Neurology Flashcards
Mechanism of action of sumatriptan?
5-HT1 agonist
How soon after LP do post-LP headaches usually develop and what is the mechanism?
Usually within 3 days
Worsens when upright
Thought to be due to leakage of CSF from the dura - when withdrawing the stylet a piece of the arachnoid can be pulled through, keeping the dural hole open
What can you treat post-LP headache with if needed?
Blood patch, epidural saline, IV caffeine
What are the symptoms of brachial neuritis?
Acute onset of unilateral severe pain, followed by shoulder and scapular weakness several days later
May be wasting of the arm muscles
What is the treatment for brachial neuritis?
Self-resolves, prognosis is good
What is the pathophysiology of GBS and most common Abs responsible?
Infection causes a production of Abs which attack the bodys nerve myelin (molecular mimicry). This demyelination then causes an acute, inflammatory, ascending polyneuropathy of peripheral nerves.
Anti-GM1 Abs in 25%
What is the most common/other infections that can cause GBS?
Campylobacter jejuni
CMV
Mycoplasma etc
What are the symptoms of GBS?
1-2 weeks post-infection: symmetrical, ascending muscle weakness +/- numbness
Progresses from distal to proximal over 4 weeks
Can get loss of reflexes, develop neuropathic pain and autonomic dysfunction (sweating, tachy etc)
In 20%, resp muscle and facial muscles are affected
What are the investigations in GBS?
LP: increased proteins, WCC normal
Nerve conduction studies: slow nerve conduction (due to lack of myelin), prolonged distal motor latency and increased F wave latency
Spirometry to monitor FVC
Management and prognosis of GBS?
IVIg
Can also do plasma exchange but less common
Monitor respiratory function
15% have ongoing muscle weakness
5% die
Poor prognostic factors associated with GBS?
age > 40 years poor upper extremity muscle strength previous history of a diarrhoeal illness (specifically Campylobacter jejuni) high anti-GM1 antibody titre need for ventilatory support
What is Miller Fisher syndrome? Triad of symptoms? Antibody?
Variant of GBS
Opthalmoplegia, ataxia, areflexia
Usually a descending paralysis
Anti-GQ1b positive in 90%
What is the management of medication-overuse headaches?
simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches)
opioid analgesics should be gradually withdrawn
When does neuroleptic malignant syndrome occur and what are the symptoms?
Occurs within a few days of starting antipsychotic
fever
muscle rigidity
autonomic lability: typically HTN, tachycardia and tachypnoea
Confusion
Investigations (e.g. blood test) and management in neuroleptic malignant syndrome?
Raised CK
Mx: stop drug, IV fluids to prevent renal failure
Can treat with dantrolene or bromocriptine
What is the pathophysiology and symptoms of acoustic neuromas?
- Usually benign SOLs arising from proliferation of Schwann cells around the nerve
- Many are symptomless and only found on autopsy
Unilateral SN hearing loss
Unilateral tinnitis
Vertigo
Absent corneal reflex
CN VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
CN V: absent corneal reflex
CN VII: facial palsy
Investigations and management of acoustic neuromas?
Audiometry
MRI
Mx is with either surgery, radiotherapy or observation
What is Creutzfeldt-Jakob disease (CJD)?
A rapidly progressive neurological condition caused by PRION proteins. These proteins misfold and induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases.
sporadic: accounts for 85% of cases
10-15% of cases are familial
mean age of onset is 65 years
What are the features of Creutzfeldt-Jakob disease (CJD)?
dementia (rapid onset)
behavioural changes
Focal signs
myoclonus
(think of the woman found wandering the streets)
Investigations in CJD?
CSF is usually normal
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
MRI: hyperintense signals in the basal ganglia and thalamus
Which should you replace first, B12 or folate?
B12
What are the symptoms of subacute degeneration of the spinal cord?
UMN + LMN signs
Investigations in MS? Diagnostic criteria?
Visual evoked potential studies - delayed nerve conduction
MRI FLAIR scan - periventricular lesions, >90% show white matter irregularities, see Dawson’s Fingers
CSF - oligoclonal bands of IgG, increased protein
Over 2 CNS lesions disseminated in time and space
What type of MRI scan should be performed to visualise thyroid Abs in thyroid eye disease?
MRI STIR
What are the three main RFs for degenerative cervical myelopathy?
SMOKING
Genetics
Occupation
Symptoms of degenerative cervical myelopathy? Specific sign to look for?
Pain
Numbness
Weakness
Loss of autonomic function (urinary/bowel continence)
HOFFMAN’S SIGN - flicking one fingers causes the other fingers on that hand to flicker
How do you investigate and treat degenerative cervical myelopathy?
Investigation: MRI
Tx: Urgent referred for assessment by specialist spinal service - early surgery gives best outcomes
Which chromosomes code for neurofibromatosis T1 and T2?
T1 = chr 17 T2 = chr 22
Symptoms of neurofibromatosis T1?
Café-au-lait spots (>= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris hamatomas (Lisch nodules) in > 90% Scoliosis Pheochromocytomas
CLAPPS
Symptoms of neurofibromatosis T2?
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, mengiomas and ependymomas
What triad of symptoms is seen in sturge weber syndrome?
Port-wine stain on face
Brain abnormality called a leptomeningeal angioma
Glaucoma
In webs, there are lots of GAPs
What is the inheritence pattern and what are the features of an essential tremor?
AD
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
Management of an essential tremor?
propranolol is first-line
primidone is sometimes used (e.g. in asthmatics)
What type of Ca is Lambert-Eaton myasthenic syndrome associated with?
SCLC
What are anti- Hu/Yo/GAD/Ri associated with?
Anti-Hu associated with SCLC and neuroblastomas sensory neuropathy - may be painful cerebellar syndrome encephalomyelitis
Anti-Yo
associated with ovarian and breast cancer
cerebellar syndrome
Anti-GAD antibody
associated with breast, colorectal and SCLC (BSc)
stiff person’s syndrome or diffuse hypertonia
Anti-Ri
associated with breast and SCLC
ocular opsoclonus-myoclonus -> dancing eye syndrome
What type of anaemia is seen with anti-epileptics?
Folate deficiency anaemia
What is the pathophysiology of Parkinson’s disease?
Progressive degeneration of dopaminergic neutrons in the substantiated nigra pars compacts of the basal ganglia
Lewy bodies develop -> eosinophilic cytoplasmic inclusions of ubiquitin and alpha-synuclein
Loss of dopamine and melanin in the striatum
Symptoms of Parkinson’s disease?
Asymmetrical
Rigidity
Bradykinesia (cogwheel - due to superimposed tremor)
Resting tremor (pill-rolling, 3-5Hz, worse when stressed/tired)
Other: mask-like facies flexed posture micrographia drooling of saliva psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis REM sleep behaviour disorder postural hypotension
What are the features of drug-induced parkinsonism?
Slightly different features to Parkinson’s disease:
motor symptoms are generally rapid onset and BILATERAL
rigidity and rest tremor are uncommon
Main differentiating factor = ASYMMETRY of symptoms in idiopathic Parkinson’s
If difficult to differentiate between essential tremor and Parkinson’s, what can you use?
single photon emission CT (SPECT)
SEs and limitations of levodopa?
N&V, palpitations, hypotension, dry mouth
Induces dyskinesia (involuntary muscle movements)
On-off effect: fluctuations in motor performance
Reduced efficacy over time
Which medications should you start with in Parkinson’s?
if the motor symptoms are affecting the patient’s quality of life: levodopa
if the motor symptoms are not affecting the patient’s quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO‑B) inhibitor
What are the SEs and examples of dopamine agonists?
Ropinirole, pramiprexole
SEs: drowsiness, nausea
HALLUCINATIONS, COMPULSIVE BEHAVIOUR
Note: bromocriptine/carbergoline - may cause pulmonary, retroperitoneal and cardiac fibrosis, therefore need close monitoring
What are examples of MAO-B inhibitors, MOA and SEs?
selegiline, rasagiline
MOA: reduce dopamine breakdown
SEs: postural hypotension, AF
What are examples of COMT inhibitors, MOA and SEs?
Entacapone, tolcapone
Inhibit the enzyme COMT which is involved in the breakdown of dopamine
Tolcapone can cause liver damage
What is the MOA and SEs of Amantadine?
M2 inhibitor
mechanism is not fully understood, probably increases dopamine release and inhibits its uptake at dopaminergic synapses
side-effects include ataxia, slurred speech, confusion, dizziness and livedo reticularis
How do you treat balance, speech and gait disturbances in parkinson’s?
Don’t respond to medication
Need physiotherapy
Which drugs are CI in Parkinson’s?
ANTIPSYCHOTICS
What are the four criteria where you would start anti epileptics after the first seizure in epilepsy?
1) the patient has a neurological deficit
2) brain imaging shows a structural abnormality
3) the EEG shows unequivocal epileptic activity
4) the patient or their family consider the risk of having a further seizure unacceptable
First line medication for T-C/myoclonic/focal/absence seizures?
T-C: Na valproate
Myoclonic: Na valproate
Absence: Na valproate or ethosuxamide
Focal: Carbamazepine or lamotrigine
Where is Broca’s area located?
Inferior frontal gyrus
Where is Wernicke’s area located?
Brodmann area 22 in the superior temporal gyrus
Where is the arcuate fasiculus and what is caused by a problem here?
The connection between Wernicke’s and Broca’s area
Conductive aphasia
Speech is fluent but repetition is poor
NICE treatment steps for neuropathic pain?
first-line treatment: amitriptyline, duloxetine, gabapentin or pregabalin
Try 2 out of 4 as monotherapies before moving on
tramadol may be used as ‘rescue therapy’ for exacerbations of neuropathic pain
topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia)
What is the inheritance pattern, pathophysiology and symptoms of otosclerosis?
Management?
AD, affects young adults
replacement of normal bone by vascular spongy bone causes fixation of the stapes bone at the oval window
conductive hearing loss, tinnitus and positive family history
Mx: hearing aid
stapedectomy
What does CADASIL stand for?
cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
genetic condition due to a mutation in the NOCTH3 gene
Rare cause of multi-infarct dementia
Patient usually present with migraines
What occurs if there are lesions in each part of the brain?
Parietal lobe
sensory inattention
apraxias
Gerstmann’s syndrome (lesion of dominant parietal): alexia (can’t write), acalculia (can’t do maths), finger agnosia (can’t identify fingers) and right-left disorientation
Occipital lobe
cortical blindness
visual agnosia
Temporal lobe
Wernicke’s aphasia
auditory agnosia
prosopagnosia (difficulty recognising faces)
Frontal lobe expressive (Broca's) aphasia: in the inferior frontal gyrus disinhibition perseveration anosmia inability to generate a list
Absolute CI to thrombolysis in stroke?
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected SAH
- Stroke or traumatic brain injury in preceding 3 months
- LP in preceding 7 days
- GI haemorrhage in preceding 3 weeks
- Active bleeding
- Pregnancy
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
Relative CI to thrombolysis in stroke?
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
What pre-stroke functional status is needed for thrombectomy to be considered? And what type of stroke can it be given in?
<3 on the modified Rankin scale
>5 on the National Institutes of Health Stroke Scale (NIHSS)
<6hrs since stroke, offer if: confirmed occlusion of the proximal anterior circulation demonstrated by CT/MR angio
6-24hrs since stroke, offer if: above, plus confirmed evidence on scans of salvageable brain tissue
CONSIDER within 24hrs, if: confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) and confirmed evidence of salvageable brain tissue
Who should be offered a carotid artery endarterectomy?
If patient has suffered stroke/TIA in the carotid territory and are not severely disabled
Only consider if carotid stenosis > 70% according ECST criteria or > 50% according to NASCET criteria
Outside of the immediate CT criteria, what is the criteria for doing a CT within 8hrs?
CT head scan within 8 hours of the head injury - for adults with any of the following RFs who have experienced some LOC/amnesia since the injury:
age 65 years +
any Hx of bleeding or clotting disorders
dangerous mechanism of injury
>30 mins retrograde amnesia of events immediately before the head injury
Which neuropathic drugs are CI in glaucoma and why?
Amitriptyline - antimuscarinic
What drug can be given to manage spasticity in MS and what is its mechanism of action?
Baclofen - acts on GABA AGonist
Can also give gabapentin
After that - diazepam etc
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
What causes normal pressure hydrocephalus and what is the pathophysiology?
SAH, meningitis, head injury
Thought to be due to decreased levels of absorption at the arachnoid villi
What are the three main symptoms of normal pressure hydrocephalus?
Gait disturbance - looks Parkinsonian (d/t distortion of corona radiata)
Dementia (due to distortion of limbic system)
Bladder incontinence
What are the CT/MRI findings of normal pressure hydrocephalus?
Ventriculomegaly without equal sulcal enlargement
Treatment of normal pressure hydrocephalus?
Ventriculoperitoneal shunt -> high rate of complications (10%)
Medical - can try acetazolamide + repeated LPs to decrease pressure
What are the features of progressive suprnuclear palsy?
Parkinson plus syndrome
Early postural instability and falls
Vertical gaze palsy (difficulty looking down)
Parkinsonism (bradykinesia ++, no tremor)
Cognitive decline
What are the features of multiple system atrophy?
Parkinsonism Autonomic features - postural hypotension - bladder dysfunction - erectile dysfunction Cerebellar signs
What are the EEG findings and prognosis in absence seizures?
EEG: 3Hz generalized, symmetrical
good prognosis: 90-95% become seizure free in adolescence
What are the three different findings in juvenile myoclonic epilepsy? Age of symptom onset + treatment?
onset: teens; F:M = 2:1
1. Infrequent GENERALISED seizures, often in morning//following sleep deprivation
2. Daytime ABSCENCES
3. Sudden, shock like MYOCLONIC seizure (these may develop before seizures)
usually good response to sodium valproate
Which anti epileptics can you take whilst breast-feeding?
breast-feeding is acceptable with all antiepileptic drugs, taken in normal doses, with the possible exception of barbiturates
What are the congenital effects of the main anti epileptics?
Na valproate: Neural tube defects
Phenytoin: cleft Palate
carbamazepine: often considered the least teratogenic of the older antiepileptics
lamotrigine: studies suggest the rate of congenital malformations may be low. Dose may need to be increased in pregnancy
Which spinal tracts are affected in subacute combined degeneration of the spinal cord?
dorsal columns (loss of proprioception and vibration) lateral corticospinal tracts (motor weakness)
Which tracts are affected in Freidrich’s ataxia?
Same as SACD of spinal cord
In addition cerebellar ataxia → other features e.g. intention tremor
Which tracts are affected in Brown-Sequard syndrome?
- Lateral corticospinal tract
- Dorsal columns
- Lateral spinothalamic tract
Causes:
Ipsilateral spastic paresis below lesion
Ipsilateral loss of proprioception and vibration sensation
Contralateral loss of pain and temperature sensation
Which tracts are damaged in anterior spinal artery occlusion?
Lateral corticospinal tracts
Lateral spinothalamic tracts
What tracts are damaged in syringomyelia?
Ventral horns
Lateral spinothalamic tract
Treatment of brain abscesses?
Craniotomy with debridement
IV 3rd gen cephalosporins + metronidazole
(remember, may present with focal neurology)
What are the symptoms of facioscapulohumeral muscular dystrophy and at what age does it usually present?
Usually in 20s, AD inheritence
facial muscles are involved first - difficulty closing eyes, smiling etc
weakness of the shoulder + upper arm muscles
‘winging’ shoulder blades
lower limb: hip girdle weakness, foot drop
What is the name of the score and what are the negative points on it in assessment of suspected stroke? Over what number is a stroke ‘likely’?
ROSIER score
-1 point for: seizure activity, LOC
>0 = stroke likely
Should exclude hypoglycaemia first
Part of the brain that is affected to cause a chorea and conditions that cause it?
Basal ganglia, specifically the caudate nucleus
Huntington’s disease, Wilson’s disease, ataxic telangiectasia
SLE, anti-phospholipid syndrome
rheumatic fever: Sydenham’s chorea
pregnancy: chorea gravidarum thyrotoxicosis polycythaemia rubra vera carbon monoxide poisoning cerebrovascular disease
Where do each of the CNs arise from?
CN 1+2 - cerebrum CN 4 - midbrain CN 3 - midbrain-pontine junction CN5 - pons CN6+7+8 - pontine-medulla junction CN 9+10+11+12 - medulla
Which anti epileptic can cause haemorrhagic disease of the newborn and what should you do to prevent this if taken in pregnancy?
It is advised that pregnant women taking phenytoin are given vitamin K in the last month of pregnancy to prevent clotting disorders in the newborn
Treatment of Bell’s palsy?
Prednisolone within 72hrs
Add antiviral - controversial - if severe
If no improvement in 3 weeks -> refer to ENT urgently
Should resolve in 3-4 months
Untreated, 15% have mod-severe permanent damage
What is Hereditary sensorimotor neuropathy (HSMN)? What is the difference between T1 and T2?
Charcot-Marie-Tooth
present with LMN signs in all limbs and reduced sensation (more pronounced distally)
Champagne bottle legs
HSMN type I: primarily due to demyelinating pathology
HSMN type II: primarily due to axonal pathology
What is the pathophysiology and cause of motor neurone disease? What age does it present?
Genetic 10% - SOD1
Primarily sporadic and unknown trigger
Unknown cause - results in UMN and LMN signs due to progressive degeneration of motor neurones in the spine, CNs and within the cortex
Rarely presents before 40
What is the El Escorial Criteria for the diagnosis of ALS and what other symptoms might hint towards ALS?
UMN signs
LMN signs
Evidence of spreading, with the absence of:
- Electrophysiological evidence of other disease
- Neuroimaging evidences of other disease
Also get:
- fasciculations
- lack of sensory symptoms
- wasting of the small muscles of the hand
Management of ALS?
Riluzole - extends life by 3 months, works by suppressing glutamate activity
Non-invasive ventilation (at night) - extends life by 7 months
Baclofen for spasms, PEG tube when needed, MDT approach
What are the 4 different types of motor neurone disease?
Amyotrophic lateral sclerosis - most common, UMN and LMN. Associated with frontotemporal dementia
Primary lateral sclerosis - UMN only
Progressive muscular atrophy - LMN only (best prog)
Progressive bulbar palsy - UMN and LMN of the lower cranial nerves. Early bulbar symptoms (worst prog)
What is autonomic neuropathy and what is affected? What can cause it?
Autonomic neuropathy occurs when the nerves that control involuntary bodily functions are damaged. It can affect blood pressure, temperature control, digestion, bladder function and even sexual function.
- postural hypotension (BP)
- inability to sweat (temp regulation)
- impotence (sexual)
DIABETES, GBS, parkinson’s, infections: HIV, Chagas’ disease, neurosyphilis, drugs: antihypertensives, tricyclics
craniopharyngioma
What causes Lambert-Eaton syndrome?
Ab directed against presynaptic voltage-gated calcium channel in the peripheral nervous system
What are the clinical features of Lambert-Eaton syndrome?
repeated muscle contractions lead to increased muscle strength
limb-girdle weakness (affects lower limbs first)
hyporeflexia
autonomic symptoms: dry mouth, impotence, difficulty micturating
What would you see on EMG in Lambert-Eaton and what is the management?
EMG: incremental response to repetitive electrical stimulation
Tx of underlying cancer
immunosuppression e.g. with prednisolone +/or azathioprine
IVIg/plasma exchange may be helpful
Management of Ramsay Hunt syndrome?
PO corticosteroids and PO aciclovir
What is the pathophysiology, usual demographic and cardinal symptoms of Meniere’s disease?
Affects young to middle-aged adults
Due to too much fluid in the inner ear, causes pressure to build
Can be unilateral or bilateral
Cardinal features:
• Vertigo (lasts mins-hours, the patient is normal between attacks)
• Hearing loss (uni/bilateral, but level fluctuates)
• Tinnitus (usually precedes an attack of vertigo)
• Aural fullness (described as a pressure, fullness or warm feeling in the ear)
• Can also get nystagmus
- Attacks tend to occur in clusters (average 6-11 attacks/yr)
- Likely to vomit during acute attacks
How to manage Meniere’s disease?
Inform DVLA
Medical management – prochlorperazine for acute attacks. For prophylaxis: beta-histamine, gentamicin injections (saw the lady in GP), low salt diet and diuretics (to prevent fluid build-up)
Surgical management – decompressing the inner ear (draining the endolymphatic sac), disconnecting the labyrinth (vestibular neurectomy) or labyrinthectomy (destruction of the labyrinth)
What are the symptoms of Wernicke’s, what to do need to remember to do if the patient is hypoglycaemic and how do you investigate?
Ataxia, confusion, ophthalmoplegia, also NYSTAGMUS
Give thiamine before glucose
Ix: RBC transketolase activity (decreased)
Inheritance pattern of neurofibromatosis, which chr found on and difference between T1 and T2?
AD
T1 - chr 17 (17 letters in neurofibromatosis)
Cafe au last spots - start in 1st yr of life, DO NOT carry risk of skin cancer
Freckling - by 10yrs old
Dermal neurofibromas - small, violet nodules
Nodular neurofibromas - from nerve trunks, can give abnormal sensation when pressed
Lisch nodules - harmless brown/translucent mounds on iris
T2 - chr 22 Cafe au lait spots BILATERAL vestibular schwannomas Meningiomas A form of childhood cataracts can develop
What ophthalmological problem can one of the prophylactic anti-migraine medications cause?
Topiramate - acute open angle glaucoma
What is the pathophysiology and demographic of Myasthenia Gravis?
Abs form against the nicotinic acetylcholine receptors
Depletes the number of ‘working’ post-synaptic receptors
If <50 - more common in women
If >50 - more common in men
What triggers myasthenia gravis?
Pregnancy, infection, emotion, exercise
What are the investigations, Abs and treatments in myasthenia gravis?
Ix: Anti-AChR, MUSK Abs
Neurophsiology
CT - thymus (thymomas in 15%, hyperplasia in 60%)
Mx: Acetylcholinesterase inhibition - Pyridostigmine
Tx relapses with prednisolone +/- azathioprine/Mtx
Thymectomy
Plasma exchange/IVIg in severe exacerbations
Management of myasthenia crisis?
Immunoglobulins, plasma exchange
How to Mx otitis externa?
Ear drops – containing abx + steroids e.g. gentisone-HC contains gentamicin and hydrocortisone
Oral abx (rarely indicated) – flucloxacillin, if pseudomonas is suspected use ciprofloxacin
If fungal infection suspected – use anti-fungal agent e.g. clotrimazole 1% ear drops
Refer for aural toilet if no response
What are the features and management of malignant otitis external? RF?
OE that has spread to cause osteomyelitis of the skull base
RF: diabetes
o Features: Otalgia + HEADACHE (worse than expected), bone in canal, facial nerve palsy occurs in 50% of pt
o CT scan/MRI
o May be life-threatening – requires urgent admission, Tx: IV ciprofloxacin
Which meds should you give in the event of a stroke and AF?
Aspirin
Don’t start anticoagulation until 14 after an ischaemia stroke (risk of haemorrhage transformation)
Symptoms and investigations in SAH?
Symptoms: Thunderclap headache N&V Meningism Coma/seizures
Ix:
CT head (7% show no bleed)
LP at least 12hrs later for xanthochromia (to differentiate from bloody tap)
Causes of a spontaneous SAH?
Berry aneurysms 85% of cases, associated conditions include adult PKD, Ehlers-Danlos syndrome and coarctation of the aorta
AV malformation
Pituitary apoplexy (bleeding into benign pituitary tumour)
Arterial dissection
Mycotic (infective) aneurysms
How to manage SAH?
Coil aneurysm within 24hrs
Consider craniotomy for other causes
Vasospasm is prevented using a 21-day course of nimodipine (a calcium channel inhibitor targeting the brain vasculature)
Complications of SAH?
Re-bleeding - most (c) by 12hrs Vasospasm - usually occurs day 7/8, give nimodipine to prevent Hyponatraemia (commonly d/t SIADH) Seizures Hydrocephalus
Damage to which nerve causes foot drop?
Common peroneal nerve
Symptoms of Bell’s? UorLMN?
LMN
Weakness including forehead
may also be post-auricular pain (may precede paralysis)
Altered taste, dry eyes, hyperacusis (sounds are louder)
What is the most common psych complication in Parkinson’s?
Depression
Symmetrical leg weakness, UMN signs, viral illness/HIV =
transverse myelitis
Neuro and cutaneous changes in tuberous sclerosis?
Cutaneous features
depigmented ‘ash-leaf’ spots which fluoresce under UV light
Shagreen patches: roughened patches of skin over lumbar spine
Angiofibromas: butterfly distribution over nose
Fibromata beneath nails
café-au-lait spots* may be seen
Neurological features
epilepsy (infantile spasms or partial)
intellectual impairment
Difference between hyoscine hydrobromide, hyoscine butylbromide and glycopyrronium bromide?
hyoscine hydrobromide
hyoscine butyl bromide - buscopan
glycopyrronium bromide - decrease resp secretions
How long should you not drive for following a first unprovoked or isolated seizure if brain imaging and EEG normal?
for patients with established epilepsy or those with multiple unprovoked seizures?
withdrawawl of epilepsy medication?
First - 6m
Epilepsy + seizure free - 12m
withdrawawl of epilepsy medication: should not drive whilst anti-epilepsy medication is being withdrawn and for 6 months after the last dose
What is astereognosis and what lobe is responsible?
Parietal
The inability to identify objects by feel alone
Medical Tx of neuroleptic malignant syndrome and severe serotonin syndrome?
NMS - dantrolene
SS - cyproheptadine
DVLA driving rule for seizure/TIA/narcolepsy/cataplexy/craniotomy?
SeIXure = 6 months (1st seizure)
2nd seizure - x2 = 12 months
T1A = 1 month
Multiple TIAs = multiple months = 3 months
CraniotomYEAR = 1 year
NOrcoplexy / CEAtaplexy = No driving / Cease driving (until controlled)
Causes of gingival hyperplasia?
PANIC
phenytoin, AML, natural/normal, icolosporin and ca channel blocker
Deficit if stroke in: anterior cerebral art? Middle cerebral art? posterior cerebral art? Brainstem?
Anterior:
Contralateral hemiparesis and sensory loss
Lower limb > upper limb
Ants have lots of legs
Middle:
Contralateral hemiparesis and sensory loss
Upper limb > lower limb
Monkeys use their arms more
Aphasia (MCA supplies Broca’s and Wernicke’s)
Posterior:
Contralateral homonymous hemianopia with macular sparing
Visual agnosia (can’t interpret visual info)
Prosopagnosia (can’t recognise faces)
Brainstem: wide range of effects
Quadriplegia
Vision disturbances
Basilar artery = locked in syndrome
How does a lacuna stroke present?
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
Causes of cerebellar syndrome?
P - paraneoplastic syndrome
A - ataxic telangectasia
S - stroke (posterior circulation), sclerosis (MS)
T - trauma, thyroid (hypothyroid)
R - Rx (medications) - phenytoin, lithium, valproate, metronidazole
I - infection (HIV, lyme, syphilis)
E - ethanol (wernicke’s encephalopathy or chronic alcohol use)
S - SOL (posterior fossa tumour), spinocerebellar ataxia (friedreich’s ataxia)
Adverse effects and CIs for triptans?
AE: ‘triptan sensations’ - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure
CIs: history of/RFs for IHD or cerebrovascular disease
What is the inheritence pattern, chromosome and symptoms of von Hipple Lindau?
AD Chr 3 (3 letters in VHL)
Kidney:
renal cysts (premalignant)
clear-cell renal cell carcinoma
Brain:
cerebellar haemangiomas: these can cause SAH
retinal haemangiomas: vitreous haemorrhage
Other:
phaeochromocytoma
What is the only cause of an UMN facial palsy?
Stroke
Causes of bilateral facial nerve palsy?
sarcoidosis
GBS
Lyme disease
bilateral acoustic neuromas (as in NF type 2)
What is the anti-NMDA receptor?
a paraneoplastic syndrome, presenting as prominent psychiatric features including agitation, hallucinations, delusions and disordered thinking; seizures, insomnia, dyskinesias and autonomic instability
Linked to ovarian teratomas
What is responsible for pupillary response?
CN 2, CN3
Light hits the retina, travels down CN2 and synapses with CN3 in the Edinger-Westphal nucleus
Then travels down CN3 to cause pupil contraction
Where is the medial longitudinal fasciculus located and what does it do? What can cause this?
In between the midbrain and pons
Connects CN 3, 4 and 6 - therefore damage causes problems with eye movements:
- impaired adduction of the eye on the same side as the lesion
- horizontal nystagmus of the abducting eye on the contralateral side
(i.e. if lesion is on the L, if looks right, the L eye will not fully adduct and the R eye will display nystagmus)
Causes: MS, vascular disease
Drugs that cause Myasthenia Gravis?
P(pp)M supports LGBTQ(q)
P: penicillamine
P: phenytoin
P: procainamide
M: macrolide
L: lithium G: gentamicin B: beta blocker T: tetracycline Q: quinidine Q: quinolone
What is Froin’s syndrome?
coexistence of xanthochromia, high protein level and marked coagulation of CSF. It is caused by meningeal irritation (e.g. during spinal meningitis) and CSF flow blockage by tumour mass or abscess
Phenytoin SEs?
PHENYTOIN
P- Pseudolymphoma
pancytopenia
P450 interaction
H-Hirsutism,Acne
E-Enlarged gums
N-Nystagmus-cerebellar syndrome
Y-Yellow browning of skin
T-teratogenic
O-Osteomalacia
I-Interference with folic acid absorption
Idiosyncracy
N-Neuropathies
What is a pituitary apoplexy and what are the symptoms, Ix and management?
Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction
Symptoms of SAH
bitemporal superior quadrantic defect
Symptoms of hypopituitarism
Extraocular nerve palsys (CN3)
Ix: MRI
Mx: urgent steroid replacement due to loss of ACTH
careful fluid balance
surgery
Cluster headaches RFs, symptoms + length of attacks and Ix?
RF: male, smoking
Triggers: can be alcohol, at night/early morning
Symptoms: pain 1-2x/day, lasting 15m-2hrs
Typically lasts for 1-3months
Intense pain around one eye
accompanied by redness, lacrimation, lid swelling
Miosis +/- ptosis
May be vomiting
Ix: clinical Dx
Management of cluster headaches?
100% 15L O2
S/c sumatriptan
Prevention: verapamil
Avoid alcohol during cluster periods
May be evidence for tapering dose of prednisolone
What are Gelastic seizures?
Laughing seizures
The laugh is the seizure
Lennox Gaustaut syndrome?
Drop attacks, often present in childhood
Which HLA is narcolepsy associated with, what are the symptoms and management?
HLA-DR2
associated with low levels of orexin (hypocretin), a protein which is responsible for controlling appetite and sleep patterns
Symptoms: hypersomnolence (excessive sleepiness) cataplexy sleep paralysis vivid hallucinations on going to sleep or waking up
Mx: daytime stimulants (e.g. modafinil) and nighttime sodium oxybate (CNS depressant)
What is the advice regarding stopping anti-epileptic drugs?
Can be considered if seizure free for > 2 years, with AEDs being stopped over 2-3 months
What will you see on nerve conduction studies with axonal vs demyelinating pathology?
(A)xonal -> reduced (A)mplitude
D)emyelinating -> (D)eceleration (reduced conduction velocity
Symptoms of myotonic dystrophy?
Haggered faces Ptosis, hollow cheeks, cataracts, frontal baldness Distal muscle wasting Cardiac conduction problems MYOTONIA - difficulty relaxing muscles
All the D's distal weakness initially difficulty relaxing muscles autosomal dominant diabetes dysarthria
Types of myotonic dystrophies?
Most common = DM1
CTG trinucleotide repeat in affected gene (DMPK) on Chr 19
DM2 = ZNF9 gene on Chr 3
What is a Chiari malformation and what may result?
Herniation of the cerebellar tonsils through the foramen magnum
Can cause non-communicating hydrocephalus + syringomyelia
What can cause syringomyelia and what are the symptoms?
Chiari malformation, tumour, trauma, no cause identified
‘Cape-like’ loss of temperature/pain sensation (spinothalamic tract)
What is foster-kennedy syndrome?
a frontal lobe tumour leading to ipsilateral optic atrophy and papilloedema of the contralateral optic nerve
Treatment of restless leg syndrome?
Simple measures: walking, stretching, massaging
Tx any iron deficiency
Dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
Benzodiazepines
Gabapentin
What is neuromyelitis optica? Diagnostic criteria?
monophasic or relapsing-remitting demyelinating CNS disorder
Previous thought to be a variant of MS
Typically involves optic nerves and cervical spine, disease triggered by auto-immune NMO-IgG
MRI often normal
Diagnosis is requires bilateral optic neuritis, myelitis (spinal cord inflammation) and 2/3 of:
- Spinal cord lesion involving 3+ spinal levels
- Initially normal MRI brain
- Aquaporin 4 positive serum antibody
What is the trinucleotide repeat in Freidrich’s ataxia and what is unusual about this?
GAA (stumbling around - gone GAA GAA)
Autosomal recessive
No anticipation
Age of onset and symptoms of Freidrich’s ataxia?
Between age 10-15
Neurological features absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration
Other features
HOCM (90%, most common cause of death)
DM (10-20%)
high-arched palate
Which area of the brain does HSV encephalitis usually affect and which strain of HSV is most common?
Temporal lobe, sometimes inferior frontal lobe
HSV-1 in 95%
Symptoms of encephalitis?
fever, headache, vomiting
confusion, behavioural change, seizures
focal features e.g. aphasia
Investigations and treatment in encephalitis?
CSF - moderate increase in protein, lymphocytes
- decrease in glucose
Leucocytosis on bloods
EEG - diffuse abnormal slow waves
CT - some changes in temporal/inferior frontal lobe e.g. petechial haemorrhages
Tx: IV aciclovir, if treatment is started promptly the mortality is 10-20%. Left untreated the mortality approaches 80%
Most common complication of meningitis?
SN hearing loss
When to obtain specialist follow-up for pts with TIA?
If within 7 days - f/u within 24hrs
If >7 days since attack - f/t within 7 days
What medication should all suspected TIA patients receive immediately and what are the exceptions to this?
- the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage)
- the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
- Aspirin is contraindicated: discuss management urgently with the specialist team
Longterm management of stroke?
Clopidogrel
If not tolerated, aspirin and dipyridamole
Treatment of migraines?
Sumatriptan + NSAID/paracetamol +/- antiemetic
Non-oral metoclopramide/prochlorperazine
Prophylaxis of migraines?
Propranolol or topiramate Consider amitriptyline If not working: 10 session of acupuncture Riboflavin daily
Management of trigeminal neuralgia?
carbamazepine is first-line
May need surgical decompression
May remit after 6-12 months spontaneously
failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
Management of status epilepticus?
ABC, 100% O2
Benzodiazepines e.g. diazepam (rectal)/lorazepam (IV)
This may be repeated once after 10-20 minutes
Then, Phenytoin
If no response -> GA
What is Huntington’s disease?
trinucleoutide repeat - expansion of CAG
Chr 4
results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia
Symptoms and management of huntington’s?
psychotic and behavioural symptoms chorea personality changes dystonia saccadic eye movements
Mx: none
Symptom control - benzos etc for chorea
