examples of each)
a) DOPA decarboxylase and Catechol-O-methyltransferase (COMT)
b) - DDC inhibitor (e.g. carbidopa)
- COMT inhibitor (e.g. entacapone)
PD symptom management
a) Depression
b) Dementia
c) Psychosis
d) Orthostatic hypotension - 1st line, 2nd line
e) REM sleep disorder
f) Drooling
a) SSRI
b) Cholinesterase inhibitor
c) Quetiapine (or clozapine)
- NOT first generation anti-psychotic (haloperidol, chlorpromazine, etc.)
d) Midodrine, then fludrocortisone
e) Clonazepam or melatonin
f) Glycopyrronium
Wilson’s disease.
a) What is it?
b) Symptoms usually appear when?
c) Clinical features (usually appear at what age?)
d) 2 things required for diagnosis
e) Other Ix
f) Management
a) Genetic disorder (AR, Ch13) or copper deposition, mainly affecting liver, brain and eye
b) Symptom onset usually in 20s.
- Generally have liver cirrhosis by the time they present with neuropsychiatric features
c) - Opthalmological: Kayser-Fleischer rings (95%), sunflower cataracts
- Liver: hepatitis, acute liver failure, liver cirrhosis
- Brain: first tremor, then other PD features, later chorea
- Psych features common
- May also have cardiac, renal, MSK and other features
d) - Low ceruloplasmin
- Kaiser-Fleischer rings
e) - LFTs
- Liver scan and biopsy
- Brain imaging
- ECG
- Urine - copper excretion
f) Wilson’s is VERY treatable:
- Penicillamine (copper chelator) - beware in SLE
- Zinc supplements
- Avoid alcohol and copper-rich foods (eg. liver, chocolate, mushroom)
- Chorea? - tetrabenazine (also used for tardive dyskinesia)
- Liver transplantation
PD tremor vs Essential tremor:
- distinguishing features.
- Postural/action tremor (ET) vs resting tremor (PD)
- Distractibility (induces tremor = suggests PD)
- Symmetry (usually starts unilateral in PD)
- Amplitude
- Body parts (head/ vocal involvement suggests ET)
- PD features (bradykinesia, rigidity, etc.)
- Handwriting (micrographia suggests PD)
- Effect of L-dopa (improvement suggests PD)
- Effect of alcohol (improvement suggests ET)
Dopamine (D2) agonists
a) Two types - example of each
b) Main side effects
c) Which one may be given as injection?
a) - Ergot-derived (e.g. carbergoline)
- Non-ergot derived (e.g. ropinirole - preferred)
b) Sleepiness, impulsive and compulsive behaviours
c) Apomorphine
Non-pharmacological management of PD
MDT.
- Parkinson’s disease nurse specialists.
- Physiotherapy: improve gait and stability, avoid Zimmer frames unless they have wheels; improve fitness
- Occupational therapy
- Speech and language therapy - improve speech volume, help with swallowing in later stages
- Nutritional support
- Psychological support
Patient education.
- Compliance
-
Patient with movement disorder. CT scan shows generalised atrophy, and in particular atrophy of the caudate nucleus and putamen.
- Diagnosis?
- Cause of this disease?
- Classic features and progression?
- Management?
Huntington’s Disease.
Cause.
- AD inherited disorder - huntingtin gene - Ch 4
- Trinucleotide cytosine-adenine-guanine (CAG) repeats
- Leads to neurodegeneration
Features.
- Onset around age 40
- Initially frontal lobe symptoms - apathy, neglect, loss of inhibition, depression
- Then movement disorder (chorea, Parkinsonism) and dementia
Management.
- No curative therapies; just symptomatic and non-drug
- Hungtington’s disease care homes
- Genetic counselling and psychological support (may offer testing to family members)
- Chorea - benzo or tetrabenazine
- Parkinsonism - D2-agonist
- Depression - SSRI
- Psychosis - quetiapine, clozapine (not 1st gen APs)
Multiple system atrophy (MSA)
a) Predominant symptoms (give 4)
b) Features suggesting MSA over PD
c) Tests to support (though diagnosis is clinical)
d) Management
a) Urinary dysfunction, erectile dysfunction, postural hypotension, cerebellar ataxia, Parkinsonism
b) Rapid progression, poor L-Dopa response, prominent autonomic symptoms
c) Autonomic tests, PET scan
d) Symptomatic:
- postural hypo (midodrine)
- constipation (laxatives)
- urinary incontinence (anticholinergics)
- parkinsonism (dopamine), SALT, PT, OT, etc.
Progressive supranuclear palsy (PSP)
a) 6 key features
b) Management
a) - Supranuclear ophthalmoplegia,
- early cognitive dysfunction
- parkinsonism
- autonomic dysfunction and falls
- pseudobulbar palsy
- neck dystonias
b) Symptomatic:
- dopamine agonists
- SALT, PT, OT
Tremor.
a) Physiological causes
b) Neurological causes
c) Examination
d) Investigations
e) Management
a) Physiological.
- Benign essential tremor (familial)
- Anxiety
- Fever
- Metabolic: hyperthyroid, hypo/hyperglycaemia
- Drugs: beta-agonist, steroid, lithium, thyroid rx, caffeine, other stimulants
- Alcohol withdrawal
b) Neuro.
- PD and Parkinson’s plus syndromes
- Drug-induced Parkinsonism
- Wilson’s
- Cerebellar syndrome (intention tremor)
c) ?
d) - Bedside: ECG, urine toxiology
- Bloods: FBC, CRP, U+Es, TFTs, calcium, glucose
- Imaging: CT/MRI head if indicated
- Special tests: eg. NCS
e) - ET: propanolol, primidone
- Treat any underlying causes
Diagnosing PD.
a) Stage 1 (Parkinsonian)
b) Stage 2 (exclusion)
c) Stage 3 (for diagnosis of definite PD)
d) Investigations
a) Diagnose Parkinsonian syndrome:
- Bradykinesia, plus at least one out of…
1. Rigidity
2. Resting tremor 4 - 6 Hz
3. Postural instability (not caused by visual, vestibular, cerebellar or proprioceptive dysfunction)
b) Exclusion of other causes:
- eg. head injury, vascular (stroke), cerebellar signs, supranuclear gaze palsy, early autonomic features
c) Unilateral onset, resting tremor, response to L-Dopa, progressive
d) - Refer to specialist for diagnosis
- Clinical diagnosis (imaging not usually required)
- If uncertainty - may do SPECT (dopaminergic neuron loss in substantia nigra), MRI, etc.
Lewy-body dementia
Dementia Fluctuating attention/concentration Parkinsonism Visual hallucinations REM sleep disorder
Creutzfeld-Jakob Disease (CJD).
a) What is it?
b) Types
c) Features
d) Investigations and findings
e) Management
a) Prion disease causing neurodegeneration
b) Sporadic (85%), hereditary (< 15%), iatrogenic (< 1%), new-variant* (< 1%)
* new variant is related to bovine spongiform encephalopathy (BSE) - i.e. mad cow disease - may be caused by eating infected meat products (VERY rare!)
c) - Progressive ataxia, movement disorder (chorea, myoclonus, rigidity, dystonia, etc.) and dementia
d) - EEG: periodic wave complexes
- LP: 14-3-3 protein
- Brain biopsy is only definite way to diagnose (done at post-mortem)
e) - No cure
- Supportive, as for diseases like HD (manage chorea, PD, dementia, depression, etc.)
