1
Q
what are the names of cells that produce myelin in the CNS?
A
oligodendrocytes
2
Q
what are the names of cells that produce myelin in the PNS?
A
schwann cells
3
Q
which nervous system does MS affect?
A
central
4
Q
what is the most common presentation of MS?
A
optic neuritis
5
Q
what are 5 signs of upper motor neurone lesions?
A
Inspection - No significant wasting
Tone - Increased (spasticity/rigidity) + Ankle Clonus
Power - Pyramidal (extensors weaker in arms, flexors weaker in legs)
Reflexes - Hyperreflexia
Plantars - Upgoing (Babinski)
6
Q
What are 5 signs of lower motor neurone lesions?
A
Inspection - Fasciculations + Wasting
Tone - Reduced (or normal)
Power - reduced in affected nerve distribution
Reflexes - reduced or absent
7
Q
what are 2 eye signs of MS?
A
optic neuritis (pain on eye movement and temporary vision loss to one eye)
Eye movement abnormalities (Internuclear opthalmoplegia or conjugate lateral gaze disorder - 6th CN palsies)
8
Q
what are 4 motor signs of MS?
A
Bells palsy
horners syndrome
limb paralysis
incontinence
9
Q
what are 4 sensory symptoms of MS?
A
triegeminal neuralgia
Numbness
Paraesthesia
Lhermitte’s sign
10
Q
what is Lhermitte’s sign? What condition does it signify?
A
MS
Electric shock sensation that travels down spine into limbs when flexing neck (due to disease in DCML of cervical spine)
11
Q
what is Uhtoff’s phenomenon?
A
worsening symptoms of demyelinating disorders (MS) when the body becomes overheated hot weather or hot showers
12
Q
what are 2 coordination symptoms of MS?
A
sensory ataxia (due to loss of proprioception +ve romberg)
cerebellar ataxia
13
Q
what is the mcdonald criteria for MS diagnosis?
A
2 or more episodes of central nervous system damage disseminated in time and space
14
Q
What is one episode of an MS like attack called?
A
clinically isolated syndrome
15
Q
what are 3 patterns of disease in MS?
A
relapsing-remitting
secondary progressive (starts relapsing-remitting then progresses)
Primary progressive
16
Q
what 2 investigations can be done to diagnose MS?
A
MRI brain and spinal cord
Lumbar puncture
17
Q
what is seen on LP in someone with MS?
A
oligoclonal bands in the CSF
18
Q
what is seen on LP in someone with MS?
A
oligoclonal bands in the CSF
19
Q
what are 4 key features of optic neuritis?
A
Central scotoma (enlarged blindspot)
pain on movement
impaired colour vision
relative afferent pupillary defect
20
Q
what is the treatment for acute MS relapse?
A
Methylprednisolone 500mg orally OD for 5 days
OR
1g IV daily for 3-5 days (in severe cases)
(plasma exchange can be used in V severe disability)
21
Q
What is the ongoing treatment for MS?
A
Immunomodulators and Biologcal therapy
Interferon beta
Glatiramer
Teriflunomide
22
Q
What medications can be used to treat neuropathic pain in MS?
A
amitriptyline or gabapentin
23
Q
what medication can be used to treat urge incontinence in MS?
A
anticholinergics - tolterodine or oxybutynin
24
Q
what medications can be used to treat spacticity in MS?
A
baclofen
gabapentin
Physio
25
What is the pathophysiology of parkinson's disease?
progressive reduction in the dopamine of the basal ganglia leading to disorders of movement
26
where is dopamine produced in the brain?
substantia nigra
27
what is are 6 features of benign essential tremor?
Symmetrical
5-8 Hz (5-8 times a second)
improves at rest
Worse with intentional movement
No other parkinsons features
Improves with alcohol
28
what are 6 features of parkinson tremor?
pill rolling
asymmetrical (starts unilaterally)
4-6 Hz (times a second)
worse at rest and with distraction
improves with intentional movement
no change with alcohol
29
what are 3 motor symptoms of parkinsons? (core diagnostic)
(Unilateral) tremor
(cogwheel) rigidity
bradykinesia - movements slows and gets smaller
30
what are 5 manifestations of bradykinesia in parkinsons?
handwritting gets smaller (micrographia)
Shuffling gait
Difficulty initiating movement
Difficulty in turning
reduced facial movement and expressions (hypomimia)
31
what are 5 non-motor symptoms of parkinsons?
Depression
sleep disturbance and insomnia
anosmia
cognitive impairment and memory problems
Postural hypotension
32
what is multiple system atrophy?
degeneration in multiple systems in the brain - causes parkison's like symptoms as well as autonomic and cerebellar dysfunction
33
what are 4 symptoms of autonomic dysfunction?
postural hypotension, constipation, abnormal sweating and sexual dysfunction
34
what is the treatment in dysfunctional essential tremor?
propanalol
35
what is the 1st line investigation for parkinsons?
dopaminergic trial
36
What is the first line treatment of parkinsons?
Levodopa (co-beneldopa)
37
what are 4 treatment options for parkinsons?
Levodopa
Dopamine agonist
Monoamine oxidase B (MAO-B) inhibitors
COMT inhibitors (extends life of levodopa)
38
what are 2 dopamine agonists?
Cabergoline
Pergolide
39
what are 2 monoamine oxidase B inhibitors?
Selegiline
Rasagiline
inhibits enzyme that breaks down dopamine
40
what are 8 side effect of levodopa?
dyskinesia
impulsive and compulsive behaviours
wearing off
dry mouth
anorexia
palpitations
postural hypotension
psychosis
41
when medication shouldn't be prescribed with levodopa?
iron - reduces absorption of levodopa
42
what are the 4 diagnostic criteria for lewy body dementia?
Fluctuating cognition with pronounced variation in attention and alertness
Recurrent visual hallucinations
REM sleep behaviour disorder
1+ key feature(s) of Parkinsonism
43
what protein are lewy bodies made up of?
alpha-synuclein
44
what is the first line pharmacological treatment for lewy body dementia?
acetylcholinesterase inhibitors
45
what are 3 examples of acetylcholinesterase inhibitors?
Donpezil
Rivastigmine
galantamine
46
what medication can be used to treat REM sleep disorder in lewy body dementia?
Clonazepam
or melatonin
47
what is the most common cause of dementia?
Alzheimer's disease
48
what is the 2nd most common cause of dementia?
Vascular dementia
49
what scan should be done in all patients presenting with dementia?
MRI head
50
what is the first line treatment for vascular dementia?
Aspirin
(clopidogrel 2nd line)
51
what is the definition of dementia?
a chronic progressive neurodegenerative diseasecharacterised by global, non-reversible impairement in cerebral functioning
52
what is the pathophysiology of alzheimers disease?
excess of beta-amyloid deposited in plaques along neurones
aggregation of tau proteins in neurofibrillary tangles
53
what is the 1st line pharma management of alzheimers?
Acetylcholinesterase inhibitors - donepezil, galantamine and rivastigmine
54
what medication is indicated in severe alzheimers?
Memantine (N-methyl-D-aspartate antagonist)
55
what is Bell's palsy?
sudden acute onset unilateral facial palsy of the facial nerve which fully resolves within 72 hours
may have hyperacusis (find noises loud) due to stapedius muscle paralasis
56
which of bells palsy or stroke are forehead sparing?
Stroke is forehead sparing
57
What is the first line treatment for bells palsy?
corticosteroids and eye care (within 72 hours)
58
what can be a complication of bells palsy?
keratoconjuctivitis sicca
59
what classes as a TIA?
a sudden onset focal neurological deficit which completely resolves within 24 hours of onset
60
what scale can be used to assess for urgent need of thrombolysis?
National institute of health stroke scale
61
what is the immediate management of TIA? (2)
aspirim 300mg initially then 75mg OD
Refer to specialist to be seen within 24 hours
62
How long from presentation can alteplase be given for stroke?
up to 4.5 hours
63
How long from presentation can thrombectomy be considered for ischaemic stroke?
<6 hours
24 hours if effecting large artery can be considered
64
What is the first line treatment for stroke within 4.5 hours?
IV alteplase
65
What is the first line treatment for stroke from hours 6-24?
thrombectomy
66
what scale is used to measure neurological disability?
Modified Rankin scale
67
what is the first line investigation in stroke?
CT head (non-enhanced)
68
what is the most common cause of extradural haemorrhage?
skull trauma
69
what blood vessel is most commonly ruptured to cause extradural haemorrhage?
middle meningeal artery (MMA)
70
what kind of haematoma is visible on CT in extradural haemorrhage?
biconvex and (usually) does not cross suture lines
71
what is the most common cause of subdural haemorrhage?
sheering of bridging veins which empty the ventral sinuses
72
what two populations are at great risk of subdural haematomas?
alcoholics
elderly people
due to brain atrophy there is more room for expansion of the haematoma prior to symptoms
73
what kind of haematoma is visible on CT in subdural haemorrhage?
crescent (sickle) shaped haematoma which can cross suture lines
74
what is the textbook presentation of subarachnoid haemorrhage?
sudden onset 'thunderclap' headache which peaks in severity within 1-5 minutes and lasts more than an hour. Also may be with vomiting, photophobia and non-focal neurological signs
75
what are 3 surgical options for subdural haematoma?
Burr hole craniotomy and suction irrigation
Trauma craniotomy
Hemicraniotomy and duraplasty
76
what medication is given insubarachnoid haemorrhage to prevent vasospasm?
nimodipine (calcium channel blocker)
77
what is the most common cause of a subarachnoid haemorrhage?
rupture of berry (intracranial saccular) aneurysm
78
what are 5 genetic conditions that increase risk of subarachnoid haemorrhage?
Ehlers-danlos syndrome
marfans syndrome
autosomal dominant polycystic kidney disease
neurofibromatosis type 1
pseudoxanthoma elasticum
79
what is the surgical treatment of subarachnoid haemorrhage?
1 - endovascular coiling
surgical clipping
80
what is the CT presentation of subarachnoid haemorrhage?
pooling of blood usually around the circle of Willis or in the sylvian fissure
81
what are 2 early complication of giant cell arteritis?
vision loss
stroke
82
what are 5 symptoms of GCA?
severe unilateral headache around temple and forehead
scalp tenderness (brushing hair)
jaw claudication
blurred or double vision
painless complete sight loss
May also have fever, aches, fatigue, wt loss, loss appetite
83
what cells are found on temporal artery biopsy in GCA?
multinucleate giant cells
84
what are 2 diagnostic tests for GCA?
raised ECS (>50 mm/h usually)
temporal artery biopsy
85
what are 4 non-diagnostic investigations that can be used for GCA?
FBC - may have normocytic anaemia and throbocytosis
LFTs - can have raised alk phos
CRP raised
Duplex USS of temporal artery has hypoechoic halo sign
86
what is the management of GCA?
1- Prednisolone 40-60mg until symptoms resolve
aspirin 75mg OD
PPI - for gastric prevention
87
what are 3 late complications of GCA?
relapse
steroid related complication
stroke
aortitis - aortic aneurysm and dissection
88
How long does a migraine usually last?
4-72 hours
89
what are 7 characteristic of a migraine?
Moderate to severe intensity
Pounding or throbbing
Usually unilateral (can be bilateral)
photophobia
phonophobia
with or without aura
nausea and vomiting
90
what are 4 different types of aura?
sparks in vision
blurring vision
lines across vision
loss of different visual fields
91
what is a hemiplegic migraine?
migraine with motor symptoms that can mimic stroke
symptoms include - hemiplegia, ataxia and changes in consciousness
92
what are some triggers for migraine?
CHOCOLATE
CHeese, chocolate and caffeine
Oral Contraceptive pill (and menstruation)
Alcohol (OH) + dehydration
Anxiety (+ stress)
Travel
Exercise
93
what is the acute management of migraine?
Triptans - sumatriptan 50mg
+ Paracetamol/NSAIDs
Antiemetics (metoclopramide)
94
how do triptans work?
5HT receptor agonists (serotonin receptor)
act on smooth muscles in arteries to cause vasoconstriction
act on peripheral pain receptors to inhibit activation
reduce neuronal activity in CNS
95
what is prophylactic management of migraine?
Propanalol
Topiramate (teratogenic)
amitriptyline
acupuncture
96
what migraine prophylactic medication is teratogenic?
Topirimate - causes cleft lip/palate
97
what are the 3 branches of the trigeminal nerve?
Ophthalmic (V1)
Maxillary (V2)
Mandibular (V3)
98
How long does trigeminal neuralgia last?
seconds to hours
99
what are 3 features of trigeminal neuralgia?
intense facial pain
electricity like shooting pain
Triggered by cold weather, spicy food, caffeine and citrus fruits
100
what is the 1st line treatment for trigeminal neuralgia?
carbamezapine
101
what are 8 symptoms of cluster headache?
Unilateral
Clusters of attacks for 4-12 weeks followed by remission for at least 3 months
15 mins to 3 hours
red, swollen watery eye
pupil constriction (miosis)
Ptosis
runny nose
facial sweating
102
what are 4 triggers for cluster headache?
alcohol
strong smells
sleep/circadian disruption
weather changes
103
what are 5 risk factors for cluster headache?
male
FHx
head injury
smoking
drinking
104
what is the acute management of cluster headaches?
Triptans sub cut (sumatriptan - 6mg SC)
High flow O2
105
what is the prophylaxis of cluster headache?
verapamil
106
what monitoring is needed with verapamil
ECG, BP, LFTs
107
headache redflags
fever, photophobia, neck stiffness
new neurological symptoms
dizziness
visual disturbance
sudden onset occipital headache
worse on cough or strain
postural changes
vomiting
wakes from sleep
pregnancy
trauma
108
what is the triad of horner's syndrome?
Ptosis
miosis
anhydrosis
109
what is the pathophysiology of Horner's syndrome?
damage to the sympathetic nervous system supplying the face
sympathetic nerves from spinal cord pass through sympathetic ganglion in base of neck to be post-ganglionic nerves and travel back into the head alongside the internal carotid
110
what are 2 methods of investigating horner's syndrome?
cocaine eye drops - should usually dilate pupil - not in horners
adrenaline eye drops - won't dilate normal pupil but will dilate in horners
111
what are 4 central causes of Horner's?
4 Ss
Stroke
MS
Swelling (tumours)
Syringomyelia - cysts in spinal cord
112
what are 4 pre-ganglionic lesions that can cause horners?
4Ts
tumour
trauma
thyroidectomy
top rib (extra cervical rib)
113
what are 4 post-ganglionic lesions that can cause horners?
4Cs
carotid aneurysm
carotid artery dissection
cavernous sinus thrombosis
cluster headache
114
what is the most common cause of bacterial meningitis in adults?
1 - Neisseria meningitidis
2 - streptococcus pneumoniae
115
what does neisseria meningitidis look like?
gram negative (pink) diplococci
116
what causes the petechial rash in meningitis?
disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages
117
what is the most common cause of bacterial meningitis in neonates?
Group B strep (strep aglactiae)
118
what is Kernig's test?
for meningeal irritation in meningitis
patient lies on back, hip and knee flexed to 90 degrees then straighten knee
119
what is brudzinski's test?
for meningitis
patient flat on back, flex their neck to their chest - pos if causes involuntary flexion of hips and knees
120
what medication can be given in GP for suspected bacterial meningitis?
IM (or IV) benzylpenicilin
<1 year - 300mg
1-9 years - 600mg
>10 years - 1200mg
121
what blood test can be done for meningitis?
meningococcal PCR - quicker than blood cultures
122
what is the treatment for meningitis in <3 months?
Cefotaxime + amoxicillin (listeria cover)
123
what is the treatment for meningitis in >3 months?
ceftriaxone
124
what non-antibiotic meds can be given in bacterial meningitis?
steroids - Dexamethasone QDS > 3 months
125
what is the prophylactic for meningitis contacts?
ciprofloxacin single dose
126
what is the incubation period of meningitis usually?
7 days
127
what is the 3 most common causes of viral meningitis?
Herpes simplex virus
Enterovirus
Varicella zoster virus
128
where does the spinal cord end?
L1-2
129
what level is a lumbar puncture taken at?
L3-4
130
what is the LP picture in bacterial meningitis?
cloudy
high protein
low glucose
high neutrophils
+ve culture
131
what is the LP picture in viral meningitis?
clear
normal (may be mildly raised) protein
Normal glucose
High lymphocytes
+ve PCR
132
what is the LP picture in fungal meningitis?
Clear
Mildly raised WBC
raised protein
normal glucose
-ve culture and PCR
133
what are 5 complications of meningitis?
hearing loss
seizures and epilepsy
congnitive impairement and learning disability
memory loss
focal neurological deficits
134
what is the most common cause of encephalitis in adults?
HSV
135
what is a non-infective cause of encephalitis?
autoimmune encephalitis
136
what are 4 risk factors for encephalitis?
extremes of age
immunodeficiency
viral infection
animal or insect bite
137
what are 6 symptoms of encephalitis?
Altered consciousness
altered cognition
unusual behaviour
acute onset focal neurological symptoms
acute onset focal seizures
Fever
138
what medication can be used to treat HSV and varicellar zoster?
aciclovir
139
what medication can be used to treat CMV?
ganciclovir
140
what are 3 infections particularly associated with GBS?
campylobacter jejuni
CMV
EBV
141
what is the pathophysiology of GBS?
B cells create antibodies against antigens on pathogen of proceeding infection which match proteins on the neurones and cause them to be attacked. This is called molecular mimicry
142
what are 4 features of GBS?
symmetrical ascending weakness
reduced reflexes
loss of sensation or neuropathic pain
can progress to cranial nerves
143
what is the criteria for clinical diagnosis of GBS?
Brighton criteria
144
what is the 1st line management of GBS?
IV Ig
145
what is the leading cause of death in GBS?
PE - given VTE prophylaxis
146
what is the most common motor neurone disease?
Amyotrophic lateral sclerosis - ALS
147
what is the second most common MND?
progressive bulbar palsy
148
what is the pathophysiology of MND?
progressive degeneration of upper and lower motor neurones
149
what medication can be used to prolong survival in ALS?
Riluzole
150
what tumour is linked to myasthenia gravis?
thymoma
151
what is the pathophysiology of myasthenia gravis?
autoimmune antibodies to Ach receptors at neuromuscular junction of motor nerves bind to receptors and block triggering which would lead to muscle contraction
152
what antibodies are tested for in myasthenia gravis?
acetylcholine receptor antibodies
muscle specific kinase antibodies
LRP4 antibodies
153
what is a diagnostic test for myasthenia gravis?
edrophonium test - increases Ach at NMJ causing relief of weakness
154
what are 5 treatment options for myasthenia gravis?
reversible acetylcholinesterase inhibitors - pyridostigmine or neostigimine
immunosupression
thymectomy
rituximab
eculizumab
155
what is the treatment of myasthenic crisis?
Immunomodulatory therapies - IV Ig or plasma exchange
156
what is the most common type of neurofibromatosis?
type 1
157
what chromosome is the gene that causes neurofibromatosis type 1 on?
chromosome 17 - codes for tumour suppressor protein
158
what is the inheritance pattern of neurofibromatosis?
autosomal dominant
159
what is the diagnostic criteria for neurofibromatosis 1?
CRABBING
Cafe au lait spots (6+ over 5mm kids and 15mm adults)
Relative with NF1
Axillary or inguinal freckles
BB - Bony dysplasia - Bowing of long bone
Irish hamartomas - 2+ yellow/brown spots on iris
Neurofibromas 2+ or 1 plexiform neurofibroma
Glioma of optic nerve
160
what chromosome is the neurofibromatosis type 2 gene found on?
chromosome 22
161
what kind of tumours does neurofibromatosis type 2 lead to?
shwannomas
162
what tumour is neurofibromatosis 2 most associated with?
acoustic neuromas
163
what is an acoustic neuroma?
benign tumour of schwann cells surounding auditory nerve
also called vestibular schwannomas
164
where do acoustic neuromas occur?
cerebellopontine angle
165
what is the presentation of acoustic neuromas? (5)
unilateral sensorineural hearing loss
unilateral tinnitus
vertigo
absent corneal reflex
facial palsy
166
what is the gold standard investigation for acoustic neuroma?
MRI
167
what nerves are effected in bulbar palsy?
CN9,10,11,12
168
what is the function of CN1?
olfactory nerve
smell
169
what is the function of CN2?
optic nerve
Vision
170
what is the function of CN3?
oculomotor
Eye movement (up, nasally, down, inferior oblique)
171
what is the function of cranial nerve 5?
trigeminal
V1 - ophthalmic - scalp, forehead and nose sensation
V2 - maxillary - cheek, lower eyelid, nasal mucosa, upper lip, upper teeth and palate sensation
V3 - jaw, lower teeth, anterior 2/3 tongue sensation, muscles of mastication
172
what is the function of CN6?
Abducens
lateral rectus movement
173
what is the function of CN7?
facial nerve
sensation to ear
taste in anterior 2/3 tongue, hard and soft palate
muscles of facial expression
lacrimal, submandibular and sublingual glands and mucous glands
174
what is the function of CN8?
vestibulocochlear
hearing and balance
175
what is the function of CN9?
glossopharyngeal
taste and sensation posterior 1/3rd tongue
parotid gland
elevation of larynx and pharynx (stylopharyngeus muscle)
176
what is the function of CN10?
vagus
sensation external ear, larynx, pharynx
taste from epiglottis
smooth muscle of pharynx and larynx
177
what is the function of CN11?
spinal accessory
trapezium and sterocleidomastoid innervation
178
what is the function of CN12?
hypoglossal
intrinsic and extrinsic tongue muscles
179
what are 4 causes of bulbar palsy?
brainstem stroke or tumour
degenerative disease (MND)
Autoimmune disease (GBS)
Genetic disease - kennedy disease
180
what causes pseudobulbar palsy?
disease of corticobulbar tracts due to cerebrovascular events, MS, MND, neurosyphilis etc that causes bulbar symptoms along with exaggerated jaw jerk and emotional lability
181
what are 7 causes of cerebral palsy?
antenatal - maternal infection, trauma in pregnancy
perinatal - birth asphyxia, pre-term birth
postnatal - meningitis, severe neonatal jaundice, head injury
182
what are 4 types of cerebral palsy?
spastic - hypertonia (damage to UMNs)
dyskinetic - muscle tone control problems with abnormal movements (damage to basal ganglia)
Ataxic - coordination problems (damage to cerebellum)
Mixed
183
what are 6 presentations of cerebral palsy?
failure to meet milestones
increased/decreased tone
hand preference before 18 months
problems with coordination, speech or walking
feeding or swallowing problems
learning difficulties
184
what medications may someone with cerebral need?
muscle relaxants for spasticity and contractures - baclofen
antiepileptics
glycopyrronium bromide - excessive drooling
185
what is the grading system for hypoxic-ischaemic encephalopathy grade called?
Sarnat staging
186
what are 3 features of mild hypoxic ischaemic encephalopathy?
poor feeding, irritability, hyperalertness
resolves within 24 hours
normal prognosis
187
what are 3 features of moderate hypoxic ischaemic encephalopathy?
poor feeding, lethargy, hypotonic and seizures
can resolve in weeks
up to 40% develop cerebral palsy
188
what are 3 features of severe hypoxic ischaemic encephalopathy?
reduced consiousness, apnoea, flaccidity and reduced or absent reflexes
up to 50% mortality
90% cerebral palsy
189
what is a possible treatment of hypoxic ischaemic encephalopathy?
therapeutic hypothermia - cool down core temp of baby on ICU for 72 2hours which reduced inflammation and neurone loss after acute hypoxic injury
190
what is the inheritance pattern of muscular dystrophy?
X-linked recessive
191
what is the name of the sign in muscular dystrophy where people will use their hands and knees to push themselves up due to proximal muscle weakness?
Gower's sign
192
what is gower's sign for?
muscular dystrophy
193
what causes duchennes mucular dystrophy?
defective dystrophin gene on x-chromosome
Dystrophin holds muscles together at cellular levels
194
what is the average age of presentation for duchennes MD?
3-5 years - presents with weakened pelvic muscles
195
what is the life expectancy of duchennes?
25-35 years
196
when medication can be given to slow the progression of duchennes MD?
oral steroids
creatine suplemmentation
197
what is Beckers MD?
less severe than duchennes
presentation 8-12 years
require wheelchairs late 20s-30s
198
what is myotonic dystrophy?
usually presents in adulthood
progressive muscle weakness
prolonged muscle contractions
cataracts
cardiac arrhythmias
199
what is facioscapulohumeral muscular dystrophy?
usually presents in childhood with weakness around face progressing to shoulders and arms
classic initial symptoms - sleeping with eyes slightly open, weakness pursing lips
200
what is oculopharyngeal muscular dystrophy?
presents in late adulthood with weakness of ocular muscles and pharynx
typically presents with - bilateral ptosis, restricted eye movement, swallowing problems
201
what is limb girdle musclar dystrophy?
usually presents in teenagers with progressive weakness around limb girdles
202
what is emery-dreifuss muscular dystrophy?
usually present in childhood with contractures of elbows or ankles
also have progressive weakness and wasting of muscles starting with upper arms and lower legs
203
what is the inheritance pattern for huntingtons?
Autosomal dominant
204
what is the genetic error in huntingtons?
trinucleotide repeat disorder of the HTT gene on chromosome 4
205
what chromosome is affected in huntingtons?
chromosome 4
206
what genetic phenomenon is seen in huntingtons?
anticipation
207
what is genetic anticipation?
sucessive generations have more trinucleotide repeats resulting in earlier onset and increased severity of disease
208
what are 4 symptoms of huntingtons disease?
Cognitive. behavioural and mood changes
eye movement disorders
speech and swallow difficulties
Chorea
209
what is the life expectancy of huntingtons post diagnosis?
15-20 years
210
what are 3 medications that can be used for chorea in huntingtons?
Benzos - tetrabenazine
Antipsychotics
Amantadine
211
what causes Creutzfeldt-Jakob disease?
Prions
212
what is the general life expectancy after presentation of someone with CJD?
<1 year
213
what are 5 symptoms of prion disease?
sudden onset cognitive impairment and decline
Ataxia
Myoclonus
Psychiatric symptoms
parkinsonism
214
what are 4 ways prion disease can be passed on?
Genetically
Contaminated blood products
Contaminated surgical instruments
Consumption of contaminated beef
215
what is a specific test that can be done for CJD?
real time quaking induced conversion of the CSF
216
what do the nerves of the cauda equina supply?
sensation to lower limbs, perineum, bladder and rectum
Motor supply to lower limbs, anal and urethral sphincters
Parasympathetic control of bladder and rectum
217
what are 5 causes of cauda equina?
Herniated disc
tumours
spondylolisthesis (displacement of vertebra)
Abscess
Trauma
218
what are 9 red flags of cauda equina?
Lower back pain
Saddle anesthesia
loss of sensation to bladder and rectum
Urinary retention or incontinence
Faecal incontinence
Bilateral sciatica
Bilateral/severe motor weakness of legs
Reduced anal tone
sexual dysfunction
219
what is the management of cauda equina?
Lumbar decompression surgery
220
what is the treatment for metastatic spinal cord compression?
high dose dexamethasone
Analgesia
Surgery
radio/Chemo
221
what is a key feature of the pain in metastatic spinal cord compression?
Back pain worse on coughing or straining
222
what is the first line investigation for a metastatic spinal cord compression?
Gandolinium enhanced MRI Spine
223
what are 3 different types of spinal stenosis?
central - narrowing of central spinal canal
lateral - narrowing of nerve root canals
foramina - narrowing of intervertebral foramina
224
what are 5 causes of spinal stenosis?
Congenital
degenerative changes
Herniated discs
thickening of ligamentum falvum/posterior longitudinal ligament
Spinal fractures
spondylolisthesis
tumours
225
what exacerbates central spinal stenosis?
Standing up straight and walking
226
what relieves spinal stenosis?
bending forwards and resting
227
what is anterior cord syndrome?
incomplete spinal cord injury due to infarct of the anterior 2/3rds of the spine often because of flexion injuries to C spine
228
what is the presentation of anterior cord syndrome?
impaired pain and temperature sensation with intact vibration, proprioception and light touch
Motor deficits are also present
229
what are 3 causes of anterior cord syndrome?
Iatrogenic (throacic/abdo surgery)
Trauma
Hypo perfussion/ischaemia of the anterior spinal artery
230
what is the presentation of brown sequard syndrome?
paralysis, loss of proprioception and vibration on the side of the lesion and loss of pain and temperature on the contralateral side
231
what is a radiculopathy?
damage to the nerve root - pinched/trapped nerve
232
what examination can be done for eliciting sciatica pain?
Straight leg raise
sciatic stretch
233
what is the first line management of sciatica?
Amitriptyline
Duloxetine
234
which nerves form the sciatic nerve?
L4-S3
235
what cancers most commonly metastasise to bone?
Prostate
Renal
Thyroid
Breast
Lung
236
what medication can be given for back spasms?
benzodiazepams - diazepam => less than 5 days course
237
what are 5 symptoms of diabetic neuropathy?
peripheral sensation loss - glove and stocking
Peripheral pain - burning/prickling
reduced ankle reflexes
painless injuries
Erectile dysfunction
238
what can be used to treat painful diabetic neuropathy?
gabapentin/pregabalin
++ Glycaemic control!
239
what is narcolepsy?
chronic condition of disrupted sleep wake cycle and REM sleep intrusion into wakeful state
240
what is the classic tetrad of narcolepsy?
excessive daytime sleepiness
cataplexy
sleep paralysis
hypnagogic/hypnopompic hallucinations
241
what is the management of narcolepsy?
Sleep hygiene and lifestyle changes, trigger avoidance
Sodium oxybate - for cataplexy
Modafinil - for excessive daytime sleepiness
242
what are 4 causes of acute transverse myelitis?
Infection
Vaccination
NMO - Neuromyelitis optica
MOG - Myelin Oligodendrocyte Glycoprotein
243
what nutrients affect spinal cord functioning?
B12
copper
244
How does NOS affect B12?
effects of B12 blocked by NO though there is enough B12 in the body
245
what nutrients affect spinal cord functioning?
B12
copper
246
what are 5 causes of acute transverse myelitis?
Infection
Vaccination
MS
NMO - Neuromyelitis optica
MOG - Myelin Oligodendrocyte Glycoprotein
246
How does NOS affect B12?
causes functional deficiency of B12 => cannot be converted to active form
247
what are the 3 categories of GCS?
Eye response
verbal response
motor reponse
248
what are the GCS levels for eye response?
1 - no eye opening
2 -- to pain
3 - to verbal command
4 - spontaneously
249
what are the GCS levels for verbal response?
1 - no response
2 - incomprehensible sound
3 - inappropriate words
4 - confused
5 - orientated
250
what are the GCS levels for motor response?
1 - no response
2 - extension to pain
3 - flexion to pain
4 - withdrawal from pain
5 - localised pain
6 - obeys commands
251
what is charcot marie tooth disease?
genetic condition that causes peripheral motor and sensory deficits which usually presents <10 years but can present later in life depending on the type
252
what are 7 classical features of charcot marie tooth?
high foot arches - pes cavus
disal muscle wasting and inverted champagne bottle legs
weakness in lower legs - particularly ankle dorsiflexion
weakness in hands
reduced tendon reflexes
reduced muscle tone
peripheral sensory loss
253
what are 5 possible causes of peripheral neuropathy?
ABCDE
alcohol
B12 deficiency
Cancer and CKD
Diabetes and Drugs
Every vasculitis
254
what are 3 medications that can cause peripheral neuropathy?
isoniazid
amiodarone
cisplatin
255
what are 4 features of lambert-eaton myasthenic syndrome?
Progressive muscle strengthening
Limb girdle weakness
Hyporeflexia
autonomic symptoms - dry mouth, impotence, difficulty urinating
256
what condition most typically occurs with lambert-eaton syndrome?
small cell lung cancer
causes antibodies against voltage-gated calcium channels in presynaptic terminals of the NMJ
257
what is central cord syndrome?
incomplete cord injury caused by trauma to the cervical spine leading to weakness or neurological symptoms worse in the upper > lower limbs
258
what nerve is compressed in carpal tunnel?
median nerve
259
what is the conservative treatment of carpal tunnel?
6 weeks of
night wrist splint
corticosteroid injection into carpal tunel
hand exercises and mobalisation techniques
260
what are 5 presentations of labert-eaton syndrome?
proximal muscle weakness
intraoccular muscle weakness - double vision
levator muscle weakness - ptosis
oropharyngeal muscle weakness - dysphagia
autonomic dysfunction - dizziness, impotence, dry mouth, blurred vision
261
what is the treatment for lambert-eaton syndrome?
1 - amifampridine
imunosuppresion
IVIG
plasmapheresis
262
what are hamartomas?
benign neoplastic growths
263
what condition are hamartomas seen in?
tubular sclerosis
264
what genetic mutations cause tubular sclerosis?
TSC1 gene on chromosome 9 - codes for hamartin
TSC2 gene on chromosome 16 - codes for tuberin
265
what are 6 skin signs seen in tubular sclerosis?
ash leaf spots
shagreen patches - thickened, dimpled, pigmented patch of skin
angiofibromas - small skin coloured or pigmented papules over nose and cheeks
subungual fibromata - circular painless lumps growing from nail bed
cafe au lait spots
poliosis - isolated patch of white hair
266
what are 2 neurological features of tubular sclerosis?
epilepsy
learning disability and developmental delay
267
what are 5 non-neuro signs of tubular sclerosis?
rhabdomyomas of heart
gliomas
polycystic kidneys
lymphagioleimyomatosis
retinal hamartomas
268
damage to white nerve causes winging of the scapula?
spinal accessory - CN11
269
what is the most common cause of foot drop?
common peroneal nerve injury
270
what is the CSF picture in viral encephalitis?
raised WBCs
HSV +ve PCR
271
what is the treatment for herpes encephalitis?
IV acyclovir 10mg/kg every 8 hours for 14 days (21 days if immunocompromised)
272
what is the most common viral cause of encephalitis?
HSV
273
what is the most common cause of autoimmune encephalitis?
N-methyl D-aspartate receptor antibody encephalitis
274
what scale is used for stroke?
national institute of health stroke scale (NIHSS)
275
what is a mild NIHSS stroke?
1-5
276
what is a moderate NIHSS stroke?
5-14
277
what is a severe NIHSS stroke?
15-24
278
what is a very severe NIHSS stroke?
>25
279
what is the management for TIA or minor stroke if thrombolysis/thrombectomy are not appropriate?
dual anti-platelet - aspirin 300mg followed by 75mg daily + clopidogrel 300mg followed by 75mg daily
280
what 5 investigations should be done for stroke?
CT
MRI
ECG
carotid doppler
281
what is the management of moderate/severe stroke if thrombectomy/thryombolysis are not indicated?
aspirin 300mg then 75mg daily for 2 weeks until starting antiplatelet therapy - clopidogrel
282
what is CSF absorbed by?
arachnoid granules
283
what is the most common cause of hydrocephalus?
aqueductal stenosis
284
what are 4 congenital causes of hydrocephalus?
aqueductal stenosis
arachnoid cysts - blood csf outflow
arnold-chiari malformations - cerebellum herniates through foramen magnum
Chromosomal abnormalities and congenital malformations
285
what are 5 signs of hydrocephalus in babies?
rapid increasing head circumference
bulging anterior fontanelle
poor feeding and vomiting
poor tone
sleepiness
286
what is the management for hydrocephalus?
vetriculoperitoneal shunt
287
what are 5 ventriculoperitoneal shunt complications?
infection
blockage
excessive drainage
intraventricular haemorrhage during surgery
outgrowing them
288
what are 3 features of normal pressure hydrocephalus?
gait apraxia
cognitive impairment
urinary frequency/incontinence/urgency - also sometimes foecal
289
what is the management for normal pressure hydrocephalus?
ventriculoperitoneal shunt is suitable for surgery
290
what is the presentation of ME? (8)
persistent disabling fatigue
post exertional malaise
unrefreshing sleep
congnitive and autonomic dysfunction
myalgia
arthralgia
headaches
sore throat and lymph nodes (no lymphadenopathy)
291
what are 5 risk factors for brain abcess?
sinusitis/otitis media
dental procedure
meningitis
recent head/neck/neuro surgery
HIV/immunocompromised
292
what on MRI with contrast in brain abcess?
ring enhancing lesion
293
what is the management of brain abcess?
IV vancomycin
IV Metronidazole
IV ceftriaxone
SURGERY
294
what is mononeuritis multiplex?
progressive motor and sensory deficits in the distribution of specific peripheral nerves commonly caused by vasculitis, hypersensitivity reactions or infection.
295
what is an investigations for mononeuritis multiplex?
electromyogram
296
what is the management for mononeuritis multiplex?
corticosteroids - pred 1mg/kg/day BO
+/- immunosupression - oral cyclophosphamide
297
what are 5 symptoms of malaria?
fever
chills
sweats
headache
myalgia
298
what is the 1st line investigation for malaria?
giemsa stained blood film
299
what is the most life threatening malaria infection?
plasmodium flaciparum
300
what mosquitos cause malaria?
female anopheles
301
what is the 1st line management of uncomplicated malaria?
hydroxychloroquine
302
what is the 1st line management of complicated malaria?
artesunate IV
303
what is wernicke's aphasia?
receptive aphasia causing sentences to not make sense and comprehension to be impaired
304
where is wernicke's area?
superior temporal gyrus of dominant hemisphere
305
what blood vessel supplies wernicke's area?
inferior devision of (usually) left middle cerebral artery
306
what is broccas aphasia?
expressive aphasia causing halting speech and impaired repetition with normal comprehension
307
where is broccas area?
inferior frontal gyrus of dominant hemisphere
308
what blood vessel usually supplies broccas area?
superior division of the (usually) left MCA
309
what is conduction aphasia?
due to stroke affecting arcuate fasiculus (connection between wernickes and broccas). Fluent speech with impaired repetition
310
which tumours most commonly metastasise to brain?
lung (most common)
breast
bowel
skin (namely melanoma)
kidney
311
what is the most common primary brain tumour in adults?
glioblastoma multiforme
312
what does glioblastoma multiforme look like on imaging?
solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.
313
what cells do meningiomas arise from?
arachnoid cap
314
what genetic condition is associated with bilateral acoustic neuroma?
neurofibromatosis type II
315
what is the most common primary brain tumour in children?
Pilocytic astrocytoma
316
what phenomena are seen in occipital lobe focal seizures?
visual flashers or floaters
317
what phenomena are seen in parietal lobe focal seizures?
sensory - parasthesia
318
what phenomena are seen in frontal lobe focal seizures?
head/leg movements, posturing, jacksonian march, post-ictal weakness (todds paresis)
319
what phenomena are seen in temporal lobe focal seizures?
pre-ictal aura
automatisms
320
what is the GCS scoring?
MoVE - 6,5,4
321
what is the MOA of memantine?
NMDA receptor antagonist
322
what are 2 side effects of donepezil?
contraindicated in bradycardia
can cause insomnia
323
what dermatome is the thumb and first finger?
C6 - make a 6 with left hand thumb and first finger
324
what dermatome is the nipple?
T4 - teat pore
325
what dermatome is the umbilicus?
BellybuT-TEN = T10
326
what dermatome is the inguinal ligament?
L1 - Li
327
what dermatome is the knee caps?
ALL Fours - L4
328
what dermatome is the big toe and foot dorsum?
L5 - largest of 5 toes
329
what dermatome is little toe?
S1 - smallest one
330
what dermatome is the genitalia?
S2/3
331
what are 3 side effects of dopamine receptor agonists?
pulmonary, retroperitoneal and cardiac fibrosis
impulse control
excessive daytime sleepiness
332
what is autonomic dysreflexia?
In patients with spinal injury at or above T6 - response to painful stimuli that causes unbalanced physiological response - extreme hypertension, flushing, sweating (above level of lesion), agitation and extreme hypertension
333
what is the most common cause of autonomic dysreflexia?
faecal impaction or urinary retention
334
what is the management of autonomic dysreflexia?
removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia
335
what are 8 side effects of carbamezapine?
P450 enzyme inducer
dizziness and ataxia
drowsiness
headache
visual disturbances (especially diplopia)
Steven-Johnson syndrome
leucopenia and agranulocytosis
hyponatraemia secondary to syndrome of inappropriate ADH secretion
336
what are 5 causes of foot drop?
common peroneal nerve lesion
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
CNS lesion (stroke etc)
337
what examination findings indicate peroneal nerve injury?
weakness of foot dorsiflexion and eversion. Reflexes normal
338
what examination findings indicate L5 radiculopathy?
weakness of hip abduction
339
what is the gold standard investigation for venous sinus thrombosis?
MRI venogram
340
what is lateral medullar syndrome and it's 6 features?
Occurs due to occlusion of posterior inferior cerebellar artery (PICA)
Ataxia
Nystagmus
ipsilateral dysphagia, facial numbness, CN palsy
Contralateral - limb sensory loss
341
What is cushing's triad of raised ICP?
widening pulse pressure
bradycardia
irregular breathing
342
what is the management of raised ICP?
head elevation by 30 degrees
IV manitol
controlled hyperventilation
removal of CSF
343
what does occlusion of the retinal artery cause?
Amaurosis fugax
344
what does occlusion of the basillar artery cause?
locked in syndrome
345
what is the presentation of wernickes encephalopathy?
nystagmus or ophthalmoplegia
Ataxia
encephalopathy - confusion, disorientation
peripheral sensory neuropathy
346
what causes wernicke's encephalopathy?
Thiamine B1 deficiency
alcoholism
persistant vomiting
stomach cancer
dietary deficiency
347
what are examples of 5-HT3 antagonists?
Ondansetron
Palonesetron
Anti-emetics used in chemo
348
what are 2 side effects of ondansetron?
prolonged QT + ventricular tachycardia
constipation
349
what are 2 symptoms of a posterior cerebellar stroke?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
350
what are 2 signs of webbers syndrome (infarct of branches of the posterior cerebral artery that supply midbrain)?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
351
what artery is affected in lateral pontine syndrome?
Anterior inferior cerebellar artery
352
what are of lateral pontine syndrome?
Ipsilateral: facial paralysis and deafness
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
353
what scale measures disability?
Barthel index
354
what quick stroke assessment scale is used by medical professionals?
Rossier
355
when is statin therapy started after stroke?
cholesterol >3.5
wait 48 hours to avoid haemorrhagic transformation
356
what is the 1st line secondary prevention of stroke?
Aspirin - 75mg
clopidogrel - 75mg
357
what is the 2nd line secondary prevention of stroke?
Aspirin + modified release dipyridamole
358
what is the management of idiopathic intracranial hypertension?
1 - loose weight
2 - Acetazolamide
3+ - Lumboperitoneal shunt, Optic nerve sheath fenestration, Ventriculoperitoneal shunt
Phase 3A
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