Conditions Flashcards
1
Q
What is MND?
A
- Motor Neurone disease
- progressive neurodegenerative condition
2
Q
Symptoms of MND
A
- Muscle weakness
- problems with speech, swallow and breathing
3
Q
What percentage of MND cases have a familial link?
A
- 10%
4
Q
What is the most common subtype of MND?
A
- Amyotrophic lateral sclerosis
ALS
5
Q
What are different subtypes of MND
A
- Primary lateral sclerosis (PLS) - upper motor
- Amyotrophic lateral sclerosis (ALS) - mixed
- Progressive muscular atrophy (PMA) - lower motor
- Progressive bulbar palsy
6
Q
PLS subtype of MND is mainly a ____ neuron symptom
A
- upper motor
7
Q
PMA subtype of MND is mainly a ____ neuron symptom
A
- lower motor
8
Q
ALS is mainly a ___ neuron symptom
A
- upper and lower
- mixed
9
Q
Symptoms of an upper neuron issue
A
- increased tone
- hyperreflexia
- spastic gait
- slowed movements
10
Q
Symptoms of a lower neuron issue
A
- muscle waisting
- weakness
- fasciculations
11
Q
Investigations of MND
A
- Electrophysiology
- clinical diagnosis
12
Q
Potential drug treatment for MND
A
- riluzole
13
Q
Explain hyperkinetic disorder presentation?
A
- tremor
- tics
- chorea
- dystonia
- athetosis
14
Q
Parkinson’s is a __hyperkinetic/hypokinetic__
A
- hypokinetic disorder
15
Q
What nmemonic can be learnt for hyperkinetic disorders?
A
- TRAP
- Tremor
- Rigidity
- Akinesia
- Postural disturbances
16
Q
A pill rolling tremor is seen in?
A
- Parkinsons
- resting tremor affecting the hands
17
Q
Describe rigidity
A
- increased muscle tone during passive movement
- not velocity dependant
18
Q
Describe cog wheel sign and what disease is it seen?
A
- tremor + rigidity
- seen in parkinsons
19
Q
What is akinesia?
A
- slowness of repetitive movement
20
Q
Shuffling gait may be the sign of?
A
- Parkinsons
21
Q
3 types of tremor?
A
- rest
- postural
- kinetic
22
Q
Describe dystonia?
A
- sustained and repetitive muscle contraction
23
Q
Explain chorea?
A
- fidgety and flowing
24
Q
Explain myoclonus?
A
- sharp sudden weak movement
25
Writhing movements may be described as what?
- athetosis
26
How is Parkinson's diagnosed?
- clinical
- DaTSCAN
- Bradykineasia + (resting tremor, rigidity, postural instability)
27
Explain bradykineasia?
- slowness of initation of voluntary movement with progressive reduction in speed and amplitude
28
Symptoms of Parkinsons
- stooped posture
- hypomimia (masked face)
- shuffling
- short stepped gait
29
Lead pipe rigidity?
- constant resistance throughout passive movement
30
What are Lewy bodies?
- seen in parkinsons
| - protein deposits on nerve cells
31
What is the pathogenesis of parkinsons?
- loss of dark pigment of substantia nigra and locus coeruleus
- loss of dopaminergic cells
32
Genetic links to parkinsons?
- SNCA
- LRRK2
- PARKIN
33
Treatment of parkinsons?
- dopamine agonists (levopa)
34
Side effects of levopa
- nausea
- motor fluctuations
- dyskinesia
- drug induced psychosis
35
Define cognition
- the mental action of acquiring knowledge and understanding through thought, experience and the senses
36
Dementia definition
- significant cognitive decline
| - interference with independence in everyday activities
37
A transient form of antegrade dementia
- transient global amnesia
38
Triggers for transient global amnesia?
- emotion
| - change in temperature
39
Transient epileptic amnesia symptoms?
- forgetfulness shortlived
| - temporal lobe seizure
40
Treatment of epileptic amnesia?
- anti-epileptic medication
41
High functioning job, young person with a memory "not as it used to be"?
- functional/subjective cognitive impairment
42
Explain CJD?
- Prion disease
| - misfolding of the protein prion
43
Explain addenbrooke's test?
- cognitive examination
- orientation
- registration
- attention
- memory
- verbal fluency
44
Tests for limbic encephalitis?
- high WCC and antibodies in CSF
| - inflammatory changes on MRI
45
Treatment of limbic encephalitis
- treat tumour
| - immunosuppressive treatment
46
Explain alzheimer's disease
- amyloid neurodegenerative proteinopathy
47
Extracellular amyloid plaques and dementia is what?
- alzhimers
48
Investigations for alzhimers?
- clinical
- MRI = atrophy of temporal/parietal lobes
- CSF = decreased amyloid
49
Treatment of Alzheimer's?
- acetylcholine boosting
= cholinesterase inhibitors
= NMDA receptor blocker
50
Frontotemporal dementia is associated with what symptoms?
- behaviour
| - loss of ability to stop
51
Treatment of frontotemporal dementia?
- trazodone
| - safety management
52
What is vascular dementia?
- cerebrovascular disease + dementia
53
Dementia with Lewy bodies?
- cognitive followed by motor symptoms
- attention difficulties
- hallucinations
54
Treatment of dementia with Lewy bodies?
- levodopa
| - cholinesterase inhibitors
55
Investigations of parkinson's dementia?
- DaTscan
| - clinical
56
Huntington's disease symptoms?
- changes in mood
- chorea
- later psychosis
57
What gene is increased in huntington's disease?
- expansion of CAG gene
| - increased huntingtin gene
58
Advantages of a CT scan?
- quick
- excellent bony details
- axial acquisition
59
Disadvantages of a CT scan
- radiation
| - poor soft tissue detail
60
Advantages of MRI
- Excellent contrast and soft tissue resolution
| - marrow and cord pathologies
61
Disadvantages of MRI
- Less bony detail
- not compatible with pacemakers
- not as quick as CT
62
What are the 3 main types of MRI?
- T1
- T2
- FLAIR
63
Explain what appears white (hyperintense) on T1?
- Fat
- methaemoglobin (subacute haematoma)
- mineral deposition
- melanin
- mush
64
What appears dark (hypointense) on T1?
- Water
- air
- bone
65
White matter appears whiter than grey matter on what MRI scan?
- T1
66
White matter appears darker than grey matter on what MRI scan?
- T2
67
On a T2 scan the outer aspect of the brain will be ___lighter/darker__-
- grey matter
| - whiter in colour
68
Which scan ___T1/T2__ is better for pathologies?
- T2
69
What appears dark on a T2 scan?
- blood
70
What MRI scan shows cranial nerves?
- Flair
71
What would an infarct appear like on MRI
- Basal ganglia darker
| - loss of contrast between white and grey matter
72
CSF is dark on a __T1/T2__ scan
- T1
73
Mainstay of investigation for stroke?
- non-contrast CT
74
CT angiogram is good for picking up what size of aneurysms?
- less than 3mm
75
MRI angiogram is good for picking up what size of aneurysms?
- >3mm
76
Areas commonly affected in brain trauma?
- CT
- Orbitofrontal cortex
- anterior temporal lobe
- posterior portion of the superior temporal gyrus
77
Explain an extradural haematoma
- collection of blood between the inner surface of the skull and outer dura
- source of bleed : middle meningeal artery
78
Appearance of an extradura (epidural)l haematoma?
- biconvex
- mass effect with herniation
- limited by cranial sutures
- lemon shaped
79
Explain a subdural haematoma?
- below the dura, between the arachnoid mater
| - venous in origin?
80
Appearance of a subdural haematoma?
- banana shaped
- concave/cresent
- irrespective of cranial sutures
81
What does a subarachnoid haemorrhage look like?
- follows the sculic pattern
82
Neoplasms outside the BBB will appear like what on MRI
- greater enhancement due to contrast uptake
83
What neoplasm is "ice-cream cone" shaped
- vestibular (acoustic) schwannoma
84
Differentiating from a low grade and high grade glioma?
- low grade = more defined, less of a massive effect
| - high grade = less defined, more of a massive effect
85
Explain Chiari malformation?
- cerebellum protrudes the foramen magnum
86
Explain cortical dysplasia?
- thickening of the grey matter
87
Explain the appearance of schizencephaly
- grey matter lined cleft
| - abnormal brain development
88
What diagnoses MS?
- Multi-focal lesions of demylination
| - Dawson's fingers in brain
89
What MRI scan is best to diagnose MS?
- T2
| - FLAIR
90
Appearance of herpes encephalitis on MRI?
- Gyral enhancement
- general oedema
- often starts unilateral and becomes bilateral
91
Appearance of CJD on MRI?
- Hperintensity of globus pallidus in T1
| - Cerebral atrophy
92
Ring like mass on MRI?
- Metastasis?
| - central necrosis with surrounding oedema
93
arachnoid cysts usually contain?
- CSF
94
What combination drug is given in Parkinson's?
- Sinemet
- levodopa + carbidopa
- reduces peripheral side effects of levodopa
95
What drug is contraindicated in parkinsons? (anti-emetic)
- metoclopramide
| - as crosses the BBB
96
What anti-emetic is given in Parkinsons and why?
- domperidone
| - given 30mins before leveopoda as an adverse effect of the drug is nausea and vomiting
97
What adjunct therapy can be given in Parkinsons?
- dopamine receptor agonists (rotigotine)
| - COMT inhibitors (entacapone)
98
Explain some causes of drug induced parkinsons?
- anti-psychotics (chlorpromazine)
- anti-emetics (metoclopramide)
- CCBs (diltiazem
- lithium
99
What is neuroleptic induced parkinsonism and how is it treated?
- caused by antipsychotic medications
| - treatment by procyclidine
100
Which tremor has a frequency of usually around 4-6HZ
- Rest (Parkinsonian) tremor
101
What is the frequency of a orthostatic tremor?
- 14-16Hz
102
What suppresses an essential tremor?
- alcohol
| - beta blockers
103
Some potential causes of tremor?
- lithium
- bronchodilators
- hyperthyroidism
- brain injury
- inflammation (MS)
- Parkinsons
- Dementia with Lewy bodies
104
Describe myoclonus and some causes?
- sudden, brief involuntary jerks
- drug induced
- liver flap
105
A cerebellar tremor is an example of what tremor?
- Intention tremor
106
Explain the type of brain herniation with the greatest risk of death?
- tonsillar
- infratentorial
- spinal cord decompression
107
Which type of brain herniation can cause 3rd nerve palsy?
- uncal herniation
108
Red flags for a brain tumour?
- headaches worse in morning
- nausea and vomiting
- seizures
- behavioural changes
109
A lesion in the cerebellum will have a __contralateral/ipsilateral__ effect?
- ipsilateral
110
What is the frontal lobe responsible for?
- thought
- behaviour
- memory
- reasoning
111
What is the DANISH nemonic for cerebellum examination?
- dysdiadochokinesia
- ataxia
- nystagmus
- intention tremor
- slurred speech
- hypotonia
112
What is the commonest primary brain tumour?
- glioma
113
What is the commonest brain tumour?
- metastasis
114
What is the grading system for glioma?
- graded 1-4
- grade 1 = benign seen in children
- grade 4 = glioblastoma, highly malignant
115
Gliomas arise from what cell type?
- astrocytes
116
Tumour that is chemosensitive and appears calcified?
- oligodendrocyte tumour
117
What protocol is used for the treatment of gliomas?
- Stupp protocol
| - surgery + radiotherapy + chemotherapy
118
What is the commonest benign brain tumour?
- meningioma
119
Symptoms of a meningioma?
- headaches
- visual impairment
- seizure
120
What investigations are performed for a meningioma?
- CT (Densley enhancing, oedema)
| - = MRI (Dural tail)
121
Dural tail on MRI is suggestive of?
- memingioma
122
Treatment of meningioma?
- surgery
| - radiotherapy
123
Symptoms of a vestibular schwannoma?
- hearing loss
- tinnitus
- dizziness
124
Complication of a vestibular schwannoma surgery?
- facial nerve palsy
125
Midline tumour in children?
- pineal tumour
126
Treatment and investigation for pineal tumour?
- ETV +/- Biopsy
127
What initial investigations for a pituitary tumour should be carried out?
- early morning cortisol
| - prolactin
128
Increased prolactin from a pituitary tumour should be initially treated by what?
- cabergoline
| - should shrink the tumour/
129
Define intracranial pressure
- the pressure exerted by the cranium on to
| - the brain, CSF, Blood
130
Explain the Monroe Kellie hypothesis and also state the 1st factor to componsate
- fixed area, an increase in CSF;Blood;brain tissue will cause compensation in another volume
- first is CSF
- 2nd is blood volume
131
What is the normal range for ICP
- Between 7-15mmHg
| - constantly fluctuating
132
What is the equation for cerebral perfusion pressure (CPP)
CPP = MAP - ICP
133
What is the equation for cerbebral blood flow
CBF = CPP/Cerebral vascular resistance
134
Name some early and late clinical signs of a raised ICP
- Early = reduced consciousness, headache, papiloedema, nausea and vomiting
- later = coma, fixed dilated pupils, increased urinary output
135
List some causes of a raised ICP
- Masses (tumour, infarct, abscess)
- Swelling
- central venous thrombosis
- blockage to CSF flow
136
Describe communicating hydrocephalus?
- blocked reabsorption of CSF
- all ventricles enlarged
- causes: SAH, Meningitis
- LP can be performed
137
Describe obstructive hydrocephalus?
- blockage preventing reabsorption
- aqueductal stenosis
- lateral and 3rd ventricles enlarged
- do not perform a lumbar puncture
138
Define hydrocephalus
- increase in CSF within the head
139
Explain normal pressure hydrocephalus
- idiopathic, seen in elderly
| - hakims triad (dementia, gait abnormalities, urinary incontinence)
140
CT signs of normal pressure hydrocephalus
- disproportionate size between ventricles and lateral sulcus
141
Treatment of normal pressure hydrocephalus
- low pressure VP shunt
142
What is Hakims triad
- seen in normal pressure hydrocephalus
- abnormal gait
- dementia
- urinary incontience
143
What is a typical patient who experiences idiopathic intracranial hypertension
- young female
- child bearing age
- obeses
144
Typical symptoms of idiopathic intracranial hypertension
- headache
- vision disturbances
- diziness
- nausea and vomiting
145
Investigations for idiopathic intracranial hypertension?
- LP
- fundoscopy
- CT
- MRI
- ICP monitoring
Diagnosis of exclusion
146
Treatment of idiopathic intracranial hypertension
- weight loss
- diuretics
- carboanyhrase inhibitors
147
What are the 3 main subdivisions of GCS
- Eye opening
- verbal
- motor
148
Difference on scan between an acute and chronic subdural haematoma
- chronic = darker blood, more liquified
| - acute = blood appears whiter on scan
149
Describe a contra-coup injury
- 2 points of injury
150
What is a DAI
- Diffuse axonal injury
- harder to pick up on CT
- Sheering forces (rapid acceleration then deceleration)
151
Why should CO2 be controlled in raised ICP
- Increased CO2
- increased Cerebral blood flow
- increased ICP
152
Treatment of a chronic subdural haematoma?
- burr hole and drainage
153
Highest and lowest GCS?
- Lowest = 3 = bad
| - highest = 15 = normal
154
What is the EVM nmenomic for GCS
- Eyes (out of 4)
- Verbal (out of 5)
- movement (out of 6)
155
What defines acute and chronic back pain?
- acute <3months
| - chronic >3months
156
What is important to ask about in drug history of a patient with back pain
- corticosteroids
| - immunosuppressants
157
Cancer red flags for back pain?
- age >50
- weight loss
- history of cancer
- pain at night
158
Infection red flags for back pain?
- fever/chills
- immunosuppression
- IV drug use
- foreign travel
159
Cauda equina red flags for back pain
- bilateral sciatica
- urinary incontenence
- decreased anal tone
- loss of perianal sensation
160
AAA red flags for back pain
- age >60
- abdominal pulsatile mass
- pain at rest
161
What are the nerve roots of the sciatic nerve?
- L4 - S3
162
What nerve roots are most commonly effected in sciatica and what are their symptoms?
- L5 = Lateral leg, weak dorsiflexion of foot
| - S1 = Back of leg to foot, weak plantar flexion and reduced ankle reflexes
163
___% of back pain patients are better in 6 weeks?
- 90%
164
Describe the symptoms of sciatica?
- unilateral leg pain
- lower back and buttocks
- radiates below the knee to the foot and toes
165
Initial investigations for sciatica?
- straight leg rise
| - assess gait, power, tone, sensation
166
Initial treatment for sciatica?
- absence of red flags = conservative treatment for 12 weeks then referal
167
Differentials of sciatica?
- bone spur
- canal stenosis
- tumour
- TB
- endometriosis
- peripheral neuropathies
168
What are the 3 main tracts you must be aware of on the spinal cord?
- corticospinal (medulla)
- spinothalamic (crosses segmentally)
- dorsal column (medulla)
169
Upper motor lesion symptoms
- hypertonia
- hyperreflexia
- no muscle waisting
- no fasiculations
170
Lower motor lesion symptoms
- hypotonia
- hyporeflexia
- muscle waisting
- fasciulations
171
Explain the symptoms of a complete cord transection
- motor and sensory deficits
- initial 'spinal shock' with flacidity
- followed by an upper motor neuron sign
172
Explain brown-sequard syndrome
- cord hemisection
- ipsilateral motor loss
- ipsilateral dorsal column loss
- contralateral spinothalamic (loss of pain)
173
Explain central cord syndrome
- lesion in the centre of the cord, usually post hyper-extension or flexion event in a previous stenotic neck (elderly)
- cape like distribution affecting upper limb - motor
- no lower limb motor dysfunction
174
in chronic spinal cord decompression __lower/upper__ motor symptoms predominate
- upper motor
175
treatment of tumour compression of spinal cord
- dexamethasone
- radiotherapy
- chemotherapy
- surgical decompression and stabilisation
176
causes of spinal canal stenosis
- osteophyte formation
- subluxation
- bulging of intervertebral disc
177
Treatment of spinal canal stenosis
- decompress and stabilisation
178
Causes of epidural abscesses?
- blood borne (s.aureus)
- IV drug (s. epidermis)
- TB
179
Treatment of an infection abscess causing cord decompression?
- antimicrobial therapy
| - surgical drainage
180
Treatment of haemorrhage causing spinal cord compression
- reverse anticoagulation
| - surgical decompression
181
What inheritance pattern does duchenne muscular dystrophy follow?
- x linked recessive
182
What is the chance of children being affected if their mother is a carrier for duchenne muscular dystrophy
- x linked recessive
- 50% chance of daughter being carrier
- 50% chance of son being effected
183
Symptoms of duchenne muscular dystrophy?
- 3-4yrs
- growers sign (using arms to compensate)
- exaggerated lumbar lordosis
- toe walking
- calf hypertrophy
184
What mutation causes duchenne muscular dystrophy?
- dystrophin gene
185
Diagnosing duchenne muscular dystrophy?
- CK
- EMG
- Muscle biopsy
- genetic testing
186
Symptoms of Huntington's disease
- chorea
- dementia
- rigidity
- bradykineasia
187
Huntington's disease is what genetic disease?
- autosomal dominant
| - age dependant penetrance
188
Explain age dependant penetrance in Huntington's disease?
- the older you are, the less likely you will show symptoms
189
CAG expression in Huntington's increases down generations?
TRUE/FALSE
- True
190
What is the pathology behind huntington's disease?
- caudate atrophy
| - loss of cells from the basal ganglia
191
CAG expansion causes?
- huntingtin gene
192
Spinal muscular atrophy is a genetic disease affecting which gene?
- SMN1 gene
193
What is the genetic pattern of spinal muscular atrophy?
- autosomal recessive
| - 1 in 4 chance
194
What are some symptoms of spinal muscular atrophy?
- proximal muscle weakness
- hypotonia
- respiratory muscle weakness
195
What causes muscle weakness in spinal muscular atrophy?
- loss of anterior horn cells
196
What is the aim in treatment in spinal muscular atrophy?
- retaining exon 7 in the SMN2 gene to give it the same functional properties as SMN1
197
The autosomal dominant variant of Alzheimer's disease is present in what percentage of cases?
And what gene mutation is it?
- APP mutation
- present in 5-10% cases
- autosomal dominant for early onset
198
Explain senile plaques?
- amyloid beta plaques
| - seen in alzhimers
199
ApoE gene increases your chances of what condition?
- alzhimers
200
What is MS?
- Inflammatory demyelination disorder of oligodendrocytes in the cns
201
What are the 2 types of MS?
- Relapsing remitting
| - primary progressive
202
Explain pyramidal weakness
- weakness of upper arm extensors
- weakness of lower limb flexors
- seen in MS
203
Symptoms of MS
- Pyramidal weakness
- spasticity
- cerebellar dysfunction
- internuclear opthaloplegia
204
Investigations for MS?
- Clinical
- blood test
- MRI (T2 or flair) - Dawsons fingers
- CSF - oligoclonal bands
205
Dissemination in time is a sign of?
- MS
206
Mild treatment of MS
- Oral steroids
207
Severe relapse of MS treatment
- IV steroids
208
1st line disease modifying treatment in MS?
- tecfidera
209
2nd line disease modifying in MS?
- monoclonal antibodies - tysabri
| - fingolimod
210
3rd line disease modifying treament in MS?
- Mitoxantrone
211
What are potential risk factors for epilepsy that you must ask in a history?
- birth
- development
- seizures
- head injury
- family history
- drugs
- alcohol
212
What can you do to determine an epileptic seizure from syncope?
- ECG
- cardiovascular examination
- lying and sitting BP
213
Episode of collapse, what is best investigation
ECG
214
Prolonged QT syndrome may first present as what?
- collapse
| - seizure
215
Differentials for epilepsy
- syncope
- non-epileptic attack disorders
- panic attack
- sleep phenomena
- TIAs
- migraine
216
Driving after 1 seizure?
- car = 6 months
| - HGV = 5 years
217
Driving with epilepsy?
- car = 1 year seizure free
| - HGV = 10years seizure free and off medications
218
What is epilepsy?
- a tendency to recurrent, usually spontaneous epileptic seizures
219
What is an epileptic seizure?
- abnormal synchronisation of neuronal activity
- spontaneous abnormal activity
- interruption of normal brain activity (focal, generalised)
220
What does SUDEP stand for?
- sudden unexpected death in epilepsy
221
What is SUDEP
- Refers to death in people with epilepsy that are not caused by injury or other known causes
222
Two main types of epilepsy?
- focal
| - generalised
223
Explain focal to generalised seizures?
- part of brain structurally abnormal
| - hits the cortical networks
224
A seizure focus causes?
- focal seizure
225
Symptoms of a motor cortex focal seizure
- jerking
- postural
- head and eye deviation
226
Symptoms of sensory cortex seizures?
- olfactory
- gustatory
- visual
227
Symptoms of psychic cortex seizure
- memories
- deja vu
- aphasia
228
Commonest epilepsy in children?
- generalised seizures
229
Why are focal epilepsies commoner in adults?
- structual abnormality in brain
| - as you age more likely to injury brain
230
Over the age of 30, what seizure?
- focal
231
Complication of benzodiazepines
- respiratory arrest
232
Primary generalised epilepsy is commoner in what age group?
- under 30s
233
Focal onset epilepsy is commoner in what age group?
- over 30s
| - structural abnormalities
234
What is helpful for diagnosis in a primary generalised seizure?
- EEG
235
What treatment is first and 2nd line in a primary generalised seizure?
- sodium valproate
| - lamotrigine
236
Side effects of sodium valproate?
- hair loss
- weight gain
- teratogenic
237
Sodium valproate is used first line for what treatment?
- primary generalised epilepsy
238
Treatment of focal onset epilepsy?
- carbamazepine
| - lamotrigine
239
Side effects of carbamazepine
- dizziness
240
What drug should you not give in generalised epilepsy?
- carbamazepine
241
Why do you avoid certain anti-convulsants in women?
- anti-convulsants that induce hepatic enzymes
- carbamazepine etc.
- effect progesterone pill
- morning after pill
242
What is status epilepticus?
- clinical emergency
| - recurrent epileptic seizures without full recovery of consciousness
243
Time frames for status epileptics?
- seizure for 30mins or more
- treat after 10 mins
- first line benzodiapines
244
Types of status epilepticus?
- generalised convulsive status
- non-convulsive status
- epilepsia partialis continua
245
Causes of status epilepticus?
- respiratory insufficiency
- hypotension
- hyperthermia
- rhabdomyolysis
246
First line treatment for status epilepticus?
- benzodiazepines
247
treatment of partial status epilepticus
- acute convulsions state
| - benzodiazepines
248
When is the botulism toxin found?
- clostridium botulinum
| - organism found in soil, food, wounds, IV drug users
249
What does the botulism toxin do?
- cleaves proteins that are responsible for vesicle formation
250
Symptoms of botulism?
- rapid onset of weakness
| - no sensory loss
251
What is the pathophysiology of LEMS?
- Antibodies to presynaptic Ca2+ channels
| - less vesicular release
252
What condition is LEMS associated with?
- bronchial small cell carcinoma
253
Symptoms of LEMS?
- Proximal limb weakness
| - improves with contract
254
What differentiates myasthenia gravis and LEMS in terms of muscle contractibility fatigue?
- LEMS improves with contractions
| - myasthenia gravis worsens with contraction
255
Treatment of LEMS
- 3-4diaminopyridine
256
What breakdown ACh in the post synaptic membrane?
- acetylcholinestrase
| - ACh = choline + acetate
257
What antibodies are associated in myasthenia gravis?
- anti-AChR IgG
258
Myasthenia gravis is also associated with what?
- thymic hyperplasia
| - thymoma
259
symptoms of myasthenia gravis?
- fatigue, worse with activity
- generalised/ocular (ptosis)
- double vision
260
What antibiotic should always be avoided in a patient with myasthenia gravis?
- gentamicin
261
Treatment of myasthenia gravis?
- acetylcholinesterase inhibitor = pyridostigmine
| - thymectomy
262
Explain myotonia?
- failure of muscle relaxation
| - disorder of chloride channel
263
Myotonic dystrophy is autosomal ___
- dominant
264
Symptoms of myotonic dystrophy?
- weakness
- cataracts
- frontal balding
265
Symmetrical proximal muscle weakness in an older patient may be?
- polymyositis
266
What is rhabdomyolysis
- dissolution of muscle
267
Causes of rhabdomyolysis
- crush injury
- extreme exercise
- toxins
268
What is the affect of rhabdomyolysis?
- large amounts of phosphate, potassium and myoglobin in circulation
269
Triad of symptoms in rhabdomyolysis?
- malgia
- muscle weakness
- myoglobinuria
270
What drug may cause myalgia?
- statins
271
Explain a tonic-clonic seizure?
- aura before
- tonic phase (rigidity, tongue biting, hypoxia)
- followed by clonic phase (convulsion, eye rollin, tachycardia)
272
How does carbamazepine work?
- inhibits Na+ channels
| - first line in focal seizures
273
How does lamotrigine work?
- inhibits Na+ channels
| - 2nd line in seizure control
274
How does sodium valoprate work?
- increases GABA, inhibits Na+ channels and glutamate decabroxylase
- 1st line in generalised seizure
275
Treatment of psychogenic non-epileptic attack disorder
- withdrawl of anti-epileptic drugs
- explanation of attacks
- treatment of anxiety or depression
276
How long does non-epileptic attacks usually last
- 10 mins or longer
277
What is an absence seizure?
- altered/loss of awareness
| - day dreaming apperance
278
What is focal cognitive seizure
- trigger: reading/writing
- abnormal responses during trigger
- loss of cognition
279
Differences on non-epileptic attacks and epilepsy?
```
- non epileptic attacks
= non-synchronous movement
= longer duration
= eyes closed
= control during attack
= physiological history?
```
280
Eye closure in epileptic seizures is unusual?
TRUE OR FALSE
- TRUE
| - eye closure is more associated with non-epileptic attack
281
Define neuropathy
- a pathological process affecting peripheral nerves
282
Define radiculopathy
- pinching of a nerve at the nerve root
- disease affecting the nerve roots
- dermatomal or myotomal distribution
283
What is the symptoms of large fibre neuropathies?
- loss of joint position and vibration sense
| - sensory ataxia
284
What is the symptoms of small fibre neuropathies?
- impairment of pain, temperature and autonomic function
285
What is pseudoathetosis?
- involuntary, slow, writing movement
- loss of proprioception
- eyes closed (converse to the athetosis)
286
Define mononeuropathy and name some examples
- only 1 nerve involved
- carpal tunnel
- ulnar nerve compression
287
Define mononeuritis multiplex and name some examples
- several individual nerves effected
- diabetes
- leprosy
- vasculitis
288
Define polyneuritis and some examples
- starts distally (glove and stocking distribution)
- diffuse, symmetrical disease
- demyelination or axonal loss
- gullian-barre syndrome
- toxins
289
What is Guillan-barre syndrome and its symptoms
- polyneuritis
- demylination
- progressive paraplegia
- sensory symptoms beforee motor
- painful
290
Causes of guillan-barre syndrome?
- campylobacter infection
291
Treatment of Gillian barre syndrome?
- immunoglobulin infusion
| - plasma exchange
292
Symptoms of HSMN1
- muscle weakness, feet, ankles, legs and hands
| - high arched foot
293
Explain the effect of axonal or demyelination damage to nerves?
- axonal - reduction in amplitude
| - demyelination - reduction in velocity
294
Some causes of axonal neuropathies?
- vasculitis
- paraneoplastic
- infection
- drugs
295
Treatment of axonal neuropathies?
- treat cause
- supportive
- neuropathic pain relief
296
Treatment of vasculitic neuropathies?
- IV methylprednisolone + cyclophasmide
297
Treatment of demylination neuropathies
- IV immunoglobulins, steroids, azathrioprine
298
Define encephalitis
- brain parenchyma inflamed
299
Define meningitis?
- meninges inflamed
300
Most common cause of bacteria meningitis?
- streptococcus pneumonia
301
What meningitis organism is commonest in neonates?
- listeria
| - group b streph
302
Commonest meningitis organism in those aged 10-21
- nisseria meningitis
303
commonest cause of viral meningitis?
- enterovirus
| - hsv 1 and 2
304
first line treatment in suspected bacterial meningitis?
- ceftriaxone and dexamethasone
305
Why are steroids given in meningitis and when should they be avoided
- reduces inflammation
| - avoid in: post-surgical meningitis, severe immunocompromised, meningococcal, septic shock
306
When is viral meningitis commoner?
- later summer/autumn
307
Diagnosis of viral meningitis?
- stool culture
- throat swab
- b CSF PCR
308
Treatment of viral meningitis?
- supportive
309
Symptoms of encephalitis?
- coma
- mental changes
- speech changes
- almost always viral = iv aciclovir
310
Treatment of viral encephalitis?
- IV aciclovir
311
Diagnosis of viral encephalitis?
- CT
| - LP
312
Symptoms of meningitis
- headache
- vomiting
- fever
- neck stiffness
- rash
- photophobia
313
Define meningo-encephalitis
- headache, fever, neck stiffness + features of encephalitis
314
Suspicious of what organism of meningitis in the immunocompromised?
- listeria monocytogenes
| - give amoxicillin
315
Complications of meningitis?
- purulence
- invasion
- cerebral oedema
- hydrocephalus
316
Causes of meningitis?
- direct extension
| - remote foci
317
When should a CT be performed before an LP in suspected meningitis?
- immunocompromised
- CNS disease
- new onset seizure
- papiloedema
318
CSF in bacterial meningitis?
- WBC > 2,000
- neutrophils >1,180
- low glucose
- high protein
319
CSF in viral meningitis?
- lymphocytes
- normal glucose
- normal/slightly high protein
320
Causes of aseptic meningitis?
- HSV1 and 2
- Syphillus
- TB
321
Close contact prophylaxis in meningitis?
- chemoprophylaxis = ciprofloxacin
322
Which vaccines have helped reduce the cases of meningitis?
- neisseria meningitis
- haemophilus influennzae
- streptococcus phenuomia
323
Possibility of penicillin resistance meningitis treatment?
- add vancomycin
324
Penicillin allergic treatment of bacterial meningitis?
- chloramphenicol + dexamethasone
325
Define negative myoclonus?
- sudden, brief interruption of a muscle contraction
326
Define positive myoclonus?
- sudden quick involuntary muscle jerk due to contraction
327
Gram stain of s.pneumoniae?
- gram positive cocci
328
Gram stain of listeria monocytogenes?
- gram postive bacillius
329
Commonest mononeuropathies?
- carpal tunnel syndrome
- cubital tunnel syndrome
- radial nerve compression
- peroneal nerve compression
330
Signs of cubital tunnel syndrome?
- clawing of the hands
| - waisting
331
Signs of radial nerve compression
- radial nerve compressed by humerus
- wrist drop
- weakness of wrist and finger extension
332
Signs of peroneal palsy?
- peroneal nerve compressed against fibula
| - foot drop and weakness of eversion
333
Explain the pattern of disease in mononeutritis multiplex?
- several nerves become affected simultaneously or one after another
334
pseudoathetosis vs athetosis?
- pseudathetosis = loss of proprioception with eyes closed
| - athetosis = loss of proprioception with eyes open
335
What haemolytic pattern does s.pneumoniae follow?
- alpha haemolytic
| - partial haemolysis
336
Define pain
- an unpleasant sensory and emotional experience associated with actual tissue damage or described in such terms of damage
337
Chronic pain is defined as pain for ____ months
- greater than 3 months
338
what is peripheral sensitisation?
- a reduction in the threshold of peripheral afferent nociceptors
339
What is central sensitisation?
- increased excitability of spinal neurons
- rewiring of the spinal cord
- changes in brain
340
symptoms of chronic regional pain syndrome?
- allodynia
- hyperalgesia
- swelling
341
what is nociceptive pain
- pain from injury relayed through a normal nervous system
342
what is neuropathic pain
- pain generated within the nervous system
343
symptoms of neuropathic pain?
- burning
- shooting
- tingling
344
treatment of neuropathic pain
- tricyclic antidepressants
- anticonvulsants
- opiodis
345
first line in neuropathic pain treatment
- non-pharmacological
- amitriptyline
- gabapentin
346
describe neuroplasticity in chronic pain
- ability of the nervous system to adapt
| - in chronic pain can lead to hypersensitive and hyperactive activity
347
Glove and stocking distribution is due to which neuropathy?
- axonal length dependant
348
In GBS (Gullian-Barre) motor symptoms preceed sensory?
True of fale?
- true
349
Nerve conduction study in Gullian Barre
- demylination
- reduction in conduction velocity
- conduction block
350
Nerve conduction study for demylination?
- reduction in conduction velocity
| - conduction block
351
Nerve conduction for axonal neuropathies?
- conduction velocity normal
| - reduction in amplitude
352
Which neuropathy?
Child presents with high arched foot, muscle weakness distally?
- HSMN type 1
| - chronic demylination neuropathy
353
Treatment of neuropathies?
- treat underlying cause
- prevent progression
- treatment for neuropathic pain
- GBS = IV immunoglobulins
- vasculitis = IV steroids
354
Complication of GBS neuropathy?
- respiratory distress
- DVT
- Death
355
Explain the pathophysiology of MND?
- Impaired glutamate uptake
- excitotoxicity
- oxidative stress
- inflammation
- synaptic failure
- muscle atrophy
356
How does the drug riluzole work in MND?
- Glutamate inhibitor
| - NMDA receptor antagonist
357
If there is no signs of diffuse axonal injury on CT this can be ruled out?
True or false?
- false
| - only will show on MRI
358
What is DAI
- Diffuse axonal injury
| - sheering forces due to a secondary impact in coup-countercoup injury
359
Commonest benign brain tumour in children?
- glioma
360
Commonest malignant brain tumour in children?
- medullaryblastoma
| - midline
361
commonest benign brain tumour in adults?
- meningioma
- blood vessels
- dural tail
362
Commonest malignant brain tumour in adults?
- glioma
363
Explain pyramidal weakness?
- weakness of extensors of upper limb
| - weakness of flexors of lower limb
364
Stocking sensory loss suggests?
- length dependant neuropathy
365
Dermatomal sensory loss suggests?
- mononeuropathy
- radiculopathy
- plexus
366
Define hemianaesthesia?
- loss of sensation to lateral half of body
| - contralateral cerebrum lesion
367
Dissociated sensory is associated with what spinal lesion?
- brown sequard
368
Temporal lobe lesion may result in what aphasia?
- Wernicke's area
| - receptive aphasia
369
Frontal lobe lesion may result in what aphasia?
- Broca's area
| - expressive aphasia
370
Red flags associated with headaches?
- age >55
- immunosuppressed
- malignancy or previous malignancy
- worsened by valsalva (coughing, sneezing)
371
Explain acephalic migraine?
- no headache
| - but associated migraine symptoms : nausea, aura
372
Basilar migraine has which symptom?
- nausea
373
Abdominal migraine is commoner in what population?
- children
374
What is the average number of migraines someone gets per month
- around 1
| - prophylaxis considered if > 3/month
375
What causes aura in a migraine?
- cortical spreading and depolarisation
376
How long does a migraine typically last for?
- 4-72hrs
377
Symptoms of a migraine?
- throbbing
- unilateral
- nausea
378
Triggers for a migraine?
- Chocolate
- hangover
- orgasm
- cheese
- oral contraceptives
- lie ins
- alcohol
- travel
- exercise
379
Acute treatment of migraine?
- NSAIDs
- triptans (5HT)
- anti-emetics
380
Migraine prophylaxis
- beta blocker (propanolol)
- amitriptyline
- topiramate
- 3 or more attacks per month
- try a drug for 2-3months
381
Side effects of amitriptyline
- dry mouth
| - hypothension
382
Typically how long does the aura before a migraine last?
- 20-60mins
383
How do triptans work?
- 5HT
- Inhibit release of substance P and pro-inflammatory neuropeptides
- blocks transmission to the trigeminal nerve
384
Symptoms of tension headaches?
- bilateral pressing
| - absence of nausea and vomiting
385
Treatment of tension headaches?
- relaxation
- antidepressants
- dopthiepin
386
Who do cluster headaches typically affect?
- young
- males > females
- circadian (night)
387
Symptoms of a cluster headache?
- pain behind one eye
| - unilateral
388
Treatment of cluster headache?
- high flow oxygen
- sumatriptan
- prophylaxis = verampril
389
Differences between migraine and paroxysmal hemicrania?
- paroxysmal hemicrania has a shorter attack (10-30mins)
- 40 per day
- autonomic symptoms
390
Treatment of paroxysmal hemicrania?
- indomethacin
391
difference between paroxysmal hemicrania and hemicrania continua?
- hemicrania continua has a constant headache
| - paroxysmal hemicrania has shorter attacks (10-30 mins)
392
Symptoms of trigeminal neuralgia?
- unilateral severe shooting pain in face
- trigeminal distribution
- short duration
393
Treatment of trigeminal neuralgia?
- carbamazepine
- gabapentin
- decompression of trigeminal nerve root
394
What does SUNCT headache stand for?
- short lived
- unilateral
- neuralgiform
- conjunctival injection
- tearing
395
Treatment of SUNCT
- Lamotrigene
| - gabapentin
396
Where do trigeminal neuralgias typically affect?
- maxillae or mandibular
397
Autonomic headaches may have what other symptoms?
- ptosis
- miosis
- nasal stuffiness
398
What is most important Non-REM or REM sleep?
- Non-REM sleep
| - profound relaxation
399
Explain Non-REM sleep
- start of night
- synthronchies rhythmic EEG activity
- partial muscle relaxation
- profound relaxation
400
What type of dreaming is present in Non-REM
- non-narrative images
| - emotionally driven image in head
401
Explain REM sleep?
- mostly at end of night
- EEG fast activity
- Atonic muscles (except diaphragm and extraocular muscles)
- important for cortical development
402
What type of dreaming is present in REM sleep?
- narrative dreaming
403
3 factors of sleep onset?
- homeostatic response
- emotional response
- circadian rhythm
404
What is the circadian rhythm?
- 25 hour cycle
- biological and physiology control of sleep
- timing changes with age
405
What controls the circadian rhythm?
- light entrains body clock
- non-rod/non-cone ganglion cell projections onto the suprachiasmatic nucleus
- blue light
406
What is the effect of exercise and sleep?
- no evidence of muscle recovery
| - sleep deprivation - physiological tiredness
407
What are the effects of sleep deprivation?
- visual illusions
- irritable
- concentration lapses
- micro sleeps
408
How long should you sleep?
- 7-7.5hours/night
| - a mid afternoon nap of 15mins is equivalent to 90mins overnight
409
Non-REM Parasomnias?
- non-dreaming
- confusional arousals
- sleep walking
- restless legs
- complex behaviours
410
REM parasomnias?
- dreaming
- simpler behaviour
- usually latter 3rd of night
411
What is the presentation of narcolepsy?
- daytime sleepiness
- cataplexy
- hypnagogic hallucinations
- sleep paralysis
412
What is cataplexy?
- loss of muscle tone triggered by emotion
| - seen in narcolepsy
413
What is hypnagogic hallucinations?
- hallucinations occurring at sleep onset
| - seen in narcolepsy
414
Investigations of narcolepsy?
- overnight polysomnography
- multiple sleep latency test
(goes into REM sleep very quickly)
415
What is insomnia?
- psychophysiological concept
416
What is a stroke
- Rapidly developing clinical symptoms and/or signs of focal and at times global loss of brain function
- functions lasting greater than 24hrs
- vascular in origin
417
Name some potential stroke mimics?
- seizure
- sepsis
- toxins
- syncope
- delierium
- vestibular dysfunction
418
What scoring system can be used in A&E for a stroke?
- ROSIER score
419
Types of stroke?
- infarction
- haemorrhage
- subarachnoid haemorrhage
420
Types of infarction strokes?
- atheroembolic
- small vessel
- cardioembolic
421
Types of haemorrhagic stroke?
- structural
- hypertensive
- amyloid angioplasty
422
First line investigation for stroke?
- CT (acute blood appears white)
| - looses sensitivity after 1 week post bleed
423
When should an MRI be used in the diagnosis of stroke?
- clinical suspicion of infarction, but no sign on CT
424
Infarction in the brain may appear as what on CT?
- Increased darkness
| - oedema
425
What is the penumbra?
- are surrounding an ischaemic event
- reduced blood flow and oxygen transport to the area
- hypoxia of cells nearby
426
What is the timeframe for thrombolysis in ischaemic strokes?
- sooner the better
| - up to 4.5hrs
427
What is the thrombolysis drug of choice for ischaemic strokes?
- Alteplase
428
What must be done before thrombolysis treatment in strokes?
- CT
- rule out haemorrhage
- CT should appear normal as ischaemic strokes don't show straight away
429
What is the endo-vascular therapy for stroke?
- thrombectomy
- physical removal of a clot using interventional radiology
- good for proximal clots
430
What antiplatelet treatment is given after an ischaemic stroke?
- after 24hrs = aspirin 300mg (for 2 weeks) then clopidegrol
431
What DVT prophylaxis is used in stroke?
- intermittent pneumatic compression
432
What is a TIA?
- Transient Ischaemic Attack
- same pathology as stroke
- lasts less than 24hrs
433
Treatment of TIA?
- Aspirin for 2 week
- then clopidogrel
- consider carotid endarterectomy
434
What is intracerebral haemorrhage associated with?
- anticoagulation treatment
(e. g. warfarin)
- treat with anticoagulation reversal (vitamin K)
435
Causes of an intercerebral haemorrhage?
- trauma
- hypertension
- arteriovenous malformation
- tumour
- amyloid
436
Focal intercerebral haemorrhage presentation?
- paresis (weakness)
- dysphasia
- numbness
- seizure
- visual
437
Global intercerebral haemorrhage presentation?
- headache
- nausea and vomiting
- reduced GCS
- pupils
438
Investigations for intercerebral haemorrhage?
- GCS
- CT
- CT angiogtaphy
- DSA
- MRI
439
Gold standard for diagnosing an intercerebral haemorrhage?
- DSA
| - Digital subtraction angiography
440
Management of an intercerebral haemorrhage?
- reduce ICP
- correct clotting
- control BP
- Reduce mass effect
- treat seizures
- prevent re bleed
441
Hypertension can cause a Intercranial haemorrhage in which arteries?
- lenticulostriae vessels
442
What is an intercerebral haemorrhage?
- bleeding occurring in the lobes, pons or cerebellum
443
What scoring system is used for strokes?
- NIHSS
- National institute of health stroke scale
- maximum score = 42
- severe stroke > 21
444
What is the maximum time up to which thrombectomy can be performed?
- up to 24hrs
445
What is the best MRI sequence to see an acute stroke?
- DWI
| - diffusion weighted imaging
