General neurology [29/10/20] Flashcards
1
Q
4 key features of Parkinson’s disease
A
Bradykinesia, rigidity, postural instability, tremor
2
Q
Parkinsonism cauases
A
- idiopathic PD
- vascular Parkinsonism
- normal pressure hydrocephalus
- Parkinson’s plus syndrome [PPS]
- iatrogenic
3
Q
Typical preparation of L-dopa for PD
A
- Co-careldopa/ co-beneldopa [L-dopa given with a dopa-decarboxylase]
- CR prep
- dispersable prep [rescue/morn.]
- can have Statevo which is co-careldopa plus etacapone [etacapone helps stop breakdfown of L-dopa and is a COMT inhibitor]
4
Q
Dopamine agonists available, and their advantage over L-dopa
A
- non-ergot [Ropinirole, Pramiexole, Rotigotine]
- ergot [Bromotcriptine etc.]
- adv: less dyskinesia, longer acting
- dis: higher risk hallucinations, compulsive behaviour, postural hypotension, daytime sleepiness, specific ergot SE like fibrosis lungs/chest so need constant monitoring ECG/CT
5
Q
Examples of MAOI and cautions related to them
A
- Selegiline, Rasagiline.
- Serotonin syndrome: pyrexia, agitation, sweating, diarrhoea
- Tramadol, Dethidine, MTX are CI
6
Q
When to start drug treatment for PD?
A
No disadvantage starting early, generally for Sx control [esp. L-dopa best for that]
7
Q
Non-motor Sx of PD
A
Mood, sleep [esp. REM sleep], cognitive, impulse control disorder, autonomic dysfunction, anosmia [pts can have Sx for 20y without Dx]
REM sleep disorder: pts acting out their dreams.
Insomnia due to stiffness/restlessness
8
Q
Psychotic PD Tx
A
- assess provoking factors [could be illness, metabolic, infection]
- refer to neuro
- disconintue meds like MAOI
- intitiate clozapine, quitiepine [consider acetylcholine esterase inhibitors]
9
Q
Autonomic Sx PD
A
orthostatic hypotnesion, N and vomiting, hypersalivation, nocturia, sweating etc.
10
Q
When can DBS be used for PD and what is the benefit?
A
used in the subthalamic nucleus, lowers requirement for L-dopa [by about half its dose]
- only for pts with motor fluctutations
11
Q
Sx of a third nerve palsy
A
Parasympethic fibres
Apex petrous part temporal bone
Fixed dilated pupil
12
Q
Sx of cerebellar syndrome
A
Ataxia, nystagmus
ipsilateral
Midraib, pons, medulla
13
Q
What is above and below the brain stem?
A
Above: thalamus, internal capsule
Below: spinal cord
14
Q
Describe the reticulating activating system and its function
A
Peri-aqueductal grey matter/ floor of the 4th ventricle
->
alertness, sleep/awake, REM/nonREM sleep, resp control, CV drive
15
Q
Disorders affecting the brainstem
A
Tumour [meningioma, schwanomma, mets etc,], inflammation [MS], metabolic, trauma, Haemorrhage [AVM, aneurysm], infarct [vertebral artery infarct], infection [cerebellar in ear]
16
Q
What is compensated type 2 respiratory failure?
A
High CO2, normal or low O2, high bicarb [to compensate]
17
Q
Mx of acute neuromuscular respiratory weakness
A
- ABCD
- ABG
- ECG
- supportive Mx respiratory and autonomic instability
- early anaesthetic call
- consider infection
- Tx underlying condition
18
Q
How to investigate MG?
A
- consider culprit [drugs like Mebeverine]
- bloods for ACh receptor antibodies [MuSK]: 85% positive [though takes 3w]
- thyroid function [autoimmune coexistence]
- CT chest [possible thymoma]
- if antibodies there: simple fibre EMG [NM] dysfunction
19
Q
Mx of generalised MG
A
- assess severity
- caution Rx [stop Mebeverine]
- Pyridostigmine 30mg qid inc. to 60 qid and higher [max 450mg per day]
- steroids 10mg al days increasing by 10mg after 3rd dose aiming for 100mg alt days [quicker if IP, beware dips]
- bone protection, PPI
- IVIg
- Tx infections early, aggressively and care with antibiotics
- LARGE list of drugs CI in MG patients
20
Q
What is a typical pyramidal [or UMN] pattern of weakness?
A
- Flexors > extensors in UL
- Extensors > flexors in LL
- consequently, pt circumducts leg to walk as cannot flex hip properly to take step
21
Q
Ix for pt with back pain, difficulty walking, pyramidal weakness
A
- MRI cervical spine [possible compression], LP, admit and observe = DONE IMMEDIATELY
- [NCS, anti-glioside antibodies, infection serology for cause]
22
Q
Mx of GBS
A
IVIg, monitor pulse and BP, monitor FVC
23
Q
Autonomic instability Mx for GBS
A
- tachycardia: drugs/DC cardioversion
- bradycardia: drugs/pacing
- HTN: labetolol
- hypotension: fluids, inotropics
24
Q
Mx of respiratory failure in MND
A
Care with O2, consider infection, NIV on ward, ITU usually not appropriate
25
Origin and function of the pyramidal and extrapyramidal tracts
Pyramidal
- origin: cerebral cortex
- function: voluntary control of muscles and face
Extrapyramidal
- origin: brainstem
- function: involuntary and autonomic control muscles such as tone, balance, posture, locomotion
26
Functionally, how are pyramidal tracts split?
- Corticospinal tracts: lateral [90%, decussate medulla and terminate ventral horn] and anterior [ipsilateral then decussates cervical and thoracic segmental levels]
- Corticobulbar
27
Functionally, how are the extrapyramidal tracts split?
Originate brainstem, motor fibres spinal cord
Four tracts:
- vestibulospinal and reitculospinal do not decussaate, ipsilateral innervation
- rubrospinal and tectospinal contralteral
28
How many somatic pairs of nerve in the NS?
31 pairs
29
UMN vs LMN features
UMN
- bulk: normal, tone: increased, strength: decreased, fasciculations: absent, reflexes: increased
LMN
- bulk: reduced [wasting], tone: normal or decreased, strength: decreased, fasciculations: may be present, reflexes: decreased or absent
30
Causes of weakness
```
Neuropathies
- peripheral neuropathy
- GBS
- MEI
- MND
Myopathies
```
31
Compare three main types peripheral nerve damage
Polyneuropathy [peripheral neuropathy]
Mononeuropathy multiplex [at least 2 neurones]
Mononeuropathy
32
Predominantly motor loss peripheral neuropathy
- GBS
- chronic inflammatory demyleinating polymuerhpathy [CIDP] i.e. chronic GBS
- hereditary sensorimotor neuroapthies [HSMN] e.g. Charcot Marie Tooth Disease
- DIptheria
- Pomphoria? or something
33
Predominantly sensory loss peripheral neuropathy
- deficiency states [B12/folate]
- DM
- alcohol/toxins/drugs
- metabolic abn. e.g. uraemia
- leprosy
- amyloidosis
34
Define mononeuritis multiplex
- Painful, asymmetrical sensory and motor neuropathy
35
Features and causes of mononeuritis multiplex
- Subacute presentation
- Inflammatory/immune mediated
- Causes: vasculitides e.g. Churg-Strauss, CT disorders [like sarcoid]
36
Mononeuropathy exmaples
Median nerve [Carpal Tunnel]
Ulnar elbow
Radial axilla
Common peroneal nerve leg
37
Neuropathy Ix
```
history and exam
- neurpathy screen
vasculitic screen
- EMG/NCS
-CSF study
- imaging/nerve biopsty
```
[all after Hx optional]
38
neuropathy Tx
20% idiopathic with no Tx [just give analgesic]
treat underlying cause [e.g. deficiency/DM]
inflammatory like CIDP then prrednisolone with steroids sparing agents like azathioprine
vasculitic neurpathy give prednisolone with immunosuppreant like cyclophosphamide
39
Features of GBS [incl. on LP]
```
AI response causing demyelination
Post infectious e.g. resp or GI
Subacute [<6w] ascending paralysis/numbness/areflexia
NCS: demyleinating
LP: raised CSF protein
```
40
Tx for GBS
IVIg or plasmapharesis, support, monoitor, FVC, ITU review
41
How long recorvry GBS?
Weeks to years
42
Features of MG
AID; antibodies against nicotinic acetylchline receptors
Common, potentially fatal
Thymus dysfunction common [hyperplasia, thymoma]
Generalised weakness:
- proximal limbs
- neck and face [head drop, ptosis]
- extraocular [complex diplopia]
bublar Sx [speech and swallow] pareticularly in elderly
ocular in 10-25% pts
risk other AID like thyroid, PA
43
Ix for MG
Tensilon test [allow accumulation of ACh in the NMJ]
- AChR antibodies
- EMG [look at NMJ]
- CT thorax
44
Tx for MG
- acetylcholine esterase inhibitors [Pyridostigmine]: in symptomatic
- immunosuppresants [steroids start slwoly, azathioprine/MTX/mycophenolate]
- thymomectomy
45
What is a myasthenic crisis?
- Severe weakness incl. respiraotry muscles
- High risk of death
- Cause: infection, natural disease cycel, under/overdosing
46
Mx for myasthenic crisis?
Take change in myasthenic condition seriously
urgent revierw neurologist
Think about breathing and monitor
Anaesthetic review
47
Simple definition of MND
Slelective degeneration of motor neurones in the motor cortex and anterior horn of the spinal cord [also CN]. presents with LMN and UMN signs.
48
Ix for MND
LP, NCS/EMG, MRI [to r/o]
49
Clinical features of MND
No sensory, visual, or B/B involvement
Asymmetrical weakness
Bulbar and limb onset
Survival typically 2-5y
50
Common muscle disorders
- steroid myopathy
- statin myopathy
- metabolic and endocrine myopathies
- myotonic dystrophy
51
List some uncommon muscle disorders
```
Most muscular dystrophies
- Duchenne, FSHD
Inflammatory muscle disorders
- polymyositis, dermatomyositis
Mitochondrial disorders
```
52
Features of muscle disroders
Stairs, chair, hair
wasting is common
Facial weakness occurs in some [FSMD]
Neck weakness and contractures can occur
Scoliosis often feature especially in Duchenne
Eye movements disorders are rare [seen in mitochrondrial]
53
Muscle Ix
CK, EMG, ESR/CRP,
| +/- genetics [like DMD], biopsy
54
General rule, nerve compared to muscle disease?
```
Nerve = distal weakness
Muscle = proximal weakness
```
55
Aetiology of MND
Sporadic [unknown], or familial [SODL, C9ORF2]
56
Types of MND
- ALS [50]: LMN and UMN. Worse bulbar onset.
- PLS: motor cortex only, so UMN
- PMA: LMN, best prognosis
- PBP: CNs 9 to 12, palsy tongue, chewing and swallowing. Worse prognosis.
57
Features of MND
Sx: weakness, bublar Sx [dysarthria, dysphagia, siarrlorrhoea]
Sx: assymetrical pattern weakness, ALS both UMn and LMN signs
Ix: no Dx test, neurophysioogy/EMG may detect denervation, MEI helps excl, LP to r/o
58
Mx for MND
Riluzole, NIV, gastrostomy
59
What are PD associated with?
Lewy bodies [tangles alpha-synuclein and ibiquitin]
60
Ix for PD
usually clincial Dx, MRi and CT to r/o, PET and SPECt scans [alsdo DaTSCAn]
61
Define Huntington's Disease
Inherited neurodegenerative disease characterised by chorea, dystonia, incoordination, cognitive decline and benhioural difficulties
62
Aetiology of HD
AD [complete penetrance], gene chromosoem 4 encodes for Huntingtin, degneration of cholinergic and GABAnergic neurones. CAG nucleotides need over 35 repeats.
63
Features of HD
after 35 y.o, chorea, personality changes, intellectual impairment, dystonia, saccadic eyes, rigidity, dementia, bulbar Sx, sleep problems
64
Ix for HD [incl. part of brain looked at]
genetic testing, CT/MRI can show atrophy of caudate nucleus
65
Mx and prognosis for HD
No DMT, 15-20 years life
66
Define dementia
Syndrome deteriorating memory, thinking, behaivour, and ability for ADLs
67
Types of dementia
AD [50%], vascular [25%], LBD [15%], FTD [5%], mixed dementia, PD+ genetic causes dementia
Also, potentially Tx causes dementia
68
potentially Tx causes of dementia
Substance abuse, hypothyroidism, SOL, NPH, syhpilis, vit. B12 defi, pellagra
69
Dx of dementia
histor yna dexam, screen impairment with e.g, MMSE
70
Ix for dementia
dementia screem, r/o other causes confusion like FBC, LFTs, U and E, EST, TFT, syphilis, cvortisol, glucose, CT head, EEG, LP [CJD]
71
Mx of dementia
acetyolcholinesterase inhibotres [e.g. donepezil] for mild to mod AD
NMDA [memantine] for 2nd line where ACHe doesn;t work
72
What is PSP?
Supranuclear palsy or Parkinson plus syndrome;
| impairments vertical gaze [hard stairs], parkinsons, falls, speech, cognitive impairment
73
Mx of PSP
poor repsonse to L-dopa
74
What is TGA~?
Loss transient memory
anxious and repeat questions
no recall events
75
Chorea cause
daamge basal ganglia
| - HD, RFs, drugs [l-dopa antipsychotics], thyrotoxicoiss, PRV, poison, ataxis trelangactasia etc.
76
Lewy body dementia Sx
```
Progressive cognitive decline:
- attention and EF decline first compared to memory loss
cognitive fluctutating
- usually CD before Parkinsons
- visual hallucinations
```
77
Cx of stroke
```
raised ICP [cerebral oedema, haemorrhage, [signs HTN, new neurology, GCS]
- aspiration
pressure sores
depression
cognitiv e impariemtn
othert medical problems]
```
