Neurology Flashcards
(300 cards)
1
Q
What are red flags for headaches
A
New headache over >60yrs Thunderclap headache Hx of malignancy Hx of infectious disease Altered Consciousness, memory or confusion Seizure Papilloedema
2
Q
What is the most common type of chronic daily recurrent headache
A
Tension Headache
3
Q
How does tension headache present
A
bilateral non pulsatile headache +/- scalp tenderness
pressure or tightness around head
No N/V or photophobia
4
Q
What is the treatment for a tension headache
A
Explanation & Reassurance
Stress Relief
Simple Analgesia e.g Paracetamol or Ibuprofen
5
Q
What is one of the main issues of using long term analgesia to treat tension headaches
A
Analgesia overuse headaches - when you stop taking analgesia you get headaches from analgesia withdrawal
(paracetamol, codine/opiates, triptans)
6
Q
What medication can be tried to relieve chronic tension headaches
A
Tricyclic Antidepressents e.g Amitriptyline
7
Q
What are the two subtypes of Migraine
A
Migraine with aura
Migraine without aura
8
Q
How does a migraine without aura present
A
Unilateral throbbing building up over minutes/hours
Nausea and Vomiting
Photophobia/Phonophobia
(patients like to sit in dark and often irritable)
9
Q
What additional features would you see in migraine with aura and when does the aura begin
A
Aura presents before the headache (temporary warning)
Eyes: Scotoma, Unilateral Blindness, Flashes and zig-zags
Motor: Weakness
Sensory: Aphasia, tingling, numbness
10
Q
What are the triggers of Migraine
A
C hoclate H angovers O rgasm C heese O ral contraceptive L ie ins A lcohol T umult (loud noise) E xercise
11
Q
What are other premonitory changes may you get before migraine
A
Fatigue
Nausea
Change in mood or appetite
12
Q
What may differentiate a migraine from a stroke
A
Migraines usually have +ve symptoms whilst stroke -ve
13
Q
How can you manage migraines conservatively
A
avoid triggers
Usually resolve through sleep
14
Q
How can you manage a mild migraine
A
Simple analgesia + Anti-emetic e.g metoclopramide
15
Q
How can you manage a migraine which is unresponsive to simple analgesia or severe migraines
A
Triptans e.g Sumatriptan
contraindicated in vascular disease cause vasoconstriction
16
Q
What can frequent use of Triptans lead to
A
Analgesia overuse headaches
17
Q
What medication is used to prevent migraines
A
1st line: B-blockers e.g propanolol
2nd line: Topiramate or Amitriptyline (if B-blockers contraindicated)
18
Q
What is a cluster headache
A
Rapid onset, severe, short lived (1-2hrs) unilateral headache with a clustering of painful attacks over weeks/months followed by periods of remission
19
Q
Who is at risk of cluster headaches
A
Men
20 -50 yrs
20
Q
How does a cluster headache present
A
Short lived, severe unilateral headache Pain begins around eye and temple Lacrimation and redness of eye Rhinorrheoa Flushing
21
Q
How can you manage an acute attack of a cluster headache
A
SC Sumatriptan or Intranasal Sumatriptan
100% O2
22
Q
What can trigger cluster headaches
A
Alcohol
Strong smelling chemicals e.g perfume, petrol
Smoking
23
Q
How can you prevent cluster headaches
A
Avoiding triggers
Verapamil - Ca2+ channel blocker
Not effective: Corticosteroids, Lithium Carbonate
24
Q
What can cause secondary headaches
A
Subarachnoid Haemorrhage
Raised Intracranial pressure
Idiopathic Intracranial HTN
Medication/Analgesia Overuse Headache
25
How does Subarachnoid Haemmorhage headache present
Thunderclap headache - focal symptoms and signs, coma if severe
26
How does raised intracranial pressure present
Typically worse walking, lying, bending and coughing
Nausea/ Vomiting
Papilloedema
Focal signs
27
What causes idiopathic intracranial HTN
Caused by raised ICP
| risk factors: drugs, obesity
28
What is giant cell arteritis/ temporal arthritis
Chronic Vasculitis - characterisied by granulomatous inflmmation in the large arteries of the scalp and neck
29
What is granulomatous inflammation
Inflammation with granulomas (collection of macrophages attempting to wall off foreign substances)
30
What is GCA closely related to and therefore what other symptoms may present alongside GCA
Polymyaligia rheumatica - Fatigue and Pain, stiffness and inflammation of the shoulders, hip and neck
31
What are the clinical features of GCA
Headache (usually unilateral in temporal area but may become bilateral)
Scalp Tenderness (e.g when combing hair)
Jaw Claudication
Features of polymyaligia rheumatica - arm, neck and pelvic stiffness and tenderness
Partial or Complete Blindness in one or both eyes - usually permanent
Amaurosis Fugax - temporary painless loss of vision in one or both eyes
Systemic Features: weight loss, fatigue, low grade fever
32
What may you find on examination of someone with temporal arteritis
If its a superfical temporal artery it may be tender, firm and pulseless - the skin overlying may be red
33
When should you suspect GCA
Over 50 with either:
- new onset localised unilateral headache in temporal area
- temporal artery abnormality - tender, firm and pulseless
34
What confirms diagnosis of GCA
*Temporal artery biopsy confirms diagnosis - although granulomatous changes may be patchy and missed
Always raised ESR
35
How do you manage GCA
High dose oral steroids e.g Prednisolone
Aspirin
PPI to protect Gut e.g Omeprazole
36
What is trigeminal neuralgia
Severe episodic face pain in the distribution of one or more branches of the trigeminal (5th) nerve
37
What are the clinical features of trigeminal neuralgia
Severe paroxysms of sharp/knife like pain in one or more divisions of the 5th nerve
38
What 'trigger factors' precipitate trigeminal neuralgia attacks
Light touch to the face
Washing
Shaving
Eating
39
What is the most common cause of trigeminal neuralgia
Vascular compression of the nerve - main cause
| Rare causes: MS, tumours
40
What are risk factors for developing trigeminal neuralgia
Increasing age
MS
Family Hx
41
How is trigeminal neuralgia managed
Check for red flags: tumours, MS, aneurysms
| Carbamazepine
42
What is a transient ischaemic attack
A transient episode of neurological dysfunction caused by temporary occlusion of cerebral circulation usually an emboli (less than 24hrs)
43
What is a stroke
Rapid onset of neurological dysfunction caused by infarction or haemorrhage in the brain lasting more than 24 hrs
44
What are the two types of stroke
Haemmorhagic Stroke - caused by intracerebral or subarachnoid haemorrhage
Ischaemic Stroke - caused by infarct
45
What two things can cause an ischaemic stroke
- Thrombus - occurs at sight of atheromatous plaque in internal carotid, vertebral, cerebral arteries
- Embolus - occurs from atheromatous plaque of the internal carotid artery breaking off or emboli from the heart e.g AF
46
What are TIAs usually caused by
Microemboli from atheromatous plaques of the internal carotid or from the heart e.g AF
47
What are risk factors for stroke/TIA
```
HTN
Diabetes
Smoking
Cardiovascular Disease
Hyperlipidaemia
Obesity
Oestrogen oral contraceptives
Alcohol
AF - major risk for emboli stroke
Rarer: Cocaine, Migraine, Vasculitis
```
48
What does the Frontal lobe control
```
Movement
Executive Function (cognitive control and behaviour)
```
49
What does the Parietal lobe control
Sensory information
50
What does the temporal lobe control
```
Hearing
Memory
Smell
Languages
Facial Recognition
```
51
What does the occipital lobe control
Vision
52
Whats does the cerebellum control
Balance and Co-ordination
53
What does the brain stem control
Heart Rate and BP
Breathing
GI function
Consciousness
54
Where is Broca's area and what does it do
It is located mainly in the left hemisphere of the frontal lobe
It is the area which controls speech production
55
What is Wernickes area and what does it do
It is located mainly in the left hemisphere of the temporal lobe
It is the area which controls understanding speech
56
What two groups of arteries supply the brain
```
Internal Carotid Arteries (L&R)
Vertebral Arteries (L&R) - come together to form basillar artery
```
57
What does the Internal carotid artery/Anterior circulation supply
Anterior Cerebral Artery - median portions of frontal and parietal lobes
Middle Cerebral Artery - lateral portions of frontal, parietal and temporal lobes
58
What does the Vertebral Artery/Posterior circulation supply
The Cerebellum
The Brainstem
The Posterior Cerebral Artery - supplies the occipital lobe, some of the temporal lobe and the thalamus
59
How can stroke be classed
Oxford Stroke (Bamford) Classification
60
What is the presentation of a total anterior circulation stroke
All three of the following:
- Unilateral weakness &/or sensory deficit of the face, arm and leg
- Homonymous Hemianopia
- Higher Central Dysfunction (dysphasia, visuospatial disorder)
61
What is the presentation of a partial anterior circulation stroke
Two of the following: - Unilateral weakness &/or sensory deficit of the face, arm and leg
- Homonymous Hemianopia
- Higher Central Dysfunction (dysphasia, visuospatial disorder)
62
What is a lacunar stroke
Occlusion of deep penetrating arteries of the brain
It is the most common type of stroke
It only affects a small amount of subcortical white matter therefore does not present with cortical features
63
What is the presentation of a lacunar stroke
One of the following:
- Pure Sensory Stroke
- Pure Motor Stroke
- Sensori-Motor Stroke
- Ataxia hemiparesis
64
What is the presentation of a posterior circulation syndrome
One of the following:
- Ipsilateral cranial nerve palsy and a contralateral motor/sensory deficit
- Bilateral motor/sensory deficit
- Conjugate eye movement disorder (gaze palsy)
- Cerebellar Dysfunction e.g ataxia, nystagmus, vertigov
- Isolated homonymous hemianopia or cortical blindness
65
What is the Acronym to recognise stroke
F ace
A rm
S peech
T ime
66
What is the initial management of someone who presents with a stroke
1. FAST
2. ABCDE
3. Bloods + BM
4. Breif Hx and Examination (time of onset, risk factors, contraindications for thrombolysis)
5. BP and ECG
6. NIHSS (national institute of health stroke scale) - to grade severity
67
What is the key investigation for stroke
Urgent CT head (+/- CT angiography)
| - Sensitive for haemorrhage, cannot usually diagnose stroke in the acute phase)
68
What is the emergency treatment for an ischaemic stroke once haemmorhagic stroke has been excluded
Thrombolysis e.g IV alteplase +/- Mechanical Thrombectomy
| Aspirin
69
What is the timeframe for using thrombolysis
4.5 hrs from onset of symptoms
70
What are contraindications for thrombolysis
```
Haemorrhage on CT
Active bleeding from any site
recent GI or urinary tract haemorrhage
Suspected known pregnancy
Active pancreatitis
Blood Pressure 185/110
```
71
What are the risks of thrombolysis
severe high blood pressure
bleeding
Haemorrhagic stroke Transformation
72
What is it essential to do post thrombolysis care
Aggressive blood pressure monitoring
Vigilance for complications
24hr CT head to check for haemmorhagic transformation
73
What is the timeframe for mechanical thrombectomy
6 hr time frame for Anterior circulation stroke (longer for posterior)
74
Can Mechanical Thrombectomy be used alongside IV Thrombolysis
Yes
75
What is the disadvantage of Thromectomy
It is a limited resource
76
Following Thrombolysis how should ischaemic stroke patients be managed
1. Investigate the cause
2. Screen and prevent further complications
3. Rehabillitation
4. Manage secondary prevention
77
What investigations should be performed following thrombolysis
Blood Tests:
FBC, ESR, U&E, Lipid Profile, LFTs, CRP, Clotting screening, Glucose
ECG: MI, Atrial flutter/fibrillation
Carotid Dopper US - carotid stenosis
Echocardiogram
MRI - confirms diagnosis of ischaemic strome
78
Who needs to be in the MDT to provide supportive care following a stroke
```
Nursing
SALT
OT
Physiotherapy
Dieticans
```
79
What lifestyle changes can be used for secondary prevention of further strokes/TIAs
```
Smoking cessation
Drinking and drugs cessation
Dietary modifications
Exercise
Driving Advice
```
80
What medication can be used for secondary prevention of further strokes/ TIAs
Antiplatelets e.g Clopidogrel superior to Aspirin
Anticoagulation if AF using CHADVASC score e.g warfarin
Antihypertensive Drugs to lower BP e.g B-blockers
Lower Cholesterol using Statin e.g Simvastatin
81
What medical management can be performed to prevent further complications of stroke
```
Prevention of DVT e.g TED stockings
Hydration
NG feeding/ PEG feeding
Botox and Physio for Spasticity
Monitor for infection
```
82
What further surgical management may be performed to prevent further strokes/TIAs causes by carotid stenosis
Carotid Endarterectomy
| Carotid Artery Stenting
83
What is the CHA2DS2 VASc Score
Estimates risk of stroke in AF patients
All worth one point accept A2 and S2
```
C HF
H ypertension
A2 ge 75 or other
D iabetes
S2 troke/TIA
```
V ascular Disease
A ge 65-74
Sc sex category
```
0 = low risk no anticoagulation
1 = moderate risk consider antiplatelet or anticoagulation
2 = anticoagulation candidate
```
84
What is the ABCD2 score
Estimates the risk of a stroke following a TIA
A ge (60 or over)
B lood pressure (140/90 or greater)
C linical features (unilateral weakness +/- speech impairment = 2, only speech impairment = 1)
D uration of symptoms (60 mins or longer =2, under 60 mins = 1)
D iabetes
85
What are the two types of haemmorhagic stroke
Subarachnoid Haemorrhage
| Intracerebral Haemorrhage
86
What is an intracerebral haemorrhage and its presentation
A bleed within the brain tissue itself
| headache and neurological deficit
87
What is a subarachnoid haemorrhage and its presentation
A bleed within the subarachnoid space usually caused by saccular aneurysms
Symptoms:
Thunderclap headache
meningeal symptoms (neck stiffness, vomiting and photphobia)
Painful 3rd cranial nerve palsy
Horners Syndrome
Reduced GCS - can lead to seizures, collapse and sudden death
88
How is haemorrhagic stroke diagnosed
MRI/CT
| Cerebral Angiography - to rule out anurysmal cause and locate anuerysm
89
In a suspected SAH what other investigation would you like to perform if the CT is -ve
LP - can be performed after 12 hrs after onset and can be detected up to 2 weeks after - will show xanthochromia
90
What is the emergency management of a haemmorhagic stroke
ABCDE
Control BP try and keep systolic between 140-160 no higher
Stop all anticoagulation and reverse any anticoagulation (Vit K for Warfarin and Protamine in Heparin/partally LMWH)
Manage underlying malformation - SAH aneurysm surgical clipping or endovascular coiling is definite management
Evaluate for neurosurgery - if continual bleeding causing brainstem compression and hydrocephalus in intracerebral haemorrhage - perform haematoma evacuation
91
What other conditions mimic strokes
Seizures
Tumours/Abscesses
Migraine
Metabolic (hypoglycaemia, hyponatramia)
92
What is the treatment for a SAH
Surgical clipping
| Endovascular Coiling
93
What are the risk factors fo haemorrhagic stroke
Smoking
HTN
Alcohol Access
Increasing age
94
What is a subdural haemorrhage
accumulation of blood due to rupture of bridging veins in the subdural space between dura and arachnoid
95
What is the pathophysiology behind subdural haemorrhage
Bleeding causes ICP to gradually rise causing shifting of midline structures away from clot
If left untreated eventual tentorial herniation and coning
96
What is the cause of a subdural haemorrhage and who is at risk of developing one
- Brain atrophy leads to tearing of bridging veins usually only from minor head trauma
- Patients with brain atrophy high risk - alcoholics and the elderly
97
How does subdural haemorrhage present
```
A progressively worsening headache
Fluctuating levels of consciousness
Confusion
Personality change
Sleepiness
Raised ICP
seizures
```
98
When can a subdural haemorrhage present
Acute - if severe head injury
| Subacute/ Chronic - over days and weeks if minor head injury
99
What could be a differential diagnosis of a subdural haemorrhage
Stroke
Dementia
Infection
100
What are the symptoms of raised ICP
Papilloedema
Vomiting
Headache
Deterioration on level of consciousness
101
What investigation would you use for suspected subdural haemorrhage and what would you see if it is a subdural haemorrhage
CT/MRI
| Show concave collection of blood (sickle shape) +/- midline shift
102
How would you treat a subdural haemorrhage
Surgical evacuation of haematoma e.g burr hole craniotomy
103
What is an extradural/epidural haemorrhage
A bleed between the bone and dura
104
How is an extradural haemorrhage usually caused
fractured temporal or parietal bone causing laceration of middle meningeal artery typically after trauma to temple
105
How do extradural haemorrhage patients present
Triphasic:
1. Brief deterioration in consciousness
2. Lucid phase where they appear to recover (can last hours)
3. Rapid deterioration - headache, falling GCS, raised ICP, vomiting, confusion, fits, hemiparesis with brisk reflexes, compression of 3rd nerve causing fixed dilated pupil, coma, breathing deep irregular (brainstem compression) - Death
106
What investigation is key for suspected Extradural haemorrhage
CT - Convex haematoma (Egg shape)
| X-ray may show skull fracture
107
What is the management of an extradural haemorrhage
Stabilse and surgical evacuation/ drainage of bleed (craniotomy)
108
What is the difference between an extradural and subdural on CT
Extradural - Egg shaped (convex)
| Subdural - Sickle shaped (concave)
109
What is Guillain -Barre Syndrome
An acute inflammatory demyelinating peripheral polyneuropathy
110
What is Guillain - Barre Syndrome usually cause by
Usually triggered by an infection
- Campylobacter jejuni
- EBV
- Cytomegalovirus
111
What are the clinical features of GBS
Symptoms are toes to nose and symmetrical:
- Progressively worsening limb weakness (starting in the hands and feet and spreading upwards) - eventual flaccid weakness
- Paresthesias (tingling/numbness) - starting in hands and feet snd spreading upwards
- Absent Reflexes
- Eventual Paralysis of Respiratory muscles leafing to life-threatening respiratory failure
- Autonomic Dysfunction: postural hypotension, cardiac arrythmias, sweating, flushing and urinary retention
112
At what rate does GBS reach maximum weakness
3 -4 weeks
113
What is miller fisher syndrome
A variant of GBS affecting the cranial nerves leading to opthalmoplegia and ataxia
114
What investigations should you do for suspected GBS
Clinical
Nerve Conduction Studies: F waves slow/absent, reduced motor conduction velocity
CSF: Usually protein is raised but may be normal
MRI to exclude cord compression
115
What is the management of GBS
Monitoring respiratory weakness - FVC, RR may need mechanical ventilation
ECG and BP - cardiac monitoring of arrhythmias and hypotension
Supportive Treatment - pain management opiates, physiotherapy and VTE prevention (heparin TED stockings)
Immunotherapy - IVIG or plasma exchange
116
What is the most commonly used treatment for GBS
IVIG - to reduce duration and severity of symptoms
| Plasma exchange - may be more side effects than IVIG
117
What is a seizure
A transient event caused by the abnormal and excessive discharge of cerebral neurones
118
What is epilepsy
An increased tendency to experience recurrent unprovoked epileptic seizures
119
What type of onset can a seizure take
Generalised onset - affect whole brain
| Focal/Partial onset - affect one part of brain may become generalised
120
What types of seizures are generalised onset
Generalised Tonic- Clonic Seizure
Absence Seizure
Myoclonic jerk seizures
121
What types of seizure are partial/focal onset
Simple partial seizures
Complex partial seizures
Secondary Generalised Tonic Clonic Seizures
122
What is a generalised tonic clonic seizure (grand mal)
- Sudden onset rigid (tonic phase) followed by convulsion (clonic phase) and rhythmic muscle jerking
- Tongue Biting
- Urinary Incontinence
- After feel drowsy, pale, confused for several hours
123
What is an absence seizure (petit mal)
- Usually disorder of childhood
- Child ceases activity and stares blanky into space
- Lasts a few seconds
124
What is a myoclonic seizure
When some or all of muscles in body start jerking
| Usually aware of event
125
What is a simple partial/focal seizure
Presents with aura symptoms you are fully aware of what is happening
- Hallucinations (vision, smell)
- Motor movements (stiffness, twitching)
- Sensory disturbances (tingling, numbness)
- Rising sensation in stomach
- Feeling strange
126
What can simple focal/partial seizures be a warning of
The development of a bigger seizure - secondary grand mal
127
What is a complex focal/partial seizure
Loss of awareness with random symptoms such as:
- smacking lips
- making weird noises
- rubbing hands
- chewing/ swallowing
128
What are secondary generalised tonic - clonic seizures
The development of generalised tonic- clonic seizure following a focal/partial seizure
129
What is Todds Paralysis
Paralysis of involved limbs for several hours after the seizure can be partial or complete
130
What can cause Seizures
```
Causes:
Idipoathic
Family Hx
Head Trauma
Genetic
Tumours
Structural Defects
Alcohol and Illegal drugs
```
131
What can precipitate seizures in epileptics
```
Precipitates:
Stress
Lack of sleep
Waking up
Drinking alcohol
Flashing lights
```
132
What are the main 3 differentials for loss of consciousness
Epileptic seizures
Syncope - transient global cerebral hypoperfusion
Psychogenic nonepileptic seizure
133
What can cause Syncope
- Reflex: vasovagal (caused by your body overeacting to triggers e.g fainting at blood)
- Cardiogenic
- Orthostatic Hypotension (sitting to standing can be caused by medication)
134
What can cause cardiac syncope
1. Conditions that predispose to transient tachycardias
2. Bradycardias
3. Cardiac Ischaemia
4. Structural heart disease
135
If someone presents with LOC what is the key investigation to perform
ECG - conditions that give rise to transient tachyarryhthmias have abnormal ECGs between events!
* They are a major cause of sudden death in younger people
136
What is a blackout during exercise until proven otherwise
Cardiogenic Syncope
137
What are Psychogenic Non-epileptic Seizures
PNES are attacks that may look like epileptic seizures but are not caused by abnormal brain electrical discharges. Instead, they are a manifestation of psychological distress e.g childhood sexual abuse
138
What are two rare but important causes of transient LOC
Hypoglycaemia
| Acute Hydrocephalus
139
What is essential for distinguishing between different causes of LOC
```
The History:
Circumstances - trigger
Prodome - any aura
Witness - what did they see
Duration
Post icital phase - what happened after
```
140
What are the key investigations for LOC
ECG - no.1
CT head
MRI
EEG - for the diagnosis and classification of epilepsy
141
What is the emergency management of someone having a seizure
- Ensure patients harm themselves as little as possible environment around them is safe as possible
- Repeated or Prolonged seizures give Rectal (if no IV access) or IV (inpatient) diazepam or lorazepam
142
What anti epileptic drugs can be used to treat epilepsy
Generalised Tonic Clonic - Sodium Valproate and Lamotrigine
Absence Seizure - Sodium Valproate
Partial Seizure - Carbamazepine
143
What is important to remember about anti epileptic drugs
They are highly teratogenic - especially Sodium Valproate
| - Must be on some form of long term contraception
144
What is the pathogenesis behind parkinsons disease
1. Progressive depletion of of dopamine secreting cells in the substantria nigra
2. Causing depletion in dopamine secretion
3. Fall in neuronal transmission from basal ganglia to cortex
145
What are the three clinical features of Parkinson disease
Tremor - resting tremor
Rigidity - hypertonia in limbs and trunk
Bradykinesia - difficultly initiating movement
146
How is Hypertonia different in Parkinsons to UMN lesion
Hypertonia is present throughout extension unlike UMN where resistance falls throughout extension
147
What are other symptoms/signs may you get in Parkinsons
```
Shuffling gait
Poor balance
Lewy Body Dementia - hallucinations
Depression
Loss of sense of smell
```
148
What investigations can be performed for suspected parkinsons disease
Diagosis is clinical
149
How is parkinsons disease managed
- Levodopa - 1st line: in Parkinsons whose motor symptoms impact QoL
- Dopamine Agonists (ropinarole) - 1st line: for young patients, or not impacting QoL to prolong use of L-DOPA
- Monoamine Oxidase B Inhibitors
- Physiotherapy
150
What is a side effect of L-Dopa and what medication can manage this
- Nausea
| - Carbidopa
151
What happens to L-Dopa overtime
It becomes less effective
152
What are the side effects of Dopa Agonists
- Impulsive control disorders e.g impulsive shopping
| - Excessive sleeping
153
What is Huntingtons Chorea
A rare autosomal dominant condition
| It is an incurable, progressive, neurodegenerative disorder presenting at middle age
154
What is the average age of onset of huntingtons chorea
40 years
155
What are the clinical features of Huntingtons Chorea
Prodomal phase: irritability, depression, incoordination
Progresses to:
- Chorea - jerky involuntary movements
- Dementia - memory problems
- Psychotic changes - presonality change, depression and psychosis
- fits +/- death
156
What is the pathophysiology behind Huntingtons Chorea
1. Expansion of CAG repeats in Huntingtons gene
2. Causes atrophy of neurones in the striatum
3. Less GABA
4. Less regulation of Dopamine
5. Increase in Dopamine - increase in movement
157
What would you see on examination of someone with Huntingtons Chorea
Abnormal eye movements
Random unpredictable movements
Ataxia Problems
Heal to Toe walking
158
What is the treatment for Huntingtons
Genetic test will reveal condition
No cure
MDT to manage patients e.g physio, occupational health
SSRIs - to manage depression
159
What is Myasthesia Gravis
An autoimmune disease characterised by weakness and fatiguability of the occular, bulbar and skeletal muscles
160
How may a myasthesia gravis patient present
- Progressive tiredness throughout the day or through repetitive movements which improves after rest:
- skeletal muscle fatigue of proximal limbs
- occular: diplopia
- bulbar: tiring from swallowing and chewing
- face and neck: head drop, ptosis
161
What may you see on examination of a myasthesia gravis
- fading voice when asking patient to count to 50
- ask patients to stretch out arms and look for downward drift
- ask patient to look up - look for ptosis
162
What investigations should you perform for myasthesia gravis
- Tensilon Test
- AchR antibodies
- MuSK antibodies
- EMG - may be normal but repetitive stimulation of nerve may demonstrate decrements in muscle action potential
- CT Thorax - look for thyoma
163
What is Myasthesia Gravis associated with
- Thymic Hyperplasia/ Thyoma
| - Other AI disease: thyroid
164
What is a differential diagnosis of Myasthesia Gravis
Generalised muscle weakness in MND
165
What is the management of Myasthesia Gravis
- Acetylcholine esterase inhibitors e.g Pyridostigmine
- Immunosupressents e.g Steroid (prednisolone) or Azathriopine/Methotrexate
- Thymectomy
166
What is a Myasthenic Crisis
Severe weakness including the respiratory muscles it is life threatening
Can be caused by infection, relapse, medication dosing
167
How is a Myasthenic Crisis managed
Urgent!!
Monitor FVC
Treat with IVIG or plasma exchange
Identify and treat trigger
168
What is Multiple Sclerosis
- Chronic inflammatory demyelinating autoimmune disorder specific to the CNS
- it causes multiple plaques of demyelination within the brain and spinal cord
169
What is the cause of Multiple Sclerosis and who is at risk
- Genetic Predispostion + Environmental Trigger (infection (EBV) or Vit D deficiency)
- Most common in women
170
What is usually the age of onset in MS
Early adulthood
171
What are the 3 types of progression of MS
- Relapsing/ Remitting - periods of relapse followed by periods of remission where they are well
- Primary Progressive - disease progressively worsens from onset
- Secondary Progressive - disease begins as relapsing/remitting followed by progression
172
How does an MS patient usually present and how does regression occur
- young adult with two or more clinically distinct episodes of CNS dysfunction followed by remission
- resolution of inflammation and partial remyelination
173
What are the symptoms of MS
```
- Optic Neuropathy: impaired vison and
unilateral eye pain
- Spinal cord lesions
+ Sensory: numness, paraesthesia
+ Motor: spastic weakness
+ Autonomic: Sexual Dysfunction and urine
retention
- Cerebellar: Charots Triad: nystagmus,
dysarthia and intention tremor
- Change in mental state: memory, impaired
concentration
```
174
What sign in MS causes electric shock sensations down spine when they flex their neck
Lhermittes Sign
175
What are MS symptoms exacerbated by
Uthoffs Sign - Fever, physical, exersion, warm water (worsen with heat)
176
What investigations should be performed in suspected MS
- Detailed Hx and Examination
- MRI - may show plaques
- Nerve Conduction Studies - Prolonged
evoked potential
- LP: increased protein and cell count
electrophoresis: Oligoclonal bands
177
What is the management of Relapsing/Remitting MS
```
Medications for relapsing/remitting
Relapses:
- 1st line: Steroids - IV methyprednisalone shortens
- 2nd line: Plasmapheresis
To prevent relapses in remission:
- 1st line: Interferon therapy: IFN-B
- 2nd line: Natalizumab
```
178
What is the management of Primary Progressive MS
No drugs to manage primary progressive
179
What supportive management can be given to those with MS especially those with primary progressive
- Spasticity: baclofen, diazapam, physio
- Paraesthesia: Amitriptyline
- Tremors: Beta Blockers
- Urinary Incontinence: Oxybutinin
180
What is Motor Neurone Disease
Neurodegenerative Disease of the UMN and LMN
181
What is MND pathophysiology
LMN: Destruction of motor neurones in anterior horn of spinal cord and brainstem
UMN: Destruction of lateral corticospinal tracts in motor cortex
182
How can you distinguish MND from MS
No sensory loss or sphincter disturbance
183
What most commonly causes death in MND patients
Respiratory failure from bulbar palsy and pneumonia
184
Who does MND most commonly present in
Middle aged men
Most cases are sporadic with no FHx
Rare familial cases
185
What are the 4 clinical patterns of MND
All 4 no involvement of sensory system or motor nerves of eyes or sphincters
- Amyotrophic Lateral Sclerosis - most common
- Progressive muscular atrophy- predominantly LMN
affected
- Progressive bulbar and pseudobulbar palsy - destruction
of UMN and LMN cranial nerves (dysarthia, dysphagia)
- Primary lateral Sclerosis
186
What are the features of Amyotrophic Lateral Sclerosis
UMN and LMN signs
UMN: spasticity, hypertonia, hyperreflexia, no fasiculations, no atrophy, babinski +ve
LMN: flaccidity, hypotonia, hyporeflexia, fasciculation's present, severe atrophy of muscles, babinski -ve
187
What are the investigations for MND
Clinical
| Fasciculations are characteristic
188
How can you differentiate between MND and cervical spine lesions with UMN and LMN signs
Cervical spine lesions often will have sensory signs
189
How is MND managed
There is no cure
Riluzole delays progression of disease by a few months
Ventilatory support
NG feeding tube
190
What are the features of spinal cord compression
- UMN deficit below the lesion due to disruption to corticospinal pathways
- Sensory impairment below the lesion due to disruption of spinothalamic and dorsal coulmn function
- Acute spinal compression can present as spinal shock (flaccid weakness and no UMN signs)
- Lhermittes Phenomenom
- Painless Atonic Bladder
191
What are the causes of spinal cord compression
Secondary malignancy
Infection e.g spinal TB
Cervical disc prolapse
Trauma
192
What investigations should you perform for spinal cord compression
Urgent MRI!!! especially acute to prevent irreversible paralysis
193
What spinal cord syndromes are not caused by compression
Inflammation
Infarction
MND
Syrinx
194
What is cauda equina
Spinal damage distal to L1
195
What does cauda equina present as
bilateral sciatica
leg weakness
bladder/bowel dysfunction
saddle paraesthesia/numbess
196
What is the management of cauda equina
Medical emergency
| Surgical Decompression
197
What is a mononeuropathy
affects a single nerve OR multiple mononeuropathy/mononeuritis multiplex affects several random nerves
198
What causes single mononeuropathy
Acute compression/entrappment of a nerve
OR
Direct damage to nerve through surgery, trauma
199
What is the most common cause of single neuropathy
Carpel Tunnel Syndrome - pressure on median nerve
200
What causes carpel tunnel syndrome
```
Idiopathic
Pregnancy
Obesity
DM
RA
Hypothyroidism
```
201
What are the clinical features of carpel tunnel
Pain and paraesthesia
Weakness and wasting of thenor muscles
Sensory loss of palm and palmer aspects of 3 radial and a half fingers
Tapping will cause pain: Tinnels and Phallens
202
How is mononeuropathys managed
Treat the cause
Nocturnal Splints (carpel tunnel)
Local Steroid injections
Surgical decompression
203
What other mononeuropathys
Ulner Nerve - Elbow - Tennis Elbow
Radial Nerve - wrist drop from pressure of humerus - Saturday night syndrome
Common Peroneal Nerve - pressure of head of fibula - lateral loss of sensation -leg crossing
204
What causes multiple mononeuropathy/ mononeuritis multiplex
Individual nerves picked off randomally
Often inflammatory/ autoimmune mediated
e.g Vasculitis and Connective Tissue Disorders
```
W egners
A IDs/Amyloid
R heumatoid
D M
S arcoidosis
```
P AN
L eprosy
C arcinoma
205
What are the three types of the peripheral neuropathys
Polyneuropathy
Mononeuropathy
Mononeuropathy Multiplex
206
What are polyneuropathys
Disorders of the peripheral nerve whos distribution is usually symmetrical and widespread
Can be sensory, motor or both
Chronic, Slow and Progressive
Starts in the most distal nerves
207
What is the most common cause of peripheral polyneuropathy
```
Diabetes
idiopathic
Uncommon:
Deficiency states B12/Folate
Alcohol/Toxins/Drugs
Hereditary Neuropathies
Paraneoplastic Syndromes/Malignancy
Metabolic Syndromes: Thyroid/ Renal Failure
```
208
What investigations should you do for neuropathies
```
Hx and Ex
Neuropathy Screening
Vasculitic Screening
EMG/NCS
CSF Study
Nerve Biopsy
```
209
What neuropathy screening tools are there
FBC, ESR
Glucose, U&E, TFT
B12/ Folate
HIV
210
What Vasculitic Screening tools are there
FBC, ESR
ANA, ANCA, compliment, RhF
CRP
211
How are neuropathys managed
Idiopathic - no treatment - manage pain with pregabalin/ amitriptyline
Treat remove/underlying cause e.g DM/glusose
Inflammatory Neuropathy - Prednisalone with steroid sparing agent e.g azathioprine
Vasculitis Neuropathy - Prednisalone + Immunosuppresent e.g cyclophosphamide
212
What is the most common cell type of primary brain tumours
Majority glial cell!!!
- Astrocytoma
- Oligodendroglioma
213
Where do secondary brain tumours originate from
```
Breast
Prostate
Thyroid
Kidney
Stomach
```
214
What are three clinical features of brain tumours
1. Progressive Neurological Deficit - dependent on site of tumour
2. Raised Intracranial Pressure - headache, vomiting and
papilloedema - can lead to pressure on brainstem
3. Epilepsy
215
What investigations should you perform for brain tumours
CT & MRI
216
What is the management for brain tumours
Surgical resection of tumour and Radiotherapy
217
What is benign ICP
headache and papilloedema in young obese females
Management:
Weight loss, loop diuretics and predinisalone
218
What are the contraindications for a lumbar puncture
Raised Intracranial Pressure!!!
| - Due to pressure gradient can cause coning and neurological deterioration even death!!!
219
What is Hydrocephalus
Excessive amounts of CSF within the ventricles causing raised ICP most often due to obstruction of outflow of CSF
220
What can cause Hydrocephalus
Congenital
Malignancy
Meningitis
Subarachnoid Haemorrhage
221
What is the presentation of Hydrocephalus
Signs of raised ICP and ataxia
222
What is the management of Hydrocephalus
Diagnosed on MRI/CT
| Surgical insertion of a shunt
223
What is meningitis
Inflammation of the meninges
224
What are the causes of meningitis
Bacterial
Viral
Fungi
225
What bacteria cause bacterial meningitis
Neisseria Meningitidis
Streptococcus Pneumoniae
Listeria Monocytogenes
Haemophilus Influenzae
226
What viruses cause Viral Meningitis
```
Enterovirus
Mumps
Herpes Simplex (HSV)
HIV
EBV
```
227
What is the presentation of acute bacterial meningitis
```
Headache
Neck Stiffness
Fever
Photophobia
Vomiting
Papilloedema
Progressive Drowsiness
```
228
What can acute bacterial meningitis lead to and what symptoms may you get
Meningococcal Septicaemia
| - a non blanching purpuric rash and signs/ symptoms of shock
229
What is the presentation of viral meningitis
Same symptoms except no rash or sepsis - usually self limiting condition
230
What is the deifferential diagnosis of meningitis
Subarachnoid Haemorrhage
Migraine
Viral encephalitis
231
What investigations may be done for someone with suspected meningitis
Head CT scan
Lumbar Puncture -B - WCC high, Protein high, low glucose
-V - WCC normal, Protein slightly raised,
glucose normal
Blood cultures
CXR
232
What is the management of a pateint with suspected meningococcal septicaemia
- Medical Emergency!!!!!!
- Non blanching rash is a sign
- LP is contraindicated in Sepsis!! - coning
- Do blood culture instead
- Start immediate treatment of IV benzylpenicillin or IV
cefotaxime
- BUFALO
233
What is the management of meningitis
Cefotaxime IV add ampicillin if risk of listeria
| Treatment depends on results from MC&S
234
What is meningococcal Prophylaxis
```
Oral Ciprofloxacin (tendon disorders and quinalones)
Vaccination
```
235
What is Encephalitis
Inflammation of the brain
236
What is the presentation of Encephalitis
Unlike meningitis - it leads to abnormal cerebral function, mental state, motor and sensory deficiencies
Mild - most common - self limiting fever, headache and
drowsy
Less common - severe focal signs, seizures and coma/death
237
What is Encephalitis caused by
Viruses - Herpes Simplex, HIV, Mumps
| Can also occur in a bacterial infection
238
Who is encephalitis most common in
Immunosuppressed
MSM
IVDU
239
What infection can be concerning in encephalitis
Herpes - death or brain injury follows herpes encephalitis for many in the UK
240
What investigations should be performed in Encephalitis
CT/MRI
CSF - high WCC and high protein
Viral serology or blood cultures
EEG
241
What is the treatment for encephalitis
Suspected HSV - start IV aciclovir immediately
242
What is the Cerebellum responsible for
Co-ordinating movements
| Maintenance of balance and posture
243
What are the pathways of the the Cerebellum
Ipsilateral
244
What causes Cerebellar Lesions
```
MS
Primary/Secondary Tumours
Haemorrhage/Infarct
Chronic Alcohol use
Anti-epileptic Drugs
```
245
What are the clinical features of Cerebellar Disease
```
Ataxia
Dysarthia
Dysphagia
Nystagmus
Clumsiness
Tremor
```
246
How is Cerebellar Disease diagnosed
Hx and Ex
| MRI
247
What are muscular dystrophys
Inherited groups of progressive myopathic disorders affecting normal muscle function
248
What is Duchenne Muscular Dystrophy
An X linked mutation
Presents in early childhood with weakness in proximal legs progressing to other muscle groups leading to severe disability and death in late teens
No curative treatment
249
What tract relays motor response
The corticospinal tract
250
Where does the corticospinal tract cross
The medulla
251
What tract relays sensory response
Dorsal Columns - Vibration and Light Touch/proprioception
| Spinothalamic - Pain and Temperature
252
Where does the posterior column cross
The medulla
253
Where does the spinothalamic cross
1-2 segments above point of entry
254
What are the 12 cranial nerves
Olfactory - smell
Optic - vision
Oculomotor - eye movements and pupil reflex (inferior oblique, medial, superior and inferior rectus)
Trochlear - eye movements (superior oblique)
Trigeminal - face sensation and chewing
Abducens - eye movements (lateral rectus)
Facial - face movment and taste
Vestibulocochlear - hearing and balance
Glossopharyngeal - throat sensation, taste and swallowing
Vagus - movement, sensation and abdominal organs
Accessory - neck movements
Hypoglossal - tongue movement
255
What would you get in a unilateral transverse lesion
Brown - Sequard Syndrome
Ipsilateral loss of vibration and proprioception
Ipsilateral weakness
Contralateral loss of pain temperature
256
What would you get in central cord syndrome
Quadraparesis
Rare: loss of pain/temperature sensation in upper > lower extremities
+/- bladder dysfunction
257
What would you get in anterior cord syndrome
```
Paraplegia
Loss of pain/temperature
Autonomic Dysfunction
Bowel, Bladder and Sexual Dysfunction
Preservation of dorsal columns
```
258
What is the most common fatal accidental poisoning
CO poisening
| Headache, N&V, Confusion, Coma, Death
259
What can Amphetamines and other stimulants cause
```
Seizures
Psychosis
Ischaemic Stroke
Intracranial Haemorrhage
Coma
```
260
What are the symptoms of alcohol overdose
Ataxia
Dysarthia
Nystagmus
Coma
261
What are the symptoms of alcohol withdrawal
6-8hrs Tremor, anxiety, nausea
24hrs Delirium Tremens - Visual Hallucinations
48hrs Generalised Tonic-Clonic Seizures
3-5 days rare hyperactivity
262
How should you treat alcohol withdrawal
Thiamine (BEFORE GLUCOSE)
Multivitamins
Benzodiazapine
263
What is Wernickes Encephalopathy
Thiamine Deficiency
Opthalmoparesis, Ataxia and Confusion
Majority improve however some develop Korsakoffs Psychosis may emerge as Wernickes resolve
Possibly irreversible
264
Apart from Wenickes Encephalopathy what else can alcohol lead to
```
Cerebellar Degeneration
Peripheral Neuropathy
Colour Blindness
Dementia
Tremor
Central Pontine Myelinolysis
```
265
What virus causes chicken pox
Varicella Zoster Virus (one of the herpes viruses)
266
What is is called when varicella zoster virus reactivates from latent phase
Herpes Zoster
267
Where does herpes zoster remain dormant before reactivation
In dorsal root ganglia and/or cranial nerve ganglia
268
What does reactivation of herpes zoster lead to
Shingles
269
What is the risk to others with shingles
Could cause chickenpox in a non-immune individual after close touch or contact
270
What are the clinical features of shingles
pain and tingling in a dermatomal distribution followed by a rash a few days later
The rash consists of papule and vesicles in the same dermatome
271
What are the most common dermatomes for shingles
Lower Thoracic Dermatome
| Ophthalmic Trigeminal Dermatome
272
What is the management of shingles
Oral Acyclovir
273
What is a complication of shingles
post herpatic neuralgia which can be sever and last for years
Meningitis or Encephalitis
Blindness - if near eye (ulceration)
274
How can you treat PHN
Carbamazipine - quick treatment of acyclovir reduces risk of PHN
275
What can be given to reduce risk of shingles
The varicella vaccine (in over 70s)
276
What may lead to you developing shingles
Weakened Immune System:
- Increasing age
- HIV/AIDS
- Stress
- Chemotherapy
- Immunosupression - organ transplant
277
What are the symptoms of DCM
DCM symptoms can include any combination of :
Pain (affecting the neck, upper or lower limbs)
Loss of motor function (loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance
Loss of sensory function causing numbness
Loss of autonomic function (urinary or faecal incontinence and/or impotence) - these can occur and do not necessarily suggest cauda equina syndrome in the absence of other hallmarks of that condition
Hoffman's sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient's hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick.
278
What is Degenerative Cervical Myelopathy
Degenerative of the cervical intravertbrae discs in the leading to spinal cord compression in the neck
279
How is DCM treated
Urgent referral for assessment to prevent permanent damage
| Decompressive Surgery!!!
280
What opiate can help with nerve pain
Tramadol
281
What is the treatment of nerve pain
1st line: amitriptyline, pregablin, gabapentin
| 2nd line: tramadol
282
What are the rules for group 1 drivers following a TIA
Can start driving if symptom free after 1 month and no need to inform DVLA
283
What is the contraindication of Triptan
Cardiovascular Disease
284
What is normal pressure hydrocephalus
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis.
285
What is the triad of symptoms of normal pressure hydrocephalus
```
Wet, Wobbly and Wacky
A classical triad of features is seen
urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson's disease)
```
286
What is a differential for GBS
Charot Marie Tooth Disease
| Autosomal Dominant - Sensorimotor Neuropathy
287
What is the treatment of Meningococcal Septicaemia in the community
IM benzylpenicillin
| Report to the proper officer at the local council
288
What is multiple system atrophy
Multiple system atrophy is a cause of Parkinsonism which can be difficult to differentiate from idiopathic Parkinson’s disease. Key features to help you differentiate are the presence of unilateral symptoms, and more severe/early onset autonomic dysfunction (postural hypotension/erectile dysfunction).
289
What is a pontine haemorrhage
Pontine haemorrhage is a life-threatening condition. It often occurs as a complication secondary to chronic hypertension. Patients often present with reduces Glasgow coma score, quadriplegia, miosis, and absent horizontal eye movements
290
What lesions are homonymous quantrantinopias
PITS (Parietal-Inferior, Temporal-Superior)
291
What dementia is associated with MND
Frontotemporal dementia
292
How does a 3rd nerve palsy present
Affected eye looks down (hypotropia) and out (exotropia)
Ptosis
Diplopia
Compression - fixed dilated pupil
ischaemic microangiopathy - pupil sparing
293
How does a 4th nerve palsy present
Extorsion of the eye
| Diplopia
294
How does a 6th nerve palsy present
Esotropia
| Diplopia
295
What is facial nerve palsy caused by
```
Bells Palsy (idiopathic)
Lymes Disease
Ramsey Hunter Syndrome
Meningits
TB, Viruses
Stroke
MS
Tumour
Guillian Barre (bilateral)
Diabetes
Sarcoidosis
```
296
What is Ramsey Hunter Syndrome what are the symptoms and how is it treated
When latent varicella zoster virus reactivates in the ganglion of the 7th cranial nerve
Symptoms:
- Painful vesicular rash on auditory canal +/- drum, pinna, tongue palate or iris
- Ipsilateral Facial Palsy
- Loss of taste
- Vertigo
- Tinnitus/Deafness
- Dry mouth and Eyes
Treatment: Acyclovir + Prednisolone (as for Bells Palsy)
297
What symptoms do you get for a facial nerve palsy
```
Abrupt Onset:
Complete Unilateral Face Weakness
Unilateral sagging of the mouth
Drooling of Saliva
Food trapped between gum and cheek
Speech Difficulty
Failure of eye closure making watery/dry eye
Ipsilateral numbness or pain around the ear
Decreased Taste
Hypersensitivity to Sound
```
298
What are the signs of Facial Nerve Palsy
UMN (brainstem) lesion - Patients are able to close eyelids and frown, symptoms present as contralateral
LMN - (peripheral) lesion - Patients are unable to close eyelids fully and frown, symptoms are present ipsilateral (Bells)
299
What tests are done for Facial Nerve Palsy
Bloods - ESR, glucose, Increased Borriella Antibodies in Lymes, Increased VZV antibiodies in Ramsey Hunter
MRI - space occupying lesions
CSF - infection
Nerve Conduction Studies
300
What is the Management of Bells Palsy
Prednisolone
