Clinical neurology Flashcards
(99 cards)
1
Q
Bilateral sensory involvement LMN diseases
A
Polyradiculopathy
Polyneuropathies- Guillain Barre
Mononeuritis multiplex
2
Q
What is Guillain-Barre?
A
Acute inflammatory demyelinating polyradiculoneuropathy
Often follows gastroenteritis- commonly campylobacter jejuni
Progresses over days to weeks and may lead to respiratory muscle failure
3
Q
Possible causes of polyneuropathies
HINT: A DANG THERAPIST
A
Acquired or inherited Diabetes Amyloid Nutritional- B12 deficiency Guillain Barre Toxic Hereditary- Charcot Marie Tooth, Friedrick's ataxia Endocrine Recurring/ Renal Alcohol Pb toxicity/ porphyria Infection Systemic- SLE, sarcoid, hypothyroid, syphilis Tumours- paraneoplastic
Around 50% are idiopathic
4
Q
How is Duchenne muscular dystrophy inherited?
A
X-linked recessive
Dystrophin absence
5
Q
Signs of Duchenne muscular dystrophy
A
Proximal lower limb weakness from the age of 4, gradually spreads to rest of muscles over time
Calf pseudohypertrophy (from infiltration)
Eventual spread to cardiac and respiratory muscles
Gower’s manoeuvre (when asked to stand from sitting)
6
Q
Acquired myopathies usual clinical features
A
Proximal muscle weakness, usually symmetrical and more weakness than pain
Polymyositis/dermatomyositis
Often affect the pelvic girdle more than the shoulder
7
Q
Investigations into acquired myopathies
A
CK- in the 1000s ESR EMG to confirm spontaneous activity Biopsy Genetic analysis Antibody testing
8
Q
LMN, bilateral, purely motor potential issues
A
Myopathy- acquired- inflammatory, endocrine, metabolic- hereditary
Anterior horn cell
NMJ- MG, MG crisis, Lambert-Eaton, botulism
9
Q
Presentation of myasthenia gravis
A
Fluctuating weakness
Proximal, ocular and bulbar- ptosis, diplopia, dysphonia, dysarthria, dysphagia
MG facies- snarl
Fatiguability
No atrophy/ fasciculations, normal tone and reflexes as not a nerve pathology `
10
Q
What is the Tensilon Test?
A
Short acting anticholinesterase is given IV to see if there is any improvement
11
Q
What imaging is done in MG?
A
CT of thymus looking for thymoma
12
Q
Treatment for MG
A
Anticholinesterase inhibitors- pyridostigmine, propantheline
Immunosuppression- especially in acute- pred, IVIg, plasmapheresis
Thymectomy
13
Q
Unilateral LMN signs
A
Radiculopathy
Plexopathy
Mononeuropathy
14
Q
Causes of plexopathy
A
Features reflect motor and sensory findings extending over multiple nerve roots Trauma- Erb's palsy and Klumpkeys Neuralgic amyotrophy Thoracic outlet syndrome Malignant infiltration Radiotherapy (may be years later) Compression
15
Q
Radiculopathy clinical features
A
Shooting, electric, sharp pain radiating down the limb
Can be across multiple levels if polyradiculopathy
16
Q
ALS MND stands for
A
Amytrophic lateral sclerosis- Motor neuron disease
17
Q
ALS differentials
A
UMN and LMN signs cervical spondylotic radiculomyelopathy Spinal tumour Spinal conus medularis lesions Vit B12 deficiency- sub acute degeneration of the cord and peripheral neuropathy
18
Q
Treatment for ALS
A
No cure Riluzole used to prolong life (1-2 months) Supportive- CPAP/NIPPV PEG
19
Q
What is MS?
A
Demyelinating disorder of the CNS
20
Q
Who gets MS?
A
Classically white, young women who live at the poles
21
Q
Types of MS
A
Clinically isolated syndrome
Relapsing-remitting
Primary progressive
Secondary progressive
22
Q
How can MS be diagnosed
A
Two events demonstrating dissemination over space and time
Can be done faster now by radiological imaging
23
Q
Management of MS
A
Acute- corticosteroids to rapidly induce recovery
Disease modifying therapy- interferons and monoclonal antibodies
Symptomatic management- spasticity, bladder, bowel, pain, depression/low mood
MDT
24
Q
Types of trigeminal autonomic cephalalgias
A
Cluster headaches
Paroxysmal hemicrania
Short-lasting unilateral neuralgiform headache (with conjunctival injection and tearing)
25
Cluster headache important other differentials
Migraine
Other types of TACs
Secondary causes of chronic headache
Hypnic headache
26
Primary headache causes
Migraine
Tension type
Trigeminal autonomic cephalalgias
Other- exercise/ sex-induced
27
Secondary headache causes
Injury
Medication overuse
Cranial or cervical vascular disorder (carotid or vertebral artery dissection)
Venous sinus thrombosis
Non-vascular- idiopathic intracranial hypertension
Painful cranial neuropathies
28
Presentation of venous sinus thrombosis
Recurrent presentations
Procoagulant state
Headache with signs of raised ICP
Normal CT head (possibly anti-delta sign)
29
Signs of idiopathic intracranial hypertension
Papilloedema- compression of the optic nerves and subsequent visual loss
Other cranial neuropathies
Raised LP opening pressure
30
Management of IIH
Conservative- analgesia, weight loss (ultimate treatment), life-style
Medical- Acetazolamide, topiramate, furosemide
CSF drainage/ shunting
31
Acetazolamide
MOA: Carbonic acid anhydrase inhibitor
S/E: haemorrhage, metabolic acidosis, nephrolithiasis, abnormal sensation
AVOID in pregnancy (especially 1st trimester) and sulphonamide reactions
32
Causes of cerebellar dysfunction
| HINT: MAVIS
```
MS
Alcohol
Vascular
Inherited
Space occupying lesion
```
33
Pyramidal tracts
Originate in the cerebral cortex
| Voluntary control of musculature of the face and body
34
Extrapyramidal tracts
Originate in the brainstem
| Involuntary and autonomic control of musculature- tone, balance, posture and locomotion
35
What is Uhthoff's phenomenon?
Transient worsening of neurological symptoms when the body becomes overheated
36
Lhermitte's phenomenon
Electric shock like complaint down the spine from flexion of the neck due to a cervical plaque
37
Clinical findings of MS due to optic nerve involvement
RAPD
Central scotoma
Red desaturation
Optic disc atrophy
38
Causes of carotid artery dissection
Traumatic- hyperextension
Iatrogenic- previous surgery, endartectomy
Spontaneous- HTN, smoking
Connective tissue disorder- Marfan's, Ehlers-Danlos
39
Signs and symptoms of a carotid artery dissection
```
MCA thrombotic events
Headache
Amaurosis fugax
Limb/facial weakness
Neck swelling
Horner's (partial if post-ganglionic)
```
40
What is Eagle syndrome?
Rare mimic of carotid artery dissection- calcified or elongated stylohyoid ligament compresses the carotid artery
41
Treatment of carotid artery dissection
Conservative- modification of risk factors
Medical- aspirin
Surgical- reconstruction
42
Miller-Fisher syndrome
Less severe version of Guillain-Barre which causes ophthalmoplegia, areflexia, ataxia
Always self-limiting, does not require treatment
Anti-GQ1b antibodies
43
Tests to diagnose MND and why
```
TFTs- thyrotoxicosis
HbA1c and BM- diabetic amyotrophy
CK- polymyositis/ dermatomyositis
EMG- confirms MND pattern of fibrillation and fasciculations and loss of lower motor neurons
Normal nerve conduction
Genetic analysis
```
44
Typical pattern of presentation of MND
| initial, progression and sparing
Isolated limb weakness (reduced grip or foot drop) which progresses to truncal and bulbar involvement
Progressive bulbar palsy- dysarthria and dysphagia
Usually spares extraocular muscles and sensory and autonomic systems
45
What enhances active MS plaques on MRI T2
Gadolinium
46
Differentials for myasthenia gravis
```
Myasthenic crisis
Lambert-Eaton Myasthenic syndrome
Polymyositis
Dermatomyositis
Botulism
```
47
Clinical features of GB
Back/leg pain in the initial stages
Progressive, symmetrical weakness of the limbs, classically ascending from the legs first, proximal muscles affected first
Reduced or absent reflexes
Mild sensory symptoms
48
Autonomic dysreflexia occurs in patients that have...
A spinal cord injury (often traumatic) above the level of T6
| Most commonly triggered by constipation or urinary retention
49
Features of multisystem atrophy
Parkinsonian features
Cerebellar signs- ataxia
Autonomic disturbance:
Postural hypotension
Erectile dysfunction
Atonic bladder
50
Features of a bacterial meningitis LP
```
Turbid appearance
Elevated opening pressure
Very raised WCC
Raised protein
Low glucose
```
51
Features of a viral meningitis LP
```
Clear appearance
Normal opening pressure
Lymphocyte predominance, raised WCC
Slightly raised protein
Normal glucose
```
52
What is Hoover's sign of leg paresis used to distinguish between?
Organic and non-organic paresis of the leg
Synergistic contraction of the contralateral leg if the patient is genuinely trying, no contraction suggests a functional/ non-organic cause
53
Management of possible TIA if the patient is anticoagulated
A&E for CT head to exclude haemorrhagic stroke- do not give aspirin 300mg until this is done
54
Conduction dysphasia
Classically due to a stroke affecting the arcuate fasciculus- connecting Broca's and Wernicke's areas
Speech is fluent and comprehension is intact but poor repetition
55
Clinical features of neuroleptic malignant syndrome
Rigidity, hyperthermia, autonomic instability, altered mental state- agitated delirium with confusion
Can cause AKI
56
Management of neuroleptic malignant syndrome
Stop causative antipsychotic
IV fluids to prevent renal failure
Dopamine agonist (bromocriptine)
Dantrolene, muscle relaxer
57
Stroke management
Exclude haemorrhage (CT head)
Aspirin 300mg (and then for 14 days)
Thrombolysis within 4.5 hours if no contraindications
+ thrombectomy within 6 hours
58
Secondary stroke prevention
Follow up in primary care at 6 months and annually
Lifestyle modification
Clopidrogrel 75mg daily
Aspirin if not tolerated and dipyridamole if both not tolerated
Duel aspirin and clopidogrel for 3 months may be initiated
Statin
Treatment for HTN
Weigh up anticoagulation risk vs benefit
Optimise co-morbidities
59
Triad of Wernicke's encephalopathy
Ataxia, ophthalmoplegia (nystagmus most commonly) and confusion
Peripheral polyneuropathy may be seen
60
Treatment of Wernicke's encephalopathy
Urgent replacement of thiamine
| IV pabrinex- vit B/C
61
Causes of a third nerve palsy
```
Vasculitis
DM
Posterior communicating artery aneurysm
MS
Weber's syndrome- ipsilateral third nerve palsy with contralateral hemiplegia- midbrain strokes
Amyloid
```
62
Features of progressive supranuclear palsy
Parkinsonian features- particularly early loss of balance when walking
More rapidly progressive
Ophthalmic and bulbar symptoms more pronounced- problems controlling eye movements and blurring of vision predominant
63
Features of corticobasal degeneration
Cortical and basal ganglia involvement
Parkinsonism
Often presents unilaterally and then progresses bilaterally
Apraxia, limb dystonia, cognitive features, visual-spatial impairment
64
Cushing's triad and other features of raised ICP
Irregular breathing
Widened pulse pressure
Bradycardia
Papilloedema
Headache
Vomiting
Reduced consciousness
65
LP of bacterial meningitis
```
Cloudy and turbid appearance
Elevated opening pressure
Primarily polymorphonuclear leukocytes- neutrophils elevated
Low glucose
Raised protein
```
66
LP of viral meningitis
```
Appears clear
Normal or elevated opening pressure
Raised WBC- primarily lymphocytes
Normal glucose
Raised protein
```
67
LP of fungal meningitis
```
Clear or cloudy appearance
Elevated opening pressure
Elevated WBC
Low glucose
Elevated protein
```
68
LP of TB meningitis
```
Appears opaque
Elevated opening pressure
Elevated WBC
Low glucose
Grossly raised protein
```
69
LP of subarachnoid haemorrhage
```
Blood stained initially
Xanthochromia after 12 hours
Elevated opening pressure
Elevated WBC
Elevated RBC
Elevated protein
Normal glucose
```
70
Guillain Barre LP results
Albuminocytologic dissociation- raised protein without a raised WBC
71
Indications for LP
Diagnostic- MS, GB, meningitis, subarachnoid haemorrhage, encephalitis, idiopathic intracranial hypertension, normal pressure hydrocephalus
Therapeutic- IIH
72
Normal pressure hydrocephalus triad of signs
Instability- ataxic gait
Cognitive dysfunction
Incontinence
73
Contraindications for LP
Meningococcal septicaemia
Intracerebral haemorrhage
Raised ICP in a non-communicating hydrocephalus
Congenital malformations- chiari, spina bifida
Coagulopathy or anticoagulated
74
Meniere's disease
Increased pressure of the endolymphatic system leading to recurrent attacks of vertigo lasting >20 minutes but <24 hours
Fluctuating sensorineural hearing loss
Aural fullness
Tinnitus
75
Vestibular schwannoma clinical features
Unilateral sensorineural hearing loss
Slow progression leading to CN being effected- 5,6,7,9,10
Absent unilateral corneal reflex
Bilaterally associated with NF2
76
BPPV clinical features
Occurs on head movement due to disruption of debris
Brief episodes of mild-intense vertigo
Dix-Hallpike manoeuvre shows fatigable nystagmus
Epley manoeuvre clears debris
77
Vestibular neuronitis clinical features
Recent viral infection
No deafness or tinnitus
Abrupt onset, severe, associated with nausea and vomiting
78
Drugs that can cause ototoxicity
Loop diuretics
| Aminoglycosides ie gentamycin
79
Spinal cord tract changes in sub-acute degeneration of the spinal cord
Dorsal column loss causing sensory and LMN signs
Corticospinal cord tract loss causing UMN signs
Spinothalamic tracts remain intact and so there is no pain or temperature change even in extreme cases
80
How many beats of clonus is normal?
3 or less
81
Management of cluster headaches
Keep calm, 100% high flow oxygen for 15 mins and sumatriptans at onset
Education to avoid triggers- could be alcohol
Consideration of corticosteroids in the short term or verapamil prophylaxis
82
Treatment of trigeminal neuralgia
Patient education
Carbamazepine prophylaxis or lamotrigine/ phenytoin/ gabapentin
Surgery can be used if medication fails- microvascular decompression
83
Triggers of migraines
```
Chocolate
Hangovers
Orgasms
Cheese/ caffeine
Oral contraceptives
Lie-ins
Alcohol
Travel
Exercise
```
84
Differentials for migraine headaches
```
Trigeminal autonomic cephalalgias (including cluster)
Tension headache
Cervical spondylosis
Raised ICP
TIA
```
85
Management of migraines
Patient education about triggers
Medical prophylaxis- propranolol, topiramate
During an acute attack- oral triptan combined with ibuprofen or paracetamol. Anti-emetics. Warm or cold compress, rebreathing into a paper bag. 10 sessions of acupuncture
86
What is a Stokes-Adams attack?
Transient arrhythmia eg bradycardia due to incomplete heart block resulting in decreased cardiac output nad LOC
Falls to the ground with no warning except palpitations, injuries are common as a result of this (screen for these!!)
Pale with a slow or absent pulse
Recovery is within seconds but if prolonged LOC there may be anoxic clonic jerks
87
Blackouts differentialas
```
Vasovagal syncope- reflex bradycardia and peripheral dilation (situation syncope if triggered specifically)
Carotid sinus syncope
Epilepsy
Stokes-Adams attacks
Hypoglycaemia
Postural hypotension
Anxiety- no LOC
Factitious
```
88
Differentials for unilateral foot drop
```
DM
Early MND
MS
Stroke
Common peroneal palsy
Prolapsed disc
```
89
Spastic gait description
Stiff
Circumduction of the legs +/- scuffing of the toes
UMN lesions
90
Acute management of stroke
A-E assessment
CT head to rule out haemorrhage
NBM until swallow screen
300mg aspirin for 14 days
Consider thrombolysis as soon as haemorrhage is ruled out (within 4.5 hours of onset)- best results within 90 minutes- Alteplase
Thrombectomy within 6 hours if large artery occlusion in the proximal anterior circulation
91
Secondary prevention of stroke
Control risk factors- treat HTN, hyperlipidaemia, DM, cardiac disease, AF, smoking
14 days of 300mg aspirin
Switch to long-term clopidogrel monotherapy 75mg OD
MDT approach- PT, OT, SALT, specialist nurse, stroke team
Rehabilitation on a stroke ward and into the community
Tests
24 hour ECG
HTN- retinopathy, neuropathy, cardiomegaly
Carotid artery stenosis- carotid doppler US +/- CT angiography
92
Pharmacological treatment of cognitive decline
Acetylcholinesterase inhibitors- donepezil, rivastigmine, galantamine- recommended by NICE for dementia
Memantine- NMDA antagonist- late stage AD
Antipsychotics acutely if extreme agitation or psychosis
Vitamin supplementation
93
Todd's palsy/paresis
Weakness following a focal seizure in the motor cortex
94
Extrapyramidal triad of Parkinson's disease
Resting tremor (unilateral presentation, gets better on voluntary movement)- often pill rolling
Hypertonia- cogwheel rigidity
Bradykinesia
95
What is Devic's syndrome/ NMO
Neuromyelitis optica
MS variant with transverse myelitis, optic atrophy
Anti-aquaporin 4 antibodies
96
MND with only LMN signs
Progressive muscular atrophy- anterior horn cell lesion so only LMN
97
MND definition
Cluster of neurodegenerative diseases characterised by selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells
UMN and LMN with bulbar inclusion
No sensory or sphincteric disturbance
NEVER affects eye movements
98
Signs of neurofibromatosis type 1
Cafe-au-lait spots, adults have more than 5, do NOT predispose to skin cancer
Dermal neurofibromas and nodular neurofibromas, increase in number with age
Lisch nodules on the iris
Mild learning disability common
Malignancy in 5%
Autosomal dominant
99
Signs of neurofibromatosis type 2
Bilateral vestibular schwannoma are characteristic
Usually become symptomatic around 20 years old
Meningiomas
Autosomal dominant
