Neuro (3A) Flashcards
(176 cards)
1
Q
What is presbycusis
A
Age related sensorineural hearing loss
2
Q
What is otosclerosis
A
Autosomal dominant replacement of normal bone with spongy vascular bone.
- Conductive deafness
- Tinnitus
- “Flamingo tinge” to tympanic membrane
- Family history
3
Q
What is meniere’s disease
A
Recurrent episodes of vertigo, tinnitus and sensorineural hearing loss, lasting mins-hours. Vertigo main complaint!
- Middle aged adults
- Feeling of aural fullness/pressure
- Nystagmus/positive romberg test
4
Q
Investigations in Meniere’s disease
A
Menieres triad
- Otoscopy - Normal ear drum
- Audiometry - Sensorineural hearing loss
- Tympanometry - normal
5
Q
Pharmacological management of Meniere’s
A
- Prochlorperazine (acute vertigo and nausea) [Acute attacks]
- Betahistine medication (H1 agonist that acts as a Vestibular sedative) [Prevention]
- Intratympanic gentamicin injection if surgical
6
Q
What is acoustic neuroma
A
AKA vestibular schwanomma. Tumour arising from schwann cells myelinating CN8. Usually presents between 40-60yo.
Associated with type 2 neurofibromatosis
Presents similar to menieres (vertigo, tinnitus, S hearing loss) BUT also has absent corneal reflex and possible facial paralysis
Affected cranial nerves:
- Men. symptoms (VIII)
- Absent corneal reflex (V)
7
Q
How can acoustic neuroma present
A
Depends on cranial nerves affected
- CN5: Absent corneal relfex
- CN7: Facial palsy
- CN8: Unilateral sensorineural hearing loss and tinnitus, vertigo.
8
Q
Investigation of acoustic neuroma
A
Audiogram and examination show sensorineural hearing loss.
MRI Gold standard imaging for diagnosis and tumour tracking.
9
Q
Management and complications of acoustic neuroma
A
- Conservative or
- Tumour excision or
- Radiotherapy
- Permanent hearing loss (CN8), permanent facial weakness (CN7)
10
Q
What is an essential tremor, give features and management
A
Autosomal dominant condition usually affecting both arms.
- Postural tremor: worse when arms stretched out
- Improved by alcohol and rest
- Most common cause of titubation (head tremor)
Managed with propanolol, or primidone second line
11
Q
How does left heart failure cause right heart failure
A
Left side of heart is unable to pump efficiently, causing blood to back up into pulmonary veins and arteries. This increases pulmonary blood pressure. This pressure is then transmitted back towards the right ventricle.
The dilation of the right ventricle stretches the AV valve, causing a regurgitation into the right atrium during systole. This causes right atrium dilation, which puts further pressure on the right ventricle causing it’s hypertrophy. Eventually neither work efficiently causing right heart failure. RHF causes an increase in blood backing up into general circulation
12
Q
How does right heart failure cause its cardinal symptoms
A
Jugular vein distension - Increased pressure in right atrium is transmitted back to the jugular veins
Hepatomegaly - Increased pressure of the hepatic veins, which usually directly drain into the inferior vena cava
Peripheral pitting oedema - Increased pressure in the systemic venous circulation, forcing fluid out of the blood into surrounding tissues
13
Q
Signs and symptoms of left heart failure
A
Signs
- Tachypnoea, tachycardia
- Cool peripheries
- Peripheral cyanosis
- Pink frothy sputum/crackles on auscultation
- Wheeze
- Third heart sound
- Displaced apex beat
Symptoms:
- Dyspnoea, Orthopnoea (SOB when lying flat), Paroxysmal nocturnal dyspnoea (SOB at night)
- Fatigue and weakness
- Weight loss
14
Q
Signs and symptoms of right heart failure
A
(usually due to pathology involving lungs/pulmonary vessels e.g. pulmonary stenosis)
Signs (due to backing up of fluid):
- Raised JVP
- Peripheral pitting oedema (thighs, sacrum, abdomen)
- Hepatosplenomegaly
- Ascites
- Facial engorgement
- Pulsing in face/neck (tricuspid regurgitation)
Symptoms:
- Fatigue/weakness
- Swelling in legs/distended abdomen
- Nausea/anxiety
- Nose bleed
15
Q
How does left heart failure cause pulmonary oedema, and how does this lead to right sided heart failure
A
LV unable to move blood out into body, causing backlog.
This increases blood stuck in LA, pulmonary veins and lungs. They leak fluid as a result and are unable to reabsorb it. This causes pulmonary oedema; lung tissues and alveoli become full of interstitial fluid, interfering with gas exchange, leading to SOB and other symptoms.
Pulmonary HTN puts pressure on right ventricle, meaning it isn’t able to pump as much blood, causing right sided heart failure.
16
Q
How might a heart failure patient present on examination?
A
- Increased resp rate
- Reduced O2 saturation
- Tachycardia
- Hypotension
- Dyspnoea
- Oedema in legs
Auscultation:
- 3rd heart sound/ displaced apex beat
- Bilateral basal crackles (that sound wet)
17
Q
Investigations in Heart failure
A
BNP (Brain Natriuretic Peptide) blood test
- Released from stressed ventricles in response to increased mechanical stress
- (NOT specific, also released in tachycardia, sepsis, PE, renal impairment, COPD)
CXR (ABCDE)
- Alveolar Oedmea, Kerley B lines (interstitial oedema), Cardiomegaly, Dilated upper lobe vessels, Pleural effusion
ECG will show wide QRS and may help diagnose causation
Echocardiography is KEY. Measures Ejection fraction, ventricular function, valvular abnormalities
18
Q
Scoring system for heart failure functional limitations
A
New York Heart Association classifications of heart failure
I (Mild) - No limitation on physical activity. Ordinary physical activity doesnt cause fatigue/palpitations/dyspnoea
II (Mild) - Slight limitation n physical activity. Comfortable at rest; dyspnoea on ordinary activity
III (Moderate) - Less than ordinary activity causes dyspnoea, which is limiting. Rest is fine.
IIII (Severe) - Symptoms present at rest, all activity causes discomfort
19
Q
3 cardinal non specific signs in heart failure
A
SOB AS FAT
Dyspnoea, Ankle Swelling, Fatigue
20
Q
Pathophysiology of ischaemic, HTN, LV hypertrophy and dilated cardiomyopathy heart failure, and what HF do these cause?
A
Cause systolic failure
- Ischaemic: Myocytes start to die, reducing ability of contraction
- HTN: Arterial pressure increase in systemic circulation means it is harder for LV to pump blood into hypertensive circulation
- LV hypertrophy: increased muscle mass requires increased oxygen supply, more likely muscles will die
- Dilated cardiomyopathy: Heart chambers dilate, become thinner, weaker contractions.
21
Q
Acute heart failure management
A
Pour SOD
Pour away fluids (Stop fluids)
Sit up
Oxygen
Diuretics
GTN may be needed
22
Q
Management of chronic heart failure
A
1) ACEi + beta blocker
2) Add spironolactone and SGLT2i if Ejection fraction not controlled with ACEi and BB
23
Q
What should be kept in mind when prescribing for heart failure? (reg ACEi)
A
ACEi contraindicated in Heart valve disease
ARB (candesartan) can be used instead of ACEi
Aldosterone antagonists added if ejection fraction not controlled with ACEi and BB
24
Q
How does anterior, middle and posterior cerebral artery stroke present
A
Contralateral weakness/paralysis, sensory loss
Anterior - lower extremities>upper
Middle - Upper>lower, contralateral homonymous hemianopia, aphasia
Posterior - Contralateral homonymous hemianopia with macular sparing and visual agnosia.
25
Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
Horner's also:
- Anhidrosis
- Meiosis
- Ptosis
26
Anterior inferior cerebellar artery (lateral pontine syndrome)
Same as PICA but with added Ipsilateral: facial paralysis and deafness
(PICA:
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus)
27
What causes locked in syndrome
Basilar artery blockage
28
What does retinal/ophthalmic artery ischaemia cause
Amaurosis fugax
29
What is a lacunar stroke
Present with either isolated hemiparesis, hemisensory loss, or hemiparesis with limb ataxia
Strong association with hypertension
30
What assessment system is used in acute strokes
ROSIER (Recognition of Stroke In Emergency Room)
Uses symptoms as + points and mimics (syncope, seizure activity) as - points
31
Management of ischaemic stroke
Once haemorrhagic ruled out:
- IV Alteplase if presents within 4.5 hours
- Mechanical thrombectomy if after 4.5 hours
Then: 300mg Oral aspirin daily for 2 weeks then clopidogrel lifelong daily
32
What are the driving rules in ischaemic stroke
Patients must not drive car for 1 month after TIA or stroke, or 1 year for HGV
33
Scoring system for risk of stroke after Atrial Fibrillation
CHA2DS2 VASc
34
Define TIA
Transient Ischaemic Attack. Acute neurological dysfunction that has a sudden onset and resolves in less than 24 hours.
NOT a stroke as involves ischaemia not infarction
- Contralateral numbness, face droop, dysphasia, vision loss (Amaurosis Fugax)
35
What acronym helps identify stroke in public
FAST
Face
Arms
Speech
Time
36
Define amaurosis fugax with pathophysiology and causes
Short lived blindness in one eye described as “curtain coming down over vision”. Due to temporary reduction in internal carotid or central retinal artery leading to ischaemia of the retina.
Occurs in GCA, Stroke, AF
37
What risk score should be completed after TIA
ABCD2 - risk of stroke after TIA
Age >60
BP >140/90
Clinical features (unilateral weakness =2, just speech disturbance =1)
Duration >60mins =2, 10-59mins =1
> 6 predicts stroke, immediate referral
4 requires referral
38
Management of TIA
<24 hours since presentation, use DAPT
- 300mg Aspirin and 300mg clopidogrel loading dose
- Followed by aspirin and clopidogrel 75mg for 21 days,
- Then clopidogrel 75mg long term.
May need PPI
39
What are the types of haemorrhagic stroke
Extradural haemorrhage - bleeding above dura mater
Sudural haemorrhage - bleeding between dura and arachnoid
Subarachnoid haemorrhage - bleeding between arachnoid and pia mater
Intracerebral haemorrhage - Bleeding within cerebrum
40
Important examples of TIA mimics that MUST be excluded
Hypoglycaemia - sweating, palpitations, hunger, anxiety. Often has insulin, metformin, sulfonylurea! use. Resolves with insulin
Intracranial haemorrhage - Stroke init. Longer, symptoms get worse instead of better.
41
General symptoms of haemorrhagic stroke
Reduced GCS
Headache
Vomiting
Seizures
One sided arm/leg/face weakness/paralysis
42
Give the scoring system for unconsciousness
Glasgow Coma Scale - assessment of eye opening, verbal and motor response.
Eye out of 4
Verbal out of 5
Motor out of 6
Minimum score 1 per category
43
Glasgow coma scale scoring system in detail (not sure if need to know but probably helpful to have decent idea)
Eye opening
4 - Spontaneous
3 - To speech
2 - To pain
1 - None
Verbal response
5 - Orientated
4 - Confused conversation
3 - Inappropriate words
2 - Incomprehensible sounds
1 - None
Motor response
6 - Obeys command
5 - Localises to pain
4 - Withdraws to pain
3 - Abnormal flexion to pain
2 - Extension of upper and lower limbs to pain
1 - No response
44
Define Extradural haemorrhage with its main cause and epidemiology
Cranial bleeding above the dura mater.
Usually caused by trauma to pterion of skull, causing rupture of middle meningeal artery in temporo-parietal region. Can associate with temporal bone fracture.
Usually found in young adults
Blood doesnt cross suture lines
45
Why can extradural stroke present slowly at first before becoming more severe
If bleeding is slow, symptom onset is slower (lucid interval) before there is a sudden, rapid decline when intracranial pressure increases enough to compress brain
46
Describe Non contrast CT appearance in Extradural haemorrhage (3)
- Biconvex, hyperdense haematoma
- Blood doesnt cross suture lines
- Shows midline shift (increased pressure can cause cause brain shifting/herniation)
47
What are the main 2 herniation complications of haemorrhagic stroke
Supratentorial herniation (cerebrum against skull, compressing arteries and causing ischaemic stroke)
Infratentorial herniation (Cerebellum pushed against brainstem, compressing area that controls consciousness, respiration, heart rate)
48
What is Cushing’s triad and how is it treated
Body’s response to increased intracranial pressure, signifies severe lack of oxygen in brain tissue
- Bradycardia
- Irregular respirations
- Widened pulse pressures (increased systolic, decreased diastolic)
Treated with IV mannitol to reduce ICP
49
Define Subdural haemorrhage with main cause and epidemiology
Bleeding below dura mater, caused by bridging vein rupture.
Usually occur in elderly/alcoholic patients but can occur in babies (shaken baby syndrome)
50
Causes of bridging vein rupture
- Brain atrophy; with age. Stretches bridging veins, meaning they stretch over gaps unsupported.
- Alcohol abuse: Causes walls of vein to thin
- Trauma
- Falls
- Shaken baby syndrome
- Acceleration/deceleration injury
51
Non contrast CT appearance of Subdural haemorrhage
Bleeding between the dura mater and arachnoid
- Follows contours of brain and crosses suture lines, forming a crescent shape
Acute (hyperdense mass)
Chronic (Hypodense mass)
Acute on Chronic (both)
52
What GCS score requires intubation
8 or below
53
Specific surgical management used in subdural haemorrhage
Burrhole washout if haemorrhage small
Craniotomy if large haemorrhage
54
Define subarachnoid haemorrhage with main cause
Bleeding below the arachnoid layer, where CSF is located.
Main cause is a ruptured saccular (or Berry) aneurysm, with majority located between anterior communicating artery and anterior cerebral artery
55
Risk factors for subarachnoid haemorrhage (7)
PKD (Associated with berry aneurysm)
Connective tissue disorders (Ehlers-Danlos, Marfans)
Family history
Increasing age
HTN
Smoking
Alcohol
56
Typical presentation of subarachnoid haemorrhage
Sudden onset occipital “thunderclap” headache, following strenuous activity, with associated neck stiffness and photophobia. Smaller, “Sentinel” headache may have preceded thunderclap
Black, female, 45-70
57
Signs/symptoms of subarachnoid haemorrhage
- Thunderclap headache
- Meningism (Headache, photophobia + neck stiffness)
- Fixed dilated pupil (third nerve palsy - especially in posterior communicating artery rupture)
- 6th nerve palsy
- Kernigs and Brudzinskis due to meningism also
- Nausea/vomiting, weakness, confusion, coma, reduced consciousness, speech reduction
58
Investigations in subarachnoid haemorrhage
Urgent non contrast CT head (blood in subarachnoid space/basal cisterns)
CT angiography to locate bleed source
ECG to detect arrhythmia/abnormality
If CT non conclusive,
- Lumbar puncture (RBCs in CSF and Xanthochromia) 12 hours after onset.
59
Define Kernigs and Brudzinskis signs
Kernig - Inability to straighten bent leg without pain when hip flexed to 90 degrees
Brudzinski - Passive flexion of neck in supine patient elicits hip and knee flexion
Suggest meningitis/meningism
60
CT Appearance in subarachnoid haemorrhage
Blood in subarachnoid space (hyperdense)
- Star shaped lesion (Blood filling in gyro pattern)
61
Management of Subarachnoid haemorrhage
Surgical 1st/GOLD
- Endovascular coiling (clipping also possible but more complications)
Nimodipine to prevent vasopasms
IV Mannitol to reduce ICP
Sodium valproate for seizures
62
Define meningitis
Inflammation of the meninges (specifically leptomeninges - pia and arachnoid). Can be due to viral, bacterial or fungal cause.
Notifiable disease
63
Viral causes of meningitis
More common but less severe
- Coxsackie virus
- HSV (Herpes simplex virus)
- Varicella Zoster virus
- Mumps
64
Bacterial causes of meningitis
Most common - S. pneumoniae and N. meningitidis
Children - ^ and H influenzae
Elderly and pregnant - Listeria Monocytogenes (pregnant avoid cheese)
Newborns - ^ and Group B strep
65
How do N meningitidis, S pneumoniae, Group B strep and Listeria monocytogenes present on gram film
N meningitidis - Gram negative diplococci (Only one that causes non blanching rash!)
S pneumoniae/Group B strep - Gram positive cocci in chains
Listeria monocytogenes - Gram positive bacillus
66
Signs/symptoms of meningitis
Signs
- Neck stiffness, headache, photophobia (avoids light)
- Phonophobia (avoid sound)
- Papilloedema (optic disk swelling)
- Kernig sign
- Brudzinski sign
- Non blanching rash (N meningitidis only)
Pyrexia, reduced GCS
67
Investigations in meningitis
Blood culture 1st line - Bacterial or negative for viral
Lumbar puncture GOLD
Bacterial
- Cloudy/yellow
- Protein high
- Glucose low (<50% normal)
- WCC high (Neutrophil)
Viral
- Clear appearance
- Protein small raise/normal
- Glucose normal (>60% normal)
- WCC high (lymphocytes)
(Gram stain identifies bacteria and CSF PCR identifies viruses)
68
Fungal appearance of CSF in meningitis
Cloudy and fibrous
Protein high
Glucose low
WCC high - Lymphocytes!
69
Management of bacterial meningitis
Primary care: Immediate IV or IM benzylpenicillin (if suspected meningococcal) and hospital referral
Hospital
- Dexamethasone (steroid)
- Cefotaxime or Ceftriaxone IV
- Give Amoxicillin if under 3 months or over 50 to cover listeria
- Contact tracing and single dose oral ciprofloxacin for contacts
70
Complications of meningitis
Hearing loss
Seizures
Cognitive impairment
Hydrocephalus
Sepsis
71
Upper motor neurone lesion signs vs lower motor neurone lesion signs
Type of paralysis
-reflexia
Fasciculations
Babinski sign
Voluntary movement
Muscle tone and power
UMN
Spastic paralysis
Hyperreflexia
No fasciculations
Babinski positive
Voluntary movement slowed
Muscle tone and power kept
LMN
Flaccid paralysis
Hyporeflexia
Fasciculations
Babinski negative
Voluntary movement gone
Muscle tone and power lost
(babinski - toes curl up when bottom of foot is stroked
fasiculations - brief spontaneous contractions under skin)
72
Define Multiple sclerosis
Type 4 hypersensitivity reaction in which there is autoimmune attack against oligodendrocytes (which create myelin) in the CNS (Brain/Spinal cord). Causes plaques of demyelination.
Lesions vary, meaning plaques are “disseminated in space and time” - affect different areas of CNS at different times/ events.
73
Disease progression types in MS
- Relapsing remitting (most common) - Episodic flare ups without full recovery in between, meaning flares worsen over time. (Most common and often progress to secondary progressive)
- Secondary progressive - Symptoms start getting worse without remission
- Primary progressive - Symptoms worsen without remission (/)
- Progressive relapsing - Constant attack with superimposed flare ups
74
What is Uhtoff’s phenomenon and why does it occur in MS
Symptoms worsen with heat (e.g. hot bath) or exercise.
New myelin is inefficient, and doesn’t tolerate temperature rise effectively.
75
What triad is associated with MS
Charcot’s neurological triad
- Nystagmus (involuntary side-to-side/up-down rapid eye movements)
- Dysarthria (slowed, slurred speech)
- Intention tremor
76
Signs and symptoms of MS
- Optic neuritis usually first (Loss of vision, eye pain, pale optic disk, double vision)
- Internuclear ophthalmoplegia (eye muscle paralysis which impairs lateral gaze)
- Lhermitte’s sign - Electric shock sensation when flexing neck
- UPPER motor neurone signs
- Bowel, bladder, erectile dysfunction
- Ataxia
- Sensation loss
(Uhthoff’s and Charcot’s neurological triad already mentioned)
77
What criteria is used in diagnosis of MS
McDonald criteria (think McDonald’s M!)
- 2 or more relapses with evidence of 2 or more lesions, or one lesion with reasonable history of relapse
78
Investigations in MS (3)
MRI Brain/Spine
- Demyelinating plaques (new enhance with contrast, old don’t - showing dissemination in space and time)
Lumbar puncture
- Oligoclonal IgG bands in CSF
Visual evoked potential studies (responses to visual stimulus)
- Shows delayed nerve conduction
79
Management of MS
During acute relapse
- Oral/IV methylprednisolone first, cladribine
- Plasma exchange
Maintenance
- Interferon beta
- IV monoclonal antibodies
Cladribine second line in ongoing secondary progressive but causes cancer and is teratogenic
80
Management of complications of MS (3)
- Spasticity - Baclofen and gabapentin
- Neuropathic pain/depression - amitriptyline
- Physiotherapy for Spasticity and mobility impairment
81
What are some disease modifying drugs in MS with their indications
- relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
- secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
- IV Natalizumab - monoclonal antibody
- IV Ocrelizumab
- Oral fingolimod
- SC beta interferon
- SC Glatiramer acetate
82
What is Huntingtons
Autosomal dominant trinucleotide repeat disorder, which causes deterioration of nervous system and an excess of dopamine.
Also known as Huntington’s chorea (Chorea= involuntary jerky movements)
HTT gene on chromosome 4 - Mutated Huntingtin proteins aggregate in neuronal cells of caudate and putamen. Causes cell death of GABAergic and cholinergic neruones, causing ACh and GABA deficiency, so less dopamine inhibition. Excess dopamine causes excess movement.
Genetic anticipation - The more copies of the protein DNA polymerase adds on in the sperm, the earlier onset and more severe the disease
83
Signs/symptoms of Huntington's
Usually asymptomatic until 30-50 years
Prodromal - Irritability, depression, cognitive problems
Chorea - Jerky involuntary movements
Eye movement disorders
Dysphagia/dysarthria
Dementia, seizures, death within 15 years
84
Investigations in Huntington’s
Clinical diagnosis
- Genetic testing GOLD
CT/MRI - Caudate and striatal atrophy - increased size of lateral ventricles
85
Other causes of chorea
Hyperthyroid
Wilson’s
SLE
Dementia
86
Management of Huntington’s
Uncurable
Chorea
- Diazepam and tetrabenazine (Benzodiazepine and dopamine depleting agent)
87
Define Parkinson’s disease
Neurodegenerative movement disorder characterised by loss of dopaminergic neurones in Substantia Nigra Pars Compacta of basal ganglia.
Misfolded a synuclein proteins called Lewy bodies also present histologically (dark eosinophilic inclusions)
Causes a dopamine deficiency
88
What are the “parkinsonism” symptoms
Resting Tremor
Bradykinesia
Rigidity
Postural instability
89
Signs and symptoms of Parkinson’s (other than parkinsonism)
Resting tremor
Cogwheel rigidity
Shuffling gait
Reduced arm swing
Non motor:
Loss of smell
Sleep disturbance
Depression, anxiety
Dementia
90
Investigations in Parkinsons
Clinical diagnosis - bradykinesia and 1 other Parkinsonism sign
(Bradykinesia = slow, difficult movements. Smaller handwriting, shuffling gait, reduced arm swing etc)
Dopamine agent trial shows improvement
91
Management of Parkinson’s
If Severe: Levodopa + Decarboxylase inhibitor (boost dopamine and Di prevents L-dopa breakdown)
- Co-careldopa (Levodopa and carbidopa)
Otherwise:
Dopamine agonist
- Ropinirole
Monoamine oxidase B inhibitor (MAOBi) (stop breakdown of circulating dopamine)
- Selegiline
92
Complications of Parkinson’s
Disease progression and motor fluctuations (off periods when treatment stops working)
- Freezing (sudden stop of movement)
- Dyskinesia
- Dementia
93
Differentials of Parkinsonism
Benign Essential Tremor
Wilson’s disease
Encephalitis causing degeneration of substantia nigra
Trauma
94
Define Myasthenia Gravis
Type 2 hypersensitivity reaction causing autoimmune destruction of the post synaptic membrane at the neuromuscular junction of skeletal muscle. Antibodies to acetylcholine receptors in 85% of cases. (Anti-AChR)
2x in women.
Mostly affects facial muscles.
Strong association with thymoma/ thymic hyperplasia
95
Signs/symptoms of Myasthenia Gravis (6)
Mostly affects proximal and small muscles of head and neck
- Muscle weakness with fatigability, worse with exertion better with rest (e.g. patient counting to 50 will struggle in later numbers)
- Ptosis (eyelid droop) and diplopia (double vision)
- Jaw weakness and weak swallow (dysphagia)
- Head drop
- Facial paresis and slurred speech
- Snarl when attempting to smile (myasthenic snarl)
96
How to check for muscle fatigability on examination (3) and what should you check?
Repeated blinking causing ptosis
Counting to 50, speech becomes slurred and quieter towards end
Repeated abduction of one arm will result in weakness in said arm compared to other
Forced Vital Capacity should also be checked
97
Investigations in Myasthenia gravis
Antibodies
- AchR antibodies (anti-MuSK and anti-LRP4 less sensitive)
- Anti-MUSK
- Anti LRP4
CT thorax - look for thymus growth/thymoma (rule out)
Tensilon test - Used to be done but causes arrhythmia so dont even think about it
98
What are some drugs that exacerbate MG muscle fatigability
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
Most common factor is exertion!
99
Management of Myasthenia Gravis
Long acting acetylcholinesterase inhibitor
- Pyridostigmine first line
Immunosuppression (not started at diagnosis, usually started later)
- Prednisolone
Thymectomy may be needed (as many patients also have thymic hyperplasia)
100
Define Guillain Barre Syndrome, with causes
Acute autoimmune demyelination of the peripheral nervous system, following an upper resp tract or GI infection (e.g. gastroenteritis)
Acute, symmetrical, ascending weakness!
Can be caused by:
Bacteria:
- Campylobacter Jejuni
- M. pneumoniae
Viral:
- Cytomegalovirus
- EBV
101
Guillain barre disease course (4)
Initial GI or URT infection
Symptoms start after 2 weeks
Symptoms peak 2-4 weeks further
Recovery period of months to years
102
Pathophysiology of Guillain Barre syndrome
Molecular mimicry.
Pathogenic antigens resemble Schwan cell proteins so when immune response is launched, there is also destruction of myelin sheath. Demyelination occurs in patches down length of axon (segmental demyelination). Schwann cells can remyelinate so patients recover over time. Affects sensory and motor nerves.
103
Signs and symptoms of Guillain Barre syndrome
Symptom onset 2-3 weeks after preceding infection. Proximal muscles affected first
Symmetrical ascending weakness beginning in legs/feet.
Areflexia
Reduced sensation
Paraesthesia
Sensory loss
Respiratory distress if lungs affected
Autonomic dysfunction (bowel/bladder, sweating, raised BP/pulse, arrhythmia)
104
Investigations in Guillain Barre syndrome
Brighton criteria used to make clinical diagnosis, can be supported by:
- Nerve conduction studies (reduced conduction)
- Lumbar puncture (high protein in CSF, normal cell count and glucose)
Antibodies: subtype of GBS
AIDP (90%) - Anti ganglioside
Miller fisher syndrome (eyes affected first) - anti GQ1b
Do spirometry to assess risk of resp failure
105
Management of Guillain barre syndrome (2 treatments + 2 complication treatments)
IV immunoglobulin 5 days (CI if IgA deficiency)
Plasma exchange
VTE Prophylaxis (LMWH)
Ventilation if low FVC
106
Upper motor neurone lesion area
Anywhere from pre central gyrus to anterior spinal cord
107
Lower motor neurone lesion area
Anywhere from Anterior spinal cord to innervated muscle
108
How is muscle power affected in upper motor neurone lesions?
In arms flexors>extensors
In legs extensors>flexors
109
How is organisation of movement conducted
1 - Idea of movement in pre motor cortex
2 - Activation of UMN in motor cortex
3 - Impulse via corticospinal tract
4 - Modulation.
- Cerebellum fine tunes and Basal Ganglia green lights signal
5 - Movement and somatosensory info obtained by sensory tracts
110
Types of motor neurone disease
Amyotrophic lateral sclerosis (50% of patients)
typically LMN signs in arms and UMN signs in legs
in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
Primary lateral sclerosis
UMN signs only
Progressive muscular atrophy
LMN signs only
affects distal muscles before proximal
carries best prognosis
Progressive bulbar palsy
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis
111
Presentation of MND
asymmetric limb weakness is the most common presentation of ALS
the mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior is common
fasciculations
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but 'never' sensory
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MG brief pathophys
Unexplained destruction of Only UMNs and LMNs.
- No effect on sensory neurones (distinguishing point from MS etc)
- No effect on eyes (distinguishing from myasthenia gravis)
- No cerebellar involvement
- SOD1 mutation association in ALS
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Management of MND (2) What is prognosis
Riluzole (protects neurones from glutamate induced damage)
Respiratory support (non invasive at home)
50% die in 3 years
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Genetic associations with Alzheimer’s
- APoE e4 - APoE usually helps break down beta amyloid but e4 version less effective.
- Down’s (Trisomy 21) - Increased APP (amyloid precursor protein) production (APP gene also on C21). APP broken down incorrectly becomes beta amyloid.
- PSEN1, PSEN2
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Key histological findings in Alzheimers
Senile plaques of beta amyloid proteins (APP incorrectly broken down into sticky, insoluble b amyloid) (extracellular)
Neurofibrillary tangles of hyperphosphorylated tau proteins (intracellular)
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Signs and symptoms of Alzheimer’s (4)
Insidious onset and slow progressive decline
- Poor memory (short term early, long term late)
- Speech problems (receptive and expressive dysphagia)
- Loss of executive function (planning/problem solving)
- Disorientation/lack of recognition of places, people or objects
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How would Alzheimers affect behaviour (4)
Emotional instability
Depression/anxiety
Withdrawal/apathy
Disinhibition (Socially/sexually inappropriate behaviour)
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How would Alzheimer’s affect daily living (3)
Loss of independence
Early on loss of higher level function (finances, difficulties working)
Later loss of basic function (washing, eating, walking)
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How is Alzheimer’s diagnosed?
Based on DSM-V criteria and MMSE (Mini mental state examination) (25+ normal, <17 severely impaired)
MRI - Generalised brain atrophy with medial temporal then later parietal predominance
Brain biopsy is GOLD but can only be done after death
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Management of Alzheimer’s
Supportive: Improve cognitive function
- Exercise
- Music
- Board games
- Cognitive stimulation program
ACh-esterase inhibitor (Donepezil)
NMDA receptor antagonist (Memantine)
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Define Vascular dementia
Dementia caused by cerebrovascular damage causing hypoperfusion of neuronal cells.
Presents in patients with Stroke/TIA history, UMN signs and general condition decline.
Shows a stepwise decline with symptoms worsening after each cerebrovascular event.
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Investigations and treatment of vascular dementia
Mini mental state exam
CT/MRI of brain
- Multiple cortical and subcortical infarcts
- Atrophy of brain cortex
Treated with management of risk factors (lower BP, cholesterol, diabetes etc)
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Define Lewy Body Dementia
Dementia with Parkinsonism (Resting tremor, bradykinesia, rigidity, postural instability).
Alpha synuclein misfolds in neurones and aggregates to form Lewy Bodies, which deposit in cortex and substantia nigra causing neuron death.
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Symptoms of Lewy Body Dementia
Presents with dementia symptoms first
(memory, focus, speech, understanding issues)
Parkinsonism develops later
Sleep disorders like sleep walking/talking, and hallucinations are also very common in LBD
If Parkinsons first, it is Parkinson Dementia
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Management of Lewy Body Dementia
Dopamine analogue - Levodopa
ACh-esterase inhibitor - Donepezil
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Define Frontotemporal dementia
Focal degeneration of frontal and temporal lobes. Pick’s disease is most common type. Loss of over 70% of spindle neurones.
- Frontotemporal dementia (Pick's disease)
- Progressive non fluent aphasia (Chronic progressive aphasia)
- Semantic dementia
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Common features of frontotemporal lobar dementias
- Onset before 65
- Insidious onset
- Relatively preserved memory and visuospatial skills
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What is Pick's disease, how does it present and what macroscopic and microscopic changes are found
AKA Frontotemporal dementia.
Personality change and impaired social conduct! Hyperorality, disinhibition, increased appetite, perserveration beahviours.
Focal gyral atrophy with knife-blade appearance
Macro
- Atrophy of frontal and temporal lobes
Micro
- Pick bodies - spherical aggregations of tau protein (silver staining)
- Gliosis
- Senile plaques and neurofibrillary tangles
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What is CPA (chronic progressive apahasia)
Clues in the name.
Short utterances that are agrammatic. Comprehension preserved
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What is semantic dementia
Fluent progressive aphasia. Speech fluent but empty, not much meaning. Memory better for recent rather than remote events
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What are pick bodies
3R isoform of tau proteins
These become hyperphosphorylated and form tangles, causing atrophy in frontoteporal lobes
(In alzheimers, the isoform is 3R+4R)
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Give Primary and secondary causes of headache
Primary
- Migraine
- Tension
- Cluster
Secondary (to other pathology)
- GCA
- Cerebrovascular disease
- Subarachnoid haemorrhage
- Truma
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Define Migraine
Episodes of recurrent, unilateral throbbing headache. May or may not have an aura and often has visual changes (e.g. photophobia, diplopia etc).
Can last up to 72 hours, and classically preceded by an aura
- Visual, progressive, lasting 5-60 minutes.
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Common migraine triggers
CHOCOLATE
Chocolate
Hangover
Orgasms
Cheese
Oral contraceptive
Lie ins (tiredness)
Alcohol
Tumult (loud noise)
Exercise
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Signs/symptoms of typical/atypical aura
Typical: Lasts 5-60 mins and fully reversible.
- Visual changes (zigzag lines, distortion etc)
- Smell changes
- Paraesthesia
Atypical: >60 mins
- Diplopia
- Motor weakness (hemiplegic migraine!)
- Poor balance
- Reduced consciousness
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Investigations in migraine
Clinical diagnosis:
Migraine with/without aura
(at least 2/4 symptoms, 1 associated symptom, no attribution to another disorder)
CT/MRI to exclude secondary haemorrhage
ESR exclude GCA
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Treatments of migraine
Acute prevention
- Oral Sumatriptan (5-HT receptor agonist (mimic serotonin))
with/without aspirin
Prophylaxis
- Propanolol
- Amitriptyline
- Topirimate (antiemetic)
AVOID Opiates
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Define Tension headache
Most common type of headache.
Bilateral “pressing/tight” headache. Lasts minutes to hours. No associated symptoms except photo OR phonophobia
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Define cluster headache
Severe, unilateral periorbital headache, with associated autonomic features, affecting same side face/eyes. Lasts 15-180 mins.
AKA Trigeminal Autonomic Cephalalgia
140
Signs/symptoms of cluster headache
Severe unilateral, periorbital, crescendo headache, lasting 15mins to 3 hours. Clusters of headaches,
(Boring/hot poker pain “worst pain ever”)
Ipsilateral autonomic symptoms
- Ptosis (eyelid droop)
- Miosis (excessive constriction of pupil of eye)
- Teary, bloodshot eye
- Nasal congestion/rhinorrhoea
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Diagnosis and management of cluster headache
Clinical diagnosis (5+ similar headaches)
Acute
- Triptans
- High flow oxygen
(AVOID paracetamol, NSAID, Opioids)
Prophylaxis
- Verapamil (CCB)
- Prednisolone, 2-3 weeks
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Define trigeminal neuralgia
Severe, unilateral “electric” pain along distribution of trigeminal nerve lines. Extremely increased risk in demyelinating disease. Attacks last seconds.
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Pathophysiology of trigeminal neuralgia
Vascular loop (MC superior cerebellar artery) compresses nerve near nerve root entry zone. Compression causes poor conduction along nerve root, causing pain.
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Common triggers of trigeminal neuralgia pain
Light touch (washing, shaving, brushing teeth)
- Talking
- Cold weather
- Spicy food
- Caffeine and citrus
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What are the branches of the trigeminal nerve
Ophthalmic
Maxillary
Mandibular
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Signs/symptoms of trigeminal neuralgia
Facial pain
- Electric/stabbing pain
- Very severe
- Trigeminal distribution
- Unilateral
- Provoked (touch, cold etc)
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Investigations and management of trigeminal neuralgia
Clinical but can MRI brain (space occupying lesion, demyelination etc)
Carbamazepine first line
Surgery (microvascular decompression)
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Define Cauda Equina
Compression of the bundle of nerves below the end of the spinal cord (known as cauda equina). Causes bilateral lower limb weakness/saddle anaesthesia
Medical emergency that requires immediate decompression
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Signs/symptoms of Cauda Equina
Severe lower back pain, bilateral lower limb weakness and reduced sensation. LMN signs!
Saddle anaesthesia (numbness/reduced tone in perianal region, groin, inner thigh)
Decreased reflexes and leg weakness/paralysis
Erectile dysfunction
Bladder/bowel dysfunction.
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Investigations and treatment of cauda equina
URGENT MRI spine GOLD
Emergency decompressive laminectomy (vertebra removal) within 24-48 hours, or permanent weakness/dysfunction
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Brief overview of the anatomy of the spine
Originates at base of medulla oblongata, exiting through foramen magnum, ending at conus medullaris at L2
Consists of 5 sections of vertebrae, with 31 spinal nerves arising from this
- Cervical (7)
- Thoracic (12)
- Lumbar (5)
(Spinal cord ends at L1, conus medullaris begins at L2)
- Sacrum (5 - fused)
- Coccyx (4 - fused)
Beyond L2 are bundle of nerves called “cauda equina”
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Causes of spinal cord compression
Vertebral body neoplasms (thoracic most likely)
- disc herniation
- disc prolapse
- infection
- Trauma
- Spinal stenosis
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How do spinal cord lesions’ sensory and motor symptoms present
Motor - Contralaterally
Sensory - Ipsilaterally (same side)
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What nerve roots are implicated in knee jerk, big toe jerk and ankle jerk reflexes
Knee jerk - L3/4
Big toe - L5
Ankle - S1
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Signs/symptoms of spinal cord compression
Progressive (Hours-weeks/months) back pain and progressive leg weakness. Motor signs contralateral
UMN signs above level of lesion
LMN below level of lesion
Sensory loss 1-2 cord segments below lesion level
bladder/sphincter involvement is a late, bad signs
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What levels are the lesions in L5 nerve root compression and sciatica
L5 nerve root compression - L4/L5
Sciatica - L5/S1
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Specific causes of peripheral neuropathy
DAVID
Diabetes
Alcoholism
Vitamin B12 deficiency
Infective/inherited (GBS/Charcot-Marie-Tooth)
Drugs e.g. isonazid
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Define Brown Sequard syndrome
Damage to one half of the spinal cord, resulting in a specific pattern of symptoms:
- Ipsilateral motor weakness
- Ipsilateral loss of proprioception (position sense), light touch, vibration at level of lesion
- Contralateral loss of pain and temperature sense below level of lesion
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Gold investigation and management of Brown sequard
Investigations
- EMG (electromyography)
- MRI Spinal cord
Management
- Treatment of underlying condition
- High dose steroids
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Define/ explain pathophysiology of Charcot Marie Tooth syndrome
Group of inherited diseases (autosomal dominant) that cause axonal/myelin dysfunction. CMT1 and CMT2 most common
CMT 1 - loss of myelin sheath (onion bulb myelin due to schwann cell repair)
CMT 2 - Neuronal mitochondrial dysfunction = neurone death
Causes atrophy of muscle when motor neurones affected
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Signs/symptoms of charcot marie tooth
Weakness in lower legs and hands.
Loss of muscle tone and reflexes
Foot drop and claw hand
Tingling/burning in hands and feet
Thickened palpable nerves and hammer toes
“Inverted champagne bottle” legs due to distal muscle wasting
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Define Duchenne Muscular Dystrophy
X linked recessive condition characterised by severe muscle dystrophy due to absence of Dystrophin protein
163
Signs/symptoms of duchenne muscular dystrophy
Usually presents 3-5 years.
- Weakness in pelvic muscles
- Waddling gait
- Gowers sign: due to inability to get up normally, they get into downward dog position then climb their hands up their legs to stand.
- Fat calves due to buildup of fat and fibrotic tissue rather than muscle
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Nerves implicated in wrist drop and claw hand
WD - Radial
Cl - Ulnar (4th and 5th fingers claw)
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What is Bells palsy
Acute, unilateral, idiopathic facial nerve paralysis.
Aetiology unknown, but suspected HSV
166
How does Bells palsy present
LMN Facial nerve palsy -> Forehead affected
(UMN spares upper face)
- Post auricular pain
- Altered taste
- Dry eyes
- Hyperacusis
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How is Bell's palsy treated
Prednisolone within 72 hours, maybe add antiviral
most people with Bell's palsy make a full recovery within 3-4 months
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What is temporal arteritis
AKA Giant cell arteritis. Branches of carotid artery (
Occurs in >50, usually ~70. Strong association with Polymyalgia Rheumatica.
Early recognition and treatment can minimise risk of complications e.g. permanent sight loss.
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How does temporal arteritis present
- Rapid <1 month onset
- Unilateral headache around temple/forehead
- Diminished/absent temporal artery
- Jaw claudication
- Blurred/double vision
- Optic disc pallor
- Scalp tenderness (Painful to comb)
- Fever, muscle aches, weight loss, loss of appetite
170
GCA typical presentation
50+ white female with unilateral temple headache, scalp tenderness (painful to comb), jaw claudication and vision changes.
171
Pathophys of GCA
Granulomatous vasculitis of large/medium arteries. Arteries become inflamed, intima is thickened and vascular lumen is narrowed. Usually cerebral (temporal) arteries affected:
- Superficial temporal artery: Headache/scalp tenderness
- Mandibular artery: jaw claudication
- Ophthalmic artery: visual loss (retinal ischaemia)
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Diagnostic criteria in GCA
3 of:
- Over 50
- New headache
- Temporal artery tenderness/diminished pulse
- ESR Raised
- Abnormal temporal artery biopsy
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Management of GCA
High dose prednisolone
- Sight loss (amaurosis fugax!)
Should be dealt with ASAP or could lead to permanent blindness
- Ischaemic cranial complications (Visual loss/stroke)
- Aortic aneurysm
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In cases where long term steroids are given, what 2 systems should be protected and how is this done?
GI (stomach and oesophagus) and Bones
- PPI (omeprazole)
- Alendronate (bisphosphonate)
- Ca2+ and vitamin D
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Define polymyalgia rheumatica
Condition that causes pain stiffness and inflammation in neck, shoulders and hips. Limits range of motion. Occurs alongside GCA often.
Morning pain/stiffness in shoulders etc. Leads to fatigue, fever, weight loss, anorexia and depression
Raised ESR but CK and EMG normal
Managed with prednisolone
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Normal pressure hydrocephalus presentation triad and sign on imaging
urinary incontinence, cognitive impairment, and gait disturbance
ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
