Neurology Flashcards
1
Q
Describe symptoms of an extradural bleed
A
- developed a headache after head trauma
- confused and drowsy
- difficult to rouse
- biconvex shape on CT
2
Q
Describe presentation of subarachnoid bleeding
A
- sudden onset
- severe thunderclap headache
- occurs due to strenuous activity
3
Q
What is Guillan-Barré syndrome?
A
- an acute paralytic polyneropathy
- affects peripheral nervous system
- symptoms peak after 2-4 weeks, recovery can take months-years
4
Q
What causes Guillan-Barré syndrome?
A
- triggered by infection
- associated with:
- campylobacter jejuni (MC)
- cytomegalovirus
- Epstein-Barr virus
5
Q
What is the pathophysiology behind Guillan-Barré syndrome?
A
- occurs due to molecular mimicry
- B cells create antibodies against antigens on the pathogen
- these antibodies match proteins on nerve cells
- Abs then target myelin sheath of motor nerve cell or nerve axon
6
Q
How does Guillan-Barré syndrome present?
A
- symmetrical ascending weakness (starting at feet)
- reduced reflexes
- peripheral loss of sensation/neuropathic pain
- progresses to cranial nerves and causes facial weakness
7
Q
How is Guillan-Barré syndrome diagnosed?
A
- Brighton criteria
- nerve conduction studies/electromyography
- lumbar puncture for CSF: raised protein, normal cell count and glucose
8
Q
How is Guillan-Barré syndrome managed?
A
- IV immunoglobulins
- plasma exchange
- supportive care
- VTE prophylaxis
9
Q
What is multiple sclerosis?
A
- chronic and progressive condition involving demyelination of neurones in the CNS
10
Q
What is the epidemiology of multiple sclerosis?
A
- young adults (under 50)
- more common in women
- symptoms improve in pregnancy and postpartum
11
Q
What is the pathophysiology of multiple sclerosis?
A
- myelin made by Schwann cells (PNS) and oligodendrocytes (CNS)
- immune cells infiltrate and damage myelin > inflammation > symptoms
- re-myelination can occur in early disease, is incomplete in late disease > permanence
12
Q
What is the aetiology of multiple sclerosis?
A
- genetic
- EBV
- low vit D
- smoking
- obesity
13
Q
Why do symptoms of MS change over time?
A
- lesions vary in location over time
- different nerves are affected and symptoms change
- early course: relapsing-remitting attacks
14
Q
How is multiple sclerosis managed?
A
- acute episodes: IV methylprednisolone
- DMDs: β interferon
- mitoxantrone
15
Q
What optical symptoms occur with multiple sclerosis?
A
- double vision: abducens (VI) lesions
- internuclear opthalmoplegia: problems with coordinating eye movements (III, IV, VI)
- conjugate lateral gaze disorder: affected eye cannot abduct (VI)
16
Q
How is multiple sclerosis diagnosed?
A
- progressive symptoms over a year
- or 2 attacks disseminated in time and space
- MRI brain + cord showing lesions
- LP shows oligoclonal IgG bands in CSF
17
Q
What is optic neuritis?
A
- unilateral reduced vision over hours to days
- central scotoma (enlarged blind spot)
- impaired colour vision
- pain on eye movement
- relative afferent pupillary defect
18
Q
What is the general presentation of multiple sclerosis?
A
- sensory and cerebellar ataxia
- tremors
- incontinence
- limb paralysis
- trigeminal neuralgia, numbness and paresthesia
19
Q
What are the types of disease course of multiple sclerosis?
A
- relapsing-remitting: episodes of disease followed by recovery - can be active and/or worsening
- 1º progressive: gradual deterioration without recovery
- 2º progressive: relapsing-remitting > worsening and incomplete remission
20
Q
What are differential diagnoses for multiple sclerosis?
A
- SLE, Sjrogen’s, encephalomyelitis
- Lyme’s, syphilis, AIDS
21
Q
What is Parkinson’s disease?
A
- progressive reduction of dopamine in the basal ganglia leading to movement disorders
- substantia nigra produces dopamine
- asymmetrical symptom
22
Q
How is Parkinson’s diagnosed and what is the classic triad?
A
- clinically based on symptoms
- resting tremor, rigidity, bradykinesia
- DaTscan
23
Q
What are the symptoms of Parkinson’s?
A
- unilateral tremor: 4-6Hz
- cogwheel rigidity: jerky movement
- bradykinesia: shuffling gait, difficulty initiating movement, reduced facial expression
- anosmia
- postural instability
24
Q
How is Parkinson’s managed?
A
- Levodopa + carbidopa (decarboxylase inhibitor)
- dopamine agonist e.g. bromocriptine
- COMT inhibitors e.g. entacapone: metabolises L-dopa
- Monoamine oxidase B inhibitors - rasagiline
25
How does levodopa work and what are the associated side effects?
- synthetic dopamine
- taken alongside decarboxylase inhibitor e.g. carbidopa
- becomes less effective over time
- leads to dyskinesias e.g. dystonia, chorea, athetosis
26
What are the risk factors and epidemiology of Parkinson's?
- family history
- male
- inc age
- e.g. 70y/o male
27
What is Huntington's chorea and what is the pathophysiology?
- autosomal dominant condition with full penetrance
- trinucleotide repeat disorder in HTT in chromosome 4
- lack of GABA and excessive nigrostriatal pathway
28
What is genetic anticipation?
- more trinucleotide repeats leads to earlier onset of disease and increased severity
29
When does Huntington's present and what is the prognosis?
- asymptomatic until age 30-50
- life expectancy: 15-20yrs after onset of symptoms
30
How does Huntington's present?
- cognitive, psychiatric and mood problems > movement disorder
- chorea
- eye movement disorders
- dysarthria and dysphagia
- depression
31
How is Huntington's diagnosed?
- genetic testing with pre and post-test counselling
- diagnosis if > 35 CAG repeats
32
How is Huntington's managed?
- antipsychotics e.g. olanzapine
- benzodiazepines e.g. diazepam
- dopamine-depleting agents e.g. tetrabenzine
- antidepressants
33
What is myasthenia gravis?
- autoimmune condition causing muscle weakness
- progressively worse weakness with activity which improves with rest
- mostly affects proximal muscles and small muscles of head and neck
34
How is myasthenia gravis diagnosed?
- testing for antibodies: acetylcholine receptor (Ach-R) and muscle-specific kinase (MuSK)
- Edrophonium test
35
What is the edrophonium test for myasthenia gravis?
- IV dose of edrophonium chloride
- edrophonium blocks cholinesterase enzymes
- stops Ach breakdown and and inc Ach levels
- relieves weakness temporarily
36
What is the epidemiology of myasthenia gravis?
- women under 40
- men over 60 (more related to thymoma)
37
What is the pathophysiology behind myasthenia gravis?
- Ach-R antibodies bind to postsynaptic NMJ receptors
- Ach unable to bind, stimulate receptor and trigger muscle contraction
- more used during activity and so more are blocked
- Abs activate complement system leading to damage at postsynaptic membrane
38
How does myasthenia gravis present?
- extraocular muscle weakness > diplopia
- eyelid weakness > ptosis
- dysphagia
- jaw fatigue
- slurred speech
- facial weakness
39
How can myasthenia gravis be examined clinically?
- repeated blinking > ptosis
- prolonged upward gazing > diplopia
- repeated abduction of one arm > unilateral weakness
40
How is myasthenia gravis managed?
- reversible Ach inhibitors: pyridostigmine
- immunosuppression
- thymectomy
- monoclonal Abs: rituximab
41
What is myasthenic crisis?
- complication causing acute worsening of symptoms
- triggered by another illness e.g. resp tract
- can lead to resp failure > ventilation
- treatment with IVIG and plasma exchange
42
What is the presentation of trigeminal neuralgia?
- intense facial pain
- comes on spontaneously, can last seconds to hours
- electricity-like shooting pain
43
How is trigeminal neuralgia treated?
- carbamazepine
44
What is the pathophysiology of trigeminal neuralgia?
- can affect any combination of V1, V2 and V3
- 90% cases are unilateral
- triggers: cold weather, spicy food, caffeine, citrus
45
What are the 3 branches of the trigeminal nerve?
- V1: ophthalmic
- V2: maxillary
- V3: mandibular
46
How do tension headaches present?
- mild ache across forehead in band like pattern
- muscle ache in frontal, temporalis and occipitalis muscles
- come on and resolve gradually: no visual changes
47
What are the causes of tension headaches?
- alcohol
- depression
- dehydration
- stress
- skipping meals
48
What are cluster headaches?
- severe and unbearable unilateral headaches
- usually around the eye
- crescendo pain which may affect temples
49
What are the symptoms of cluster headaches?
- unilateral
- red, swollen, watering eye
- pupil constriction
- ptosis
- nasal discharge
- facial sweating
50
What is the frequency of cluster headaches?
- attacks lasting 15 mins - 3hrs
- may suffer multiple attacks per day for 4-12 weeks
- followed by pain free period lasting 1-2 years
51
Who is the typical patient to suffer from cluster headaches?
- 30-50 y/o male smoker
- attacks triggered by: alcohol, strong smells, exercise
52
What are the types of migraines?
- migraine with or without aura
- silent migraine (with aura but without headache
- hemiplegic migraine
53
What is aura?
- visual changes
- sparks in vision
- blurring lines
- lines across vision
- loss of visual field
54
How do triptans work?
- 5HT receptor agonists (serotonin)
- cause vasoconstriction in artery smooth muscle
- inhibit peripheral pain receptors
- reduce neuronal activity in CNS
55
What are the symptoms of migraine?
- pounding/throbbing
- usually unilateral
- photophobia and phonophobia
- nausea and vomiting
56
What are migraine triggers?
- stress
- bright lights
- strong smells
- dehydration
- menstruation
- abnormal sleep patterns
- certain foods e.g. chocolate, cheese, caffeine
57
What is the treatment for migraine?
- paracetamol
- triptans e.g. sumatriptan
- NSAIDs
- antiemetics
58
What is prophylaxis for migraine?
- headache diary
- propanolol, topiramate, amitriptyline
- acupuncture
- riboflavin
59
What is epilepsy?
- an umbrella term for a condition where there is a tendency to have seizures
60
What is a seizure?
- a transient episode of electrical activity due to abnormally excessive or synchronous neuronal activity within the brain
61
How is epilepsy diagnosed?
- electroencephalogram: electrical activity in brain
- MRI brain
62
What is a generalised tonic-clonic seizure?
- loss of consciousness with muscle tensing (tonic) and muscle jerking (clonic) movements
- post-ictal period
- limb contraction, extension, back arching and - usually last 2-3 minutes
- ictal cry: sound resulting from chest contraction
63
What is an absence seizure?
- commonly in school aged children
- becomes blank and stares into space then abruptly returns to normal
- unaware of their surroundings and unresponsive but still conscious
- Episodes last 10-20 seconds and can disappear with age
64
What investigations are done for stroke?
- 1st line: non contrast CT head
- gold: diffusion weighted MRI
- blood test
- carotid doppler
65
What is the management for stroke?
- aspirin 300mg stat and continued for 2 weeks
- thrombolysis with alteplase (TPA) in 4.5 hr window
- keep BP high to avoid reduced perfusion
66
What are the secondary prevention guidelines for stroke?
- Clopidogrel 75mg once daily
- Atorvastatin 80mg started (not immediately)
- Carotid endarterectomy (>50%) or stenting
- Treat modifiable risk factors
67
What is subarachnoid haemorrhage?
- bleeding into subarachnoid space between Pia mater and arachnoid membrane (where CSF is)
- usually due to ruptured cerebral aneurysm
68
What are the symptoms of a subarachnoid haemorrhage?
- thunderclap headache described like being hit on the back of the head
- neck stiffness
- photophobia
- visual changes
- neurological symptoms
69
What is the presentation of brain tumours?
- headache
- seizures
- focal neurological symptoms
- other non-focal symptoms e.g. cognitive problems or behavioural changes
70
What are the symptoms of a raised ICP headache?
- woken by headache
- worse in the morning, lying down
- exacerbated by coughing, sneezing, drowsiness
- nausea and vomiting
71
Which cancers commonly metastasise to the brain?
- lung
- breast
- renal cell carcinoma
- melanoma
72
What are the types of gliomas?
- astrocytomas
- oligodendroglioma
- ependymoma
- grade 1 are benign
- grade 4 are most malignant: glioblastoma
73
What is the treatment for brain tumours?
- surgery: early resection
- combined radiotherapy and chemotherapy
74
What is motor neurone disease?
- progressive, ultimately fatal condition
- motor neurones stop functioning
75
What are the types of motor neurone disease?
- amyotrophic lateral sclerosis (ALS)
- progressive bulbar palsy
- progressive muscular atrophy
- primary lateral sclerosis
76
What is the pathophysiology behind motor neurone disease?
- progressive degeneration of upper and lower motor neurones
- sensory neurones spared
77
What are risk factors for motor neurone disease?
- genetic
- smoking
- SOD-1 mutation
- exposure to heavy metals and pesticides
78
What is the presentation of motor neurone disease?
- progressive weakness of muscles affecting limbs, trunk, face, speech
- weakness first noticed in upper limbs: fatigue when exercising
- dysarthria
- mixed LMN and UMN signs
79
What are signs of lower motor neurone disease?
- muscle wasting
- reduced tone
- fasciculations
- reduced reflexes
- Babinski -ve
80
What are the signs of upper motor neurone disease?
- increased tone or spasticity
- brisk reflexes
- no fasciculation
- babinski +ve: upgoing plantar responses
81
What is the management of motor neurone disease?
- riluzole: sodium channel blocker
- prevents stimulation of glutamate receptors
- non-invasive ventilation to support breathing at night (BIPAP)
82
What are the causes of subarachnoid haemorrhage?
- cocaine use
- sickle cell anaemia
- connective tissue disorders
- neurofibromatosis
- autosomal dominant PKD
83
What is the epidemiology of subarachnoid haemorrhage?
- black patients
- female
- age 45-70
84
What are risk factors for subarachnoid haemorrhage?
- htn
- smoking
- excess alcohol consumption
- cocaine use
- family history
85
What is the management of subarachnoid haemorrhage?
- surgical intervention for aneurysms: endovascular coiling with catheter or clipping off
- nimodipine: CCB that prevents vasospasm
86
How is a subarachnoid haemorrhage investigated?
- 1st line: NC CT head within 6hrs: hyperattenuation of blood in subarachnoid space - hyperdense/bright
- If negative: LP for CSF. Raised red cell count and xanthochromia (bilirubin)
- If +ve: angiography
87
What is a transient ischaemic attack (TIA)?
- transient neurological dysfunction secondary to ischaemia without infarction
- often precede a stroke
- caused by carotid thrombo-emboli
88
What is amaurosis fugax?
- sudden loss of vision in one eye: veiling
- caused by infarct in retinal arteries
- retinal branches from ophthalmic which branches from int carotid
89
What are the symptoms of a stroke in the anterior cerebral artery?
- contralateral hemiparesis and sensory loss
- affecting lower limbs > upper
90
What are the symptoms of a stroke in the middle cerebral artery?
- weak, numb, contralateral side of the body
- upper > lower limbs
- facial drooping
- forehead sparing
- contralateral homonymous hemianopia
- dysphasia (temporal lobe)
91
What are the symptoms of a stroke in the posterior cerebral artery?
- visual loss: loss of recognition
- contralateral homonymous hemaniopia with macular sparing
92
What is lateral medullary syndrome?
- posterior inferior cerebellar artery stroke
- ipsilateral facial pain and temperature loss
- contralateral limb/torso pain and temp loss
- ataxia
- nystagmus
93
What are the symptoms of a stroke of the anterior inferior cerebellar artery?
- lateral pontine syndrome
- ipsilateral facial paralysis and deafness
- ipsilateral facial pain and temperature loss
- contralateral limb/torso pain and temperature loss
- ataxia
- nystagmus
94
What is Weber's syndrome?
- posterior cerebral artery stroke affecting midbrain
- ipsilateral CN3 palsy: eye down and out, ptosis
- contralateral limb/torso pain and temperature loss
- ataxia
- nystagmus
95
What are the symptoms of a basilar artery stroke?
- locked in syndrome
96
What are the symptoms of a retinal/ophthalmic artery stroke?
- amaurosis fugax
97
What 3 criteria must be present for a total anterior circulation stroke?
1. unilateral weakness (and/or sensory deficit) of the face, arm and leg
2. homonymous hemianopia
3. higher cerebral dysfunction (dysphasia, visuospatial disorder)
98
What is the difference between a total and partial anterior circulation stroke?
- partial: only 2/3 criteria present
- higher cerebral dysfunction alone is classed as PACS
99
What are the criteria of a posterior circulation stroke?
- cranial nerve palsy and contralateral motor/sensory deficit
- bilateral motor/sensory deficit
- conjugate eye movement disorder
- cerebellar dysfunction
- isolated homonymous hemianopia
100
What is a lacunar stroke?
- subcortical stroke occurring secondary to small vessel disease
- no loss of higher cerebral dysfunction
101
What are the criteria for a lacunar stroke?
- pure sensory or pure motor stroke
- sensori-motor stroke
- ataxic hemiparesis
102
What special considerations should be made when treating meningitis?
- allergy to penicillin: if anaphylaxis switch to chloramphenicol
- immunocompromised: potentially caused by listeria: add amoxicillin
- recent travel: add vancomycin
103
What are contraindications for a lumbar puncture?
- abnormal clotting (platelets/coagulation)
- petechial rash
- raised ICP
104
What is the definition and presentation of encephalitis?
- inflammation of the brain
- flu-like illness
- altered GCS, fever, seizures, memory loss
105
What are the causes of encephalitis?
- usually viral
- herpes simplex
- varicella zoster
106
What are the investigations for encephalitis?
- MRI head
- LP: lymphocytic CSF and viral PCR
107
What is the management of encephalitis?
- supportive
- aciclovir if HSV or VZV
108
What is cauda equina syndrome?
- nerve roots of cauda equina at bottom of spine are compressed
- emergency
109
What is the anatomy of the cauda equina?
- collection of nerve roots travelling through spinal canal after cord terminates around L2/L3
- spinal cord tapers down into conus medullaris
- nerve roots exit at vertebral level
110
What do the nerves of the cauda equina supply?
- sensation: lower limbs, perineum, bladder, rectum
- motor: lower limbs, anal and urethral sphincters
- parasympathetic: bladder and rectum
111
What is the aetiology of cauda equina syndrome?
- herniated disc at L4/5 or L5/S1(MC)
- (metastasised) tumours
- abscess and trauma
112
What is the management of cauda equina?
- emergency MRI and neurosurgical input to consider lumbar decompression surgery
113
What are signs of cauda equina syndrome?
- saddle anaesthesia
- loss of sensation in bladder and rectum
- faecal incontinence
- bilateral sciatica and motor weakness
114
What are the causes of spinal cord compression?
- degenerative disc lesions
- degenerative vertebral lesions
- TB
- vertebral neoplasms
- epidural abscess or haemorrhage
115
What is the presentation of spinal cord compression?
- sensory loss below level of compression
- spastic paraparesis/tetraparesis
- radicular pain at level of compression
116
Where is the sciatic nerve and what does it do?
- L4-S3 form sciatic nerve
- supplies sensation to lateral lower leg and foot
- motor for posterior thigh, lower leg and foot
117
What is sciatica?
- unilateral pain from the buttock radiating down the back of the thigh to below the knee or foot
- causes: herniated disc, spondylolisthesis, spinal stenosis
118
What is the presentation of sciatica?
- electric or shooting pain
- paraesthesia
- numbness
- motor weakness
119
What symptoms are experienced in the postictal period?
- amnesia
- postictal nose wiping (ipsilateral)
- confusion, irritability, feeling low
- sore tongue from bitten
120
What is the difference between tonic and atonic seizures?
- Tonic: person becomes stiff suddenly
- Atonic: known as drop attacks where all muscles relax at once (lapse in muscle tone). These don’t tend to last for more than 3 minutes
121
What is a myoclonic seizure?
- sudden, brief muscle contractions like a jump
- Seizures are brief but can occur in clusters, usually shortly after waking up
- Patient remains conscious.
122
What is a complex partial seizure?
- consciousness may be impaired
- Often occurs in the temporal lobe
- May have post-ictal confusion
123
What are the symptoms of partial temporal lobe seizures?
- rising feeling in stomach, deja vu, unusual taste or smells, intense feelings of fear or joy
- automatisms: lip smacking, plucking
124
What are the symptoms of partial frontal lobe seizures?
- ‘wave feeling’ going through head, stiffness or twitching in a limb
- Jacksonian march: clonic movements starting in an extremity and moving proximally
125
What are the symptoms of partial parietal lobe seizures?
- numbness or tingling, arms or legs feel bigger or smaller than normal
126
What are the symptoms of partial occipital lobe seizures?
visual disturbance (coloured or flashing lights), hallucinations
127
What is a focal seizure?
- only one part of the brain is affected
- Can be aware or unaware
- start in the temporal lobes and can affect hearing, speech, memory and emotions.
- Presentation: hallucination, flashbacks, deja vu, doing things on autopilot.
128
How is epilepsy investigated?
- electroencephalogram (EEG) shows typical patterns
- MRI brain
129
What is the treatment for epilepsy?
- lamotrigine or levitiracetam
- carbamazepine: 1st line for focal seizure
130
How does sodium valproate work and what are the side effects?
- increases activity of GABA: has relaxing effect
- inhibits sodium ion channels
- teratogenic, liver damage, hair loss, tremor
131
What are the causes of seizures?
- vascular
- idiopathic
- infection
- trauma
- dementia and drugs
132
What are some seizure triggers?
- fatigue
- stress
- alcohol and drugs
- flashing lights
- monthly periods
- missing meals
- fever
133
What is the pathophysiology behind epileptic seizures?
- normal balance between GABA and glutamate
- balance shifts towards glutamate
- more excitatory > inc glutamate stimulation and inc GABA inhibition
134
What is the ABCD2 scoring system?
- risk of stroke following TIA
- Age >60 (1)
- BP > 140/90 (1)
- Clinical symptoms: unilateral weakness (2), slurred speech, no weakness (1)
- duration Sx: >1h (2). <1hr (1)
- T2DM (1)
135
What is an ischaemic stroke?
- 85% cases
- a long TIA
- caused by thrombosis/AF embolisation
136
What is a haemorrhagic stroke?
- 15% cases
- ruptured blood vessel
- caused by trauma, htn, berry aneurysm rupture
137
What is an extradural haemorrhage?
- usually caused by rupture of middle meningeal artery
- in temporo-parietal region
- associated with fracture of temporal bone
- occurs between skull and dura mater
138
Where does a subdural haemorrhage occur?
- rupture of bridging veins in outermost meningeal layer
- occurs between dura mater and arachnoid mater
139
Describe the presentation of subdural bleeding
- crescent shape on CT and possible midline shift
- drowsiness, reduced consciousness, high ICP
- seizure
140
What is the pathophysiology behind subdural bleeding?
- usually occurs due to trauma
- turning/jerking injuries cause damage to the bridging veins and causes tears
- elderly and alcoholics have more brain atrophy making rupture more likely
141
What is dementia?
- progressive global decline of cognitive function without impairment of consciousness
- mild cognitive impairment (MCI) is seen as a pre-cursor
- typically affects temporal and parietal lobes
142
What is vascular dementia?
- results from many small infarcts
- step-wise progression
- infarcts affect function if damaging white matter
143
How can vascular dementia be prevented and which conditions is it associated with?
- aspirin or warfarin therapy
- controlling BP
- raised BP, past strokes
144
What is Lewy-body dementia?
- presence of Lewy bodies in the brainstem and neocortex
- fluctuating cognitive impairment
- hallucinations and Parkinsonism
145
What are the medications used for tremors?
- primidone
- β blockers
- gabapentin
146
What is the pathophysiology behind peripheral neuropathy (6 mechanisms)?
1. demyelination (Schwann cell damage)
2. axonal degeneration
3. nerve fibres cut or crushed
4. compression causing demyelination
5. infarction
6. infiltration by infection
147
What are the aetiologies of secondary intracerebral haemorrhages?
- clotting disorder
- AV malformation
- brain tumour
- intracranial aneurysm
- trauma
148
What are the classic cerebellar signs (DANISH)?
- D: dysdiadochokinesia
- A: ataxia
- N: nystagmus
- I: intentional tremor
- S: slurred speech
- H: hypotonia
149
What is Charcot-Marie-Tooth disease?
- autosomal dominant disease affecting peripheral motor and sensory nerves
- cause dysfunction in myelin and axons
150
What are classical presentations of Charcot-Marie-Tooth disease?
- high foot arches (pes cavus)
- distal muscle wasting (stork calves)
- lower leg and hand weakness
- reduced tendon reflexes and sensory tone
151
What are the causes of peripheral neuropathy? ABCDE
- Alcohol
- B12 deficiency
- Cancer and CKD
- Diabetes and drugs (isoniazid, amiodarone and cisplatin)
- Every vasculitis
152
How is Duchenne's muscular dystrophy passed down and who does it affect?
- X-linked recessive mutation
- boys affected exclusively
153
What is the pathophysiology and presentation of Duchenne's muscular dystrophy?
- muscle is replaced with adipose tissue
- causes difficulty getting up from lying down
- skeletal deformities: scoliosis, hyperlordosis
154
What is carpal tunnel syndrome?
- compression of the medial nerve as it travels through the wrist
155
How is carpal tunnel syndrome managed?
- rest and altered activities
- wrist splints
- steroid injections
- surgery: flexor retinaculum cut to relieve pressure
156
How does carpal tunnel syndrome present?
- worse at night
- sensory symptoms: numbness, paraesthesia, burning, pain
- weakness of thumb movements, grip strength, difficulty with fine movement, wasting of thenar muscles
157
What occurs in the frontal lobe?
- voluntary and planned motor behaviours
- motor speech area (Broca's)
- personality
- planning
158
What is the function of the temporal lobe?
- hearing
- language comprehension
- semantic knowledge
- memory
- emotional behaviour
159
What is the presentation of Alzheimer's?
- temporal: episodic memory disorder
- parietal: visuospatial difficulties
- receptive and expressive dysphagia
- emotional disorder/psychosis
- problems with planning and problem solving
- early loss of language (subtle)
160
What is the pathophysiology of Alzheimer's disease?
- accumulation of β-amyloid peptides > plaques
- degrades amyloid precursor protein
- leads to neuronal damage, neurofibrillary tangles, amyloid plaques
- loss of Ach
161
Which areas of the brain are most affected by Alzheimer's?
- hippocampus
- amygdala
- temporal neocortex
- subcortical nuclei
162
What are the risk factors for Alzheimer's?
- 1st degree relative
- Down's
- depression/loneliness
- decreased exercise, physical activity
163
What are β-amyloid plaques?
- amyloid precursor protein broken down (found in cell membrane of neurones)
- leads to excess deposition of β-amyloid
- accumulate between synapses and affect nerve transmission
- cause localised inflammation
164
What is the management of dementia?
- AChE inhibitors: donepezil: inc cholinergic transmission in brain
- NMDA antagonists: memantine: inhibits glutamate receptors
- antipsychotics
165
What is Alzheimer's disease?
- leading cause of dementia
- progressive neurodegenerative disorder causing deterioration in mental performance
- general atrophy of brain
- frontal and temporal lobes affected in particular
166
What is fronto-temporal dementia?
- frontal and temporal lobe atrophy: loss of spindle neurones
- behaviour change
- disinhibition and emotional unconcern
167
How is dementia investigated?
- CT/MRI to look for lesions/atrophy
- bloods
- questionnaires: 6 CIT/MMSE
168
What is Kernig's test?
- lie patient on back
- flexing one hip and knee to 90 degrees and slowly straighten knee (keep the hip flexed)
- spinal pain or resistance to movement in meningitis
169
What is Brudisinski's test?
- lie patient on back
- gently lift head and neck and flex chin to chest
- patient will involuntarily flex hips and knees
170
What is the anatomy of the carpal tunnel?
- between the carpal bones and flexor retinaculum
- flexor retinaculum: runs across palmar side of wrist
- median nerve and flexor tendons travel through
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What does the palmar cutaneous branch of the median nerve innervate?
- sensory to palm
- thumb
- index and middle finger
- lateral half of ring finger
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What are the risk factors for carpal tunnel syndrome?
- repetitive strain
- obesity
- perimenopause
- RA
- diabetes
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What is Phalen's test?
- fully flexing the wrist and holding it there
- wrists bent inwards at 90º
- triggers numbness and parasthesia
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What is Tinel's test?
- tapping the wrist where the median nerve meets the carpal tunnel
- triggers numbness and parasthesia
175
What other investigations are done for carpal tunnel syndrome?
- nerve conduction studies
- carpal tunnel questionnaire
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What is syncope?
- temporarily losing consciousness
- due to disruption of blood flow to brain
- also called vasovagal episodes or fainting
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What is the presentation of syncope?
- hot and clammy, sweaty
- heavy
- dizzy, lightheaded
- vision going dark
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What is the management of TIA?
- 300mg aspirin as soon as suspected
- followed by clopidogrel
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What are some causes of lower motor neurone injury?
- MND, spinal muscular atrophy, polio
- Guillain Barre
- neuropathies
- myasthenia gravis
- myositis
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What is the presentation of spinal muscular atrophy?
- symmetrical, proximal muscle weakness caused by loss of motor neurons
- lower limbs more affected than upper
- affects bulbar function and breathing
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Describe SMA 1 and 2
- SMA 1: onset in first few months, death within 2 years
- SMA 2: onset in 18 months, never walk but survive to adulthood
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Describe SMA 3 and 4
- SMA 3: onset after 1y/o, walk but later lose ability, resp affected
- SMA 4: onset in 20s, ability to walk for short distances, everyday tasks > fatigue. resp + life expectancy not affected
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What is the pathophysiology behind neuropathies?
- primary demyelination > secondary axonal degeneration
- primary axonal degeneration > secondary demyelination
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What are the types of peripheral neuropathies?
- mononeuropathy: one nerve
- mononeuropathy multiplex: several nerves, no pattern
- polyneuropathy: many nerves (distal + symmetrical)
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What are the primary causes of syncope?
- dehydration
- missed meals
- extended standing
- vasovagal response to stimuli: surprise, pain
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What are the secondary causes of syncope?
- hypoglycaemia
- dehydration
- anaemia
- infection
- anaphylaxis
- arrhythmia
187
What investigations are done for syncope?
- ECG/24 hr ECG
- echocardiogram
- bloods: FBC, electrolytes, blood glucose
188
What is an acoustic neuroma?
- tumours of Schwann cells on auditory nerve
- occur along cerebellopontine angle
- symptoms: hearing loss, tinnitus, balance problems
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What is papilloedema?
- swelling of optic disc 2º to raised ICP
- blurring of optic disc margin
- Elevated optic disc
- Engorged retinal veins
- Creases in the retina and haemorrhages around optic disc
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What are red flag features of headaches?
- Constant
- Nocturnal
- Worse on waking
- Worse on coughing, straining or bending forward
- Vomiting
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What are symptoms of a brain tumour?
- raised ICP
- Cushing's triad
- focal neurology
- epileptic seizures
- lethargy + weight loss
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What is the investigation for suspected brain tumour?
- MRI
- biopsy
- NO LP > contraindicated if high ICP
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What are some types of primary brain tumour?
- astrocytoma
- oligodendrocytoma
- meningioma
- Schwannoma
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What is Lambert Eaton syndrome?
- autoimmunity against presynaptic Ca channels in NMJ
- symptoms improve with exertion
- associated with small cell lung carcinoma
- symptoms start with extremities
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What is the presentation of Lambert Eaton syndrome?
- proximal muscle weakness
- diplopia
- ptosis
- dysphagia
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What is the treatment of Lambert Eaton syndrome?
- Amifampridine
- immunosuppressants: prednisolone
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How can you differentiate between UMN and LMN lesions?
- forehead spared in UMN
- ask patient to raise eyebrows
- if can move both sides then UMN lesion
- if one side affected then LMN lesion
198
What is the presentation of a radial nerve palsy and what are the nerve roots?
- roots C5-T1
- wrist drop
- innervates extensor muscles
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What is the presentation of a ulnar nerve palsy and what are the nerve roots?
- roots C8 + T1
- claw hand (4th and 5th fingers)
200
What are differential diagnoses for epilepsy?
- syncope
- cardiac arrhythmia
- panic attack
- non-epileptic + dissociative seizures
- TIA
- migraine
201
What is Brown-Sequard syndrome?
- hemisection of the spinal cord
- involves anterolateral system and DCML pathway
- symptoms occur below the lesion
202
What are the symptoms of Brown-Sequard?
- contralateral loss of pain and temperature
- ipsilateral loss of proprioception and vibration
- ipsilateral UMN weakness below lesion
203
Describe GCS?
- Eyes, verbal response, motor response
- max score is 15, min is 3
- 8/15 or below: secure airway
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What is Cushing's triad?
- bradycardia
- irregular respiration
- widened pulse pressure
205
What are migraine triggers? (CHOCOLATE)
Chocolate
Hangovers
Orgasms
Cheese
Caffeine
Oral contraceptive pill
Lie-ins
Alcohol
Travel
Exercise
