Neurology - part 1 Flashcards
1
Q
What are two methods of investigation problems with the peripheral nervous system (2)?
A
- EMG
- Nerve conduction studies
2
Q
What are two findings in a nerve conduction study?
A
- Decreased amplitude/ smaller waves = axon loss (e.g. MND, peripheral neuropathy)
- Delayed signal = demyelination (e.g. GBS)
3
Q
What are two findings in electromyography?
A
- Myopathy = decreased action potential duration and amplitude
- Neuropathy = increased action potential duration and amplitude
4
Q
What is a myopathy vs neuropathy?
A
- Myopathy = problem with the muscle (e.g. muscular dystrophy)
- Neuropathy = problem with the nerve (e.g. MND)
5
Q
What are 3 ways of investigating problems with the CNS?
A
- EEG (electroencephalogram)
- MRI/ CT
- LP
6
Q
What is NCCT first line for (3)?
A
- Stroke
- ICH
- Raised ICP
7
Q
What is a contrast CT useful for?
A
Brain abscess
8
Q
What are some differences in of PNS and CNS lesions in terms of presentation (2)?
A
- PNS = back pain?, bi-lateral,
- CNS = headache?, uni-lateral, visual changes
9
Q
What is a lateralising sign?
A
Lesion affecting a single hemisphere (e.g. pronator drift, homonymous hemianopia, u/l weakness)
10
Q
What are 3 important tracts to know about and where do they decussate?
A
- (DCML) dorsal column medial lemniscal (medulla)
- Spinothalamic (1-2 spinal levels above)
- Corticospinal (medulla)
11
Q
What does the DCML carry (4)?
A
- Proprioception
- Fine touch
- Vibration
- 2 point discrimination
12
Q
What does spinothalamic tract carry (3)?
A
- Lateral = pain + temperature
- Anterior = crude touch
13
Q
What does the corticospinal tract carry (1)?
A
Motor signals to muscles
14
Q
What nerve roots are responsible for the key reflexes?
A
- Bicep = C5/6
- Tricep = C7
- Knee = L4
- Ankle = S1
15
Q
What nerve roots are responsible for the key dermatomes (6)?
A
- C6 = thumb
- C7 = middle + palm
- C8 = little
- T10 = bellybuT-TEN
- L5 = big toe (largest of the five)
- S1 = heel + sole
think of body doing splits with arms out
16
Q
What are the nerve roots responsible for the key myotomes (5)?
A
- C5/6 = elbow flexion + shoulder abduction
- C7 = wrist flex, elbow extend
- C8 = hand flexion
- L5 = dorsiflexion
- S1 = plantar flexion
17
Q
What are two common causes of foot drop?
A
- Common peroneal nerve lesion = MC
- L5 radiculopathy
18
Q
How could you differentiate between common peroneal nerve lesion and L5 radiculopathy (2)?
A
- Common peroneal = ankle jerk absent, weak eversion
- L5 radiculopathy = ankle jerk present, weak inversion
19
Q
What is a stroke?
A
A sudden infarct in the blood supply to part of the brain with symptoms lasting more than 24 hours
20
Q
What are the two categories of stroke?
A
- Haemorrhagic (15%)
- Ischemic (85%)
21
Q
What presentation is suggestive of a stroke?
A
Any sudden onset neurological symptom
22
Q
What is ischemia vs infarction?
A
- Ischemia = inadequate blood supply
- Infarction = complete blockage of blood –> tissue death
23
Q
What are the causes of a stroke (7)?
A
- Thrombus (+atherosclerosis)
- Embolus + infective endocarditis
- Shock
- Vasculitis
- Trauma
- Aneurysm
- Anticoagulants
24
Q
What might cause a stroke in young people (6)?
A
- ADPKD (berry aneurysms)
- Congenital heart malformations
- COCP
- Blood clotting disorders
- Cocaine
- Infective endocarditis
25
Which congenital heart malformations increase the risk of stroke (3)?
* ASD
* VSD
* Patent foramen ovale
*allow embolus from systemic circulation to enter the left ventricle and go to brain*
26
What type of stroke affects the deep brain structures?
Lacunar stroke
27
What is a TIA?
A temporary neurological dysfunction lasting less than 24 hours caused by ischemia, but without infarction
28
What are some typical symptoms of a stroke (6)?
* Limb weakness
* Facial weakness
* Dysphasia
* Visual field defect
* Sensory loss
* Ataxia and vertigo
29
What are some risk factors for an ischemic stroke (13)?
* Smoking
* Hypertension
* AF
* Previous stoke/ TIA
* Diabetes
* Obesity
* Raised cholesterol
* Family history
* Vasculitis
* Thrombophilia
* COCP
* Migraines (with aura)
* Older age
30
What are the Bamford/ Oxford stroke classifications (4)?
* Total anterior circulation stroke
* Partial anterior circulation strokes
* Posterior circulation stroke
* Lacunar stroke
31
How does a total anterior circulation stroke present (3)?
* Unilateral sensory/ motor loss
* Homonymous hemianopia
* Higher cortical function loss e.g. dysphasia
**Need all 3 for this classification**
32
How does partial anterior circulation stroke present?
2 out of 3 of the criteria for a TACS
33
How does a posterior circulation stroke present (3)?
* Isolated vision change
* Cerebella Sx
* Contralateral CN palsy
34
How does a lacunar stroke present (3)?
* Pure motor stroke
* Pure sensory stroke
* Ataxic hemiparesis
35
What would be the presentation of an ACA stroke (2)?
* **Lower limb >>>> upper limb**
* Behavioural change
*sensory and motor deficit*
36
What would be the presentation of an MCA stroke (4)?
* **Upper limb >>>> lower limb**
* Face droop (forehead spared)
* Brocca/ wernicke aphasia (in dominant hemisphere)
* Ipsilateral gaze deviation
37
How does PCA stroke present (2)?
* **Contralateral homonymous hemianopia** (with macular sparing)
* Visual agnosia
38
What is webers stroke?
Branches of PCA that supply the midbrain blocked
39
What is the presentation of webners stroke (2)?
* Ipsilateral CN 3 palsy (down and out)
* Contralateral hemiplegia (upper + lower)
40
What is Wallenberg stroke?
PICA infarct
*lateral medullary syndrome*
41
What is the presentation of Wallenberg stroke (4)?
* Ipsilateral spinothalamic face loss (pain + temp)
* Contralateral spinothalamic limb/ torso loss
* Nystagmus
* Ataxia
42
What is a basilar artery stroke known as?
Locked in syndrome
*carries blood to brain stem*
43
How does a retinal artery stroke present?
Amaurosis fugax
*transient loss of vision*
44
How does a Benedikt stroke present?
Wallenberg + **gait disturbance** (red nucleus infarct)
45
How does an AICA stroke present?
Wallenberg + hearing loss + ipsilateral facial paralysis
*no dysphasia*
*lateral pontine syndrome*
46
What tool can be used to assess the likelihood of a stroke (2)?
* FAST (community)
* ROSIER tool (ED)
47
How is a patient presenting with a stroke managed (5)?
* BG testing (exclude hypoglycaemia)
* Immediate NCCT head
* Aspirin 300mg (for 2 weeks)
* Thrombolysis < 4.5 h
* Thrombectomy < 6h
* Consider diffusion weighted MRI
* Carotid doppler USS
48
How is a TIA managed (3)?
* Aspirin 300mg (daily)
* Referral for specialist assessment **within 24 hours** (or within 7 days if > 7 days since symptoms resolved)
* Diffusion weight MRI scanning
49
What are the specialist treatments offered to those with an ischemic stroke (2)?
* Thrombolysis (alteplase)
* Thrombectomy
50
How should blood pressure be managed in those with ischemic strokes?
Only treated in hypertensive emergency or to reduce risks associated with IV thrombolysis
*reducing BP can reduce perfusion to brain*
51
What are some complications of those with ischemic stroke (5)?
* Haemorrhagic transformation
* Cerebral oedema
* Seizures
* Aspiration pneumonia
* Long-term complications
52
What is haemorrhagic transformation in those with ischemic stroke?
Damaged tissue during ischemic stroke allows bleeding from the brain
53
What underlying causes of stroke are assessed in those who have had a TIA/ stroke (2)?
* AF (using ECG)
* Carotid artery stenosis (using USS)
54
How can carotid artery stenosis be managed (2)?
* Carotid endarterectomy (when > 70% stenosed)
* Angioplasty (balloon dilation) and stenting
55
When is thrombolysis offered to those with stroke?
**4.5 hours** within onset of symptoms (slightly different for those who have woken up with stroke)
56
What are some contraindications to thrombolysis (4)?
* Previous haemorrhagic stroke
* Recent major surgery
* Active internal bleeding
* Uncontrolled hypertension
57
When is thrombectomy offered for those with a stroke?
Usually within **6 hours**, sometimes up to 24 hours if imaging is favourable
58
How are strokes/ TIAs managed long term (3)?
* Clopidogrel daily - P2Y12 inhibitor (or aspirin + dipyridamole)
* Atorvastatin
* BP + diabetes control
59
What are the 4 types of intracranial haemorrhage?
* Extradural haemorrhage
* Subdural haemorrhage
* Subarachnoid haemorrhage
* Intracerebral haemorrhage
60
Which intracranial haemorrhage constitute a stroke (2)?
* Subarachnoid haemorrhage
* Intracerebral haemorrhage
61
Where does a subdural haemorrhage bleed between?
Dura mater and arachnoid mater
62
Where does an extradural haemorrhage bleed between?
Skull and dura mater
63
What shape is a subdural haemorrhage on CT?
Banana shaped
64
What shape is a extradural haemorrhage on CT?
Lemon shaped
65
What percentage of strokes do intracerebral and subarachnoid haemorrhages account for?
10-20%
66
What are some risk factors for intracerebral haemorrhage (7)?
* Head injuries
* Hypertension
* Aneurysms
* Anticoagulants
* Bleeding disorders/ thrombocytopenia
* Brain tumours
* Alcoholism
67
What are the signs/ symptoms of intracerebral haemorrhage (5)?
* Headache
* Seizures
* Vomiting
* Reduced GCS
* Focal neurological symptoms
68
What values is the GCS between?
3-15
69
What are the scores allocated to eyes in GCS (4)?
* Spontaneous opening
* Speech
* Pain
* No response
70
What scores are allocated to verbal response in GCS (5)?
* Orientated
* Confused
* Inappropriate words
* Incomprehensible sounds
* No response
71
What scores are allocated to motor response in GCS (6)?
* Obeys command
* Localises pain
* Withdraws from pain
* Abnormal flexion to pain
* Extension to pain
* No response
72
What GCS score needs intubation?
8/15 or less
73
Damage to which blood vessel usually causes an extradural haemorrhage?
Middle meningeal artery
74
Damage to which region of the head often results in damage to the middle meningeal artery and therefore an extradural haemorrhage?
Temporal bone fracture
75
What age patient is usually affected by extradural haemorrhage?
Younger patients (20-30)
76
What is the typical presentation of a patient with extradural haemorrhage?
Head trauma and ongoing headache initially with symptoms improving then rapid worsening
77
Damage to which blood vessel usually causes an subdural haemorrhage?
Bridging veins
78
What age is typically affected by a subdural haemorrhage (2)?
* Older patients
* Babies (shaken baby)
79
What are some risk factors for subdural haemorrhage (3)?
* Head trauma
* Dementia
* Alcoholism
*both cause brain atrophy - causing arachnoid to pull away from dura
80
What time period do subdural haemorrhages usually come on over?
Longer time period than extradural as usually caused by bleeding from veins
81
How are subdural and epidural haemorrhages managed (3)?
* Correct clotting abnormalities, stabilise BP
* Craniotomy
* Burr holes
82
How does an intracerebral haemorrhage present?
Similar to an ischemic stroke but with a headache
83
What is the presentation of a patient with a SAH?
Sudden onset occipital headache 'thunderclap headache'
84
What are some other symptoms of SAH other than headache (3)?
* Meningism (hhotophobia, neck stiffness, vomiting)
* Raised ICP Sx (CN3, 6 palsy, cushings triad)
* Neurological symptoms
85
What are some risk factors for SAH (12)?
* Hypertension
* Smoking
* Alcohol
* Cocaine
* Family history
* Sickle cell
* Connective tissue disorders
* ADPKD
* Neurofibromatosis
* Aged 45-70
* Women
* Black
86
How is a SAH investigated?
CT head = hyperlattenuation in SAS
*most reliable within 6 hours*
87
What can be performed if CT head is negative for SAH?
Lumbar puncture
88
How long after symptoms should a lumbar puncture be performed for a SAH?
More than 12 hours
89
What would a lumbar puncture show for those with SAH (2)?
* Xanthocromia
* Raised red cell count
90
How can the source of the bleeding in a SAH be located?
CT angiogram
91
How is a SAH managed (2)?
* Aneurysm coiling or clipping
* Neurosurgery
92
What are 2 complications of a SAH?
* Vasospasm
* Hydrocephalus
93
How is vasospasm treated in those with SAH?
Nimodipine (CCB)
94
How is hydrocephalus treated (in those with SAH) (2)?
* Therapeutic lumbar puncture
* Shunt (ventriculoperitoneal/ external)
95
How is raised intracranial pressure treated in those with intracranial haemorrhage?
Mannitol
96
What is the most common cause of a head injury in the UK?
* **RTA** = mc
*also alcohol + drugs*
97
What criteria might suggest a patient with a head injury would need a scan (6)?
* Persistently reduced GCS (<15)
* Vomited on more than 1 occasion
* Seizure/ focal neurological deficit
* Battle sign (fluid from ear/ nose)
* > 65
* Dangerous mechanism of injury
98
A fracture in which part of the skull may cause CSF to leak from nose?
Basilar skull fracture (base of skull)
99
What are some complications of head injuries (4)?
* Epilepsy
* Mood disorders
* Personality changes
* Focal neurological deficits
100
What is dementia?
Progressive condition that causes irreversible impairment in memory, cognition, communication or personality
101
What are the main types of dementia (4)?
* Alzheimers (60%)
* Vascular dementia
* Lewy body dementia
* Fronto-temporal dementia
102
What term is often used to describe symptoms similar to dementia but not as severe?
Mild cognitive impairment
103
What age would be considered early onset for dementia?
< 65
104
What are some differential diagnoses for dementia (8)?
* **Delirium**
* Medication use
* Psychiatric conditions e.g. depression/ **bereavement**
* Parkinsons
* Brain tumours
* Hyper(para)thyroidism
* Cushings
* Nutritional deficiencies
105
What medications can cause symptoms of dementia in particular (3)?
* Anticholinergics (urological drugs e.g. Oxybutynin)
* Antihistamines
* TCA
106
What nutritional deficiencies may cause dementia like symptoms in particular (2)?
* B12 deficiency
* Thiamine (B1) deficiency - Wernicke-Korsakoff syndrome
107
What are some risk factors for developing dementia (5)?
* Sedentary lifestyle
* Lack of mental stimulation
* Obesity
* High BP
* High BG
108
What are 2 specific risk factors for the development of Alzheimers?
* Downs syndrome
* Family history
109
What are the general signs/ symptoms of dementias (4)?
* Forgetful (events/ names)
* Asking same questions
* Difficult remembering words
* Impaired decision making
110
What is the general progression of symptoms in dementia?
Slow and insidious worsening of symptoms and ability to function
111
What are the prominent features of Alzheimers?
* **Agnosia** - can't recognise
* **Apraxia**
* **Aphasia**
* **Dysphagia**
112
What is the pattern of memory loss in Alzheimers?
Short term memory worse affected
113
What is a prominent feature of vascular dementia?
Stepwise decrease in functioning/ symptoms
*symptoms vary depending on the area of the brain affected*
114
What type of person is typically affected by vascular dementia?
Stereotypically unhealthy patient - overweight, smokes, drinks
115
What is a prominent feature of fronto-temporal dementia (2)?
* Frontal = thinking and memory
* Temporal = speech and language
116
What is a prominent feature of Lewy body dementia (2)?
* Parkinsonism
* **Hallucinations**
117
What is the pathophysiology of Alzheimers dementia?
Beta amyloid proteins accumulate as plaques and tau neurofibrillary triangles --> death of brain cells
118
How is Alzheimers managed with medication (2)?
* Cholinesterase inhibitors
* Memantine
119
What are 3 examples of cholinesterase inhibitors used for Alzheimers?
* Galantamine
* Donepezil
* Rivastigmine
120
What causes Lewy body dementia?
Lewy body accumulation in the cortex
121
What is the main constituent of Lewy bodies?
Alpha synuclein
122
What is Lewy body dementia an umbrella term for?
* Dementia with Lewy bodies (memory affected first)
* Parkinsons disease dementia (movement affected first)
123
What is the difference between Parkinson's disease and dementia with Lewy bodies?
Likely due to the location of accumulation of Lewy bodies
*considered a spectrum of disorders*
124
The collection of which protein leads to fronto-temporal dementia?
Tau protein/ pick body
125
What sometimes causes frontotemporal dementia?
Autosomal dominant mutation in tau protein
126
What is frontotemporal dementia associated with?
**ALS** (15-20% of those with ALS develop FTD)
127
How are those with dementia initially managed in primary care (2)?
* Exclude physical cause (bloods, CXR MSU)
* Referral to memory clinic
128
How are those with dementia investigated in secondary care (2)?
* ACE (addenbrookes cognitive examination)
* MRI
129
What would an MRI show in those with dementia (2)?
* **Atrophy** of brain
* Lack of structural pathology
130
How are those with dementia managed (5)?
* Lasting power of attorney
* Advanced decisions
* Antidepressants
* Antipsychotics (risperidone)
* Benzos (for crisis management)
131
What are some associated behavioural and psychological symptoms of dementia (6)?
* Depression
* Anxiety
* Agressions
* Agitation
* Hallucinations
* Sleep disturbances
132
What is delirium?
Acute fluctuating disturbance in mental function
133
What are the signs/ symptoms of delirium (6)?
* Agitation
* Confusion
* Drowsiness
* Hallucinations
* Delusions
* Memory problems
134
What are the causes of delirium?
* **P**ain
* **I**nfection
* **N**utrition
* **C**onstipation
* **H**ydration status
* **M**edications
* **E**nvironvment/ electrolytes
135
What medications commonly cause delirium (5)?
* Benzos
* Opioids
* Anticholinergics
* Dopaminergics
* Steroids
136
What might make a diagnosis of delirium more likely in terms of presentation (3)?
* Hallucinations (esp. visual)
* Altered consciousness (hyperalert/ drowsiness)
* Agitation more common (looking round room, fidgety)
137
What is the second most common neurodegenerative condition after dementia?
Parkinsons disease
138
What is the underlying cause of parkisnons disease?
Loss of dopamine producing neurones in the substantial nigra pars compacta
139
What protein accumulation causes the death of these neurones in PD?
Alpha synuclein aggregating to form Lewy bodies
140
What is the mechanism by which parkinsons makes it more difficult to initiate movement?
Decrease in nigrostriatal pathway neurones --> less GABA-ergic inhibition (via indirect pathway) --> gross cortex inhibition --> more difficult to initiate movement
141
What are some risk factors for PD (3)?
* Family history
* Male (2:1)
* Older age
142
What is protective against PD?
Smoking
143
What medications can cause parkinonism or exacerbate parkinsons (2)?
* Antipsychotics (especially typical)
* Metoclopramide (antiemetic)
144
What is a feature of drug induced parkinsonism?
Bilateral resting tremor
145
What are the 4 typical symptoms of parkinsons disease?
* **Bradykinesia**
* Resting tremor
* Rigidity
*+ Postural instability (not part of the triad, but a key symptom*
146
What is the typical feature/ presentation of symptoms in PD?
Asymmetrical symptoms with one side affected much more than the other
147
What are some symptoms of PD when walking (3)?
* Stooped posture
* Reduced arm swing
* Shuffling gait
148
What are two types of rigidity found in parkinsons disease?
* Cogwheel = jerky
* Lead pipe = smooth tremor throughout
149
What are some other features of PD other than the 4 main features (7)?
* Depression
* Sleep disturbance (REM sleep disorders)
* Postural instability
* Loss of sense of smell
* Cognitive impairment/ memory problems
* Constipation
* Autonomic symptoms (e.g. postural hypotension)
150
What are the features of a tremor in parkinsons disease (3)?
* 4-6 hertz (pill rolling)
* Asymmetrical (worse on one side)
* Resting
151
What is a key differential to the tremor seen in PD?
Benign essential tremor
152
What are the features of benign essential tremor when compared to PD?
* Higher frequency 6-12 Hz
* Intention tremor
* Symmetrical
* Improves with alcohol
153
How is PD diagnosed?
Clinically by a specialist
154
How is more mild PD treated (3)?
* Da agonist
* Monoamine oxidase - B inhibitors
* Levodopa
155
What is an example of a dopamine agonist used for PD?
Bromocriptine
156
What is an example of a MAOB-i used for parkinsons?
Selegiline
157
How do MAOB-i work?
Prevent the breakdown of dopamine
158
What is a complication of long term use of dopamine agonists?
Pulmonary fibrosis
159
What medication is used in more severe PD?
Levodopa
160
What medications are levodopa combined with to prevent its breakdown/ metabolism (2)?
* COMT inhibitors
* **Peripheral decarboxylase inhibitors** - always combined with this
161
What is an example of a COMT inhibitor?
Entacapone
162
What is an example of a peripheral decarboxylase inhibitor?
Carbidopa
163
What treatments are offered to those with PD where initial medications are ineffective (2)?
* Deep brain stimulation
* Apomorphine (dopamine agonist) sub cut infusion
164
What is a side effect of levodopa (4)?
* Postural hypotension
* Confusion
* Psychosis
* Dyskinesia/ chorea
165
What can be given for those with PD who develop dyskinaesia due to levodopa?
Amantadine (glutamate antagonist)
166
What is a phenomena seen in those who take levodopa for a long time?
"On-off phenomena" motor fluctuations throughout the day
167
Overall what is the typical treatment of parkinsons (3)?
1. Levodopa + decarboxylase inhibitor (co-careldopa)
2. COMT inhibitors, DA agonists
3. DBS
168
What two conditions are similar to PD but important differentials especially in those not responding to treatments?
* Progressive supranuclear palsy (PSP)
* Corticobasal degeneration
169
How is essential tremor treated (5)?
* Propanolol = 1st
* Primidone
* Gabapentin
* Botox injections
* Beep brain stimulation
170
What are 3 primary causes of headaches?
* Migraine
* Tension headache
* Cluster headache
171
What are some other (secondary) causes of headache (8)?
* Meningitis/ infection
* Sinusitis
* **Giant cell arteritis**
* Intracranial haemorrhage
* **Medication overuse headache**
* Glaucoma
* Pre-eclampsia
* Raised ICP
* **Trigeminal neuralgia**
172
What is the most common type of primary headache disorder?
Tension headache
173
What type of pain is felt in tension headaches?
Bilateral tight band around sides of head - temples, occipital region and neck
174
What are other associated symptoms with a tension headache?
There are not really any
*no N+V, motion sickness or visual changes*
175
What are some triggers for tension headaches (5)?
* Stress
* Alcohol
* Dehydration
* Stagnant head/ neck position
* Lack of sleep
176
How can the symptoms of tension headaches be treated medically?
Analgesics - paracetamol, Ibuprofen
177
What can be used to treat for chronic/ frequent tension headaches?
Amitriptyline
178
What is the most common type of recurrent primary headache disorder?
Migraine
179
Which gender is most commonly affected by migraines?
Females
180
What age do migraines typically begin?
< 40 year olds
*most common in young adults/ adolescents*
181
What are the types of migraine (4)?
* Migraine with aura
* Migraine without aura
* Silent migraine
* Hemiplegic migraine
182
What is a silent migraine?
Migraine that does not cause a headache only an **aura**
183
What are the 5 stages of a migraine?
* Prodrome
* Aura
* Headache
* Resolution
* Postdrome
184
What does a prodrome refer to in the development of a migraine?
Change in mood/ fatigue up to days before an attack
185
What does an aura refer to in the development of a migraine?
Neurological changes in the minutes before an attack
186
What are the most common areas an aura may affect in the development of migraines (4)?
* Visual = MC
* Sensory (parasthaesia)
* Speech (dysphasia)
* Motor (weakness)
187
What are some common visual changes associated with migraines with aura (3)?
* Sparks in vision
* Blurred vision
* 'QR code' vision
188
What is a migraine that causes motor symptoms known as?
Hemiplegic migraine
189
What are the symptoms of a hemiplegic migraine (3)?
* Unilateral limb weakness
* Ataxia
* Reduced consciousness
190
What is an important condition to rule out in those with migraines and associated symptoms?
Stroke/ TIA
191
What can sometimes cause the abrupt end to symptoms of migraines (2)?
* Vomiting
* Sleeping
192
What is the types of pain experienced in a migraine?
Unilateral moderate - severe throbbing headache
*may come on quickly or gradually*
193
What are some associated symptoms with migraines (other than headache) (5)?
* Nausea/ vomiting
* Photo/phono/osmo phobia
* Aura
* Motion sickness
* Facial weekness
194
What are some triggers for migraines (8)?
* Stress
* Over/under sleeping
* Caffeine
* Chocolate
* Alcohol
* Dehydration
* Menstruation
* Exercise/ orgasm
195
How are migraines managed acutely first line?
Triptans (e.g. sumatriptan) + NSAID/ paracetamol
196
What is the mechanism of triptans?
Serotonin receptor agonist
*various effects including cranial vasoconstriction*
197
What are some contraindications for triptans (3)?
* Hypertension
* CAD
* Stroke
*things vasoconstriction would negatively affect*
198
How are migraines treated prophylactically (2)?
* 1st = **Propanolol**
* 2nd = TCAs e.g. amitriptyline
199
What is given as migraine prophylaxis first line in asthmatics?
Topiramate
200
What is important to check in females diagnosed with migraines?
Take them off oral contraceptive pill
201
What type of pain is felt in those with cluster headaches?
Severe unilateral pain, usually around the eye
202
What is the pattern of attacks seen in cluster headaches?
Periods of pain 15min - 3hours, 3-4 times per day, for 4-12 weeks, followed by a period of pain free lasting weeks, months or years
203
What are some risk factors for cluster headaches (4)?
* Male
* 30-50
* Genetics/ family history
* Smoking
204
What are some other features of cluster headaches other than pain (4)?
* Ptosis
* Red, swollen watery eye
* Rinorrhoea
* Miosis
205
How are cluster headaches treated acutely (2)?
* SC triptans (sumatriptan)
* High flow oxygen 100%
206
How are cluster headaches treated with prophylaxis?
Verapamil (CCB)
207
What is the presentation of a patient with trigeminal neuralgia?
Shooting/ stabbing/ electric shock pain usually on ONE side of the face
208
Where can trigeminal neuralgia affect?
Any combination of the trigeminal nerve branches:
* V1 ophthalmic
* V2 maxillary
* V3 mandibular
209
What condition is trigeminal neuralgia associated with?
Multiple sclerosis
210
What are some other risk factors for trigeminal neuralgia other than multiple sclerosis (2)?
* Female
* Older age
211
What is the treatment for trigeminal neuralgia?
* 1st = **Carbamazepine**
* Surgical intervention
212
What is the most common secondary headache?
Medication overuse headache
213
How many days per months should analgesia be taken for a headache to be termed a medication overuse headache?
**15/ 30 days**
*10/30 for opiates*
214
What often precedes sinusitis?
Viral infection
215
Where is pain felt in sinusitis?
Areas above the eyebrows and below the eyes
216
When might sinusitis need to be treated?
After 10 days
217
How is sinusitis treated (2)?
* Steroid nasal spray
* Penicillin V (phenoxymethylpenicillin)
218
What is giant cell arteritis?
Large cell vasculitis mainly affecting temporal artery
219
What are some risk factors for GCA (3)?
* Female
* >50 years
* Caucasian
220
What are the signs/ symptoms of GCA (3)?
* Jaw claudication
* Temporal tenderness
* Amaurosis fugax
221
How is GCA investigated (2)?
* Bloods = raised **ESR**
* Temporal artery biopsy = granulomas + skip lesions
222
How is GCA treated (2)?
* IV methylprednisolone (if vision loss)
* PO prednisolone (if no vision loss)
223
What is an epileptic seizure?
Transient episodes of abnormal electrical activity in the brain
224
What is another type of seizure (not epileptic)?
Non-epileptic seizure (functional seizure)
225
What are some causes of non-epileptic seizures (5)?
* Psychological
* Syncope
* Hypoglycaemia
* Arrhythmias
* Functional neurological disorder
226
What are two ways to differentiate non epileptic seizures from epileptic seizures?
* Epileptic = eyes open
* Non-epileptic = injuries less common
227
What defines epilepsy (in terms of when the seizures happen)?
2 or more unprovoked seizures that occur more than 24 hours apart
228
What are some causes of seizures (8)?
* Epilepsy (idiopathic)
* Vascular
* Infections
* Fever
* Metabolic/ electrolyte disturbances
* Trauma
* Eclampsia
* Autoimmune disease e.g. SLE
229
What are some conditions associated with epilepsy (5)?
* Brain tumours
* NFM1
* Dementia
* Cerebral palsy
* History of trauma/ infection
230
What are the features of an epileptic seizure (3)?
* Eyes open
* Synchronous movements
* Can happen at night
231
What is thought may be a cause of epilepsy?
Imbalances between the inhibitory GABA and excitatory glutamate neurotransmitters
232
How long does an epileptic seizure typically last?
< 2 minutes
233
What can lower the seizure threshold in those with epilepsy (4)?
* Hypoglycaemia
* Alcohol
* Stress + lack of sleep
* Medications (e.g. ciprofloxacin, lithium, TCAs)
234
What are the phases of epileptic seizures (4)?
* Prodrome
* Aura
* Ictal event
* Post-ictal period
235
What are symptoms of a prodrome before a seizure (3)?
* Changes in mood
* Changes in sensation
* Changes in behaviour
236
What are the symptoms of an aura associated with a seizure (5)?
* Change in smell/ taste
* Lip smacking
* Visual changes
* Sensory disturbances
* Deja vu
* Sudden emotional changes
*the beginning of the seizure - a focal seizure that then spreads to become a generalised seizure*
237
How long does a prodrome last before a seizure?
Days before the seizure
238
How long does an aura last before a seizure?
Minutes/ seconds
239
What are the symptoms associated with the post-ictal period after a seizure (4)?
* Confusion
* Drowsiness
* Headache/ body ache
* Emotional changes
240
How long does the post-ictal period last?
Minutes to hours
241
How are epileptic seizures classified depending on the part of the brain they affect (2)?
* **Generalised** - bilateral, often lose consciousness
* **Focal** - features confined to one region
242
What are the main types of generalised seizures?
* Tonic clonic
* Absence
* Myoclonic
* Tonic
* Atonic
* Clonic
243
What is the presentation of a patient with a tonic clonic seizure?
Loss of consciousness, muscles tense --> repeated jerking of muscles
244
What other symptoms may the patient have during a tonic clonic seizure (4)?
* Tongue biting
* Incontinence
* Groaning
* Irregular breathing
245
What is the presentation of a patient with an absence seizure?
Period of loss of consciousness and awareness for 10-20 seconds
246
Who is most commonly affected by absence seizures?
Children, most adults have grown out of them
247
What is the presentation of a myoclonic seizure?
Sudden brief muscle jerks affecting any part of the body, usually remain conscious
248
What is the presentation of atonic seizures?
Sudden brief loss of muscle tone, consciousness maintained
249
What is the presentation of tonic seizures?
Sudden stiffening of muscles, resulting in a fall if standing (around 20 seconds) - sometimes lose consciousness
250
What is the presentation of a clonic seizure?
Repeated rhythmic muscle jerking in the upper body/ face (last a few minutes) - sometimes lose consciousness
251
What is a feature of absence seizures on an EEG?
3 Hz spike
252
What are the two types of focal seizures?
* Simple
* Complex
253
What is the difference between a simple and complex focal seizure?
* Simple = remain aware
* Complex = lose awareness
254
What are the symptoms of a focal seizure in each hemisphere of the brain?
* Temporal = repeated movements + dysphasia
* Frontal = repeated muscle twitching/ paralysis
* Parietal = paraesthesia
* Occipital = visual changes
255
What two neurological phenomena sometimes occur with frontal lobe seizures?
* Todds paralysis
* Jacksonian marching (twitching along the motor homunculus)
256
What extra brain structure is often involved in complex focal seizures?
Basal ganglia
257
What are some differentials for epileptic-seizures (6)?
* Vasovagal syncope (fainting)
* Hemiplegic migraine
* Psychogenic non epileptic seizures
* Arrhythmias
* Hypoglycaemia
* TIA/ stroke
258
How is epilepsy investigated (5)?
* EEG
* MRI/ CT
* ECG
* Serum electrolyte
* BG
259
What blood marker is commonly raised in those with epileptic seizures?
Prolactin
260
How are generalised seizures treated (2)?
* Sodium valproate (in all except women who may become pregnant)
* Lamotrigine/ levetiracetam (for women of childbearing age)
261
How are focal seizures treated?
* 1st = Lamotrigine or levetiracetam
* 2nd = carbamazepine
262
What medication is first line in those with absence seizures?
Ethosuximide
263
What medication is first line for myoclonic seizures (2)?
* Male = sodium valproate
* Female = levetiracetam
264
What medication is first line for tonic or atonic (2)?
* Male = sodium valproate
* Female = lamotrigine
265
What are some other important steps to take in the management of people with epilepsy (2)?
* Cannot drive until seizure free for 1 year (must tell DVLA)
* Advise not to swim/ operate heavy machinery
266
In what way is sodium valproate teratogenic?
Increases likelihood of neural tube defects
267
What is a side effect of lamotrigine?
Stephen Johnson syndrome
268
What is a side effect of ethosuxamide?
Agranulocytosis (like clozapine)
269
What AED has lots of different side effects and isn't used very much any more?
Phenytoin
270
What is important to tell women on AEDs?
Lots of them interact with hormonal contraceptives
*the same goes with some other medications*
271
What is a severe complication of those who have seizures?
Status epilepticus
272
What is status epilepticus?
* Seizure lasting more than 5 minutes
or
* Multiple seizures without regaining consciousness
273
How is status epilepticus treated (5)?
1. ABCDE management
2. IV lorazepam/ other benzo
3. IV lorazepam (after 5 min)
4. IV levetiracetam/ phenytoin/ valproate
5. Phenobarbital/ general anaesthesia
274
What is multiple sclerosis?
Autoimmune condition causing demyelination in the CNS
275
What type hypersensitivity is MS?
Type 4
276
What cell is attacked in MS?
Oligodendrocytes
277
Who is typically affected by MS (2)?
* **Younger** adults especially
* **Women** (<40)
278
What phase is often used to describe MS and can be useful in radiological and clinical diagnosis?
Disseminated in space and time
*lots of parts of the body affected at different points in time*
279
What are some causes/ risk factors/ precipitating factors of MS (7)?
* Smoking
* EBV/ other infections
* Obesity
* Genetics
* Vitamin D deficiency
* Other autoimmune diseases
* Female
280
How quickly do symptoms of MS come on at initial presentation?
Over 24 hours
281
How long do symptoms last at initial MS presentation?
Days to weeks, then improve
282
What is the **most** common presentation of MS?
Optic neuritis
283
What are the symptoms/ signs of optic neuritis (4)?
* Unilateral reduced vision
* Pain (with eye movement)
* Loss of colour vision (especially red)
* Relative afferent pupillary defect
284
What is a relative afferent pupillary defect?
Pupil of affected eye constricts more when shining light in the contralateral eye than the affected eye
285
How should optic neuritis be managed?
* Urgent ophthalmology review
* High dose steroids
286
What are some other symptoms of MS other than optic neuritis (5)?
* Weakness (incontinence, limb weakness, Horners syndrome)
* Sensory symptoms (paraesthesia, trigeminal neuralgia)
* Eye movement abnormalities
* Ataxia
* Hyper-reflexia
* Intention tremor
287
What is a specific sensory sign seen in MS when flexing the neck?
Lhermittes sign
288
What other sensory sign is found in MS amongst other neurological conditions?
Transverse myelitis - site of inflammation in the spinal cord
289
What eye movement disorder sometimes present in those with MS (2)?
* Intranuclear ophthalmoplegia
* Horizontal lateral gaze disorder
290
What causes intranuclear ophthalmoplegia (INO)?
Lesion in the medial longitudinal fasciculus
291
What is the clinical feature of INO?
Impaired adduction on the same side of the lesion and nystagmus in the contralateral abducting eye
292
What is the clinical feature of horizontal lateral gaze disorder?
When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct
293
What is the group of 3 signs/ symptoms found in MS known as?
Charcots neurological triad
294
What is in charcots neurological triad?
* Nystagmus
* Dysarthria
* Intention tremor
295
What are two types of ataxia that may occur in MS?
* Sensory ataxia = due to loss of proprioception (causes +ve Rombergs test)
* Cerebellar ataxia
296
What are some other causes of optic neuritis other than MS (4)?
* SLE
* Sarcoidosis
* Infections e.g. syphilis, Lyme disease
* Neuromyelitis optica
297
What are the 4 patterns of disease in those with MS?
* Clinically isolated syndrome
* Relapsing-remitting
* Primary progressive (never has remission)
* Secondary progressive (started as relapsing-remitting
298
How is MS investigated (3)?
* MRI
* Lumbar puncture
* NCS = decreased velocity (due to demyelination)
299
What would be suggestive of MS on a lumbar puncture?
Oligoclonal bands
300
What criteria are sometimes helpful in the diagnosis of MS?
Mc Donald criteria
301
How are relapses of MS treated (2)?
* Methylprednisolone
* Plasmapheresis + IV Ig (if severe)
302
What sometimes precipitates a flare of MS?
Infections!!
303
How is MS managed in the long term?
Disease modifying drugs
304
What are some disease modifying drugs used to treat MS (3)?
* Natalizumab
* Glatiramer
* Beta interferon
305
What is a complication of some of the disease modifying therapies used in MS?
Progressive multifocal leukoencephalopathy (reactivation of the JC virus - living in glial cells)
306
What are some differential diagnoses for MS?
* Migraine with aura
* Stroke
* Hypoglycaemia/ hypothyroidism
* Infections
* Brain tumour
* **Neuromyelitis optica**
307
What is neuromyelitis optica?
Sister disease to MS with lots of similar symptoms but WORSE
*only affects eyes and cord (not brain)*
308
What are 3 anterior horn diseases?
* MND = mc
* Poliomyelitis
* Spinal muscular atrophy
* Anterior spinal artery infarct
309
What is the anterior horn of the spinal cord?
The place where motor information exits the spinal cord
310
What are the symptoms of anterior horn diseases?
Purely motor symptoms
311
What is usually the cause of an anterior spinal artery infarct (2)?
* AAA repair
* AD repair
312
What are the signs/ symptoms of an anterior spinal artery infarct (2)?
* Bilateral paralysis (below lesion)
* Bilateral loss of pain + temp
313
How is polio spread?
Faeco-oral spread
*VERY contagious*
314
What is a complication of polio?
Attacks anterior horns --> lower motor neurone signs + paralysis
315
What is spinal muscular atrophy?
Auto recessive condition causing floppiness that can present at various ages (including in newborns) with progressing muscle weakness
316
What is motor neurone disease?
A neurodegenerative disorder that selectively targets motor neurones leading to their degeneration through unknown mechanisms
317
What are some risk factors for motor neurone disease (4)?
* Genetics/ family history
* Exposure to toxic, viral, heavy metals
* Smoking thought to increase risk
* Male
318
What is the typical patient affected by MND?
60 y/o male
319
What gene mutation has been associated with MND?
SOD-1 gene mutation
320
What condition is MND linked to?
Frontotemporal dementia
321
What is the types of MND (4)?
* **Amytrophic lateral sclerosis**
* Progressive bulbar palsy
* Progressive muscular atrophy
* Primary lateral sclerosis
322
Which neurones are affected in ALS?
Upper and lower motor neurones
323
What are the signs/ symptoms of ALS (4)?
* Muscle weakness + waisting
* Muscle fasciculations
* Dysarthria/phagia
* Generalised fatigue
324
What might a patient complain of with ALS to a GP in the initial stages (3)?
* More clumsy
* Dropping things
* Tripping over
325
What are the two initial presentations found in those with ALS?
* Bulbar onset symptoms
* Limb onset symptoms
326
What is the prognosis for those diagnosed with ALS?
Poor typically 3-5 year survival
327
What nerves are affected in progressive bulbar palsy?
Lower motor neurones of cranial nerves 9, 10, 12
328
What are the signs/ symptoms of PBP (3)?
* Dysphagia
* Dysarthria
* Tongue atrophy
*worst prognosis*
329
What nerves are affected in progressive muscular atrophy?
Lower motor neurones
330
What are the signs/ symptoms of progressive muscular atrophy (3)?
* Muscle weakness
* Muscle atrophy
* Fasciculations
331
What nerves are affected in primary lateral sclerosis?
Upper motor neurones
332
What are the signs/ symptoms of primary lateral sclerosis (2)?
* Spasticity
* Weakness
333
How can progressive muscular atrophy be distinguished from ALS and PLS?
Absence of:
* Brisk reflexes
* Spasticity
* Babinski
334
How can primary lateral sclerosis be distinguished from ALS and PMA?
No muscle atrophy
335
How is MND diagnosed?
Clinically after excluding other conditions
*EMG (can be abnormal), nerve conduction and MRI can help*
336
How is MND managed (2)?
* Riluzole (can slow progression)
* Supportive care
337
How is spasticity in MND treated?
Baclofen
338
How is drooling in MND treated?
Hyoscine
339
What type of ventilation is often required in MND?
BiPAP
340
What is usually the most suitable way to feed people with MND?
Percutaneous endoscopic gastroscopy tube (PEG)
341
What symptoms are never found in those with MND (3)?
* Sensory Sx
* Eye Sx
* Sphincters never affected
342
What are the general signs/ symptoms of UMN lesions (3)?
* Increased reflexes
* Hypertonia / increased spasticity
* Babinski sign +ve (toes upward)
343
What are the general signs/ symptoms of LMN lesions (5)?
* Atrophy
* Fasciculations
* Reduced tone
* Reduced reflexes
* -ve Babinski
344
What is Huntington's chorea?
Progressive neurodegenerative genetic disorder characterised by gradual onset of involuntary movements
345
What is the inheritance pattern of Huntington's chorea?
Autosomal dominant
346
What gene and chromosome are affected in Huntington's?
HTT (huntingtin) gene on chromosome 4
347
What is the dysfunction of the DNA in huntingtons chorea?
Trinucleotide repeat disorder of CAG
348
How many repeats of CAG are required for people to develop huntingtons?
**36** or more
349
What changes in the development of huntingtons disease are seen from generation to generation?
Anticipation - symptoms present earlier and more severely
350
What are the signs/ symptoms of huntingtons chorea (6)?
* **Chorea**
* **Dementia **
* **Psychiatric issues/ depression**
* Dystonia - abnormal muscle tone --> abnormal postures
* Dysphagia/arthria
* Rigidity
* Eye movement disorders
351
What is usually the first symptoms of those with huntingtons?
Cognitive decline/ depression
352
What age does huntingtons usually present?
30 - 50
353
How is huntingtons investigated?
* Clinical suspicion e.g. symptoms + family history
* Genetic testing = diagnostic
354
How is huntingtons chorea managed (2)?
Symptomatic relief:
* **Tetrabenazine** for chorea
* Valproic acid for muscle twitching
355
What are the two most common causes of death in huntingtons?
1. Resp arrest
2. Suicide
356
What are two neuromuscular junction disorders?
* Myasthenia gravis
* Lambert eaton myasthenia disorder
357
What is myasthenia gravis?
Autoimmune condition attacking the neuromuscular junction
358
What is the pathophysiology of MG?
ACh receptor antibodies bind to postsynaptic ACh receptors, blocking them and preventing stimulation by ACh
359
As well as AChR antibodies what two other antibodies sometimes cause MG?
* Muscle specific kinase (MuSK)
* Low-density lipoprotein receptor-related protein 4 (LRP4)
360
What is another mechanism by which ACh receptor antibodies cause symptoms?
They activate the compliment system leading to cell damage at postsynaptic membrane
361
What are MuSK and LRP4 proteins important for the creation and organisation of?
Acetylcoline receptors
362
What is the key feature of those with MG?
**Fatiguability** - weakness that improve with rest/ worsens with activity
363
Why does MG improve with rest?
AChR are cleared from the postsynaptic ACh receptors when resting
364
What two groups of people are most commonly affected by MG?
* Men over 60 (with thyomas)
* Women under 40 (autoimmune)
365
Which two muscle groups are most commonly affected in MG?
* Small muscles of the head and neck
* Proximal muscles of the limbs
366
What are the signs/ symptoms of MG (5)?
* Ptosis
* Diplopia
* Dysphagia/arthria
* Weakness in limbs e.g. tripping over
* Fatigue in jaw when chewing
367
What condition is there a link to with MG?
Thymomas (30% develop MG)
368
How can you exacerbate symptoms of MG on a physical examination (3)?
* Repeated blinking exacerbates ptosis
* Prolonged upward gazing exacerbates diplopia
* Repeated abduction of one arm will result in unilateral weakness
369
How is MG investigated (3)?
* Antibodies tests (85% have AChR, 10 MuSK, 5 LRP4)
* CT/ MRI thymus (to look for thymoma)
* Edrophonium test if any doubt
370
How does edrophonium test work in MG?
Edrophonium blocks cholinesterase --> more ACh available at postsynaptic receptor --> weakness of muscle improves
371
How is MG managed (4)?
* **Pyridostigmine** (cholinesterase inhibitor)
* **Immunosurpression** (e.g. azathioprine, prednisolone)
* **Thymectomy** - can improve symptoms even if no thymoma
* **Rituximab**
372
What is a serious complication of MG?
Myasthenia crisis
373
How can severity of myasthenic crisis be monitored?
Forced vital capacity (easy test involves counting numbers in 1 breath)
374
How is myasthenic crisis managed (3)?
* NIV/ mechanical ventilation
* IVIg (against AChR)
* Plasmapheresis
375
How long does MG last?
It is lifelong
376
What is Lambert eaton myasthenic syndrome?
Autoimmune condition affecting neuromuscular junction
377
What is the pathophysiology of Lambert eaton syndrome?
Antibodies are formed against voltage gated calcium channels and destroy them --> less ACh released into the synapse
378
What is the importance of voltage gated calcium channels in the NMJ?
They assist in the release of ACh
379
What condition is usually associated with cases of Lambert eaton myasthenic syndrome?
SCLC (paraneoplastic syndrome)
*can also occur as a primary autoimmune disorder*
380
What are the signs/ symptoms of Lambert eaton myasthenic syndrome?
* **Proximal muscle weakness** e.g. difficulty climbing stairs
* **Autonomic dysfunction** e.g. dry mouth, impotence
* **Reduced/ absent reflexes**
381
What is a key feature of Lambert eaton myasthenia syndrome?
Muscle strength improves after use
382
How is Lambert eaton myasthenic syndrome investigated (3)?
* Excluding SCLC (CT)
* Nerve conduction studies/ EMG
* Blood tests for voltage gated calcium antibodies
383
How is Lambert eaton myasthenic syndrome managed (3)?
* Diaminopyridine (amifampridine)
* Immunosurpression
* IVIg/ plamapheresis
384
How does diaminopyradine work?
Blocks voltage gated potassium channels in presynaptic membrane --> prolongs depolarisation --> assists calcium channels in carrying out their function
