Neurology Flashcards
example and action of 5-HT3 antagonists
e.g., ondansetron
antiemetics - used in management of chemo-therapy-related nausea
act in chemoreceptor trigger zone of medulla oblongata
S/E - QT prolongation, constipation
features of wernicke’s aphasia
receptive aphasia
sentences make no sense, word substitution, neologisms, fluent speech - word salad
impaired comprehension
lesion in wernicke’s aphasia
due to lesion of superior temporal gyrus
supplied by inferior division of left MCA
lesion in broca’s aphasia
lesion of the inferior frontal gyrus
supplied by the superior division of the left MCA
features of broca’s aphasia
non-fluent, laboured, and halting speech
impaired repitition
normal comprehension
features of conduction aphasia
speech fluent, repetition poor
aware of errors
normal comprehension
lesion in conduction aphasia
due to a stroke affecting the arcuate fasiculus - the connection between Wernicke’s and Broca’s area
features of global aphasia
non fluent, comprehension impaired, severe expressive and receptive aphasia
definition of arnold chiari malformation
herniation of the cerebellar tonsils through the foramen magnum
may be congenital or acquired (traumatic)
features of arnold chiari malformation
non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow
headache
syringomyelia
causes of ataxia
Cerebellar hemisphere lesions cause peripheral (‘finger-nose ataxia’)
Cerebellar vermis lesions cause gait ataxia
features of ataxia telangiectasia
an autosomal recessive disorder caused by defective ATM gene (encodes for DNA repair enzymes - combined immunodeficiency disorders
cerebellar ataxia - inability to coordinate muscle movement
telangiectasia
IgA deficiency
describe autonomic dysreflexia
clinical syndrome in those with SC injury above T6
afferent signals (due to faecal impaction/urinary retention) cause sympathetic spinal reflex via thoracolumbar outflow
parasympathetic response is prevented due to lesion
therefore unbalanced physiological response leads to - extreme hypertension, flushing and sweating above the level of the cord lesion, agitation
definition of bells palsy
acute, unilateral, idiopathic, facial nerve paralysis
features of bells palsy
LMN - forehead affected
UMN - forehead sparing
post-auricular pain (may precede paralysis)
altered taste
dry eyes
hyperacusis
management of bells palsy
oral prednisolone <72 hours of onset
eye care - prevent exposure keratopathy
if paralysis not improved after 3 weeks - urgent ENT referral
refer to plastic surgery if long standing weakness
normally full recovery <3-4m
branchial plexus nerve roots
C5 to T1
erb duchenne paralysis
damage to C5,6 roots
winged scapula
may be caused by a breech presentation
klumpke’s paralysis
damage to T1
loss of intrinsic hand muscles
due to traction
presentation of brain abscess
depends upon the site of the abscess
considerable mass effect, raised ICP
dull persistent headache
fever
focal neurology (due to raised ICP) - and nausea, papilloedema, seizures
management of brain abscess
surgery - craniotomy, abscess cavity debrided
IV ceftriaxone + metronidazole
ICP mx dexamethasone
features of parietal lobe lesions
sensory inattention
apraxias
astereognosis (tactile agnosia)
inferior homonymous quadrantanopia
Gerstmann’s syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
features of occipital lobe lesions
homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia
features of temporal lobe lesions
Wernicke’s aphasia: this area ‘forms’ the speech before ‘sending it’ to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
superior homonymous quadrantanopia
auditory agnosia
prosopagnosia (difficulty recognising faces)
features of frontal lobe lesions
expressive (Broca’s) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting
disinhibition
perseveration
anosmia
inability to generate a list
features of cerebellar lesions
midline lesions: gait and truncal ataxia
hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
common source of brain metastases
lung (most common)
breast
bowel
skin (namely melanoma)
kidney
features of gliobastoma multiforme
most common primary tumour in adults
poor prognosis
solid tumours, central necrosis, rim enhances with contrast
surgical tx with postoperative chemotherapy and/or radiotherapy
features of meningioma
second most common primary brain tumour in adults
benign, extrinsic tumours of CNS, located next to dura, cause compressive sx
contrast enhancing on CT
observation, radiotherapy, surgical resection
features of vesticular schwannoma
AKA acoustic neuroma
benign tumour arising from CN8
seen in cerebellopontine angle
presentation - hearing loss, facial nerve palsy, tinnitus
observation, radiotherapy or surgery
features of medulloblastoma
aggressive paediatric brain tumour
spreads through CSF
surgical resection and chemo
features of ependymoma
Commonly seen in the 4th ventricle
May cause hydrocephalus
features of pituitary adenoma
benign
secretory or non-secretory, microadenoma or macroadenoma
presents with hormone excess or hormone depletion
features of brown sequard syndrome
caused by lateral hemisection of the spinal cord
ipsilateral weakness below lesion
ipsilateral loss of proprioception and vibration sensation
contralateral loss of pain and temperature sensation
indications and moa of carbamazepine
used in the treatment of epilepsy, particularly partial seizures
binds to sodium channels and increases their refractory period
features of cataplexy
sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy.
Features range from buckling knees to collapse.
what are the cavernous sinuses
paired and are situated on the body of the sphenoid bone. It runs from the superior orbital fissure to the petrous temporal bone
features of cerebellar disease
D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear ‘Drunk’
A - Ataxia (limb, truncal)
N - Nystamus (horizontal = ipsilateral hemisphere)
I - Intention tremour
S - Slurred staccato speech, Scanning dysarthria
H - Hypotonia
unilateral cerebellar lesions cause ipsilateral signs
causes of cerebellar syndrome
Friedreich’s ataxia, ataxic telangiectasia
neoplastic: cerebellar haemangioma
stroke
alcohol
multiple sclerosis
hypothyroidism
drugs: phenytoin, lead poisoning
paraneoplastic e.g. secondary to lung cancer
definition of cerebral perfusion pressure
net pressure gradient causing blood flow to the brain
tightly autoregulated to maximise cerebral perfusion
sharp rise = rising ICP, fall = cerebral ischaemia
CPP= Mean arterial pressure - Intra cranial pressure
features of charcot marie tooth disease
most common hereditary peripheral neuropathy - causes predominantly motor loss
foot drop
high arched feet - pes cavus
distal muscle weakness + atrophy
hyporeflexia
physical and occupational therapy
features of cluster headache
intense sharp, stabbing pain around one eye lasting 15 mins - 2 hours
clusters typically last 4-12 weeks
accompanied by redness, lacrimation, lid swelling
nasal stuffiness
miosis and ptosis in a minority
management of cluster headache
acute
100% oxygen (80% response rate within 15 minutes)
subcutaneous triptan (75% response rate within 15 minutes)
prophylaxis
verapamil is the drug of choice
some evidence to support a tapering dose of prednisolone
features of common peroneal nerve lesion
foot drop
weakness of foot dorsiflexion
weakness of foot eversion
weakness of extensor hallucis longus
sensory loss over the dorsum of the foot and the lower lateral part of the leg
wasting of the anterior tibial and peroneal muscles
features of creutzfeldt jakob disease
apidly progressive neurological condition caused by prion proteins
dementia (rapid onset)
myoclonus
investigation findings of CJD
CSF is usually normal
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
MRI: hyperintense signals in the basal ganglia and thalamus
features of degenerative cervical myelopathy
Pain (neck, upper or lower limbs)
loss of motor function and dexterity
loss of sensory function - numbness
loss of autonomic function (urinary/faecal incontinence)
Hoffman sign - flick one finger on patients hand, positive test causes reflex twitching
management of degenerative cervical myelopathy
MRI cervical spine - disc degeneration, ligament hypertrophy
refer urgently for assessment
early tx = better prognosis
decompressive surgery
risk factors for degenerative cervical myelopathy
smoking
genetics
occupation - high axial loading
drugs causing peripheral neuropathy
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
DVLA rules for epilepsy and seizures
all patient must not drive and must inform the DVLA
1st seizure - 6 months off if idiopathic, 12 months off if positive EEG/structural abnormality
established epilepsy - can drive if seizure free for 12m, full license if no seziures for 5 years
no driving during medication withdrawal and <6m of last dose
dvla rules for syncope
simple faint: no restriction
single episode, explained and treated: 4 weeks off
single episode, unexplained: 6 months off
two or more episodes: 12 months off
DVLA rules for stroke/TIA
stroke or TIA: 1 month off driving, may not need to inform DVLA if no residual neurological deficit
multiple TIAs over short period of times: 3 months off driving and inform DVLA
dvla rules on brain tumours
pituitary tumour: craniotomy: 6 months; trans-sphenoidal surgery ‘can drive when there is no debarring residual impairment likely to affect safe driving’
dvla rules for chronic neurological disorders
chronic neurological disorders e.g. multiple sclerosis, motor neuron disease: DVLA should be informed, complete PK1 form (application for driving licence holders state of health)
features of duchenne muscular dystrophy
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower’s sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
neuropathy findings on EMG
Neuropathy causes
increased action potential duration (slow)
increased action potential amplitude
myopathy findings on EMG
reduced action potential duration (fast)
reduced action potential amplitude
features of encephalitis
fever, headache, psychiatric symptoms, seizures, vomiting
focal features e.g. aphasia
HSV-1 is responsible for 95% of cases in adults
typically affects temporal and inferior frontal lobes
investigation findings in encephalitis
CSF - lymphocytosis, elevated protein, PCR - HSV, VZV, enterovirus
neuroimaging - can be normal
EEG - lateralised periodic discharges at 2 Hz
features of infantile spasms
flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
progressive mental handicap
EEG: hypsarrhythmia
secondary to serious neurological abnormality
treat with vigabatrin, steroids
poor prognosis
features of typical absence seizures
duration few-30 secs; no warning, quick recovery; often many per day
EEG: 3Hz generalized, symmetrical
sodium valproate, ethosuximide
features of juvenile myoclonic epilepsy
typical onset is in the teenage years, more common in girls
features:
infrequent generalized seizures, often in morning//following sleep deprivation
daytime absences
sudden, shock-like myoclonic seizure (these may develop before seizures)
treatment: usually good response to sodium valproate
classification of seizures
- Where seizures begin in the brain - focal vs generalised, unknown onset
- Level of awareness during a seizure - aware vs impaired awareness
- Other features of seizures - motor, non motor, aura
features of focal seizures
start in a specific area, on one side of the brain
level of awareness can vary
focal aware
focal impaired awareness
awareness unknown
motor/non motor/other features e.g., aura
features of generalised seizures
engage or involve networks on both sides of the brain at the onset
consciousness lost immediately
motor, non motor
features of temporal lobe seizures
can be with or without impairment of consciousness/awareness
aura - rising epigastric sensation - psychic phenomena, possible hallucinations (olfactory, auditory)
seizures last ~1min
may have automatisms - lip smacking etc
features of frontal lobe seizures
often motor signs
Head/leg movements, posturing, post-ictal weakness, Jacksonian march
features of parietal lobe seizures
Paraesthesia
sensory symptoms
features of occipital lobe seizures
visual factors
floaters, flashes
indications for sodium valproate
used in males for
generalised TC seizures
myoclonic seizures
tonic or atonic seizures
indications of lamotrigine/levetiracetam
used first line for focal seizures in males and females
used in females
either for TC seizures
lamotrigine - tonic/atonic
levetiracetam - myoclonic
indications for ethosuximide
first line for absence seizures
indications for starting anti epileptics
NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present:
the patient has a neurological deficit
brain imaging shows a structural abnormality
the EEG shows unequivocal epileptic activity
the patient or their family or carers consider the risk of having a further seizure unacceptable
features of essential tremor
autosomal dominant condition which usually affects both upper limbs
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
causes of extradural haematoma
‘low-impact’ trauma e.g., fall
collection is often in the temporal region since the thin skull at the pterion overlies the middle meningeal artery and is therefore vulnerable to injury
features of extradural haematoma
lucid intervals - consciousness eventually lost due to expanding haematoma and brain herniation around tentorium cerebelli – causes fixed and dilated pupil due to compression of CN3
biconvex/lentiform, hyperdense collection
limited by suture lines
functions of the facial nerve
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
causes of bilateral facial nerve palsy
sarcoidosis
Guillain-Barre syndrome
Lyme disease
bilateral acoustic neuromas (as in neurofibromatosis type 2)
as Bell’s palsy is relatively common it accounts for up to 25% of cases f bilateral palsy, but this represents only 1% of total Bell’s palsy cases
causes of foot drop
Foot drop is a result of weakness of the foot dorsiflexors.
Possible causes :
common peroneal nerve lesion - the most common cause
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
other possible includes central nerve lesions (e.g. stroke) but other features are usually present
features of CN4 palsy
supplies superior oblique (depresses eye, moves inward)
vertical diplopia
classically noticed when reading or going downstairs
subjective tilting of objects (torsional diplopia)
compensatory head tilt
affected eye appears to deviate upwards and is rotated outwards
features of friedreich’s ataxia
onset 10-15y
autosomal recessive trinucleotide repeat disorder
Gait ataxia and kyphoscoliosis
HOCM, DM
Neurological features
absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration
pathogenesis of guillain barre syndrome
immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni)
cross-reaction of antibodies with gangliosides in the peripheral nervous system
features of guillain barre syndrome
back/leg pain
progressive, symmetrical weakness of all the limbs
ascending weakness
reflexes are reduced or absent
mild sensory sx
may progress to involve respiratory muscles and cranial nerves
investigation findings of guillain barre syndrome
LP - rise in protein, normal WCC
nerve conduction studies - reduced motor nerve conduction velocity (demyelination), prolonged distal motor latency
features of temporal arteritis
Typically patient > 60 years old
Usually rapid onset (e.g. < 1 month) of unilateral headache
Jaw claudication (65%)
Tender, palpable temporal artery
Raised ESR
red flag symptoms in headache
vomiting
worsening headache with fever
new-onset neurological deficit
new-onset cognitive dysfunction
change in personality
impaired level of consciousness
recent (typically within the past 3 months) head trauma
thunderclap
triggered by cough, sneeze, exercise
orthostatic
sx suggesting GCA/acute narrow angle glaucoma
definition and types of hereditary sensorimotor neuropathy
HSMN type I: primarily due to demyelinating pathology
HSMN type II: primarily due to axonal pathology
features of huntingtons
develop >35y
chorea
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movements
risk factors for idiopathic intracranial hypertension
obesity
female sex
pregnancy
drugs (combined oral contraceptive pill, steroids, tetracyclines, retinoids (isotretinoin, tretinoin) / vitamin A, lithium)
features of idiopathic intracranial hypertension
headache
blurred vision
papilloedema (usually present)
enlarged blind spot
sixth nerve palsy may be present
management of idiopathic intracranial hypertension
weight loss
carbonic anhydrase inhibitors e.g. acetazolamide
topiramate
overview of internuclear ophthalmoplegia
causes horizontal disconjugate eye movement
due to lesion in medial longitudinal fasciculus which controls horizontal eye movements by connecting CN3, 4 and 6
features of internuclear ophthalmoplegia
ipsilateral impaired adduction
contralateral horizontal nystagmus in abducting eye
features of intracranial venous thrombosis
may cause cerebral infarction - less common than arterial causes
headache (may be sudden onset)
nausea & vomiting
reduced consciousness
investigation findings of intracranial venous thrombosis
MRI venography/CT venography
non contrast CT head
D dimer
management of intracranial venous thrombosis
anticoagulation - LMWH then long term warfarin
features of sagittal sinus thrombosis
seizures, hemiplegia
parasagittal biparietal/bifrontal haemorrhagic infarctions
features of cavernous sinus thrombosis
periorbital oedema
ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
central retinal vein thrombosis
causes of lambert eaton myasthenic syndrome
association with small cell lung cancer (+ breast and ovarian)
may be autoimmune
caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system
features of lambert eaton myasthenic syndrome
non fatiguable - repeated muscle contractions lead to increased muscle strength (but will eventually decrease)
limb girdle weakness
hyporeflexia
autonomic sx - dry mouth, impotence, difficulty micturating
EMG - incremental response to repetitive electrical stimulation
management of of lambert eaton myasthenic syndrome
treat underlying cancer
immunosuppression - pred, azathioprine
IVIG
plasma exchange
indications for lamotrigine
sodium channel blocker
Adverse effects
Stevens-Johnson syndrome
features of lateral medullary syndrome
Wallenberg’s - occurs following occlusion of posterior inferior cerebellar artery
cerebellar fx - ataxia, nystagmus
brainstem fx - ipsilateral dysphagia, facial numbness, CN palsy (horners), contralateral limb sensory loss
median nerve supply
Motor supply (LOAF)
Lateral 2 lumbricals
Opponens pollicis
Abductor pollicis brevis
Flexor pollicis brevis
sensory supply
Over thumb and lateral 2 ½ fingers
On the palmar aspect this projects proximally, on the dorsal aspect only the distal regions are innervated with the radial nerve providing the more proximal cutaneous innervation.
features of median nerve damage at wrist
e.g., carpal tunnel
paralysis and wasting of thenar eminence muscles and opponens pollicis (ape hand deformity)
sensory loss to palmar aspect of lateral (radial) 2 ½ fingers
features of median nerve damage at elbow
unable to pronate
wrak wrist flexion
ulnar deviation of wrist
features of medication overuse headache
most common cause of chronic daily headache
present for >15 days per month
developed/worsened due to medication - usually opoids and triptans
withdraw opioids gradually, triptans/simple analgesics abruptly
meningococcal disease vs meningitis
meningococcal disease = any disease caused by neisseria meningitidis
can both present similarly to less serious, viral infections - classical signs often absent in infants with bacterial meningitis
features of meningitis
headache
fever
nausea/vomiting
photophobia
drowsiness
seizures
neck stiffness
purpuric rash (particularly with invasive meningococcal disease)
CSF findings in bacterial meningitis
cloudy, low glucose, high protein, high polymorphs
CSF findings in viral meningitis
clear/cloudy, 60-80% plasma glucose, normal protein, high lymphocytes
CSF findings in TB meningitis
slightly cloudy, fibrin web, low glucose, high protein, high lymphocytes
complications of meningitis
sensorineural hearing loss (most common)
seizures
focal neurological deficit
infective
sepsis
intracerebral abscess
pressure
brain herniation
hydrocephalus
characteristics of migraine
severe, unilateral, throbbing headache
nausea, photophobia and phonophobia
up to 72h
precipitated by aura - visual progressive 5-60mins
triggers for migraine
tiredness, stress
alcohol
combined oral contraceptive pill
lack of food or dehydration
cheese, chocolate, red wines, citrus fruits
menstruation
bright lights
management of migraine
5-HT receptor agonists (triptans) - acute tx
5-HT receptor antagonists are used in prophylaxis
1st line - triptans + NSAID/paracetamol
prophylaxis - propranolol, topiramate
features of motor neuron disease
neuro condition, unknown cause
asymmetric limb weakness
UMN and LMN signs
wasting of small hand muscles
fasciculations
absence of sensory signs and symptoms
doesnt effect external ocular muscles
no cerebellar signs
investigation findigns in motor neuron disease
EMG - reduced number of action potentials with increased amplitude
management of motor neuron disease
riluzole - used in AML - prevents stimulation of glutamate receptors
respiratory care = NIV
PEG tube
types of motor neuron disease
Amyotrophic lateral sclerosis (50% of patients) - LMN sx in arms, UMN sx in legs
primary lateral sclerosis - UMN signs only
progressive muscular atrophy - LMN only, distal muscles affected first
progressive bulbar palsy - palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
overview of multiple sclerosis
chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system
F>M
diagnosed in 20-40
subtypes of multiple sclerosis
relapsing remitting - acute attacks followed by periods of remission
secondary progressive - relapsing remitting patients who have deteriorated and now have neuro signs between relapses
primary progressive - progressive deterioration from onset
features of multiple sclerosis
visual
optic neuritis: common presenting feature
optic atrophy
Uhthoff’s phenomenon: worsening of vision following rise in body temperature
internuclear ophthalmoplegia
Sensory
pins/needles
numbness
trigeminal neuralgia
Lhermitte’s syndrome: paraesthesiae in limbs on neck flexion
Motor
spastic weakness: most commonly seen in the legs
Cerebellar
ataxia: more often seen during an acute relapse than as a presenting symptom
tremor
Others
urinary incontinence
sexual dysfunction
intellectual deterioration
investigation findings in multiple sclerosis
MRI - periventricular plaques
CSF - oligoclonal bands (none in blood), increased intrathecal synthesis of IgG
managing acute relapse of multiple sclerosis
High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse
managing multiple sclerosis
natalizumab IV/ocrelizumab IV both prevent relapse
for spasticity - baclofen and gabapentin are first-line
bladder dysfuction - intermittent self-catheterisation, anticholinergics
fatigue - mindfulness, CBT
types of multiple system atrophy
MSA-P - Predominant Parkinsonian features
MSA-C - Predominant Cerebellar features
Shy-Drager syndrome
features of multiple system atrophy
parkinsonism
autonomic disturbance
erectile dysfunction: often an early feature
postural hypotension
atonic bladder
cerebellar signs
overview of myasthenia gravis
autoimmune disorder resulting in insufficient functioning acetylcholine receptors - due to antbodies to acetylcholine receptors
associated with thymomas, AI disorders
features of myasthenia gravis
extraocular muscle weakness: diplopia
proximal muscle weakness: face, neck, limb girdle
ptosis
dysphagia
fatiguability - muscles become progressively weaker during activity, slowly improve after rest
investigation findings in myasthenia gravis
EMG
CT thorax - exclude thymoma
normal CK
Ach R antibodies
tensilon test - IV edrophonium temporarily reduces muscle weakness
management of myasthenia gravis
long-acting ACHesterase inhibitors - pyridostigmine
immunosuppression - prednisolone, azathioprine, cyclosporine, MMF
managing myasthenic crisis - plasmapheresis, IVIG
overview of myotonic dystrophy
autosomal dominant inherited myopathy, features develop around 20-30y
affects skeletal, cardiac and smooth muscle
DM1 - distal weakness more prominent
DM2 - proximal weakness more prominent
features of myotonic dystrophy
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
weakness
myotonia - tonic muscle spasm
mild mental impairment
DM
testicular atophy
overview of narcolepsy
associated with low levels of orexin (hypocretin), a protein which is responsible for controlling appetite and sleep patterns
early onset REM sleep
features of narcolepsy
onset in teens
hypersomnolence
cataplexy (sudden loss of muscle tone often triggered by emotion)
sleep paralysis
vivid hallucinations on going to sleep or waking up
features of neurofibromatosis type 1
Café-au-lait spots (>= 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamatomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas
features of neurofibromatosis type 2
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, mengiomas and ependymomas
overview of neuroleptic malignant syndrome
rare but dangerous
occurs in patients taking antipsychotics and dopaminergic drugs - dopamine blockade triggers massive glutamate release and subsequent neurotoxicity and muscle damage
occurs <hrs to days of starting an antipsychotic
features of neuroleptic malignant syndrome
pyrexia
muscle rigidity
autonomic lability: typical features include hypertension, tachycardia and tachypnoea
agitated delirium with confusion
raised CK
AKI secondary to rhabdomyolysis if severe
leukocytosis
management of neuroleptic malignant syndrome
stop antipsychotic
IV fluids - prevent renal failure
dantrolene
possible ICU
common causes of neuropathic pain
pain which arises following damage or disruption of the nervous system
diabetic neuropathy
post-herpetic neuralgia
trigeminal neuralgia
prolapsed intervertebral disc
treatment of neuropathic pain
first-line treatment*: amitriptyline, duloxetine, gabapentin or pregabalin
can switch between them if not effective
tramadol - rescue therapy
features of normal pressure. hydrocephalus
reversible cause of dementia seen in elderly patients - secondary to reduced CSF absorption at the arachnoid villi
onset few months
urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson’s disease)
management of normal pressure hydrocephalus
CT - ventriculomegaly out of proportion to sulcal enlargement
manage with ventriculoperitoneal shunting
pathophysiology of parkinsons disease
progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra.
The reduction in dopaminergic output results in a classical triad of features: bradykinesia, tremor and rigidity.
asymmetrical symptoms
features of parkinsons disease
bradykinesia - or hypokinesia, shuffling gait, difficulty initiating movement
tremor - prominent at rest, worst if stressed/tired, improves with voluntary movement, ‘pill rolling’
rigidity - lead pipe, cogwheel (due to superimposed tremor)
mask-like facies
flexed posture
micrographia
drooling of saliva
psychiatric features
postural hypotension
REM sleep behaviour disorder
treatment options for parkinsons
for motor sx - levodopa
for nonmotor sx - dopamine agonist, MAOB, levothyroxine
causes of parkinsonism
Parkinson’s disease
drug-induced e.g. antipsychotics, metoclopramide*
progressive supranuclear palsy
multiple system atrophy
Wilson’s disease
post-encephalitis
dementia pugilistica (secondary to chronic head trauma e.g. boxing)
toxins: carbon monoxide, MPTP
overview of patoxysmal hemicrania
attacks of severe, unilateral headache, usually in the orbital, supraorbital or temporal region
autonomic features
<30 mins
multiple times a day
responsive to treatment with indomethacin
peripheral neuropathies causing motor loss
Guillain-Barre syndrome
porphyria
lead poisoning
hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
chronic inflammatory demyelinating polyneuropathy (CIDP)
diphtheria
peripheral neuropathies causing sensory loss
diabetes
uraemia
leprosy
alcoholism
vitamin B12 deficiency
amyloidosis
features of alcoholic neuropathy
secondary to both direct toxic effects and reduced absorption of B vitamins
sensory symptoms typically present prior to motor symptoms
features of vitamin B12 deficiency
subacute combined degeneration of spinal cord
dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia
definition and causes of pituitary apoplexy
Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction
Precipitating factors
hypertension
pregnancy
trauma
anticoagulation
features of pituitary apoplexy
sudden onset headache similar to that seen in subarachnoid haemorrhage
vomiting
neck stiffness
visual field defects: classically bitemporal superior quadrantic defect
extraocular nerve palsies
features of pituitary insufficiency
e.g. hypotension/hyponatraemia secondary to hypoadrenalism
management of pituitary apoplexy
MRI - diagnostic
urgent steroid replacement due to loss of ACTH
careful fluid balance
surgery
features of post-lumbar puncture headache
occurs in 1/3 patients - due to leak in CSF following dural puncture
usually develops within 24-48 hours following LP but may occur up to one week later
may last several days
worsens with upright position
improves with recumbent position
managment of post-lumbar puncture headache
supportive initially (analgesia, rest)
if pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural haematoma
treatment options include: blood patch, epidural saline and intravenous caffeine
features of progressive supranuclear palsy
parkinson plus syndrome
postural instability and falls - stiff, broad-based gait
impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
parkinsonism
bradykinesia
cognitive impairment
primarily frontal lobe dysfunction
poor response to L Dopa
features of pseudoseizures
pelvic thrusting
family member with epilepsy
much more common in females
crying after seizure
don’t occur when alone
gradual onset
features of radial nerve damage
wrist drop
sensory loss to small area between 1st and 2nd metacarpals
paralysis of triceps if axillary damage
features of raised intracranial pressure
headache
vomiting
reduced levels of consciousness
papilloedema
Cushing’s triad
widening pulse pressure
bradycardia
irregular breathing
management of raised intracranial pressure
ix and tx underlying cause
elevate head
IV mannitol
controlled hyperventilation - reducing PCO2 - vasoconstriction of cerebral arteries - reduces ICP
CSF removal
common reflexes and nerve roots
Ankle S1-S2
Knee L3-L4
Biceps C5-C6
Triceps C7-C8
definition and causesof restless legs syndrome
syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia
common
positive family history in 50% of patients
causes
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy
features of restless legs syndrome
akathisia - uncontrollable urge to move legs
symptoms occur at night then day
sx worse at rest
paraesthesias - crawling/throbbing sensation
features of reye’s syndrome
severe, progressive encephalopathy in children
assx with aspirin use
may have preceding viral illness
encphalopathy - confusion, seizures, cerebral oedema, coma
fatty infiltration of liver, kidneys, pancreas
hypoglycaemia
supportive management
management of acute seizures
check the airway and apply oxygen if appropriate
place the patient in the recovery position
if the seizure is prolonged give benzodiazepines
rectal diazepam, repeat after 10-15mins if necessary
adult 10-20mg max 30mg
midazolam oromucosal solution
adverse effects of sodium valproate
teratogenic - neural tube defects, neurodevelopmental delay
p450 inhbitor
nausea
increased appetite, weight gain
ataxia
tremor
hepatotoxicity
pancreatitis
thrombocytopaenia
hyponatraemia
features of brown sequard syndrome
= spinal cord hemisection
- Lateral corticospinal tract - ipsilateral spastic paresis
- Dorsal columns - ipsilateral loss of proprioception and vibration sensation
- Lateral spinothalamic tract - contralateral loss of pain and temperature
features of subacute combined degeneration of the spinal cord
- Lateral corticospinal tracts - bilateral spastic paresis
- Dorsal columns - bilateral loss of proprioception and vibration
- Spinocerebellar tracts – bilateral limb ataxia
features of friedrich’s ataxia
- Lateral corticospinal tracts - bilateral spastic paresis
- Dorsal columns - bilateral loss of proprioception and vibration
- Spinocerebellar tracts – bilateral limb ataxia
+ cerebellar ataxia
features of anterior spinal artery occlusion
- Lateral corticospinal tracts - bilateral spastic paresis
- Lateral spinothalamic tracts - bilateral loss of pain and temperature sensation
features of syringomyelia
- Ventral horns - flaccid paresis
- Lateral spinothalamic tract - loss of pain and temperature sensation
features of neurosyphilis (tabes dorsalis)
dorsal columns - loss of proprioception and vibration sensation
features of spontaneous intracranial hypotension
rare cause of headaches, results from CSF leak from thoracic nerve root sleeves
headache generally much worse when upright
management of status epilepticus
single seizure lasting >5 minutes, or
>= 2 seizures within a 5-minute period
ABC
benzos - PR diazepam/buccal midazolam prehospital, IV lorazepam in hospital
if ongoing ‘established’ - 2nd line agent levetiracetam, phenytoin or sodium valproate
if no response ‘refractory’ <45mins - induction of general anaesthesia
features of anterior cerebral artery stroke
Contralateral hemiparesis and sensory loss, lower extremity > upper
features of middle cerebral artery stroke
Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia
features of posterior cerebral artery stroke
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
features of weber syndrome
branches of the posterior cerebral artery that supply the midbrain
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
features of posterior inferior cerebellar artery stroke
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
features of anterior inferior cerebellar artery stroke
Symptoms are similar to Wallenberg’s (see above), but:
Ipsilateral: facial paralysis and deafness
features of lacunar stroke
present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
strong association with hypertension
common sites include the basal ganglia, thalamus and internal capsule
issues to consider in stroke management
fluids - oral preferable, IV isotonic saline if needd
glycaemic control - close monitoring needed, high mortality in hyperglycaemia
diabetic patients need intense management
BP management - cautious lowering of BP due to blood flow compromise
feeding - SALT assessment, NG if unsafe swallow
use of scoring systems in stroke
The FAST screening tool (Face/Arms/Speech/Time) is widely known by the general public following a publicity campaign. It has a positive predictive value of 78%.
A variant of FAST called the ROSIER score is useful for medical professionals. It is validated tool recommended by the Royal College of Physicians.
investigation findings in stroke
non-contrast CT head - first line
acute ischaemic stroke - low density in grey and white matter, hyperdense artery (responsible arterial clot)
acute haemorrhagic stroke - areas of hyperdense (blood) surrounded by low density (oedema)
management of stroke
dont lower BP unless complications
aspirin 300mg orally after excluding haemorrhagic
start statin if cholesterol >3.5
managing acute ischaemic stroke
thrombolysis with alteplase if given <4.5h of stroke sx onset
thrombectomy <6h of sx onset if acute ischemic stroke and confirmed occlusion of proximal anterior circulation
thrombectomy if well 6-24h previously if confirmed occlusion of proximal anterior circulation
secondary prevention of stroke
clopidogrel
2nd line - aspirin + MR dipyridamole
carotid artery endarterectomy if stenosis >70%
features of subacute degeneration of spinal cord
dorsal column involvement - vibration and proprioception, tingling/burning/sensory loss
lateral corticospinal tract involvement - muscle weakness, hyperreflexia, spasticity, UMN signs in legs
spinocerebellar tract involvement - sensory ataxia, gait abnormalities, positive rhombergs
features of subdural haemorrhage
head trauma - lucid interval - gradual decline of consciousness
altered mental status
fluctuations in level of consciousness
focal neurological deficits - weakness, aphasia, visual field defects etc
headache - localised, worsening
seizures - if acute/expanding
papilloedema, pupil changes, gait abnormalities, hemiparesis
behavioural/cognitive changes - memory loss, personality changes, cognitive impairment
N/V, drowniness, raised ICP (cushings triad)
classification of subdural haemorrhage
collection of blood deep to the dural layer of the meninges
extra-axial
can be unilateral/bilateral
acute (<48h)
subacute - days to weeks post injury
chronic - weeks to months, elderly
causes of chronic subdural haematoma
Rupture of the small bridging veins within the subdural space rupture and cause slow bleeding
Elderly and alcoholic patients are particularly at risk of subdural haematomas since they have brain atrophy and therefore fragile or taut bridging veins.
crescentic in shape, not restricted by suture lines and compress the brain (‘mass effect’)
hypodense (dark) on CT
causes of syringomyelia
collection of CSF in spinal cord
Causes include:
a Chiari malformation: strong association
trauma
tumours
idiopathic
syringobulbia - fluid filled cavity in medulla of brainstem
features of syringomyelia
cape-like loss of sensation to temperature
(preservation of light touch, proprioception and vibration)
spastic weakness (predominantly of the lower limbs)
neuropathic pain
upgoing plantars
autonomic features
treat cause of syrinx/if persistent, place shunt
features of third nerve palsy
eye is deviated ‘down and out’
ptosis
pupil may be dilated (sometimes called a ‘surgical’ third nerve palsy)
causes of third nerve palsy
diabetes mellitus
vasculitis e.g. temporal arteritis, SLE
false localizing sign* due to uncal herniation through tentorium if raised ICP
posterior communicating artery aneurysm
pupil dilated
often associated pain
cavernous sinus thrombosis
Weber’s syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes
other possible causes: amyloid, multiple sclerosis
features of thoracic outlet syndorme
disorder involving compression of brachial plexus, subclavian artery or vein at the site of the thoracic outlet - can be neurogenic or vascular
neurogenic
painless muscle wasting of hands
hand weakness
numbness, tingling
vascular
subclavian vein compression - painful diffuse arm swelling with distended veins
subclavian artery compression - painful arm claudication
causes of thoracic outlet syndrome
neck trauma - single acute incident or repeated stresses
anatomical anomalies can either be in the form of soft tissue (70%) or osseous structures (30%) - presence of cervical rib
management of thoracic outlet syndrome
education, rehabilitation, physiotherapy, or taping
surgical decompression
definition of transient ischaemic attack
a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction. REF
management of TIA
aspiring 300mg immediately unless bleeding RF
follow on with antiplatelet therapy - clopidogrel (or aspirin + dypyridamole)
lipid modification
carotid artery endarterectomy - if stroke/TIA in carotids and not severely disabled
specialise review - if multiple, if <7 days rv <24h, if >7days rv <7days
types of tremor
postural
resting
intention
action
features of essential tremor
Postural tremor: worse if arms outstretched
Improved by alcohol and rest
Titubation
Often strong family history
definition of trigeminal neuralgia
pain syndrome characterised by severe unilateral pain - usually idiopathic, may be due to trigeminal roots by tumours/vascular problems
features of trigeminal neuralgia
brief electric shock pains
abrupt onset/termination in divisions of trigeminal nerve
evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth
small areas in nasolabial folds/chin more susceptible to pain precipitation
treatment of trigeminal neuralgia
carbamazepine is first-line
poor response or atypical features (e.g., <50y) - refer to neurology
features of tuberous sclerosis
genetic condition of autosomal dominant inheritance
neurocutaneous symptoms - depigmented ash leaf spots, shagreen rough patches over spine, angiofibromas on nose, subungual fibromata
neurological features - developmental delay, epilepsy, intellectual impairment
features of ulnar nerve damage
claw hand
wasting and paralysis of intrinsic hand muscles and hypothenar muscles
sensory loss to medial fingers
radial deviation of wrist
features of vestibular schwannoma
account for 90% of cerebellopontine angle tumours
vertigo, hearing loss, tinnitus, absent corneal reflex
cranial nerves affected:
VIII - vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
V - absent corneal reflex
VII - facial palsy
management of vestibular schwannoma
urgent ENT referral
tumors usually slow-growing, benign
MRI cerebellopontine angle
audiometry
pathophysiology of homonymous hemianopias
incongruous defects: lesion of optic tract
congruous defects: lesion of optic radiation or occipital cortex
macula sparing: lesion of occipital cortex
pathophysiology of homonymous quadrantanopias
superior: lesion of the inferior optic radiations in the temporal lobe (Meyer’s loop)
inferior: lesion of the superior optic radiations in the parietal lobe
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
pathophysiology of bitemporal hemianopia
lesion of optic chiasm
upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour
lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
features of von hippel lindau syndrome
AD condition predisposing to neoplasia
cerebellar haemangiomas: these can cause subarachnoid haemorrhages
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
clear-cell renal cell carcinoma
features of wernicke’s encephalopathy
oculomotor dysfunction
nystagmus (the most common ocular sign)
ophthalmoplegia: lateral rectus palsy, conjugate gaze palsy
gait ataxia
encephalopathy: confusion, disorientation, indifference, and inattentiveness
peripheral sensory neuropathy
management of wernicke’s encephalopathy
give thiamine
if untreated, may develop into korsakoff syndrome – + antero- and retrograde amnesia and confabulation
