Neurology Flashcards
(208 cards)
1
Q
What is epilepsy?
A
(a) 2 unprovoked seizures occurring >24hrs within each other.
Or
(b) 1 unprovoked seizure where the recurrence probability within 10yrs is same as recurrence rate of (a).
Or
(c) diagnosed with epilepsy syndrome.
A seizure is a single event in time whereas epilepsy is the tendency to have multiple seizures.
2
Q
What are the RF for developing epilepsy?
A
Mainly unknown Premature birth Dementia/neurodegenerative disorders Head trauma/infection FHx Cerebral malformation Co morbid conditions- common with older people (stroke, cerebrovascular disease) Vascular Infection Trauma Tumour
3
Q
What are the causes/triggers of seizures?
A
Cerebral tumours Intracranial infections Head injury Illicit drugs Alcohol (acute intoxication/withdrawal) Hypoxia Stroke Low Na, Ca and Mg
4
Q
What should be asked about in an epilepsy history?
A
Presence of triggers?
Before the attack- any aura?
Attack- short lived? Sudden muscle tone loss? Any generalised stiffening, rhythmic jerking of limbs, urinary incontinence, tongue biting? Any behavioural arrest? Any brief shock like jerks?
After attack- Any post ictal phenomena? (Have some remaining symptoms I.e. amnesia, headache, drowsiness).
5
Q
How would a suspected seizure be investigated?
A
Physical examination-
Oral mucosa- Any signs of lateral tongue biting?
Any injuries sustained during the attack?
Cardiac, mental state, neuro and maybe developmental assessment.
Baseline tests including FBC, U+Es, LFTs, Ca and glucose.
12 lead ECG.
6
Q
What are the different types of seizures?
A
Generalised tonic-clonic seizures
Focal
Myoclonic
Absence
7
Q
What is a generalised tonic-clonic seizure?
A
Tonic:
First phase where there is a loss of consciousness and the Px may fall. Strong spasms of muscles forcing air out of the lungs, cry/moan.
Can have foam/saliva from the mouth, tongue biting and impaired breathing due to stiffness.
Clonic:
Jerking movement of face, arms, legs becoming rapid and intense.
After 1-2 mins slows down and body relaxes, including bladder and bowel.
Px lets out deep sigh and normal breathing is restored.
Post ictal phase:
Px can remain unconscious for a few minutes until the brain recovers.
Regains awareness, may be exhausted/confused/sore for few hrs.
May have post ictal amnesia.
8
Q
What is a focal seizure?
A
Complex partial seizure. Lasting 30 seconds to 1 minute. Aura may occur as a warning (visual, auditory, olfactory, fright, unfamiliarity to a place etc) Lip smirking Hand at the chest Stop behaviour momentarily
9
Q
What is a myoclonic seizure?
A
Sudden, brief involuntary muscle jerks (similar to when everyone foot jerks in bed).
May be mild, affecting one part of the body.
May be strong enough to throw a person on the floor.
More clear in extreme scenarios I.e. Px falling down or car accident.
10
Q
What is an absence seizure?
A
Common in children, most grow out in teenage years.
Momentary loss of awareness.
Looks like a vacant/blank stare.
Lasting less than 30 seconds, occurring a few times a day.
Eyes turn upwards and eyelids flutter
Don’t realise they have had the seizure and carry on where they left off.
11
Q
How is the first seizure in epilepsy managed?
A
Needs immediate specialist referral
Advise to stop driving until seen specialist
Advise Px and family of possible epilepsy diagnosis
Advise on triggers of seizures i.e. alcohol, sleep deprivation etc
Advise witness of seizure to attend with the Px to see the specialist.
Discharge with outpatient MRI and baseline EEG.
12
Q
How is a generalised tonic-clonic seizure managed?
Same management for unclassifiable seizures
A
Px is woman of child bearing potential;
First line AED- Lamotrigine
Alternatives- Levatiracetum, Valporate
Px is not a woman of child bearing potential;
First line AED- Valporate
Alternatives- Lamotrigine, Levatiracetum
13
Q
How is a focal seizure managed?
A
First line AED- Carbamazepine
Alternatives- Lamotrigine, Levatiracetum, Valporate, Phenytoin
14
Q
What is status epilepticus?
A
This is a medical emergency.
Defined as having a seizure lasting >5 minutes or >1 seizure within 5 minutes.
Can lead to permanent brain damage or even death.
15
Q
What are the causes of status epilepticus?
A
AED withdrawal Non compliance Alcohol use/withdrawal Illicit drug Current infection Progression of underlying disease (tumour, encephalitis etc)
16
Q
How is status epilepticus managed?
A
ABCDE
Start timing seizure
>5mins give 4mg IV lorazepam. (In the community would give buccal midazolam or rectal diazepam if unavailable or preferred) If uncertain of duration of seizure still give.
Repeat again if after 5 mins still seizure
Prepare the phenytoin incase the seizure still persists.
If >25mins seizure give 20mg/kg over 20mins of phenytoin.
IV valproate
Notify ITU.
Refractory status >30mins
General anaesthesia-high dose Propofol
Continue for 12-24hrs
17
Q
What is the differential for epilepsy?
A
Vasovagal syncope
More likely epilepsy if Px has cyanosis, lateral tongue bite, post ictal amnesia(not confusion), rhythmic tonic clinic jerk, duration >2mins.
Less likely epilepsy- tongue tip bite, pallor, post event fatigue, brief twitching and jerking, associated trigger- may encourage collapsing.
Both can have incontinence, confusion on arousing.
18
Q
How would you address a neurological problem?
A
Take a detailed Hx.
Look at distribution of symptoms
Onset- if sudden usually vascular
Type of symptoms? Motor or sensory?
These can help localise the lesion to a location- left/right/cerebellum/brainstem etc.
19
Q
What are the causes of neuromuscular weakness?
A
Motor neurone disease Myasthenia gravis Guillain Barre syndrome Muscular dystrophies Multiple sclerosis
20
Q
What is myasthenia gravis?
A
Disease of the neuromuscular junction.
Autoantibodies bind to the Ach receptors.
Leading to muscle weakness with easy fatigability.
Increases with exercise relieves with rest.
Varies from mild weakness of a few muscle groups to more severe weakness of several muscle groups.
21
Q
How is myasthenia gravis classified?
A
Based off :
Antibody specific- The antibody specificity: acetylcholine; muscle-specific receptor tyrosine kinase (MuSK), LRP4 or seronegative.
Thymus histology- Thymitis, atrophy or thymoma.
Age of onset- Bimodal distribution (females usually get early, males usually late)
Course- Ocular or generalised
22
Q
What is seronegative myasthenia gravis?
A
Seronegative to usual test but may have MUsk.
Usually female, <40yrs, 1/3 don’t respond to acetylcholinesterase.
23
Q
How does myasthenia gravis present?
A
The main and usually first presentation is ocular symptoms.
Most then progress to generalised MG.
Muscle fatigue post exercise:
-Ask Px to count to 50 then notice their voice is less audible as they fatigue.
-Can ask Px to look at a point over their forehead, can’t do this for more than a few seconds if ocular involvement.
Ptosis, diplopia.
Weakness of proximal muscles
Symmetrical weakness of muscles can lead to difficulty walking, sitting, holding head up.
Muscle tone, reflexes and sensation is normal, no muscle wasting.
May have seizure.
Bulbar involvement
24
Q
How does myasthenia gravis progress?
A
Disease progression occurs in the first year, if not second year of diagnosis. If has not progressed after this, then less likely to progress.
If restricted to extrinsic ocular muscles and LPS then ocular myasthenia.
Remission is rare, although if they do occur most will occur in the first 3 yrs of treatment.
Respiratory compromise- Weakness of muscles of ventilation or pharyngeal muscles can lead to this. Need ventilation, monitor O2 sats and vital capacity.
25
How is myasthenia gravis investigated?
If serum AChE is +ve then no further testing required.
Clinical diagnosis + detection of specific antibodies (i.e. anti-AChR, anti-MuSK or anti-LRP4) + ice test for ptosis.
TFTs
Negative serology can warrant MRI brain
Thymus CT/MRI
EMG- repetitive nerve stimulation to look for fatigability
CT thorax
26
How is myasthenia gravis managed?
First line- AChE inhibitors (pyristagmine) along with immunosuppression.
If unresponsive then corticosteroids, azathioprine or thymectomy (beneficial with/out thymoma present) are used as first line.
[Alternatives to azathioprine include ciclosporin, methotrexate or cyclophosphamide.]
Rituximab is becoming increasingly used.
Swallowing difficulty- Dietary advice.
New born babies can have transient myasthenia from myasthenic mother, regardless of if she is well controlled- need neonatal high dependency support.
27
What is myasthenia crisis?
Refers to worsening muscle weakness leading to respiratory failure requiring intubation and mechanical ventilation.
Occurs in first yr, can be first indicator of MG.
Increased risk post surgery or RTI.
Manage with immunoglobulins, corticosteroids, and plasma exchange.
28
How would you distinguish between a cholinergic crisis and a myasthenic crisis?
Cholinergic crisis would occur after having too much anticholinergic medicine. Px presents with muscle fasciculation, pallor, sweating, hypersalivation and small pupils.
29
DifferitatingH
Brisk reflex- UMN (up going toe)
| Missing reflex- LMN
30
Areas and symptoms
Brain- left/right- opposite side
| Brain stem- stroke
31
Double vision differential
Cranial nerve? 3/6th
MG
Thyroid eye disease (asymmetric infiltration)
32
Apthophysiology
Thymidine tumour/hyperplasia
| Associated with other Ai diseases I.e SLE, RA, thyroid etc
33
Precipitants of MG
Emotional stress
Pregnancy
Messes
Drugs- aminoglycosides, BB, CCB, chloroquine etc- can still give but be aware of precipitating symptoms
34
What is Guillain Barre syndrome (GBS)?
Affects the PNS- demyelination and axonal degeneration resulting in acute, ascending and progressive peripheral neuropathy.
Weakness, paraesthesia and hyporeflexia.
Usually triggered by an infection
Clinical diagnosis
Length dependent neuropathy- i.e. the nerves further away e.g. the feet are affected first.
35
What is the pathophysiology of GBS?
Usually following a GI or respiratory infection; Campylobacter jejuni, HIV, EBV, CMV.
Thought the antibodies to the infectious organisms also attack the peripheral nerves antigens.
36
What are the subtypes of GBS?
AIDP- 95% of GBS
AMAN
AMSAN
Dyautonomia- extremely disabling; vision, dry mouth, urination, can’t get up when extremely flat since low B
37
What are the RF for developing GBS?
| What are the complications of GBS?
Respiratory/GI infection 1-3wks prior.
Zika virus.
Vaccinations: live and dead vaccines have been implicated.
Malignancies - eg, lymphomas, especially Hodgkin's disease.
Pregnancy: incidence decreases during pregnancy but increases in the months after delivery.
Complications- Respiratory failure, aspiration, DVT autonomic
38
How does GBS present?
```
LMN signs
Muscle weakness-
Starts at lower extremities and ascends symmetrically.
Max severity reached after 2wks
Facial weakness, dysarthria, dysphagia.
Severe- Respiratory failure
```
Neuropathic pain, especially in legs and back.
Reduced/absent reflexes
Sensory symptoms starting at lower extremities. Glove and stocking pattern of sensory loss.
Autonomic symptoms: reduced sweating, reduced heat tolerance, paralytic ileus and urinary hesitancy. Severe autonomic dysfunction may occur.
39
How would GBS present on examination?
Hypotonia.
Demonstrable altered sensation or numbness.
Reduced or absent reflexes.
Fasciculation may occasionally be noted.
Facial weakness - may be asymmetrical.
Autonomic dysfunction - fluctuations of heart rate and arrhythmias, labile blood pressure and variable temperature.
Respiratory muscle paralysis.
40
How is GBS investigated?
Diagnosis is clinical but following investigations may prove useful.
Electrolytes- Some get SIADH
Lumbar puncture- Most have increased CSF protein count
Antibody screen
Spirometry- FVC can indicate need for ICU
Nerve conduction studies
ECG- 2nd/3rd AV block, ST depression etc.
Stool culture/throat swab- looking for cause.
Otherwise prognosis if much different if not improved after 4 weeks- CIDP- treated then improve then treated. Multiphasic
41
How is GBS managed?
| What is the prognosis of GBS?
```
Plasma exchange
IV immunoglobulin if started within the first 2wks of onset can be as useful as PE.
DVT prophylaxis
ITU admission
Pain relief
```
Most make a full recovery, 20% still have some neurological problems, where 50% of these are severely disabled.
42
What are muscular dystrophies?
Inherited disorders of muscle weakness.
Different depending on age of onset, severity of muscle weakness, muscles affected etc.
Inherited gene leads to lack or deficiency in a particular protein. This leads to damaged muscle fibres and muscle weakness. The resulting weakness will be dependent on the gene and protein involved.
43
How do muscular dystrophies present?
```
Muscle weakness
Can occur at any age-
Baby- floppiness
Older babies/young children- Failure to reach motor milestones
Muscle wasting
Muscle hypertrophy
Myopathy
Contractures
Some types of MD can affect the heart. In some cases, there may be symptoms of heart disease without much in the way of muscle weakness.
```
44
What are the different types of muscular dystrophies?
Duchenne MD- Most common and most severe. Affecting arms and legs, with early muscle weakness. Age 12- need wheelchair. Affects boys but girls carriers. Heart and breathing problems become serious- live till 20yrs old.
Becker's MD- Similar to DMD but les severe and occurs aged 20 so at 40/50yrs old unable to walk with developing heart and breathing problems. Boys affected, women carry.
Limb-girdle MD- Affect top of arms and legs. Severity dependent on type, affects both genders.
Fascioscapulohumeral MD- Affecting males and females, muscles of the face/shoudlers/upper arm. Onset 40-50yrs.
Emery Driefuss MD- Child/Adolescence. Affecting muscles of shoulder/upper arms and lower limbs.
Oculopharyngeal MD- Onset 50-60yrs, affecting muscles of eyes/throat. Present droopy eyes/difficulty swallowing.
45
How are muscular dystrophies investigated?
```
Bloods- Elevated CK
Muscle biopsy- protein analysis
Genetic analysis
Electromyogram- observes muscle activity.
Muscle US
```
46
How are muscular dystrophies managed?
```
No cure
Physiotherapy- For joint mobility
Steroids- For muscle strength
Practical aids
Follow ups
Specific treatment i.e. of contractures.
```
47
What is motor neurone disease (MND)?
| What are the main RF?
Neurodegenerative condition affecting the brain and spinal cord leading to paralysis and eventually death.
Commonly due to ALS (amyotrophic lateral sclerosis)
Other forms do exist and may occasionally occur, although rare.
Male, increasing age (60-70yrs), genetics.
48
What is the pathophysiology of MND?
Cause is unknown although importance of mitochondrial abnormality and oxidative stress of motor neurones is a possibility.
Leads to a LMN and UMN picture of muscle paralysis with LMN signs predominating.
49
How does MND present?
Progressive muscular weakness, first presenting as isolated symptoms.
First presents with emotional lability, behavioural change, FT dementia.
Functional muscle weakness (falling, reduced dexterity)
Muscle wasting, twitching, cramps, stiffness
Speech/dysphagia problems
Dyspnoea/orthopnoea
```
Particularly ALS-
Starts in a particular region:
Limb (commonly)
Bulbar (20%)
Respiratory (less likely)
```
50
How is MND investigated?
No investigation to diagnose the condition, but can be used to exclude other causes.
EMG and nerve conduction studies show characteristic pattern requiring careful interpretation.
CT/MRI brain/SC can rule out similar presenting pathologies.
Blood test to exclude i.e. B12/folate deficiency, HIV etc.
Muscle biopsies for exclusion.
51
How is MND managed conservatively?
Incurable disease with a distressing disability preceding it.
If suspect refer to get diagnosis
Offer MDT support.
Encourage physiotherapy and speech therapy.
Dietician
Exercise
PEG tube when Px can't feed
Encouraging other modes of communication.
NIV
52
How is MND managed medically?
```
Riluzole used in ALS since disease modifying efficacy.
Muscle cramps- quinine
Increased tone/stiffness- baclofen
May need anti depressants
May need pain relief
```
Discuss end of life care
Survival is 2-4yrs.
53
What is multiple sclerosis (MS)?
| What are the types of MS?
Autoimmune condition leading to repeated episodes of inflammation of the CNS.
Relapsing remitting MS- Px has an episodes of good health/remission followed by sudden relapse. Don't always get 100% remission. (Commonly teenage/adolescent females)
Secondary progressive- Progression from RRMS. More/worsening symptoms with fewer remissions.
Primary progressive- From beginning symptoms gradually develop and worsen over time. (Usually middle aged men)
54
What is the pathophysiology of MS?
Autoimmune; may be precipitated by either environment (early viral infections) or genetics.
Leads to multiple areas of sclerosis along neurones, this leads to slowed/blocked transmission to brain/SC and so reduced sensation and movement.
55
What are the RF for MS?
F:M is 2:1
FHx
Pregnancy does not change the risk, although lowered risk of relapse during pregnancy, higher post partum.
56
How does MS present initially?
The main first symptoms are as followed:
1) Optic neuritis- sight impairment/hemianopia. Optic neuritis.
2) Brainstem lesion- Double vision, facial pain, dysphagia, vertigo, facial weakness, diminished taste, hearing impairment a, weakness nystagmus.
3) Transverse myelitis- An acute episode of weakness or paralysis of both legs, with sensory loss and loss of control of bowels and bladder; requires urgent hospital admission.
57
What are the other presentations of MS?
Facial- Bells palsy, trigeminal neuralgia, paroxysmal dysarthria, ataxia.
Hearing/balance- Deafness, vertigo, vomiting
Cognitive; visual, auditory, higher mental function
Depression
Taste and smell alters
Symptoms persists for several wks-months then will either get better and resume the original gradual decline or will get worse.
Several different presentations.
58
How is MS investigated?
Check FBC, inflammatory markers, U&E, LFT, TFT, glucose, HIV serology, calcium and B12 levels to exclude differentials.
Electrophysiology to detect demyelination.
MRI- 95% of patients have periventricular lesions and over 90% show discrete white matter abnormalities. (May show lesions of inflammation within the spinal cord or within the hemispheres, less commonly medulla)
CSF- Rise in protein level.
Diagnose with Mc Donald criteria.
59
How is MS managed conservatively?
- Px to notify DVLA
- Exercise
- Eat healthy
- No smoking
- Vitamin D OTC everyday
- Referred to MS nurse
After diagnosis of MS, and having checked bloods for any latent TB or dormant infections, a Px is initiated on DMT.
If sudden onset of relapse (within 12-24hrs) then give either 500mg/day methylprednisolone for 5 days or admit for IV infusion. Give gastric protection. Rule out UTI first as cause of symptoms.
60
What is the role of DMT in MS?
DMT is recommended for RRMS to help prevent the recurring of a relapse, by working as an immunosuppressor. There are different levels of intensity and different formats. I.e. the more low level ones are tablet, whereas other higher forms are 6 monthly infusions.
Examples include Interferon beta and Teriflunomide (used in active MS- two relapses within 2yrs).
Recently, Alemtuzumab has found to be the only DMT useful in treating PPMS.
NB- Women should stop DMT 12 months prior to becoming pregnant.
61
Autonomic dysfunction and complications
```
Arrhythmia
By
Pseudo obstruction- lieu’s
Repisroty failure-
Most people make a full recovery- self limiting- takes weeks to months to achieve
25% need help with breathing- ITU
```
62
Other complications
Pain
63
Manage
Close obs etc
Severe- IV IG, plasmapheresis in first 2 weeks
64
Prognosis s
25% need ventilation
65
Testing visual field defect
Test by confrontation- check your vision against a Px.
Ask when the Px can see the finger/white pen.
To test macular function use red pen and ask when it appears red.
Need Px to maintain point of fixation I.e. on bridge of your nose.
Move the pen slowly
Use systematic approach
Repeat if you find a defect- need for find at least 2 points of defect to find the boundaries. Move the pen horizontally, moving down each time and move vertically moving across each time.
66
What is a bitemporsl hemianopia?
Normal visual acuity so unlikely cataract fr ipbilateral optic nerve disorder.
Not homologous since in two different VF of the eye; therefore not at optic tract, radiations or behind the chasm.
This is therefore from the chiasm.
67
What
Optic chiasm- bilateral hemianopia
Complete lesion of optic tract- no Ono iOS complete hemianopia
Incomplete- bilateral incomplete hemianopia
Geniculate- typically vascular injury- wedged shaped homonimous- lateral genicukjs
Lesions of optic radiations- quadrantinopias
Visual cortex lesions homonomous hemianopia with macular sparring
Affecting the higher orders of vision- a
68
Causes of bitmportal hemianopia
Affecting the optic chiasm
Neoplasm
Inflammatory
Vascsuly
69
Chronic open angle glaucoma
Find optic disc with fundocspy- if found easily ask has this pt got atrophy? Easy to find boundaries.
If difficult then does this Px have papillodedma?
70
Optic neuritis
Central Scotoma
| Colour vision
71
Pupils
Equal diameter?
Reaction to light?
Round?
```
Abnormal one usually doesn’t react to light, unilateral ptosis.
Common causes-
3rd nerve palsy
Hornets syndrome
Tonic pupil
Pharmacological pupil
```
Binocular Diplopoda likely neurological.
Monocular persists after covering eye- functional or ophthalmic cause.
72
Posterior communicating artery aneurysm
Pupil involved initially.
Can compress on 3rd nerve- giving third never palsy
Painful
Short history
Diagnose using angiogram- can lead to subarachnoid haemorrhage.
73
Horners syndrome
```
Sympathetic palsy
Partial ptosis, constricted pupil
Congenital horners gives paler iris
Test by turning lights off- pupils will dilate passively if horners syndrome as opposed to actively.
May get anhydrosis
No visual symptoms
```
74
Causes of horners
Sympathetic chain
No decussation therefore if ls lesion then LS hornrrs.
Causes me congenital , clusterbheadache, structural pathology
Pan coast tumour
75
Pancoust turner @ti
Pain @ medial forearm. Muscle wasting @ thenart intercede.
76
Tonic pupil
```
Pupil larger than another one t
No ptosis o eye movement usually reacts to light but after prolonged illumination reluctant to dilate again
Parasympathetic version of howlers
Oval pupil
No symptoms
```
77
4th nerve palsy
Notice when looking to down, reading
Tilt heads away from affected site- esp in congenital
Looking out and down- difficult to look down
Common congenital
78
6th nerve palsy
LR
79
Ocular myasthenia
Bilateral pto
80
Brainstem
```
Compact therefore small lesions can have large effects
Feautures
Cranial nerve palsies
Complex eye movement disorder
2+ is BS territory.
```
81
UMN
Ok
82
LM
Mdd
| Think of cutting a nerve
83
Inter nuclear ophthlmaplegia
```
Impairment of addiction
Abducting eye develops nystagmus
Related to MS esp in young
Doesn’t look like anything else
ask the Px to look between two points- look right then left then right- looking for slow abduction. I.e. eye moving towards nose is slower
```
84
What to ask in AMU?
```
PMH- gestational- distressed baby, vaginal delivery?
Birth
Developmental milestones
Schooling
Head injury
```
Drugs-opioids, alcohol, illicit
Fhx- strongly genetic
85
Clinical
```
general medical exam
Full neurological exam-
GCS/MOCA
Cranial nerves
Fundoscopy
```
86
What are the roles of the facial nerve?
General sensory- small part of pinna of ear.
Special sensory- Anterior 2/3 tongue
Motor- Muscles of facial expression, nerve to stapedius (acuity of noise).
Parasympathetic- Lacrimal glands, mucosal glands, salivary glands.
If any damage to the facial nerve, all these functions would be affected.
87
What is Bell's palsy?
Refers to a palsy (paralysis) of the facial nerve, lasting from 3wks upto 9 months.
A LMN lesion- Px wouldn't have forehead sparing (lesion is outside of the pons/brainstem).
88
What are the causes of Bell's palsy?
LMN-
Idiopathic- more common in diabetes/pregnancy.
Iatrogenic- Local anaesthetic (dental)
Infection- Herpes simplex (type 1), Herpes zoster (Ramsey-Hunt- suspect if significant pain), HIV, EBV etc.
Neuropathic- GBS
Trauma
Neoplastic
89
How is Bell's palsy investigated?
Hx
Examination, focusing on CNVII
Serology looking for infectious causes.
90
How is Bell's palsy managed?
Prednisolone for 10 days.
If not responding to medical treatment then consider a facial nerve decompression.
If not resolving within 6-9 motnhs consider plastic surgeon referral. May consider cosmetic surgery also.
91
What is Parkinson's disease (PD)?
2nd most common neurodegenerative disease after Alzheimer's.
Typically 55-65 yrs old
Due to degeneration of dopaminergic pathways in the substantia nigra.
92
What are the RF for PD?
Increasing age
Men slightly more likely than females
Pesticide exposure
93
What are the three characteristic features of PD?
The clinically diagnostic features of PD are:
1) Rest tremor- Seen at rest but can be induced by concentration i.e. pill rolling)
2) Bradykinesia- Slow voluntary movements and reduced automatic movements. Noticeable when walking through a door frame- Px will pause. Also when walking only one arm may swing.
3) Rigidity- Increased resistance to passive movement, producing a flexed posture. I.e. Lead-pipe/ cog wheeling.
94
What are other presentations of PD?
Gait imbalances
Orthostatic HTN
Constipation
Sleep disorders
Urinary disturbances
Difficulty with movement requiring dexterity i.e buttons, opening pill bottles etc.
Fixed facial expression with drooling, infrequent bleeding etc.
Can get dementia one yr after diagnosis (P dementia- parkinsonism in the limb, frequent hallucinations, fluctuations in lucidity).
Depression
Psychosis
95
How is PD investigated?
Clinical diagnosis.
Investigations are to rule out other possible diagnosis.
MRI/CT- To exclude other diagnosis and for Px who fail to respond to L-Dopa for 12 wks.
Genetic testing- Huntington's gene
Olfactory testing
96
How is PD managed?
```
PD specialist nurses
Physiotherapy
Exercise
SALT
Nutritional support
Review every 6-12 months
```
```
Drugs-
Mono-amine oxidase B inhibitors- rasagiline, selegiline.
Oral/transdermal dopamine agonist
L-dopa (+carbidopa)
Anticholinergic/amantidine
```
If non dopaminergic drug not working, add dopaminergic drug.
If dopamine agonist not working increase dose, switch between agonists or add L-dopa.
If L-dopa not working increase dose, add agonist or add COMT inhibitor.
If drug therapy fails consider deep brain stimulation, where electrodes are placed in the basal ganglia.
97
What are the common management problems faced with treating PD?
What is the rescue drug for PD?
- Wearing off phenomenon
- 'On-off- fluctuations- where Px switch from severe dyskinesia (involuntary movements) to immobility within a few minutes.
- Dyskinesia- Can occur at the beginning, end or at the peak of a dose.
Apomorphine- Injected dopamine agonist, used to treat 'off episodes' where Px are frozen but can move hands enough to take the injection and inject themselves.
98
What is Parkinsonism?
| What are the potential causes?
Any condition causing a combination of the characteristic features of PD.
```
Drugs- Antipsychotics, metoclopramide.
Repeated head trauma (boxing)
LB dementia
Wilsons disease
Cerebral lesions i.e. tumours
```
99
What is an essential tremor?
Benign condition
Men and women equally affected
FHx +ve
Familial tremor occurs in childhood (gradual onset), sporadic ET occurs >40yrs old.
100
How does an essential tremor present?
Distal symmetrical postural tremor of the upper limb. I.e when the UL against gravity e.g. outstretched arms.
May begin as transient but becomes persistent.
May get head tremor
Frequency of tremor remains constant but amplitude can vary.
Tremor is absent in sleep.
Small amounts of alcohol will improve the tremor.
101
How is an essential tremor investigated?
No need to investigate if they have the characteristic features of an essential tremor.
But can look at EMG, TFTs, metabolic derangements (U+Es, LFTs, FBC)
102
How is an essential tremor managed?
Mild- No functional disability therefore no treatment.
Mild-Moderate- Disabling in exacerbations i.e. stressful situations. Give intermittent treatment in these situations.
Persistent disability- Need continuing treatment.
Persistent despite medications. Other non-conventional treatments should be considered.
Treat with propranolol and primidone.
103
What is carpal tunnel syndrome?
Compression, irritation or entrapment of median nerve at the carpal tunnel.
Mostly idiopathic but can be due to pregnancy (fluid retention), obesity, wrist overuse, hypothyroidism, diabetes, renal failure etc.
104
How does carpal tunnel syndrome present?
Tingling, numbness or pain in the distribution of the median nerve in the hand.
Wasting thenar eminence (more severe cases)
Positive Phalen test- Flex wrist for 60 seconds, brings on pain/paraesthesia.
Positive Tinel's sign- Tap over median nerve lightly at the wrist, causes paraesthesia in distribution.
Most cases are bilateral.
105
How is carpal tunnel syndrome investigated?
```
Not needed for diagnosis, unless CTS is suspected but does not respond to treatment.
Electroneurography- Gold standard
EMG (less accurate than ENG)
Ultrasonography- faster, non-invasive
MRI
```
106
How is carpal tunnel syndrome managed?
Conservative management if early/mild disease or advanced with minimal symptoms.
Typically resolve within 6 months.
Splints worn during the night
Minimise activities exacerbating the symptoms.
If treatment not working after 3 months then refer to specialist.
Local corticosteroid injections
Surgical release of the nerve through cutting transverse carpal ligament.
107
What is an Ulnar nerve palsy?
| What are the causes?
```
Dysfunction of the ulnar nerve.
Causes include;
Inflammation of the nerve
Injury to the nerve
Compression of the nerve
Elbow fracture/dislocation
```
108
How does an Ulnar nerve palsy present?
Loss of sensation in your hand, especially in your ring and little fingers
Loss of coordination in your fingers
a tingling or burning sensation in your hand
Pain
Hand weakness that may get worse with physical activity
Loss of grip strength
In more severe cases, loss of control and sensation can lead to ulnar claw.
109
How is an Ulnar nerve palsy investigated?
```
Hx and Ex
Bloods- rule out anaemia, hypothyroidism, RA and DM- all of which could cause neuropathy.
MRI
EMG and nerve conductions
USS of cubital tunnel
X-Ray
```
110
How is an Ulnar nerve palsy managed?
```
Splinting
NSAIDs to relieve nerve irritation
Physiotherapy
Corticosteroid injection
Surgical intervention
```
111
What is the Ulnar paradox?
A high ulnar nerve injury will result in a less prominent ulnar claw, whereas a low ulnar nerve injury will result in a more prominent ulnar claw.
112
What is cervical spondylosis?
Chronic degeneration of the cervical discs leading to disc herniation, calcification and osteophytic outgrowths. This can cause:
Radiculopathy due to compression or stretching of the nerve roots.
Myelopathy due to compression, compromised BS or repeated microtrauma to the spinal cord.
Affects women more than men, but men are affected earlier.
113
How does cervical spondylosis present?
```
Neck pain
Referred pain to between the shoulder blades, upper limbs.
Orbital/temporal pain
Neck stiffness
Poor balanced
Limited range of movement
Poor localisation of tenderness
```
When there is unilateral neck, shoulder or arm pain to one dermatome suspect radiculopathy.
114
How is cervical spondylosis investigated?
Most don't need investigating and diagnosis is on clinical grounds only.
X-Ray: Although these findings are common to middle aged people, will see osteophytes, narrowing of disc spaces.
MRI of cervical spine
115
How is cervical spondylosis managed?
First 3-4wks don't do anything; encourage neck movement, refrain from work absence etc.
4-12wks, if pain still persisting then refer to physiotherapist, psychologist etc.
If pain >12wks then continue with physio, consider psychosocial cause, might need surgery.
```
Mechanical- A few sessions of cervical manipulation may prove useful.
Pharmacological- Amitriptyline may be more effective than other analgesics.
#Xurgery- Consider if person has intractable pain, evidence of nerve compression or progressive neurological deficit.
```
116
What is lumbar spondylosis?
More likely due to exposure to mechanical stress.
Wear and tear of the disc and facet joints in the lumbar region of the spine.
Discs become thinner and osteophytes can form.
117
How does lumbar spondylosis present?
Pain
Stiffness
Referred pain to the buttocks and/or legs
Restricted movement.
118
How is lumbar spondylosis investigated?
Clinical diagnosis, where investigations are necessary in chronic pain.
CT/MRI etc.
Femoral stretch test
Straight leg raise etc.
119
How is lumbar spondylosis managed?
Encourage good posture
Keep moving i.e. avoid staying in one position.
Take care when lifting- bend at the knees.
Engage in physiotherapy exercises.
Lumbar facet injections
Corticosteroid injections
Common surgery- Spinal fusion. Can also consider laminectomy, discectomy, disc replacement etc.
120
What is a subarachnoid haemorrhage (SAH)?
Potential emergency, due to bleeding from a berry aneurysm (saccular-ballooning from one wall) in the Circle of Willis. The aneurysm is likely due to atherosclerosis or HTN. Usually occur after the age of 50yrs.
121
What are the RF for a SAH?
```
Larger aneurysm
HTN
Smoking
Alcohol intake
Female
FHx
Genetic conditions such as neurofibromatosis, Ehlers-Danlos, Marfans etc.
```
122
How does a SAH present?
Sudden onset severe diffuse headache lasting a few seconds to a minute. This is usually the only symptom present and so since can't distinguish reliably from a normal headache all Px should have a hospital consultation.
May also get:
Vomiting
Altered conscious state
Seizure
Confused state
Neck stiffness (due to meningeal irritation by blood in the CSF)
123
What are the 'warning' symptoms of SAH?
These occur 2-3wks prior to the SAH and are representations of sentinel bleeds (small leaks/expansion of the aneurysm).
These include characteristic SAH headaches, dizziness, orbital pain, visual loss etc.
Also can get motor/sensory deficit.
If sentinel bleeds are suspected Px should be treated as if had SAH.
124
What are possible signs of SAH on examination?
```
Reduced consciousness
Neck stiffness
Ophthalmoscope- Intraocular haemorrhages
Loss of pupillary light reflex due to brain herniation
HTN- due to sympathetic reflex
Impaired oculomotor nerve
```
125
How is SAH investigated?
Cannot just be a clinical diagnosis.
Initial- NEED CT W/O contrast- hyperdense appearance of blood in basal cisterns.
*Gold Standard for detection, demonstration and localisation of aneurysm is cerebral panangiography- either cerebral or CT angiography.*
CT can be negative in a few Px and so should do a lumbar puncture within 12hrs of headache onset- Take CSF to look for signs of bleeding.
ECG- ST elevation, dysrhythmias.
126
How is SAH manage
Aim is to stabilise the SAH and prevent the complications of SAH.
Initially all Px referred to a specialist unit for supportive care i.e. swallowing assessment, regular monitoring, intubation, nasogastric feeding etc.
Complications to prevent:
Vasospasms (common)- persistent constriction of the BV leading to cerebral ischaemia. Treat with nifedipine.
Re-bleeding risk- An untreated aneurysm has a risk of re bleeding which will lead to death. Px can either have neurovascular lipping through a craniotomy or endovascular coiling through the femoral artery.
Seizures- Some may experience so need to prevent it.
Ventricular drainage- To prevent the short and long term complications of hydrocephalus.
127
What is meningitis?
| What are the RF for meningitis?
Refers to inflammation of the meninges. Viral meningitis is the most common form and more likely to be benign, BUT all cases of meningitis should be treated as bacterial until proven otherwise.
Infants, teenagers and young adults are most at risk of BM.
Other RF include: Immunosuppression, CSF shunts, spinal procedures i.e. anaesthetic can introduce pseudomonas, splenectomy and sickle cell, diabetes, alcohol etc.
128
What are the common causative organisms of meningitis?
Neonates- E.coli
Infants- Neisseria.meningitidis
Older children and adults- H.influenza, S.pneumoniae
129
How does meningitis present?
Can get non-specific symptoms i.e. fever, headache, nausea, vomiting, lethargy, irritability etc.
Specific symptoms include: neck stiffness, photophobia, non-blanching rash, back rigidity, altered mental state, shock, +ve Kernigs sign.
In neonates/young children and elderly the symptoms are not that obvious.
130
How is meningitis investigated?
Don't delay treatment because of no investigations.
Need a lumbar puncture within 1hr of coming into hospital and commence treatment immediately. NB Don't do a LP if signs of shock, increased ICP, frequent fits etc. CSF to look for WBC, gram stain, PCR etc. In early stages may not show anything so repeat if symptoms persist.
For young Px should also do ABG, FBC, blood culture, PCR of blood, CRP etc.
All Px should also have U+Es, CXR, virology etc.
131
How is meningitis managed?
Supportive- Antipyretics, anti-emetics, fluids, analgesia, nutrition etc.
Viral: Treatment is supportive.
Bacterial: Need immediate referral to hospital. If a person is showing signs of sepsis or live >1hr from a hospital or has a non-blanching rash they should be given benzylpenicillin (IV/IM)
<1yrs- 300mg
1-9yrs- 600mg
>10yrs- 1200mg
Don't give if Hx of penicillin anaphylaxis.
In secondary care- Adults given 10mg QDS IV dexamethasone for four days.
Initial 'blind' therapy:
3 months- 60yrs old should be given IV ceftriaxone. If Px having a Ca infusion then cefotaxime is preferable.
<3 months, >60yrs or immunocompromised + ampicillin/amoxicillin.
132
What is encephalitis?
Inflammation of the brain commonly due to a viral infection i.e. Herpes simplex virus type 1.
Other causes include autoimmune, post infectious (i.e. encephalitis secondary to a viral infection elsewhere in the body), bacterial, HIV, tic-borne etc.
133
How does encephalitis present?
Most present with signs of meningitis (neck stiffness, fever, headache) as well as altered consciousness, movement disorder, convulsions, vertigo etc.
May start off as a flu-like illness/headache but then rapidly develop into the symptoms described above.
134
How is encephalitis investigated?
LP- Test CSF for proteins, cells, glucose, lactate and virology. All Px need PCR for HSV 1+2, varicella zoster virus and enterovirus (these are 90% of the causes).
CT- Useful to rule out and SOL as the cause, and will identify any increased ICP which would delay LP.
FBC, U+Es, LFTs, blood film, glucose, ESR, CRP.
MRI- Detects any oedematous changes.
EEG- More useful than CT in first week. Abnormal findings.
135
How is encephalitis treated?
Need immediate hospital admission.
If suspected meningitis also, then give IV/IM benzylpenicillin.
Treat early on with IV acyclovir- will drastically improve the Px.
Supportive therapy is very important in managing a viral cause. (Be careful with fluids in case of cerebral oedema.
Many Px are left with residual physical/neuropsychological deficits requiring life long MD management.
136
What is temporal arteritis (GCA)?
Immune mediated vasculitis of medium sized/large vessels.
Association with PMR
If not treated can lead to bilateral vision loss.
RF include female, 60-80yrs old, +ve FHx, European.
137
How does GCA present?
Recent onset temporal headache, malaise, myalgia, fever, weight loss etc. Also scalp tenderness, transient vision loss, amaurosis fugax, facial pain and diplopia. Some Px can get acute vision loss.
A lot of Px have jaw claudication, more prominent in eating/talking.
NB- Visual disturbances are due to inflammation of branches of the ophthalmic artery, leading to ischaemic optic neuritis.
Temporal artery may appear prominent, beaded, tender. May hear bruits over the carotid/axillary/brachial arteries.
138
What are the criteria for GCA?
Need to meet at least 3/5:
1) Age of onset ≥ 50yrs old.
2) Temporal artery biopsy shows signs of vasculitis/mononuclear cell invasion.
3) ESR ≥50 mm/hour.
4) New onset headache/localised pain in the head.
5) Temporal artery tender to palpation or have decreased pulsation.
139
How is GCS investigated?
Need TAB within 2wks of starting steroids.
ESR but if normal then CRP
Colour duplex ultrasonography
Normocytic normochromic anaemia and thrombocytosis present
May have deranged LFTs
140
How is GCS managed?
TAB may be -ve if skip lesions or if suboptimal biopsy, in which case treat as GCS if Hx and clinical signs are suggestive.
Immediately start on high dose of oral steroids, in a suspected GCA.
40mg daily, or if jaw claudication present then 60mg daily. As this is long term steroid therapy also give bisphosphonates to avoid osteoporosis.
When the symptoms and abnormal tests resolve then wean off steroids every 2 weekly by 10mg until reach 20mg. Thereafter wean off by 2.5mg.
If the symptoms relapse the Px should taper the dose up to that which controlled their symptoms.
Also start on aspirin, where a Px should be given PPI for gastric protection.
141
What is speech?
Voluntary tasks comprised of two components:
Phonation- Using the vocal chords of the larynx to produce sound.
Articulation- Using the pharynx, palate, tongue and lips to convert that sound into speech.
Also involves controlled expiration.
The nerves which are involved in speech are therefore the vagus, facial, hypoglossal and phrenic.
142
What is bulbar palsy?
LMN in the medulla leading to a functional impairment of CN VII-XII.
This can lead to an impairment of speech and swallowing amongst other symptoms.
Can be progressive or non-progressive, where progressive is more common.
The cause of the bulbar palsy can be due to:
Disorder of the muscles itself.
Disease of the medulla/pons
Disease of the intramedullary nerves of the spinal cord
Disease of the peripheral nerves to the muscles.
143
How does a bulbar palsy present?
- Tremulous lips
- Wasted and weak tongue, sits in the mouth with fasciculations.
- Collection of saliva in the mouth, leading to drooling- since develop dysphagia.
- Difficulty pronouncing 'r', which eventually becomes consonants as they develop dysarthria.
- Absent palatal movements
- Dysphonia- Rasping tone if unilateral, nasal tone if bilateral.
If pathology progresses, speech begins to slur and eventually becomes indistinct.
144
How is bulbar palsy investigated?
New neurophysiological investigations including electromagnetic articulography (EMA), electropalatography (EPG) and pressure-sensing EPG.
Also conduct bloods and imaging CT/MRI to identify any lesions in the brain/brainstem.
145
How is bulbar palsy managed?
Neurology referral.
Treat underlying cause.
Postural adjustments to help with drooling and reduce aspiration pneumonia.
Supportive- Give baclofen for spasticity, anti-cholinergic for drooling and treat any aspiration pneumonia.
If child then conduct genetic analysis.
146
What is acute respiratory distress syndrome (ARDS)?
Devastating syndrome which can affect any Px.
Non cardiogenic pulmonary oedema due to acute alveoli damage leading to acute respiratory failure. This leads to hypoxia and multiple organ failure.
147
What are the RF for ARDS?
```
Common RF:
Sepsis
Pneumonia
Gastric aspiration
Hypovolaemia
Massive trauma with shock with multiple transfusions
```
```
Less common RF:
Smoke inhalation
Burns
DKA
TLS
etc
```
148
How does ARDS present?
```
Hx of injury followed by dyspnoea (within one week)
Cyanosis
Tachypnoea
Tachycardia
Peripheral vasodilation
Bilateral fine inspiratory crackles
```
149
How is ARDS investigated?
| What is the diagnostic criteria?
FBC, U+Es, LFTs, amylase, clotting, CRP, ABG.
CXR shows bilateral alveolar shadowing
Onset of symptoms one week after the insult.
Presence of bilateral lung shadowing.
Respiratory failure w/o cardiac cause.
Presence of hypoxaemia.
150
How is ARDs managed?
ITU, supportive treatment, manage underlying cause.
Main treatment is O2 therapy to manage the hypoxaemia.
Early ARDS 40-60% O2 via CPAP is adequate.
Ventilate if O2<6.3kPa or CO2>6kPa.
Circulatory support/monitoring to maintain a good cardiac output.
Also treat the underlying cause of ARDS.
151
What is a head injury?
Can either be:
Direct- due to blunt or penetrating injury which would affect the same side of the brain.
Indirect- where movement of the brain on one side leads to contusion on the other side.
Or injuries can be disruptive where shearing/rotational forces lead to axon or blood vessel damage.
Main causes of minor head injury are assaults and falls, also RTA (these make up a lot of the moderate/severe).
152
How would you assess a Px with head injury?
Need to reach hospital within an hour for a CT scan.
Perform ATLS/resuscitation if needed. Following this guide:
- If alert with a normal/near normal GCS: Check haemodynamic status (pulse, fluid status, BP), neurological assessment, look for other injuries to examine.
- If reduced GCS: Resuscitate and make note of GCS and pupils.
Airway, Breathing- Need to ensure adequate O2 supply beginning ventilation ASAP. Also monitor CO2 as hyperventilation can lead to cerebral vasoconstriction. Circulation- Need to maintain a BP>90 systolic, to ensure cerebral perfusion.
May also aim to immobilise cervical spine if not contraindicated.
153
When would you admit a head injury Px to A&E?
```
GCS<15
Loss of consciousness
Amnesia regarding before/after
Vomiting
Focal neurological problem i.e. limb weakness
Headache since
Any seizures since
Irritability/altered behaviour
>65yrs
Hx of bleeding/clotting disorder
Drug/alcohol intoxication
etc
```
154
How would you investigate a head injury?
```
Need a CT scan within 1 hr if:
GCS <13 (within the first hr after injury ideally) or <15 two hours after injury.
>1 episode of vomiting
Seizure after the injury
Suspected/signs of skull fracture.
```
A cervical spine CT may also be indicated, especially if a diagnosis is needed before surgery, GCS<13, Px >65yrs, mechanism of fall etc.
155
How is head injury managed?
Early nutritional support
| High dose Mannitol- in the case of acute intra-cranial haematomas for preoperative Px.
156
What is an acute stroke?
A cerebrovascular event caused by a disruption to the blood supply of the brain. This leads to focal/generalised neurological deficits lasting >24hrs or death.
About 85% of strokes are ischaemic and 15% are haemorrhagic.
157
What are the RF for an acute stroke?
```
Smoking
Obesity
Hypertension
Diabetes
Alcoholism
PAD
Post TIA
Heart disease
COCP
etc
```
158
How does a stroke present?
Typical to have sudden onset or step-wise depression of symptoms/signs over hrs to days.
For sudden onset use FAST (face, arms, speech and time) to diagnose a stroke/TIA.
```
Cerebral infarct-
Contralateral hemiplegia- initially flaccid then limbs are spastic.
Contralateral sensory loss
Homonymous hemianopia
Dysphagia
```
```
Posterior circulation ischaemia-
Motor deficits
Homonymous hemianopia
Vertigo
Diplopia
Ataxia
Dysphagia/dysarthria
```
Lacunar-
Pure motor loss, pure sensory loss, mixed motor and sensory loss or ataxia.
Intact cognition and consciousness
159
How is an acute stroke investigated?
Need non-enhanced CT immediately (within 24hrs) if:
- Considering anticoagulation or thrombolysis
- Px on anti-coagulants
- GCS<13
- Severe headache
- Bleeding tendency
- Fluctuating/progressive symptoms
- Papilledema, neck stiffness etc
```
FBC
HTN- retinopathy, ECG, CXR
Test for sickle cell
ESR (Rule out GCS)
Carotid duplex
```
160
How is an acute stroke managed?
```
Supportive-
Give O2 if sats <95%
Maintain blood glucose between 4-11mM
BP kept at 185/110 if Px considered for thrombolysis
Screen for swallowing and nutrition
Encourage active movement
```
Anti-platelet therapy:
Start 300mg Aspirin as soon as a haemorrhagic stroke has been excluded.
Then for long term anti-platelet (so long as no permanent/paroxysmal AF) give 75mg Clopidogrel or aspirin if not tolerated.
Thrombolytic treatment:
Given within 4.5hrs (preferably 3hrs) of symptom onset so long as haemorrhagic stroke has been excluded and there are facilities to image and reimage.
Surgically can consider thrombectomy (within 6hrs).
161
What are the causes of raised ICP?
Localised mass lesions- extra-dural, subdural or subarachnoid haemorrhage.
Neoplasms- Glioma, meningioma or metastasis
Disruptive CSF system i.e. obstructive/communicating hydrocephalus.
Cerebral oedema i.e. secondary to meningitis, encephalitis etc.
162
How do Px with raised ICP present?
- Papilledema, vomiting and headache combination is common for presentation.
- Headache- Worried more if nocturnal (i.e. on waking), worse with cough/sneeze, altered mental state.
- Changes in mental state (early-lethargy, irritability which if untreated can lead to coma and death.
- Vomiting which can become projectile
- Pupillary changes
- Fundoscopy- blurred disc margins, flame-shaped haemorrhages, later on will develop retinal haemorrhages.
163
How would you investigate a Px with suspected raised ICP?
CT/MRI for underlying cause
| Monitor blood glucose, renal function, electrolytes.
164
How is raised ICP managed?
In the acute emergency need to ensure maintenance of O2, glucose, BP.
First line-
Avoid pyrexia- this can increase ICP
Manage seizures aggressively as these can increase ICP
Drain CSF fluid
Elevate the head of the bed 30 degrees to improve jugular outflow and drainage
Neuromuscular blocks. I.e. increase in thoracic muscle movement may compress drainage from brain.
Analgesia i.e. Morphine, Alfentanil
Sedation i.e. Midazolam
Mannitol- although be ware of hypovolaemia.
165
What is a spinal cord compression?
Neurological emergency which needs to be identified and managed immediately to avoid any permanent damage.
166
What are the causes of spinal cord compression?
```
Trauma
Tumour (metastasis mainly from breast, lung and prostate)
Infection
Inflammatory (RA)
Disc prolapse
Haematoma (subdural/epidural)
```
167
What are the red flags of spinal cord compression?
Insidious progression (gradual but harmful)
Neurological symptoms- Weakness, gait disturbance, loss of sexual/bladder/bowel function.
Neurological signs-
Lhermittes sign- Flexion of neck sends electric shock down entire spine.
UMN signs in lower limb
LMN signs in upper limbs
Variable sensation- noticeable in hands.
168
How does spinal cord compression present?
Motor (easily fatigued, disturbed gait), sensory (sensory loss, paraesthesia) and autonomic dysfunction (priapism, urinary retention, paralytic ileus, neurogenic shock etc)
Cervical spine lesion- quadriplegia
Thoracic spine lesion- hemiplegia
Reflexes- Increased below level of injury, absent at the level of injury and decreased above the level of injury.
Sphincter disturbances
169
How is spinal cord compressions investigated?
Need baseline Hb and Haematocrit to monitor blood loss.
Renal function and electrolytes for dehydration.
MRI of whole spine.
170
How is spinal cord compression managed?
Initially:
- Lie the Px flat with the spine in neutral position.
- If not contraindicated start the Px on a course of dexamethasone.
- Ensure the Px is catheterised.
- Help Px cough anything which could be blocking their airways.
- Take preventative measures against pressure ulcers and DVT.
- Give psychosocial counselling.
Management:
-Pain with analgesia, radiotherapy or surgery.
-Give bisphosphonates to those who have cord compression due to metastasis.
-Definitive treatment should be started before Px loses the ability to walk or before progression of neurological symptoms. DT includes laminectomy, posterior decompression, radiotherapy.
Planned discharge with community rehabilitation.
171
What is cauda equina syndrome?
| What are the RED FLAGS?
Compression of the cauda equina, typically starting from L1/2.
```
Urinary incontinence
Faecal incontinence
Saddle anaesthesia
Bilateral sciatica
Erectile dysfunction
```
172
How is cauda equina syndrome investigated?
| How is cauda equina syndrome managed?
Investigated- Hx, O/E, emergency MRI.
Managed:
- Immobilise spine if the cause is due to trauma.
- Surgery if need to remove bone fragments, blood, tumour, herniated disc or abnormal bone growth.
- Radiotherapy for malignant disease being the cause, where surgery is not possible.
- Some Px may benefit from Abx therapy, anti-inflammatory agents etc depending on the cause.
173
What is a migraine?
A migraine can either be classified as a migraine with aura, w/o aura or migraine aura w/o headache.
Episodic migraines occur for <15 days per month.
Chronic migraines occur for >15 days per month for 3 months, where at least 8 days have migraine features.
174
How do migraines present?
Best diagnostic tool is a good Hx, therefore encourage Px to keep a diary.
Painful unilateral headache.
Some may have premonitory phase (i.e. can feel a migraine coming on hrs-days before; photophobia, vomiting), may have an aura.
Resolution phase occurs as the headache fades away.
Px after the migraine may feel tired, irritable, difficulty concentrating.
175
What is a migraine w/o aura?
Recurrent- Need 5 attacks to define as a MWA.
Lasting 4-72hrs with at least 2 of the following:
-Unilateral
-Moderate/severe pain
-Pulsating
-Aggravated by or leading to the avoidance of physical activity.
Plus one of the following:
- Vomiting/Nausea during the attack
- Photo/phonophobia
176
What is a migraine with aura?
Aura features last minutes to hrs and occur before the migraine.
Visual- Can be in one eye or homonymous (affecting one VF in both eyes, may have scintillating scotoma, hallucinations.
Numbness starting from hand and moving up the arm into the face. Can be followed by paraesthesia.
Begins before the end of an aura or within an hr of the aura occurring.
Some Px may have migraines with aura and migraines without.
177
How are migraines investigated?
Only needed to rule out alternative diagnosis.
| If second line treatment fails or the diagnosis of a migraine is unclear then refer to neurologist.
178
How are migraines managed?
Reduce triggers; stress, anxiety, bright lights, trauma, dietary sensitivities, sleep deprivation or XS.
Work through this ladder, but if a Px has moderate-severe migraines then skip to step 3.
1) Analgesics- Either anti-emetic or non anti-emetic. Use either aspirin or ibuprofen. Can use prochlorperazine to treat N/V.
2) Rectal analgesia/antiemetics- i.e. diclofenac.
3) Anti-migraine specific medications (triptans) i.e. almotriptan, eletriptan.
179
What is Horner's syndrome?
| How does Horner's syndrome present?
Rare condition, due to the sympathetic disruption of the sympathetic chain to the eye. Results in the triad;
Partial ptosis
Miosis
Anhidrosis
```
Symptoms include:
Asymptomatic
Above mentioned triad
Facial flushing (if preganglionic)
Orbital pain/headache (if post ganglionic)
```
180
How is Horner's syndrome investigated?
| How is Horner's syndrome managed?
CXR- May indicate apical lung cancer.
CT/MRI- looking for cerebrovascular accident
CT angiography/carotid US
Horner's syndrome is a sign, should treat the underlying disease.
181
What is subacute combined degeneration of the cord?
| What are the RF of SCD?
Vitamin B12 deficiency affecting the spine, brain and nerves. Initially the myelin sheath is affected, but eventually the entire nerve is.
This 'combined' disease affects the nerve, brain and SC.
The aetiology is unknown but thought lack of Vitamin B12 leads to a build up of FA around the nerve cells.
RF include Vitamin B12 deficiency (pernicious anaemia), poor absorption i.e. Crohns or gastric surgery.
182
How does SCD present?
Progressive paraesthesia, numbness and weakness.
As the disease progresses, get change in mental state, clumsiness, stiffness, depression, decreased vision, sleepiness, unsteady gait.
Reduced/absent reflexes.
183
How is SCD managed?
Give Vitamin B12 injections for OD for a week.
Then once a week for a month.
Then monthly.
A Px should remain on life long Vitamin B12 supplements.
Treatment at early presentation of symptoms has the best recovery.
184
What is cavernous sinus syndrome?
The cavernous sinus if a definite space in the cranium containing CNII, CNIII, CNIV, CNVb, CNVI, part of the sympathetic plexus and the internal carotid artery.
Therefore any pathology affecting the cavernous sinus has the opportunity to manifest in man different ways.
185
What are some of the causes of CSS?
| How does CSS present?
Commonly tumour compression by; meningioma, pituitary adenoma, neuroblastoma etc.
Inflammation; sarcoidosis, Tolosa-Hunt syndrome.
Trauma; basal skull fracture.
Vascular; cavernous sinus thrombosis.
Infection; Aspergillosis, herpes zoster.
Can present with ophthalmoplegia (dependent on which CN palsy exists), facial sensory loss, Horner's syndrome, chemosis (swelling of conjunctiva) and proptosis (both due to increase pressure in CS). If CST presents with- fever, tachycardia, hypotension, rigors, altered mental state.
186
How is CSS investigated?
| How is CSS managed?
Mainly a clinical diagnosis.
Confirm with MRI and test suspected underlying diagnosis i.e. if CN palsy then test that CN.
Manage the underlying cause. I.e. trauma will self resolve, tumour may need surgery/radiotherapy, infection will need supportive management and anti-microbials, vascular causes may need endovascular intervention etc.
187
What is a pituitary tumour?
| What are the characteristic effects of the pituitary tumour?
```
Almost always benign and curable.
Leads to:
Local effects of the tumour.
XS hormone production
Decreased hormone production by the unaffected portion of the pituitary gland.
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188
How does a pituitary tumour present?
1) Local effects of the tumour:
- Visual loss (being the first sign usually), typically get bitemporal hemianopia and ocular nerve palsies can result in squint.
- Headaches, usually retro-orbital or bitemporal.
- Changes to appetite, thirst, temperature regulation and consciousness if compresses on the hypothalamus.
2) Hypopituitarism in the following order; LH, GH, TSH, ACTH, FSH.
3) Hyperpituitarism leading to acromegaly, hyperprolactinaemia, Cushing's disease, thyrotoxicosis.
189
How are pituitary tumours investigated?
| How are pituitary tumours managed?
Endocrine studies (to look for hormone XS/deficiency)
Lateral skull X-Ray
MRI
VF
Management is dependent on the type. I.e. if it affects hormones then consider surgery, radiotherapy or drugs.
If it is a tumour then trans-sphenoidal surgery. Radiotherapy is used post surgery, or where the tumour has not been completely removed.
190
What is Wernicke's encephalopathy?
| How do Px present?
Chronic alcohol consumption leading to thiamine deficiency due to poor absorption, poor intake or poor utilisation.
This can lead to neuronal loss in the thalamus, hypothalamus and mamillary bodies.
Px present with:
Double vision, ptosis, eve movement affected.
Loss of muscle coordination, unsteady movement, may have atrophy
Loss of memories- can be profound.
Inability to form new memories
Low BP, low body temperature
191
How is Wernickes encephalopathy investigated?
| How is Wernickes encephalopathy managed?
Diagnosis is based mainly on Hx an Examination.
Bloods- FBC, U+Es, LFTs, Glucose, cholesterol, serum thiamine levels, MRI more useful than a CT.
Managing the alcohol misuse is the mainstay of treatment.
Assess and reassess memory and intellectual impairment.
Occupational therapy and neuropsychological assessments.
192
What is Argyll Robertson pupil?
Commonly associated with neurosyphilis, following yrs on untreated syphilis.
Pupil reacts poorly to light but briskly to accommodation.
Usually bilateral- thought to be due to bilateral damage to the nuclei in the midbrain.
193
What is CJD?
| How does CJD present?
Transformation of normal host encoded prions to protease resistant isoforms, where most cases are sporadic.
Common presentation of sporadic; Myoclonus, visual disturbances, cerebellar/pyramidal/extra-pyramidal signs also i.e. slurred speech, numbness, paraesthesia, progressive ataxia, dementia and involuntary movements.
194
How is CJD investigated?
| How is CJD managed?
Usually diagnosed by experienced neurologist and is correct when confirmed by brain biopsy post mortem 95% of times.
- Brain biopsy if there is an alternative possible diagnosis.
- MRI can be used to differentiate between the types.
- EEG
There is currently no cure for this disease and so management is purely supportive.
195
What us syringomyelia?
How is it investigated?
How is it managed?
Rare condition, where there is a fluid filled cyst in the centre of the spinal cord (usually cervical).
-‘Cape-like’ (neck, shoulders and arms)
loss of sensation to temperature but the preservation of light touch, proprioception and vibration
Classic examples are of patients who accidentally burn their hands without realising
Spastic weakness (predominantly of the lower limbs)
Neuropathic pain
Upgoing plantars
Autonomic features:
Horner’s syndrome due to compression of the sympathetic chain, bowel and bladder dysfunction.
Scoliosis will occur over a matter of years if the syrinx is not treated
Diagnose with an MRI
Manage with physiotherapy and rehab to preserve neurological functions and Px are taught how to avoid damage due to lack of feeling pain stimuli.
Undergo surgery where there are symptoms present.
196
How does a CNI palsy present?
| What are the common causes?
Reduced taste and smell.
| Trauma, frontal lobe tumour, meningitis.
197
How does a CNII palsy present?
| What are the common causes?
VF defects, pupil abnormalities, optic neuritis, optic atrophy, papilledema.
GCA, demyelination, tumour, ischaemia etc.
198
How does a CNIII palsy present?
| What are the common causes?
Fixed dilated pupil, ptosis, outward deviation of eye.
| DM, GCA, syphilis, aneurysm of PCA, idiopathic, raised ICP.
199
How does a CNIV palsy present?
| What are the common causes?
Vertical diplopia, head tilt.
| Trauma of orbit, DM, HTN.
200
How does a CNV palsy present?
| What are the common causes?
Reduced sensation, weakness of jaw, fasciculations of temporalis/masseter.
Trigeminal neuralgia, herpes zoster, bulbar palsy, acoustic neuroma.
201
How does a CNVI palsy present?
| What are the common causes?
Medially deviated eye.
| MS, pontine CVA.
202
How does a CNVII palsy present?
| What are the common causes?
Fascial weakness, forehead sparing in UMN, forehead affected in LMN.
LMN- Bell's palsy, polio, otitis media, skull fracture etc.
UMN- stroke, tumour
203
How does a CNVIII palsy present?
| What are the common causes?
Unilateral SNHL, tinnitus.
| Paget's disease, herpes zoster, neurofibroma, acoustic neuroma, Meniere's.
204
How does a CNIX palsy present?
| What are the common causes?
Only bilateral lesions will cause an effect i.e. pseudobulbar palsy.
Trauma, BS lesion, polio, GBS.
205
How does a CNX palsy present?
| What are the common causes?
Palatal weakness causing 'nasal speech', nasal regurgitation of food.
Trauma, BS lesion, polio, GBS etc.
206
How does a CNXI palsy present?
| What are the common causes?
Weakness/wasting of SCM and trapezius.
| Trauma, BS lesion, polio, GBS etc.
207
How does a CNXII palsy present?
| What are the common causes?
Wasting of ipsilateral side of tongue with fasciculations, tongue deviates towards affected side.
Polio, syringomyelia, TB, median branch thrombosis of vertebral artery.
208
What is hypoxic ischaemic encephalopathy?
This refers to when the brain does not receive enough oxygen (due to reduced blood supply) and so this leads to ischaemia and brain damage.
Can occur in any age, where children drowning/unconsciousness is more common than adults and cardiac arrest/CVA.
