Neurology Flashcards
1
Q
Define headache.
A
Pain in the face, head or neck.
2
Q
Acute new headache: what are the three main features of meningitis?
A
Fever, photophobia, stiff neck.
3
Q
Acute new headache: what are the three main features of encephalitis?
A
Fever, confusion, decreased GCS.
4
Q
Acute new headache: what are the main features of subarachnoid haemorrhage?
A
Thunderclap headache, stiff neck, vomiting, fits, confusion, eye pain.
5
Q
Acute new headache: what are the main features of head injury?
A
Bruising, decreased GCS, lucid periods, amnesia.
6
Q
Acute new headache: what are the main features of self-limiting illness?
A
Sore throat, low grade fever.
7
Q
Acute new headache: what are the main features of sinusitis?
A
Tender of sinuses, history of upper respiratory tract infection.
8
Q
Acute recurrent headache: what are the main features of migraine?
A
Aura, visual + speech changes, nausea + vomiting, numbness.
9
Q
Acute recurrent headache: what are the main features of cluster headache?
A
Intense sharp stabbing eye pain, redness, lid swelling.
10
Q
Acute recurrent headache: what is the main feature of exertional headache?
A
History of association with activities.
11
Q
Acute recurrent headache: what are the main features of trigeminal neuralgia?
A
Intense stabbing eye pain, redness, lid swelling.
12
Q
Acute recurrent headache: what are the main features of glaucoma?
A
Red eye, halos, decreased visual acuity, pupil abnormality.
13
Q
Subacute headache: what are the main features of giant cell arteritis?
A
Age > 50, scalp tenderness, increased ESR, anaemia.
14
Q
Chronic headache: what are the main features of tension headache?
A
Bilateral tightening, associated with stress + anxiety.
15
Q
Chronic headache: what are the main features of medication overuse headache?
A
Rebound headaches on stopping analgesia, taking medication for longer than three months.
16
Q
Chronic headache: what are the main features of intracranial hypertension?
A
Non-pulsatile, bilateral, worse in morning, visual changes.
17
Q
Give examples of headache red flags.
A
Aged over 50, new onset, vomiting w/o cause, postural differences, eye pain, occurs after trauma, new with hx of cancer, seizures, loss of consciousness.
18
Q
What is meningitis?
A
A life-threatening condition of acute inflammation of the meninges.
19
Q
What are the meninges?
A
The protective membranes of the brain and spinal cord (dura mater, arachnoid mater, pia mater).
20
Q
What are the two mechanisms by which meningitis may develop from an infection?
A
Haematogenous spread.
Direct spread.
21
Q
Meningitis: What is haematogenous spread?
A
Pathogen enters the bloodstream and moves through the blood brain barrier.
22
Q
Meningitis: What is direct spread?
A
Pathogen penetrates CSF due to anatomical defects.
23
Q
Meningitis: describe how cerebral oedema and raised intracranial pressure develop.
A
WBCs identify the pathogen in the blood and the CSF. WBCs release cytokines to recruit more WBCs… the BBB becomes more permeable and more fluid crosses… increasing CSF pressure. Inflammation and endothelial damage = cerebral oedema and raised ICP.
24
Q
Meningitis: describe how hydrocephalus develops.
A
Fibrin deposits block resorption of CSF by arachnoid villi, causing hydrocephalus.
25
What are the three types of meningitis?
Viral meningitis.
Bacterial meningits.
Non-infectious meningitis.
26
Of the three types of meningitis, which is most common?
Viral meningitis.
27
What are the three most common causes viral meningitis?
Coxsackie (enteroviruses).
Herpes simplex virus.
Varicella zoster virus.
28
What are the two most common causes of bacterial meningitis in adults?
Neisseria meningitidis.
| Streptococcus pneumoniae.
29
What are the two most common causes of bacterial meningitis in neonates?
Group B streptococcus.
| E. coli.
30
What are non-infectious causes of meningitis?
Malignancy.
Autoimmune disease.
Adverse drug reactions.
31
How does meningitis present?
Fever, headache, photophobia and neck stiffness. As well as confusion and focal neurological deficits.
32
How is meningitis investigated?
Lumbar puncture.
33
What does lumbar puncture reveal in bacterial meningitis?
Raised white cells, polymorphonuclear cells (e.g. neutrophils), low glucose and high protein.
34
What does lumbar puncture reveal in viral meningitis?
Raised white cells, lymphocytes, normal glucose, normal/high protein.
35
What does lumbar puncture reveal in tuberculosis meningitis?
Raised white cells, lymphocytes, v. low glucose, v. high protein.
36
Meningitis: investigation by lumbar puncture is contraindicated under what circumstances?
In patients with cardiorespiratory instability or in patients with signs of raised intracranial pressure (vomiting, visual changes, behaviour changes, weakness).
37
Why is lumbar puncture contraindicated with raised intracranial pressure?
Risk of coning the cerebellum.
38
How is bacterial meningitis managed in the community?
In cases with non-blanching rash give single dose IM/IV benzylpenicillin before immediate transfer to hospital.
39
How is bacterial meningitis managed in the hospital setting?
Aged < 3 months: IV cefotaxime + amoxicillin.
Aged ≥ 3 months: IV ceftriaxone.
Give dexamethasone to reduce inflammation and complications.
40
How is viral meningitis managed?
Normally resolves spontaneously, can give aciclovir.
41
Who should receive meningococcal chemoprophylaxis and in what form?
Household members should receive ciprofloxacin.
42
What is the most common complication of bacterial meningitis?
Sensorineural hearing loss.
43
What is encephalitis?
Inflammation of the cerebral cortex.
44
What groups of people does encephalitis typically affect?
The immunocompromised.
| Those at the extremes of age (e.g. < 1 yr, > 65yrs).
45
What is the presentation of acute encephalitis?
Fever, confusion, decreased consciousness, headache, seizures and vomiting.
46
What is the most common cause of acute encephalitis?
Herpes simplex virus type-1.
47
How is acute encephalitis investigated?
Lumbar puncture.
48
What are the results of lumbar puncture in acute encephalitis?
Lymphocytosis and elevated protein in the CSF.
49
What is the management of acute encephalitis?
Intravenous acyclovir.
50
What is subarachnoid haemorrhage?
Bleeding beneath the arachnoid mater into the cerebrospinal fluid.
51
What are the two causes of subarachnoid haemorrhage?
Trauma.
| Spontaneous development.
52
Give examples of possible triggers for spontaneous subarachnoid haemorrhage.
Strenuous activity such as weight lifting or sex that can cause an aneurysm to rupture.
53
What is the characteristic presenting feature of subarachnoid haemorrhage?
Acute severe occipital headache (thunderclap!)... often described as a blow to back of the head.
54
Other than thunderclap headache, what are the features of subarachnoid haemorrhage?
Stiff neck, decreased consciousness, photophobia, vision, changes and focal neurological deficit.
55
Give four risk factors for subarachnoid haemorrhage?
Hypertension, smoking, excessive alcohol consumption and cocaine use.
56
What is the primary imaging investigation in suspected subarachnoid haemorrhage?
CT head.
57
What are the results of CT head in subarachnoid haemorrhage?
Hyperattenuation in the subarachnoid space. Note: negative imaging doesn't exclude SAH.
58
SAH: What investigation can be performed if CT head negative but SAH still suspected?
Lumbar puncture.
59
What are the results of lumbar puncture in subarachnoid haemorrhage?
Red blood cells and xanthochromia (bilirubin... breakdown of RBCs).
60
What pharmacological intervention can be provided in subarachnoid haemorrhage?
Nimodipine.
61
Management of SAH: explain why nimodipine is given.
Nimodipine is a calcium channel blocker which prevents cerebral artery vasospasm (risk of brain ischaemia).
62
What investigation may be performed in subarachnoid haemorrhage prior to neurosurgical intervention?
Cerebral angiography for the detection, demonstration and localisation of ruptured aneurysms.
63
What are the neurosurgical management options of subarachnoid haemorrhage?
Neurosurgical clipping.
| Endovascular coiling.
64
What is the Glasgow Coma Scale?
A universal assessment tool for assessing the level of consciousness.
65
What three factors contribute to the Glasgow Coma Scale?
Eyes, verbal response and motor response.
66
How are eyes scored on the Glasgow Coma Scale?
4 - open spontaneously.
3 - open to voice.
2 - open to pain.
1 - no opening.
67
How is verbal response scored on the Glasgow Coma Scale?
```
5 - orientated, normal conversation.
4 - confused conversation.
3 - inappropriate words.
2 - incomprehensible sounds.
1 - no verbal response.
```
68
How is motor response scored on the Glasgow Coma Scale?
```
6 - obeys commands.
5 - localises to pain.
4 - normal flexion to pain.
3 - abnormal flexion to pain.
2 - extends to pain.
1 - no motor response.
```
69
What GCS score indicates that a patient may need their airway securing?
8/15 or below.
70
What is chronic subdural haematoma?
Collection of blood within the subdural space (between the dural and arachnoid mater).
71
What are the clinical features of subdural haemorrhage?
Progressive history of confusion, reduced consciousness or neurological deficit.
72
Subdural haemorrhage commonly occurs due to rupture of which vessels?
Bridging veins which cross the subdural space.
73
Which groups of people are more vulnerable to subdural haemorrhage and why?
Alcoholics and the elderly. Both have brain atrophy which makes the bridging veins more exposed and vulnerable to rupture.
74
What does CT head reveal in chronic subdural haematoma?
Crescentic, hypodense collection.
75
What is the difference between acute subdural haemorrhage and chronic subdural haematoma on CT head?
Blood is hyperdense in acute haemorrhage and hypodense in chronic haematoma.
76
Chronic subdural haematoma is defined as existing for how long a period of time?
At least three weeks.
77
In subdural haemorrhage, why does blood form a crescentic pattern on CT head?
Haemorrhage is not limited by suture lines.
78
What is the management of acute subdural haemorrhage and chronic subdural haematoma?
Surgical decompression for both. Acute haemorrhage requires craniotomy while chronic haematoma can be resolved with burr holes.
79
What is extradural haemorrhage?
Bleeding between the skull and dura (the outermost layer of the meninges).
80
Extradural haemorrhage most commonly develops as a result of rupture of which vessel?
Middle meningeal artery.
81
Why is the middle meningeal artery particularly vulnerable to rupture?
The middle meningeal artery overlies the pterion, a particularly thin and weak point of the skull (where the temporal, frontal, parietal and sphenoid bones join).
82
What is the classic presentation of extradural haemorrhage?
Patient loses, briefly regains, then loses consciousness again following a low-impact head injury.
83
How does extradural haemorrhage appear on CT imaging?
Biconvex, hyperdense collection around the surface of the brain.
84
Why do extradural haemorrhages appear as biconvex on CT imaging?
The haemorrhage is limited by suture lines.
85
What is the management of extradural haemorrhage?
Craniotomy and surgical evacuation of the haemorrhage.
86
Give three possible causes of brain abscess?
Extension of sepsis from the middle ear or sinuses.
Penetrating head injuries.
Embolic events from endocarditis.
87
What is the presentation of a brain abscess?
Fever, headache and focal neurology (depending on the site of the abscess).
88
What are focal neurologic signs?
Impairments of nerve, spinal cord or brain function that affect a specific region of the body.
89
What are five causes of focal neurologic signs?
Head trauma, tumours, stroke, meningitis or encephalitis.
90
Frontal lobe signs most commonly involve what system?
The motor system.
91
Give examples of frontal lobe signs.
Unsteady gait, hypertonia, limb paralysis, expressive aphasia, change in personality, grand mal / tonic-clonic seizures,
92
Parietal lobe signs most commonly involve what system?
Somatosensory system.
93
Give examples of parietal lobe signs.
Impairment of tactile sensation and proprioception, sensory/visual neglect, loss of ability to read, write and calculate.
94
Temporal lobe signs most commonly involve what system?
Auditory sensation and memory systems
95
Give examples of parietal lobe signs.
Cortical deafness, tinnitus, auditory hallucinations, sensory aphasia, amnesia/memory loss, complex partial seizures.
96
Occipital lobe signs most commonly involve what system?
Visual sensation.
97
Give examples of occipital lobe signs.
Cortical blindness, visual field impairment, visual agnosia, visual hallucinations.
98
Cerebellar signs most commonly involve what system?
Balance and coordination.
99
Give examples of cerebellar signs.
Cerebellar ataxia (broad based gait), intention tremor, dysdiadochokinesia, nystagmus, dysarthria.
100
What is a common pathological consequence of brain abscess?
Abscesses have a considerable mass effect in the brain and raised intracranial pressure is common.
101
How are brain abscesses investigated?
CT imaging.
102
How are brain abscesses managed medically?
Intravenous ceftriaxone and metronidazole.
103
How are brain abscesses managed surgically?
Craniotomy and debridement of the abscess cavity.
104
Define migraine.
Recurrent, sever headache which is usually unilateral and throbbing in nature.
105
What clinical features are typically associated with migraine?
Along with unilateral throbbing headache... aura, nausea and photosensitivity.
106
Migraine: How long can the headache last?
4-72 hours.
107
Migraine: What is aura?
Visual changes associated with migraines.
108
Migraine: Give examples of aura.
Sparks in vision.
Blurring vision.
Lines across vision.
Loss of different visual fields.
109
Migraine: How long can aura last?
15-60 minutes.
110
Give examples of potential migraine triggers.
Chocolate, oral contraceptive, caffeine, alcohol, travel, exercise (chocolate mnemonic). As well as stress, bright lights, dehydration and menstruation.
111
What is the pharmacological management of acute migraine?
```
Oral triptan (e.g. sumatriptan) + NSAID.
//or//
Oral triptan + paracetamol.
```
112
What is pharmacological prophylaxis for migraine?
Topiramate or propranolol.
113
When should people be offered migraine prophylaxis?
Having two attacks of migraine per month.
114
Migraine management: what effects choice of topiramate v propranolol in migraine prophylaxis?
Topiramate should not be given to women of childbearing potential (teratogenic).
Propranolol should be avoided in asthmatics.
115
How is migraine management different in women?
Women may find that frequency and severity of migraines increases around menstruation. Treatment with mefenamic acid or a combination of aspirin, paracetamol or caffeine.
116
Describe the pattern by which cluster headaches can occur.
Headaches occur once or twice a day in clusters lasting several weeks... with a cluster occurring once a year.
117
Cluster headaches are more common in which two groups?
Men.
| Smokers.
118
What is the characteristic presentation of a cluster headache?
Intense, sharp, stabbing pain around one eye.
119
How long do episodes of cluster headache last for?
15 minutes to 3 hours.
120
As well as sharp pain around the eye, what are other features of cluster headaches?
Redness, miosis, ptosis, lacrimation, eyelid swelling and nasal congestion.
121
How are cluster headaches managed acutely?
100% oxygen via a non-rebreathe mask with 6mg subcutaneous sumatriptan.
122
What short-term prevention regimens can be used in the management of cluster headaches?
Oral prednisolone.
123
What longer-term prevention regimens can be used in the management of cluster headaches?
Oral verapamil.
124
Give three examples of potential triggers of cluster headache?
Alcohol.
Exercise.
Strong smells.
125
How are cluster headaches investigated?
MRI to exclude secondary causes e.g. tumours.
126
What is trigeminal neuralgia?
Syndrome characterised by severe unilateral facial pain along the branches of the trigeminal nerve (ophthalmic, maxillary, mandibular).
127
What is the clinical presentation of trigeminal neuralgia?
Brief, unilateral, electric shock-like pains that are abrupt in onset and termination. Pain is commonly evoked by light touch (washing, shaving, talking).
128
What are the causes of trigeminal neuralgia?
Idiopathic.
Compression of nerve roots by tumours.
Vascular problems.
129
What is the first-line pharmacological management of trigeminal neuralgia?
Carbamazepine.
130
What is temporal arteritis?
A large vessel vasculitis that commonly presents with unilateral headache.
131
What is the clinical presentation of temporal arteritis?
Unilateral headache, jaw claudication, visual disturbances, tender + palpable temporal artery.
132
What is the result of investigations in temporal arteritis?
Raised inflammatory markers.
| Skip lesions present on biopsy of the temporal artery.
133
What is the treatment of temporal arteritis?
High-dose prednisolone.
134
Describe the features of tension headache.
Recurrent, non-disabling, bilateral headache often described as a tight-band.
135
What is the management of tension headaches?
Advise yoga, meditation and exercise to try to alleviate stress and anxiety. If necessary provide simple analgesia.
136
Define raised intracranial pressure and what is normal intracranial pressure?
Pressure exerted inside the skull is increased (normal intracranial pressure is 7-15 mmHg in the supine position).
137
What are five potential causes of raised intracranial pressure?
Idiopathic intracranial hypertension, traumatic head injuries, infection, tumours and hydrocephalus.
138
How does raised intracranial pressure present clinically?
Headache (non-pulsatile, bilateral, worse in mornings), vomiting, reduced consciousness, visual disturbance (including transient visual darkening).
139
Raised ICP: What is Cushing's triad?
Signs indicative of raised ICP: widened pulse pressure, bradycardia and irregular respirations.
140
Raised ICP: What does investigation with fundoscopy reveal?
Bilateral papilloedema.
141
How is raised ICP treated?
Intravenous mannitol or CSF removal via shunt or repeated lumbar puncture.
142
Idiopathic intracranial hypertension typically affects what group of people?
Young, obese women.
143
What is spontaneous intracranial hypotension?
A rare cause of hypotension that develops due to a low level of CSF.
144
What is the clinical presentation of spontaneous intracranial hypotension?
Headache worse on standing and improves when lying flat.
145
What is tuberous sclerosis?
Genetic condition that involves the growth of hamartomas (benign neoplastic growth of the tissue that they originate from) on the brain and other organs.
146
What are neurocutaneous features of tuberous sclerosis?
Ash leaf spots (depigmented areas of skin). Roughened patches of skin over lumbar spine. Angiofibromas (papules across nose and cheeks). Fibromata beneath nails. Poliosis (isolated patch of white hair).
147
What are neurological features of tuberous sclerosis?
Epilepsy.
| Learning disability with developmental delay.
148
What is neurofibromatosis?
Group of conditions characterised by benign tumours that grow in the nervous system (neuromas).
149
What are the two types of neurofibromatosis and which is most common?
Neurofibromatosis type 1 (more common).
| Neurofibromatosis type 2.
150
What chromosome is the neurofibromatosis type 1 gene found on?
Chromosome 17.
151
Neurofibromatosis type 1 gene codes for what protein?
Neurofibromin (a tumour suppressor protein).
152
What is the inheritance pattern of mutations to the neurofibromatosis type 1 gene?
Autosomal dominant.
153
What are the diagnostic criteria of neurofibromatosis type 1?
C – café-au-lait spots.
R – relative with NF1.
A – axillary/inguinal freckles.
BB – bony dysplasia e.g. bowing of a long bone.
I – iris hamartomas (yellow/brown spots on the iris).
N – neurofibromas (2 or more).
G – glioma of the optic nerve.
154
What chromosome is the neurofibromatosis type 2 gene found on?
Chromosome 22.
155
Neurofibromatosis type 2 gene codes for what protein?
Merlin (a tumour suppressor protein important in Schwann cells).
156
What is the inheritance pattern of mutations to the neurofibromatosis type 2 gene?
Autosomal dominant.
157
What is the most common type of schwannoma that develops in neurofibromatosis type 2?
Acoustic neuroma.
158
Neurofibromatosis type 2: What are the clinical features of acoustic neuromas?
Hearing loss, tinnitus and balance problems.
159
Define transient ischaemic attack (TIA).
Transient episode of neurological dysfunction cause by focal brain, spinal cord or retinal ischaemia without acute infarction.
160
What is 14-day risk of stroke following TIA? (%)
10%.
161
What is the management of transient ischaemic attack?
300mg aspirin.
| Clopidogrel for three months.
162
Define stroke.
Sudden interruption in the vascular supply of the brain that causes neuronal death and thus irreversible damage.
163
What are the two main types of stroke?
```
Ischaemic stroke (majority).
Haemorrhagic stroke.
```
164
What are clinical features of stroke?
Motor weakness, dysphasia, swallowing problems, visual field defects and balance problems.
165
Total anterior circulation infarcts involve which vessels?
Middle and anterior cerebral arteries.
166
Total anterior circulation infarcts present with which three features?
Unilateral hemiparesis and/or hemisensory loss of face, arm and leg.
Homonymous hemianopia.
Higher cognitive dysfunction (dysphagia).
167
Partial anterior circulation infarcts involve which vessels?
Smaller arteries of anterior circulation (upper/lower division of middle cerebral arteries).
168
Partial anterior circulation infarcts present with two of which three features?
Unilateral hemiparesis and/or hemisensory loss of face, arm and leg.
Homonymous hemianopia.
Higher cognitive dysfunction (dysphagia).
169
Lacunar infarcts involve which vessels?
Penetrating arteries around the internal capsule, thalamus, basal ganglia.
170
Lacunar infarcts present with one of which three features?
Unilateral weakness of face + arm, arm + leg or all three.
Pure sensory stroke.
Ataxic hemiparesis.
171
Posterior infarcts involve which vessels?
Vertebrobasilar arteries.
172
Posterior infarcts present with one of which three features?
Cerebellar or brainstem syndromes.
Loss of consciousness.
Isolate homonymous hemianopia.
173
Suspected strokes require which urgent investigation and why?
CT or MRI head to classify strokes into ischaemic or haemorrhagic.
174
What is the management of ischaemic strokes which present within 4.5 hours of symptom onset?
Intravenous alteplase (thrombolytic therapy).
175
What are contraindications for alteplase use in the management of ischaemic stroke?
Previous intracranial haemorrhage, seizure at onset of stroke, intracranial neoplasm, pregnancy, preceding gastrointestinal bleeding and active bleeding.
176
What is the management of ischaemic strokes which aren't suitable for thrombolytic therapy but present within 24 hours of symptom onset?
Mechanical thrombectomy.
177
What is provided for secondary prevention of ischaemic strokes?
75mg clopidogrel.
178
What structural disease can increase the risk of an ischaemic stroke?
Carotid artery stenosis.
179
How is carotid artery stenosis investigated?
Duplex ultrasound followed by CT angiography.
180
What is the management of carotid artery stenosis?
Carotid artery endarterectomy or stenting.
181
What are two causes of haemorrhagic stroke (and which is most common)?
Hypertension (most common).
| Amyloid angiopathy.
182
Where do haemorrhagic strokes secondary to hypertension most commonly occur?
Cerebellum, thalamus and/or basal ganglia.
183
Describe the pathophysiology behind amyloid angiopathy and the development of haemorrhagic strokes?
Amyloid angiopathy is characterised by protein deposition which increases vascular fragility and thus the risk of rupture... bleeding.
184
Where do haemorrhagic strokes secondary to amyloid angiopathy most commonly occur?
Cerebral cortex.
185
What is the management of haemorrhagic stroke?
Stop anticoagulation and reverse (if applicable).
Control hypertension with labetalol (aim for SBP < 140).
If amenable refer to neurosurgery for craniotomy.
186
How is anticoagulation by warfarin reversed?
Give dried prothrombin complex (beriplex) and vitamin K (phytomenadione).
187
What is a facial nerve palsy?
Isolated dysfunction of the facial nerve and weakness of structures it supplies.
188
What are the functions of the facial nerve?
Motor: muscles of facial expression and stapedius muscle. Sensory: taste sensation from the anterior 2/3 of the tongue. Parasympathetic: salivary and lacrimal glands.
189
How is an upper motor neurone and lower motor neurone facial nerve palsy differentiated?
Upper motor neurone lesions spare the upper face (forehead) whereas lower motor neurone lesions affect all facial muscles.
190
Give examples of a cause of an upper motor neurone lesion and lower motor neurones facial nerve palsy?
Upper - stroke.
| Lower - Bell's palsy.
191
What is Bell's palsy?
Acute, unilateral, idiopathic, facial nerve paralysis.
192
How does Bell's palsy present clinically?
Lower motor neurone facial nerve palsy affecting all facial muscles (including forehead). Post-auricular pain, altered taste, dry eyes and hyperacusis.
193
What is the management of Bell's palsy?
10 day course of prednisolone given within 72 hours of onset.
194
What is the prognosis of Bell's palsy if left untreated?
15% of untreated patients will develop permanent moderate-to-severe weakness. The rest resolve.
195
What is a third nerve palsy?
Eye condition resulting from damage to the oculomotor nerve (or a branch thereof).
196
What is the function of the oculomotor nerve?
Supplies a majority of muscles controlling eye movement EXCEPT lateral rectus and superior oblique.
197
How does a third nerve palsy present clinically?
'Down and out' eye deviation and ptosis.
198
A defect to the left oculomotor nerve affects which eye (right or left)?
Left - cranial nerves don't cross sides (except CNIV)
199
What is a surgical third nerve palsy?
Third nerve palsy (down and out) + dilated pupil with absent light reflex.
200
What is the management of a surgical third nerve palsy?
Requires urgent brain imaging to exclude bleed/aneurysm.
201
What is epilepsy?
A condition characterised by recurrent seizures.
202
How is epilepsy classified?
Depending on where the seizure begins in the brain, the level of awareness of the individual during the seizure, motor, non-motor and other features.
203
What are the main clinical features of epilepsy?
Loss of consciousness, limb jerking, tongue biting and incontinence.
204
What are the two main groups of seizure?
Generalised.
| Focal.
205
What is the difference between generalised and focal seizures?
Generalised seizures result in loss of consciousness whereas focal seizures result in varying consciousness.
206
What is the first-line treatment of generalised seizures?
Sodium valproate (avoid in females of childbearing potential).
207
What is the first-line treatment of focal seizures?
Carbamazepine.
208
What is a common second-line option in treatment of epilepsy?
Lamotrigine.
209
Epilepsy: What typically follows seizure?
Postictal period (drowsy and tired) for around fifteen minutes.
210
Following an epileptic seizure and EEG / brain imaging is normal... how long must a patient not drive for?
Six months.
211
Following an epileptic seizure and EEG / brain imaging is abnormal... how long must a patient not drive for?
Twelve months.
212
What are the typical features of a generalised tonic-clonic seizure?
Loss of consciousness, stiffening, body jerking.
213
What are the typical features of a temporal lobe (focal) seizure?
Plucking clothes, lip smacking, aura, deja-vu.
214
What are the typical features of a frontal lobe (focal) seizure?
Motor abnormalities and Jacksonian movements.
215
What are the typical features of a parietal lobe (focal) seizure?
Sensory abnormalities (tingling, numbness).
216
What are the typical features of juvenile myoclonic epilepsy?
Absence, myoclonus, tonic-clonic seizure.
217
What are absence seizures?
Form of generalised epilepsy mostly seen in children.
218
How do absence seizures present?
Absence/vacant lasting for a few seconds followed by quick recovery.
219
What does EEG reveal in absence seizures?
Bilateral, symmetrical 3Hz spike and wave pattern.
220
What is the management and prognosis of absence seizures?
Manage with sodium valproate / ethosuximide. Most become seizure-free in adolescence.
221
What are psychogenic non-epileptic seizures?
Pseudoseizures. Events resemble epileptic seizures but lack the characteristic electrical discharges associated with epilepsy.
222
What are features of psychogenic non-epileptic seizures?
Gradual onset with pelvic thrusting. Crying post-seizure.
223
What investigation can be used to differentiate pseudoseizures from true epilepsy?
Serum prolactin - prolactin raised 10-20 minutes following true epilepsy (but not pseudoseizure).
224
What is status epilepticus?
Seizures lasting longer than five minutes.
225
Why is status epilepticus dangerous?
Prolonged seizures = hypoxia = irreversible brain damage.
226
What is the treatment of status epilepticus in hospital?
Intravenous lorazepam.
227
Status epilepticus: How soon can intravenous lorazepam be repeated?
Repeated once after 10 minutes.
228
Status epilepticus: if seizures don't resolve after two doses of lorazepam, what treatment should be given?
Intravenous phenytoin.
229
What is the treatment of status epilepticus in the prehospital setting?
Buccal midazolam or rectal diazepam.
230
What is dementia?
A syndrome encompassing progressive deficits across several cognitive domains.
231
Give four causes of dementia.
Alzheimer's disease.
Vascular dementia.
Lewi-body dementia.
Fronto-temporal dementia.
232
How does dementia present?
Memory loss over months or years. Non-cognitive symptoms include agitation, aggression or apathy.
233
Give examples of two assessment tools used in the diagnosis of dementia.
10-point cognitive screener (10-CS).
| 6-item cognitive impairment test (6CIT).
234
What are two differential diagnoses to dementia of memory loss over hours/days?
Infection.
| Stroke.
235
What are differential diagnoses to dementia of memory loss over several weeks?
Depression.
Hypothyroidism.
B12/folate/thiamine deficiency.
236
What investigations are performed for dementia in primary care?
Confusion screen (FBC, U&Es, LFTs, calcium, glucose, TFTs, vitamin B12, folate).
237
In the diagnosis of dementia, neuroimaging is important in the exclusion of which conditions?
Subdural haematoma.
| Normal pressure hydrocephalus.
238
Give examples of social support that can be provided to dementia patients.
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Laundry services.
District nurses.
Attendance allowance.
Respite care in hospital.
Day care and lunch clubs.
Council tax rebate.
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239
What is the most common cause of dementia?
Alzheimer's disease.
240
What is the presentation of Alzheimer's dementia?
Enduring, progressive and global cognitive impairment.
241
Describe the pathophysiology of Alzheimer's dementia.
Accumulation of beta-amyloid peptide (degradation product of amyloid precursor) results in progressive neuronal damage, neurofibrillary tangles and increased numbers of amyloid plaques.
242
What are the clinical features of Alzheimer's dementia?
Impairment of visuo-spatial skill (gets lost), memory, verbal abilities and executive function (planning).
243
What are later clinical features of Alzheimer's dementia?
Irritability, mood disturbance and behavioural change (wandering, aggression, disinhibition).
244
What pharmacological intervention can be considered first-line in Alzheimer's dementia?
Acetylcholinesterase inhibitors (donepezil, rivastigmine, galantamine).
245
What pharmacological intervention can be considered second-line in Alzheimer's dementia?
Memantine (NMDA receptor antagonist).
246
What is vascular dementia?
The cumulative effect of many small strokes.
247
How does vascular dementia present?
Sudden onset, stepwise deterioration of cognitive impairment.
248
What are the clinical features of vascular dementia?
Focal neurological abnormalities, attention/concentration difficulty, seizures, memory disturbance and gait/speech/emotional disturbance.
249
What is Lewy-body dementia?
Dementia characterised by alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas.
250
What are the clinical features of Lewy-body dementia?
Fluctuating cognitive impairment, Parkinsonism and hallucinations.
251
What is fronto-temporal dementia?
A type of cortical dementia characterised by frontal and temporal atrophy without Alzheimer histology.
252
What is the presentation of fronto-temporal dementia?
Insidious onset typically before the age of 65 years with personality change and social conduct problems.
253
What is Pick's dementia?
The most common subtype of fronto-temporal dementia.
254
What are the clinical features of Pick's dementia?
Personality change, impaired social conduct, hyperorality, disinhibition, increased appetite and preservation behaviour.
255
What is normal pressure hydrocephalus?
Reversible cause of dementia caused by reduced CSF absorption at the arachnoid villi (secondary to head injury, subarachnoid haemorrhage and meningitis).
256
What is the classic triad of features of normal pressure hydrocephalus?
Urinary incontinence, gait abnormality and dementia (wet, wobbly and wacky).
257
What investigation is performed in normal pressure hydrocephalus and what are the results?
Neurological imaging (CT or MRI). Hydrocephalus with ventriculomegaly.
258
How should normal pressure hydrocephalus be managed?
Ventriculoperitoneal shunting.
259
What is benign essential tremor?
Neurological disorder that causes involuntary and rhythmic shaking in voluntary muscles.
260
What are clinical features of essential tremor?
Fine tremor of the hands, head and jaw. Pt may also have a vocal tremor.
261
What is the management of essential tremor?
Rest typically improves symptoms. Pharmacological management includes propranolol (beta-blocker).
262
What is Guillain-Barre syndrome?
Syndrome of rapid-onset muscle weakness as a result of autoimmune damage to the peripheral nervous system.
263
What is the most common pathophysiological process behind the development of Guillain-Barre syndrome?
Acute inflammatory demyelinating neuropathy
264
Describe the process behind the acute inflammatory demyelinating neuropathy in Guillain-Barre syndrome?
White blood cells produce antibodies against the preceding infection which inappropriately match with proteins on the myelin sheath... causing damage.
265
Two thirds of cases of Guillain-Barre syndrome are preceded by what?
Gastrointestinal or upper respiratory tract infections.
266
When does Guillain-Barre syndrome typically develop post gastrointestinal / upper respiratory tract infection?
Four weeks.
267
What are the most common bacterial pathogens that precede Guillain-Barre syndrome?
Campylobacter jejuni.
| Mycoplasma pneumoniae.
268
What are the two most common viral pathogens that precede Guillain-Barre syndrome?
Cytomegalovirus.
| Epstein-Barr virus.
269
What is the characteristic presentation of Guillain-Barre syndrome?
Progressive ascending weakness (starting in the hands and feet) with/without paraesthesia.
270
What are the potential clinical features of Guillain-Barre syndrome (other than weakness)?
Loss of sensation, absence of reflexes, cranial nerve involvement (double vision, dysarthria, difficulty swallowing), autonomic dysfunction (blood pressure irregularity, constipation, urinary incontinence).
271
How is diagnosis of Guillain-Barre syndrome made?
Clinically with supportive investigations (nerve conduction studies and CSF analysis).
272
What do nerve conduction studies reveal in Guillain-Barre syndrome?
Focal conduction block is characteristic of GBS.
273
What does CSF analysis reveal in Guillain-Barre syndrome?
Elevated protein.
274
How is Guillain-Barre syndrome treated?
Give intravenous immunoglobulin to neutralise harmful antibodies. If no response perform plasmapheresis. Give VTE prophylaxis (LMWH).
275
How should neuropathic pain be managed in Guillain-Barre syndrome?
Mod-severe pain give tramadol.
276
How should individuals with Guillain-Barre be monitored for life-threatening complications?
Respiratory muscles may be affected - four-hourly monitoring of forced vital capacity is essential in anticipating respiratory failure. FVC < 20mL/kg warrants transfer to ICU for potential intubation and ventilation.
277
What is Miller-Fisher syndrome?
A variant of Guillain-Barre syndrome that presents as a descending paralysis (causing ophthalmoplegia, ataxia and areflexia).
278
What is Huntington's disease?
An incurable progressive neurodegenerative condition.
279
What is the inheritance pattern of Huntington's disease?
Autosomal dominant.
280
What type of mutation results in the development of Huntington's disease?
Trinucleotide repeat disorder - mutation in the huntingtin (HTT) gene on chromosome 4.
281
Huntington's disease: Trinucleotide repeat disorders are characterised by what genetic phenomenon?
Genetic anticipation.
282
Huntington's disease: Explain genetic anticipation.
The disease presents at an earlier age with increased severity in successive generations.
283
Features of Huntington's disease typically develop at what age?
After 35 years.
284
How does Huntington's disease present initially?
Personality changes (irritability, apathy, depression) and intellectual impairment.
285
Huntington's disease: Following the development of personality changes, how does the disease progress?
Development of chorea (jerky body movements), dystonia and saccadic eye movements.
286
How is Huntington's disease diagnosed?
Genetic testing.
287
How is Huntington's disease managed?
No cure. Antipsychotics (olanzapine) and benzodiazepines (diazepam) can be given to suppress disordered movements.
288
What is the prognosis of Huntington's disease?
Life expectancy is 15-20 years following diagnosis. Most common cause of death is pneumonia.
289
What is Parkinson's disease?
A progressive neurodegenerative condition caused by degeneration of dopaminergic neurones in the substantia nigra.
290
What is the mean age of diagnosis of Parkinson's disease?
65 years.
291
Parkinson's disease: Describe the physiology of the basal ganglia.
The basal ganglia normally exert a constant inhibitory influence on a wide range of motor symptoms, preventing them from becoming active at inappropriate times.
292
Parkinson's disease: Describe the function of dopamine in normal physiology.
Dopamine facilitates a reduction in inhibition when a decision is made to perform an action.
293
Describe the pathophysiology of Parkinson's disease.
Death of dopaminergic neurones leads to lower levels of dopamine in the striatum and thus greater exertion of effort demanded for any given movement.
294
Where is dopamine produced?
Substantia nigra pars compacta.
295
What are the three characteristic features of Parkinson's disease?
Bradykinesia, tremor and cogwheel rigidity.
296
Describe the tremor associated with Parkinson's disease.
Asymmetrical resting tremor.
297
Other than the three main features of Parkinson's disease... how else may the condition present?
Mask-like facies.
| Postural instability.
298
How is Parkinson's disease treated?
Levodopa.
299
What is levodopa?
Dopamine precursor used in the management of Parkinson's disease.
300
Levodopa is typically combined with what to increase its effectivity?
Decarboxylase inhibitor (e.g. carbidopa).
301
Why is co-careldopa (levodopa + carbidopa) more effective than levodopa alone?
Carbidopa prevents the peripheral metabolism of levodopa to dopamine.
302
Give examples of side effects of levodopa.
Dyskinesia, dry mouth, anorexia, palpitations, postural hypotension, psychosis and drowsiness.
303
What is Parkinson's disease dementia?
Dementia presents in the later stages of Parkinson's (at least one year of parkinsonian symptoms).
304
What are the features of drug-induced Parkinsonism?
Rapid-onset, bilateral motor symptoms while rigidity and resting tremor are uncommon.
305
What is multiple system atrophy?
A Parkinsonian-plus syndrome characterised by Parkinsonism with autonomic, corticospinal and cerebellar dysfunction.
306
What are the features of multiple system atrophy?
Bradykinesia and cogwheel rigidity with urinary urgency, constipation and postural hypotension.
307
How does the parkinsonian features of multiple system atrophy differ from Parkinson's disease?
MSA does not typically involve resting tremor. Note that in MSA the features are typically symmetrical (as opposed to asymmetrical in Parkinson's).
308
How is multiple system atrophy treated?
Levodopa.
309
What is motor neurone disease?
Motor neurone disease is a progressive neurological condition of unknown caused characterised by degenerative of motor neurones in the brain, brain stem and spinal cord.
310
Give examples of patterns of motor neurone disease.
Amyotrophic lateral sclerosis.
Progressive bulbar palsy.
Progressive muscular atrophy.
311
What is the most common pattern of motor neurone disease?
Amyotrophic lateral sclerosis (AML).
312
What group of people are most commonly affected by motor neurone disease?
Late-middle aged men.
313
Amyotrophic lateral sclerosis is characterised by what features?
Lower motor neurone signs in the arms and upper motor neurone signs in the legs.
314
What is the clinical presentation of motor neurone disease?
Insidious progressive weakness of muscles of limbs, trunk, face and speech. Features include stumbling, foot drop, weakened grip, slurred speech, cramp, muscle wasting, twitching and fatigue.
315
How is diagnosis of motor neurone disease made?
Clinical diagnosis with nerve conduction studies to exclude neuropathies.
316
Motor neurone disease: give examples of lower motor neurone signs.
Muscle wasting, reduced tone, fasciculations and reduced reflexes.
317
Motor neurone disease: give examples of upper motor neurone signs.
Increased tone/spasticity, brisk reflexes and upgoing plantars.
318
What is the management of motor neurone disease?
Riluzole may prolong life for up to three months. Be honest with patients about progression, provide analgesia and respiratory care.
319
Explain how riluzole prolongs life in people with motor neurone disease.
Riluzole prevents the stimulation of glutamate receptors (excessive stimulation is thought to play a role in the destruction of motor neurones).
320
What is Brown-Sequard syndrome?
Condition that results from hemisection of the spinal cause.
321
Give examples of causes of Brown-Sequard syndrome.
Tumour, trauma, ischaemia and infectious/inflammatory diseases.
322
Brown-Sequard: What are the clinical features of a corticospinal tract lesion?
Ipsilateral weakness below the lesion.
323
Brown-Sequard: What are the clinical features of a dorsal column lesion?
Ipsilateral loss of proprioception and vibration sensation.
324
Brown-Sequard: What are the clinical features of a spinothalamic tract lesion?
Contralateral loss of pain and temperature sensation.
325
How is Brown-Sequard syndrome investigated?
MRI.
326
What is syringomyelia?
A collection of CSF fluid within the spinal cord due to anatomical abnormalities... can expand over time to destroy the spinal cord.
327
Give examples of causes of syringomyelia.
Chiari malformations, intramedullary tumours, trauma.
328
What is the clinical presentation of syringomyelia?
Cough, lower motor neurone weakness in hands and arms, upgoing plantars and dissociate sensory loss affecting spinothalamic sensation (temperature and pain)... patients may accidentally burn their hands without realising.
329
What is multiple sclerosis?
An inflammatory demyelinating disease of neurones in the central nervous system (the immune system attacks and destroys myelin).
330
What type of hypersensitivity reaction is multiple sclerosis?
Type 4 (cell-mediated) hypersensitivity reaction.
331
Describe the pathophysiology of multiple sclerosis.
T lymphocytes bypass the blood-brain barrier and identify myelin-producing oligodendrocytes as foreign. T cells release cytokines which recruit more T cells as well as B cells and macrophages. B cells produce antibodies which mark the myelin sheath and macrophages attack oligodendrocytes - leading to demyelination.
332
What are the four types of multiple sclerosis?
Relaxing-remitting multiple sclerosis (RRMS).
Secondary progressive multiple sclerosis (SPMS).
Primary progressive multiple sclerosis (PPMS).
Progressive-relapsing multiple sclerosis (PRMS).
333
What is the most common type of multiple sclerosis?
Relapsing-remitting multiple sclerosis (RRMS).
334
Describe the disease course of releasing-remitting multiple sclerosis.
Bouts of autoimmune attacks months/years apart which cause increasing disability... there will be some improvement in symptoms initially (remitting) but overtime symptoms will resolve incompletely leaving residual disability.
335
Describe the disease course of secondary progressive multiple sclerosis.
Initially similar to RRMS until eventually the immune attack becomes constant causing a steep progression in disability.
336
Describe the disease course of primary progressive multiple sclerosis.
A constant attack causing steady progression of disease.
337
Describe the disease course of progressive-relapsing multiple sclerosis.
A constant attack as well as superimposed bouts of disease.
338
At what age does multiple sclerosis typically present?
20-40 year old.
339
What is the most common presentation of multiple sclerosis?
Unilateral optic neuritis (pain on eye movement and rapid decrease in central vision).
340
How may multiple sclerosis present (other than optic neuritis)?
```
Brainstem or cerebellar symptoms (diplopia, ataxia, nystagmus, intention tremor).
Sensory symptoms (numbness or tingling in limbs).
Spastic weakness.
```
341
How is a diagnosis of multiple sclerosis made (what criteria are used)?
Clinically and guided by the McDonald criteria.
342
Multiple sclerosis: What are the McDonald criteria?
Multiple lesions in the CNS disseminated in space and time and causes symptoms that last longer than 24 hours.
343
What investigations are used in the diagnosis of multiple sclerosis?
MRI.
| CSF analysis.
344
Multiple sclerosis: What do MRI and CSF analysis reveal?
MRI - white matter plaques.
| CSF - oligoclonal bands.
345
What is the treatment of an acute relapse of multiple sclerosis?
Corticosteroids (methylprednisolone) - three day high dose intravenous, or five day oral.
346
What is the treatment for long-term remission of multiple sclerosis?
Biological agents / disease-modifying drugs.
347
Multiple sclerosis: What treatment is provided for spasticity?
Gabapentin or baclofen.
348
What are characteristic features of cauda equina syndrome?
Lower back pain, urinary incontinence/retention, reduced sensation to the perianal area and decreased anal tone.
349
What is myasthenia gravis?
A long-term neuromuscular disease that leads to varying degrees of skeletal muscle weakness.
350
What type of hypersensitivity reaction is myasthenia gravis?
Type 2 (antibody-mediated) hypersensitivity reaction.
351
Describe the pathophysiology of myasthenia gravis.
B-cells produce antibodies against post-synaptic nicotinic acetylcholine receptors at the neuromuscular junction leading to depletion of these receptors (normal action potentials cannot be generated).
Autoantibodies may also target muscle-specific receptor tyrosine kinase (MuSK) protein.
352
What are the clinical features of myasthenia gravis?
Fluctuating muscle weakness and fatiguability. Patients become progressively weaker over the course of a day.
353
What is the most common presenting feature of myasthenia gravis?
Vision impairment (ptosis, diplopia) due to extra ocular muscle groups being affected.
354
Myasthenia gravis: Give examples of affected muscle groups and the corresponding features.
Extraocular - ptosis, diplopia.
Bulbar - swallowing difficulty, dysarthria.
Face - facial weakness, facial expression loss.
Neck - cannot keep head up.
Limbs - proximal asymmetric muscle weakness.
Respiratory - respiratory failure.
355
20% of cases of myasthenia gravis are associated with what tumour?
Thymoma
356
What clinical features are associated with myasthenia w/ thymoma?
Cough, shortness of breath.
357
What investigations are performed in myasthenia gravis Gravis?
```
Antibody testing (acetylcholine receptor antobides and muscle-specific tyrosine kinase).
Nerve conduction studies.
```
358
What does antibody testing reveal in myasthenia?
Raised AChR-Abs.
| Raised MuSK.
359
What do nerve conduction studies reveal in myasthenia?
Repetitive stimulation results in progressive decline in muscle action potential.
360
How is myasthenia gravis treated?
Pyridostigmine (acetylcholinesterase inhibitor).
| High dose prednisolone during relapse.
361
How does pyridostigmine treat myasthenia gravis?
Acetylcholinesterase inhibitor prevents the degradation of acetylcholine to increase the concentration of acetylcholine in the synaptic cleft to enhance neuromuscular transmission.
362
What is the primary life-threatening complication of myasthenia gravis?
Myasthenic crisis (decrease in function of respiratory muscles).
363
How are people with myasthenia monitored for the development of myasthenic crisis?
Regular forced vital capacity (FVC) measurements.
