Neurology Flashcards
(258 cards)
1
Q
two types of stroke
A
Ischaemia or infarction of the brain tissue secondary to a disrupted blood supply (ischaemic stroke)
Intracranial haemorrhage, with bleeding in or around the brain (haemorrhagic stroke)
2
Q
blood supply disrupted by 4 possible things
A
thrombus/embolus
atherosclerosis
shock
vasculitis
3
Q
TIA definition
why different from stroke
A
temporary neurological dysfunction (lasting less than 24 hours) caused by ischaemia but without infarction. Symptoms have a rapid onset and often resolve before the patient is seen. TIAs may precede a stroke.
4
Q
crescendo TIA’s define
A
2 or more TIA’s within a week…high risk of stroke
5
Q
clinical fx of stroke
A
sudden onset
asymmetrical
Limb weakness
Facial weakness
Dysphasia (speech disturbance)
Visual field defects
Sensory loss
Ataxia and vertigo (posterior circulation infarction)
6
Q
risk factors for stroke
A
Previous stroke or TIA
Atrial fibrillation
Carotid artery stenosis
Hypertension
Diabetes
Raised cholesterol
Family history
Smoking
Obesity
Vasculitis
Thrombophilia
Combined contraceptive pill
7
Q
increased risk of stroke with COCP
A
migraines with aura
smoker >34 yrs
hx of stroke/TIA
8
Q
how assess possible stroke in community
A
FAST
9
Q
what tool in secondary care risk of stroke
A
based on clinical fx and duration
ROSIER (recognition of stroke in the emergency room)
10
Q
mx TIA
A
Aspirin 300mg daily (started immediately)
Referral for specialist assessment within 24 hours (within 7 days if more than 7 days since the episode)
Diffusion-weighted MRI scan
11
Q
initial management of stroke
A
Exclude hypoglycaemia
Immediate CT brain to exclude haemorrhage
Aspirin 300mg daily for two weeks (started after haemorrhage is excluded with a CT)
Admission to a specialist stroke centre
12
Q
what type of stoke must be excluded first
A
haemorrhagic
13
Q
once haemorrhage excluded what next
A
thrombolysis with alteplase
thrombectomy is considered if confirmed blockage in proximal ant circ or proximal posterior circ…can be done alongside thrombolysis
14
Q
blood pressure tx
haemorrhagic v ischaemic stroke
A
haemorrhagic - aggressively tx
ischaemic - lowering can worsen ischaemia, only tx in hypertensive emergency or to reduce risks in thrombolysis
15
Q
possible underlying cause/risk fx of stroke
A
afib
cartoid artery stenosis
16
Q
how assessed for underlying causes of stroke
A
carotid imaging
ECG
17
Q
secondary prevention of stroke
A
clopidogrel
atorvastatin
BP and DM control
modifiable risk fx
18
Q
4 types of IC haemorrhage
A
Extradural haemorrhage (bleeding between the skull and dura mater)
Subdural haemorrhage (bleeding between the dura mater and arachnoid mater)
Intracerebral haemorrhage (bleeding into brain tissue)
Subarachnoid haemorrhage (bleeding in the subarachnoid space)
19
Q
risk factors for IC haemorrhage
A
Head injuries
Hypertension
Aneurysms
Ischaemic strokes (progressing to bleeding)
Brain tumours
Thrombocytopenia (low platelets)
Bleeding disorders (e.g., haemophilia)
Anticoagulant
20
Q
clinical fx of IC haemorrhage
A
sudden onset headache
seizures
vomit
reduced GCS
focal neurological sx (weakness)
21
Q
extradural haemorrhage
which artery
associations
on scan
sx
A
-middle meningeal in temporparietal region
- fracture of temporal bone
- CT - biconvex shape, do not cross cranial sutures
- period of improved neuro sx and consciousness follow by rapid decline over hrs
22
Q
subdural haemorrhage
- vein
- imaging
- clinical fx
A
- rupture of bridging vein
- ct - crescent shape, cross sutures
- elderly and alcoholics - more atrophy make vessels more prone to rupture
23
Q
intracerebral haemorrhage
- clinical fx
- cause
A
- sudden onset neuro sx - limb, facial weakness, dysphagia or vision loss
- spontaenous, secondary to ischaemic stroke, tumour or aneurysm rupture
24
Q
location of intracerebral haemorrhage
A
Lobar intracerebral haemorrhage
Deep intracerebral haemorrhage
Intraventricular haemorrhage
Basal ganglia haemorrhage
Cerebellar haemorrhage
25
subarachnoid
- cause
- hx
ruptures cerebral aneurysm
sudden onset occipital headache during strenuous activity, thunderclap
26
IC haemorrhage inv
CT head
blood - FBC and coag sceen
27
IC haemorrhage mx
Admission to a specialist stroke centre
Discuss with a specialist neurosurgical centre to consider surgical treatment
Consider intubation, ventilation and intensive care if they have reduced consciousness
Correct any clotting abnormality (e.g., platelet transfusions or vitamin K for warfarin)
Correct severe hypertension but avoid hypotension
28
surgical options indicated fo
extradural or subdural
29
what are surgical options
craniotomy
burr holes
30
more common in SAH
45-70
woman
black
31
risk factors SAH
HTN
smoking
excess alcohol
32
SAH associations
Family history
Cocaine use
Sickle cell anaemia
Connective tissue disorders (e.g., Marfan syndrome or Ehlers-Danlos syndrome)
Neurofibromatosis
Autosomal dominant polycystic kidney disease
33
clinical fx SAH
sudden onset occipital headache - thunderclap
Neck stiffness
Photophobia
Vomiting
Neurological symptoms (e.g., visual changes, dysphasia, focal weakness, seizures and reduced consciousness)
34
SAH investigations
CT head (if normal does not exclude SAH)
if normal - LP 12 hrs after sx to allow bilirubin to accumulate...raised RBC, xanthochromia (caused by billirubin)
CT angiography - locate source
35
CAH mx
Intubation and ventilation
surgery for aneurysms ....endovascular coiling or neurosurgical clipping
36
complication of SAH and how tx
Vasospasm...resulting in brain ischaemia...tx with nimodipine
hydrocephalus - LP, external ventricular drain, VP shunt
seizures - anti drugs
37
MS definition
autoimmune condition involving demyelination in the central nervous system. The immune system attacks the myelin sheath of the myelinated neurones.
38
MS age and sex
<50
women
39
oligodendrocytes
CNS
40
scHwann cells
PNS
41
MS affect what
CNS
42
patho MS attack
there are often other demyelinating lesions throughout the central nervous system, most of which are not causing symptoms
43
early disease v later patho MS
re-myelination can occur, and the symptoms can resolve. In the later stages of the disease, re-myelination is incomplete, and the symptoms gradually become more permanent.
44
characteristic fx of MS
sx vary as lesions vary in location
45
causes of MS
Multiple genes
Epstein–Barr virus (EBV)
Low vitamin D
Smoking
Obesity
46
onset MS
progress over more than 24 hrs
sx last days to week at first presentation and then improve
47
most common presentation of MS
optic neuritis - demyelination of optic nerve
unilateral reduced vision over hrs to days
Central scotoma (an enlarged central blind spot)
Pain with eye movement
Impaired colour vision
Relative afferent pupillary defect (pupil constricts more in contralateral eye than in affected eye)
direct reflex redcued response and normal consensual
48
other causes of optic neuritis
Sarcoidosis
Systemic lupus erythematosus
Syphilis
Measles or mumps
Neuromyelitis optica
Lyme disease
49
optic neuritis mx
urgent opthal input
high dose steroids
50
possible eye movement abnormalities MS
diplopia
nystagmus
oscillopsia (environment moving)
internuclear opthalmoplegia
conjugate lateral gaze disorder
51
internuclear opthalmoplegia
caused by a lesion in the medial longitudinal fasciculus. The nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei (“internuclear”) that control eye movements (the 3rd, 4th and 6th cranial nerve nuclei). These fibres are responsible for coordinating the eye movements to ensure the eyes move together. It causes impaired adduction on the same side as the lesion (the ipsilateral eye) and nystagmus in the contralateral abducting eye.
52
conjugate lateral gaze disorder
lesion in abducens
Conjugate means connected. Lateral gaze is where both eyes move to look laterally to the left or right. When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion involving the left eye, when looking to the left, the right eye will adduct (move towards the nose), and the left eye will remain in the middle
53
MS focal neurological signs
Incontinence
Horner syndrome
Facial nerve palsy
Limb paralysis
Trigeminal neuralgia
Numbness
Paraesthesia (pins and needles)
Lhermitte’s sign
54
Lhermitte's sign
an electric shock sensation that travels down the spine and into the limbs when flexing the neck. It indicates disease in the cervical spinal cord in the dorsal column. It is caused by stretching the demyelinated dorsal column.
55
ataxia MS
types
sensory or cerebellar
56
sensory ataxia
loss of proprioception
positive romberg's test
caused by lesion in dorsal column
57
cerebellar ataxia cause
cerebellar lesion
58
disease patterns of MS
clinically isolated syndrome - the first episode of demyelination and neurological signs and symptoms. Patients with clinically isolated syndrome may never have another episode or may go on to develop MS. Lesions on an MRI scan suggest they are more likely to progress to MS.
relapsing-remitting - most common, episodes followed by recovery. different areas. further classified (active, not active, worsening, not worsening)
secondary progressive - was relapsing remitting but not progressive
primary progressive - worsening disease from point of disease without remissions
59
diagnosis MS
neurologist
clinical fx
MRI - lesions
LP - oligoclonal bands in CSF
60
mx of MS
MDT
disease modifying therapies
relapses with steroids
exercise
fatigue tx xwith amantadine, modafinil
neuropathic pain tx
depression tx
urge incontinence tx
spasitisity tx with baclofen
oscillopsia tx with gabapentin
61
MND definition
encompasses variety of specific diseases affecting motor nerves
no sensory sx
62
types of MND
Amyotrophic lateral sclerosis
progressive bulbar palsy
progressive muscular atrophy
primary lateral sclerosis
63
MND patho
Motor neurone disease involves a progressive degeneration of both the upper and lower motor neurones. The sensory neurones are spared.
64
increased risk MND
many genes - family hx
smoking
exposure to heavy metals and pesticides
generally 60 yr old man
65
clinical fx MND
There is an insidious, progressive weakness of the muscles throughout the body, affecting the limbs, trunk, face and speech
increased fatigue
clumsiness
slurred speech (dysarthria)
66
signs of LMND
Muscle wasting
Reduced tone
Fasciculations (twitches in the muscles)
Reduced reflexes
67
signs of UMND
Increased tone or spasticity
Brisk reflexes
Upgoing plantar reflex
68
mx of MND
riluzole can slow progression
NIV
mdt
Baclofen for spasticity
antimuscarinic for saliva
benzo to help anxiety
advanced directive and end of life care
69
cause of death MND
resp failure or pneumonia
70
parkinsons classical triad
Resting tremor (a tremor that is worse at rest)
Rigidity (resisting passive movement)
Bradykinesia (slowness of movement)
71
parkinsons patho
progressive reduction in dopamine in basal ganglia...disorders of movement
72
clinical fx parkinsons
tremor worse on one side, 4-6 Hz frequency, pill rolling, resting, worse when distracted, performing task with other hand exaggerates tremor
rigidity - when passive flex and extend arms...results in jerks called cogwheel rigidity
bradykinesia - micrographia, shuffling gait, festinating gait, difficulty initiating movement and turning around, hypomimia
depression
insomnia
anosmia
postural instability
cognitive impairment
73
parkinsons v essential tremor
Parkinson’s Tremor
Benign Essential Tremor
Asymmetrical
Symmetrical
4-6 hertz
6-12 hertz
Worse at rest
Improves at rest
Improves with intentional movement
Worse with intentional movement
Other Parkinson’s features
No other Parkinson’s features
No change with alcohol
Improves with alcohol
74
parkinson's-plus syndromes
multiple system atrophy - neurones of various systems in brain degenerate...including basal ganglia = parkinsons
dementia with lewy bodies
progressive supranuclear palsy
corticobasal degeneration
75
parkinson's diagnosis
diagnostic criteria
specialist
76
parkinson's mx
levodopa (combined with peripheral decarboxylase inhibitors)
COMT inhibitors
dopamine agonist
MAO B inhibitors
or combination drugs such as co-careldopa
77
main s/e of levodopa and how tx
dyskinesia - dystonia, chorea, athetosis
tx with amantadine
78
dopamine agonists
examples
s/e
bromocriptine
pergolide
cabergoline
pulmonary fibrosis
79
benign essential tremor define
a relatively common condition associated with older age. It is characterised by a fine tremor affecting all the voluntary muscles. It is most notable in the hands but can affect other areas, for example, causing a head tremor, jaw tremor and vocal tremor.
80
clinical fx of benign essential tremor
Fine tremor (6-12 Hz)
Symmetrical
More prominent with voluntary movement
Worse when tired, stressed or after caffeine
Improved by alcohol
Absent during sleep
81
ddx of tremor
Parkinson’s disease
Multiple sclerosis
Huntington’s chorea
Hyperthyroidism
Fever
Dopamine antagonists (e.g., antipsychotics)
82
mx of benign tremor
not treated, harmless
sx management - propranolol, primidone
83
more common types of seizures in adult
Generalised tonic-clonic seizures
Partial seizures (or focal seizures)
Myoclonic seizures
Tonic seizures
Atonic seizures
84
more common types of seizures in children
Absence seizures
Infantile spasms
Febrile convulsions
85
generalised tonic clonic seizures fx
tonic (tense) and clonic (jerk)
complete loss of consciousness
AKA grand mal
before might experience aura
tongue biting, incontinence, groaning and irregular breathing
post-ictal period
86
partial/focal seizures fx
isolated brain area -> temporal lobe usually
affect hearing, speech, memory and emotions. Patients remain awake during partial seizures. They remain aware during simple partial seizures but lose awareness during complex partial seizures
Déjà vu
Strange smells, tastes, sight or sound sensations
Unusual emotions
Abnormal behaviours
87
myoclonic seizures fx
sudden, brief muscle contractions
remain awake
can occur as part of juvenile myoclonic epilepsy
88
tonic seizures fx
a sudden onset of increased muscle tone, where the entire body stiffens. This results in a fall if the patient is standing, usually backwards. They last only a few seconds, or at most a few minutes.
89
atonic seizures fx
sudden loss of muscle tone...fall
only brief and pt aware
often begin in childhood
associated with lennox-gastaut syndrome
90
absence seizures fx
usually children
blank, stare into space, abruptly returns to normal, unaware, last 10-20 secs
91
infantile spasms fx
AKA west syndrome
rare
6mths of age
cluster of full body spasms
hypsarrhythmia on EEG
developmental regression and poor prognosis
tx with ACTH and vigabatrin
92
febrile convulsions fx
tonic clonic
during high feve
6 mths to 5 yrs
not usually any damage
increase risk of developing epilepsy
93
ddx seizures
Vasovagal syncope (fainting)
Pseudoseizures (non-epileptic attacks)
Cardiac syncope (e.g., arrhythmias or structural heart disease)
Hypoglycaemia
Hemiplegic migraine
Transient ischaemic attack
94
investigations seizure
EEG
MRI brain
ECG, serum electrolytes, blood glucose, blood culture/urine/LP
95
seizures mx
inform DVLA
taking showers than baths
caution with heights, swimming and dangeous equiptment
96
generalised tonic clonic medical mx
men/no children - sodium valp
women who can have children - lamotrigine or levetiracetam
97
partial medical mx
lamotrigine or levetiracetam
98
myoclonic medical mx
men - sod valporate
women - levetiracetam
99
tonic and atonic medical mx
men - sod valporate
women - lamotrigene
100
absence medical mx
ethosuximide
101
sodium valp
- mechanism
- s/e
- risk
increasing the activity of gamma-aminobutyric acid (GABA), which has a calming effect on the brain.
Teratogenic (harmful in pregnancy)
Liver damage and hepatitis
Hair loss
Tremor
Reduce fertility
neural tube defects/developmental delay...Valproate Pregnancy Prevention Programme
102
status epilepticus definition
A seizure lasting more than 5 minutes
Multiple seizures without regaining consciousness in the interim
103
initial mx of status epilepticus
A-E
secure airway
high flow o2
check blood glucose
IV access
benzo repeat after 5-10 mins, then try IV levetiracetam/phenytoin/sod valporate, then general anaesthesia
104
benzo doses and route
Buccal midazolam (10mg)
Rectal diazepam (10mg)
Intravenous lorazepam (4mg)
105
define pain
“An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue damage”
106
acute v chronic pain
chronic = >3 or more months
107
basic physiology of pain
sensory - from pain receptor
affective - unpleasant emotional reaction to pain
pain is subjective
pain threshold - the point at which sensory input is reported as painful
allodynia - when pain is experienced with sensory inputs that do not normally cause pain (e.g., light touch).
Pain tolerance is different to pain threshold. It is more difficult to define and generally refers to a person’s response to pain. One person may experience pain but think little of it and carry on with their activities as usual.
108
how feel pain?
Nociceptors at ends of nerves detect damage or potential damage to tissues -> nerve signals transmitted along afferent nerves->spinal cord
Afferent sensory nerves that transmit pain signals are part of the peripheral nervous system and are called primary afferent nociceptors
Nerve fibres transmit pain to CNS , up CNS (mainly spinothalamic tract and spinoreticular tract) to brain where it is interpreted as pain, mainly in thalamus and cortex
109
which fibres transmit pain
- speed and type of pain
C fibres (unmyelinated and small diameter) – transmit signals slowly and produce dull and diffuse pain sensations
A-delta fibres (myelinated and larger diameter) – transmit signals fast and produce sharp and localised pain sensations
110
types of pain sensory inputs
Mechanical (e.g., pressure)
Heat
Chemical (e.g., prostaglandins)
111
why feel referred pain
Nerves may share the innervation of multiple parts of the body (e.g., the heart and left arm)
Pain in one area amplifies the sensitivity in the spinal cord to signals coming from other areas
Activation of the sympathetic nervous system in response to pain results in pain in other areas
112
neuropathic pain cause
caused by abnormal functioning or damage of the sensory nerves, resulting in pain signals being transmitted to the brain.
113
ways to rate pain
Visual analogue scale
numerical rating scale
graphical rating scale
114
analgesic ladder
Step 1: Non-opioid medications such as paracetamol and NSAIDs
Step 2: Weak opioids such as codeine and tramadol
Step 3: Strong opioids such as morphine, oxycodone, fentanyl and buprenorphine
115
opioids in palliative care
Background opioids (e.g., 12-hourly modified-release oral morphine)
Rescue doses for breakthrough pain (e.g., immediate-release oral morphine solution)
The rescue dose is usually 1/6 of the background 24-hour dose
116
oral morphine to IV/SC mophine
/2
117
oral morphine to oral codeine
x10
118
chronic pain two types
Chronic primary pain – where no underlying condition can adequately explain the pain
Chronic secondary pain – where an underlying condition can explain the pain (e.g., arthritis)
119
options for mx of chronic pain
Supervised group exercise programs
Acceptance and commitment therapy (ACT)
Cognitive behavioural therapy (CBT)
Acupuncture
Antidepressants (e.g., amitriptyline, duloxetine or an SSRI)
avoid all form sof analegesia
120
common causes of neuropathic pain
Post-herpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk
Nerve damage from surgery
Multiple sclerosis
Diabetic neuralgia (typically affecting the feet)
Trigeminal neuralgia
Complex regional pain syndrome
121
four first line tx for neuropathic pain
Amitriptyline – a tricyclic antidepressant
Duloxetine – an SNRI antidepressant
Gabapentin – an anticonvulsant
Pregabalin – an anticonvulsant
122
1st line for trigeminal neuralgia
carbamezapine
123
CRPS definition and clinical fx
areas of abnormal nerve functioning, causing neuropathic pain, abnormal sensations and skin changes. It is often triggered by an injury and isolated to one limb.
The area can become hypersensitive, with pain associated with normal sensations (allodynia). There may be intermittent swelling, colour changes, temperature changes, skin flushing and abnormal sweating.
124
facial nerve palsy definition
isolated dysfunction of facial nerve...unilateral facial weakness
125
facial nerve pathway
facial nerve exits the brainstem at the cerebellopontine angle. On its journey to the face, it passes through the temporal bone and parotid gland.
It then divides into five branches:
Temporal
Zygomatic
Buccal
Marginal mandibular
Cervical
126
functions of facial nerve
- motor
-sensory
- parasympathetic
Motor function for:
Facial expression
Stapedius in the inner ear
Posterior digastric, stylohyoid and platysma muscles
Sensory function for taste from the anterior 2/3 of the tongue.
Parasympathetic supply to the:
Submandibular and sublingual salivary glands
Lacrimal gland (stimulating tear production)
127
2 types of facial nerve palsy
UMN and LMN facial nerve palsy
128
difference between UMN and LMN facial nerve palsy
UMN - immediate mx as possible stroke, foreheard spared (innervation from both sides of brain)
LMN - less urgently mx, forehead not spared (innervation from one side of brain)
129
upper motor neurone lesions types
unilateral
bilateral
130
unilateral UMN lesions causes
stroke
tumour
131
b/l UMN lesions causes
pseudobulbar palsies
MND
132
bell's palsy presentation
unilateral LMN facial nerve palsy
133
bells palsy prognosis
fully recover mostly over several week...can take 12 mths
may have residual weakness
134
bell's palsy tx
prednisolone within 72 hrs of sx onset for 10 days
lubricating eye drops, taped at nite (risk of exposure keratopathy)
135
ramsay hunt syndrome cause
varicella zoster virus
136
ramsay hunt syndrome clinical fx
unilateral LMN facial nerve palsy
painful tender vesicular rash in ear canal, pinna and can extend to tongue and hard palate
137
ramsay hunt syndrome tx
aciclovir and prednisolone
lubricating eye drops
138
other causes of LMN facial nerve palsy
Infections:
Otitis media
Otitis externa
HIV
Lyme disease
Systemic diseases:
Diabetes
Sarcoidosis
Leukaemia
Multiple sclerosis
Guillain–Barré
Tumours:
Acoustic neuroma
Parotid tumour
Cholesteatoma
Trauma:
Direct nerve trauma
Surgery
Base of skull fractures
139
presentation of brain tumours
can be asx
progressive focal neuro sx
raised ICP (intracranial HTN)...eg: change in personality frontal lobe tumour
140
causes of ICP
Brain tumours
Intracranial haemorrhage
Idiopathic intracranial hypertension
Abscesses or infection
141
concerning fx of headache indicate IC HTN
Constant headache
Nocturnal (occurring at night)
Worse on waking
Worse on coughing, straining or bending forward
Vomiting
Papilloedema on fundoscopy
Altered mental state - personality, mental state and memory
Visual field defects
Seizures (particularly partial seizures) - may have aura
Unilateral ptosis (drooping upper eyelid)
Third and sixth nerve palsies
142
investigations raised ICP
non contrast CT usually (contrast if suspect tumour or haemorrhage, if renal function ok)
MRI with contrast
diffusion weighted MRI - different tumour
143
how to describe space occupying lesion on MRI/CT
Particularly Interesting Surgeons Love Carefully Drilling Massive Burr Holes
Patient
Intra or extra axial - in brain parenchyma? Pituitary lesions are extra axial
location - supra or infratentorial, lobes
Density - hypo/hyper (how white)
Border - defined?, oedema
Contrast enhancement - homogenous/heterogenous
mass effect - effacement of sulci, midline shift, ventricle compression
hydrocephalus
144
ddx for raised ICP/space occupying lesion
haemorrhage, infarct, vascular malformation, aneurysm
abscess
mets, primary brain tumour
cyst
MS, granulomatous disease
145
why raised icp causes papilloedema
Papilloedema describes swelling of the optic disc secondary to raised intracranial pressure. Papill- refers to a small, rounded, raised area (the optic disc) and -oedema refers to the swelling.
The sheath around the optic nerve is connected with the subarachnoid space. The raised cerebrospinal fluid (CSF) pressure flows into the optic nerve sheath, increasing the pressure around the optic nerve behind the optic disc causing the optic disc to bulge forward.
146
how papilloedema seen on fundoscopy
Blurring of the optic disc margin
Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation)
Loss of venous pulsation
Engorged retinal veins
Haemorrhages around the optic disc
Paton’s lines, which are creases or folds in the retina around the optic disc
147
gliomas definition
tumours of the glial cells in the brain or spinal cord. Glial cells surround and support the neurones. Glial cells include astrocytes, oligodendrocytes and ependymal cells.
148
gliomas grade
1-4
149
types of gliomas
Astrocytoma (the most common and aggressive form is glioblastoma)
Oligodendroglioma
Ependymoma
150
meingiomas definition and how serious
tumours growing from the cells of the meninges. They are usually benign. However, they take up space, and this “mass effect” can lead to raised intracranial pressure and neurological symptoms
151
cancers most often spreading to brain
Lung
Breast
Renal cell carcinoma
Melanoma
152
pituitary tumours clinical fx
press on optic chiasm - bitemporal hemianopia
Acromegaly (excessive growth hormone)
Hyperprolactinaemia (excessive prolactin)
Cushing’s disease (excessive ACTH and cortisol)
Thyrotoxicosis (excessive TSH and thyroid hormone)
153
management of pituiray tumours
Trans-sphenoidal surgery (through the nose and sphenoid bone)
Radiotherapy
Bromocriptine to block excess prolactin
Somatostatin analogues (e.g., octreotide) to block excess growth hormone
154
acoustic neuroma definition
benign tumours of the Schwann cells that surround the auditory nerve (vestibulocochlear nerve) that innervates the inner ear. Schwann cells provide the myelin sheath around neurones of the peripheral nervous system.
AKA vestibular schwannomas
AKA cerebellopontine angle tumours
155
bilateral acoustic neuromas associated
neurofibromatosis type 2
156
acoustic neuromas clinical fx
40-60 year old presenting with a gradual onset of:
Unilateral sensorineural hearing loss (often the first symptom)
Unilateral tinnitus
Dizziness or imbalance
Sensation of fullness in the ear
Facial nerve palsy (if the tumour grows large enough to compress the facial nerve)
157
acoustic neuroma mx
monitor
surgery to remove
radiotherapy
158
1st line investigation for suspected brain tumour
MRI Brain
biopsy for histological diagnosis
159
brain atumours mx options
surgery
chemo
radio
palliative
160
huntington's chorea inheriteance
aut dom
161
huntington's genetics
trinucleotide repeat disorder involving a genetic mutation in the HTT gene on chromosome 4, which codes for the huntingtin (HTT) protein.
162
huntingtons age
30-50
163
genetic anticipation
Anticipation is 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
164
presentation huntington's
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)
165
huntington's inv
genetic testing
166
huntingtons mx
genetic counselling
MDT
physio to improve mobility, maintain joint function
SALT
Tetrabenazine - chorea sx
antidepressants
advanced directives
167
prognosis huntington's
progressive
life expectancy 10-20yrs after sx onset
death due to aspiration penumonia most commonly
suicide also
168
myasthenia gravis definition
an autoimmune condition affecting the neuromuscular junction. It causes muscle weakness that progressively worsens with activity and improves with rest.
169
myasthenia gravis sex and age
women <40
men >60
170
myasthenia gravis link
thymomas
171
myasthenia gravis patho
The axons release acetylcholine from the presynaptic membrane. Acetylcholine travels across the synapse and attaches to receptors on the postsynaptic membrane, simulating muscle contraction.
Acetylcholine receptor (AChR) antibodies are found in most patients with myasthenia gravis. These antibodies bind to the postsynaptic acetylcholine receptors, blocking them and preventing stimulation by acetylcholine. The more the receptors are used during muscle activity, the more they become blocked.
Antibodies also activate complement system within neuromuscular junction...cell damage at postsynaptic membrane
172
other antibodies causing myasthenia gravis
Muscle-specific kinase (MuSK) antibodies
Low-density lipoprotein receptor-related protein 4 (LRP4) antibodies
173
clinical fx of myasthenia gravis
vary
weakness worse with muscle use and improves with rest, sx best in morning...affect proximal limbs and small muscles of head and neck
Difficulty climbing stairs, standing from a seat or raising their hands above their head
Extraocular muscle weakness, causing double vision (diplopia)
Eyelid weakness, causing drooping of the eyelids (ptosis)
Weakness in facial movements
Difficulty with swallowing
Fatigue in the jaw when chewing
Slurred speech
174
examinations myasthenia gravis
elicit fatigue - Repeated blinking will exacerbate ptosis
Prolonged upward gazing will exacerbate diplopia on further testing
Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
- check for thymectomy scar
- test FVC
175
investigations myasthenia gravis
antibody tests - AChR antibodies (around 85%)
MuSK antibodies (less than 10%)
LRP4 antibodies (less than 5%)
CT/MRI of thymus
edrophonium test (reduce breakdown of Ach...temporarily relieves weakness)
176
tx myasthenia gravis
Pyridostigmine is a cholinesterase inhibitor that prolongs the action of acetylcholine and improves symptoms
Immunosuppression (e.g., prednisolone or azathioprine) suppresses the production of antibodies
Thymectomy
rituximab
177
myasthenic crisis
- what
- triggers
-clinical fx
- tx
potentially life-threatening complication of myasthenia gravis. It causes an acute worsening of symptoms, often triggered by another illness, such as a respiratory tract infection. Respiratory muscle weakness can lead to respiratory failure. Patients may require non-invasive ventilation or mechanical ventilation.
Treatment is with IV immunoglobulins and plasmapheresis.
178
lambert eaton myasthenic syndrome definition
an autoimmune condition affecting the neuromuscular junction, similar to myasthenia gravis. The symptoms tend to be more insidious and less pronounced than myasthenia gravis
179
lambert eaten myasthenic syndrome associations
paraneoplastic syndrome - SCLC
180
LEMS patho
results from antibodies against voltage-gated calcium channels. These antibodies may be produced in response to small-cell lung cancer (SCLC) cells that express voltage-gated calcium channels. They target and damage voltage-gated calcium channels in the presynaptic membrane of the neuromuscular junction. responsible for release of ach into synapse...attaches to post synpatic membrane..less ach release so weaker muscle contraction
181
presenting fx LEMS
proximal muscle wekaness
dry mouth
blurred vision
impotence
dizziness
reduced or absent tendon reflexes
182
how presenting fx different to myasthenia gravis
improves after periods of muscle contraction
183
LEMS mx
exclude underlying malignancy
Amifampridine - block VG K channels in pre synaptic membrnae...prolongs depolarisation of cell membrane and assists calcium to carry out their action
other options - pyridostigmine, immunosuppressants, IV IG, plasmapheresis
184
charcot marie tooth disease definition
an inherited disease that affects the peripheral motor and sensory neurones. It is also known as hereditary motor and sensory neuropathy. There are various types, with different genetic mutations and pathophysiology, causing myelin or axon dysfunction
185
CMT disease inheritance
aut dom
186
CMT age
before age of 10 but can be until 40 or later
187
classical fx CMT disease
High foot arches (pes cavus)
Distal muscle wasting causing “inverted champagne bottle legs”
Lower leg weakness, particularly loss of ankle dorsiflexion (with a high stepping gait due to foot drop)
Weakness in the hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss
188
other causes of peripheral neuropathy
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
189
mx of CMT disease
progressive
Neurologists and geneticists to make the diagnosis
Physiotherapists to maintain muscle strength and joint range of motion
Occupational therapists to assist with activities of living
Podiatrists to help with foot symptoms and suggest insoles and other orthoses to improve symptoms
Analgesia for neuropathic pain (e.g., amitriptyline)
Orthopaedic surgeons for severe joint deformities
190
definition GB syndrome
an acute paralytic polyneuropathy that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms.
191
GB syndrome triggered
Campylobacter jejuni, cytomegalovirus (CMV) and Epstein-Barr virus (EBV).
192
GB syndrome patho
caused by molecular mimicry. B cells create antibodies against antigens on pathogen. these antibodies can also target proteins on the myelin sheath or the nerve axon itself
193
presentation of GB syndrome
sx begin within 4 weeks of triggering infection...begin in feet and progress upwards
peak within 2-4 wks
recovery period lasts mths to yrs
- symmetrical ascending weakness
- reduced reflexes
- peripheral loss of sensation or neuropathic pain
- may cause facial weakness
- can lead to urinary retention, ileus or heart arrythmias
194
diagnosis GB syndrome
brighton criteria supported by investigations - nerve conduction studies, LP (raised protein)
195
mx GB syndrome
Supportive care
VTE prophylaxis (pulmonary embolism is a leading cause of death)
IV immunoglobulins (IVIG) first-line
Plasmapheresis is an alternative to IVIG
intubation and ventilation
196
prognosis GB syndrome
mths to yrs
can continue regaining function up to 5 yrs...usually full recovery, some have signifcant disability
mortality low but due to resp or CVS complications
197
neurofibromatosis definition
a genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system. These tumours are benign but can cause neurological and structural problems.
2 types but 1 is most common
198
neurofibromatosis type 1 gene
found on chromosome 17
codes for a protein called neurofibromin, which is a tumour suppressor protein.
199
neurofibromatosis inheritance
aut dom
200
diagnostic criteria of neurofibromatosis type 1
CRABBING pneumonic:
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
201
skin neurofibromas
skin coloured raised nodules or papules with a smooth, regular surgace. two or more of these are significant
plexiform neurofibroma - a larger, irregular complex neurofibroma, a single one is significant
202
neurofibromatosis mx
no tx
monitor and mx sx and complications
203
complications of neurofibrotomasis
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)
204
neurofibromatosis type 2 genetics
gene is found on chromosome 22. It codes for a protein called merlin, a tumour suppressor protein important in Schwann cells. Schwann cells provide the myelin sheath that surrounds neurones of the peripheral nervous system. Mutations in this gene lead to schwannomas (benign tumours of the Schwann cells). Inheritance is also autosomal dominant.
205
neurofibromatosis type 2 associations
bilateral acoustic neuromas
206
tuberous sclerosis inheritance
aut dom
207
characteristic fx of tuberous sclerosis
hamartomas - benign tissue growths, affecting
Skin
Brain
Lungs
Heart
Kidneys
Eyes
208
tuberous sclerosis mutation in
TSC1 gene on chromosome 9, which codes for hamartin
TSC2 gene on chromosome 16, which codes for tuberin
...interact with each other to control size and growth of cells
209
skin fx tuberous sclerosis
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)
210
neurological fx of tuberous sclerosis
epilepsy
learning disabilities
brain tumours
211
multi system fx of 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
212
tuberous sclerosis mx
no tx
supportive
tx complications - epilepsy
mTOR inhibitors (everolmus or sirolimus) - suppress growth of brain, lung or kidney tumours
213
causes of headaches
Tension headaches
Migraines
Cluster headaches
Secondary headaches
Sinusitis
Giant cell arteritis
Glaucoma
Intracranial haemorrhage
Venous sinus thrombosis
Subarachnoid haemorrhage
Medication overuse
Hormonal headache
Cervical spondylosis
Carbon monoxide poisoning
Trigeminal neuralgia
Raised intracranial pressure
Brain tumours
Meningitis
Encephalitis
Brain abscess
Pre-eclampsia
214
red flag sx of headaches
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)
215
tension headaches
- sx
- cause
-mx
mild ache or band like restriction
resolve gradually
depression, stress, alcohol, skipping meals, dehydration
reassurance and analgesia
if frequent or recurrent = amitriptyline
216
secondary headaches
- presentation
- cause
similar to tension
causes include Infections (e.g., viral upper respiratory tract infection)
Obstructive sleep apnoea
Pre-eclampsia
Head injury
Carbon monoxide poisoning
217
sinusitis
- clinical fx
- cause
- tx
pain and pressure following recent URTI, tenderness on palpation
>10 days tx with steroid nasal spray or phenoxymethylpenicillin
218
medication overuse headache
non specific fx
withdrawal of analgesia
219
hormonal headaches
- cause
- clinical fx
- when
- tx
low oestrogen
unilateral, pulsatile, nausea
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)
tx with triptans and nsaids
220
cervical spondylosis
- cause
- clinical fx
degenerative changes in spine
neck pain, worse on movement, headache
221
trigeminal neuralgia
- fx
- associations
- tx
intense facial pain in distribution of trigeminal nerve
unilateral usually
suddenly and lasts secs to hts, shooting/burning, triggered by touch or eating
MS
carbamazepine
222
migraines more common in
women
teenagers/young adults
223
4 types of migraine
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
224
5 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
225
typical fx of migraine
last 4-72 hrs
Usually unilateral but can be bilateral
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
226
what is aura
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).
227
sx of hemiplegic migraine
hemiplegia
ataxia
impaired consciousness
...must exclude tia/stroke
228
familial hemiplegic migraine
aut dom
229
triggers of migraines
Stress
Bright lights
Strong smells
Certain foods (e.g., chocolate, cheese and caffeine)
Dehydration
Menstruation
Disrupted sleep
Trauma
230
migraine tx
dark room, sleep
NSAIDS
paracetamol
triptans
antiemetics
231
triptans mechanism
5-HT receptor agonists (they bind to and stimulate serotonin receptors), specifically 5-HT1B and 5-HT1D.
Cranial vasoconstriction
Inhibiting the transmission of pain signals
Inhibiting the release of inflammatory neuropeptides
232
when take triptans
ASAP
if attack resolves and reoccurs second dose can be taken but not if in same attack
233
c/i triptans
HTN
CAD
stroke
MI
234
prophylaxis migraine
headachde diary
Propranolol (a non-selective beta blocker)
Amitriptyline (a tricyclic antidepressant)
Topiramate (teratogenic and very effective contraception is needed)
Pizotifen
Candesartan
Sodium valproate
Monoclonal antibodies (e.g., erenumab and fremanezumab)
CBT
mindfulness
acupuncture
vit b2
235
menstrual migraines prophylactic
frovatriptan
zolmitriptan
236
migraine with aura contraception
COCP C/I
237
cluster headaches how often
clusters of attacks and then disappear
attack lasts 15 mins to 3 hrs
238
cluster headache typical person
30-50 yr old male smoker
239
cluster headache triggers
alcohol
strong smells
exercise
240
sx cluster headache
SEVERE
unilateral
Red, swollen and watering eye
Pupil constriction (miosis)
Eyelid drooping (ptosis)
Nasal discharge
Facial sweating
241
cluster headache mx
triptans
high flow o2
242
cluster headaches prophylaxis
verapamil is 1st line
occipital nerve block
prednisolone
lithium
243
causes of spinal cord compression
trauma
prolapsed intervertebral disc
atlantoaxial subluxation (RA)
infection (pott's, discitis)
bony mets
244
CES definition
compression of nerve roots caudal to termination of cord resulting in characteristic sx
245
SCC sx
above level of T1 - tetraplegia
below T1 - paraplegia
minor - paraesis or parathesia
major - complete paralysis
L1 - CES
246
SCC red flag sx
weakness
paraesthsia
ataxia
urinary retenion
UMN signs
247
mets to bone
breast
kidney
thyroid
lung
prostate
248
most common prolapse site
L4/5 or L5/S1
249
clinical sx CES
sudden onset - hours
saddle paraesthesia
bowel etc dysfunction
CES-I (incomplete - reduced urinary function)
CES-R (retention)
loss of lower limb reflexes
reduced anal tone
lower limb weakness/sensory
250
ix for CES
urgent MRI of lumbar spine
251
mx of CES
surgical decompression within 48 hrs of sx onset
some causes can be tx without surgery - eg radiotherapy or if anklyosing sponydlitis - steroids
252
giant cell arteries clinical fx
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
vision testing is a key investigation in all patients
anterior ischemic optic neuropathy accounts for the majority of ocular complications. It results from occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) → ischaemia of the optic nerve head. Fundoscopy typically shows a swollen pale disc and blurred margins
may result in temporary visual loss - amaurosis fugax
permanent visual loss is the most feared complication of temporal arteritis and may develop suddenly
diplopia may also result from the involvement of any part of the oculomotor system (e.g. cranial nerves)
tender, palpable temporal artery
around 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats
253
giant cell arteritis association
polymyalgia rheumaticag
254
giant cell arteritis mx
urgent high-dose glucocorticoids should be given as soon as the diagnosis is suspected and before the temporal artery biopsy
if there is no visual loss then high-dose prednisolone is used
if there is evolving visual loss IV methylprednisolone is usually given prior to starting high-dose prednisolone
there should be a dramatic response, if not the diagnosis should be reconsidered
urgent ophthalmology review
255
s/e of steroids
DM, osteoporosis, mood changes, weight gain
256
dopamine agonists for parkinson's examples
ropinirole, apomorphine
257
s/e of MAO-B inhibitors
headache, wpost hypotension, abdo pain
258
