neurology Flashcards
mx of tension-type headache?
acute- aspirin, paracetamol, NSAID
prophylaxis- acupuncture
amitriptyline
features of migraine?
severe, unilateral throbbing headache
associated with nausea, photophobia, phonophobia
diagnostic criteria for migraine? ABCDE
At least 5 attacks fulfilling criteria B-D:
B- attacks lasting 4-72 hours
C- 2 of the following characteristics:
- unilateral location
-pulsating quality
- moderate or severe pain headache
- aggravation by or causing avoidance of routine physical activity
D- during headache at least one of the following:
1. Nausea and/or vomiting
2. Photophobia and phonophobia
E- not attributed to any other disorder
triggers for migraine
Chocolate Hangovers Orgasms Cheese Oral contraceptives- CI'd if aura Lie-ins Alcohol Tumult Exercise
mx of acute migraine?
1st line- oral triptan and NSAID or paracetamol
metoclopramide or prochlorperazine
prophylaxis of acute migraine?
if 2+ attacks a month
Topiramate or propranolol
acupuncture
riboflavin
what is wrong with topiramate?
cleft palate and reduces effectiveness
RFs for cluster headache?
male, smokers
features of cluster headaches?
Unilateral pain around one eye- episodes last 15 minutes-2 hours
Restless and agitation
Eye- redness, lacrimation, lid swelling, miosis, ptosis
Rhinorrhoea
mx of cluster headaches?
100% O2
sub cut triptan (sumatriptan)
prophylaxis- verapamil, short course prednisolone may help
what is trigeminal neuralgia?
a pain syndrome characterised by severe unilateral pain
majority are idiopathic but could have serious underlying cause such as tumour
features of trigeminal neuralgia?
a unilateral disorder of brief electric-shock like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve
triggers= washing, shaving, brushing teeth
RFs for trigeminal neuralgia?
hypertension, >50
Ix of trigeminal neuralgia?
MRI
mx of trigeminal neuralgia?
1st line= carbamazepine
failure to respond needs prompt referral to neurology
RFs of GCA?
over 50s, associated with polymyalgia rheumatica
features of GCA?
scalp tenderness jaw claudication pulseless temporal arteries temporal pulsating headache amaurosis fugax
ix of GCA?
ESR
raised CRP, platelets, alk phos, reduced Hb
temporal artery biopsy
mx of GCA?
Steroids- prednisolone 60mg
PPI and bisphosphonates
complications of GCA?
Visual loss
signs of raised ICP?
Worse walking, bending forwards, lying down, coughing associated with vomiting papilloedema focal signs seizures
layers of meninges of the brain
Dura mater
Arachnoid mater
Subarachnoid space
Pia mater
causes of SAH?
Berry aneurysm- associated with adult PKD, Ehlers Danlos syndrome, coarctation of the aorta AV malformation Pituitary apoplexy Arterial dissection Mycotic aneurysms
features of SAH?
Thunderclap headache N&V Meningism: photophobia, neck stiffness Coma seizures
Ix of SAH?
CT head
LP- done if CT is negative, perform 12 hours after symptom onset to allow development of xanthochromia (RBC breakdown)
refer to neurosurgery as soon as SAH confirmed
Tx of SAH?
CT intracranial angiogram
clipping of aneurysm
vasospasm is prevented with nimodipine
external ventricular drain for hydrocephalus
maintain cerebral perfusion with dexamethasone to decrease cerebral oedema
causes of subdural haemorrhage?
tearing of bridging veins between the venous sinuses and the cortex
causes increased ICP
RF for subdural haemorrhage?
traumatic head injury
cerebral atrophy/increasing age
alcoholism
anticoagulation meds
Signs and symptoms of subdural haemorrhage?
acute- raised ICP, confusion, seizure
chronic- cognitive decline, personality change, headache
Ix of subdural haemorrhage?
CT- gold standard
diffusely spreading crescent shaped mass
mx of subdural haemorrhage?
neurosurgery- monitor ICP and decompressive craniotomy
if chronic and no neuro deficit- can be managed conservatively
what is an extra dural haemorrhage?
collection of blood between the dura mater and bone
symptoms of extra dural haemorrhage?
briefly consciousness after a low impact injury then lucid interval:
then altered consciousness, severe headache, N&V, confusion, seizures
may lead to rapid increase in intra-cranial pressure- ipsilateral pupil dilatation, signs of brainstem injury/ compression
Ix of extradural haemorrhage?
CT- hyperdense biconvex (lemon shape) adjacent to skull
mx of extradural haemorrhage?
craniotomy and clot evacuation
give mannitol if raised ICP
causes of encephalitis?
viral infection- HSV 1&2, VZV, EBV, CMV, HIV, measles and mumps
Secondary to bacterial meningitis, TB, malaria, listeria etc
symptoms of encephalitis?
features of a viral infection
LOC
behavioural change
focal neurological deficit
Ix of encephalitis?
LP and CSF studies (PCR)- moderate increase in protein level. lymphocytes and decreased glucose
bloods and cultures
mx of encephalitis?
if viral- aliclovir IV high dose for 14 days
IM benpen in meningitis
anti-seizure meds
what is guillain barre syndrome?
an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically campylobacter jejuni)
features of GBS?
Progressive weakness in all 4 limbs some sensory signs history of gastroenteritis areflexia CN involvement e.g. diplopia autonomic involvement e.g. urinary retention, diarrhoea
Ix of GBS?
LP- rise in protein found with a normal WCC
nerve conduction studies
if resp muscle involvement -> ITU admission
mx of GBS?
IVIG for 5 days
causes of epilepsy?
idiopathic CP tuberous sclerosis mitochondrial diseases head injury
types of epilepsy?
primary generalised- LOC, bilateral discharges, no ofcal brain abnormality
partial/focal- one hemisphere involved.
partial seizure is focal structural abnormality until proven otherwise
types of primary generalised epilepsy?
-tonic-clonic- stiffness and rigidity followed by rhythmic jerking. can have tongue biting and incontinence. post-ictal confusion and todd’s paralysis
- tonic
- clonic
- absence
- myoclonic
- atonic
name some non-epileptic causes of seizures?
febrile convulsions
Alcohol withdrawal seizures
Psychogenic non-epileptic seizures
types of focal seizures?
focal aware
focal impaired awareness
awareness unknown
focal to bilateral seizure (secondary generalised)
features of temporal lobe seizure?
HEAD Hallucinations (auditory, olfactory, gustatory) Epigastric rising/emotion Automatisms (lip smacking) Deja vu/ dysphasia (Wernicke's area)
features of frontal lobe seizure? (motor)
head/leg movements
posturing
post-ictal weakness
Jacksonian march (up and down motor homunculus)
features of parietal lobe seizures? (sensory)
paraesthesia
features of occipital lobe seizures? (visual)
floaters/flashes
Ix of epilepsy?
EEG and MRI
mx of epilepsy?
1st line for generalised seizures- sodium valproate 2nd line lamotrigine
1st line for partial seizures- carbamazepine
driving and epilepsy?
cannot drive for 6 moths following a seizure, 12 months if established epilepsy
How can epilepsy meds contraindicate other meds e.g. warfarin?
they can induce/inhibit the P450 system affecting metabolism of other drugs
epilepsy and pregnancy?
sodium valproate is teratogenic
breastfeeding is generally considered safe
epilepsy and contraception?
they both reduce the effectiveness of eachother
how does sodium valproate work and AEs?
increases GABA activity
AEs- weight gain and increased appetite, alopecia, ataxia, tremor, hepatitis, pancreatitis, thrombocytopenia, P450 enzyme inhibitor
how does carbamazepine work? and AEs
binds to sodium channels increasing their refractory period
AEs- P450 inducer, dizziness and ataxia, drowsiness, agranulocytosis, SIADH, visual disturbances
SE of lamotrigine?
stevens- johnson syndrome
how does phenytoin work?
binds to sodium channels and increases their refractory period
AEs- P450 inducer, dizziness and ataxia, drowsiness, hirsutism, megaloblastic anaemia, peripheral neuropathy, osteomalacia, lymphadenopathy
management of status epilepticus?
ABCDE
check BG levels
benzodiazepines e.g. diazepam (rectally or intranasally or sublingual) or lorazepam (IV) 4mg
wait 10 mins
phenytoin, sodium valproate and phenobarbital IV
GA in no response within 30 mins
what should be checked to rule out other causes of status epilepticus?
BG
PaO2
what is Parkinson’s disease?
degeneration of the dopaminergic neurons in the substantia nigra
symptoms are classically symmetrical
development of lery bodies
features of PD?
Bradykinesia
tremor- 3-5Hz, most at rest
rigidity- cog wheel or lead pipe
mask-like facies flexed posture micrographia fatigue REM sleep behaviour disorder psych disturbance
diagnosis of PD?
clinical
MRI will be normal initially but then show atrophy
mx of PD?
If motor symptoms are affecting QOL-> 1st line levodopa
if they aren’t -> dopamine agonist e.g. ropinirole or MAOB inhibitor
Combine drugs
Can add COMT inhibitor
what is levodopa?
usually combined with a decarboxylase inhibitor to prevent peripheral metabolism of levodopa to DA to reduce AEs
- reduces effectiveness with time (usually by 2 years)
AEs of levodopa?
dyskinesia (involuntary writhing movements) on-off effect dry mouth anorexia palpitations postural hypotension psychosis drowsiness
Don’t stop suddenly -> can cause acute dystonia
AEs and monitoring of dopamine receptor agonists?
bromocriptine, ropinirole
prior to tx- ECHO, ESR, CXR, creatinine
AEs- impulse control disorder, excessive daytime sleepiness, hallucinations, nasal congestion, postural hypotension
how do MAO-B inhibitors work?
e.g. selegiline
inhibits the breakdown of DA
AEs- postural hypotension, AF
how do COMT inhibitors work?
e.g. entocapone, tolcapone
stops breakdown of DA
AEs- can cause liver damage
name some parkinson plus syndromes?
Progressive supra-nuclear palsy
Multiple system atrophy
Corticobasal degeneration
Lewy body dementia
features of progressive supra-nuclear palsy?
early postural instability and falls vertical gaze palsy rigidity of limbs < trunks no tremor symmetrical
features of multiple system atrophy?
early autonomic features (postural hypotension, bladder dysfunction)
cerebellar signs
no tremor
features of corticobasal degeneration?
akinetic rigidity involving one limb
features of lewy body dementia?
dementia before motor symptoms= LBD
dementia one year after motor symptoms= PD
what is huntington’s disease?
AD neurodegenerative condition
trinucleotide repeat disorder: CAG
results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia
features of huntington’s disease?
typically after 35 years of age chorea personality change dystonia saccadic eye movements
diagnosis of huntington’s disease?
CT/MRI
clinical
genetic testing
mx of huntington’s disease?
no cure
chorea- benzodiazepines, valproic acid, tetrabenazine
what is motor neuron disease?
cause is unknown
presents with upper and lower motor neuron signs
clues which point towards a diagnosis of MND?
Fasisculations
the absence of sensory S&S
mixture of LMN and UMN signs
wasting of the small hand muscles/ tibialis anterior is common
types of MND?
1) Amytrophic lateral sclerosis- (50%)- typically presents with LMN signs in arms and UMN signs in legs. Associated with fronto-temporal dementia
2) Primary lateral sclerosis- UMN signs only
3) Progressive muscular atrophy- LMN signs only, affects distal muscles before proximal, carries best prognosis
4) progressive bulbar palsy- palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
- carries worse prognosis
diagnosis of MND?
Clinical
nerve conduction studies
EMG
MRI to exclude cervical cord compression and myelopathy
mx of MND?
Riluzole- prevents stimulation of glutamate receptors. Used mainly in amytrophic lateral sclerosis. Prolongs life by about 3 months
Resp care- NIV e.g. BiPAP at night
Spasms- baclofen
prognosis of MND?
50% die within 3 years
what is MS?
Chronic cell-mediated autoimmune disorder characterised by demyelination in the CNS
epidemiology of MS?
3x more common in women
age 20-40 years
more common at higher altitude
types of MS?
- Relapsing-remitting disease- (85%) acute attacks (last 1-2 months) followed by periods of remission
- secondary progressive disease- neurological S&S between relapses
- primary progressive disease (10%)
diagnosis of MS?
2 OR MORE RELAPSES AND EITHER
OBJECTIVE CLINICAL EVIDENCE OF 2 OR MORE LESIONS OR
OBJECTIVE CLINICAL EVIDENCE OF 1 LESION TOGETHER WITH REASONABLE HISTORICAL EVIDENCE OF A PREVIOUS RELAPSE
Features of MS?
Visual- optic neuritis, optic atrophy, Uhthoff’s phenomenon (worsening symptoms following increased body temp), internuclear ophthamoplegia
Sensory- pins and needles, numbness, trigeminal neuralgia, Lhermitte’s syndrome (paraesthesia in limbs on neck flexion)
Motor- spastic weakness
Cerebellar- ataxia, tremor
Others- urinary incontinence, sexual dysfunction, intellectual deterioration
Ix of MS?
MRI- periventricular plaques
CSF- oligoclonal bands, IgG
Visual evoked potentials- delayed, but well preserved waveform
mx of MS?
Acute relapse- oral or IV methylprednisolone for 5 days
Disease modifying drugs- DMARDS (beta-interferon), glatiramer acetate, natalizumab, fingolimod
mx of specific problems of MS- fatigue, spasticity, bladder dysfunction?
fatigue- CBT, mindfulness, amantadine
spasticity- baclofen and gabapentin, PT, diazepam, cannabis and botox
bladder dysfunction- self-catheterisation, anticholinergics e.g. doxizosin
what is myasthenia gravis?
an autoimmune affecting the neuromuscular junction
affects acetylcholine receptors- antibodies against them
associations with myasthenia gravis?
thymomas autoimmune antibodies (pernicious anaemia, thyroid, RA, SLE), thymic hyperplasia
features of myasthenia gravis?
muscle fatiguability extraocular muscle weakness: diplopia ptosis dysphagia peek sign= can't keep eyes closed for long
Ix of myasthenia gravis?
single fibre electromyography CT thorax to exclude thymoma CK normal auto-antibodies- anti-AChR or anti-MuSK Tensilon test- IV edrophonium reduces muscle weakness temporarily
mx of myasthenia gravis?
long-acting anticholinesterase inhibitors e.g. pyridostigmine
immunosuppression e.g. prednisolone initially
thymectomy
mx of myasthenic crisis?
plasmapheresis
IVIG
exacerbating factors for myasthenic crisis?
beta blockers penicllamine lithium phenytoin Abx
causes of dementia?
alzheimer's disease CV disease- multi-infarct dementia lewy body dementia huntingtons CJD Pick's disease HIV
assessment of dementia?
6CIT, 10-CS
GP setting- MMSE, GPCOG
Blood screen to exclude reversible causes- FBC, U&E, LFT, Ca, glucose, TFT, vit B12 and folate
Neuroimaging to excluse organic causes
genetics of Alzheimer’s disease?
most cases are sporadic
5% of cases are inherited as an autosomal dominant trait
mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form
apoprotein E allele E4 - encodes a cholesterol transport protein
risk factors include Down’s syndrome
macroscopic changes of AD?
Widespread cerebral atrophy, particularly involving the cortex and hippocampus
microscopic changes of AD?
Cortical plaques (due to deposition of type A-Beta amyloid protein) Intraneuronal neurofibrillary tangles (caused by anbormal aggregation of the tau protein)
mx of alzheimer’s disease?
non-pharmacological- exercise, group therapy, cognitive rehab
pharmacological-
-3 acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine) 1st line
- memantine (NMDA receptor antagonist) 2nd line
- donepezil contraindicated in bradycardia, can cause insomnia*
features of fronto-temporal lobar dementia?
onset before 65
insidious onset
relatively preserved memory and visuo-spatial skills
personality change and social conduct problems
3 types of fronto-temporal lobar dementia?
- Pick’s disease
- Progressive non-fluent aphasia/ CPA-non-fluent speech
- Semantic dementia- fluent progressive aphasia
features of pick’s disease?
personality change impaired social conduct hyperorality dis-inhibition increased appetite
macroscopic changes associated with pick’s disease?
atrophy of frontal and temporal lobes
microscopic changes associated with pick’s disease?
aggregation of tau proteins
gliosis
neurofibrillary tangles
plaques
features of lewy body dementia?
associated with PD
progressive cognitive impairment
parkinsonism
visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen)
Ix of lewy body dementia?
clinical
SPECT (DAT scan)
tx of lewy body dementia?
both acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine can be used as they are in Alzheimer’s
RF of brain tumours?
ionising radiation
immunosuppression
genetic syndromes- neurofibromatosis
types of brain tumours?
high grade- gliomas- astrocytomas or oligodendromas
- primary cerebral lymphoma
- medulloblastoma
low grade- meningioma, acoustic neuromas, neurofibromas, pituitary adenoma, pineal tumour
features of brain tumours?
increased ICP
neurological focal symptoms- personality change, cognitive or behavioural symptoms, confusion
Coning can occur- herniation of cerebellar tonsils through foramen magnum -> resp depression, bradycardia, death
diagnosis of brain tumours?
CT/MRI
tx of brain surgery?
neurosurgery
cause of extra dural haemorrhage?
fracture of temporal or parietal bone causing laceration of the middle meningeal artery
ususally young adults
causes of cerebellar disease?
PASTRIES Paraneoplastic Alcohol Stroke Trauma Rare- Gluten's ataxia, Friedrich's ataxia Iatrogenic- drugs e.g. phenytoin Endocrine SOL/MS
what is a neurofibromatosis?
a genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system
2 types- NF1 most common
features of neurofibromatosis type 1?
at least 2 of CRABBING.
C – Café-au-lait spots (6 or more) measuring ≥ 5mm in children or ≥ 15mm in adults
R – Relative with NF1
A – Axillary or inguinal freckles
BB – Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
I – Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris
N – Neurofibromas (2 or more) or 1 plexiform neurofibroma
G – Glioma of the optic nerve
mx of neurofibromatosis type 1?
There is no treatment of the underlying disease process or to prevent the development of neurofibromas or complications.
Management is to control symptoms, monitor the disease and treat complications.
complications of neurofibromatosis type 1?
Migraines
Epilepsy
Renal artery stenosis causing hypertension
Learning and 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)
Leukaemia
what is Neurofibromatosis type 2 commonly associated with?
acoustic neuromas
These are tumours of the auditory nerve innervating the inner ear.
bilateral almost always indicates NF2
mx= surgery
mx of benign essential tremor?
propranolol
primidone
features of benign essential tremor?
Fine tremor Symmetrical More prominent on voluntary movement Worse when tired, stressed or after caffeine Improved by alcohol Absent during sleep
what is lambert eaton myasthenic syndrome?
similar to myasthenia gravis
damage to NM junction causes progressive muscle weakness
common cause of lambert eaton myasthenic syndrome?
small cell lung cancer
antibodies against voltage-gated calcium channels in SCLC cells
medical mx of lambert eaton syndrome?
amifampridine allows more ACh to be released in the NM junction
what is charcot marie tooth disease?
an inherited disease that affects the peripheral motor and sensory nerves
features of charcot marie tooth disease?
- high foot arches (pes cavus)
- distal muscle wasting causing inverted champagne bottle legs
- weakness in lower legs (ankle dorsiflexion)
- weakness in hands
- reduced tendon reflexes
- reduced tone
- peripheral sensory loss
differentials of peripheral neuropathy?
A – Alcohol B – B12 deficiency C – Cancer and Chronic Kidney Disease D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin) E – Every vasculitis
mx of charcot marie tooth disease?
neurologists
PT, OT, podiatrists
ortho surgeons to correct disabling joint deformities
What is tuberous sclerosis characterised by?
harmatomas- benign neoplastic growths of the tissue (skin, brain, lungs, heart, eyes)
can cause epilepsy
skin features of tuberous sclerosis?
ash leaf spots shagreen patches angiofibromas café au lait spots poliosis
where does the facial nerve exit the brainstem?
cerebellopontine angle
passes through the temporal bone and parotid gland
what are the 5 branches of the facial nerve?
temporal zygomatic buccal marginal mandibular cervical
functions of the facial nerve?
motor- muscles of facial expression, stapedius (ear), some neck muscles
sensory- taste anterior 2/3 tongue
parasympathetic- submandibular and sublingual salivary glands and lacrimal gland
why is the forehead not spared in a LMN lesion?
each side of the forehead only has innervation from one side of the brain therefore in a lesion it can’t be supplied by the other side of the brain
examples of unilateral and bilateral UMN lesions of the facial nerve?
Unilateral- CVAs, tumours
Bilateral- pseudobulbar palsies, MND
causes of LMN facial nerve palsies?
Infection- otitis media, malignant otitis externa
Systemic disease- DM, sarcoidosis, leukaemia, MS, GBS
Tumours- acoustic neuroma, parotid tumour, cholesteatomas
Trauma
