Neurology Flashcards

Question

Why may patients see a return of seizures after 3-4 weeks of treatment?

Answer 1

Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.

Answer 2

Lamotrigine is an antiepileptic used second-line for a variety of generalised and partial seizures. Mechanism of action sodium channel blocker

Answer 3

Adverse effects Stevens-Johnson syndrome

Answer 4

Phenytoin is used in the management of seizures. Mechanism of action binds to sodium channels increasing their refractory period

Answer 5

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.

Answer 6

Acute initially: dizziness, diplopia, nystagmus, slurred speech, ataxia later: confusion, seizures

Answer 7

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

Answer 8

Idiosyncratic fever rashes, including severe reactions such as toxic epidermal necrolysis hepatitis Dupuytren's contracture* aplastic anaemia drug-induced lupus

Answer 9

Teratogenic associated with cleft palate and congenital heart disease

Answer 10

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

Answer 11

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

Answer 12

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.

Answer 13

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.

Answer 14

Rectal diazepam or oromucosal midazolam

Answer 15

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)

Answer 16

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)

Answer 17

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

Answer 18

Phenytoin Carbamazepine Phenobarbital Primodine

Answer 19

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`

Answer 20

Recurrent, non-disabling, bilateral headache, often described as a 'tight-band' Not aggravated by routine activities of daily living

Answer 21

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

Answer 22

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.

Answer 23

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

Answer 24

Typically patient > 60 years old Usually rapid onset (e.g. < 1 month) of unilateral headache Jaw claudication (65%) Tender, palpable temporal artery Raised ESR

Answer 25

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

Answer 26

Management (from 2008 SIGN guidelines) simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches) opioid analgesics should be gradually withdrawn

Answer 27

-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

Answer 28

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)

Answer 29

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

Answer 30

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'

Answer 31

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

Answer 32

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

Answer 33

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

Answer 34

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

Answer 35

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

Answer 36

Migraine and hormone replacement therapy (HRT) safe to prescribe HRT for patients with a history of migraine but it may make migraines worse

Answer 37

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.

Answer 38

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

Answer 39

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

Answer 40

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

Answer 41

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

Answer 42

Management carbamazepine is first-line failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology

Answer 43

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.

Answer 44

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.

Answer 45

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

Answer 46

Features headache blurred vision papilloedema (usually present) enlarged blind spot sixth nerve palsy may be present

Answer 47

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

Answer 48

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

Answer 49

Common features headache (may be sudden onset) nausea & vomiting reduced consciousness

Answer 50

Sagittal sinus thrombosis may present with seizures and hemiplegia parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen 'empty delta sign' seen on venography

Answer 51

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

Answer 52

Lateral sinus thrombosis 6th and 7th cranial nerve palsies

Answer 53

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.

Answer 54

Visual optic neuritis: common presenting feature optic atrophy Uhthoff's phenomenon: worsening of vision following rise in body temperature internuclear ophthalmoplegia

Answer 55

Sensory pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion

Answer 56

Motor spastic weakness: most commonly seen in the legs

Answer 57

Cerebellar ataxia: more often seen during an acute relapse than as a presenting symptom tremor

Answer 58

urinary incontinence

Answer 59

sexual dysfunction intellectual deterioration

Answer 60

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)

Answer 61

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)

Answer 62

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

Answer 63

ocrelizumab humanized anti-CD20 monoclonal antibody like natalizumab, it is considered a high-efficacy drug that is often used first-line given intravenously

Answer 64

fingolimod sphingosine 1-phosphate (S1P) receptor modulator prevents lymphocytes from leaving lymph nodes oral formulations are availabl

Answer 65

beta-interferon not considered to be as effective as alternative disease-modifying drugs given subcutaneously/intramuscularly

Answer 66

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

Answer 67

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

Answer 68

Spasticity baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine physiotherapy is important cannabis and botox are undergoing evaluation

Answer 69

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

Answer 70

Oscillopsia (visual fields appear to oscillate) gabapentin is first-line

Answer 71

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

Answer 72

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)

Answer 73

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

Answer 74

Associations thymomas in 15% autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE thymic hyperplasia in 50-70%

Answer 75

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

Answer 76

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

Answer 77

Management of myasthenic crisis plasmapheresis intravenous immunoglobulins

Answer 78

The following drugs may exacerbate myasthenia: penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics: gentamicin, macrolides, quinolones, tetracyclines

Answer 79

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.

Answer 80

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)

Answer 81

EMG incremental response to repetitive electrical stimulation

Answer 82

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

Answer 83

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.

Answer 84

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.

Answer 85

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.

Answer 86

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.

Answer 87

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.

Answer 88

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

Answer 89

Features typical develop after 35 years of age chorea personality changes (e.g. irritability, apathy, depression) and intellectual impairment dystonia saccadic eye movements

Answer 90

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.

Answer 91

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

Answer 92

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

Answer 93

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

Answer 94

Riluzole prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis prolongs life by about 3 months

Answer 95

Respiratory care non-invasive ventilation (usually BIPAP) is used at night studies have shown a survival benefit of around 7 months

Answer 96

Nutrition percutaneous gastrostomy tube (PEG) is the preferred way to support nutrition and has been associated with prolonged survival

Answer 97

Prognosis poor: 50% of patients die within 3 years

Answer 98

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.

Answer 99

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

Answer 100

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

Answer 101

General features myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria

Answer 102

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

Answer 103

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

Answer 104

Café-au-lait spots (>= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris hamatomas (Lisch nodules) in > 90% Scoliosis Pheochromocytomas

Answer 105

Bilateral vestibular schwannomas Multiple intracranial schwannomas, mengiomas and ependymomas

Answer 106

Vestibular schwannomas (sometimes referred to as acoustic neuromas) account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle tumours.

Answer 107

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

Answer 108

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.

Answer 109

Management is with either surgery, radiotherapy or observation.

Answer 110

Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neurocutaneous.

Answer 111

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

Answer 112

Neurological features developmental delay epilepsy (infantile spasms or partial) intellectual impairment

Answer 113

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

Answer 114

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)

Answer 115

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

Answer 116

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.

Answer 117

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

Answer 118

Other features hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate

Answer 119

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

Answer 120

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

Answer 121

Becker muscular dystrophy develops after the age of 10 years intellectual impairment much less common

Answer 122

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

Answer 123

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)

Answer 124

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

Answer 125

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)

Answer 126

- 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

Answer 127

- Concurrent anticoagulation (INR >1.7) - Haemorrhagic diathesis - Active diabetic haemorrhagic retinopathy - Suspected intracardiac thrombus - Major surgery / trauma in the preceding 2 weeks

Answer 128

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)

Answer 129

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)

Answer 130

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

Answer 131

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

Answer 132

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

Answer 133

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

Answer 134

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

Answer 135

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

Answer 136

Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia May still be able to communicate using gestures

Answer 137

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.

Answer 138

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.

Answer 139

Signs of Increased Intracranial Pressure: Such as bradycardia, hypertension, and respiratory irregularities (Cushing's triad).

Answer 140

d: a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.

Answer 141

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

Answer 142

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

Answer 143

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

Answer 144

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

Answer 145

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

Answer 146

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

Answer 147

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

Answer 148

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

Answer 149

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

Answer 150

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

Answer 151

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

Answer 152

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

Answer 153

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

Answer 154

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

Answer 155

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

Answer 156

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.

Answer 157

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.

Answer 158

Consider glycopyrronium bromide to manage drooling of saliva in people with Parkinson's disease.

Answer 159

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).

Answer 160

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

Answer 161

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

Answer 162

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

Answer 163

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

Answer 164

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

Answer 165

MAO-B (Monoamine Oxidase-B) inhibitors e.g. selegiline inhibits the breakdown of dopamine secreted by the dopaminergic neurons

Answer 166

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

Answer 167

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

Answer 168

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)

Answer 169

domperidone

Answer 170

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

Answer 171

Overview aka Steele-Richardson-Olszewski syndrome a 'Parkinson Plus' syndrome

Answer 172

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

Answer 173

Management poor response to L-dopa

Answer 174

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.

Answer 175

Features parkinsonism autonomic disturbance erectile dysfunction: often an early feature postural hypotension atonic bladder cerebellar signs

Answer 176

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.

Answer 177

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

Answer 178

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

Answer 179

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.

Answer 180

Imaging hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement

Answer 181

Management ventriculoperitoneal shunting around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages

Answer 182

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

Answer 183

Investigations decreased red cell transketolase MRI Treatment is with urgent replacement of thiamine

Answer 184

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.

Answer 185

Predominately motor loss Guillain-Barre syndrome porphyria lead poisoning hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth chronic inflammatory demyelinating polyneuropathy (CIDP) diphtheria

Answer 186

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.

Answer 187

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

Answer 188

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

Answer 189

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

Answer 190

Predominately sensory loss diabetes uraemia leprosy alcoholism vitamin B12 deficiency amyloidosis

Answer 191

Vitamin B12 deficiency subacute combined degeneration of spinal cord dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

Answer 192

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

Answer 193

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

Answer 194

spinocerebellar tract involvement sensory ataxia → gait abnormalities positive Romberg's sign

Answer 195

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

Answer 196

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.

Answer 197

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.

Answer 198

Causes include: a Chiari malformation: strong association trauma tumours idiopathic

Answer 199

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

Answer 200

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.

Answer 201

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

Answer 202

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

Answer 203

Features eye is deviated 'down and out' ptosis pupil may be dilated (sometimes called a 'surgical' third nerve palsy)

Answer 204

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

Answer 205

Motor supply (LOAF) Lateral 2 lumbricals Opponens pollicis Abductor pollicis brevis Flexor pollicis brevis

Answer 206

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.

Answer 207

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

Answer 208

Damage at elbow, as above plus: unable to pronate forearm weak wrist flexion ulnar deviation of wrist

Answer 209

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

Answer 210

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

Answer 211

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

Answer 212

Homonymous hemianopia incongruous defects: lesion of optic tract congruous defects: lesion of optic radiation or occipital cortex macula sparing: lesion of occipital cortex

Answer 213

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)

Answer 214

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

Answer 215

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

Answer 216

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

Answer 217

Management early physiotherapy is important neuropathic analgesia in-line with NICE guidelines specialist management (e.g. Pain team) is required

Answer 218

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.

Answer 219

Ankle S1-S2 Knee L3-L4 Biceps C5-C6 Triceps C7-C8

Answer 220

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)

Answer 221

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

Answer 222

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

Answer 223

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)

Answer 224

Management propranolol is first-line primidone is sometimes used

Answer 225

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.

Answer 226

Chemotherapy-induced nausea and vomiting in adults, persisting with optimised conventional anti-emetics - Nabilone, under specialist supervision

Answer 227

Chronic pain - Do not use cannabis-based medicines, but if already initiated continue until appropriate to stop under specialist supervision

Answer 228

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

Answer 229

Severe-treatment resistant epilepsy NICE currently evaluating use of cannabidiol with clobazam for treating seizures associated with Lennox-Gastaut syndrome and Dravet syndrome

Answer 230

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

Answer 231

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%.

Answer 232

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

Answer 233

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

Answer 234

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

Answer 235

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'

Answer 236

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)

Answer 237

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

Answer 238

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

Answer 239

Post-herpetic neuralgia - Amitriptylline Trigeminal neuralgia - Carbamazepine

Answer 240

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)

Answer 241

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

Answer 242

Management simple measures: walking, stretching, massaging affected limbs treat any iron deficiency dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) benzodiazepines gabapentin

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