Neurology Flashcards

1
Q

Which body parts fall into the territory of the anterior cerebral artery in the sensory and motor cortices?

A

Trunk, legs, feet

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2
Q

Which body parts fall into the territory of the middle cerebral artery in the sensory and motor cortices?

A

Arms, head and neck

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3
Q

What fasciculi are within the dorsal column? Which is medial and which is lateral?

A
Medial = Gracile fasciculus
Lateral = Cuneate fasciculus
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4
Q

What sensory/motor role does the dorsal column have?

A

Fine touch + proprioception (sensory)

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5
Q

Where does the sensory input for the gracile fasciculus of the dorsal column come from?

A

Lower limbs

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6
Q

Where does the sensory input for the cuneate fasciculus of the dorsal column come from?

A

Upper limb

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7
Q

What are the 3 neurones involved in the dorsal column tract?

A

Dorsal root ganglion - gracile/cuneate nucleus (medulla)
Gracile/cuneate nucleus - ventral posterolateral thalamus
ventral posterolateral thalamus - post-central gyrus

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8
Q

Describe the path of the first neurone in the dorsal column tract

A

Sensory neurones in limbs and dorsal root gangion outside spinal cord
Enters gracile and cuneate fasciculi in spinal cord
Goes up to ipsilateral gracile and cuneate nuclei in medulla oblongata

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9
Q

Describe the path of the second neurone in the dorsal column tract

A

Gracilie/cuneate nuclei - decussates in pyramids
Travels through medial lemniscus of the pons
Reaches ventral posterolateral nucleus of thalamus

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10
Q

Describe the path of the third neurone in the dorsal column tract

A

Ventral posterolateral nucleus of thalamus to post-central gyrus in brain (sensory cortex)

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11
Q

What role does the spinothalamic tract have?

A

Pain, temperature and crude touch (sensory)

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12
Q

Where are the 3 neurons in the spinothalamic tract?

A

Sensory neuron/dorsal root gangion - dorsal horn of grey matter
Dorsal horn - contralateral thalamus
Thalamus - post-central gyrus

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13
Q

Describe the path of the 1st neuron of the spinothalamic tract

A

Sensory neuron/dorsal root ganglion
Enters spinal cord through Lissauer’s fasciculus
Ascends in spinal cord by 1 or 2 segments
Synapses in dorsal horns of grey matter

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14
Q

Describe the path of the 2nd neuron of the spinothalamic tract

A

Dorsal horn of grey matter - decussates in Anterior White Commissure at same level
Ascends through spinal cord and brain stem
Synapses at thalamus (contralateral to dorsal horn)

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15
Q

Describe path of 3rd neuron of spinothalamic tract

A

Thalamus to post-central gyrus

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16
Q

What is the role of the corticospinal tract?

A

Axial and limb motor

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17
Q

What is the role of the lateral corticospinal tract?

A

Limb motor

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18
Q

What is the role of the anterior corticospinal tract?

A

Axial motor

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19
Q

Describe the path of the lateral corticospinal tract

A

Pre-central gyrus (motor cortex) - internal capsule - brainstem
75-90% corticospinal tract decussate at pyramids (lateral corticospinal)
Descend through spinal cord and synapse at anterior horn cell
Lower motor neuron from anterior horn cell to limb muscles

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20
Q

Describe the path of the anterior corticospinal tract

A

Pre-central gyrus (motor cortex) - internal capsule - brainstem
10-25% do not decussate at pyramids (anterior corticoospinal)
Descend to level in spinal cord where decussates through anterior white commissure
Synapses at contralateral anterior horn cell
Lower motor neuron from anterior horn cell to axial muscles

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21
Q

Give 4 nerve endings for fine touch

A

Pacinian corpuscle
Meisner’s corpuscle
Ruffini endings
Merkel endings

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22
Q

What 3 tests do you do for a mini neurological exam?

A

General appearance + vital signs
GCS
Lateralising signs
Pupil reflexes

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23
Q

What are the 3 domains of the Glasgow Coma Scale?

A

Best Motor Response
Best Verbal Response
Best Eye-opening Response

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24
Q

Which of the 3 domains of the Glasgow Coma Scale is the best prognostically?

A

Best Motor Response

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25
What might you do if patient is unconscious to assess them?
Collateral history Mini Neurological Exam GCS Pupils
26
What are the criteria for the scores on BMR of GCS?
``` 6 = obeys commands 5 = Localises to pain 4 = Flexes to pain 3 = spastic flexion/withdrawal (don't use) 2 = Extends to pain 1 = None ```
27
What do lateralising signs tend to indicate? Give examples of lateralising signs
One hemisphere lesion | E.g. gaze paresis, inattention to one side, upper limb drift, asymmetrical flexion/extension
28
What causes a fixed dilated pupil?
Compression of parasympathetic fibres on outside of CN3, usually over petrous temporal bone
29
What shape does a subdural bleed show on imaging? Why?
Crescent-shaped | Low pressure venous blood causes slow bleed
30
What shape does an extradural bleed show on imaging? Why?
Convex shaped | Needs high pressure arterial blood to peel periosteal dura off cranium
31
What does a subarachnoid haemorrhage present with?
Sudden onset, severe headache - pressure on meninges Nausea, vomiting, stiff neck, photophobia, restless, agitation May have seizures
32
What symptom might an occipital lobe lesion cause?
contralateral visual defect
33
What might occur with dominant parietal lobe syndrome?
Confusion Difficulty writing and reading (agraphia) Difficulty with mathematics (acalculia) Visuospatial impairment - 3D (agnosia) Apraxia - inability to comprehend verbal commands and motor impairment Contralateral sensory impairment
34
Where is Broca's area? What is its role?
Dominant hemisphere frontal lobe (usually left) | Speech production
35
Where is Wernicke's area? What is its role?
Dominant hemisphere temporal lobe (usually left) Surrounds auditory cortex, close to lateral fissure Comprehension of speech
36
What might frontal lobe lesions present with?
Bilateral lesion = personality | Unilateral lesion = hemiparesis
37
What might temporal lobe lesions present with?
Memory loss - verbal memory loss if dominant hemisphere, non-verbal if non-dominant
38
What is the general role of the basal ganglia? How might problems present?
General role = control of movement and reward Too much control = rigidity Too little = chorea
39
What is the most likely cause of sudden onset hemiparesis? Where do most of these originate?
Stroke | More are ischaemic - embolic from carotid bifurcation (75%) or from heart (25%)
40
What would occur if a middle cerebellar artery stroke happened? What happens instead?
MCA stroke = unconscious | Most strokes in this territory are perforator artery emboli affecting internal capsule
41
Where is the lesion in myelopathy?
Spinal cord
42
What are the features of the A fibres in peripheral nerves?
Long, fast, myelinated | Role: proprioception, vibration, fine touch
43
What are the features of the C fibres in peripheral nerves?
Short, slow, non-myelinated | Role: pain, crude touch, temperature
44
Which nerve fibres are affected first in diabetic peripheral neuropathy? What test of sensation is most sensitive for peripheral neuropathy?
A fibres - long fibres affected first | Therefore - VIBRATION test - picks up neuropathy before symptoms/numbness appears
45
What is the most common cause of spinal cord lesions?
Disc osteophytes from arthritis
46
Give a rhyme for proximal muscle weakness. What are the presenting features?
Can't do hair, stair, chair Weak grasp, handwriting and flapping gait - finer movements lost first Fatigue
47
Where is the lesion in a radiculopathy?
Nerve root - initial segment of a nerve leaving the spinal cord
48
Where is the most common cervical radiculopathy? Give the dermatome and myotome distributions of these nerve roots.
C6 and C7 C6 dermatome = thumb, myotome = biceps C7 dermatome = middle and index finger, myotome = triceps
49
Where are the most common lumbar radiculopathies? What are the dermatomes and myotomes affected for these nerve roots?
L5 and S1 L5 dermatome = big toe, dorsal foot Myotome = dorsiflexion of foot (stand on heels) S1 dermatome = ltitle toe, lateral and sole of foot Myotome = plantar flexion foot
50
What features might suggest cerebellar lesion?
IPSILATERAL dysdiadochokinesia, ataxia, nystagmus, intention tremor, slurred speech, hyperreflexia (drunk)
51
Where are the most common sites for compression if these cranial nerves are affected together? a) CNIII, IV, VI b) CNVII, VIII c) CNIX, X, XI, XII
a) cavernous sinus b) petrous part of temporal bone c) jugular foramen
52
What are features of an intracranial mass?
epilepsy focal neurological signs raised intracranial pressure - headache, dizziness, nausea, vomiting, blurred vision
53
What is the commonest cause of proximal myopathy? How might you test for proximal myopathy clinically?
Corticosteroid use e.g. transplant, chemotherapy, autoimmune | Stand on one leg and squat
54
If there is a lesion at a disc level, which nerve root will be affected?
Nerve root below | e.g. disc lesion at C6/C7 - present with C7 radiculopathy
55
What are other ways of saying "long tract signs"?
UMN, pyramidal, descending tract, corticospinal tract signs
56
Give some examples of long tract signs
``` spastic gait hypertonia, hyperreflexia clonus + babinski cross-adductors Hoffman's sign loss of fine finger movements Deltopectoral reflex ```
57
What frequency is vibration sensation measured at?
128Hz
58
Where should pain stimulus in Best Motor and Best Eye response for GCS be done?
to CN distribution so not to elicit spinal reflex | e.g. behind ear and mastoid bilaterally
59
What is the formula for cerebral perfusion pressure? What should cerebral perfusion pressure be?
CPP = mean arterial pressure - intracranial pressure | Should be 60-70mmHg
60
Describe the relationship between intracranial volume and intracranial pressure
Little increase in ICP with increasing volume until decompensates Then exponential large increase in ICP with small increase in volume
61
How long do the majority of seizures last for?
1-2 minutes
62
Give the management of a seizure
Time it! Insert nasopharyngeal airway and oxygen mask, monitor O2 sats If >10 minutes - IV midazolam Loading dose: IV phenytoin, valproate, levotiracetam 15 min infusion with cardiac monitoring
63
Give post-seizure management
U+Es Possibly CT head Beware of peri-ictal aspirations and risk of LRTI
64
What drug can you use for acute management of raised ICP?
Mannitol - 100ml 10% or 50ml 20%
65
When might hydrocortisone cover be given?
If on long-term corticosteroids, cover with hydrocortisone to prevent adrenal crisis
66
When might dexamethasone be given? What cautions need to be taken?
Given peri-operatively for tumour neurosurgery Discontinue after 2 weeks (avoid Cushings) Monitor blood glucose - risk hyperglycaemia Beware of steroid psychosis
67
What risks do anticoagulants and anti-platelets have in neurology? How would you manage these?
Increased risk intracranial haemorrhage and chronic subdural bleeds If pathology found, stop meds, and reverse acutely Discuss with haematologist and only re-start if agreed by neurosurgeon
68
Give the pathophysiology of cranial diabetes insipidus. Give management
Decreased ADH secretion - can be feature of hypopituitarism Excessive diuresis, thirst and hypernatraemia Give diuresis post-surgery Fluid-balance chart, drink to thirst, consider desmopressin
69
Why might hyponatraemia occur in neurology?
Water moves into plasma from cells e.g. cerebral oedema, raised ICP Brain injury releases natriuretic peptides causing "cerebral salt wasting" SIADH
70
What are the effects of hyponatraemia?
potentially life-threatening >48hrs or severe Seizures, coma, cardio-respiratory arrest Chronic: falls, gait problems, concentration and cognitive decline, osteoporosis
71
How would you manage hyponatraemia?
IV crystalloid and consider mineralocorticoid If SIADH, either fluid restriction OR sodium replacement Careful - Too rapid Na replacement risks central pontine myelinosis
72
How might you manage neuropathic pain?
Trial and error with neurotropics Gabapentin + pre-gabalin for radicular pain Carbamazepine - trigeminal neuralgia Topirimate - chronic refractive head pain
73
Give the total capacity of the CSF and of the ventricles
CSF total = 120ml | Ventricles = 20ml
74
Give 3 functions of CSF
Protection (cushion) Buoyancy - reduces pressure on base of brain Environment for brain function and neurotransmission e.g. low K
75
What lines the ventricles and what produces CSF?
Ependymal cells | Choroid plexus produces CSF
76
What foramen connects 1st and 2nd ventricles to the 3rd?
Foramen of Monro
77
Where is the 3rd ventricle and what connects it to the 4th ventricle?
Between left and right thalamus | Connects to 4th via CEREBRAL AQUEDUCT
78
Where is the 4th ventricle and how does it drain to spinal canal and subarachnoid cisterns?
4th ventricle between pons and medulla | Drains laterally via Foramina of Luschka and foramen of Magendie medially
79
Give some presenting features of myelopathy
Back pain Long tract signs - UMN signs Loss of fine finger movements Radiating limb pain
80
Give features of vertebral syndrome
Lower back pain - dull, aching | Radiating pain not as far as radiculopathy
81
Give features of radiculopathy
Sensory loss/pain in dermatome AND/OR motor loss in myotome | Possible back pain
82
What difference in presentation is there between L5 radiculopathy and common peroneal nerve palsy?
L5 radiculopathy - weakness on foot inversion | common peroneal nerve palsy - weakness on foot eversion
83
Give the features of Brown-Sequard syndrome
Lesion in one half of spinal cord Ipsilateral UMN weakness below lesion Ipsilateral loss of fine touch, proprioception and vibration below lesion Contralateral loss of pain, temp and crude touch 1-2 segments below lesion
84
What is paracellar syndrome and what features present?
Pituitary tumour or mass in pituitary cellar Upward pressure on optic chiasm - bitemporal hemianopia Hypopituitarism
85
What are the 2 types and relative prevalence of strokes?
Ischaemic (85%) Haemorrhagic (15%) (also can be due to systemic hypoperfusion)
86
What is the WHO definition of a stroke
Clinical syndrome consisting of RAPIDLY developing clinical signs of focal (or global in coma) disturbance of cerebral function lasting MORE THAN 24 HRS or leading to death with no apparent cause other than a VASCULAR origin
87
Given common symptoms of an anterior/carotid circulation stroke
Weakness (face, legs, arms) Impaired speech/language Amaurosis fugax
88
Give common symptoms of a posterior circulation stroke
``` Dysarthria Dysphagia Diplopia Dizziness Ataxia Diplegia ```
89
Give the 3 types of ischaemic stroke
Embolic Thrombotic Systemic hypoperfusion
90
What does the acute CT scan show after a stroke? Why might a repeat CT be done after 48hrs?
Acute - bleeds/haemorrhagic stroke | Ischaemia appears normal, but after 48hrs may see dark patches
91
What arteries supply anterior circulation?
Internal carotid arteries - ACA and MCA
92
What arteries supply posterior circulation?
Posterior cerebral artery and vertebral arteries
93
What arteries connect anterior and posterior circulations?
Posterior communicating arteries
94
What criteria defines a total anterior circulation infarct?
Contralateral weakness/sensory loss Homonymous Hemianopia/visual inattention Higher cortical dysfunction - dysarthria, dysphasia
95
What criteria defines a partial anterior circulation infarct?
2 of the following: Contralateral weakness/sensory loss Homonymous hemianopia/visual inattention Higher cortical dysfunction - dysarthria, dysphasia
96
What are the presenting features of a posterior circulation infarct?
``` Cranial nerve palsy Contralateral sensory/motor deficit (pyramidal lesion) Cerebellar dysfunction Isolated homonymous hemianopia Bilateral events - reduced GCS ```
97
What is a lacunar infarct?
= occlusion of deep penetrating arteries
98
Why does a lacunar infarct not present with higher cortical features?
Only affect small volumes of SUB-cortical white matter so does not present with cortical features e.g. dysarthria, dysphasia, visual field loss
99
What syndromes are common in lacunar infarcts?
``` pure motor hemiparesis Ataxic hemiparesis "clumsy hand" + dysarthria pure hemisensory loss Mixed sensorimotor ```
100
Why do small lacunar infarctions cause large clinical syndromes?
White matter infarcts - nerve fibres narrowed down so small area has large clinical consequences
101
What is the acute management of a stroke?
ABCDE + bloods (including BM!) Hx: TIME of onset, risk factors, contraindications to thrombolysis, blood pressure, NIHSS Urgent CT head (no contrast within 1h) BEFORE thrombolysis/aspirin Thrombolysis/thrombectomy OR Aspirin 300mg
102
What might be the next stage and long-term management of a stroke?
Investigate cause (full history, exam, investigations) Screen and prevent complications (infection, sores) Secondary prevention (lifestyle, meds, surgery) Rehabilitation (PT/OT/SLT)
103
What is the NIHSS?
National Institute of Health Stroke Scale Score 0-42 Grades severity of stroke and can use to monitor response to acute treatment
104
How might ischaemic regions appear on an acute CT?
May not be visible Subtle blurring and decreased attenuation of grey-white junction due to oedema may be early sign May see hyperdense vessel - sign of intravascular clots e.g. MCA
105
What type of infarcts are MRIs better at viewing?
Posterior circulation infarcts | Lacunar infarcts
106
Give the indications for thrombolysis post-stroke
Within 4.5 hours of symptom onset | Non-haemorrhagic stroke on CT
107
Give some contra-indications for thrombolysis
``` Haemorrhagic stroke/bleed current or previous anti-coagulation/coagulopathy pregnancy high blood pressure Aneurysm Severe liver disease, varices, portal HTN Seizures at presentation Hypo/erglycaemia Intracranial neoplasm history Rapidly improving symptoms ```
108
What is given for thrombolysis? How should you follow up this treatment?
IV Tissue Plasminogen Activator (ALTEPLASE 0.9mg/kg) Monitor blood pressure and complications of bleeding 24hr CT to check for haemorrhagic transformation
109
Give the indications and methods for thrombectomy
Indications - within 6 hours of symptom onset in anterior circulation, later if basilar thrombosis. Methods - CT angiography to remove clot, can be used alongside thrombolysis
110
What are the 2 parts of ischaemic cerebral tissue called and which is more likely to survive when blood supply returns?
``` Ischaemic core (central) surrounded by Ischaemic Penumbra (outer) Core dies 1st, but penumbra may live if blood supply returned The smaller the core, the better ```
111
What investigations might you do to investigate the cause of the stroke?
Bloods: FBC, UE, ESR, Lipids, LFTs, CRP, clotting, glucose/Hba1c ECG + 72 hour tape (paroxysmal AF) Carotid Doppler USS (carotid stenosis) Echo (endocarditis/valvular disease) MRI (confirm diagnosis, infarcts) or delayed CT if not tolerated
112
What appears white in a a) T1 weighted image? b) T2 weighted image?
a) fat | b) water
113
What is the best type of MRI for detecting acute infarcts?
Diffusion-weighted MRI - infarct appears WHITE
114
What type of MRI is matched with DW MRIs to detect infarcts?
ADC - infarct appears BLACK Match black lesion to white lesion on DW MRI DWI-ADC match
115
Give examples of MDT management post-stroke
nursing | PT, OT, SLT, dietitian, orthoptics
116
What lifestyle advice might you give someone after a stroke?
``` smoking cessation limit alcohol and drug use dietary modifications exercise driving advice ```
117
What medications might you give as secondary prevention for strokes?
Anti-platelets - Aspirin 300mg for 2 weeks, then Clopidogrel lifelong Anticoagulation: if have AF (HASBLED vs CHADSVASC score to determine this) Anti-hypertensives: if haemorrhagic, be careful if ischaemic - aim <130/80 Statins - aim 40% decrease in non HDL cholesterol
118
How might you surgically manage extracranial carotid stenosis?
If 70-99% occluded - carotid endarterectomy | If 50-69% occluded - consider CEA or carotid artery stenting
119
How could you manage malignant MCA syndrome?
Decompressive hemicraniotomy
120
How could you manage posterior circulation infarct surgically?
External ventricular drainage or Posterior fossa decompression to prevent risk of hydrocephalus
121
What are the different methods of management for haemorrhagic stroke?
Manage hydrocephalus Reversal of anticoagulation Blood pressure control
122
What are the indications for medical and surgical management of haemorrhagic stroke?
Small deep haemorrhages, lobar haemorrhage without hydrocephalus, rapid neurological deterioration, large haemorrhage and significant comorbidities before stroke, GCS <8, posterior fossa haemorrhage
123
What are the indications for reversal of anticoagulation in haemorrhagic stroke management?
Primary intracerebral haemorrhage and taking anticoagulants with elevated INR
124
What are the indications for rapid blood pressure lowering in acute management of haemorrhagic stroke?
Within 6 hours symptom onset systolic BP 150-220 Consider if >6hrs or BP>220 Aim systolic 130-140 within 1h and maintain for 7 days
125
What are the contraindications to rapid blood pressure lowering in management of haemorrhagic strokes?
Underlying structural cause GCS<6 Awaiting neurosurgery for haematoma Massive haematoma with poor prognosis
126
What medications can be used for reversal of anticoagulation in haemorrhagic stroke management?
Warfarin - Beriplex and vit K LMWH - protamine DOACs - Beriplex partial Dabigatran - Idaruciumab
127
What causes can mimic stroke presentations?
``` Seizures Tumours/abscess Migraine Metabolic Functional Myelopathy, peripheral neuropathy, cranial nerve ```
128
What might you want to determine about the distribution and pattern of muscle weakness and why?
Proximal (muscle) or distal (nerve) Symmetrical (genetic/metabolic) or asymmetrical Mono or poly Cranial involvement (bulbar/ophthalmoplegia) Variability (fatigueability/relapse-remission) Sensory deficit?
129
Give clinical features of Motor neurone disease and exclusion criteria
UMN (+LMN) No sensory deficit or sphincter disturbance Never affects eye movements
130
What are the 4 types of motor neuron disease? Which is the most common?
Most common = amyotrophic lateral sclerosis (ALS) Progressive bulbar palsy Progressive muscular atrophy Primary lateral sclerosis (rare)
131
What are the pathophysiological features of MND?
Loss of motor neurones in cortex, cranial nerve nuclei and anterior horn cells
132
Where are the loss of motor neurones in ALS and what symptoms appear?
Loss motor neurons in motor cortex AND anterior horn cell UMN+LMN signs Worse prognosis if bulbar onset, older age, reduced FVC
133
Where are the loss of neurons in progressive bulbar palsy?
Cranial nerves 9-12 | UMN (corticobulbar) and LMN (bulbar)
134
Where are the loss of neurons in progressive muscular atrophy? What type of signs do you see?
Anterior horn cell loss | LMN signs only
135
Where is loss of neurons in primary lateral sclerosis and what signs do you get?
Loss of Betz cells in motor cortex Mainly UMN Pseudobulbar palsy Spastic leg weakness
136
What is the classical presentation of someone with MND?
``` Usually >40 (median 60yrs) Stumbling spastic gait Footdrop +/- proximal myopathy Weak grip/shoulder abduction Aspiration pneumonia UMN+LMN, potential bulbar signs Frontotemporal dementia in 25% ```
137
How is MND diagnosed?
By EXCLUSION Brain/cord MRI to exclude structural LP to exclude inflammatory Nerve conduction studies - detect loss of motor neurones and exclude mimicking motor neuropathies
138
What is the prognosis of MND?
Poor, <3 years in 50% patients
139
How would you manage MND?
MDT approach Riluzole - NMDA receptor antagonist, improves survival slightly Supportive
140
What supportive measures might you need to do for someone with MND?
excess saliva - positioning, suction, oral care, anti-muscarinics, botulinum toxin A dysphagia - blend food, gastrostomy Spasticity - baclofen, exercise, orthotics communication - SLT, alternative methods palliative care - from diagnosis! ventilation - if wanted, only increases prognosis few months
141
Give 3 branches of peripheral nerve disease
Polyneuropathy Mononeuropathy Mononeuritis multiplex
142
Give some causes of polyneuropathy
``` Peripheral neuropathy Guillain-Barre Syndrome Lead poisoning Charcot-Marie tooth Leprosy ```
143
Give features of peripheral neuropathy
Chronic and progressive Starts in longer nerves (vibration, proprioception sense) Starts in legs first, "glove and stocking" Sensory/LMN/both signs - numbness, pain, difficulty handling small objects/fine motor loss Trophic changes - hair loss, shiny skin, calluses, nail changes, cold, pallor
144
What are the common causes of peripheral neuropathy?
``` Commonest = DIABETES 20% = idiopathic ```
145
What are some less common causes of peripheral neuropathy?
``` B12 or folate deficiency Alcohol/toxins/drugs hereditary neuropathies paraneoplastic syndromes metabolic - uraemia, thyroid ```
146
How would you manage peripheral neuropathy?
Treat cause - e.g. diabetes, deficiencies, alcohol XS PT + OT - orthotics Splinting joints to prevent contractures in prolonged paralysis
147
Give features of Guillain-Barre syndrome
Subacute <6 weeks Ascending paralysis/numbness/areflexia Sensory loss and LMN signs Post-infectious autoimmune response
148
What investigations would you do for Guillain-Barre syndrome and what would you see?
NCS - demyelination | LP - raised protein, normal or decreased WCC (cyto-albuminologic dissociation)
149
How would you treat Guillain-Barre syndrome?
IV Ig Supportive measures Monitor FVC
150
Give some features of Charcot-Marie tooth disease
Hereditary motor and sensory neuropathy (HMSN) Muscle weakness/sensory loss in feet, legs and hands Abnormal gait High arched or very flat feet Usually presents age 5-15 Managed: supportive, OT/PT, walking aids
151
Describe the distribution and presentation of mononeuritis multiplex. How might you localise the site of lesions?
Random distribution of mononeuropathies (2 or more) Subacute presentation <6 weeks Electromyography
152
Give the causes of mononeuritis multiplex
vasculitides Connective tissue disorders e.g. SLE, RA, sarcoidosis Leprosy
153
What is the commonest mononeuropathy?
Carpal tunnel - median neuropathy
154
What other common mononeuropathies occur in the upper limb?
Ulnar neuropathy - compression at elbow | Radial neuropathy - compression in axilla
155
What is the are the comonest mononeuropathies of the leg?
Common peroneal neuropathy - fibular head | Lateral cutaneous thigh neuropathy - anterolateral burning thigh pain - inguinal ligament
156
What are the signs of common peroneal nerve palsy?
Footdrop Dorsal foot sensory loss Weak ankle dorsiflexion and EVERSION
157
Give the pathophysiology of myasthenia gravis
Autoantibodies to nicotinic post-synaptic acetylcholine receptors at neuromuscular junction - both B and T cells implicated
158
Where does spasticity tend to develop when there is UMN/pyramidal weakness?
In stronger muscle groups arms = flexors legs = extensors
159
Describe spasticity
Increased tone Velocity-dependent - faster movement = greater resistance, until gives way Clasp-knife response
160
Describe spasticity
Increased tone Velocity-dependent - faster movement = greater resistance, until gives way Clasp-knife response
161
Which groups are more likely to have myasthenia gravis?
<50 = women (thymic hyperplasia), >50 = men (thymic atrophy or thymoma) Autoimmune conditions
162
Give presenting features of myasthenia gravis
Slowly progressive or relapsing muscular fatigue. Fatigue increases on repetition of movement of VOLUNTARY muscles Order of muscles affected: extraocular, bulbar, face, neck, limb girdle, trunk Ptosis, diplopia, "peek sign" - orbicularis fatiguability Myasthenic snarl on smiling Voice fades when counting to 50 - dysphonia Normal reflexes
163
What can exacerbate myasthenia gravis symptoms?
``` pregnancy hypokalaemia infection over-treatment change of climate emotion exercise gentamicin or tetracyclines or quinine or beta blockers opiates ```
164
What investigations could you do in someone presenting with myasthenic features?
Anti-AchR antibodies (90%) or MuSK Abs Electromyography - decreasing muscle response to repeated nerve stimulation CT thorax to exclude thymoma Ice on eyelid test (not diagnostic) Tensilon test - rare (inject edrophonium)
165
How would you treat myasthenia gravis?
Pyridostigmine (Achesterase inhibitor) for symptoms Prednisolone + osteoporosis prophylaxis OR azathioprine/methotrexate/mycophenolate Thymectomy (with or without thymoma can be helpful)
166
What is myasthenic crisis?
Life-threatening weakness of respiratory muscles | Can be caused by infection, natural disease or overdosing cholinergic meds (pyridostigmine)
167
How would you manage a myasthenic crisis?
Monitor FVC, ventilatory support if needed Plasmapheresis or IV Ig Treat cause Call anaesthetics and urgent neurology review
168
What is the pathophysiology of Lambert-Eaton myasthenic syndrome (LEMS)?
Paraneoplastic or autoimmune production of antibodies to Ca channels on pre-synaptic membrane of neuromuscular junction
169
What clinical features might you see in LEMS?
Gait difficulty before eye signs Autonomic involvement (dry mouth, constipation, impotence) Hyporeflexia and weakness, symptoms and EMG amplitude improve after exercise
170
What are common causes of proximal myopathy?
Steroids Statins Metabolic and endocrine Myotonic dystrophy
171
What are less common causes of proximal myopathy?
Duchenne's, Becker's, muscular dystrophies Inflammatory muscle disease (polymyositis, dermatomyositis) Mitochondrial disorders
172
What are features of proximal myopathy?
``` Gradual onset Preserved reflexes, no fasciculation Can't do hair, stairs, chairs Muscle wasting Facial and neck weakness (sometimes) Contractures (Scoliosis) (Eye movement disorders) ```
173
How would you investigate suspected proximal myopathy?
CK (Duchenne's),ESR, AST, LDH, CRP EMG (Muscle biopsy) (genetic testing)
174
How would you treat proximal myopathy?
Remove cause/medications Supportive (OT, PT, back, renal, diet) Immunosuppressants if inflammatory
175
Give some features of Duchenne's muscular dystrophy
Proximal myopathy, commonest of muscular dystrophies, X-linked recessive Presents 4 yrs old clumsy walking, difficulty standing and respiratory failure Pseudohypertorphy in calves Raised serum CK Supportive treatment and ventilatory support
176
Give some features of Becker's muscular dystrophy
Proximal myopathy, less common of muscular dystrophies, X-linked recessive Presents later and milder than Duchenne's Better prognosis
177
Give some features of Faciospaculohumeral muscular dystrophy (FSHD, Landouzy-Dejerine)
Proximal myopathy, common as Duchenne's, autosomal dominant Onset 12-14 Inability to puff out cheeks, or raise arms above head
178
What is a myotonic disorder? What is the commonest form and its 2 types?
= tonic muscle spasm, long chains central nuclei within muscle fibres Myotonic dystrophy - DM1 and DM2 DM1 more common and severe, both types autosomal dominant Mexiletine may help with disabling myotonia, genetic counselling
179
What is the MRC grading for power?
``` 5 = normal power 4 = active movement against gravity and resistance 3 = active movement against gravity 2 = active movement with gravity eliminated 1 = flicker movement/contraction 0 = no movement/contraction ```
180
What is subacute combined degeneration of spinal cord?
B12 deficiency | Affects dorsal column first - proprioception
181
Which way does someone tend to fall in an ataxic gait?
towards the side of the lesion
182
What are the 3 broad differentials for transient loss of consciousness (TLOC)?
1. syncope 2. epileptic seizure 3. non-epileptic (psychogenic)
183
What causes syncope as a whole category?
Transient global cerebral hypoperfusion
184
What are the 3 types of syncope?
Reflex Cardiogenic Orthostatic hypotension
185
What are the 3 types of reflex syncope?
vasovagal situational - micturition, exercise carotid sinus hypersensitivity
186
What causes vasovagal syncope? aka neurocardiogenic
Reflex bradycardia +/- peripheral vasodilation | Provoked by emotion, pain, standing too long
187
What are the typical features of vasovagal syncope?
Onset over seconds - not instant Prodromal symptoms (nausea, pallor, sweating, narrowing of vision) Brief clonic jerking possible, but no tonic clonic Duration - 2mins Rapid recovery (3Ps - precipitating, prodrome, posture)
188
What are the typical features of situation syncope?
``` Clear precipitant - cough, effort/exercise, micturition onset over seconds prodromal symptoms may jerk but not tonic-clonic Duration - 2 mins Rapid recovery ```
189
What are the typical features of carotid sinus hypersensitivity?
Baroreceptors in carotid sinus hypersensitive - reflex bradycardia + vasodilation on minimal stimulation e.g. head turning or shaving Same as vasovagal in features
190
What are the features of cardiogenic syncope?
Transient arrhythmias or structural stenosis causing tachycardia or bradycardia No warning except palpitations, falls to ground Pale, slow or absent pulse Recovery within seconds, patient flushes, pulse speeds up Anoxic clonic jerks may occur in prolonged LOC May happen several times a day and in any posture
191
What are some other causes of blackouts outside of syncope, epilepsy and pscychogenic causes? What features might you see?
Hypoglycaemia - tremor, hunger, light-headed Anxiety - hyperventilation, tremor, sweating, tachy, weakness, light-headed, no LOC Drop attacks - sudden drops without LOC (narcolepsy, catoalexy, acute hydrocephalus) Factitious blackouts - pseudoseizures, Munchausens
192
What is an epileptic seizure?
Clinical manifestation of abnormal electrical discharge in the brain
193
What is epilepsy?
Tendency to recurrent epileptic seizures
194
What are the features of an epileptic seizure?
Attacks when asleep or lying down Aura, (identifiable triggers?), sudden onset Altered breathing + cyanosis Typical tonic-clonic movements (symmetrical) Jerking changing frequency Urinary incontinence Tongue-biting - lateral Duration <3mins Prolonged post-ictal drowsiness/confusion - 15 mins Amnesia Transient focal paralysis (Todd's palsy)
195
Why is TIA not a differential for TLOC?
TIA is a focal hypoperfusion whereas TLOC indicates GLOBAL hypoperfusion!
196
Give the features of a non-epileptic seizure
``` May be trigger e.g. anxious, stress No prodrome or sparingly described Jerking not synchronous, random, constant frequency Rhythmic pelvic movements, head side to side Eyes often close May bite tongue but often tip of tongue Duration >3 mins (longer than GES) May feel tired after ```
197
What is your first investigation following a TLOC?
ECG - rule out cardiac causes e.g. heart block, arrhythmias, long QT May do ambulatory ECG/24 hr ECG to see transient arrhythmia
198
What other investigations might you do after ECG for TLOC?
``` U+E, FBC, Mg, Ca, glucose Tilt-table test (exclude cardiac cause first) Echocardiogram EEG/sleep EEG CT/MRI ```
199
What are the 3 classifications of focal seizures?
Simple - no LOC complex - impaired consciousness, often temporal Starts partial then evolves to bilateral generalised convulsive seizure
200
What are the different types of generalised seizures?
``` Absence seizures Tonic-clonic Myoclonic Atonic - sudden loss muscle tone, no LOC Infantile spasms ```
201
Give some features of temporal lobe focal seizures
Impaired awareness + complex motor e.g. lip smacking, chewing, swallowing, manual movements Dysphasia, deja vu, jamais vu Emotional disturbance Olfactory, gustatory, auditory hallucinations, delusions Post-ictal confusion
202
Give some features of frontal lobe focal seizures
``` Posturing or peddling movements of legs Focal motor seizure starting with thumb/face (Jacksonian march) Motor arrest Subtle behavioural disturbances Dysphasia or speech arrest Post-ictal Todd's palsy ```
203
Give some features of parietal lobe focal seizures
Sensory disturbances - tingling, numbness, pain (rare) | Motor symptoms if spreads to pre-central gyrus
204
Give some features of occipital lobe focal seizures
Visual phenomena - spots, line, flashes
205
What is amaurosis fugax?
Occlusion of retinal artery causing unilateral progressive vision loss "like a curtain descending"
206
What can cause TIAs?
Carotid emboli Cardiac emboli Hyperviscosity - polycythaemia, SCD, myeloma Vasculitis - rare non-embolic cause
207
How would you manage a TIA?
Control CVD RFs - antihypertensives, statins, diabetic medication, smoking cessation Antiplatelets - aspirin 300mg 2 wks, then clopidogrel 75mg OD Anticoagulation if cardiac emboli Carotid endarterectomy in 2wks if 1st TIA and can tolerate (preferred to carotid angioplasty with stent) Advise not to drive for 1 month
208
What tool gives the risk for a stroke following a TIA?
ABCD2 score
209
What is a TIA?
= transient ischaemic attack | ischaemic/embolic neurological event with symptoms lasting less than 24 hours
210
What factors are considered on ABCD2 score?
``` Age >60 Blood pressure >140/90 Clinical: unilateral weakness, speech disturbance without weakness Duration: symptoms 10-59 mins or >1h Diabetes ```
211
What is a high risk score on the ABCD2?
4+ = high risk, see specialist within 24h
212
What are the typical features of meningitis?
``` Rapid onset headache Fever Photophobia Stiff neck Purpuric rash Coma ```
213
What are the typical features of encephalitis?
Rapid onset headache Fever Odd behaviour Fits or reduced consciousness
214
Give the differentials for a rapid onset headache
``` Subarachnoid haemorrhage Meningitis Encephalitis Post-coital headache Cluster headaches ```
215
Give the features of venous sinus thrombosis
subacute, gradual onset headache | papilloedema
216
Give the features of sinusitis
Dull, constant headache over frontal or maxillary sinuses with tenderness Ethmoid or sphenoid pain is deep in midline at nose root, common with coryza Postnasal drip Pain worse on bending over Lasts 1-2 weeks
217
Give the features of intracranial hypotension
CSF leakage e.g. iatrogenic after LP, epidural | Headaches worse on standing
218
How would you manage intracranial hypotension due to CSF leak post-LP?
Epidural blood patch over leak | Conservative management with IV fluids and caffeine fails
219
Give the features of a tension headache
Tight band Bilateral Non-pulsatile Scalp muscle tenderness
220
Give the features of migraines
Prodrome - precedes by days (mood changes, tired) Visual or other aura lasting 15-30 mins, followed by (1h unilateral, throbbing headache) Nausea, vomiting, photophobia, phonophobia Allodynia
221
Give some examples of visual aura experience in migraines
``` Chaotic distorting Melting and jumbling of lines Dots Zigzags Scotomata (blind spots) Hemianopia ```
222
What aura might you get with migraines other than visual
Paraesthesiae spreading from fingers to face Dysarthria, ataxia, ophthalmoplegia, hemiparesis Dysphasia or paraphasia
223
What are some triggers of migraines?
CHOCOLATE C = chocolate, H = hangovers, O = orgasms, C = cheese/caffeine, O = oral contraceptive, L = lie-ins, A = alcohol, T = travel, E = exercise
224
What is the diagnostic criteria for migraines with no aura?
>5 headaches lasting 4-72hrs with nausea/vomiting/photo/phonophobia + 2 of unilateral, pulsating, impairs routine activity
225
How would you manage migraines?
``` Avoid triggers (consider progesterone only/non-hormonal contraceptives in young women) Prophylaxis = propanolol/topiramate/amitryptiline Attack = Oral triptan + NSAID/paracetamol, anti-emetic Other = warm or cold packs to head, rebreathing into paper bag, Butterbur extracts, riboflavin supplements, acupuncture, transcutaneous nerve stimulation ```
226
Give some contraindications and side effects of triptans
``` CI = IHD, coronary spasm, uncontrolled HTN, recent lithium, SSRI, ergot SE = arrhythmias, angina, MI ```
227
Give features of a headache due to raised ICP
chronic, progressive headache - worse on waking, lying, bending forward or coughing AS - vomiting, papilloedema, seizures, false localising signs, irritability
228
If suspect ICP, what investigation would you do first and why?
Imaging - CT/MRI FIRST before LP (risk herniation/coning) | If no abnormalities, consider idiopathic intracranial hypertension
229
What medications can cause medication overuse headache/analgesic rebound headache?
Combined analgesics (paracetamol + codeine/opiates) Ergotamine Triptans Episodic headache becomes chronic daily headache
230
What are the symptoms of cluster headaches?
Rapid onset excruciating pain around orbit unilaterally Eye watery and bloodshot, lid swelling Facial flushing, rhinorrhoea, miosis or ptosis Duration 15-180 mins, OD or BD, often nocturnal Clusters last 4-12 weeks, then pain-free periods of months-2 years
231
How would you treat cluster headaches?
Acute attack - 100% O2 for 15 mins via non-rebreathe mask + sumatriptan at onset Prophylaxis - avoid triggers. Consider corticosteroidds, verapamil, lithium
232
What are the symptoms of trigeminal neuralgia?
Intense unilateral stabbing pain lasting seconds in trigeminal nerve distribution Often mandibular or maxillary distributions Triggers - washing area, shaving, eating, talking, dental prostheses
233
How would you manage suspected trigeminal neuralgia?
MRI - to exclude secondary inflammatory or structural causes Carbamazepine, lamotrigine, phenytoin or gabapentin Microvascular decompression
234
What is the minimum GCS score and what is the maximum?
``` Min = 3 Max = 15 ```
235
Give some common causes of coma
``` Drugs//toxins/opiates/ethanol Anoxia Mass lesions e.g. bleeds, SAH Infections (HSE, meningitis Infarcts of brainstem Metabolic (DKA, wernicke's) Epilepsy ```
236
Give less common causes of coma
``` Tumour Carbon monoxide Venous sinus occlusions Hypothermia Psychiatric catatonia Pituitary apoplexy Fat embolism ```
237
How does blood appear on CT in sub/extradural haemorrhage initially and then after some time?
High density or white initially | Then becomes hypodense after time
238
How would you investigate for a non-haemorrhagic ass lesion?
CT with contrast - illuminates wall of mass
239
How would an MCA infarct appear on a CT? What criteria would make it a malignant MCA infarct?
Reduced white-grey matter differentiation and sulcal effacement if 2/3 artery territory infarcted = malignant
240
What would you expect to see on a lumbar puncture without culture in bacterial meningitis?
Raised protein, decreased glucose
241
What is the pathophysiology and signs for phenobarbital overdose?
GABA-A chloride channels open longer Cerebellar signs and diplopia Coma, respiratory depression, death
242
What are the signs of CO poisoning?
headache, nausea, confusion, syncope, convulsions, coma, death low CO levels can still kill if have IHD
243
What symptoms and signs can amphetamines and other stimulants cause?
seizures psychosis ischaemic stroke, intracranial haemorrhage angiitis (stroke, mononeuritis multiplex) rhabdomyolosis delirium, coma posterior reversible encephalopathy syndrome
244
What neurological events can occur shortly after ingesting cocaine?
Seizures, stereotypic movements (tic-like) Anxiety, confusion, paranoia, visual hallucinations Stroke - usually haemorrhagic
245
What are the symptoms of ethanol overdose?
ataxia nystagmus dysarthria coma
246
What are the symptoms of ethanol/alcohol withdrawal a) within 6-8hrs? b) within 24 hrs? c) within 3-5 days?
a) tremor, anxiety, nausea b) hallucinations (visual, olfactory, auditory) c) delirium tremens - hyperactivity (motor+sensory) and altered mental state
247
How would you treat alcohol withdrawal?
Thiamine, then glucose, multivitamins Benzodiazepine (Lorazepam), Chlordiazepoxide Fluids - check hydration, K and Mg Check clotting, monitor glucose
248
What is the main concern that chronic ethanol overuse can cause? What are features of this?
``` = Wernicke's encephalopathy Thiamine deficiency Ophthalmoparesis Ataxia Confusion 25% get Korsakoff's psychosis ```
249
What other conditions can chronic ethano overuse cause except for WE?
cerebellar degeneration - LL and trunk ataxia peripheral neuropathy amblyopia central pontine myelinosis (if hyponatraemia corrected too rapidly) dementia tremor
250
What is a sub-hyaloid haemorrhage?
Intra-ocular bleed between retina and posterior layer of vitreous
251
What features do you see in 3rd CN palsy?
Parasympathetic palsy = fixed dilated pupil, droopy eyelid | Sympathetic = Horner's syndrome, partial ptosis
252
What are the features of Horner's syndrome?
``` Loss of sympathetic oculomotor function - triad = Constricted pupil (miosis) Partial ptosis Hemifacial sweating (anhidrosis) Sometimes enophthalmos ```
253
What might you see on an LP after a sub-arachnoid haemorrhage? When should a CT be done?
CT most sensitive within 24 hrs | LP = CSF xanthochromia (yellow due to bilirubin) - most sensitie within 2 weeks
254
What are 2 common treatments for a sub-arachnoid haemorrhage?
1. surgical clipping | 2. Endovascular coiling
255
When would maximum weakness need to occur in acute paresis?
Within 4 weeks
256
What features may suggest a brainstem lesion for UMN weakness?
Cranial nerve involvement Horner's syndrome Internuclear ophthalmopegia
257
What features may suggest a spinal cord injury for UMN weakness?
Bilateral weakness Radicular symptoms may give level Sensory loss below level
258
What are some acute causes of weakness?
Vascular (haemorrhagic or embolic) Compression (haemorrhagic usually) Hypoglycaemia
259
What are some subacute causes of weakness?
``` UMN = mass lesion, subdural, inflammatory, infection, metabolic LMN = GBS, peripheral neuropathy, myopathy, MG, malignant meningitis ```
260
Why is it useful to check if weakness involves dorsal and spinothalamic tracts?
If it does, then lesion must be beyond arterial distribution because each tract has separate arterial blood supply
261
What is spinal shock?
Acute complete transverse lesion across spinal cord can cause spinal shock Flaccid weakness below lesion but no UMN signs
262
What are the features of spinal cord compression?
``` UMN deficit below lesion Sensory impairment below lesion Possible spinal shock if acute Lhermitte's phenomenon Painless atonic bladder Urgency Impotence Respiratory paralysis if high cervical lesion ```
263
What are the 4 types of cancer that can metastasise to the spine?
Lung Breast Prostate Kidney
264
What are some primary tumours that could be in the spine?
``` Neuroma Meningioma Glioma Sarcoma Ependymoma ```
265
What are some non-compressive causes of myelopathy?
Inflammation Infarction MND Syrinx (fluid-filled cavity in spinal cord) AVM, spinocerebellar syndromes, radiation, fluorosis, sarcoidosis
266
What supportive treatments might you do for GBS?
Thromboprophylaxis Dysautonomia - 2-4hrly BP, cardiac monitor Monitor FVC, RR Pain - opiates, amitryptiline Rehab - splints, pressure sores, nutrition
267
What types of immunotherapy can you give for GBS?
IV Ig Plasma exchange monoclonal antibodies??
268
What is status epilepticus?
When a seizure lasts longer than 5 minutes or when seizures occur close together with no recovery between them
269
How would you manage a sub-arachnoid haemorrhage?
Re-examine neuro often - BP, pupils, GCS Repeat CT if deteriorating Keep hydrated - maintain perfusion Nimodipine - prevent vasospasm, cerebral ischaemia Surgery - Endovascular coiling or surgical clipping
270
Give some primary and secondary causes of orthostatic hypotension
``` Primary = MSA, PoTS Secondary = diabetes, medication, Parkinsons ```
271
Give some potential triggers of epileptic seizures
sleep deprivation flashing lights menstruation alcohol/alcohol withdrawal
272
How useful are EEGs in diagnosing epilepsy?
Not very! Should be done after ECG, bloods, imaging Some may have abnormal epileptic wave forms inter-ictally, but some none unless during seizure Often not useful in outpatients, send home with EEG Can provoke seizure - sleep deprivation, hyperventilation, flashing lights Useful if not sure if in status epilepticus
273
What is the role of MRI/CT in patients with recurrent blackouts?
Indications: FOCAL or new onset seizures >25yrs old MRI preferred over CT - CT if urgent e.g. bleed None needed if GTC, MC, absence seizures
274
Give pathophysiology of epileptic seizures
Excitatory and inhibitory phases Prolonged depolorisation in neurones which spreads to adjacent areas + failure of GABA transmission
275
Give treatment of focal seizures
Carbamazepine Lamotrigine If refractory - vagal nerve stimulator
276
Give treatment of tonic clonic seizures
Valproate (not if young woman) | Lamotrigine
277
Give treatment of myoclonic seizures
Valproate Levoteracetam Topiramate
278
What does a visual acuity of 6/60 mean?
Able to see clearly at 6m, but average population can see clearly at 60m distance
279
Describe Marcus-Gunn pupil
= relative afferent pupillary defect (RAPD) Pupil constricts less/dilates when light moved from unaffected eye to affected eye Indirect and direct responses diminished when light in affected eye, but normal when light shines on unaffected eye Efferent constriction is OK, problem is with afferen pathway - light detection
280
What is the role of pinhole correction?
To exclude corneal defects, myopia, hypermetropia
281
What are the different causes of relative afferent pupillary defect?
Optic nerve lesion or neuritis Vascular causes Retinal detachment or vitreous haemorrhage Acute angle closure glaucoma
282
What might you experience with retinal detachment or vitreous haemorrhage?
Flashes/floaters in vision Decrease in vision Vitreous haemorrage associated with diabetic retinopathy, macular degeneration
283
What might you experience with acute angle closure glaucoma?
painful red eye, nausea, vomiting
284
What causes anterior ischaemic optic neuropathy? What might you see on examination?
ischaemia of posterior choroidal artery that supplies optic nerve head (optic disc) Swollen optic disc on fundoscopy Think GCA
285
What are the features of central retinal artery occlusion?
Occlusion of central retinal artery which supplies retina (branch of ophthalmic artery) PALE optic disc CHERRY RED SPOT on macula
286
What are the features of central retinal vein occlusion?
Dilated branch veins Multiple retinal haemorrhages Cotton wool patches STORMY SUNSET
287
What are the features of optic neuritis?
Painful eye movements, blurred vision over few days, exacerbated by heat/exercise, afferent pupillary defect, dyschromatopsia Inflammation of optic nerve at the optic nerve head (optic disc) If behind optic disc - often normal in 2/3
288
Give an example of a transient painless monocular loss of vision
Amaurosis fugax | Migraines
289
Give an example of a permanent painless monocular loss of vision
central retinal artery occlusion | central retinal vein occlusion
290
Give an example of permanent painful monocular loss of vision?
Optic neuritis Acute angle closure glaucoma Vitreous haemorrhage
291
What investigation might you do to confirm optic neuritis?
Visual evoked potentials - EEG during light stimulus
292
Give some causes of optic neuritis
1. Multiple Sclerosis! | 2. Lyme, syphilis, HIV, B12 deficiency, GCA
293
What is the prognosis of optic neuritis?
50% develop MS | Vision improves over 6 weeks, may have ongoing dyschromatopsia or decreased acuity
294
Give the diagnostic criteria for multiple sclerosis
McDonald's criteria Inflammatory plaques of demyelination disseminated in SPACE and TIME causing focal loss with relative preservation of axons >30 days between attacks >1hr duration attacks
295
Describe the pathophysiology of multiple sclerosis
Relapse-remission part - inflammatory response causes demyelination of axons leading to relapse and remitting symptoms Neurodegenerative part - demyelination heals poorly leading to axonal degeneration causing progressive and fixed deficits
296
What are the 4 types of multiple sclerosis?
1. Benign 2. Relapsing-remitting 3. Secondary chronic progressive 4. Primary progressive
297
What is a pseudo-relapse of MS?
flare-up of symptoms not due to MS, but due to other factors e.g. stress, infection
298
Give some symptoms of MS
``` Unilateral vision loss Fatigue, cognitive impairment Weakness (pyramidal), spastic paresis Sensory disturbance Cerebellar - ataxia, nystagmus, intention tremor, vertigo, dysarthria Bladder involvement/sexual dysfunction Lhermitte's Uhtoff's Cranial nerve/bulbar dysfunction - swallowing, facial movements, eye movements ```
299
Give some signs of MS
UMN signs - spastic paresis, brisk reflexes Sensory loss Cerebellar signs Optic atrophy, RAPD Internuclear ophthalmoplegia CN involvement - swallowing, face and eye movements
300
Give the investigations and findings for MS
1. MRI - T2 weighted - white matter lesions often peri-ventricular, or in brainstem, cerebellum, spinal cord 2. LP - oligoclonal IgG bands 3. Visual evoked potentials - delayed 4. MRI with contrast - lesions in last 3 months more enhanced - to see if disseminated in time
301
What is Lhermitte's sign?
radiating pain from back of neck down spine to arms and legs on flexion of neck (plaques in spinal cord cause nerve irritation)
302
What is Uhtoff's sign?
Worsening of neurological symptoms in demyelinating conditions due to heat
303
Give short-term management of multiple sclerosis
RULE OUT INFECTION 1g methylprednisolone IV 24 hrs or PO for 3-5 days (don't use more than once a year)
304
What are the short-term risks of high dose steroid treatment?
delirium/psychosis immunosuppression peptic ulcers pancreatitis
305
What is the long-term conservative management for MS?
Smoking cessation Psychological support Regular exercise PT/OT/SLT
306
What medical long-term disease-modifying management is there for MS?
Dimethylfumarate - mild/moderate relapse-remitting Alemtuzumab or Natalizumab (Interferon beta and glatiramer - not recommended)
307
What medical symptomatic control is there for MS?
Baclofen/gabapentin - spasticity Botulinum type A injections - tremor Amantadine - fatigue Tolterodine - urinary frequency/urgency Duloxetine - bladder instability, neuropathic pain Gabapentin, pregabalin, amitryptiline - neuropathic pain
308
What do you see in internuclear ophthalmoplegia?
Ipsilateral eye cannot adduct while contralateral eye abducts with nystagmus
309
What causes INO?
Communication between CN3 and CN6 disrupted due to lesion in medial longitudinal fasciculus
310
What are the top 2 risk factors for stroke?
1. Hypertension | 2. Atrial Fibrillation
311
What is the CHADS-VASC Score for?
To determine stroke risk in a patient with atrial fibrillation
312
What are the parameters on the CHADS-VASC score?
CHF, HTN, Age, Diabetes, Stoke/TIA/VTE history, Vascular disease, Sex
313
What type of seizures in status epilepticus are an emergency and which type are not?
``` Convulsive = emergency Non-convulsive = not emergency ```
314
What are T1 and T2 in status epilepticus?
``` T1 = 10 mins, where unusually prolonged T2 = 30 mins, where expect impairment ```
315
How would you manage status epilepticus?
1. 2 doses benzodiazepines 10 mins apart (IV/buccal midazolam, IV lorazepam/diazepam) 2. If not work - valproate or levoteracetam, consider glucose + thiamine 3. If not work - anaesthesia
316
Give mechanism of action of benzodiazepines
Increase frequency of chloride ion channel opening, increasing GABA inhibitory effect
317
What investigations would you do for status epilepticus?
Toxicology, FBC, U+E, CRP, BM, Calcium CT head (quick) especially if focal neurological signs EEG during if not sure it's status epilepticus
318
What is true vertigo?
Hallucination of movement, often rotatory, of the patient and their surroundings
319
What might be some associated symptoms of vertigo?
``` Difficulty walking or standing Relief on lying, sitting still - worse on movement Nausea, vomiting Pallor, sweating Possible hearing loss, tinnitus ```
320
Give some causes of true vertigo
1. vestibular neuritis 2. BPPV 3. Meniere's disease 4. Ototoxicity 5. Acoustic neuroma 6. Trauma 7. HZV 8. Central/brainstem causes 9. Alcohol intoxication
321
What does BPPV stand for? What are its typical features?
Benign paroxysmal positional vertigo - true vertigo on head MOVEMENT, stops when head still - decreases over time due to brain adapting (self-terminating) - Fatigueable nystagmus on Hall-pike manoeuvre is diagnostic
322
What manoeuvre helps to resolve BPPV?
Epley manoeuvre - clears debris from semicircular canals of ears
323
Give another name for acute labrynthitis. What are its typical features
``` = vestibular neuronitis Abrupt onset severe vertigo, nausea and vomiting Prostration No deafness or tinnitus Self-limiting (complete 3-4wks) ```
324
What causes acute labrynthitis? How would you manage?
Virus Vascular lesion Mx: Reassure and sedation if severe
325
What are the features of Meniere's disease?
Increased pressure in endolymphatic system of inner ear Recurrent attacks vertigo lasting >20 mins, fluctuating/permanent sensorineural hearing loss, tinnitus, aural fullness and falling to one side
326
How would you manage Meniere's disease?
Bed rest and reassurance in acute attacks | If prolonged - antihistamine (cinnarizine or buccal prochlorperazine if severe)
327
What ototoxic drugs can cause vertigo and/or deafness?
Aminglycosides e.g. gentamicin Loop diuretics Cisplatin
328
What type of tumour is an acoustic neuroma?
Schwannoma arising from vestibular nerve | Accounts for 80% cerebellopontine angle tumours
329
How do acoustic neuromas present?
Unilateral hearing loss Followed by vertigo Can be predicted with serial MRIs, slow growth Once progressed - CN5,6,9,10 may be affected and Ipsilateral cerebellar signs
330
What kind of trauma might cause vertigo?
Trauma affecting petrous temporal bone or cerebello-pontine angle, may damage CN8 causing vertigo, deafness or tinnitus
331
What features might you see in Herpes zoster external meatus infection?
Facial palsy, deafness, tinnitus and vertigo
332
What test would you do to check vertigo is not from a central/brainstem lesion?
Dolls-eye test Move head and ask patient to keep eyes fixed on stationary object. If eyes move with head rather than stay fixed = negative - likely vestibular system and brainstem intact??
333
What are the features of congenital nystagmus?
Usually bidirectional, pendular (no particular beating direction) Compensation - usually no vision effect
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What is the inheritance pattern of Friedrich's ataxia? What are its typical features?
autosomal recessive Ataxia, sensory loss arms and legs, impaired speech Spinocerebellar degeneration
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What is the inheritance pattern of Spino-Cerebellar Ataxia? What is the commonest type and its features?
Autosomal dominant SCA 6 is commonest in UK Pure ataxia
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What inheritance pattern is ataxia telangiectasia? What are its features?
Autosomal recessive Progressive ataxia starting in childhood Susceptible to cancer and oculomotor apraxia
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Give some toxicities that may cause ataxia
Alcohol Phenytoin and anti-convulsants Lithium Amiodarone
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What is a degenerative cause of cerebellar ataxia?
MSA - either MSA P or MSA C | Early onset speech impairment and autonomic decline
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What are immune-mediated causes of cerebellar ataxia?
post-viral cerebellitis - VZV in children Paraneoplastic degeneration Gluten ataxia (with/without coeliac) Primary autoimmune ataxia
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What might down-beat nystagmus mean?
Obstruction in foramen magnum
341
Give some features of Bell's palsy
= idopathic facial nerve palsy Abrupt onset complete unilateral facial weakness at 24-72hrs Ipsilateral numbness or pain around the ear Decreased taste Hypersensitivity to sounds
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How would you differentiate between LMN (Bell's palsy) and UMN facial weakness?
UMN weakness - forehead sparing as bilateral innervation | LMN weakness - no forehead sparing, unable to wrinkle forehead
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What conditions might you see a high steppage gait in?
Peripheral neuropathy | L5 radiculopathy, common peroneal nerve palsy
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What gait might you see in Parkinson's disease?
wide base, shuffling, stooped posture, asymmetrical reduced armswing Freezing at obstacles Apraxia - struggle to stand and start walking
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What gait might you see in hemiplegia?
Arm flexed and ipsilateral leg circumduction
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What gait might you see in proximal myopathy?
Waddling gait
347
Give the triad of extrapyramidal features of Parkinson's disease
1. Tremor 2. Hypertonia/rigidity "cogwheel rigidity" 3. Bradykinesia (slow movements, decrease in amplitude with repetition, festinant gait, freezing at doors and obstacles, expressionless face)
348
What is the cause of Parkinson's disease?
Loss of dopaminergic neurons in substantia nigra, associated with Lewy bodies in basal ganglia, brainstem and cortex Mostly sporadic, some genetic
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Give some non-motor features of Parkinson's
``` Autonomic dysfunction - postural hypotension, constipation, urinary frequency/urgency, saliva dribbling Sleep disturbance Reduced sense of smell Depression Dementia Psychosis ```
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How is Parkinson's disease diagnosed?
Exclude cerebellar and frontotemporal disease Clinical diagnosis and based on response to dopaminergic treatment If suspect alternative cause - MRI or DaTscan, PET
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What is the pharmacological management for Parkinson's disease?
Symptom control, not reversing disease so start late as tolerance increases and SE get worse with higher doses Levodopa Dopamine agonists to delay levodopa initiation - ropinirole, pramipexole Apomorphine Anticholinergics MAO-B inhibitors COMT inhibitors
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Why should you not withdraw Levodopa suddenly?
Acute kinesia risk | Neuroleptic malignant syndrome
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What is some non-pharmacological management of Parkinsons?
Deep brain stimulation (if partly dopamine-responsive) | Surgical ablation of overactive basal ganglia circuits