Neurology Flashcards
Focal seizures
-What were these previously known as?
-where do these stem from in the brain
-how can these types of seizures be classified?
Focal seizures
previously termed partial seizures these start in a specific area, on one side of the brain the level of awareness can vary in focal seizures. The terms focal aware (previously termed 'simple partial'), focal impaired awareness (previously termed 'complex partial') and awareness unknown are used to further describe focal seizures further to this, focal seizures can be classified as being motor (e.g. Jacksonian march), non-motor (e.g. déjà vu, jamais vu; ) or having other features such as aura
Generalised seizures
-where do these stem from in the brain?
-give 5 types?
Generalised
these engage or involve networks on both sides of the brain at the onset
consciousness lost immediately. The level of awareness in the above classification is therefore not needed, as all patients lose consciousness
generalised seizures can be further subdivided into motor (e.g. tonic-clonic) and non-motor (e.g. absence)
specific types include:
tonic-clonic (grand mal)
tonic
clonic
typical absence (petit mal)
atonic
What is a focal to bilateral seizure?
Focal to bilateral seizure
starts on one side of the brain in a specific area before spreading to both lobes previously termed secondary generalized seizures
What is west’s syndrome aka
-What is this characterised by?
-What is seen on EEG?
-what can this be secondary to?
-What are possible treatments?
-What prognosis does this have?
Infantile spasms (West’s syndrome)
brief spasms beginning in the first few months of life
key features:
flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
progressive mental handicap
EEG: hypsarrhythmia
usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic
possible treatments include vigabatrin and steroids
has a poor prognosis
Absence seizures
-When do these onset?
-what are they characterised by?
-What is seen on EEG?
-What are treatment options?
-What prognosis do these have?
Typical (petit mal) absence seizures
onset 4-8 yrs duration few-30 secs; no warning, quick recovery; often many per day EEG: 3Hz generalized, symmetrical sodium valproate, ethosuximide good prognosis: 90-95% become seizure free in adolescence
Lennox-gastaut syndrome
-When do these onset?
-Give 4 clinical features
-What is seen on EEG
-What is the treatment?
Lennox-Gastaut syndrome
may be an extension of infantile spasms
onset 1-5 yrs
features:
atypical absences, falls, jerks
90% moderate-severe mental handicap
EEG: slow spike
treatment: ketogenic diet may help
What is benign rolandic epilepsy?
-Who is this most common in?
-What are the clinical features?
Benign rolandic epilepsy
most common in childhood, more common in males features: paraesthesia (e.g. unilateral face), usually on waking up
Juvenile myoclonic epilepsy
-What is the typical onset?
-What are 4 clinical features
-What is the treatment
Juvenile myoclonic epilepsy (Janz syndrome)
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
Absence seizures:
-Who is this most commonly seen in?
-Who is affected?
Absence seizures (petit mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old and girls are affected twice as commonly as boys
Give 4 clinical features of absence seizures
Features
absences last a few seconds and are associated with a quick recovery seizures may be provoked by hyperventilation or stress the child is usually unaware of the seizure they may occur many times a day
Describe the EEG pattern in absence seizures
EEG: bilateral, symmetrical 3Hz spike and wave pattern
What treatment in given first line for absence seizures?
What is the prognosis?
Management
sodium valproate and ethosuximide are first-line treatment good prognosis - 90-95% become seizure free in adolescence
When are anti-epileptics usually started?
Most neurologists now start antiepileptics following a second epileptic seizure.
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
What treatment is used for generalised tonic-clonic seizures?
-Males
-Females
Generalised tonic-clonic seizures
males: sodium valproate females: lamotrigine or levetiracetam girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line
What is used first vs second line in focal seizures?
first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
What is used first vs second line in absence seizures?
What may exacerbate absence seizures?
Absence seizures (Petit mal)
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
What is used to treat myoclonic seizures?
Myoclonic seizures
males: sodium valproate females: levetiracetam
What is used to treat tonic or atonic seizures?
Tonic or atonic seizures
males: sodium valproate females: lamotrigine
How does sodium valproate work?
Sodium valproate is used in the management of epilepsy and is first-line therapy for generalised seizures. It works by increasing GABA activity.
Sodium valproate
-What can this cause to unborn fetus?
teratogenic
neural tube defects maternal use of sodium valproate is associated with a significant risk of neurodevelopmental delay in children guidance is now clear that sodium valproate should not be used during pregnancy and in women of childbearing age unless clearly necessary. Women of childbearing age should not start treatment without specialist neurological or psychiatric advice.
Give 11 side effects of sodium valproate in addition to teratogenicity?
P450 inhibitor
gastrointestinal: nausea
increased appetite and weight gain
alopecia: regrowth may be curly
ataxia
tremor
hepatotoxicity
pancreatitis
thrombocytopaenia
hyponatraemia
hyperammonemic encephalopathy: L-carnitine may be used as treatment if this develops
What is carbamazepine used for?
Carbamazepine is chemically similar to the tricyclic antidepressant drugs. It is most commonly used in the treatment of epilepsy, particularly partial seizures, where carbamazepine remains a treatment medication. Other uses include
trigeminal neuralgia bipolar disorder
What is the mechanism of action of carbamazepine?
Mechanism of action
binds to sodium channels increases their refractory period
Give 8 adverse effects of carbamazepine?
Adverse effects
P450 enzyme inducer dizziness and ataxia drowsiness headache visual disturbances (especially diplopia) Steven-Johnson syndrome leucopenia and agranulocytosis hyponatraemia secondary to syndrome of inappropriate ADH secretion
Why may patients see a return of seizures after 3-4 weeks of treatment?
Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.
Lamotrigine mechanism of action
Lamotrigine is an antiepileptic used second-line for a variety of generalised and partial seizures.
Mechanism of action
sodium channel blocker
What are the adverse effects of lamotrigine?
Adverse effects
Stevens-Johnson syndrome
What is the mechanism of action of phenytoin?
Phenytoin is used in the management of seizures.
Mechanism of action
binds to sodium channels increasing their refractory period
How can you divide the adverse effects of phenytoin?
Phenytoin is associated with a large number of adverse effects. These may be divided into acute, chronic, idiosyncratic and teratogenic. Phenytoin is also an inducer of the P450 system.
Give 7 acute adverse effects of phenytoin?
Acute
initially: dizziness, diplopia, nystagmus, slurred speech, ataxia later: confusion, seizures
Give 9 chronic adverse effects of phenytoin
Chronic
common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness megaloblastic anaemia (secondary to altered folate metabolism) peripheral neuropathy enhanced vitamin D metabolism causing osteomalacia lymphadenopathy dyskinesia
give 6 idiosyncratic effects of phenytoin
Idiosyncratic
fever rashes, including severe reactions such as toxic epidermal necrolysis hepatitis Dupuytren's contracture* aplastic anaemia drug-induced lupus
Describe why phenytoin is teratogenic?
Teratogenic
associated with cleft palate and congenital heart disease
When should phenytoin levels be taken?
Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if:
adjustment of phenytoin dose
suspected toxicity
detection of non-adherence to the prescribed medication
What are the mechanisms of action of topiramate?
Topiramate was developed as an antiepileptic and is used alone or as adjunctive treatment in generalised tonic-clonic seizures.
There appear to be multiple mechanisms of action:
blocks voltage-gated Na+ channels
increases GABA action
carbonic anhydrase inhibition: this results in a decrease in urinary citrate excretion and formation of alkaline urine that favours the creation of calcium phosphate stone
How can topiramate effect the contraceptives?
Topiramate is an inducer of the P450 enzyme CYP3A4. This may result in hormonal contraception being less effective. As a result, the Faculty of Sexual and Reproductive Health (FSRH) suggests the following for patients taking topiramate:
combined oral contraceptive pill and progestogen-only pill: UKMEC 3 (disadvantages outweigh advantages):
implant: UKMEC 2 (advantages generally outweigh the disadvantages)
The injection (Depo-Provera) and intrauterine system are not affected by topiramate.
Give 6 adverse effects of topiramate?
Adverse effects of topiramate include:
reduced appetite and weight loss
dizziness
paraesthesia
lethargy and poor concentration
rare but important: acute myopia and secondary angle-closure glaucoma
Topiramate is associated with a risk of foetal malformations.
what is the acute management of a prolonged seizure?
Rectal diazepam or oromucosal midazolam
What is the dose of rectal diazepam for:
-Neonate
-Child 1mth to 1 year
-Child 2yrs - 11yrs
-Child 12 yrs - 17 yrs
-Adult
-Elderly
Neonate 1.25 - 2.5 mg
Child 1 month - 1 year 5 mg
Child 2 years - 11 years 5 - 10 mg
Child 12 years - 17 years 10 mg
Adult 10 - 20 mg (max. 30 mg)
Elderly 10 mg (max. 15 mg)
What is the dose of oromucosal midazalam for:
-Neonate
-Child 1-2 mths
-Child 3-11mths
-Child 1-4yrs
-Child 5-9yrs
-Child 10-17 yrs
-Adult
Neonate 300 mcg/kg (unlicensced)
Child 1 - 2 months 300 mcg/kg (max. 2.5mg, unlicensced)
Child 3 - 11 months 2.5 mg
Child 1 - 4 years 5 mg
Child 5 - 9 years 7.5 mg
Child 10 - 17 years 10 mg
Adult 10 mg (unlicensced)
What is status epilepticus defined as?
Status epilepticus is defined as:
a single seizure lasting >5 minutes, or >= 2 seizures within a 5-minute period without the person returning to normal between them
What 4 antiepileptics should be prescribed by brand?
Phenytoin
Carbamazepine
Phenobarbital
Primodine
Describe migraine
Recurrent, severe headache which is usually unilateral and throbbing in nature
May be be associated with aura, nausea and photosensitivity
Aggravated by, or causes avoidance of, routine activities of daily living. Patients often describe ‘going to bed’.
In women may be associated with menstruation`
Describe tension headache
Recurrent, non-disabling, bilateral headache, often described as a ‘tight-band’
Not aggravated by routine activities of daily living
Describe cluster headache
-How often does this occur?
-How long does it last?
-What is this assoc with?
-Who is this more common in?
Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours with clusters typically lasting 4-12 weeks
Intense pain around one eye (recurrent attacks ‘always’ affect same side)
Patient is restless during an attack
Accompanied by redness, lacrimation, lid swelling
More common in men and smokers
Who does cluster headache more commonly affect?
Cluster headaches are more common in men (3:1) and smokers. Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep.
Give 6 features of cluster headaches
Features
-intense sharp, stabbing pain around one eye
pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
the patient is restless and agitated during an attack due to the severity
clusters typically last 4-12 weeks
accompanied by redness, lacrimation, lid swelling
nasal stuffiness
miosis and ptosis in a minority
Describe the headache in 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
Medication overuse headache:
-How often is this present?
-What are suspect medications?
Present for 15 days or more per month
Developed or worsened whilst taking regular symptomatic medication
Patients using opioids and triptans are at most risk
May be psychiatric co-morbidity
What is the treatment for medication overuse headache?
Management (from 2008 SIGN guidelines)
simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches)
opioid analgesics should be gradually withdrawn
Give 3 red flag cause for headache (14)
-compromised immunity, caused, for example, by HIV or immunosuppressive drugs
-age under 20 years and a history of malignancy
-a history of malignancy known to metastasis to the brain
-vomiting without other obvious cause
-worsening headache with fever
-sudden-onset headache reaching maximum intensity within 5 minutes - ‘thunderclap’
-new-onset neurological deficit
-new-onset cognitive dysfunction
-change in personality
-impaired level of consciousness
-recent (typically within the past 3 months) head trauma
headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked), sneeze or exercise
-orthostatic headache (headache that changes with posture)
-symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma
-a substantial change in the characteristics of their headache
Give diagnostic criteria for migraine
A At least 5 attacks fulfilling criteria B-D
B Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated)
C Headache has at least two of the following characteristics:
1. unilateral location
in children, attacks may be shorter-lasting, headache is more commonly bilateral, and gastrointestinal disturbance is more prominent.
2. pulsating quality (i.e., varying with the heartbeat)
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)
D During headache at least one of the following:
1. nausea and/or vomiting*
2. photophobia and phonophobia
E Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)
Give the acute treatment of migraine
Acute treatment
first-line: offer combination therapy with
an oral triptan and an NSAID, or
an oral triptan and paracetamol
for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan
if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide or prochlorperazine and consider adding a non-oral NSAID or triptan
caution should be exercised when prescribing metoclopramide to young patients as acute dystonic reactions may develop
When should prophylaxis be offered for migraine?
prophylaxis should generally be given if ‘Migraine attacks are having a significant impact on quality of life and daily function, for example they occur frequently (more than once a week on average) or are prolonged and severe despite optimal acute treatment’
What can be used for migraine prophylaxis 1st line?
NICE CKS advise one of the following:
propranolol
topiramate: should be avoided in women of childbearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives
amitriptyline
What can be used if 1st line prophylactic treatment for migraine is ineffective?
if measures fail NICE recommends ‘a course of up to 10 sessions of acupuncture over 5-8 weeks’
NICE recommend: ‘Advise people with migraine that riboflavin (400 mg once a day) may be effective in reducing migraine frequency and intensity for some people’
for women with predictable menstrual migraine treatment NICE recommend either frovatriptan (2.5 mg twice a day) or zolmitriptan (2.5 mg twice or three times a day) as a type of ‘mini-prophylaxis’
pizotifen is no longer recommended. Adverse effects such as weight gain & drowsiness are common
What are 2 specialist options for migraine prophylaxis?
treatment options that may be considered by specialists, but fall outside the NICE guidelines:
candesartan: recommended by the British Association for the Study of Headache guidelines
monoclonal antibodies directed against the calcitonin gene-related peptide (CGRP) receptor: examples include erenumab
What can be given for migraine in pregnancy?
Migraine during pregnancy
paracetamol 1g is first-line
NSAIDs can be used second-line in the first and second trimester
avoid aspirin and opioids such as codeine during pregnancy
What treatment can be used for migraine and menstruation?
Migraine and menstruation
many women find that the frequency and severity of migraines increase around the time of menstruation
SIGN recommends that women are treated with mefanamic acid or a combination of aspirin, paracetamol and caffeine. Triptans are also recommended in the acute situation
IS it safe to prescribe HRT with migraine?
Migraine and hormone replacement therapy (HRT)
safe to prescribe HRT for patients with a history of migraine but it may make migraines worse
What are triptans?
Triptans are specific 5-HT1B and 5-HT1D agonists used in the acute treatment of migraine. They are generally used first-line in combination therapy with an NSAID or paracetamol.
When should triptans be taken?
Prescribing points
should be taken as soon as possible after the onset of headache, rather than at onset of aura oral, orodispersible, nasal spray and subcutaneous injections are available
What are the adverse effects and contraindications of triptans?
Adverse effects
'triptan sensations' - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure
Contraindications
patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease
Trigeminal neuralgia
-Describe the pain
-What is the pain evoked by
The International Headache Society defines trigeminal neuralgia as:
a unilateral disorder characterised by brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve the pain is commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas) the pains usually remit for variable periods
Give 7 red flag signs in trigeminal neuralgia
Sensory changes
Deafness or other ear problems
History of skin or oral lesions that could spread perineurally
Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally
Optic neuritis
A family history of multiple sclerosis
Age of onset before 40 years
What is the management of trigeminal neuralgia?
Management
carbamazepine is first-line failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
What is paroxysmal hemicrania?
Paroxysmal hemicrania (PH) is defined by attacks of severe, unilateral headache, usually in the orbital, supraorbital or temporal region. These attacks are often associated with autonomic features, usually last less than 30 minutes and can occur multiple times a day.
How is paroxysmal hemicrainia treated?
PH sits within the group of disorders called trigeminal autonomic cephalgias which also contains cluster headache, a condition which shares many features with PH.
Importantly, PH is completely responsive to treatment with indomethacin.
What is idiopathic intracranial hypertension? what are 4 risk factors?
Idiopathic intracranial hypertension (also known as pseudotumour cerebri and formerly benign intracranial hypertension) is a condition classically seen in young, overweight females.
Risk factors
obesity
female sex
pregnancy
drugs
combined oral contraceptive pill
steroids
tetracyclines
retinoids (isotretinoin, tretinoin) / vitamin A
lithium
What are 5 features of idiopathic intracranial hypertension?
Features
headache blurred vision papilloedema (usually present) enlarged blind spot sixth nerve palsy may be present
Give 6 management options for MS
Management
weight loss
whilst diet and exercise are important, medications such as semaglitide and topiramate may be considered by specialists.
Topiramate is particularly beneficial as it also inhibits carbonic anhydrase
carbonic anhydrase inhibitors e.g. acetazolamide
topiramate is also used, and has the added benefit of causing weight loss in most patients
repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management
surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
Intracranial venous thrombosis
-What can this cause?
-what types do people present with?
Overview
can cause cerebral infarction, much lesson common than arterial causes 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses
What are common features of VST?
Common features
headache (may be sudden onset) nausea & vomiting reduced consciousness
What specific features may be seen in sagittal sinus thrombosis? 3
Sagittal sinus thrombosis
may present with seizures and hemiplegia parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen 'empty delta sign' seen on venography
What specific features may be seen in cavernous sinus thrombosis? 5
Cavernous sinus thrombosis
other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma 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
What specific features may be seen in lateral sinus thrombosis?
Lateral sinus thrombosis
6th and 7th cranial nerve palsies
HOw is MS diagnosed?
Patient’s with multiple sclerosis (MS) may present with non-specific features, for example around 75% of patients have significant lethargy.
Diagnosis can be made on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse.
How can MS affect vision? 4
Visual
optic neuritis: common presenting feature
optic atrophy
Uhthoff’s phenomenon: worsening of vision following rise in body temperature
internuclear ophthalmoplegia
Describe the sensory features that can occur with MS?
Sensory
pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion
Describe the motor features that can occur with MS?
Motor
spastic weakness: most commonly seen in the legs
Describe the cerebellar features that can occur with MS?
Cerebellar
ataxia: more often seen during an acute relapse than as a presenting symptom tremor
How can MS affect urinary system?
urinary incontinence
How can MS affect sexual functions and intellectual functions?
sexual dysfunction
intellectual deterioration
What can be used to treat acute relapses in MS?
High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse. It should be noted that steroids shorten the duration of a relapse and do not alter the degree of recovery (i.e. whether a patient returns to baseline function)
What are the indications for disease modifying drugs in MS?
A number of drugs have been shown to reduce the risk of relapse in patients with MS. Typical indications for disease-modifying drugs include:
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
Natalizumab in MS
-What is this?
-How does this work?
-When is this used and how?
natalizumab
a recombinant monoclonal antibody that antagonises alpha-4 beta-1-integrin found on the surface of leucocytes inhibit migration of leucocytes across the endothelium across the blood-brain barrier generally considered to have the strongest evidence base for preventing relapse of the disease-modifying and hence is often used first-line given intravenously
Ocrelizumab in MS
-What is this?
-When is this given and how?
ocrelizumab
humanized anti-CD20 monoclonal antibody like natalizumab, it is considered a high-efficacy drug that is often used first-line given intravenously
Fingolimod in MS
-What is this?
-how does this work?
-How is thie given?
fingolimod
sphingosine 1-phosphate (S1P) receptor modulator prevents lymphocytes from leaving lymph nodes oral formulations are availabl
Beta-interferon in MS
-How effective is this?
-How is this given?
beta-interferon
not considered to be as effective as alternative disease-modifying drugs given subcutaneously/intramuscularly
Glatiramer acetate
-How does this work?
-How is this given?
-Is this effective?
glatiramer acetate
immunomodulating drug - acts as an 'immune decoy' given subcutaneously along with beta-interferon considered an 'older drug' with less effectiveness compared to monoclonal antibodies and S1P) receptor modulators
What can be used for fatigue in MS?
Fatigue
once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine other options include mindfulness training and CBT
What can be used for spasticity in MS?
Spasticity
baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine physiotherapy is important cannabis and botox are undergoing evaluation
What can be used for bladder dysfunction in MS?
Bladder dysfunction
may take the form of urgency, incontinence, overflow etc guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients if significant residual volume → intermittent self-catheterisation if no significant residual volume → anticholinergics may improve urinary frequency
What can be used for oscillopsia in MS?
Oscillopsia (visual fields appear to oscillate)
gabapentin is first-line
Give 6 good prognostic features of MS?
Good prognosis features
female sex age: young age of onset (i.e. 20s or 30s) relapsing-remitting disease sensory symptoms only long interval between first two relapses complete recovery between relapses
Ways of remembering prognostic features
the typical patient carries a better prognosis than an atypical presentation
What is myaesthenia gravis?
Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases*. Myasthenia is more common in women (2:1)
Describe the key features of myaesthenia gravis? 4
The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest:
extraocular muscle weakness: diplopia proximal muscle weakness: face, neck, limb girdle ptosis dysphagia
What are 3 associations with myaesthenia gravis?
Associations
thymomas in 15% autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE thymic hyperplasia in 50-70%
What are 5 investigations for myaesthenia gravis?
Investigations
single fibre electromyography: high sensitivity (92-100%)
CT thorax to exclude thymoma
CK normal
antibodies to acetylcholine receptors
positive in around 85-90% of patients
n the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies
Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia
What are 3 management options for myaesthenia gravis?
Management
long-acting acetylcholinesterase inhibitors
pyridostigmine is first-line
immunosuppression is usually not started at diagnosis, but the majority of patients eventually require it in addition to long-acting acetylcholinesterase inhibitors:
prednisolone initially
azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy
What is the management of a myaesthenic crisis?
Management of myasthenic crisis
plasmapheresis intravenous immunoglobulins
What 6 drugs may exacerbate myaesthenia gravis?
The following drugs may exacerbate myasthenia:
penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics: gentamicin, macrolides, quinolones, tetracyclines
What is lambert eaton myaesthenic syndrome?
Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.
What are 4 features of lambert eaton syndrome? is ophthalmolegia and ptosis seen?
Features
repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis)
in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease
limb-girdle weakness (affects lower limbs first)
hyporeflexia
autonomic symptoms: dry mouth, impotence, difficulty micturating
ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)
What is seen on EMG in lambert eaton syndrome?
EMG
incremental response to repetitive electrical stimulation
What are 4 management options of lambert eaton syndrome?
Management
treatment of underlying cancer
immunosuppression, for example with prednisolone and/or azathioprine
3,4-diaminopyridine is currently being trialled
works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate
intravenous immunoglobulin therapy and plasma exchange may be beneficial
What is cataplexy?
Cataplexy describes the 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 risk factors for degenerative cervical myelopathy? What is the presentation?
Degenerative cervical myelopathy (DCM) has a number of risk factors, which include smoking due to its effects on the intervertebral discs, genetics and occupation - those exposing patients to high axial loading [1].
The presentation of DCM is very variable. Early symptoms are often subtle and can vary in severity day to day, making the disease difficult to detect initially. However as a progressive condition, worsening, deteriorating or new symptoms should be a warning sign.
What are 5 symptoms of degenerative cervical myelopathy?
DCM symptoms can include any combination of [1]:
Pain (affecting the neck, upper or lower limbs) Loss of motor function (loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance Loss of sensory function causing numbness Loss of autonomic function (urinary or faecal incontinence and/or impotence) - these can occur and do not necessarily suggest cauda equina syndrome in the absence of other hallmarks of that condition Hoffman's sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient's hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick.
What is the investigation for degenerative cervical myelopathy?
An MRI of the cervical spine is the gold standard test where cervical myelopathy is suspected. It may reveal disc degeneration and ligament hypertrophy, with accompanying cord signal change.
What is the management of degenerative cervical myelopathy?
All patients with degenerative cervical myelopathy should be urgently referred for assessment by specialist spinal services (neurosurgery or orthopaedic spinal surgery). This is due to the importance of early treatment. The timing of surgery is important, as any existing spinal cord damage can be permanent. Early treatment (within 6 months of diagnosis) offers the best chance of a full recovery but at present, most patients are presenting too late. In one study, patients averaged over 5 appointments before diagnosis, representing >2 years.
Currently, decompressive surgery is the only effective treatment. It has been shown to prevent disease progression. Close observation is an option for mild stable disease, but anything progressive or more severe requires surgery to prevent further deterioration. Physiotherapy should only be initiated by specialist services, as manipulation can cause more spinal cord damage.
Huntingdon’s disease
-What is this?
-HOw is this inherited?
-what is this due to?
Huntington’s disease is an inherited neurodegenerative condition. It is a progressive and incurable condition that typically results in death 20 years after the initial symptoms develop.
Genetics
autosomal dominant
trinucleotide repeat disorder: repeat expansion of CAG
as Huntington's disease is a trinucleotide repeat disorder, the phenomenon of anticipation may be seen, where the disease is presents at an earlier age in successive generations
results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia
due to defect in huntingtin gene on chromosome 4
What 4 features develop in huntingdon’s and when do they typically develop?
Features typical develop after 35 years of age
chorea personality changes (e.g. irritability, apathy, depression) and intellectual impairment dystonia saccadic eye movements
What is motor neuron disease? how can this present?
-what 3 patterns of disease are recognised?
-When does this usually present?
Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis (ALS), progressive muscular atrophy and bulbar palsy.
What clinical features exist in motor neuron disease? 6
There are a number of clues which point towards a diagnosis of motor neuron disease:
asymmetric limb weakness is the most common presentation of ALS
the mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior is common
fasciculations
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but ‘never’ sensory signs
Motor neuron disease?
-does this affect ocular muscles?
-is there cerebellar signs?
-are abdominal reflexes and sphincters affects?
Other features
doesn't affect external ocular muscles no cerebellar signs abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature
What is the diagnosis of motor neuron disease?
The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a reduced number of action potentials with increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy
Riluzole in MND
-how does this work
-When is this used?
-How effective is this?
Riluzole
prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis prolongs life by about 3 months
Describe respiratory care in MND?
Respiratory care
non-invasive ventilation (usually BIPAP) is used at night studies have shown a survival benefit of around 7 months
Describe nutritional care in MND?
Nutrition
percutaneous gastrostomy tube (PEG) is the preferred way to support nutrition and has been associated with prolonged survival
What is the prognosis of MND?
Prognosis
poor: 50% of patients die within 3 years
Myotonic dystrophy
-What is this?
-What does this affect?
-What are the different types?
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
Myotonic dystrophy
-How is this inherited?
-DM1 - what is this caused by?
-DM2 - what is this caused by?
Genetics
autosomal dominant a trinucleotide repeat disorder DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19 DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
DM1 vs DM2
-Where is weakness seen?
-Is congenital form seen?
DM1
- DMPK gene on chromosome 19
- Distal weakness more prominent
DM2
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
- Severe congenital form not seen
Describe 5 general features of myotonic dystrophy? 5
General features
myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria
Myotonic dystrophy
-What is seen in the muscles?
-how does this affect mental / endocrine/ reproductive / cardiac / oesophgagus?
Other features
myotonia (tonic spasm of muscle) weakness of arms and legs (distal initially) mild mental impairment diabetes mellitus testicular atrophy cardiac involvement: heart block, cardiomyopathy dysphagia
How many types of neurofibromatosis exist? how are they inherited?
Which gene is affected in NF1?
Which gene is affect in NF2?
There are two types of neurofibromatosis, NF1 and NF2. Both are inherited in an autosomal dominant fashion
NF1 is also known as von Recklinghausen’s syndrome. It is caused by a gene mutation on chromosome 17 which encodes neurofibromin and affects around 1 in 4,000
NF2 is caused by gene mutation on chromosome 22 and affects around 1 in 100,000
What are 6 features of NF1?
Café-au-lait spots (>= 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamatomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas
What are 4 features of NF2?
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, mengiomas and ependymomas
How common are vestibular schwannoma?
Vestibular schwannomas (sometimes referred to as acoustic neuromas) account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle tumours.
what is the classic history of vestibular schwannoma (acoustic neuroma) and what cranial nerves are affected?
The classical history of vestibular schwannoma includes a combination of vertigo, hearing loss, tinnitus and an absent corneal reflex. Features can be predicted by the affected cranial nerves:
cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus cranial nerve V: absent corneal reflex cranial nerve VII: facial palsy
How should patients be managed in primary care with suspected acoustic neuroma? What is the investigation of choice?
Patients with a suspected vestibular schwannoma should be referred urgently to ENT. It should be noted though that the tumours are often slow growing, benign and often observed initially.
MRI of the cerebellopontine angle is the investigation of choice. Audiometry is also important as only 5% of patients will have a normal audiogram.
What is the management of acoustic neuroma?
Management is with either surgery, radiotherapy or observation.
What is tuberous sclerosis? How is this inherited?
Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neurocutaneous.
Describe the cutaneous features of tuberous sclerosis? 5
Cutaneous features
depigmented 'ash-leaf' spots which fluoresce under UV light roughened patches of skin over lumbar spine (Shagreen patches) adenoma sebaceum (angiofibromas): butterfly distribution over nose fibromata beneath nails (subungual fibromata) café-au-lait spots* may be seen
Describe the neurological features of tuberous sclerosis? 3
Neurological features
developmental delay epilepsy (infantile spasms or partial) intellectual impairment
How does tuberous sclerosis affect:
-retina
-heart
-brain
-kidneys
-lungs
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
lymphangioleiomyomatosis: multiple lung cysts
what is ataxia telangiectasia? what is this caused by? How is this inherited?
Ataxia telangiectasia is an autosomal recessive disorder caused by a defect in the ATM gene which encodes for DNA repair enzymes. It is one of the inherited combined immunodeficiency disorders. It typically presents in early childhood with abnormal movements. (age 1-5)
Give 4 features of ataxic telangiectasia
Features
cerebellar ataxia telangiectasia (spider angiomas) IgA deficiency resulting in recurrent chest infections 10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
What is friedrich’s ataxia? how is this inherited?
Friedreich’s ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich’s ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.
What is the typical age of onset of friedrichs ataxia? what are 4 neurological features?
The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.
Neurological features
absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration
How does friedrichs ataxi affect:
-heart
-endocrine
-palate
Other features
hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate
Dystrophinopathies
-How are these inherited?
-what is this due to?
-what is dystrophin?
-What happens in duchenne muscular dystrophy?
-What happens in becker muscular dystrophy?
X-linked recessive
due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21
dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleton
in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form
in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form
Duchenne muscular dystrophy, give 4 features
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
Give 2 features of becker muscular dystrophy
Becker muscular dystrophy
develops after the age of 10 years intellectual impairment much less common
Describe the rosier score used in stroke assessment
Assessment Scoring
Loss of consciousness or syncope - 1 point
Seizure activity - 1 point
New, acute onset of:
* asymmetric facial weakness + 1 point
* asymmetric arm weakness + 1 point
* asymmetric leg weakness + 1 point
* speech disturbance + 1 point
* visual field defect + 1 point
What is seen on CT in acute ischaemic stroke vs acute haemorrhagic storke
acute ischaemic strokes
may show areas of low density in the grey and white matter of the territory. These changes may take time to develop other signs include the 'hyperdense artery' sign corresponding with the responsible arterial clot - this tends to visible immediately
acute haemorrhagic strokes
typically show areas of hyperdense material (blood) surrounded by low density (oedema)
Stroke management
-When is aspirin given
-When is anticoagulation given
-when is a statin given?
aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excluded
with regards to atrial fibrillation, the RCP state: ‘anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke’
if the cholesterol is > 3.5 mmol/l patients should be commenced on a statin. Many physicians will delay treatment until after at least 48 hours due to the risk of haemorrhagic transformation
when is thrombolysis for stroke considered?
Thrombolysis with alteplase should only be given if:
it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial) haemorrhage has been definitively excluded (i.e. Imaging has been performed)
give 11 absolute contraindicates to thrombolysis?
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Pregnancy
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
Give 4 relative contraindications to thrombolysis for stroke?
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
What functional status is required to consider thrombectomy? What score is needed on NIHSS?
NICE recommend a pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)
When should you Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours)?
Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours), to people who have:
acute ischaemic stroke and
confirmed occlusion of the proximal anterior circulation demonstrated by computed tomographic angiography (CTA) or magnetic resonance angiography (MRA)
When should you Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes)
Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes):
confirmed occlusion of the proximal anterior circulation demonstrated by CTA or MRA and if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
When should you Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes)?
Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes):
who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
When should carotid endarterectomy be considered?
With regards to carotid artery endarterectomy:
recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis > 70% according ECST** criteria or > 50% according to NASCET*** criteria
Describe wernickes aphasia? Is comprehension affected?
Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA
This area ‘forms’ the speech before ‘sending it’ to Broca’s area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent - ‘word salad’
Comprehension is impaired
Describe brocas aphasia? Is comprehension affected?
Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA
Speech is non-fluent, laboured, and halting. Repetition is impaired
Comprehension is normal
What is comprehension aphasia? is comprehension affected?
Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke’s and Broca’s area
Speech is fluent but repetition is poor. Aware of the errors they are making
Comprehension is normal
What is global aphasia?
Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia
May still be able to communicate using gestures
How can subdural haematomas be classified?
Subdural haematomas can be classified in terms of their age:
Acute: Symptoms usually develop within 48 hours of injury, characterised by rapid neurological deterioration Subacute: Symptoms manifest within days to weeks post-injury, with a more gradual progression. Chronic: Common in the elderly, developing over weeks to months. Patients may not recall a specific head injury.
what can be seen on physical examination in subdural haematoma?
Physical Examination Findings:
Papilloedema: Indicates raised intracranial pressure. Pupil Changes: Unilateral dilated pupil, especially on the side of the haematoma, indicating compression of the third cranial nerve. Gait Abnormalities: Including ataxia or weakness in one leg. Hemiparesis or Hemiplegia: Reflecting the mass effect and midline shift.
What is cushings triad?
Signs of Increased Intracranial Pressure: Such as bradycardia, hypertension, and respiratory irregularities (Cushing’s triad).
What is a TIA?
d: a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.
What is the immediate treatment for TIA?
Immediate antithrombotic therapy: NICE
give aspirin 300 mg immediately, unless:
1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage)
2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
3. Aspirin is contraindicated: discuss management urgently with the specialist team
how fast should TIA get specialist review?
Specialist review
if the patient has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis:
discuss the need for admission or observation urgently with a stroke specialist
If the patient has had a suspected TIA in the last 7 days:
arrange urgent assessment (within 24 hours) by a specialist stroke physician
if the patient has had a suspected TIA which occurred more than a week previously:
refer for specialist assessment as soon as possible within 7 days
What imaging is indicated for TIA?
Neuroimaging NICE
NICE recommend that CT brains should not be done 'unless there is clinical suspicion of an alternative diagnosis that CT could detect'
MRI (including diffusion-weighted and blood-sensitive sequences) is preferred to determine the territory of ischaemia, or to detect haemorrhage or alternative pathologies
it should be done on the same day as the specialist assessment if possible
Carotid imaging
atherosclerosis in the carotid artery may be a source of emboli in some patients all patients should therefore have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy
Give 5 features of bells palsy?
Features
lower motor neuron facial nerve palsy → forehead affected
in contrast, an upper motor neuron lesion 'spares' the upper face
patients may also notice
post-auricular pain (may precede paralysis)
altered taste
dry eyes
hyperacusis
What is the treatment of bell’s palsy?
in the past a variety of treatment options have been proposed including no treatment, prednisolone only and a combination of antivirals and prednisolone
there is consensus that all patients should receive oral prednisolone within 72 hours of onset of Bell’s palsy
there is an ongoing debate as to the value of adding in antiviral medications CKS
NICE Clinical Knowledge Summaries state: ‘Antiviral treatments alone are not recommended. Antiviral treatment in combination with a corticosteroid may be of small benefit, but seek specialist advice if this is being considered.’
UpToDate recommends the addition of antivirals for severe facial palsy
eye care is important to prevent exposure keratopathy
prescription of artificial tears and eye lubricants should be considered
If they are unable to close the eye at bedtime, they should tape it closed using microporous tape
Describe the follow up in bells palsy
Follow-up
if the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT a referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months
what is the prognosis of bells palsy
Prognosis
most people with Bell's palsy make a full recovery within 3-4 months if untreated around 15% of patients have permanent moderate to severe weakness
What is thoracic outlet syndrome? who does this usually affect and when?
Thoracic outlet syndrome (TOS) is a disorder involving compression of brachial plexus, subclavian artery or vein at the site of the thoracic outlet. TOS can be neurogenic or vascular; the former accounts for 90% of the cases.
Epidemiology
given the lack of widely agreed diagnostic criteria, the epidemiology of TOS is not well documented patients are typically young thin women possessing long neck and drooping shoulders peak onset occurs in the 4th decade
What causes thoracic outlet syndrome?
Aetiology
TOS develops when neck trauma occurs to individuals with anatomical predispositions neck trauma can either be a single acute incident or repeated stresses anatomical anomalies can either be in the form of soft tissue (70%) or osseous structures (30%) a well-known osseous anomaly is the presence of cervical rib examples of soft tissue causes are scalene muscle hypertrophy and anomalous bands there is usually a history of neck trauma preceding TOS
What is the clinical presentation of neurogenic TOS? 3
Clinical presentation of neurogenic TOS
painless muscle wasting of hand muscles, with patients complaining of hand weakness e.g. grasping sensory symptoms such as numbness and tingling may be present if autonomic nerves are involved, the patient may experience cold hands, blanching or swelling
What is the clinical presentations of vascular TOS?
Clinical presentation of vascular TOS:
subclavian vein compression leads to painful diffuse arm swelling with distended veins subclavian artery compression leads to painful arm claudication and in severe cases, ulceration and gangrene
what are 4 investigations of TOS?
Investigations:
chest and cervical spine plain radiographs to check for any obvious osseous abnormalities e.g. cervical ribs, exclude malignant tumours or other differentials e.g. cervical spine degenerative changes
other imaging modalities may be helpful e.g. CT or MRI to rule out cervical root lesions
venography or angiography may be helpful in vascular TOS
an anterior scalene block may be used to confirm neurogenic TOS and check the likelihood of successful surgical treatment
give 3 treatment options of TOS
Treatment:
there is a limited evidence base conservative management with education, rehabilitation, physiotherapy, or taping is typically the first-line management for neurogenic TOS surgical decompression is warranted where conservative management has failed especially if there is a physical anomaly. Early intervention may prevent brachial plexus degeneration in vascular TOS, surgical treatment may be preferred other therapies being investigated include botox injection
What is parkinson’s disease? What is the classical triad?
arkinson’s disease is a 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. The symptoms of Parkinson’s disease are characteristically asymmetrical.
Epidemiology
around twice as common in men mean age of diagnosis is 65 years
Describe first line treatment in parkinsons disease?
For first-line treatment:
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 do you do if a patient still has problems after starting first line meds in parkinsons?
If a patient continues to have symptoms despite optimal levodopa treatment or has developed dyskinesia then NICE recommend the addition of a dopamine agonist, MAO‑B inhibitor or catechol‑O‑methyl transferase (COMT) inhibitor as an adjunct.
what can be used for orthostatic hypotension in parkinsons?
If orthostatic hypotension develops then a medication review looking at potential causes should be done. If symptoms persist then midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance) can be considered.
What can be considered for drooling in parkinsons?
Consider glycopyrronium bromide to manage drooling of saliva in people with Parkinson’s disease.
What can be given for parkinson’s disease dementia?
Of the acetylcholinesterase inhibitors (donepezil, rivastigmine and galantamine) used to treat cognitive symptoms in mild to moderate Alzheimers dementia, only rivastigmine is currently licenced for use in Parkinsons related dementia (mild to moderate).
what is levodopa almost always given with?
Levodopa
nearly always combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide)
this prevents the peripheral metabolism of levodopa to dopamine outside of the brain and hence can reduce side effects
what are 5 adverse effects of levodopa? What can be difficult to achieve with levodopa and what can this lead to?
common adverse effects:
dry mouth anorexia palpitations postural hypotension psychosis
some adverse effects are due to the difficulty in achieving a steady dose of levodopa
end-of-dose wearing off: symptoms often worsen towards the end of dosage interval. This results in a decline of motor activity 'on-off' phenomenon: large variations in motor performance, with normal function during the ‘on’ period, and weakness and restricted mobility during the ‘off’ period dyskinesias at peak dose: dystonia, chorea and athetosis (involuntary writhing movements) these effects may worsen over time with - clinicians therefore may limit doses until necessary
What is important not to do in giving levodopa?
it is important not to acutely stop levodopa, for example, if a patient is admitted to hospital
if a patient with Parkinson's disease cannot take levodopa orally, they can be given a dopamine agonist patch as rescue medication to prevent acute dystonia
dopamine receptor agonists:
-give some examples
-what is needed prior to treatment
Dopamine receptor agonists
e.g. bromocriptine, ropinirole, cabergoline, apomorphine ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored
What are the adverse effects of dopamine receptor agonists? 5
patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence
more likely than levodopa to cause hallucinations in older patients. Nasal congestion and postural hypotension are also seen in some patients
MAO-B inhibitors
-what do these do and give an example
MAO-B (Monoamine Oxidase-B) inhibitors
e.g. selegiline inhibits the breakdown of dopamine secreted by the dopaminergic neurons
Amantadine
-how does his work?
-What are the side effects? 5
Amantadine
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
COMT inhibitors
-Give example
-When is this used?
COMT (Catechol-O-Methyl Transferase) inhibitors
e.g. entacapone, tolcapone COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy used in conjunction with levodopa in patients with established PD
Antimuscarinics in parkinsons
-How do these work?
-what are these used for?
-what symptoms do these help with?
-Give some examples?
Antimuscarinics
block cholinergic receptors now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease help tremor and rigidity e.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)
What can be given for nausea in parkinsons disease
domperidone
Given some causes of parkinsonism 8
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
Progressive supranuclear palsy:
-What is this AKA
Overview
aka Steele-Richardson-Olszewski syndrome a 'Parkinson Plus' syndrome
Give 4 features of progressive supranuclear palsy
Features
postural instability and falls
patients tend to have a 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 is prominent
cognitive impairment
primarily frontal lobe dysfunction
What is the response of L-dopa to progressive supranuclear palsy?
Management
poor response to L-dopa
How many types multiple system atrophy exist?
There are 2 predominant types of multiple system atrophy
1) MSA-P - Predominant Parkinsonian features 2) MSA-C - Predominant Cerebellar features
Shy-Drager syndrome is a type of multiple system atrophy.
Give 5 features of multiple system atrophy?
Features
parkinsonism
autonomic disturbance
erectile dysfunction: often an early feature
postural hypotension
atonic bladder
cerebellar signs
What can cause neuroleptic malignant syndrome?
Neuroleptic malignant syndrome is a rare but dangerous condition seen in patients taking antipsychotic medication. It carries a mortality of up to 10% and can also occur with atypical antipsychotics. It may also occur with dopaminergic drugs (such as levodopa) for Parkinson’s disease, usually when the drug is suddenly stopped or the dose reduced.
When does neuroleptic malignant syndrome occur?
-what are 4 features of neuroleptic malignant syndrome?
-what 3 things may be seen on bloods tests?
It occurs within hours to days of starting an antipsychotic (antipsychotics are also known as neuroleptics, hence the name) and the typical features are:
pyrexia muscle rigidity autonomic lability: typical features include hypertension, tachycardia and tachypnoea agitated delirium with confusion
A raised creatine kinase is present in most cases. Acute kidney injury (secondary to rhabdomyolysis) may develop in severe cases. A leukocytosis may also be seen
what are 5 management options of neuroleptic malignant syndrome?
Management
stop antipsychotic
patients should be transferred to a medical ward if they are on a psychiatric ward and often they are nursed in intensive care units
IV fluids to prevent renal failure
dantrolene may be useful in selected cases
thought to work by decreasing excitation-contraction coupling in skeletal muscle by binding to the ryanodine receptor, and decreasing the release of calcium from the sarcoplasmic reticulum
bromocriptine, dopamine agonist, may also be used
what is normal pressure hydrocephaluse? what is the classic triad?
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis.
A classical triad of features is seen
urinary incontinence dementia and bradyphrenia gait abnormality (may be similar to Parkinson's disease)
It is thought around 60% of patients will have all 3 features at the time of diagnosis. Symptoms typically develop over a few months.
What does imaging show in normal pressure hydrocephalus?
Imaging
hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
What is the management of normal pressure hydrocephalus?
Management
ventriculoperitoneal shunting around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
Wernicke’s encephalopathy
-What is the classic triad?
-What are other 4 features?
A classic triad of ophthalmoplegia/nystagmus, ataxia and encephalopathy may occur.
In Wernicke’s encephalopathy, petechial haemorrhages occur in a variety of structures in the brain including the mamillary bodies and ventricle walls.
Features
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
What is the investigation and management for wernickes encephalopathy?
Investigations
decreased red cell transketolase MRI
Treatment is with urgent replacement of thiamine
What can develop if wernickes is not treated?
If not treated Korsakoff’s syndrome may develop as well. This is termed Wernicke-Korsakoff syndrome and is characterised by the addition of antero- and retrograde amnesia and confabulation in addition to the above symptoms.
Give 6 peripheral neuropathies that cause predominately motor loss
Predominately motor loss
Guillain-Barre syndrome porphyria lead poisoning hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth chronic inflammatory demyelinating polyneuropathy (CIDP) diphtheria
What is charcot-marie-tooth disease?
Charcot-Marie-Tooth Disease is the most common hereditary peripheral neuropathy. It results in a predominantly motor loss. There is no cure, and management is focused on physical and occupational therapy.
Give 8 features of charcot marie tooth disease?
Features:
There may be a history of frequently sprained ankles Foot drop High-arched feet (pes cavus) Hammer toes Distal muscle weakness Distal muscle atrophy Hyporeflexia Stork leg deformity
What is guillain-barre? describe the pathogenesis
Guillain-Barre syndrome describes an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni)
Pathogenesis
cross-reaction of antibodies with gangliosides in the peripheral nervous system correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated anti-GM1 antibodies in 25% of patients
What is miller-fisher syndrome? What are the symptoms? what antibodies are present?
Miller Fisher syndrome
variant of Guillain-Barre syndrome associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome anti-GQ1b antibodies are present in 90% of cases
Give 6 peripheral neuropathies that cause predominately sensory loss?
Predominately sensory loss
diabetes uraemia leprosy alcoholism vitamin B12 deficiency amyloidosis
What does vitamin B12 deficiency cause? how does this present?
Vitamin B12 deficiency
subacute combined degeneration of spinal cord dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia
Describe the dorsal column involvement in subacute combined degeneration of the spinal cord?
dorsal column involvement
distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms impaired proprioception and vibration sense
Describe the lateral corticospinal tract involvement in subacute combined degeneration of the spinal cord?
lateral corticospinal tract involvement
muscle weakness, hyperreflexia, and spasticity upper motor neuron signs typically develop in the legs first brisk knee reflexes absent ankle jerks extensor plantars
Describe the spinocerebellar tract involvement in subacute combined degeneration of the spinal cord?
spinocerebellar tract involvement
sensory ataxia → gait abnormalities positive Romberg's sign
What causes Absent ankle jerks, extensor plantars, and give examples?
Typically caused by lesion producing both upper motor neuron (extensor plantars) and lower motor neuron (absent ankle jerk) signs
Causes
subacute combined degeneration of the cord motor neuron disease Friedreich's ataxia syringomyelia taboparesis (syphilis) conus medullaris lesion
what is autonomic dysreflexia? what is the management?
This clinical syndrome occurs in patients who have had a spinal cord injury at, or above T6 spinal level. Briefly, afferent signals, most commonly triggered by faecal impaction or urinary retention (but many other triggers have been reported) cause a sympathetic spinal reflex via thoracolumbar outflow. The usual, centrally mediated, parasympathetic response however is prevented by the cord lesion. The result is an unbalanced physiological response, characterised by extreme hypertension, flushing and sweating above the level of the cord lesion, agitation, and in untreated cases severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke.
Management of autonomic dysreflexia involves removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.
What is syringomyelia? what is syringobulbia?
Syringomyelia (‘syrinx’ for short) describes a collection of cerebrospinal fluid within the spinal cord.
Syringobulbia is a similar phenomenon in which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding.
Give 4 causes of syringomyelia?
Causes include:
a Chiari malformation: strong association trauma tumours idiopathic
Give 6 features of syringomyelia
Features
a ‘cape-like’ (neck, shoulders and arms)
loss of sensation to temperature but the preservation of light touch, proprioception and vibration
classic examples are of patients who accidentally burn their hands without realising
this is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected
spastic weakness (predominantly of the lower limbs)
neuropathic pain
upgoing plantars
autonomic features:
Horner’s syndrome due to compression of the sympathetic chain, but this is rare
bowel and bladder dysfunction
scoliosis will occur over a matter of years if the syrinx is not treated
What are the investigations and management of syringomyelia?
Investigations
full spine MRI with contrast to exclude a tumour or tethered cord a brain MRI is also needed to exclude a Chiari malformation
Treatment will be directed at treating the cause of the syrinx. In patients with a persistent or symptomatic syrinx, a shunt into the syrinx can be placed.
Common peroneal nerve lesion
-How can this occur?
-What is the most characteristic features?
-Give 5 other features
The sciatic nerve divides into the tibial and common peroneal nerves. Injury often occurs at the neck of the fibula
The most characteristic feature of a common peroneal nerve lesion is foot drop.
Other features include:
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
What does the fourth nerve supply?
What is found on fourth nerve palsy?
Overview
supplies superior oblique (depresses eye, moves inward)
Features
vertical diplopia
classically noticed when reading a book or going downstairs
subjective tilting of objects (torsional diplopia)
the patient may develop a head tilt, which they may or may not be aware of
when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards
What are the features of third nerve palsy?
Features
eye is deviated 'down and out' ptosis pupil may be dilated (sometimes called a 'surgical' third nerve palsy)
Give 7 causes of third nerve palsy
Causes
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
Give the motor supply of the median nerve in the hand
Motor supply (LOAF)
Lateral 2 lumbricals Opponens pollicis Abductor pollicis brevis Flexor pollicis brevis
Give the sensory supply of the median nerve at the hand
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.
What is found if there is median nerve damage at the wrist?
Damage at wrist
e.g. carpal tunnel syndrome paralysis and wasting of thenar eminence muscles and opponens pollicis (ape hand deformity) sensory loss to palmar aspect of lateral (radial) 2 ½ fingers
What is found if there is median nerve damage at the elbow?
Damage at elbow, as above plus:
unable to pronate forearm weak wrist flexion ulnar deviation of wrist
What is found if there is damage of the anterior interosseous nerve?
Anterior interosseous nerve (branch of median nerve)
leaves just below the elbow results in loss of pronation of forearm and weakness of long flexors of thumb and index finger
Describe the pattern of damage in radial nerve paralysis? What if there is axillary damage?
Patterns of damage
wrist drop sensory loss to small area between the dorsal aspect of the 1st and 2nd metacarpals
Axillary damage
as above paralysis of triceps
Describe the findings in an ulnar nerve palsy where there is damage at the wrist? What if there is damage at the elbow?
Damage at wrist
'claw hand' - hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals) wasting and paralysis of hypothenar muscles sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects)
Damage at elbow
as above (however, ulnar paradox - clawing is more severe in distal lesions) radial deviation of wrist
Visual field defects - homonymous hemianopia
Where is the lesion if the hemianopia is congruous vs incongruous?
Homonymous hemianopia
incongruous defects: lesion of optic tract congruous defects: lesion of optic radiation or occipital cortex macula sparing: lesion of occipital cortex
Visual field defects - Homonymous quadrantanopias
Where is the lesion if this is superior vs inferior?
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)
Visual field defects - bitemporal hemianopia
Where is the lesion if there is an upper quadrant vs a lower quadrant defect?
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
What is complex regional pain syndrome?
-What are the different types?
Complex regional pain syndrome (CRPS) is the modern, umbrella term for a number of conditions such as reflex sympathetic dystrophy and causalgia. It describes a number of neurological and related symptoms which typically occur following surgery or a minor injury. CRPS is 3 times more common in women.
There are two types of CRPS:
type I (most common): there is no demonstrable lesion to a major nerve type II: there is a lesion to a major nerve
Give 6 features of complex regional pain syndrome?
Features
progressive, disproportionate symptoms to the original injury/surgery allodynia temperature and skin colour changes oedema and sweating motor dysfunction the Budapest Diagnostic Criteria are commonly used in the UK
Give 3 management options of complex regional pain syndrome
Management
early physiotherapy is important neuropathic analgesia in-line with NICE guidelines specialist management (e.g. Pain team) is required
What is the treatment for leg cramps?
Leg cramps are common especially over the age of 60. NICE does not recommend quinine as first line treatment due to poor benefit-to-risk ratio. The first line management is self-care measures such as stretching exercises for calves. If leg cramps are not improving, then quinine may be tried for a short period - one should stop if no benefit. Referral to secondary care is indicated if symptoms persist and affect quality of life greatly.
Give the nerves that supply the following reflexes
Ankle
Knee
Biceps
Triceps
Ankle S1-S2
Knee L3-L4
Biceps C5-C6
Triceps C7-C8
Give the afferent and efferent limb of each reflex:
Corneal
Jaw jerk
Gag
Carotid sinus
Pupillary light
Lacrimation
Reflex Afferent limb Efferent limb
Corneal Ophthalmic nerve (V1) Facial nerve (VII)
Jaw jerk Mandibular nerve (V3) Mandibular nerve (V3)
Gag Glossopharyngeal nerve (IX) Vagal nerve (X)
Carotid sinus Glossopharyngeal nerve (IX) Vagal nerve (X)
Pupillary light Optic nerve (II) Oculomotor nerve (III)
Lacrimation Ophthalmic nerve (V1) Facial nerve (VII)
Von hippel-lindau
-What is this?
-Where is the gene abnormality?
-Give 7 clinical features
Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3
Features
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
What are 5-HT3 antagonists?
-Where do they act?
-Give 2 examples?
-Give 2 adverse effects
5-HT3 antagonists are antiemetics used mainly in the management of chemotherapy-related nausea. They mainly act in the chemoreceptor trigger zone area of the medulla oblongata.
Examples
ondansetron
palonosetron
second-generation 5-HT3 antagonist
main advantage is reduced effect on the QT interval
Adverse effects
prolonged QT interval constipation is common
What is essential tremor? What are 3 features>
Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs
Features
postural tremor: worse if arms outstretched improved by alcohol and rest most common cause of titubation (head tremor)
What is the management of essential tremor?
Management
propranolol is first-line primidone is sometimes used
Who can prescribe cannabis-based medicinal productS?
Initial prescriptions of cannabis-based medicinal products must be made by a specialist medical practitioner with experience in the condition being treated. Subsequent prescriptions can be issued by another practitioner under a shared care agreement.
What cannibis-based medicine can be used for chemotherapy-induced nausea and vomiting in adults?
Chemotherapy-induced nausea and vomiting in adults, persisting with optimised conventional anti-emetics - Nabilone, under specialist supervision
What cannibis-based medicine can be used for cchronic pain in adults?
Chronic pain - Do not use cannabis-based medicines, but if already initiated continue until appropriate to stop under specialist supervision
What cannibis-based medicine can be used for Spasticity in adults with multiple sclerosis if other treatments are not effective?
Spasticity in adults with multiple sclerosis if other treatments are not effective 4-week trial of THC:CBD spray, continue if 20% subjective reduction in spasticity symptoms, under specialist supervision
What cannibis-based medicine can be used for Severe-treatment resistant epilepsy ?
Severe-treatment resistant epilepsy NICE currently evaluating use of cannabidiol with clobazam for treating seizures associated with Lennox-Gastaut syndrome and Dravet syndrome
What is reye’s syndrome?
Reye’s syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas. The aetiology of Reye’s syndrome is not fully understood although there is a known association with aspirin use and a viral cause has been postulated
What are 4 features of reye’s syndrome? what is the management?
The peak incidence is 2 years of age, features include:
there may be a history of preceding viral illness encephalopathy: confusion, seizures, cerebral oedema, coma fatty infiltration of the liver, kidneys and pancreas hypoglycaemia
Management is supportive
Although the prognosis has improved over recent years there is still a mortality rate of 15-25%.
Epilepsy and driving
-How long off driving after first unprovoked/isolated seziure
-How long off driving if established epilepsy
-How long off driving if established epilepsy and there is withdrawal of epilepsy medications
Epilepsy/seizures - all patient must not drive and must inform the DVLA
first unprovoked/isolated seizure: 6 months off if there are no relevant structural abnormalities on brain imaging and no definite epileptiform activity on EEG. If these conditions are not met then this is increased to 12 months
for patients with established epilepsy or those with multiple unprovoked seizures:
may qualify for a driving licence if they have been free from any seizure for 12 months
if there have been no seizures for 5 years (with medication if necessary) a ’til 70 licence is usually restored
withdrawawl of epilepsy medication: should not drive whilst anti-epilepsy medication is being withdrawn and for 6 months after the last dose
Syncope and driving
-How long off driving if simple faint
-if single episode, explained and treated
-if single episode, unexplained
-if two or more episodes
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
How long off driving if stroke or TIA? if multiple TIAs?
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
How long off driving after craniotomy for meningioma? How long off after craniotomy for pituitary tumour?
craniotomy e.g. For meningioma: 1 year off driving*
pituitary tumour: craniotomy: 6 months; trans-sphenoidal surgery ‘can drive when there is no debarring residual impairment likely to affect safe driving’
How long off driving if narcolepsy/cataplexy? how long off if chronic neurological disorders?
narcolepsy/cataplexy: cease driving on diagnosis, can restart once ‘satisfactory control of symptoms’
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)
What is neuropathic pain and what can cause this? 4
Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system. It is often difficult to treat and responds poorly to standard analgesia.
Examples include:
diabetic neuropathy post-herpetic neuralgia trigeminal neuralgia prolapsed intervertebral disc
What four drugs are first line for neuropathic pain? What can be used for rescue therapy? What can be trialled for localised neuropathic pain? for people with resistant problems, what may be helpful?
NICE updated their guidance on the management of neuropathic pain in 2013:
first-line treatment*: amitriptyline, duloxetine, gabapentin or pregabalin
if the first-line drug treatment does not work try one of the other 3 drugs
in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added
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)
pain management clinics may be useful in patients with resistant problems
What is given for post-herpetic neuralgia vs trigeminal neuralgia?
Post-herpetic neuralgia - Amitriptylline
Trigeminal neuralgia - Carbamazepine
what is restless legs syndrome? give 3 clinical features?
Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present
Clinical features
uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest paraesthesias e.g. 'crawling' or 'throbbing' sensations movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
What are 5 causes of restless legs syndrome? what investigations can be done?
Causes and associations
there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy
The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anaemia may be appropriate
Give 5 management options of restless legs
Management
simple measures: walking, stretching, massaging affected limbs treat any iron deficiency dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) benzodiazepines gabapentin
