neurology Flashcards
1
Q
first line for muscle spasticity in MS
A
baclofen and gabapentin
2
Q
how to treat a brain abscess
A
IV 3rd-generation cephalosporin (ceftriaxone) + metronidazole
(+ intercranial pressure mx (dexamethasone) )
3
Q
what does it show on CT scan w brain abscess
A
ring enhancing lesion
4
Q
what would you find on a head CT for Alzheimer’s
A
atrophy of cortex + hippocampus
5
Q
what would you find on a head CT for frontotemporal dementia (pick’s disease)
A
atrophy of the frontal + temporal lobes
knife-blade appearance
6
Q
what do you find on a head CT for SAH
A
Hyper-attenuating area in the basilar cistern (Circle of Willis)
7
Q
most common cancers than spread to the brain
A
lung (most common)
breast
bowel
skin (namely melanoma)
kidney
8
Q
tx for cerebral oedema in context of malignancy
A
dexamethasone
9
Q
tx increased icp
A
mannitol
10
Q
what happens if you give folate to someone who is b12 deficient
A
it can precipitate subacute combined degeneration of the cord
11
Q
features of subacute combined degeneration of the cord
A
dorsal column involvement
- distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
- impaired proprioception and vibration sense
lateral corticospinal tract involvement
- muscle weakness, hyperreflexia, and spasticity
- upper motor neuron signs typically develop in the legs first
- brisk knee reflexes
- absent ankle jerks
- extensor plantars
spinocerebellar tract involvement
- sensory ataxia → gait abnormalities
- positive Romberg’s sign
12
Q
most common site of berry aneurysm
A
anterior communicating artery
13
Q
what condition is assoc w berry aneurysms
A
AD PKD
14
Q
presentation of normal pressure hydrocephalus
A
dementia
gait abnormalities
urinary incompetence
15
Q
neuro-imaging in normal pressure hydrocephalus
A
ventriculomegaly in the absence of sulcal enlargement
16
Q
tx normal pressure hydrocephalus
A
ventriciuloperitoneal shunting
17
Q
what is the barthel index
A
scale that measures disability/dependence in ADLs in stroke px
18
Q
what is the most common cause of brain mets
A
lung tumours
19
Q
SEs of levodopa
A
on-off effect
cardiac arrhythmias
N&V
psychosis
reddish discol of urine
dyskinesias
20
Q
parkinson’s presentation
A
cogwheel rigidity
bradykinesia
- small writing
- shuffling gait
- can’t initiate movement/turn
tremor
- asymmetric, AT REST, improves w voluntary activity
mask-like facies
postural instability
21
Q
describe parkinson’s tremor
A
resting
pin-rolling
4-6 Hz
improves w voluntary movement, worsens when distracted
No change with alcohol
Asymmetrical
22
Q
what is levodopa usually combined w + why
A
peripheral decarboxylase inhibitors e.g. carbidopa
stops levodopa being broken down in the body before it gets the chance to enter the brain
23
Q
what is dystonia
A
This is where excessive muscle contraction leads to abnormal postures or exaggerated movements.
24
Q
what is chorea
A
These are abnormal involuntary movements that can be jerking and random.
25
what is athetosis
These are involuntary twisting or writhing movements usually in the fingers, hands or feet.
26
what is syringomyelia
cape-like loss of pain + temp sensation due to a collection of cerebrospinal fluid within the spinal cord
27
what is brown-sequard
damage to lateral 1/2 of the spinal cord
ipsilateral loss of proprioception + vibration (dorsal column decussates at medulla after leaving spinal cord)
contralateral loss of pain + temp (spinothalamic tract decussates asap)
28
sx optic neuritis
unilateral decrease in visual acuity over hours or days
poor discrimination of colours, 'red desaturation'
pain worse on eye movement
relative afferent pupillary defect (respond diff to light)
central scotoma (blind spot)
think ms?!
29
3rd nerve palsy
ptosis
dilated pupil
looking down + out
30
encephalitis sx
fever
headache
altered mental status
31
viral encephalitis tx
IV aciclovir
32
bacterial encephalitis tx
IV ceftriaxone
33
what is Ramsay Hunt syndrome
infection of facial nerve by HZV
34
presentation of Ramsay Hunt syndrome
ipsilateral LMN facial palsy (forehead not spared)
ear pain
hearing loss
vertigo
vesicular rash in outer ear
35
what is bell's palsy
LMN facial nerve lesion
36
presentation of bell's palsy
ipsilateral facial paralysis (forehead not spared)
post-auricular pain
altered taste
dry eyes
hyperacusis (reduced tolerance to sound)
37
tx bell's palsy
oral prednisolone 10 days
if no improvement after 3 wks, refer urgently to ENT
eye drops
38
presentation of acoustic neuroma (vestibular schwannoma)
vertigo
hearing loss
tinnitus
absent corneal reflex
fullness in ear
facial nerve palsy if big
39
ix acoustic neuroma (vestibular schwannoma)
MRI of cerebellopontine angle
audiometry
40
mx acoustic neuroma (vestibular schwannoma)
urgent ENT referral
41
what is acoustic neuroma (vestibular schwannoma) assoc w
neurofibromatosis type 2 (AD)
42
what is charcot-marie-tooth
most common hereditary sensory + motor peripheral neuropathy
43
charcot-marie-tooth presentation
Patients can present with lower motor neurone signs in all limbs and reduced sensation (more pronounced distally).
motor loss
distal muscular weakness + atrophy
hyporeflexia
hx of freq sprained ankles
foot drop
high arched feet - pes cavus
hammer toes
stork leg deformity
44
what is chronic inflammatory demyelinating polyneuropathy
chronic version of GBS
progressive weakness + impaired sensory func in legs + arms
45
tx of chronic inflammatory demyelinating polyneuropathy
corticosteroids
46
what is degenerative cervical myelopathy + presentation
spinal cord compression in neck
loss of fine motor func in upper limbs, pain, numbness
Hoffman's sign
50% of patients were initially incorrectly diagnosed and sometimes treated for carpal tunnel syndrome
47
dx degenerative cervical myelopathy
MRI cervical spine = disc degeneration + ligament hypertrophy
cord signal change
48
what is autonomic dysreflexia
syndrome that occurs in px w spinal cord injury at/> T6.
sympathetic reflex caused by trigger
49
what commonly triggers autonomic dysreflexia
faecal impaction or urinary retention
px cld:
- bed bound/immobile
- meds (opioids)
- anal fissure
- Hirschsprungs
- dementia
- spinal trauma
50
sx of autonomic dysreflexia
extreme HTN
flushing + sweating above lesion level
agitation
51
where does the spinal cord end
L1-2 - splits to cauda equina
52
what does the dorsal column do
ascending tract
carries sensory info to the brain
fine touch
vibration sense
proprioception
53
where does the dorsal column decussate
after leaving the spinal cord at medulla
therefore stays in same lane for ages
therefore if damaged problems are on ipsilateral side
54
what does the spinothalamic tract do
ascending tract
carries sensory info to brain
pain + temperature
55
where does the spinothalamic tract decussate
as soon as in spinal cord so ascends contralaterally
therefore crosses lanes immediately
therefore if damaged problems on opposite side (2 segments below injury)
56
what is the corticospinal tract
descending pyramidal tract sending motor signals to muscles
voluntary muscle control
immediately changes lanes + travels contralaterally
57
what is the corticobulbular tract
descending pyramidal tract
voluntary muscle control of the face, head + neck
58
what are upper motor neurons
originate in cerebral cortex + travel down to brainstem or spinal cord
59
what are lower motor neurons
begin in spinal cord + innervate muscles + glands throughout the body
60
what are signs of upper motor neuron lesions
increased tone
- spasticity
- clonus?
increased reflexes
positive babinski sign
positive hoffman sign
upper limb has weak extensor muscles
lower limb has weak flexor (bend) muscles
pyramidal drift
61
what are signs of lower motor neuron lesions
decreased tone - flaccid
decreased reflexes/none
fasciculations
muscle wasting
- bulbar = speech + swallow
- resp - diaphragm higher up
- hand
- foot drop
62
what do extrapyramidal tracts do
involuntary control i.e. tone + balance
63
what are manifestations of extrapyramidal injury
parkinsonism
- rigidity
- bradykinesia
- tremors
- postural deficits
chorea
athetosis
dystonia
64
initial dementia blood screen
FBC
U&Es
LFTs
Ca
glucose
ESR/CRP
TFTs
vitamin B12
folate levels
65
what is wernicke's encephalopathy + how does it present
thiamine (v B1) deficiency (in alcoholics)
ophthalmoplegia
ataxia
confusion
66
wernicke's encephalopathy tx
thaimine replacement -> pabrinex
67
what do you get if you don't treat wernicke's encephalopathy + how does it present
Korsakoff syndrome
antero + retrograde amnesia
confabulation (false mems)
68
presentation of multiple system atrophy
parkinsonism
autonomic disturbance
- erectile dysfunction: often an early feature
- postural hypotension
- atonic bladder
cerebellar signs
69
Posterior inferior cerebellar artery stroke (PICA) (lateral medullary syndrome) (Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia
Nystagmus
sudden vomiting/vertigo
to differentiate from ANTERIOR....
POSTERIOR has no deafness or facial paralysis
70
Anterior inferior cerebellar artery stroke (AICA) (lateral pontine syndrome)
Ipsilateral: facial paralysis and deafness, facial pain + temp loss
contralateral: body pain + temp loss
pin point pupils
to differentiate from POSTERIOR....
ANTERIOR has deafness and facial paralysis as well as the ipsilateral face sx + contralateral body sx
71
what does a basilar artery stroke cause
locked in syndrome
72
what does a retinal/ophthalmic artery stroke cause
Amaurosis fugax
73
Anterior cerebral artery stroke
Contralateral hemiparesis and sensory loss
Lower extremity more affected (ants have legs)
logical thinking/personality
74
Middle cerebral artery stroke
Contralateral hemiparesis and sensory loss
Upper extremity more affected
Contralateral homonymous hemianopia
Aphasia
Most common
75
Posterior cerebral artery stroke
Contralateral homonymous hemianopia with macular sparing
Visual agnosia (can't rec faces)
76
Weber's syndrome
Ipsilateral CN III palsy (down + out)
Contralateral weakness of upper and lower extremity
(branches of the posterior cerebral artery that supply the midbrain)
77
what do total anterior circulation infarcts have to have
involves middle and anterior cerebral arteries
all 3 of the criteria are present:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
78
what do partial anterior circulation infarcts have to have
involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery
2 of the criteria are present:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
79
what do lacunar infarcts have to have
presents with 1 of the following:
1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
2. pure sensory stroke.
3. ataxic hemiparesis
80
ix TIA
MRI brain w diffusion-weighted imaging
only do to exclude other dx
all patients should have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy (to check as a source of emboli)
81
immediate tx TIA
aspirin 300mg
82
what to do if px has had more than 1 TIA or has a suspected cardioembolic source or severe carotid stenosis
discuss the need for admission or observation urgently with a stroke specialist
83
what to do if If the patient has had a suspected TIA in the last 7 days
arrange urgent assessment (within 24 hours) by a specialist stroke physician
84
what to do if the patient has had a suspected TIA which occurred more than a week previously
refer for specialist assessment as soon as possible within 7 days
85
driving + TIAs
Advise the person not to drive until they have been seen by a specialist.
Can start driving if sx free after 1 month
86
2ndary prev after TIA
antiplatelet therapy after initial aspirin
- clopidogrel
high-intensity statin
87
when to do a carotid artery endarterectomy in TIA
carotid stenosis > 70% + sx
88
tx ischaemic stroke
aspirin 300 mg
< 4.5 hrs of onset = thombolysis w alteplase AND thrombectomy
4.5 - 6 hrs of onset = thrombectomy
or thrombectomy after 6 hrs if there is the potential to salvage brain tissue, as shown by imaging
(but not usually after 24 hrs)
89
features of acute subdural haemorrhage
4-7 wks following high impact trauma
fluctuating conc + sx come on gradually
crescent shaped
hyperdense
90
features of chronic subdural haemorrhage
wks/months
confusion
decreased conc
neuro def
more likely elderly + alcoholic as brain atrophy
or in shaken baby syndrome
hypodense
crescent shaped
slow bleeding
91
what ruptures in subdural haemorrhage
bridging veins
92
tx acute subdural haemorrhage
decompressive craniotomy
93
tx chronic subdural haemorrhage
burr hole evacuation
94
features of extradural haemorrhage
sudden onset soon after injury following brief lucid interval
headache
compression of CN III - fixed + dilated pupil
lemon shaped (biconvex)
95
what ruptures in extradural haemorrhage
middle meningeal artery
96
tx extradural haemorrhage
burr holes
craniotomy
ligation of bleeding artery
97
ix SAH
CT = acute blood (hyperdense/bright on CT) is typically distributed in the basal cisterns, sulci and in severe cases the ventricular system.
if done within 6 hrs of sx onset + normal, no LP needed - consider an alt dx
if CT is done 6+ hrs after sx onset + normal
-> LP (at least 12 hrs after sx) =
xanthochromia (RBC breakdown) + normal/raised opening pressure
-> refer to neurosurgery
98
ix is to identify a causative pathology of SAH
CT intracranial angiogram (to identify a vascular lesion)
99
mx SAH
coil/clip
IV nimidopine 21 days to prevent vasospasm
100
what signs do you get in GBS
LMN
101
features of temporal lobe seizure
Hallucinations
Epigastric rising/Emotional
Automatisms (lip smacking)
Deja Vu/Dysphasia post ictal
102
features of a frontal lobe seizure
Head/leg movements, posturing
Post ictal Todd's palsy (weakness)
Jacksonian march (clonic movements travelling proximally)
103
what is an atonic seizure
suddenly fall to the ground due to sudden loss of muscle tone
confused after
104
EEG on absence seizure
3Hz spike + wave
105
what is status epilepticus
seizure > 5 mins / > 2 in 5 mins not returning to normal
106
drug tx for male generalised tonic clonic epilepsy
sodium valproate
107
drug tx for female generalised tonic clonic epilepsy
lamotrogine/levetiracetam
108
drug tx for males in myoclonic epilepsy
sodium valproate
109
drug tx for focal seizures in epilepsy
1 = lamotrogine/levetiracetam
2 = carbamazepine
110
drug tx for females in myoclonic epilepsy
levetiracetam
111
tx status epilepticus
ABC
- airway adjunct
- oxygen
- check blood glucose
Pre-hosp = PR diazepam / buccal midazolam
Hosp = IV lorazepam. Repeat after 5-10 mins
Ongoing = phenytoin
Anaesthesia
112
what are infantile spasms (West syndrome)
rare disorder starting in infancy at around 6 months of age
brief spasms of sudden uncontrolled movements including flexion of the head, trunk, limbs, and extension of the arms (Salaam attack)
poor prognosis
usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic
113
EEG of infantile spasms
Hypsarrhythmia
114
drug tx absence seizures
ethosuximide
115
drug tx for female tonic / atonic seizures in epilepsy
lamotrigine
116
drug tx for male tonic / atonic seizures in epilepsy
sodium valproate
117
features of MG
Autoimmune autoantibodies at post-synaptic membrane = AChR. These prevent ACh from being able to stim the receptor + trigger muscle contractions
Assoc w thymomas (CT to exc) -> dry cough, breathlessness
Muscle fatiguability
- extraocular
- proximal
- ptosis
- dysphagia
118
ix in MG
Acetylcholine receptor (ACh-R) antibodies (85% of patients)
Muscle-specific kinase (MuSK) antibodies (10% of patients)
LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%)
EMG (nerve conduction studies)
CT
Edrophonium Test
119
MG tx
anticholinesterase inhibitors = PYRIDOSTIGMINE
Prednisolone
Thymectomy
120
tx for myasthenic crisis
plasmapheresis
IV immunoglobulin
121
what can make sx of MG worse
BBs
gentamicin is CI
122
features of Lambert Eaton syndrome
AI antibodies against VG calcium channels in pre-synaptic membrane so ACh cannot be exocytosed
assoc w small cell lung cancer
proximal muscle weakness espesh in lower limbs
strength increases w effort
autonomic features
123
tx Lambert Eaton syndrome
Amifampridine
124
features of GBS
progressive, symmetrical weakness of all the limbs
- classically ascending
- reduced or absent reflexes
hx viral infection e.g. gastroenteritis
125
ix GBS
LP
- rise in protein with a normal WBCS (albuminocytologic dissociation) - found in 66%
nerve condution studies
- decreased motor nerve conduction velocity (due to demyelination)
- prolonged distal motor latency
- increased F wave latency
126
what is GBS
an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni)
127
most common causative organism of viral encephalitis
herpes simplex virus 1
128
CSF in viral encephalitis
high lymphocytes
high protein
129
EEG in viral encephalitis
lateralised periodic discharges at 2 Hz
130
causes of bacterial meningitis
NEISSERIA MENINGITIDIS
Streptococcus pneumoniae (pneumococcus)
Haemophilus influenzae
Group B streptococcus (GBS) (particularly in neonates as GBS may colonise the vagina)
Listeria monocytogenes (particularly in neonates)
131
causes of viral meningitis
Enteroviruses (e.g., coxsackievirus)
Herpes simplex virus (HSV)
Varicella zoster virus (VZV)m
132
meningitis presentation
Fever
Neck stiffness
Vomiting
Headache
Photophobia
Altered consciousness
Seizures
meningococcal septicaemia (when it has entered the blood) = non-blanching rash.
133
special to look for meningeal irritation
Kernig’s test
Brudzinski’s test
134
LP for bacterial meningitis
cloudy
high protein
low glucose (relative to serum)
high neutrophils (polymorphs)
135
LP for viral meningitis
clear
mildly raised/normal protein
normal glucose
high lymphocytes
136
features of benign essential tremor
fine tremor
6-12 Hz
symmetrical
more prominent w voluntary movement
worse when tired, stressed or after caffeine
improved by alcohol
absent during sleep
137
mx benign essential tremor
just to improve sx:
propranolol
primidone (a barbiturate anti-epileptic medication)
138
what is MS
chronic + progressive AI condition involving demyelination in the CNS (oligodendrocytes) (multiple sites)
T cell mediated type 4 hypersensitivity reaction
begins early adulthood, more common in F
139
typical presentations of MS
LOSS NB
- Lhermitte’s sign - electric shock runs down back + radiates to limbs on neck flexion
- Optic neuritis – impaired vision + eye pain
- Spasticity + other pyramidal signs
- Sensory symptoms + signs - ataxia
- Nystagmus, double vision + vertigo
- Bladder + sexual dysfunction
Exacerbated by heat – showers, hot weather, saunas (Uhthoff’s phenomenon)
140
types of MS
relapsing + remitting (80%)
- onset over days recovery over weeks
- periods of gd health in betweeen
secondary progressive
- gradually worsening sx + fewer remissions
- evolves from relapsing + remitting
primary progressive
- gradually worsening disability WITHOUT relapses or remissions
141
diagnosis of MS
TWO+ attacks affecting DIFFERENT PARTS of CNS; that is 2 CNS lesions disseminated in time + space
142
ix in MS
MRI brain + spinal cord
* 95% have periventricular lesions
* Over 90% show discrete white matter abnormalities
* Multiple scattered plaques are usually seen
- with contrast - active lesions will take up contrast + appear white in colour
= Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum
LP
* oligoclonal IgG bands in CSF
143
tx for acute relapse MS
High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten duration of relapse
144
First line drug for reducing the risk of relapse in MS
natalizumab IV - a recombinant monoclonal antibody
145
drug for fatigue in MS
amantadine
(once other probs - anaemia, thyroid, depression - have been excluded)
146
tx for bladder incompetence in MS
get US first to assess bladder emptying
if significant residual volume → intermittent self-catheterisation
if no significant residual volume → anticholinergics may improve urinary frequency
147
tx for oscillopsia in MS
gabapentin
148
inheritance of huntington's disease
AD
149
what is huntingtons disease (+what is its genetics)
genetic condition that causes progressive neurological dysfunction.
trinucleotide repeat (CAG) disorder involving a genetic mutation in the HTT gene on chromosome 4, which codes for the huntingtin (HTT) protein.
150
examples of trinucleotide repeat disorders
Huntington's disease
Fragile X syndrome
Spinocerebellar ataxia
Myotonic dystrophy
Friedrich ataxia
151
what is genetic anticipation
a feature of trinucleotide repeat disorders where successive generations have more repeats in the gene, resulting in:
Earlier age of onset
Increased severity of disease
152
Huntington's disease presentation
insidious, progressive worsening of symptoms.
It typically begins with cognitive, psychiatric or mood problems, followed by the development of movement disorders:
Chorea (involuntary, random, irregular and abnormal body movements)
Dystonia (abnormal muscle tone, leading to abnormal postures)
Rigidity (increased resistance to the passive movement of a joint)
Eye movement disorders
Dysarthria (speech difficulties)
Dysphagia (swallowing difficulties)
153
dx huntington's disease
genetic testing
154
med for chorea sx in huntington's
Tetrabenazine
155
prognosis huntington's
Life expectance is around 10-20 years after the onset of symptoms. As the disease progresses, patients become more frail and susceptible to illness (e.g., infections, weight loss, falls and pressure ulcers). Death is often due to aspiration pneumonia. Suicide is also a common cause of death.
156
what is motor neurone disease
progressive degeneration of both the upper + lower motor neurones
sensory neurones are spared
157
classic features of MND
asymmetric limb weakness is the most common presentation of ALS (usually upper limbs first)
the mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior is common
fasciculations
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but 'never' sensory signs
rarely presents before 40 yrs
does NOT affect
- external occular muscles
- no cerebellar signs
- abdo reflexes preserved
158
types of MND
Amyotrophic lateral sclerosis (ALS) - most common - LMN + UMN
Progressive bulbar palsy - tongue , worst prog
Progressive muscular atrophy - LMN
Primary lateral sclerosis - UMN
159
pattern of signs in ALS
LMN signs in the arms and UMN signs in the legs
Asymmetric limb weakness
160
MND dx
clinical
exclude
done by specialist
161
what drug can slow progression of MND + how does it work
Riluzole
- prevents stim of glutamate receptors
- used mainly in ALS
- prolongs life by about 3 months
162
other tx MND
Respiratory care
non-invasive ventilation (usually BIPAP) is used at night
Nutrition
percutaneous gastrostomy tube (PEG) is the preferred way to support nutrition and has been associated with prolonged survival
163
causes of peripheral neuropathy
ABCDE:
A – Alcohol
B – B12 deficiency
C – Cancer (e.g., myeloma) and Chronic kidney disease
D – Diabetes and Drugs (e.g., isoniazid, amiodarone, leflunomide and cisplatin)
E – Every vasculitis
164
charcot-marie-tooth inheritance
AD
165
what is neurofibromatosis
genetic condition that causes neuromas to develop throughout the nervous system
benign tumours but cause neuro + structural probs
166
Neurofibromatosis Type 1 Gene
found on chromosome 17
codes for neurofibromin (a tumour suppressor protein)
Mutations in this gene are inherited in an autosomal dominant pattern.
167
diagnostic criteria for neurofibromatosis type 1
“CRABBING” mnemonic:
C – Café-au-lait spots (more than 15mm diameter is significant in adults)
R – Relative with NF1
A – Axillary or inguinal freckling
BB – Bony dysplasia, such as Bowing of a long bone or sphenoid wing dysplasia
I – Iris hamartomas (Lisch nodules), which are yellow-brown spots on the iris
N – Neurofibromas
G – Glioma of the optic pathway
168
what is a neurofibroma + when are they significant
Skin-coloured, raised nodules or papules with a smooth, regular surface.
Two or more are significant.
A plexiform neurofibroma is a larger, irregular, complex neurofibroma containing multiple cell types.
A single plexiform neurofibroma is significant.
169
complications of neurofibromatosis
Migraines
Epilepsy
Renal artery stenosis, causing hypertension
Learning disability
Behavioural problems (e.g., ADHD)
Scoliosis of the spine
Vision loss (secondary to optic nerve gliomas)
Malignant peripheral nerve sheath tumours
Gastrointestinal stromal tumour (a type of sarcoma)
Brain tumours
Spinal cord tumours with associated neurology (e.g., paraplegia)
Increased risk of cancer (e.g., breast cancer and leukaemia)
170
Neurofibromatosis Type 2 genetics
The gene is found on chromosome 22
It codes for merlin (a tumour suppressor protein important in Schwann cells).
Mutations in this gene lead to schwannomas
Inheritance is also autosomal dominant.
Assoc w acoustic neuromas
171
what is tuberous sclerosis
autosomal dominant genetic condition
leads to development of harmartomas (benign tissue growths)
commonly affect:
Skin
Brain
Lungs
Heart
Kidneys
Eyes
172
Tuberous sclerosis is caused by mutations in either:
TSC1 gene on chromosome 9, which codes for hamartin
TSC2 gene on chromosome 16, which codes for tuberin
Hamartin and tuberin interact with each other to control the size and growth of cells. Abnormalities in one of these proteins lead to abnormal cell size and growth.
173
Tuberous sclerosis skin features
Ash leaf spots (depigmented areas of skin shaped like an ash leaf)
Shagreen patches (thickened, dimpled, pigmented patches of skin)
Angiofibromas (small skin-coloured or pigmented papules that occur over the nose and cheeks)
Ungual fibromas (circular painless lumps that slowly grow from the nail bed and displace the nail)
Cafe-au-lait spots (light brown “coffee and milk” coloured flat pigmented lesions on the skin)
Poliosis (an isolated patch of white hair on the head, eyebrows, eyelashes or beard)
174
Tuberous sclerosis neuro features
Epilepsy
Learning disability
Brain tumours
175
systemic features tuberous sclerosis
Rhabdomyomas in the heart
Angiomyolipoma in the kidneys
Lymphangioleiomyomatosis in the lungs
Subependymal giant cell astrocytoma in the brain
Retinal hamartomas in the eyes
176
headache red flags
Fever, photophobia or neck stiffness (meningitis, encephalitis or brain abscess)
New neurological symptoms (haemorrhage or tumours)
Visual disturbance (giant cell arteritis, glaucoma or tumours)
Sudden-onset occipital headache (subarachnoid haemorrhage)
Worse on coughing or straining (raised intracranial pressure)
Postural, worse on standing, lying or bending over (raised intracranial pressure)
Vomiting (raised intracranial pressure or carbon monoxide poisoning)
History of trauma (intracranial haemorrhage)
History of cancer (brain metastasis)
Pregnancy (pre-eclampsia)
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why to do fundoscopy in headaches
to check for papilloedema - suggests raised ICP
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first-line for chronic or frequent tension headaches
Amitriptyline
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Tension headaches may be associated with:
Stress
Depression
Alcohol
Skipping meals
Dehydration
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mx tension headaches
Reassurance
Simple analgesia (e.g., ibuprofen or paracetamol)
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first line abx for sinusitis that has lasted > 10 days
phenoxymethylpenicillin
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why do you get hormonal headaches + how do they present
low oestrogen
unilateral, pulsatile headache associated with nausea
They may occur:
- Two days before and the first three days of the menstrual period
- In the perimenopausal period
- Early pregnancy (headaches in the second half of pregnancy should prompt investigations for pre-eclampsia)
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tx hormonal headaches
Triptans and NSAIDs (e.g., mefenamic acid)
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what is trigeminal neuralgia
intense facial pain in the distribution of the trigeminal nerve, which has three branches:
Ophthalmic (V1)
Maxillary (V2)
Mandibular (V3)
Can affect any combo of branches
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presentation trigeminal neuralgia
>90% are unilateral
pain comes on suddenly + can last seconds to hrs
an electricity-like, shooting, stabbing or burning pain
may be triggered by touch, taking, eating, shaving or cold
Attacks may worsen over time.
more common in px w MS
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first line tx trigeminal neuralgia
carbamazepine
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4 types of migraine
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
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stages of migraine
Premonitory or prodromal stage (can begin several days before the headache)
Aura (lasting up to 60 minutes)
Headache stage (lasts 4 to 72 hours)
Resolution stage (the headache may fade away or be relieved abruptly by vomiting or sleeping)
Postdromal or recovery phase
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sx migraine
Usually unilateral
Moderate-severe intensity
Pounding or throbbing in nature
Photophobia (discomfort with lights)
Phonophobia (discomfort with loud noises)
Osmophobia (discomfort with strong smells)
Aura (visual changes)
Nausea and vomiting
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what is aura + its sx
Can affect vision, sensation or language. Visual symptoms are the most common:
Sparks in the vision
Blurred vision
Lines across the vision
Loss of visual fields (e.g., scotoma)
Sensation changes may include tingling or numbness.
Language symptoms include dysphasia (difficulty speaking).
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what is a hemiplegic migraine
rare subtype of migraine with aura that is hemiplegia / ataxia / impaired consciousness
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inheritance of familial hemiplegic migraine
AD
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migraine triggers
Stress
Bright lights
Strong smells
Certain foods (e.g., chocolate, cheese and caffeine)
Dehydration
Menstruation
Disrupted sleep
Trauma
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medical mx for acute migraine attack
NSAIDs (e.g., ibuprofen or naproxen)
Paracetamol
Triptans (e.g., sumatriptan) - taken as soon as it starts to halt the attack, do not take again in same migraine if does not work
Antiemetics if vomiting occurs (e.g., metoclopramide or prochlorperazine)
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CI for triptans
risks associated with vasoconstriction, for example, hypertension, coronary artery disease or previous stroke, TIA or myocardial infarction
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migraine prophylaxis
headache diary to identify triggers
Propranolol (CI asthma)
Topiramate (teratogenic and very effective contraception is needed)
Amitriptyline
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tx for menstrual migraines
Prophylactic triptans (e.g., frovatriptan or zolmitriptan)
Sx tend to occur two days before until three days after the start of menstruation
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sx cluster headaches
Severely painful headache
- unilateral
- centred around the eye
Red, swollen and watering eye
Pupil constriction (miosis)
Eyelid drooping (ptosis)
Nasal discharge
Facial sweating
Comes in clusters of attacks lasting 15mins-3hrs
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who typically gets cluster headaches
A typical patient is a 30-50 year old male smoker. They may have triggers, such as alcohol, strong smells or exercise.
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acute mx cluster headaches
Triptans (e.g., subcutaneous or intranasal sumatriptan)
High-flow 100% oxygen (may be kept at home)
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cluster headache prophylaxis
Verapamil
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RFs Alzheimer's
age
FHx - 5% of cases are inherited as an AD trait
apoprotein E allele E4 - encodes a cholesterol transport protein
Caucasian ethnicity
Down's syndrome
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medical mx mild-moderate Alzheimer's
Acetylcholinesterase inhibitors
- donepezil
- galantamine
- rivastigmine
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medical mx severe Alzheimer's / when 1st line not worked/CI
memantine - an NMDA receptor antagonist
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blood screen when ix dementia
FBC
U&E
LFTs
calcium
glucose
ESR/CRP
TFTs
vitamin B12 and folate levels
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characteristic feature Lewy body dementia
alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas
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presentation of Lewy body dementia
progressive cognitive impairment
- BEFORE parkinsonism, but usually both features occur within a year of each other (in Parkinson's disease, motor sx typically present at least one year before cognitive symptoms)
- cognition may be FLUCTUATING, in contrast to other forms of dementia
- early impairments in attention and executive function rather than just memory loss
parkinsonism
visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen)
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mx Lewy body dementia
same as Alzheimer's
avoid neuroleptics
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what is vascular dementia
a group of syndromes of cognitive impairment caused by different mechanisms causing ischaemia or haemorrhage secondary to cerebrovascular disease
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subtypes of vascular dementia
Stroke-related VD – multi-infarct or single-infarct dementia
Subcortical VD – caused by small vessel disease
Mixed dementia – the presence of both VD and Alzheimer’s disease
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RFs vascular dementia
History of stroke or TIA
AF
HTN
Diabetes
Hyperlipidaemia
Smoking
Obesity
Coronary heart disease
A family history of stroke or cardiovascular
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presentation vascular dementia
Several months or several years of a history of a sudden or stepwise deterioration of cognitive function.
Sx and the speed of progression vary:
Focal neurological abnormalities e.g. visual disturbance, sensory or motor symptoms
The difficulty with attention and concentration
Seizures
Memory disturbance
Gait disturbance
Speech disturbance
Emotional disturbance
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dx vascular dementia
Presence of cognitive decline that interferes with activities of daily living, not due to secondary effects of the cerebrovascular event
+
Cerebrovascular disease
+
A relationship between the above two disorders inferred by:
- the onset of dementia within three months following a recognised stroke
- an abrupt deterioration in cognitive functions
- fluctuating, stepwise progression of cognitive deficits
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types of frontotemporal lobar dementias
Frontotemporal dementia (Pick's disease)
Progressive non fluent aphasia (chronic progressive aphasia, CPA)
Semantic dementia
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Common features of frontotemporal lobar dementias
Onset before 65
Insidious onset
Relatively preserved memory and visuospatial skills
Personality change and social conduct problems
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features of pick's disease
personality change
impaired social conduct
hyperorality (putting things in mouth)
disinhibition
increased appetite
perseveration behaviours (get stuck on idea)
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microscopic changes in pick's disease
Pick bodies - spherical aggregations of tau protein (silver-staining)
Gliosis
Neurofibrillary tangles
Senile plaques
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Progressive non fluent aphasia (chronic progressive aphasia, CPA)
non-fluent speech
short utterances that are agrammatic
comprehension is relatively preserved.
219
Semantic dementia
fluent progressive aphasia
speech is fluent but empty and conveys little meaning
memory is better for recent rather than remote events (unlike alzheimer's)
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what spinal tract does neurosyphilis affect and what does that cause
dorsal columns
-> Loss of proprioception and vibration sensation
(posterior cord syndrome)
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presentation of anterior cord syndrome
acute onset
loss of pain + temp sensation
loss of movement
bilaterally
Autonomic dysfunction: abnormal blood pressure
Bladder dysfunction: Urinary incontinence
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what is anterior cord syndrome
A clinical syndrome due to damage to the anterior two-thirds of the spinal cord.
Damage to:
1. Lateral corticospinal tracts
2. Lateral spinothalamic tracts
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causes of anterior cord syndrome
ischaemia of the anterior spinal artery:
Thromboembolism
Trauma
Hypotension
Aortic disease
Other pathology:
Disc herniation
Tumour
Trauma
Epidural collection
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features of Intracranial venous sinus thrombosis
headache (may be sudden or gradual onset)
nausea & vomiting
reduced consciousness
225
GS ix for Intracranial venous sinus thrombosis
MRI VENOGRAPHY
CT venography is an alternative
non-contrast CT head is normal in around 70% of patients
D-dimer levels may be elevated
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Mx Intracranial venous sinus thrombosis
anticoagulation
- typically with low molecular weight heparin acutely
- warfarin is still generally used for longer term anticoagulation
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tx degenerative cervical myelopathy
decompressive surgery
228
sign of potentially salvageable tissue on CT
limited infarct core
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what does the facial nerve supply
'face, ear, taste, tear'
face: muscles of facial expression
ear: nerve to stapedius
taste: supplies anterior two-thirds of tongue
tear: parasympathetic fibres to lacrimal glands, also salivary glands
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what is erb's palsy a result of
injury to the C5/C6 nerves in the brachial plexus during birth
associated with shoulder dystocia, traumatic or instrumental delivery and large birth weight
231
what does erb's palsy lead to
weakness of shoulder abduction and external rotation, arm flexion and finger extension. This leads to the affected arm having a “waiters tip” appearance:
Internally rotated shoulder
Extended elbow
Flexed wrist facing backwards (pronated)
Lack of movement in the affected arm
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Pontine haemorrhage
reduced GCS, paralysis (tetraparesis ) and bilateral pin point pupils
may have facial droop
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Idiopathic intracranial hypertension RFs
obesity
female sex
pregnancy
drugs: COCP, steroids, tetracyclines, retinoids (isotretinoin, tretinoin) / vitamin A, lithium
234
Idiopathic intracranial hypertension features
headache
blurred vision
papilloedema (usually present)
enlarged blind spot
sixth nerve palsy may be present
235
Idiopathic intracranial hypertension tx
weight loss
carbonic anhydrase inhibitors e.g. acetazolamide
topiramate is also used, and has the added benefit of causing weight loss in most patients
repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management
surgery: optic nerve sheath decompression and fenestration
lumboperitoneal or ventriculoperitoneal shunt to reduce ICP
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which are of the brain does herpes simplex encephalitis affect
temporal lobes
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which antiemetic to prescribe in parkinsons
Domperidone
238
what to do if not tolerating clopidogrel after stroke
aspirin and modified release dipyramidole
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where is the lesion for
broca's (expressive) aphasia
inferior frontal gyrus
240
where is the lesion for
wernicke's (receptive) aphasia
superior temporal gyrus
241
prev of vasospasm after SAH
nimodipine
