Neurology Flashcards
1
Q
What are the risk factors for MS?
A
- Female sex
- Age 25-35
- Family history
- Latitude
- EBV
2
Q
Describe the two main types of MS
A
1) Relapsing/remitting - clear relapses followed by recovery (can become secondary progressive)
2) Primary progressive (about 10%)
3
Q
Describe the potential clinical features of MS
A
- Sensory (most common presentation, first symptom in 40% people)
- Numbness/coldness/pin and needles
- Swelling/tightness
- Vibration and proprioception loss
- Lhermitte’s phenomenon
- Optic neuritis
- Pain on eye movement
- Relative afferent pupillary defect
- Uhtoff’s phenomenon
- Leg weakness/paraplegia
- UMN signs
- Bladder/bowel/sexual dysfunction
- Tremors
- Double vision/nystagmus
- Cranial nerve palsies
- Dizziness
- Dysarthria
- Gait abnormalities/ataxia/impaired ambulation
- Fatigue
- Minor cognitive impairment
- Epilepsy
- Depression
- Hallucinations/psychosis
4
Q
How would you diagnose MS?
A
Two separate episodes of CNS demyelination separated in space and time - can be clinical diagnosis or imaging (MRI brain/spine, CSF)
Diagnosis is made using the McDonald Criteria 2017.
5
Q
What would you expect to see in the lumbar puncture of a patient with MS?
A
- Oligoclonal bands from antibodies
- Slightly elevated WCC
6
Q
What condition may you suspect if there was oligoclonal bands present in the CSF but NOT the serum?
A
MS
7
Q
What condition may you suspect if there was oligoclonal bands present in the CSF and the serum?
A
- Neurosyphilis
- Lyme disease
- Behcet’s disease
- SLE
8
Q
What conditions would produce plaque lesions visible on MRI?
A
MS Old age Cerebral ischaemia Sarcoidosis Behcet's syndrome
9
Q
What differentials would you consider in a patient with suspected MS?
A
Optic neuritis
Spinal cord syndromes e.g. compression, vitamin B12 deficiency, HTLV-1 myelopathy, ALS
Brain stem syndromes e.g. tumour, encephalitis
Inflammatory disease e.g. SLE, sarcoid, Behcet’s
Infection e.g HIV, Lyme disease, syphilis
10
Q
How would you assess the level of disability in a patient with MS?
A
Kurtzke disability status scale
11
Q
What treatments could you consider for symptomatic relief in a patient with MS?
A
Accelerate recovery after relapse: IV methylprednisolone for 3 days
Relieve pain and treat depression: amitriptyline, Gabapentin
Reduce Lhermitte’s phenomenon or trigeminal neuralgia: Carbamazepine
Reduce tremor: stereotactic thalamotomy
Maximise function and reduce spasticity: physiotherapy/OT/speech therapy
Reduce spasticity: baclofen, dantrolene, tizanidine, botox
Reduce fatigue: amantadine, pemoline, modafinil
Reduce ataxia: isoniazid
Reduce unstable bladder symptoms: intermittent self-catheterisation
Reduce uncoordinated bladder symptoms: oxybutynin, tolterdoine
Treat erectile dysfunction: sildenafil
Constipation: bulking agents and stool softeners
12
Q
What disease-modifying treatments could you consider in a patient with MS?
A
Beta-inteferons Natalizumab Glatiramer acetate Daclizumab Alemutzumab Dimethyl fumarate Teriflunomide Fingolimod Cladribine Ocrelizumab
13
Q
What monitoring would you do on a patient with MS being treated with beta-interferon?
A
LFTs
FBC
14
Q
What are the side effects of beta-interferon?
A
Local irritation
Flu-like symptoms
Deranged LFTs
15
Q
What are the side effects of Natalizumab?
A
Dizziness Itch Rash Shivering Infection PML
16
Q
What MS treatments can cause PML?
A
Natalizumab
DImethyl fumarate
Fingolimod
17
Q
What monitoring would you do on a patient with MS being treated with Natalizumab?
A
FBC LFT U&E JCV antibody ttest MRI scan
18
Q
What are the side effects of Glatiramer acetate?
A
Injection site reaction, lipoatrophy
19
Q
What monitoring would you do on a patient with MS being treated with Glatiramer acetate?
A
None
20
Q
What are the side effects of Daclizumab?
A
New autoimmune disease
Infection
21
Q
What are the side effects of dimethyl fumarate?
A
Flushing
GI upset
Lymphopenia
PML
22
Q
What monitoring would you do on a patient with MS being treated with Dimethyl fumarate?
A
FBC
LFTs
U&Es
MRI scan
23
Q
What are the side effects of Teriflunomide?
A
GI upset
Hair thinning
Rash
24
Q
What monitoring would you do on a patient with MS being treated with Teriflunomide?
A
FBC
LFTs
25
What are the side effects of Fingolimod?
```
Bradycardia
Heart block
Infection
Lymphopenia
Liver dysfunction
PML
```
26
What monitoring would you do on a patient with MS being treated with Fingolimod?
```
VZV screen
FBC
LFTs
BP
Eye and skin examination
MRI scan
```
27
What are the side effects of Cladribine?
Lymphopenia
| Headache
28
What monitoring would you do on a patient with MS being treated with Cladribine?
FBC
LFT
TB, HIV, Hep B, VZV screen
MRI scan
29
What are the side effects of Alemutzumab?
Infusion reaction
Infection
Thyroid problems
Clotting disorders
30
What monitoring would you do on a patient with MS being treated with Alemutzumab?
```
FBC
U&E
TFTs
VZV antibody
HPV, Hepatitis, FB screening
MRI scan
```
31
What are the side effects of Ocrelizumab?
Infusion reaction
Chest infection
Herpes infection
32
What monitoring would you do on a patient with MS being treated with Ocrelizumab?
FBC
| Hep B screen
33
Which factors would indicate a better prognosis in a patient with MS?
Relapsing-remitting disease
Female sex
Sensory symptoms or optic neuritis at onset
34
Risk factors for stroke
```
Hypertension
Diabetes
Smoking
Family history
High cholesterol
Excess alcohol intake
COCP
Obesity
Depression
Heart disease e.g. AF, MI, left ventricular dilatation
```
35
Define a stroke
A clinical syndrome characterised by acute onset neurological deficit due to dysfunction of the brain, caused by a problem with the blood supply, which does not resolve within 24 hours
36
What percentage of strokes are ischaemic?
85%
37
Define a TIA
A clinical syndrome characterised by acute onset neurological deficit due to dysfunction of the brain, caused by a problem with the blood supply, which resolves within 24 hours
38
What are the causes of intracerebral haemorrhage
Hypertension
Charcot-Bouchard aneurysms
Arteriovenous malformation
Bleeding disorders
39
Where do intracerebral haemorrhages most commonly occur?
Basal ganglia (50%)
Lobar white matter (20%)
Pons (10%)
Cerebellum (10%)
40
Which features in a history would make you suspect a patient had a TACI stroke?
```
Rapid onset
Hemiparesis affecting face, arm and leg
Homonymous hemianopia
Drowsiness
Complete aphasia (if dominant side affected)
Inattention/neglect (if non-dominant side affected)
Transient dysarthria
Impaired swallowing
Incontinence
```
41
Which features in a history would make you suspect a patient had a PACI stroke?
MCA inferior branch infarction:
Hemianopia
Wernicke's aphasia (if dominant side affected)
Constructional aphasia (if non-dominant side affected)
MCA superior branch infarction:
Hemiparesis
Broca's aphasia (if dominant side affected)
Neglect (if non-dominant side affected)
MCA distal branch infarction:
Weakness of one limb
Isolated higher function deficit
42
Which features in a history would make you suspect a patient had a POCI stroke?
Large vessel syndrome:
Contralateral hemisensory homonymous hemianopia
Contralateral hemisensory loss
Higher function disturbance
Basilar artery occlusion:
Locked in state
```
Vertebral artery occlusion:
No deficit OR
Nystagmus
Dysarthria
Diplopia
```
43
Which features in a history would make you suspect a patient had a LACI stroke?
Internal capsule lesion:
Face, arm and leg weakness
Posterior internal capsule, midbrain or pons lesion:
Motor hemiparesis with cerebellar type ataxia on ipsilateral side
Thalmic lesion:
Hemisensory loss
Dysarthria
Tongue/face weakness
Hand clumsiness
44
Which features in a history would make you suspect a patient had an anterior circulation TIA?
Amaurosis fungax (fleeting blindness in one eye)
Aphasia
Dyslexia
Dysgraphia
Unilateral weakness or sensory loss in face, arm or leg
45
Which features in a history would make you suspect a patient had a posterior circulation TIA?
Homonymous visual field loss
Dysarthria
Combined brain stem symptoms
Bilateral or unilateral weakness or sensory loss in face, arm or leg
46
What investigations would you consider in a patient with a suspected stroke?
1) MRI/CT SCAN
2) Risk factors for atheroma:
- BP
- Blood glucose
- Cholesterol
- TFTs
- LFTs
3) Sources of embolism
- ECG
- Echo
- Blood cultures
- 24-hour tape
- Carotid doppler, angiogram or MRI angiography
4) Causes of thrombosis
- FBC
- Thrombophilia screen
- Sickle cell screen
5) Causes of inflammatory vascular disease
- ESR
- ANA, anticardiolipin antibodies
- Syphilis serology
- Temporal artery biopsy
47
Which factors worsen the outcome for a stroke patient?
Hypoxia
Hyperglycaemia
Over-hydration
48
What might you find on examination and investigation of a patient with a TIA?
No neurological abnormality
49
What would you do if a patient presented with a suspected stroke?
1) CT/MRI scan (within an hour)
2) Thrombolysis with alteplase within 4 hours
3) CT angiogram
4) Consider thrombectomy within 6 hours of onset
5) Aspirin 300mg
50
What are the indications for doing a CT/MRI scan in a patient with a suspected stroke?
- Indication for thrombolysis or early anticoagulant treatment
- On anticoagulants
- Known bleeding tendency
- GCS <13
- Unexplained progressive or fluctuating symptoms
- Papilloedema, neck stiffness or fever
- Severe headache at onset of symptoms
51
What are the contra-indications to treating a stroke patient with alteplase?
- Surgery within 2 weeks
- Ischaemic stroke within 3 months
- Significant trauma
- INR >1.5
- On anticoagulants
- No motor deficit
- Symptom onset >4 hours
- Impaired consciousness
- Recent eplieptic seizure
- History of intra-cranial haemorrhage
- Pregnant or delivered within 15 days
- Lumbar puncture or ABG within 7 days
- BP >185/110
52
What are the indications for a decompressive hemicraniectomy in a stroke patient?
- Age <60
- Clinical deficits suggestive of MCA infarction, NIHSS score >15
- Decrease in consciousness
- Signs of infarct of >50% of MCA territory on CT scan
53
How would you prevent a second stroke?
- Smoking cessation
- Reduce alcohol intake
- Aspirin 300mg for 2 weeks + clopidogrel/dipyridamole
- Anticoagulation (Apixiban)
- Antihypertensives
- Good glycaemic control
- Lower cholesterol (statin)
- Symptomatic Carotid endarectectomy
54
Complications of stroke
```
Raised intracranial pressure and herniation
Aspiration pneumonia
Contractures
DVT
Bed sores
UTI
Constipation
Depression
Epilepsy
Thalmic pain
Social problems
Financial problems
```
55
What screening tests could you use in a patient with confusion?
- Addenbrooke's Cognitive Assessment (ACE-III)
- Mini-Mental State Examination
- Clock Drawing Test
- Mini-Cog
- Time and Change
- 7-Minute Screen
- Abbreviated Mental Test
56
What investigations would you consider in a patient with confusion/delirium?
1) Evidence of biological derangement
- FBC
- U&Es
- TFTs
- B12/folate levels
- LFTs
- Inflammatory markers
- Syphilis/HIV screen
- Glucose
- ABG
2) Infection screen
- Urine/sputum culture
3) Radiology
- CXR
- CT/MRI head
4) Specialised blood tests
- Autoimmune/vasculitis screen
- Paraneoplastic onconeural antibodies
- Autoimune encephalitic antibodies (VGKC and NMDA receptor)
5) EEG
6) Lumbar Puncture
57
List some potential causes of delirium
- Hyper/hyponatremia
- Hypercalcemia
- Hypoglycaemia
- Hepatic failure
- Hypothyroidism
- Drugs - antiparkinsonian medications, recreational drugs, alcohol, alcohol withdrawal
- Infection - sepsis, UTI, meningitis, encephalitis, pneumonia, malaria
- Epilepsy
- SLE
- Limbic encephalitis
- Stroke
- Subarachnoid haemorrhage/subdural haematoma
- Trauma
- Hydrocephalus
58
What are the 4 defining features of delirium
```
1) Acute onset and fluctuating symptom course
AND
2) Inattention
AND
3) Disorganised thinking
OR
4) Altered level of consciousness
```
59
List the clinical features of delirium
```
Alterations in arousal
Fluctuating attention
Distracted
Disorganised thinking
Slow responses, going off on tangents
Slurred speech
Hallucinations
Disputed sleep
Slow movements
Short-term memory loss
Emotional instability
```
60
What differentials would you consider in a patient with suspected delirium?
- Dementia
- Aphasia
- Schizophrenia/psychosis
61
List the 3 key features of dementia
1) Progressive decline
2) Acquired widespread loss of mental function in >1 cognitive domain
3) in clear consciousness
62
List the clinical features of dementia
- Memory loss
- Language impairment
- Disorientation
- Abstract thinking
- Personality changes
- Psychiatric symptoms
63
What investigations would you consider in a patient with suspected dementia?
- CT/MRI head (loss of tissue, wide sulk, large ventricles)
- FBC and ESR
- LFTs
- U&Es
- TFTs
- Vitamin B12 and folic acid
- Syphilis serology
- Neuropsychological assessment
- EEG
64
Describe the pathophysiology of Alzheimer's disease
- 70% of all dementias
- Insidious onset and progressive gradual decline
- Extracellular neuritic beta amyloid plaques
- Intracellular neurofibrillary tangles
- Cholinergic system degerneation and neuron loss
- Cerebral atrophy, particularly in the temporal lobes
65
List the 3 stages that Alzheimer's disease commonly presents with
1) Memory disturbance
2) Global cognitive decline with relatively intact personality
3) Severe global decline with disorders of social behaviour and function
66
List the clinical features of Alzheimer's disease
- Short-term memory loss
- Language difficulties
- Praxis
- Visuospatial difficulties
- Executive functioning difficulties
- Behavioural changes
- Anosognia
- Depression
- Delusions/hallucinations
67
What might you find on examination of a patient with Alzheimer's disease
Gegenhalten pattern of increased tone
| Relapse of primitive reflexes
68
What treatment could you consider in a patient with Alzheimer's disease?
- Central cholinesterase inhibitors (donepezil, galantamine, rivastigmine)
OR
- NMDA-receptor antagonists (Memantine)
69
Risk factors for vascular dementia
- Smoking
- AF
- Diabetes
- Hypertension
70
List the 5 sub-types of front-temporal dementia
1. Behavioural variant FTD
2. Primary progressive aphasia
3. Semantic dementia
4. Progressive non-fluent aphasia
5. FTD-MND
71
List the clinical features of fronto-temporal dementia
- Behaviour and personality changes
- Lack of insight
- Progressive memory deficit
- Variable movement disorders
- Language impairment
- Hyperorality
- Perservation
- Hypersexual behaviour
72
Describe the pathology of Lewy Body dementia
Lewi bodies in the cerebral cortex
73
Clinical features of Lewy Body dementia
- Fluctuations in mental state
- Early delusions and hallucinations
- Mild extrapyramidal signs
- Neuroleptic hypersensitivity
- Unexplained falls/transient changes in consciousness
74
List the risk factors for a patient with Parkinson's developing dementia
- Age >70
- Depression
- Confusion/psychosis on Levadopa
- Facial masking at presentation
75
List some potentially reversible causes of dementia
- Drug toxicity
- Metabolic disturbance
- Autoimmune/paraneoplastic encephalopathy
- Mass/lesions
- Infection (meningitis, syphilis)
- Inflammatory disorders (SLE, sarcoid)
- Endocrine disorders (thyroid, parathyroid)
- Nutritional disease (B12, thiamine, folate)
76
List the clinical features of Horner's syndrome
- Ptosis (may be partial)
- Miosis
- Anhydrosis
- Enopthalmos (eye looks sunken)
- (different colour pupils - only in congenital Horner's or lesions in young children)
77
Describe the pathology of Horner's syndrome
A lesion in the sympathetic trunk
78
List the causes of Horner's syndrome
- Idiopathic
- Pancoast tumours
- Neuroblastoma (paediatric)
- Neck/eye/cerebellar tumours
- Stroke
- Migraine/cluster headache
- Internal carotid dissection/thrombosis
- Birth trauma to shoulder/neck
- Arnold-Chiari malformation
- Demyelinating disease - MS, Guillain-Barre syndrome
- Inflammatory disease
79
What investigations would you consider in a patient with suspected Horner's syndrome?
- CXR
- CT/MRI head
- Carotid doppler
- Bloods
- Cerebral angiogram
- LP
80
What is the treatment for Horner's syndrome?
No treatment available - treat underlying disease
81
Describe the pathophysiology of Parkinson's disease
Loss of dopaminergic cells in the substantia nigra caused by Lewy bodies
82
Which drugs can induce Parkinsonism?
- Chlorpromazine/prochlorperazine
- Phenothiazines
- Antipsychotics
83
Describer the 4 cardinal features of Parkinson's
1. Resting tremor
2. Rigidity
3. Bradykinesia
4. Postural instability
84
Describe the clinical features of Parkinson's disease
- Insidious onset and gradually progressive symptoms
- Often unilateral at presentation
- Resting tremor (coarse and slow, usually affecting the hands, pill-rolling tremor)
- Cogwheel rigidity
- Bradykinesia
- Postural instability - stooped posture with flexed elbows
- Difficulty in fine movement e.g. writing
- Fatigue
- Lack of facial expression
- Slowed blink rate
- Dysrathria - monotonous voice which trails off
- Micrographia
- Loss of arm swing
- Shuffling gait
- Difficulty starting or stopping movements
- Altered higher function
85
What differentials would you consider in a patient with suspected Parkinson's disease
- Drug-induced Parkinson's
- Essential tremor (not present at rest, more prominent on sustained posture or movement)
- Wilson's disease (in younger patients)
- Lewy Body dementia (in patients with loss of higher function and prominent hallucinations on low dose therapy)
- Unilateral hemiparesis
- Depression
- Diffuse cerebrovascular disease
- Normall pressure hydrocephalus
86
How would you diagnose Parkinson's disease?
Clinical diagnosis - Bradykinesia, rigidity, tremor, postural instability
87
What investigations could you consider in a patient with suspected Parkinson's disease with diagnostic uncertainty?
- CT/MRI head (look for normal pressure hydrocephalus or small vessel disease)
- Trial of dopaminergic drugs
- DAT scan
- Test eye movements and standing/lying BP
88
How would you treat a patient with Parkinson's who was under 70 years old?
1) Amantadine OR selegiline/rasagiline
2) Dopamine agonists (Bromocriptine, lisuride, pergolide OR carbergoline, apomorphine, ropinirole, pramipexole, rotigotine)
89
How would you treat a patient with Parkinson's who was over 70 years old?
1) Amantadine OR selegiline/rasagiline
| 2) Levodopa preparations (Sinemet or Madopar)
90
Side effects of Sinemet or Madopar
- Wearing off
- Dyskinesia
- Impulsive/compulsive behaviour
- GI upset e.g. nausea
- Hypotension
- Psychological problems
- Withdrawal
91
What anti-emetic would you use to treat nausea in a patient with Parkinson's?
Domperidone
92
Side effects of dopamine agonists (bromocriptine, carbergoline)
- Cardiac valvular fibrosis, pulmonary fibrosis, abdominal fibrosis (requires monitoring)
- Nausea
- Dizziness
- Confusion
- Sudden bouts of sleepiness
- Altered behaviour e.g. gambling, hyper-sexuality
93
Side effects of dopamine-releasing agents (amantadine)
- Dizziness, falls
- Dry mouth
- Peripheral oedema
- GI upset
- Insomnia
94
Side effects of monoamine oxidase B inhibitors (Selegiline, Rasagiline)
- Dizziness, falls
- Dry mouth
- Peripheral oedema
- GI upset
- Insomnia
95
Benefits of Amantadine or selegiline/rasagiline
Smooth out delivery of levodopa
96
Side effects of Co-methyl transferase (COMT) inhibitors e.g. Entacapone
- Behavioural disturbances
- Stiff muscles
- Fever, sweating
- Tachycardia
- Tremors
- Diarrhoea
97
What is an effective treatment option for patients with tremor caused by Parkinson's?
Anticholinergics (Procyclidine or benzotropine)
98
Benefits of anticholinergics in Parkinson's disease
- Effective in mild, early tremor
| - Effective in salivary drooling
99
Side effects of anticholinergics (procyclidine or benzotropine)
- Drowsiness
- Dry mouth
- Dizziness
- GI upset
- Flushing
- Blurred vision
100
List 3 complications of long-term treatment of Parkinson's disease
1) Fluctuations
2) Dyskinesia
3) Drug failure
101
How could you deal with fluctuations in control of Parkinson's disease?
- Increase dose frequency
- Add selegiline, COMT inhibitor or a dopamine agonist
- Change to a controlled release form of Levodopa
102
How would you deal with a dyskinesia caused by treating Parkinson's disease?
- Add selegiline/rasagiline, dopamine agonist or amantidine
| - Reduce dose of levodopa
103
Name 2 common forms of 'Parkinson's plus syndromes and their symptoms
1) Multisystem atrophy - autonomic failure, cerebellar signs, UMN signs
2) Progressive supranuclear palsy/Steele-Richardson syndrome - reduced ability to move eyes voluntarily, loss of postural reflexes and dsyrrthria
104
Diagnostic criteria for a TACS stroke
ALL of
1) Total hemiparesis and/or hemiparathesis
2) Higher function dysfunction e.g. cognitive impairment, dysphasia, visuo-spatial defects (visual neglect, tactile neglect)
3) Homonymous hemianopia
105
Diagnostic criteria for a PACS stroke
2 of:
1) Total hemiparesis and/or hemiparathesis
2) Higher function dysfunction e.g. cognitive impairment, dysphasia, visuo-spatial defects (visual neglect, tactile neglect)
3) Homonymous hemianopia
106
Diagnostic criteria for a LACS stroke
1 of:
1) Total hemiparesis and/or hemiparathesis
2) Higher function dysfunction e.g. cognitive impairment, dysphasia, visuo-spatial defects (visual neglect, tactile neglect)
3) Homonymous hemianopia
107
Diagnostic criteria for a POCS stroke
Signs of:
1) Cerebellar signs
2) Cranial neuropathy
3) Isolated hemianopia
4) Bilateral motor/sensory loss
5) Conjugate eye movements
108
What is the most common cause of headache seen in general neurology clinic?
Chronic daily headache syndrome with angles excess
109
How would you treat an acute migraine attack?
1) 300-900mg of aspirin at start of attack
| 2) Triptan (if not relieved by aspirin)
110
How could you prevent migraines?
1) Propanolol
2) Topiramate
3) Amitryptilline
4) Nortriptylline
5) Candesartan
6) Botulin toxin
7) Sodium valproate in men
111
Risk factors for functional disorders
- Female
- Young
- History of abuse/trauma
112
Which features in a history would make you suspect a functional disorder?
- Long list of symptoms
- History of previous functional symptoms
- Dissociative symptoms
- Emotional symptoms
- History of deliberate self-harm or drug overdose
- 'Belle indifference'
- Collapsing weakness
113
What general features should you ask about in a patient with suspected functional disorder?
- Pain
- Fatigue
- Sleep disturbance
- Memory and concentration problems
114
Describe the main features which would differentiate a pseudo-seizure from an epileptic seizure?
- Semi-purposeful thrashing
- No cyanosis
- Normal respiration
- No tongue biting
- No incontinence
- Feeling 'crazy'
- Numbness and tingling
- Sweating
- Shortness of breath
- Heart racing
- Chest pain
- Gradual onset
- Asynchronous limb movements
- Side to side head shaking
- Closed eyelids, resistant to opening
- Retained pupillary light reflex
- Convulsions >2 mins
- Quick post-ictal recovery
115
What investigations would you consider to diagnose pseudo-seziures?
1) EEG during attack - must be NORMAL
| 2) Post-seizure prolactin - will be raised 15-20 mins after a tonic-clonic epileptic seizure
116
Which features would differentiate functional paralysis from true paralysis?
- Inconsistency of power in formal testing and during general use
- Collapsing weakness
- Co-contraction
- Slow and jerky arm drop test
- Positive Hoover's sign
- Normal plantar response
- Normal movement restored when using hypnosis or sedatives
117
Clinical features of functional weakness of the face
- Persistently depressed eyebrow
- Variable inability to elevate frontalis
- Over-activity of orbicular
- Photophobia
118
What are the general features common to all functional movement disorders?
- Rapid onset
- Variability in frequency, amplitude or distribution
- Improvement with distraction/worsening with attention
119
How would you make a diagnosis of functional movement disorder?
- Complete remission demonstrated after admission of general anaesthetic
120
What are the general features of a functional gait disturbance?
- Variability
- Improvement with distractions
- Excessive slowness
- Falling towards/away from the doctor
121
Describe the typical patterns of functional gait disturbance
- 'Walking on ice' pattern - cautious, broad base, decreased stride length and height, stiff knees and ankles, arms abudcuted)
- Uneconomic postures with waste of muscle energy (flexed knees/hips)
- Sudden knee buckling
- Pseudo-ataxia (crossed legs, unsteady gait, sudden side steps)
- Dragging gait
122
What are the general features of a functional sensory loss?
- Non-recognisable pattern
- Complete sensory loss in all modalities
- Inconsistencies in repeat testing
- Discrepancies in functional loss
123
Describe the clinical features of hemi-sensory syndrome
- Intermittent visual blurring in ipsilateral eye
- Ipsilateral hearing problems
- Chronic generalised and regional pain
- 'Midline splitting' of sensory loss
- Differences in sensation at sternum
124
Describe the main clinical feature of functional visual loss
'Tube' field loss
125
Describe the clinical features of physiologically-induced bodily sensations or the physical symptoms of anxiety
- Dizziness/light-headedness
- Tingling in hands, feet and around mouth
- Some visual disturbances
- Loss of consciousness
- Reduced breath holding time
- Symptoms reproduced on forced hyperventilation
- Tremor
126
Describe the clinical features of chronic fatigue syndrome
- Female
- Sore throat
- Low-grade fever
- Joint and muscle pain
- Headache
- Poor concentration
- Sleep disturbances
- Features of depression
127
Define chronic fatigue syndrome
Disabling fatigue lasting >6 months where no other medical cause has been found
128
How would you treat chronic fatigue syndrome?
- Tricyclic antidepressants
- Graded exercise
- CBT
129
Describe the clinical features of fibromyalgia
- Prominent muscle aching
- Fatigue
- Arthralgia
- Malaise
- Sleep disturbances
- Headache
- No abnormality on examination except some muscle tenderness
130
How would you treat fibromyalgia?
- Tricyclic antidepressants
- Graded exercise
- CBT
131
Define a seizure
Paroxysmal neurological event caused by abnormal discharge of neurons
132
Define epilepsy
Tendency to recurrent seizures
133
List the 4 types of focal seizures
1) Simple partial
2) Complex partial (with loss of consciousness)
3) Secondary generalised
4) Focal status epilepticus
134
Describe the typical clinical features of a generalised seizure
- Usually starts in childhood/adolesence
Clusters of tonic-clonic, absence and myclonic jerks
- Generalised stiffness (tonic)
- Repeated generalised jerking (clonic)
- Intermittent symmetrical jerks (myoclonic)
- Absence with no focal symptoms
- Atonic drop attacks
Characteristic EEG signature
135
Describe the typical clinical features of a focal seizure
- Occurs at any age
- May be associated with structural brain disease
- May be post-ictal confusion and automatisms
- Inter-ictal EEG may show localised spikes or sharp waves
- Focal limb jerking (motor cortex)
- Focal tingling (somatosensory cortex)
- Olfactory or gustatory hallucination (temporal lobe)
- Visual hallucination (occipital lobe)
- Limb posturing (supplementary motor area)
- Swallowing/chewing movements (temporal lobe/insula)
136
What can provoke a seizure?
- Metabolic disturbance e.g. renal/liver failure
- Drugs
- Alcohol
- Alcohol withdrawal
137
What differentials would you consider in a patient presenting with suspected epilepsy?
- Vasovagal syncope
- Dissociative attacks
- Sleep disorders
138
Define status epilepticus
Seizures occurring for >30 minutes without recovery (either continuously or intermittently)
(>5 minutes of continuous seizure or >2 seizures without complete resolution in between)
139
What are the potential secondary manifestations of status epilepticus?
- Hypoxia
- Acidosis
- Myoglobulinuria
- Renal failure
- Disseminated intravascular coagulation
- Hyperthermia
140
What are the indications for further investigation in a patient who has had a single seizure?
>20 years old
Younger patients with clinical or EEG evidence of focal seizures
Seizures which are difficult to control
141
What investigation would you perform (if indicated) in a patient with a single seizure?
CT/MRI head
142
How would you diagnose epilepsy?
- Clinical diagnosis - 2 or more spontaneous seizures
| - May be supported with EEG (but mainly for classification)
143
When can a patient with epilepsy safely drive again?
After they have been seizure free for 2 years
144
When would you commence patients on anti-epileptic therapy?
When a person has suffered two or more seizures within 2 years
145
What treatment would you recommend for focal epilepsy?
1) Lamotrigine
| 2) Carbamazepine or Levetiracetam
146
Side effects of lamotrigine
- Sedation
- Diplopia
- Ataxia
- Rash
147
Benefit of lamotrigine
Lower risk of teratogenicity
148
Side effects of Carbamazepine
- Sedation
- Diplopia
- Ataxia
- Rash
- GI upset
- Weight gain
- Teratogenicity
149
When is Carbamazepine contra-indicated?
- Women of child-bearing age
- Not well tolerated in the elderly
- Hepatic enzyme inducer (increases COCP elimination)
150
Side effects of Levetiracetam
- Sedation
| - Mood disturbance
151
What treatment would you recommend for a patient with general epilepsy?
1) Sodium valproate OR lamotrigine
| 2) Topiramate OR lecetiracetam
152
Side effects of sodium valproate
- Sedation
- GI upset
- Weight gain
- Reversible hair loss
153
Side effects of lamotrigine
- Sedation
- Diplopia
- Ataxia
- Rash
154
When would sodium valproate be contra-indicated?
Women of child-bearing age
155
Benefits of sodium valproate
Favoured in the elderly as lower risk of ataxia and falls (than Carbamazepine)
156
Side effects of Topiramate
- Sedation
| - Weight loss
157
When would topiramate be conta-indicated
Women of child-bearing age
| also a hepatic enzyme inducer
158
Which epilepsy patients would you consider neurosurgery in?
Patients with:
- Focal onset seizures
- Not controlled by medication
- Epilepsy demonstrated to originate in single part of brain which could be removed without any major neurological deficit
159
How would you treat status epilepticus?
1) Benzodiazepam e.g. Lorazepam/Diazepam IV OR diazepam PR or midazolam buccal
2) Repeat benzodiazepine
3) Loading dose of Phenytoin IV OR sodium valproate IV
4) Admit to ITU
160
What features in a history would make you suspect a diagnosis of tension headache?
- Continuous or episodic pain
- Tight, dull band pain/pressure around the head
- Pain lasts 30 mins-7 days
- Triggers: stress, fatigue
161
What features in a history would make you suspect a diagnosis of medication overuse headache?
- Chronic headache >15 days per month
| - Regular overuse >3 months of codeine, triptans, caffeine, paracetamol
162
What features in a history would make you suspect a diagnosis of migraine?
- Episodic unilateral throbbing headache over the temples
- Lasts 4-72 hours
- Relieved by sleep
- Triggers: cheese, chocolate, coffee, red wine, lying in, relaxation/stress relief, hormonal changes, COCP, menstruation
- Worsened by activity
- Associated symptoms: nasa, photophobia, phonophobia, aura
- Premonitory symptoms 24 hours before headache - mood swings, hunger, drowsiness
163
What features in a history would make you suspect a diagnosis of cluster headache?
- Severe unilateral retro-orbital pain
- Lasts 15 mins-3 hours
- Occurs once or more per day for several weeks before subsiding
- Associated symptoms: red eye, lacrimation, nasal stuffiness, ptosis, Horner's syndrome, restlessness, forehead/facial sweating
- Triggered by alcohol
164
What features in a history would make you suspect a diagnosis of sinusitis?
- Tender frontal and maxillary sinuses
| - Associated symptoms: fever, nasal discharge
165
What features in a history would make you suspect a diagnosis of trigeminal neuralgia?
- Sudden severe pain lasting seconds-minutes followed by a dull ache
- Occurs in bouts many times each day
- Triggers: touch, movement (brushing teeth, eating, speaking), cold
166
How would you treat a tension headache?
- Relaxation exercises
| - Amitryptilline
167
How would you treat medication overuse headache?
- Withdraw analgesics
168
How would you treat cluster headaches?
1) Oxygen
2) Sumatriptan injections
3) Steroids
4) Verapamil
169
How would you treat trigeminal neuralgia?
1) Carbamazepine
2) Glycerol injections, electrical lesion
3) Surgical decompression
170
What features in a history would make you suspect a diagnosis of subarachnoid haemorrhage?
- Sudden severe headache at back of head, lasting >1 hour
| - Associated symptoms: loss of consciousness, seizures, focal neurological signs, neck stiffness, vomiting
171
What features in a history would make you suspect a diagnosis of temporal arteritis?
- Typical patient >50 years old, female, associated with PMR
- Insidious onset
- Bitemporal pain
- Associated symptoms: jaw claudication, scalp tenderness
172
What features in a history would make you suspect a diagnosis of meningitis?
- Progressive headache developing over hours/days
| - Associated symptoms: fever, neck stiffness, rash, impaired consciousness
173
What features in a history would make you suspect a diagnosis of raised intra-cranial pressure?
- Generalised headache triggered/worsened by coughing, bending over or lying down
- Headache worse in morning
- Associated symptoms: vomiting, false localising signs (nerve palsies), papilloedema, altered consciousness
- Varying time course
174
What features in a history would make you suspect a diagnosis of idiopathic intracranial hypertension?
- Young, obese women
- Caused by tetracycline
- Generalised headache triggered/worsened by coughing, bending over or lying down
- Associated symptoms: machinery noise in the ears
175
What features in a history would make you suspect a diagnosis of cranial venous sinus thrombosis?
- Causes: COCP, dehydration, clotting abnormalities, ear infections
- Generalised headache worsened/triggered by coughing, bending over, lying down
176
What features in a history would make you suspect a diagnosis of carotid arterial dissection?
- Sudden onset head or neck pain
- Associated Horner's syndrome
- Associated TIA/stroke
177
What investigations would you do in a case of suspected subarachnoid haemorrhage, and what would they show?
- Fundoscopy (papilloedema)
- CT
- CT angiography
- Lumbar puncture - elevated RBC, WCC and bilirubin (after 12 hours)
178
What investigations would you do in a case of suspected temporal arteritis, and what would they show?
- Inflammatory markers - elevated CSR
| - Temporal artery biopsy
179
What investigations would you do in a case of suspected idiopathic intracranial hypertension, and what would they show?
- MRI
- MR venography
- Lumbar puncture - increased ICP with normal CSF constituents
180
What investigations would you do in a case of suspected cranial venous sinus thrombosis, and what would they show?
- MR venography
181
How would you treat a patient with suspected idiopathic intracranial hypertension
- Lumbar puncture
- Acetazolamide
- Weight loss
- Surgical drainage through lumboperitoneal drainage
182
How would you treat a patient with suspected cranial venous sinus thrombosis?
- Anticoagulants
183
What features in a history would make you suspect a diagnosis of subarachnoid haemorrhage?
- 40-60 years old
- Sudden severe generalised headache - 'hit by bat on back of head'
- Transient loss of consciousness, drowsiness or coma
- Vomiting
- Neck stiffness
- Focal neurological signs
- Hypertension
184
Describe the pathophysiology os subarachnoid haemorrhage
- Most result from ruptured saccular intracranial haemorrhage in the circle of Willis
- 5% caused by arteriovenous malformation
185
What differentials would you consider in a patient with suspected subarachnoid haemorrhage?
- Thunderclap headache
| - Meningitis
186
What investigations would you request in a patient with suspected subarachnoid haemorrhage?
1) CT head
1a) Lumbar puncture at least 6 hours after onset (if CT negative) - would be blood stained with xanthochromic supernatant
2) 4-vessel cerebral angiogram OR MRI/CT angiography
187
How would you manage a patient with subarachnoid haemorrhage?
1) Transfer to neurosurgical centre
2) Frequent neurological observations
3) Endovascular occlusion of the aneurysm OR surgical clipping
4) Sustained hypervolaemia (3l of normal saline per day) + nimodipine (to prevent ischaemic complications caused by vasospasm)
Fludrocortisone OR hypertonic saline to treat hyponatremia
Drainage used to treat hydrocephalus
188
Which group of drugs are contra-indicated in patients with a subarachnoid haemorrhage
Anti-hypertensives
189
Complications of subarachnoid haemorrhage
- Re-bleeding from a ruptured aneurysm
- Cerebral ischaemia
- Hydrocephalus
- Hyponatremia
- Neurogenic pulmonary oedema
- Cardiac arrhythmias
- Bedrest complications: DVT, aspiration/basal pneumonia
190
How would complications of a subarachnoid haemorrhage present?
- Deterioration in consciousness and focal neurological signs
191
How would you investigate a patient with suspected subarachnoid haemorrhage complications
Repeat CT head
192
Define vertigo
One, or more, of the following:
1) A distortion of static gravitational orientation
2) An erroneous perception of movement of the sufferer
3) An erroneous perception of movement of the environment
193
What tests can be used to distinguish between peripheral and central vertigo?
1) Unidirectional nystagmus
- Positive in peripheral lesion
- Negative in central lesion
2) Vor/head impulse test
- Impaired in peripheral lesion
- Normal in central lesion
3) Suppression of nystagmus
- Positive in peripheral lesion
- Negative in central lesion
194
Describe the clinical features of acute idiopathic unilateral peripheral vestibulopathy
- 30-60 year olds
- May precede URTI
- Severe rotary vertigo
- Accompanying vertigo disturbances e.g. imbalance, vomiting
- Symptoms exacerbated by head movement
- Symptoms last around a week
195
What would you expect to find on examination of a patient with acute idiopathic unilateral peripheral vestibulopathy?
- Unidirectional nystagmus
- Fast phase to unaffected ear
- No deafness or other features
196
How would you treat a patient with acute idiopathic unilateral peripheral vestibulopathy?
1) Reassurance and explanation
- Gradual full recovery or compensation
- Good prognosis - vertigo on sudden head movements may persist
2) Symptomatic drug treatment
- Nausea - anti-emetics
- Vertigo - vestibular suppressants (cinnarizine, cyclizine)
3) ?corticosteroids
197
What red flag symptom would make you suspect a central lesion in a patient presenting with vertigo?
- Deafness or other neurological features
198
Describe the clinical features of benign paroxysmal positional vertigo
- Mainly >40s
- Female
- Attacks of rotational vertigo lasting 10-20 seconds
- Symptoms provoked by positional changes
- Brief nausea
- Other symptoms rare
199
What features would you see on examination of a patient with BPPV?
- Delayed torsional nystagmus on positional testing, which spontaneously resolves and is fatiguable
200
How would you manage a patient with BPPV?
1) Reassurance and explanation
- May resolve spontaneously but may recur
2) Particle repositioning manoeuvres
Medication is ineffective
201
Describe the triad of Meniere's disease
1) Vertigo
2) Hearing Loss
3) Tinnitus
202
Describe the typical clinical features of Meniere's disease
- 30-50 year olds
Typical attack lasts a few hours with classic evolution:
1. Sensation of fullness in ear
2. Reduced hearing
3. Occurrence/increases tinnitus
4. Rotational vertigo
5. Postural imbalance
6. Nystagmus
7. Nausea
203
Risk factors for MND
- Age >50
| - Family history - 5% of cases are familial
204
3 main patterns of MND
1) Amyotrophic lateral sclerosis
2) Progressive bulbar palsy
3) Progressive muscular atrophy
205
What must be normal to make a diagnosis of MND?
Sensation
206
What investigations could you consider in a patient with suspected MND?
- Nerve conduction studies - exclude neuropathy
- Electromyography - confirm denervation and show fasciculations
- MRI brain/neck - exclude other causes, may show corticospinal degenertion
207
Clinical features of ALS
- Stiff and weak hands
- Muscle cramps and discomfort
- Onset may be more proximal and resemble plexopathy or foot drop
Progresses to:
- Widespread patch weakness affecting many muscles (extra-ocular muscles spared)
Progresses to:
- Weakness spreads to truncal and bulbar muscles
208
Which muscles are spared in ALS?
Extra-ocular muscles
209
What would you expect to see on examination of a patient with ALS?
- Hands severely wasted
- Interosseous fasciculations
- Normal muscle tone
- Patchy weakness
- Brisk reflexes
- Extensor plantars
210
What differentials would you consider in a patient with suspected ALS?
- Cervical myeloradiculopathy
211
Clinical features of progressive bulbar palsy
- Progressive dysarthria
- Dysphagia
- Rapid weight loss
- Repeated aspiration pneumonias
- Emotional lability
212
What features would you see on examination of a patient with progressive bulbar palsy?
- Tongue wasting and fasciculations
- Tongue spasticity
- Sagging facial muscles
- Brisk facial and jaw reflexes
213
What differentials would you consider in a patient with suspected progressive bulbar palsy?
- Myasthenia Gravis
- Brain stem lesions
- Cerebrovascular disease
- MS
- X-linked bulbospinal neuronopathy
214
Clinical features of progressive muscular atrophy
- Slow progressive muscle wasting, usually symmetrical in both hands
- Progresses to proximal muscles and legs
215
What would you see on examination of a patient with progressive muscular atrophy?
- Fasciculations
216
What differentials would you consider in a patient with suspected progressive muscular atrophy?
- Multifocal motor neuropathy with conduction block
- Inherited spinal muscular atrophy
- Post-polio syndrome
217
Describe the potential treatment options for patients with MND
- Spasticity - baclofen
- Bulbar involvement (maintaining nutrition and preventing aspiration pneumonia) - percutaneous endoscopic gastrostomy tube (PEG)
- Respiratory weakness, fatigue or headache - nocturnal or diurnal respiratory support
- Riluzole (glutamate antagonist) - shown to improve 18 month survival by 7%
218
Describe the clinical features of polio/post-polio syndrome
- Foreign travel
- Expousre to nappies of babies who have recently received oral, live attenuated polio vaccine
- Mild upper respiratory tract infection symptoms
- Meningitis or encephalitis symptoms
- Severe muscle weakness developing over 24 hours affecting any pattern of muscles
- Atrophic muscles which do not recover - subsequent skeletal development impaired and muscles flaccid and small
219
Describe the features of inherited spinal muscular atrophies
- LMN symptoms
- Variable inheritance, all forms are allelic variations of the same gene
- 3 forms:
1) rapidly fatal infantile/Werdnig-Hoffman syndrome
2) slowly progressive juvenile/Kugelberg-Welander syndrome
3) adult onset
220
Describe the features of hereditary spastic paraparesis
- Autosomal dominant
- Leg spasticity
- Compensatory hypertrophy in arms
221
Describe the features of Adrenoleucodystrophy
- X-linked recessive
- Mixed UMN and LMN signs
- Mild adrenal insufficiency
222
Describe the features of X-linked bulbospinal neuronopathy
- X-linked
- Defect in androgen receptor
- Much better prognosis than MND
- Bulbar palsy
- Pronounced action-induced fasciculations
- Diabetes
- Gynaecomastia
- Infertility
- Mild neuropathy
223
A monocular blindness would suggest a lesion in the...
Optic nerve
224
A bitemporal hemianopia would suggest a lesion in the...
Optic chiasm
225
A left nasal hemianopia would suggest a lesion in the...
Left lateral optic chiasm
226
A right homonymous hemianopia would suggest a lesion in the...
Left optic tract
227
A homonymous superior quadrantanopia would suggest a lesion in the...
Contralateral temporal lobe (inferior optic radiation
228
An inferior homonymous quadrantanopia would suggest a lesion in the...
Contralateral parietal lobe (superior optic radiation)
229
Differentials for sudden-onset monocular visual loss
- Retinal detachment
- Virtuous haemorrhage
- Retinal vein thrombosis
230
The pupil response will be normal in disease of the ... but impaired in disease of the ...
Eye
| Optic nerve
231
Differentials for progressive monocular visual loss
- Senile macular degeneration
- Diabetic retinopathy
- Chronic (open angle) glaucoma
- Compression of optic nerve by meningioma or glioma
232
Clinical features of amaurosis fugax
- Caused by embolism from the ipsilateral carotid artery to the retinal artery
- Middle-aged to elderly
- Sudden reversible vision loss lasting up to 30 mins with complete and rapid recovery ('curtain coming down over one eye')
- May have associated TIA symptoms
- Examination: no ocular signs/no abnormalities between episodes, cholesterol plaques, carotid bruit
233
Clinical features of retinal migraine
- Occasionally occurs in younger patients
- Gradual vision loss and slower recovery
- Typical migraine headache
234
Clinical features of acute glaucoma
- Young to middle aged
- Transient painful visual loss associated with coloured halos in vision preceding loss
- Examination: no abnormalities between episodes, normal visual acuity, dilated pupils during episodes, long-sightedness, raised intra-ocular pressure
- Diagnosis requires ophthalmological assessment
235
Clinical features of optic neuritis
- Usually occurs in young adults
- Visual loss that evolves over 3-10 days then gradually improves over days-weeks
- Associated with: retro-orbital pain, flashing lights on eye movement
- Other MS symptoms
- Examination: pink or pale atrophic optic disc, reduced colour vision, reduced visual acuity, afferent pupillary defect
236
Clinical features of acute anterior ischaemic optic neuropathy
- Caused by atheromatous disease in <55s
- Caused by giant cell arteritis in >55s
- Middle-aged to elderly
- Altitudinal field defect evolving over minutes-days
- Associated with: headache, weight loss, malaise
- Examination: reduced visual acuity, relative afferent pupil defect, optic nerve head swelling, fundal haemorrhages, elevated ESR
- Treat with 60-80mg prednisolone
237
Clinical features of a bilateral optic nerve lesion
- Causes: optic neuritis, idiopathic intracarnial hypertension
- Examination: reduced visual acuity, optic atrophy, incongrous field defects with central scotomas which cross the midline, colour desaturation and abnormal pupil response
238
Differentials for acute bilateral vision loss
- Pituitary lesions
- Temporal arteritis
- Acute demyelinating diseases
- Leber's optic atrophy
- Bilateral intra-ocular disease (anterior uveitis)
- Bilateral occipital infarction (cortical blindness)
239
Differential for bitemporal hemianopia
- Chiasmal lesions e.g. pituitary tumour
| - Associated with: reduced visual acuity, optic atrophy
240
Differentials for symmetrical anterior visual pathway disturbance
- Vitamin B12 deficiency
- Folate deficiency
- Tobacco-alcohol amblyopia
- Retinitis pigmentosa
241
Clinical features of an optic nerve tumour
- Examination: reduced visual acuity, relative afferent pupillary defect, optic atrophy or papilloedema
242
Clinical features of open angle glaucoma
- Middle-aged to elderly
| - Examination: normal visual acuity, normal pupil response, cupped optic disc
243
Clinical features of a pituitary tumour
- Middle-aged to elderly
- Bitemporal hemianopia
- Associated endocrine abnormalities or extra-ocular nerve palsies
- Examination: initially normal visual acuity, optic atrophy or papilloedema
244
Clinical features of a optic radiation infarct
- Middle-aged to elderly
- Hemiparesis, hemi-sensory loss, dysphasia
- Examination: hemianopia or quadrantanopia
245
Clinical features of a bilateral occipital infarct
- Middle-aged to elderly
- Brain stem symptoms
- Vision loss
- Examination: blind, normal pupil response, possible brain stem signs
246
How would you investigate a patient with suspected amaurosis fugax?
- Bloods: FBC, glucose, lipids, ESR
| - Carotid Doppler
247
How would you investigate a patient with suspected glaucoma?
- Ocualr pressure
248
How would you investigate a patient with suspected optic neuritis?
- Visual evoked potentials
- CSF
- MRI
249
Clinical features of an intra-cranial tumour
1) Progressive focal neurological deficit
2) Symptoms of raised intracranial pressure
- Headache which is worse on lying down, bending or straining (initially may be present each morning and clear after rising)
- Associated symptoms: nausea, vomiting, unsteadiness, fatigue, drowsiness
- On examination: gait ataxia, papilloedema, failure of up gaze and 3rd/6th cranial nerve palsies
(- May cause ventricular obstruction - sudden onset severe headache with collapse of loss of consciousness)
3) Focal seizures
4) Endocrine disturbances
250
Differentials for a patient with a suspected intracranial tumour
- Chronic subdural haematoma
- Intracranial abscess
- Giant aneurysm
- Obstructive hydrocephalus from non-malignant cause
251
How would you investigate a patient with a suspected intracranial tumour?
- MRI head
| - Intracranial biopsy
252
How would you manage the symptoms of an intracranial tumour
- Cerebral oedema - dexamethasone OR IV mannitol (for rapid but transient reduction in critically raised intracranial pressure)
- Maintain arterial blood pressure - IV fluids
- Cerebral hypoperfusion - artificial ventilation with hyperventilation
- Seizures - IV phenytoin
- Obstructive hydrocephalus - ventriculoperitoneal shunts or ventriculostomy
253
Clinical features of pituitary adenomas
- Endocrine malfunction
- Infertility
- Amenorrhoea
- Loss of libido
- Hypothyroidism
- Lactation
- Acromegaly
- Cushing's syndrome
- Diabetes insipidus
- Hypoadrenalism
- Bitemporal hemianopia
- Reduced acuity
- Papilloedema or optic atrophy
- Headache
254
Clinical features of pituitary apoplexy
- Acute visual disturbances
- Headache
- Malaise
- Systemic collapse
255
How would you investigate a patient with suspected pituitary adenoma?
- Serum prolactin
- MRI/CT head
- Endocrine assessment e.g. TFTs, LH, FSH
256
How would you manage a patient with a pituitary tumour
1) Dopamine agonists e.g. bromocriptine, carbergoline
2) Surgery
3) Radiotherapy
257
Clinical features of craniopharyngiomas
- Children
- Endocrine disturbance
- Headache
- Bitemporal hemianopia
- Mental dulling
- Diabetes insipidus
- Eating disorders
- Disturbances of thermoregulation
- Hydrocephalus
258
Clinical features of cerebellopontine angle tumours/acoustic nerve Schwannomas
- Progressive deafness in one ear
- Ipsilateral face weakness
- Facial sensory loss
- Ataxia
- Headache
- Neurological deficit in the limb may occur later with brain stem compression
259
What investigations would you carry out in a patient presenting with unilateral sensorineural hearing loss (and to rule out what diagnosis)?
- MRI head and audiometry
| - To rule out acoustic nerve Schwannoma
260
How would you treat an acoustic nerve Schwannoma?
- Surgical resection via suboccipital transmeatal micro-dissection
- Monitor function with facial nerve electromyography and auditory evoked potentials
261
Clinical features of pineal region tumours
- Visual disturbances
- Headache
- Parinaud's syndrome
- Hydrocephalus
- Papilloedema
262
Features of Parinaud's syndrome
- Failure of up gaze
- Eyelid retraction
- Dilated unreactive pupils
- Conervergence-retraction nystagmus
263
How would you investigate a patient with suspected pineal tumour?
- CT/MRI head
| - A-fetoprotein or beta-human chorionic gonadotrophin
264
Clinical features of malignant meningitis
- History of: adenocarcinoma, lymphoma, leukaemia and melanoma
- Symptoms evolving over a few weeks
- Headache
- Radicular pain
- Multiple cranial nerve palsies
- Polyradiculopathy in the limbs
265
How would you investigate a patient with suspected malignant meningitis?
- Clinical diagnosis
- CSF examination for malignant cells
- MRI head (if 2 negative LPs)
266
Clinical features of neurofibromatosis I
- Optic nerve glioma (children)
- Cortical dysgenesis
- Mental retardation
- Seizures
- Syringomyelia
- Hydrocephalus
- Peripheral nerve neurofibromas
- Epidermal molluscum fibrosum
- Cafe au Lait patches
- Massive plexiform neurofibromas
- Axillary freckles
- Pigmented Lisch nodules in iris by age 5
- Bone cysts
- Precocious puberty
- Phaeochromocytoma
267
Clinical features of neurofibromatosis II
- Bilateral acoustic Schwannoma
- Multiple spinal neurofibroma
- Multiple menigioma, glioma
- Few cutaneous lesions
268
Clinical features of Tuberous Sclerosis
- Seizures
- Mental retardation
- Cortical dysplasia and tubers
- Hypomelanotic regions
- Adenoma sebaceum
- Subungal fibromalas
- Shagreen patches
269
Clinical features of Von Hippel-Lindau
- CNS haemangioblastomas, especially cerebellum, spinal cord and retina
270
Clinical features of cerebellar degeneration (paraneoplastic syndrome)
- History of bronchus/breast/female genital tract cancer/lymphoma
- Progressive cerebellar syndrome over weeks/months
- Sometimes myoclonus, diplopia, hearing loss
- Anti-Purkinje cell antibodies
271
Clinical features of Limbic Encephalitis
- History of malignancy
- Personality change
- Subacute confusion
- Seizures
- Movement disorders
- K+ channel antibodies
272
Clinical features of myasthenia gravis
- Females aged 15-30 OR males aged 50-70
- Variable muscle weakness with fatiguability
- Weakness of eye movements, eyelid, facial, neck and bulbar muscles, proximal arm muscles, respiratory muscles
- Ptosis
- Diplopia
- Normal muscle appearance, tone, reflexes and sensation
- Reduced muscle power on examination
273
Describe a myasthenia crisis
Bulbar or respiratory failure, precipitated by intercurrent illness or drugs
274
How would you manage a patient at risk of myasthenia crisis
- Measure FVC every hour - an FVC <2 is borderline and an FVC <1.5 may require ventilation.
- Consider NG feeding - bulbar failure may cause aspiration pneumonia
275
Differentials for myasthenia gravis
- Chronic progressive external ophthalmoplegia
- Midbrain lesions
- Guillain-Barre syndrome
- MND
276
How would you investigate a patient with suspected myasthenia gravis?
- ACh receptor antibody detection
- Electromyography - may show typical decrement on repetitive stimulation
- Single-fibre EMG - may show increased jitter
- Edrophonium test - give a short-acting cholinesterase inhibitor to overcome symptoms for 2-3 minutes
- CT/MRI head (identify associated thymus disease)
277
What are the risks of an edrophonium test, and how would you reduce these risks?
- Overactivity at cardiac muscarinic receptors --> bradycardia
- May tip over-treated patients into respiratory failure
- Risks reduced by pre-dosing with atropine
278
How would you manage a patient with myasthenia gravis?
1) Pyridostigmine or neostigmine (cholinesterase inhibitors) for symptomatic relief
2) Corticosteroids/azathiorprine/methotrexate to reduce production of abnormal antibodies
279
Side effects of pyridostigmine/neostigmine
- Diarrhoea (treated with anti-muscarinics e.g. atropine, propantheline)
- Cholinergic crisis
280
Side effects of corticosteroids in myasthenia gravis treatment
- Paradoxical deterioration in condition in first 7-10 days of treatment - need to monitor patients closely
281
How would you treat a patient with myasthenia gravis who was acutely unwell?
- IV immunoglobulins or plasmapheresis
282
When would you consider thymectomy in a patient with myasthenia gravis?
- Patient <40
| - Older patient with thyme enlargement
283
What drugs are contra-indicated in myasthenia gravis?
- Sedatives
- Calcium channel blockers
- Beta-blockers
- Aminoglycoside antibiotics
- Erythromycin
- Benzodiazepines
- Curare-type muscle relaxants
284
Clinical features of Lambert-Eaton Myasthenia Syndrome
- Usually male
- History of lung/breast/prostate/stomach cancer/lymphoma
- Subacute proximal weakness
- Sensory disturbances
- Autonomic disturbances e.g. dry mouth
(Bulbar and ocular muscles are rarely affected)
285
Describe the investigations and findings you would see in a patient with Lambert-Eaton Myasthenia syndrome
- EMG - low amplitude muscle action potentials, increment at high stimulation frequencies
- Single fibre EMG - increases jitters
- Presence of anti-voltage-gated calcium channel antibodies
286
How would you manage a patient with Lambert-Earton Myasthenic syndrome
- 3,4-diaminopyridine (symptomatic relief)
| - Immunosuppression and plasmapheresis
287
List the 4 normal phenomena which occur in sleep
1) Dreams/nightmare
2) Sleep talking
3) Hypnic jerks
4) Sleep paralysis
288
What features would you want to include in a sleep history?
- What time they go to bed
- How long it takes them to fall asleep
- When they wake up
- How often they wake through the night
- What they do when they wake
- How they feel on waking in the morning
- Whether they sleep again through the day (when, where and how long)
- If and when they fall asleep
- Parallel history from a sleep partner (movement and snoring)
289
Which factors can interfere with sleep?
- Previous sleep history
- Work pattern/domestic circumstances
- Exercise
- Psychiatric history
- Alcohol
- Caffeine
- Medication
- Pain/medical conditions
- Nocturia
- Daytime naps
290
General clinical features of a sleep disorder
- Lethargy
- Poor concentration
- Memory loss
- Depression
- Personality changes - irritability
291
Features of disrupted sleep
- Caused by non-neurological problems
- Difficulty sleeping
- Excess daytime sleepiness
- Repeated awakening
- Periodic limb movement during sleep
- Sleep apnoea
- Treated by treating the underlying disorder/factors
292
Describe the pathophysiology and treatment of insomnia
- Disrupted sleep, poor sleep hygiene and idiopathic insomnia
- Treated with eliminate sleep disruption and improve sleep hygiene (avoiding naps, optimising time of sleep and sleeping environment).
- Nocturnal sedatives can be used if there is a short-term reversible cause for poor sleep but should be avoided for long-term use
293
3 causes of excessive sleepiness
1) Obstructive sleep apnoea
2) Disrupted sleep
3) Narcolepsy
294
Most common cause of excessive sleepiness
- Obstructive sleep apnoea
295
Risk factors for obstructive sleep apnoea
- Obesity
- Upper airways disorder
- Neuromuscular disorders
- Neurodegenerative disease
- Sedative medication
296
Clinical features of obstructive sleep apnoea
- Patients report they sleep well but wake feeling drowsy
- Headache on waking
- Fall asleep easily throughout the day
- Snoring and other apnoea spells
297
How would you diagnose a patient with obstructive sleep apnoea?
- Overnight oximetry - frequent dips corresponding to apnoeic spells are recorded.
- If this is not diagnostic, more formal sleep studies (EEG monitoring, recording of respiratory function)
298
Treatment of obstructive sleep apnoea
- Weight loss
- Continuous positive airway pressure therapy delivered via a face mask
- Modafinil
- Driving is restricted until symptoms are controlled
299
Clinical features of narcolepsy
- Excessive daytime sleepiness - bouts of uncontrollable sleepiness and refreshing naps of 20-60 mins
- Cataplexy
- Hypnagogic hallucinations
- Sleep paralysis
300
How would you diagnose a patient with narcolepsy?
- Multiple sleep latency test - mean time to fall asleep is measured on a series of occasions throughout the day
- Patients with narcolepsy fall asleep in <8 minutes and progress rapidly to REM sleep
- Hypocretin levels in CSF will be low (not a routine test)
301
Treatment of narcolepsy
- Modafenil
- Fluoxetine or clomipramine are useful to control cataplexy
- Dexamphetamine or sodium oxybate can be used if ineffective
302
Clinical features of sleep terrors
- Usually brief
- Patient typically sits up and screams/appears bewildered then goes back to sleep
- Occurs in first hour after going to sleep
- No recollection the next day
303
Clinical features of sleep walking
- Associated with more complex automatic behaviours (e.g. going to fridge and eating)
304
Treatment options for sleep walking
1) Avoid sleep deprivation
| 2) Benzodiazepines
305
Clinical features of hyping jerks
- Brief generalised jerks on falling asleep
306
Clinical features of 'exploding head syndrome'
- Sensation of painless explosion on falling asleep
307
Clinical features of periodic limb movements of sleep
- Brief repeated movements, predominantly of the lower limb
| - Often associated with restless leg syndrome and daytime sleepiness
308
Treatment for Periodic limb movements of sleep
1) Dopamine agonists e.g. Ropinirole or Pramipexole
309
Clinical features of REM sleep disorder
- Elderly patients
- Associated with altered development of Parkinson's or dementia
- Limited recall of a dream but complain of injuries sustained during the night
- Partner reports episodes vigorous/violent purposeful behaviour
310
Treatment of REM sleep disorder
1) Clonazepam
311
Clinical features of nocturnal seizures
- Tongue biting
- More sterotyped than parasomnias
- Generally brief (<2 mins)
- Occur at any stage of sleep
312
Causes of sleep occurring at the wrong time
- Jet lag
- Delayed-phase sleep disorder (run on 25 hour internal clock)
- Advanced-phase sleep disorder (23-hour internal clock)
313
Causes of bacterial meningitis
Neonates
- E.Coli
- Group B strep
Adults
- Haemophilus influenzae
- Neisseria meningitidis
- Strep. pneumoniae
Developing countries
- Tuberculous meningitis
314
Risk factors for meningitis
- Overcrowding
| - Poverty
315
Clinical features of bacterial meningitis
- Headache
- Fever
- Neck stiffness
- Photophobia
- Positive Kernig's sign
- Purpuric rash (meningococcal meningitis)
- Altered consciousness
- Seizures
- Focal signs
316
How would you investigate a patient with suspected bacterial meningitis
1) Blood cultures
2) CT/MRI head - rule out contra-indications for lumbar puncture
3) Lumbar puncture
317
When would a lumbar puncture be contra-indicated in a patient with bacterial meningitis?
- Severely ill children - may lead to deterioration
| - Evidence of raised intra-cranial pressure on CT head
318
Normal CSF constituents
- <5 cell count
- lymphocytes
- <0.45 protein
- Glucose >60% blood glucose
319
CSF appearance in bacterial meningitis
- Cell count >200
- Polymorphs
- Protein >1.5
- Glucose <40%
320
CSF appearance in viral meningitis
- Cell count 50-200
- Lymphocytes
- Protein <1
- Normal glucose
321
CSF appearance in tuberculous meningitis
- Cell count 50-500
- Lymphocytes
- Protein >1
- Glucose <40%
322
CSF appearance in partially treated bacterial meningitis
- Cell count 50-500
- Mainly lymphocytes
- Variable protein
- Normal or reduced glucose
323
Treatment of bacterial meningitis
IV ceftriaxone + IV dexamethasone + IV amoxicillin
324
Most common complications of bacterial meningitis
1) Hearing loss
2) Higher function deficits
3) Epilepsy
325
Most common cause of viral meningitis
Enterovirus
326
Clinical features of viral meningitis
- Less severe headache
- Fever
- Less severe neck stiffness
327
Define aseptic meningitis
- Patients with a clinical and CSF picture of meningitis but without bacterial culture from the CSF
328
Clinical features of tuberculous meningitis
- More insidious onset
- General malaise
- Progressive headache
- Multiple lower cranial nerve palsies
- Multiple radiculopathies
329
What investigations would you do in a patient with suspected tuberculous meningitis, and what would they show?
- Lumbar puncture - CSF will show lower levels of lymphocyte pleocytosis, raised protein and low glucose
- Culture - culture of Mycobacterium tuberculosis takes 6 weeks
- PCR
330
Treatment for tuberculous meningitis
- Rifampicin
- Isoniazid
- Pyrazinamide
- Ethambutol
- Treat once diagnosis is suspected
- Treat for 9 months
331
Causes of viral encephalitis
- HSV - typically affects the temporal lobes
- EBV
- Adenovirus
- Arbovirus
- Rabies
332
Clinical features of viral encephalitis
- History of prodromal infection
- Fever
- Altered behaviour
- Seizures
- Confusion
- Coma
- Temporal lobe symptoms - behavioural changes, speech disturbances, hemiplegia, seizures (HSV)
333
How would you investigate a patient with suspected viral encephalitis, and what would they show?
- Lumbar puncture - CSF will show slightly lymphocytic pleocytosis, elevated protein and normal glucose
- CT/MRI head - usually normal
- Serum PCR or virus culture
- EEG - slow and cannot distinguish between infective and other encephalopathies
334
How would you manage a patient with viral encephalitis?
- IV aciclovir (to cover possibility of HSV)
335
Most common and disabling sequelae of HSV viral encephalitis
- Short-term memory loss
336
Clinical features of cerebral abscess
- Progressive headache
- Focal neurological symptoms and signs
- Fever
- Seizures
- Features of primary infection or markers of systemic infection
337
Main differential for a cerebral abscess
Cerebral tumour
338
How would you investigate a patient with a suspected cerebral abscess, and what would it show?
- CT/MRI head - one or more ring enhancing lesions with associated oedema
339
Treatment for cerebral abscess
- IV ceftriaxone AND metronidazole PO AND surgical drainage
340
Name a common complication of cerebral abscess
- Epilepsy
341
Clinical features of subacute sclerosing panencphalitis
- Presents in late childhood/early adolescence
- Presents after measles
- Progressive intellectual deterioration
- Seizures
- Myoclonus
- Progressive tetra-paresis
342
Clinical features of Whipple's disease
- GI upset
- Supra-nuclear gaz disorder
- Bizarre rhythmical movements of the eyes and mouth
343
Clinical features of CJD
- 50-70 year olds
- Family history (5%)
- Dementia
- Myoclonus
- Typical EEG changes
- Median survival <1 year
344
Clinical features of new-variant CJD
- Young people
- Symptoms progress over months
- Psychiatric disturbances
- Dementia
- Ataxia
- Dystonia
- No myoclonus or typical EEG changes
345
Clinical features of HTLV-1
- Common in West Indies, Africa and South America, and immigrants from these areas
- Progressive weakness and stiffness in the legs
- Sensory symptoms
- Prominent bladder symptoms
- Arms early affected
346
How would you investigate a patient with suspected HTLV-1 infection, and what would it show?
- Lumbar puncture - oligoclonalbands
347
Clinical features of a pyogenic infection/epidural abscess
- Back pain
- Fever
- Progressive radicular pain
- Symptoms/signs of spinal cord/cauda equina involvement
348
How would you investigate a patient with suspected pyogenic infection/epidural abscess?
- Urgent spinal MRI
349
Clinical features of shingles
- Usually affecting thoracic spine
- Rash affecting one dermatome
- Neuralgic pain
- If it occurs in unusual or more than one dermatome, consider underlying causes of immunosuppression
350
How would you manage a patient with shingles?
- Shorten illness and reduce frequency of post-herpetic neuralgia --> oral aciclovir (acyclovir eye drops in ophthalmic zoster)
- Neuralgic pain--> carbamazepine and amitryptilline
351
When would you be concerned about a patient with shingles?
- When it occurs in the ophthalmic branch of the trigeminal nerve - this innervates the cornea, and corneal ulcers can occur
352
4 neurological syndromes of syphilis infection, and how they would present
1) Meningeal syphilis (6-12 months after infection)
- Chronic meningitis
- Multiple cranial nerve palsies
2) Meningovascular syphilis (5-10 years after infection)
- Young stroke
3) Tabes Dorsalis (15+ years after infection)
- Lightening pain in the legs
- Loss of sensation in the legs
- Charcot joints
- Wide-based, high stepping gait
4) Paretic syphilis (15+ years after infection)
- Progressive dementia
- Prominent delusions
- Personality changes
- Weakness
- Gait disturbance
353
How would you investigate a patient with suspected neurological syphilis?
1) Blood serology - TPHA, VDRL, FTA
| 2) Lumbar puncture - CSF examination of cell count, protein and VDRL titre
354
Treatment of syphilis
21 days of supervised IM penicillin
355
Potential neurological complications of HIV infection
- Chronic demyelinating neuropathies
- Gillain-Barre syndrome
- Facial weakness
- Opportunistic infection e.g. toxoplasmosis, CMV, PML, cryptococcus, HSV, HZ
- Tumours e.g. primary CNS lymphoma
356
Clinical features of toxoplasmosis
- Immunocompromised patients
- Symptoms develop over a few weeks
- Focal neurological deficit
- Headache
- Seizures
357
How would you investigate a patent with suspected toxoplasmosis, and what would it show?
- CT/MRI head- single or multiple ring-enhancing masses with oedema
358
How would PML appear on MRI head?
- Non-enhancing lesions without mass effect limited to the white matter
359
Which region does PML usually affect?
Occipitoparietal region
360
Clinical features of cryptococcal meningitis
- Immunocompromised patient
- Headache
- Malaise
- Confusion
- multiple cranial nerve palsies
361
How would you investigate a patient with suspected cryptococcal meningitis?
- Lumbar puncture with India ink staining and antigen detection
362
Treatment of cryptococcal meningitis
- Amphotericin B
- Flucytosine
- Fluconazole
363
Treatment for CMV
- Ganciclovir and foscarnet
364
Clinical features of HIV encephalopathy
- Immunocompromised patient
- Apathy
- Behavioural abnormalities
- Marked memory problems
- Brisk reflexes
- Prominent frontal withdrawal signs
365
What would you see on investigation of a patient with HIV encephalopathy?
- Cerebral atrophy
| - Multinucleate giant cells with myelin pallor and gliosis
366
Most common cause of head injury in <15s and >65s
Falls
367
Most common cause of head injury in 15-65s
Assault
368
Clinical features of uncal herniation
- Typically occurs after a squamous temporal bone injury which tears the middle meningeal artery
- Headache
- No loss of consciousness
- Contralateral limb weakness
- Dilated pupils on ipsilateral side
- Reduced consciousness
- Cushing's response (increasing BP with decreasing HR)
- May progress to bilateral pupillary dilation
369
Treatment for uncal herniation
- Immediate surgical evacuation (before development of bilateral fixed pupils)
370
Clinical features of skull fractures
- Raccoon eyes/periorbital bruising
- Battle's sign
- Auditory and facial nerve damage
- Blood behind ear drum or in external ear
- CSF rhinorrhoea
371
If a patient had clear fluid leaking from their nose, how would you test if it was mucus or CSF?
- Glucose (in CSF, not mucus)
| - Isotransferrin - more specific but takes longer
372
Indicators of severity of a head injury
- GCS
- Signs indicative of a basal skull fracture
- Pupil reactions
- Focal neurological signs
- Duration of post-traumatic amnesia
- Imaging - fractures, penetrating injuries, haemorrhage, oedema, brain stem or deep brain involvement
373
Define a mild head injury
- GCS >12
- Post-trauamtic amnesia <24 hours
- Loss of consciousness <30 min
374
Define a moderately severe head injury
- GCS 9-12
- Post-trauamtic amnesia 1-7 days
- Loss of consciousness <24 hours
375
Define a severe head injury
GCS <9
- Post-trauamtic amnesia >7 days
- Loss of consciousness >24 hours
376
What patients with a head injury can you send home with a responsible adult?
- Mild head injury
- No loss of consciousness or loss of consciousness <5 mins
- Normal neurological examination
- No skull fracture
377
Indications for admitting a patient with a head injury to hospital
- Amnesia
- Brief or impact seizure
- Coagulation or bleeding diathesis
- Drowsiness, headache, irritability, lethargy
- Intoxication
- Loss of consciousness >5-10 mins
- No responsible carer at home
- Persistant episodes of vomiting
- Suspicion of base of skull fracture
- Unreasonable time/distance from home
378
Indications for an urget CT/MRI head in a patient with a head injury
- GCS <14
- GCS <15 2 hours after injury
- Focal neurological signs or symptoms
- Focal or sustained seizure
- Suspicion of compound/depressed skull fracture
- Penetrating skull injury
379
Indications for a skull x-ray in a patient with a head injury
- Age 1 unless trivial mechanism of injury
- Suspicion of non-accidental injury
- Full thickness laceration
- Large boggy swelling
380
Aims of treatment for a patient with a head injury
Avoid hypotension
Maintain oxygenation
Avoid raised intracranial pressure
- Head elevation
- Reduce temperature
- Surgical procedures o evacuate intracranial haematoma
- Surgical shunt for hydrocephalus
- Medical interventions with mannitol, mechanical ventilation and forced hyperventilation
- Monitor with intracranial pressure monitors
Rehabilitation
381
Complications of a head injury
Post-traumatic syndromes
- Anxiety, difficulty sleeping, poor concentration
- Recollections of the accident
- Behavioural changes e.g. avoiding driving
- Migraine-like headaches (usually spontaneously improve over 2 years)
Permanent anosmia
Post-traumatic vertigo
Post-traumatic epilepsy
382
Clinical features of a lesion in the corticospinal tract
- Weakness and unsteadiness in walking
- Stiffness on walking
- Spontaneous leg spasms
- Increased tone and reflexes
- Extensor plantar responses
383
Clinical features of a lesion in the dorsal column
- Ataxia
- Clumsiness in the hands
- Loss of joint position and vibration senses
384
Clinical features of a lesion in the spinothalamic tract
- Loss or altered cutaneous sensation and pain sensation
| - Painless injuries/burns and secondary deformities (Charcot joints)
385
Clinical features of a lesion in the lateral columns
- Altered sphincter function
386
Clinical features of a total spinal transection
- Loss of all function below the level of the lesion
- Urinary retention
- Constipation
387
Clinical features of Brown-Sequard syndrome
- Ipsilateral spasticity
- Ipsilateral loss of vibration and proprioception
- Contralateral loss of fine touch and pain
388
Clinical features of a central cord lesion
- Constipation
- Urinary retention
- Bilateral loss of fine touch and pain sensation
- Muscle wasting
- Weakness
- Areflexia
- Spasticity
389
Clinical features of anterior cord syndrome
- Loss of all functions
EXCEPT
- Preservation of proprioception and vibration sense
390
What investigation would you use in a suspected myelopathy?
- MRI spine
391
How would you treat cervical spondylosis?
- Posterior laminectomy OR
- Excision of disc material OR
- Anterior cervical decompression and fusion with iliac crest grafts
392
How would you treat non-infective inflammatory causes of spinal cord compression
High dose corticosteroids
393
Common primary malignancies which metastasise to the vertebrae
- Bronchus
- Breast
- Myeloma
- Lymphoma
- Prostate
394
Causes of inflammatory spinal cord disease
- MS
- Sarcoidosis
- Sjogren's syndrome
- SLE
395
Infective causes of spinal cord disease
- Paraspinal/epidural abscesses
- Brucellosis
- Syphilis
- HIV
- HTLV-1
396
What is the most common presentation of vascular spinal cord syndrome
- Sudden onset of anterior spinal cord syndrome at 8-T11
| - Symptoms exacerbated by exercise
397
Most common cause of spinal cord compression
Degenerative cervical disc disease
398
Clinical features of vitamin B12/folate deficiency
- Sudden myelopathy affecting dorsal columns
- Megaloblastic anaemia
- Mental slowing
- Cerebellar ataxia
- Peripheral neuropathy
399
Clinical features of Freidreich's Ataxia
- Symptoms onset at 8-15 years
- Loss of reflexes (axonal neuropathy)
- Optic atrophy
- Cardiomyopathy
- Wheelchair bound by 40
400
Clinical features of familial spastic paraparesis
- Severe spasticity of the legs
| = Relative preservation of power and compensatory hypertrophy in the arms
401
Clinical features of syringomyelia
- History of congenital abnormality (Chiari malformation), tumour or trauma
- Constipation
- Urinary retention
- Bilateral loss of fine touch and pain
- Muscle wasting, weakness and areflexia
- Muscle spasticity
- May have lower cranial nerve signs (syringobulbia)
402
Most common causes of radiculopathy
- Displaced intervertebral discs (in young people with history of recent injury/straining)
- Degernative spine disease (spondylosis in older adults)
- Tumours (breast, bronchus, kidney, thyroid, lymphoma)
- Inflammation (e.g. shingles)
403
Clinical features of radiculopathy
- Pain radiating from spine in distribution of affected nerve root
- Constant pain
- Pain exacerbated by moving the limb
- Movements painful and limited
- Weakness in distribution of affected nerve root
- Muscle wasting or fasciculations
- Areflexia
- Sensory loss in a dermatomal distribution
- In disc prolapse: acute onset and related to physical exertion
- In mechanical causes: pain worsened by coughing, sneezing or straining
404
What investigations would you consider in a patient with a suspected radiuclopathy?
- MRI spine of relevant level
- EMG - may show ridiculer pattern of denervation
- Nerve conduction studies - exclude neuropathy
- Lumbar puncture (in multiple radiculopathies with evidence of systemic illness)
405
How would you manage a patient with lumbar disc disease?
1) Period of rest
2) Mobilisation and education about avoiding back strain/injury
3) Surgical management e.g. microdiscectomy, laminectomy (consider earlier if neurological deficit present)
406
How would you manage a patient with cervical disc protrusions
1) Physiotherapy, soft collar or traction
| 2) Surgery e.g. single level discectomy, anterior disc removal and bone grafts
407
Clinical features of lumbar central disc prolapse
MEDICAL EMERGENCY
- Severe back pain radiating into both legs (usually sciatic distribution)
- Bilateral foot drop
- Bilateral hip and knee flexor weakness
- Sphincter disturbance
- Loss of ankle jerk and sacral reflex
- Sensory loss in feet
- Saddle anaesthesia
408
Management of lumbar central disc prolapse
Urgent surgical decompression
409
Clinical features of lumbar canal stenosis
- Increasing leg weakness on walking (neurogenic claudication)
- Stooped posture
- Symptoms ease on walking uphill
- No neurological signs
410
Management of lumbar canal stenosis
Surgical decompression
411
Clinical features of normal pressure hydrocephalus
- Urinary incontinence
- Gait ataxia
- Dementia
412
Clinical features of Korsakoff's psychosis
- Retrograde amnesia
| - Confabulations
413
Clinical features of Wernicke's encephaloapthy
- Confusion
- Ataxia
- Nystagmus
- Opthamoplegia
- Peripheral neuropathy
414
Driving advice for a patient with a single unprovoked seizure or blackout with seizure markers
Licence revoked for 1 year (6 months if ECG and scan are normal)
415
Driving advice for a patient with a single provoked seizure
Inform DVLA and await guidance before driving again
416
Driving advice for a patient with recurrent seizures
License revoked until seizure free for 1 year
417
Driving advice for a patient with seizures in sleep only
May drive despite continuing seizures providing all seizures have been in sleep for at least 3 years
418
Driving advice for a patient with a single TIA
Can drive 1 month after episode
419
What investigations would you do in a patent with suspected syncope?
- Fasting glucose
- 24-hour ECG
- Echocardiogram
- Tilt table test
420
What investigations would you do in a patient with a suspected seizure?
- MRI/CT head
- EEG
- 24-hour EEG
- Serum calcium
421
Define coma
A state of unconsciousness in which a person
- Can not be awakened
- Fails to respond normally to painful stimuli, light or sound
- Lacks a normal sleep/wake pattern
- Does not initiate voluntary actions
422
Equal pinpoint pupils would make you think of a diagnosis of...
- Opiate overdose
| - Pontine lesion
423
Equal small/mid-sized, reactive pupils would make you think of a diagnosis of...
- Metabolic encephalopathy
424
Equal mid-sized, unreactive pupils would make you think of a diagnosis of...
- Midbrain lesion
425
Equal large pupils would make you think of a diagnosis of...
- Drug overdose - cocaine, ecstasy, anti-depressants, cholinesterase inhibitors
426
Unequal small, reactive pupils would make you think of a diagnosis of...
- Horner's syndrome with partial ptosis
427
Unequal large, unreactive pupils would make you think of a diagnosis of...
- 3rd nerve palsy (ptosis and dilated pupil) - can be caused by herniation
428
What would you look for on examination of a patient in a a coma
Vital signs
- BP
- Pulse
- Respiratory rate
External signs of trauma
- Battle's sign
- Evidence of injections
- Bitten tongue
- Neck stiffness
Neurological examination
- GCS
- Pupillary examination
- Doll's head eye movements
- Fundoscopy
- Facial/limb asymmetry
- Tone, posture and repsonse to pain
- Reflex/plantar response asymmetry
429
What investigations would you do to determine the cause of coma?
Bloods:
- FBC
- Glucose
- U&Es
- LFTs
- Toxicology screen
- Ammonia
- Red cell transketolase
ABG
CT/MRI head
Lumbar puncture
EEG
430
Differentials for a patient in a suspected coma
- Locked in syndrome (high midbrain lesion or severe generalised neuropathy) - normal consciousness, eyes open, normal sleep-wake pattern
- Catatonia - psychiatric state of unresponsiveness
- Vegetative state (diffuse bihemispheric disease but normal brain stem function) - open eyes and normal sleep-wake pattern
431
What supportive management would you provide a patient in a coma?
- Intubation and ventilation
- IV fluids and inotropes as required
- NG tube
- TED stockings
- Urinary catheter
432
How would you investigate/manage a patient with coma, neck stiffness and fever?
1) Broad-spectrum antibiotics to cover meningitic organisms
| 2) MRI/CT head and lumbar puncture
433
How would you investigate a patient with coma and neck stiffness?
1) CT brain
434
How would you investigate/manage a patient with coma and focal signs?
1) CT/MRI head
2) Mannitol infusion
3) Dexamethasone
4) Intubation and hyperventilation
435
How would you manage a patient with coma caused by hyponatremia?
Slow correction to avoid pontine myelinolysis
436
How would you manage a patient with coma caused by encephalitis
Antiviral therapy
437
How would you manage a patient with coma caused by hypoglycaemia?
25-50ml glucose OR 250-500ml 5% IV dextrose
438
How would you manage a patient with coma caused by opiate overdose?
Naloxone 800micrograms
439
How would you manage a patient with coma caused by benzodiazepine overdose?
Flumazenil 400mg
440
How would you manage a patient with coma caused by Wernicke's encephalopathy?
Pabrinex IV
441
How would you manage a patient with coma caused by Addisonian crisis?
Steroids
442
The 3 elements of brain stem death in the UK
1) Cause of failure known to be irreversible
2) Absence of cerebral function, patient is unresponsive and unreceptive
3) Absence of brain stem function
443
How would you test brainstem function to confirm brainstem death
- Correct metabolic abnormalities
- Allow time for sedative medication to clear
- Tests undertaken by two senior physicians on two occasions
No response must be found to any of the following:
- Pupillary response
- Doll's head eye movements
- Eye movements on caloric testing
- Corneal responses
- Gag responses
- Response to painful stimuli
- Respiratory response to hypercapnia
444
Risk factors for peripheral neuropathy
- Diabetes
- Malignancy
- Connective tissue disease
- Sarcoidosis
- Rheumatoid arthritis
- SLE
- AIDS
- Uraemia
- Hypothyroidism
- Critical illness
- Thiamine deficiency
- Niacin deficiency
- B6 deficiency
- B12 deficiency
- Folate deficiency
- Vitamin E deficiency
- Alcohol excess
- Industrial toxins
- Drugs - amiodarone, cisplatin, dapsone, gold, isoniazid, metronidazole, thalidomide, vincristine
445
What investigations could you consider to help you diagnose a peripheral neuropathy and its cause?
- Nerve conduction studies
- Lumbar puncture
- Molecular genetics
- Nerve biopsy
- Formal autonomic testing
446
Most common cause of peripheral neuropathy
Diabetes
447
Clinical features of diabetic neuropathy
- Poor glycaemic control
- Predominantly symmetrical sensory symptoms e.g. painful parasthesia or numbness
- Autonomic nervous system involvement e.g. postural hypotension, tachycardia, nausea/vomiting, intermittent diarrhoea/constipation, urinary retention/overflow, impotence, disordered sweating
- Can lead to foot ulcers and neuropathic arthropathies (Charcot joints)
- May present with asymmetrical painful proximal muscle weakness in the legs
448
Clinical features of nutritional deficiency neuropathies
- Painful, predominantly sensory distal symmetrical axonal neuropathy
- Vitamin B12 deficiency often has associated myelopathy - progressive prominent distal paraparesis, loss of proprioception and associated dementia
449
How would you diagnose a vitamin B12 deficiency neuropathy?
- Vitamin B12 measurement
- Demonstration of anti parietal cell antibodies
- Demonstration of malabsorption
450
Clinical features of toxic neuropathy
- Sensorimotor distal symmetrical peripheral neuropathy (except lead, predominantly motor)
451
Clinical features of Lyme disease neuropathy
- Facial nerve neuropathy
- Radiculopathies
- Lymphocytic meningitis
- Later presents with distal symmetrical neuropathy
452
Clinical features of Charcot-Marie-Tooth disease
- Insidious onset
- Minor symptoms with more marked signs
- Distal symmetrical neuropathy
- Preferential atrophy of distal leg muscles ('inverted champagne bottle legs')
453
Most common inherited neuropathy
Charcot-Marie-Tooth disease
454
Clinical features of Guillain-Barre syndrome
- Any age
- History of illness - typically Campylobacter jejune, CMV or EBV
- Symptoms develop over days to 4 weeks
- Affects limbs and cranial nerves
- Mild sensory symptoms and signs
- Significant weakness
- Areflexia
- Autonomic nervous system involvement e.g. arrhythmia, hypo/hypertension
455
Clinical features of Miller-Fisher variant of Guillain-Barre syndrome
- Areflexia
- Opthalmoplegia
- Ataxia
456
How would you diagnose Guillain-Barre syndrome
- Clinical diagnosis
Supported by
- Neurophsyiological studies
- Lumbar puncture - shows raised CSF protein
457
Treatment for Guillain-Barre syndrome
Plasma exchange OR IV immunoglobulin
and supportive measures
458
Main risk factor for vasculitis neuropathies
Systemic vascular disease
- Wegener's granulomatosis
- Polyarteritis nodosa
- RA
- Sjogren's syndrome
459
Clinical features of neuralgic amyotrophy
- Uncommon
- History of infection or vaccination
- Severe neuralgic pain in one arm followed by weakness
- Pain eases after a few weeks and patient notices muscle weakness
- Typically affects shoulder girdle - winging of the scapula
- Sensory loss in lateral aspect of upper arm
460
Risk factors of median nerve neuropathy
- Pregnant
- Rheumatoid arthritis
- Hypothyroidism
- Diabetes
- Acromegaly
- Myeloma
- Female sex
- Age 40-60
461
Most common mononeuropathy
Median nerve
462
Clinical features of Carpal Tunnel Syndrome
- Age 40-60
- Dominant hand affected first
- Patient awoken with tingling or pain in hand - progresses to occur throughout the day
- Pain relieved by shaking/dangling hand
- Weakness of prolonged grip/clumsy hands
- Abductor pollicus brevis wasting
- Sensory changes in median nerve distribution
- Positive Tinel's test
- Positive Phalen's test
463
How would you diagnose a mononeuropathy?
- Nerve conduction studies
464
Management of carpal tunnel syndrome
1) Wrist splints
2) Corticosteroid injections into carpal tunnel
3) Surgical decompression
465
Factors which indicate a poor prognosis for surgical decompression in carpal tunnel syndrome
- Duration of symptoms >6 months
- Increasing age
- Pre-op muscle atrophy
- Poor response to corticosteroid injections
466
Clinical features of ulnar nerve lesion
- Injury/compression/bony deformities at the elbow
- Numbness/tingling in pinky finger
- Arm pain
- Hand weakness
- Weakness and wasting of interpose and long flexors of ring and pinky finger
- Sensory loss in ring and pinky finger
467
Management of ulnar nerve lesion
- Avoidance of further compression
| - Surgical decompression or transposition
468
Clinical features of radial nerve lesion
- Deep sleep/unconsciousness
- Humeral shaft fracture
- Weakness of wrist and finger extension
- Impaired triceps reflex
- Sensory loss at base of thumb
469
Management of a radial nerve lesion
- Most improve spontaneously
| - Lively splint
470
Clinical features of a common peroneal nerve lesion
- External pressure or trauma to the fibula
- Foot drop
- Tibilais anterior wasting
- Weakness of ankle dorsiflexion, toe extension and foot eversion
- Sensory loss down lateral aspect of leg and top of foot
471
Management of a common peroneal nerve lesion
- Foot drop splint
| - Surgical decompression or transposition
472
Clinical features of a lateral cutaneous nerve of the thigh lesion
- Related to weight changes
| - Burning/numbness on the lateral aspect of the thigh
473
How would you investigate and manage a lateral cutaneous nerve of the thigh lesion?
- No investigation
| - No treatment - self-limiting
474
Clinical features of a femoral nerve lesion
- Diabetes
- Weakness and wasting of the quadriceps
- Sensory loss on inner aspect of thigh
475
Clinical features of raised intracranial pressure
- Headache
- Ataxia
- Confusion
- Drowsiness
- Coma
- Papilloedema
- Sixth nerve palsy
- 3rd nerve palsy (temporal lobe herniation)
- Failure of up gaze (mid brain compression)
- Rise in systolic BP (medullary involvement)
- Fall in pulse (medullary involvement)
476
Causes of reduced intracranial pressure
CSF leakage through torn meninges caused by:
- trauma
- iatrogenic
- tumours
477
Clinical features of reduced intracranial pressure
- Headache on standing
| - CSF rhinorrhoea
478
Clinical features of spontaneous intracranial hypotension
- Generalised headache, worse on standing and improved on lying flat
- 6th nerve palsy
479
What would you see on imaging of a patient with spontaneous intracranial hypotension?
- On MRI scanning with gadolinium you would see marked dural enhancement reflecting engorged normal veins (as CSF is reduced)
480
Features of tectorial herniation
- Unilateral 3rd nerve palsy involving the pupils
- Coma
- Bilateral pupil changes
- Tetraparesis
- Occipital infarct - worsens herniation
481
Features of foramen magnum herniation
- Severe neck pain
- Erratic breathing
- Progressive tetraparesis
- Coma
482
Causes of a asymmetrical gait abnormality
- Orthopaedic problems e.g. fixed hip joint, shortened limbs
- Pain
Neurological abnormalities (usually reflect unilateral weakness or abnormal tone)
- Hemi-Parkinsonian gait - arm swing reduced on one side, posture is slightly stiff and gait is hesitant
- Hemiplegic/circumducting gait - one leg is swift and swung out and around, often catching the toes
- Foot drop - high stepping gait where the heel is brought up to avoid catching toe, usually a slap as the foot is brought down
483
Causes of a symmetrical gait abnormality with broad-based gait
- Loss of proprioception (sensory ataxia) - usually have a positive Romberg's test
- Loss of coordination (ataxia) caused by midline cerebellar ataxia or widespread cerebellar disease
484
Symmetrical gait with narrow-based gait
- Parkinsonism - short-paced, stooped, poor arm swing. May have difficulty stopping and starting. When walking they appear to be falling froward, often trying to catch up with themselves (festinant gait)
- Marche a petit pas - small steps, upright posture, good arm swings - caused by cerebellar disease
485
Cause of scissoring gait
- Bilateral leg spasticity with circumlocution from both legs e.g. spinal cord disease or bilateral hemisphere disease
486
Causes of bizarre gait
- Chorea - Huntingtons, Parkinsons treatments
- Multiple diseases
- Functional disorder
487
Cause of apraxia (great difficulty in starting, with a broken up gait pattern)
- Normal pressure hydrocephalus
| - Diffuse cerebral disease
488
Causes of high stepping gait
- Sensory ataxia
| - Peripheral neuropathy
489
Cause of a waddling gait (patient with marked hip movement abnormalities with prominent hip rotation on walking)
- Hip disease
| - Proximal pelvis weakness
490
Causes of marche a petit pas
- Diffuse cerebrovascular disease
491
Causes of cerebellar syndromes
- MS (most common cause in younger patients)
- Stroke (most common cause in older patients)
- Tumour
- Trauma
- Degeneration
- Metabolic e.g. hypothyroidism
- Paraneoplastic
- Toxic e.g. anti-convulsnats and alcohol
492
Main 3 features of cerebellar disease
- Clumsiness
- Incoordination
- Ataxia
493
Most common inherited muscle disease
DMD
494
Clinical features of Duchenne Muscular Dystrophy
- Weakness of lower limb girdle by age 3-6
- Gower manoeuvre
- Weakness spreads to other muscles in arms and legs
- Pseudohyertrophy of the calves (rubbery)
- Low IQ
495
How would you diagnose a patient with DMD?
- Muscle creatinine kinase - highly elevated
- Muscle biopsy - no fibre staining for dystrophin
- ECG
496
Clinical features of myotonic syndrome
- Stiffness
- Limitation of movement
- Weakness
- Contractions provoked by cold or direct muscle stimulation
497
Clinical features of dystrophic myotonia
- Muscle cramps triggered by the cold
- Hand weakness
- Difficulty releasing grip
- Characteristic facial appearance - frontal balding, severe temporals and sternocleidomastoid muscle wasting, bilateral ptosis, facial weakness
- Limb weakness worse distally
- Areflexia
498
Treatment options for myotonia
- Phenytoin
- Procainamide
- Quinine
499
Complications of myotonia
- Cardiac conduction abnormalities
- Impaired respiratory drive
- Diabetes
- Hypothyroidism
- Cataracts
- Male infertility
- Bowel dysmotility
- Mental retardation
500
Causes of acquired muscle disease
- Renal/hepatic failure
- Hypocalcaemia
- Hypokalemia
- Hypomagnesaemia
- Hypo/hyperthyroidism
- Cushing's syndrome
- Addison's disease
- Polymyositis
- Dermatomyositis
- Vasculitis
- Acute viral myositis
- Sarcoidosis
- Drugs e.g. statins, tyrptophan, zidovudine
- Parasites
501
Clinical features of polymyositis
- Woman
- Age 30-60
- Proximal limb, trunk, neck,, pharyngeal, oesophageal weakness
- Painful muscles
- Elevated CK
- EMG shows myopathic changes with increased spontaneous activity
502
How would you diagnose polymyositis?
- Diagnosed confirmed by demonstrating inflammatory changes on muscle biopsy
503
Treatment for polymyositis
- Corticosteroids and immunosuppression
| - Treatment response monitored using CK levels
504
Clinical features of dermatomyositis
- Children
- Purple heliotrope rash round the eyes
- Gottron's papules
505
Dermatomyositis and polymyositis are associated with an increased risk of...
Lymphoma and ovarian cancer
506
Clinical features of Bell's palsy
- Ipsilateral loss of control of facial muscles
- Symptoms come on over 48 hours
- Ptosis
- Change in taste
- Pain around the ear
- Phonophobia
- Inability to show teeth or raise eyebrows (forehead NOT spared)
507
Risk factors for Bell's palsy
- Diabetes
- URTI
- Pregnancy
508
Management options for a patient with Bell's palsy
1) Eyedrops or an eyepatch
2) Corticosteroids
3) Antiviral medication
