Neurodegenerative Conditions Flashcards
Name some common signs of Parkinson’s disease on examination
- 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
Name some extra-motor signs/symptoms of Parkinson’s disease
- Sleep disturbance: REM
- Anosmia
- Urinary incontinence, nocturia
- Constipation
- Postural hypotension
- Dementia
What are the Parkinsonian disorders? Name some other causes of Parkinsonism
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
Describe the key characteristics of multiple system atrophy.
- Alpha synucleinopathy
- Prominent autonomic symptoms
- Cerebellar ataxia
- Parkinsonism
Describe the key characteristics of progressive supranuclear palsy
- Parkinsonism, symmetrical onset
- Vertical gaze palsy (intentional)
- Pseudobulbar palsy
- Tendency to early falls
Describe the key characteristics of corticobasilar degeneration
- Parkinsonism
- Unilateral
- Alien hand
Describe the key characteristics of lewy body dementia
- Dementia onset prior to Parkinsonism
- Visual hallucinations (Lilliputian)
- Fluctuating course
Describe the treatment for Parkinson’s disease
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
What are some complications of Parkinson’s treatment?
- Dyskinesias
- On-off phenomena
- End of dose wearing off
- N+V
- Psychosis
- Dry mouth
- Insomnia
What investigations should be done for someone presenting with Parkinsonism?
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
If asked to examine a patient who has Parkinsonism, what will you specifically look for?
- 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
Name the different forms of dementia in order of frequency
Alzheimer’s
Vascular
Lewy body
Frontotemporal
Briefly describe the characteristic features of Alzheimer’s
- Progressive permanent global cognitive decline
- F >M
- Amnesia, nominal aphasia, Agnosia, Disorientation, Apraxia
- Progress into behavioural and psychiatric symptoms
Briefly describe the characteristic features of vascular dementia
- Sudden step-wise deterioration in cognitive function
- May affect any area
- In vasculopaths eg. HTN, T2DM
Briefly describe the characteristic features of lewy body dementia
- Fluctuating cognition
- Parkinsonism
- Lilliputian hallucinations
Briefly describe the characteristic features of frontotemporal dementia
- Personality change
- Behavioural problems eg. disinhibition, hypersexuality
- Poor executive functioning
Describe the treatments for Alzheimer’s disease
Conservative:
- MDT at old age psych/memory clinic
- DVLA
- Psychological interventions
- Social needs
Medical:
- 1st line- AChE i: donepezil, rivastigmine, etc
- 2nd line- Memantine
What are the treatment options for non-Alzheimer’s dementia?
Vascular- manage RFs
LBD- AChEi, galantamine
Frontotemporal- psychological
Name some causes of dementia
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
Describe the key features of Huntington’s disease
- 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)
How is Huntington’s diagnosed?
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
Define multiple sclerosis
Demyelinating inflammatory condition of the central nervous system, with at least 2 lesions separated by space and time
Describe the epidemiology of MS
2 peaks
Young females (20s-30s). Northern Euro descent
Elderly males
Describe the symptoms and signs of MS
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
Describe the pathogenesis of MS
Unknown Strong familial disposition ? viral trigger Inflammation and axonal degeneration eg. relapsing remitting -> inflammatory episodes, eventually degeneration
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%
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
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
Describe the presentation of Wernicke’s
Triad of:
- Delirium
- Ataxia
- Ophthalmoplegia, nystagmus
How is Wernicke’s treated?
Parenteral thiamine
-Treatment dose: 2-3 pairs, TDS, 3-5 days
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
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,
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
Describe the treatment of ALS
MDT approach
-Conservative: OT, PT, SALT. Psych. Dietician
-Medical: Riluzole. NIPPV when resp dysfunction
Monitoring:
-FVC monitoring
