Neuro

Question 1

What is an essential tremor? What are the features of it?

Answer 1

autosomal dominant condition that affects both upper limbs
postural tremor - worse if arms outstretched
improved by alcohol and rest
moss common cause of titubation (head tremor)

Question 2

Management of an essential tremor

Answer 2

first line - propanolol
primidone sometimes used

Question 3

Causes of tremor

Answer 3

Parkinsonism
essential tremor
anxiety
thyrotoxicosis
hepatic encephalopathy
CO2 retention
cerebellar disease
drug withdrawal

Question 4

What is motor neuron disease?

Answer 4

neuro condition of unknown cause
can present w/ both upper and lower signs
sensory neurones spared
rarely presents before 40yrs various patterns of disease are recognised

Question 5

What are the types of motor neuron disease patterns?

Answer 5

amyotrophic lateral sclerosis
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy

Question 6

Amytrophic lateral sclerosis

Answer 6

50% of pts
typically LMN signs in arms and UMN signs in legs
in familial cases gene responsible lies on C21 and codes for superoxide dismutase

Question 7

Primary lateral sclerosis

Answer 7

UMN signs only

Question 8

Progressive muscular atrophy

Answer 8

LMN signs only
affects distal muscles before proximal
carries best prognosis

Question 9

Progressive bulbar palsy

Answer 9

palsy of tongue, muscles of chewing / swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis

Question 10

What suggests a motor neurone disease diagnosis?

Answer 10

asymmetric limb weakness
mix of UMN and LMN signs
wasting of small hand muscles/tibialis anterior is common
fasciculations
absence of sensory signs / symptoms (vague sensory symptoms may occur early in disease but ‘never’ sensory signs

Question 11

Other features of motor neurone disease

Answer 11

doesn’t affect external ocular muscles
no cerebellar signs
abdo reflexes usually preserved and sphincter dysfunction if present is a late feature

Question 12

motor neurone disease diagnosis

Answer 12

clinical
nerve conduction studies = normal motor conduction, can help exclude neuropathy
Electromyography shows reduced number of action potentials w/ increased amplitude
MRI to exclude differential diagnosis of cervical cord compression and myelopathy

Question 13

Motor neurone disease prognosis

Answer 13

poor - 50% of pts die w/in 3 yrs

Question 14

Motor neurone disease management

Answer 14

riluzole
resp care - non-invasive ventilation (usually BIPAP) at night
nutrition - PEG

Question 15

Riluzole

Answer 15

prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis

Question 16

Signs of lower motor neurone disease

Answer 16

muscle wasting
reduced tone
fasciculations (muscle twitch)
reduced reflexes

Question 17

Signs of upper motor neurone disease

Answer 17

increased tone or spasticity
brisk reflexes
upgoing plantar reflex

Question 18

What is multiple sclerosis?

Answer 18

chronic cell-mediated autoimmune disorder characterised by demyelination in CNS

Question 19

Multiple sclerosis epidemiology

Answer 19

3x more common in women
most commonly diagnosed in ppl 20-40
more common at higher latitudes

Question 20

Multiple sclerosis genetics

Answer 20

monozygotic twin concordance = 30%
dizygotic twin concordance = 2%

Question 21

Multiple sclerosis subtypes

Answer 21

Relapsing-remitting disease = most common, acute attack (1-2 months) followed by periods of remission
Secondary progressive disease = relapsing-remitting pts who have deteriorated and developed neuro signs and symptoms between relapses, gait and bladder disorders
Primary progressive disease = progressive deterioration from onset, more common in older ppl

Question 22

Prevelance of multiple sclerosis types

Answer 22

relapsing-remitting = around 85%
around 65% of relapsing-remitting go on to develop secondary progressive w/in 15yrs
primary progressive = around 10%

Question 23

Sensory and motor features of multiple sclerosis

Answer 23

Sensory = pins n needles, numbness, trigeminal neuralgia, Lhermitte’s syndrome
Motor = spastic weakness - most commonly in legs

Question 24

Visual and cerebellar features of multiple sclerosis

Answer 24

Visual = optic neuritis, optic atrophy, Uhtoff’s phenomenon, internuclear ophthalmoplegia
Cerebellar = more often in an acute relapse than as presenting symptom, tremor

Question 25

Uhthoff's phenomenon

Answer 25

neuro features eg vision are exacerbated when body temp rises, associated w/ multiple sclerosis

Question 26

Lhermitte's syndrome

Answer 26

paraesthesiae in limbs on neck flexion Lhermitte's sign = sudden electric shock feeling that runs down neck into spine

Question 27

Other features of multiple sclerosis

Answer 27

around 75% pts have significant lethargy urinary incontinence sexual dysfunction intellectual deterioration

Question 28

Multiple sclerosis investigations

Answer 28

diagnosis requires demonstration of lesions disseminated in time and space MRI CSF features visual evoked potentials test - delayed, well preserved waveform

Question 29

MRI signs in multiple sclerosis

Answer 29

high signal T2 lesions periventricular plaques Dawson fingers - often on FLAIR images - demyelinating plaques on ventricles or fluid filled spaces

Question 30

CSF in multiple sclerosis

Answer 30

oligoclonal bands (and not in serum) increased intrathecal synthesis of IgG

Question 31

Multiple sclerosis acute relapse management

Answer 31

high dose steroids eg oral or IV methylprednisolone 5 days to shorten length of actual relapse

Question 32

Typical indications for disease-modifying drugs to reduce risk of relapse in patients w/ MS

Answer 32

relapsing-remitting disease and 2 relapses in past 2 yrs and able to walk 100m unaided secondary progressive disease and 2 relapses in past 2 yrs and able to walk 10m

Question 33

Drug options for reducing risk of relapse in MS

Answer 33

natalizumab IV ocrelizumab IV fingolimod beta-interferon glatiramer acetate

Question 34

Natalizumab

Answer 34

recombinant monoclonal antibody that antagonises alpha-4 beta-1 integrin found on surface of leucocytes inhibit migration of leucocytes across endothelium across BBB often used first line for MS

Question 35

Ocrelizumab

Answer 35

humanised anti-CD20 monoclonal antibody like natalizumab, considered high efficacy drug so used first line for MS

Question 36

Fingolimod

Answer 36

sphingosine 1-phosphate (S1P) receptor modulator prevents lymphocytes from leaving lymph nodes

Question 37

Glatiramer acetate

Answer 37

immunomodulating drug - acts as 'immune decoy' along w/ beta-interferon considered 'older drug' w/ less effectiveness compared to monoclonal antibodies and S1P receptor modulators

Question 38

Spasticity treatment

Answer 38

baclofen and gabapentin other options = diazepam, dantrolene and tizanidine physio

Question 39

Bladder dysfunction treatment

Answer 39

US bladder significant residual vol = intermittent self catheterisation no significant residual vol = anticholinergics may improve urinary frequency

Question 40

Oscillipsia treatment

Answer 40

oscillopsia = visual fields appear to oscillate 1st line = gabapentin

Question 41

What is Duchenne muscular dystrophy?

Answer 41

x-linked recessive inherited disorder in dystrophin genes required for normal muscular function

Question 42

Features of Duchenne muscular dystrophy

Answer 42

progressive proximal muscle weakness from 5 yrs calf pseudohypertrophy Gower's sign = child uses arms to stand up from squatted position 30% have intellectual impairment

Question 43

Investigations of Duchenne muscular dystrophy

Answer 43

raised creatinine kinase genetic testing - replaced muscle biopsy

Question 44

Management of Duchenne muscular dystrophy

Answer 44

largely supportive most children can't walk by 12 pts typically survive to around 25-30 associated w/ dilated cardiomyopathy

Question 45

What is myotonic dystrophy?

Answer 45

dystrophia myotonica inherited autosomal dominant myopathy w/ features developing around 20-30 affects skeletal, cardiac and smooth muscle 2 main types = DM1 and DM2

Question 46

DM1 v DM2

Answer 46

DM1 = DMPK gene on C19. Distal weakness more prominent DM2 = ZFN9 gene on C3. Prox. weakness more prominent, severe congenital form not seen

Question 47

Features of myotonic dystrophy

Answer 47

myotonic facies (long, haggard appearance) frontal balding bilateral ptosis cataracts dysarthria myotonia weakness of arms and legs mild mental impairment DM

Question 48

Duchenne muscular dystrophy pathology

Answer 48

x-linked recessive mutation in gene encoding dystrophin on Xp21 dystrophin is part of a large membrane associated protein in muscle which connects muscle membrane to actin, part of muscle cytoskeleton frameshift mutation resulting in 1 or both binding sites lost leading to severe form

Question 49

Becker muscular dystrophy

Answer 49

non-frameshift insertion in dystrophin gene resulting in both binding sites being preserved to milder form develops after age of 10 intellectual impairment much less common

Question 50

What is Huntington's disease?

Answer 50

inherited neurodegenerative condition progressive and incurable condition that typically results in death 20yrs after initial symptoms

Question 51

Huntington's disease genetics

Answer 51

autosomal dominant trinucleotide repeat disorder of CAG anticipation may be seen (presents earlier in successive generations) results in degeneration of cholinergic and GABAergic neurons in striatum of basal ganglia defect in huntingtin gene on C4

Question 52

Huntington's disease features

Answer 52

typically develop after 35yrs: chorea personality changes (irritability, apathy, depression) and intellectual impairment dystonia saccadic eye movements

Question 53

Huntington's disease management

Answer 53

no treatment options to slow or stop disease physio SLT tetrabenazine - chorea antidepressants

Question 54

Causes of a brain abscess

Answer 54

extension of sepsis from middle ear or sinuses trauma surgery to scalp penetrating head injuries embolic events from endocarditis

Question 55

Symptoms of a brain abscess

Answer 55

headache - dull, persistent fever focal neurology - due to raised intracranial pressure nausea papilloedema seizures

Question 56

Brain abscess investigations

Answer 56

CT scan

Question 57

Brain abscess management

Answer 57

surgery - craniotomy, cavity debrided. Abscess may reform bcs head is closed following abscess drainage IV antibiotics - cephalosporin & metronidazole dexamethasone for intracranial pressure management

Question 58

Meningitis causes 0 - 3 months

Answer 58

grp B streptococcus (most common in neonates) e.coli listeria monocytogenes

Question 59

Meningitis causes 3 months - 6yrs

Answer 59

neisseria meningitidis streptococcus pneumoniae haemophilus influenzae

Question 60

Meningitis causes 6yrs - 60yrs

Answer 60

neisseria meningitidis streptococcus pneumoniae

Question 61

Meningitis causes >60yrs

Answer 61

neisseria meningitidis streptococcus pneumoniae listeria monocytogenes

Question 62

Meningitis causes in immunosuppressed ppl

Answer 62

listeria monoctyogenes

Question 63

What is meningitis?

Answer 63

inflammation of the meninges (lining of brain and spinal cord), usually due to infection CSF is contained w/in meninges (subarachnoid space)

Question 64

Symptoms of meningitis

Answer 64

headache fever nausea / vomiting photophobia drowsiness seizures

Question 65

Signs of meningitis

Answer 65

neck stiffness purpuric rash (particularly w/ invasive meningococcal disease)

Question 66

Bacterial meningitis in infants

Answer 66

classical signs of meningitis often absent

Question 67

Bacterial meningitis features

Answer 67

CSF appearance = cloudy Glucose = low Protein = high WCs = 10 - 5000 polymorphs/mm3

Question 68

Viral meningitis features

Answer 68

CSF appearance = clear/cloudy Glucose = 60 - 80% of plasma glucose Protein = normal / raised WCs = 15 - 1000 lymphocytes/mm3

Question 69

Tuberculous meningitis features

Answer 69

CSF appearance = slightly cloudy, fibrin web Glucose = low Protein = high WCs = 10 - 1000 lymphocytes/mm3

Question 70

Ziehl-Neelson sensitivity

Answer 70

only 20% in detection of tuberculous meningitis PCR is sometimes used (sensitivity = 75%)

Question 71

Fungal meningitis features

Answer 71

CSF appearance = cloudy Glucose = low Protein = high WCs = 20 - 200 lymphocytes/mm3

Question 72

Contraindications to lumbar puncture

Answer 72

any signs of raised ICP focal neuro signs papilloedema significant bulging of fontanelle disseminated intravascular coagulation signs of cerebral herniation meningococcal septicaemia (do blood cultures and PCR)

Question 73

Meningitis management

Answer 73

antibiotics (<3mnths = IV amox & Iv cefotaxime. >3mnths = IV cefotaxime) steroids fluids cerebral monitoring public health notification and abx prophylaxis of contacts

Question 74

Meningitis complications

Answer 74

Neurological sequalae - sensorineural hearing loss, seizures, focal neurological deficit Infective - sepsis, intracerebral abscess Pressure - brain herniation, hydrocephalus meningococcal meningitis has risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency 2nd to adrenal haemorrhage)

Question 75

When is the meningitis B vaccine given?

Answer 75

2 months 4 months 12-13 months

Question 76

Causes of viral meningitis

Answer 76

non-polio enteroviruses eg coxsackie virus, echovirus mumps herpes simplex virus, cytomegalovirus, herpes zoster viruses, HIV, measles

Question 77

Risk factors of viral meningitis

Answer 77

pts at extremes of age (<5yrs and elderly) immunocompromised eg pts w/ renal failure, w/ DM IV drug users

Question 78

Viral meningitis management

Answer 78

supportive while waiting for LP viral meningitis is self-limiting, symptoms improve after 7-14 days and complications are rare in immunocompetent pts Aciclovir may be used if pt is suspected of having meningitis 2nd to HSV

Question 79

Immediate management of bacterial meningitis

Answer 79

if in a pre-hospital setting, IM benzylpenicilin can be given if meningococcal disease is suspected

Question 80

Bacterial meningitis warning signs

Answer 80

rapidly progressive rash poor peripheral perfusion resp rate <8 or >30 / min pulse rate <40 or >140 / min pH <7.3 WBC <4 *109/L lactate >4 mmol/L GCS <12 or drop of 2 pts poor response to fluid resuscitation

Question 81

Management of pts w/ raised intracranial pressure

Answer 81

critical care input secure airway and high flow O2 IV access - blood and cultures IV dexamethasone IV abx arrange neuroimaging

Question 82

Bacterial meningitis investigations

Answer 82

GBC renal function glucose lactate clotting profile CRP

Question 83

Features of encephalitis

Answer 83

fever, headache, psychiatric symptoms, seizures, vomiting focal features eg aphasia peripheral lesions (eg cold sores) have no relation to presence of HSV encephalitis

Question 84

Encephalitis pathophysiology

Answer 84

HSV-1 is responsible for 95% of cases in adults typically affects temporal and inferior frontal lobes

Question 85

Encephalitis investigations

Answer 85

Bloods CSF- lymphocytes, elevated protein PCR for HSV, VSV and enteroviruses Neuroimaging - medial temporal and inferior frontal changes. Normal in 1/3 pts, MRI is better EEG - lateralised periodic discharges at 2Hz

Question 86

Encephalitis Management

Answer 86

IV aciclovir should be started in all cases of suspected encephalitis

Question 87

Meningitis v encephalitis

Answer 87

both present w/ fever, headache, altered mental state however, pts w/ meningitis have more neck stiffness and photophobia pts w/ encephalitis present w/ focal neuro deficits eg aphasia or hemiparesis CSF: encephalitis = lymphotic pleocytosis, meningitis = neutrophil predominance

Question 88

What is Guillain-Barre syndrome?

Answer 88

post infectious, immune-mediated rare, acute polyradiculoneuropathy rapidly evolving ascending muscle weakness w/ mild sensory changes

Question 89

Guillain-Barre syndrome aetiology

Answer 89

autoimmune often precipitated by infection, most commonly campylobacter jejuni, epstein-barr virus or cytomegalovirus

Question 90

Risk factors of Guillain-Barre syndrome

Answer 90

infections vaccinations surgery

Question 91

Underlying causes of Guillain-Barre syndrome

Answer 91

theories: - molecular mimicry - antibodies generated in response to infection cross-react w/ gangliosides present on periph nerves due to structural similarities, resulting in nerve damage - bystander activation - infection leads to non-specific activation of immune system, which subsequently attacks peripheral nerves. More relevant in cases associated w/ viral infections - correlation between anti-GM1 and clinical features. Anti-GM1 in 25% of pts

Question 92

Clinical features of Guillain-Barre syndrome

Answer 92

- symptoms usually w/in 4 wks of triggering infection, may start as gastroenteresis - symmetrical ascending weakness - reduced reflexes - peripheral loss of sensation, neuropathic pain - can progress to CNs and cause facial weakness - autonomic dysfunction (urinary retention, ileus or heart arrhythmias)

Question 93

Guillain-Barre syndrome investigations and diagnosis

Answer 93

- clinical diagnosis (Brighton criteria), based on characteristic presentation of progressive, usually symmetric muscle weakness w/ or w/out sensory disturbances - lumbar puncture - rise in protein w/ normal white cell count (in 66%) - nerve conduction studies

Question 94

Guillain-Barre syndrome management

Answer 94

supportive care VTE prophylaxis (PE is leading cause of death) 1st line = IV immunoglobulins plasmapheresis alternative to IVIG

Question 95

Miller Fisher syndrome

Answer 95

variant of Guillain-Barre syndrome associated w/ ophthalmoplegia, areflexia and ataxia eye muscles typically affected first usually presents as descending paralysis rather than ascending as seen in other forms of GBS anti-GQ1 antibodies present (90% cases)

Question 96

What is herpes zoster infection?

Answer 96

Shingles acute, unilateral, painful blistering rash caused by reactivation of varicella-zoster virus (VSV) following primary infection (chickenpox), virus lies dormant in dorsal root or cranial nerve ganglia

Question 97

Herpes zoster risk factors

Answer 97

increasing age HIV (15x more common) other immunosuppressive conditions eg steroids, chemo

Question 98

What are the most commonly affected dermatomes in herpes zoster?

Answer 98

T1-L2

Question 99

Features of herpes zoster infection

Answer 99

Prodromal period = burning pain over affected dermatome 2-3 days, severe pain - interfere w/ sleep, around 20% get fever, headache and lethargy Rash - initially erythematous, macular rash over affected dermatome. Quickly become vesicular, characteristically well demarcated by dermatome and doesn't cross midline. However, 'bleeding' into adjacent areas may be seen

Question 100

Herpes zoster diagnosis and management

Answer 100

diagnosis = clinical management = remind pt they are infectious analgesia - paracetamol, NSAIDs anti-virals = w/in 72hrs for majority of pts - aciclovir, famciclovir or valaciclovir

Question 101

How long are herpes zoster pts infectious for?

Answer 101

until vesicles have crusted over, usually 5-7 days following onset

Question 102

Why should you prescribe antivirals for herpes zoster infection?

Answer 102

reduced incidence of post-herpetic neuralgia, particularly in older ppl don't use antivirals if pt is <50 and has 'mild' truncal rash associated w/ mild pain and no underlying risk factors

Question 103

Herpes zoster complications

Answer 103

post-herpetic neuralgia herpes zoster ophthalmicus (shingles affecting ocular division of trigeminal nerve) herpes zoster oticus (Ramsey Hunt syndrome) - may result in ear lesions and facial paralysis

Question 104

Herpes zoster ophthalmicus (HZO)

Answer 104

reactivation of varicella-zoster virus in area supplied by ophthalmic division of trigeminal nerve around 10% of shingles cases vesicular rash round eye, Hutchinson's sign management = oral antiviral treatment for 7-10 days, topical corticosteroids to treat secondary inflammation of eye complications = conjunctivitis, keratitis, ptosis, post-hepatic neuralgia

Question 105

What is Hutchinson's sign?

Answer 105

rash on tip or side of nose indicates nasociliary involvement and is strong risk factor for ocular involvement

Question 106

Herpes simplex keratitis

Answer 106

usually presents w/ dendritic corneal ulcer red, painful eye photophobia epiphora treat w/ topical aciclovir and ophthalmologist referral

Question 107

What 4 different species causes malaria in humans?

Answer 107

plasmodium falciparum - most common plasmodium vivax plasmodium ovale plasmodium malariae

Question 108

Protective factors of malaria

Answer 108

sickle cell anaemia G6Pd deficiency HLA-B53 absence of Duffy antigens

Question 109

Falciparum malaria presentation

Answer 109

paroxysms of fever, chills and sweating - may occur every 48 hrs corresponding to erythrocyte cycle of parasite GI - anorexia, nausea, vomiting, abdo pain resp - cough MSK - body aches and joint pains neuro - headache, dizziness CV - tachy, htn

Question 110

Features of severe falciparum malaria

Answer 110

schizonts on blood film parasitaemia >2% hypoglycaemia acidosis temp >39 severe anaemia

Question 111

Falciparum malaria complications

Answer 111

cerebral malaria - seizures, coma acute renal failure - blackwater fever acute resp distress syndrome hypoglycaemia disseminated intravascular coagulation

Question 112

Uncomplicated falciparum malaria management

Answer 112

artemisinin-based combo therapies (ACTs) eg artemether + lumefantrine artesunate + amodiaquine artesunate + mefloquine

Question 113

Severe falciparum malaria management

Answer 113

parasite counts >2% need parenteral treatment IV artesunate if parasite count >10% consider transfusion

Question 114

Malaria diagnosis

Answer 114

malaria blood film EDTA bottle (FBC) 3 -ve samples over 3 consecutive days required to exclude malaria

Question 115

Features of non-falciparum malaria

Answer 115

fever, headache, splenomegaly plasmodium vivax / ovale - cyclical fever every 48hrs plasmodium malariae - cyclical fever every 72hrs plasmodium malariae - associated w/ nephrotic syndrome

Question 116

Which malaria types have a hypnozoite stage?

Answer 116

ovale and vivax may therefore relapse following treatment

Question 117

Treatment of non falciparum malaria

Answer 117

artemisinin-based combo therapy (ACTs) or chloroquine ovale or vivax = give primaquine following acute treatment to destroy liver hypnozoites and prevent relapse

Question 118

Where is chloroquine resistant malaria strains prevelant?

Answer 118

certain areas of Asia and Africa

Question 119

Where are the majority of brain tumours in adults?

Answer 119

supratentorial - above tentorium cerebelli eg cerebrum, ventricles and upper part

Question 120

Where are the majority of brain tumours in children?

Answer 120

infratentorial below tentorium cerebelli, separates the cerebellum from cerebrum lower back part of brain, contains the cerebellum, brainstem, and fourth ventricle

Question 121

What is the most common form of brain tumour?

Answer 121

metastatic brain cancer

Question 122

Tumours that most commonly spread to the brain

Answer 122

lung breast bowel skin kidney

Question 123

Glioblastoma prognosis

Answer 123

~1 year

Question 124

What does a glioblastoma look like on imaging?

Answer 124

solid tumours w/ central necrosis rim that enhances w/ contrast disruption of BBB and therefore associated w/ vasogenic oedema

Question 125

Glioblastoma histology

Answer 125

pleomorphic tumour cells border necrotic areas

Question 126

Glioblastoma treatment

Answer 126

surgical w/ post-op chemo and/or radiotherapy dexamethasone to treat oedema

Question 127

What are gliomas?

Answer 127

glial cell tumours in brain or spinal cord glial cells surround and support neurones eg astrocytes (astrocytoma - most common and aggressive glioblastoma) oligodendrocytes (oligodendroglioma) ependymal cells (ependymoma)

Question 128

Brain tumour presentation

Answer 128

progressive focal neurological symptoms raised ICP

Question 129

Causes of increased intracranial pressure

Answer 129

brain tumours intracranial haemorrhage idiopathic intracranial HTN abscesses or infection

Question 130

Symptoms of raised intracranial pressure

Answer 130

constant headache nocturnal worse on waking worse on coughing, straining or bending forward vomiting papilloedema altered mental state visual field defects seizures unilateral ptosis

Question 131

Papilloedema on fundoscopy

Answer 131

blurring of optic disc margin elevated optic disc loss of venous pulsation engorged retinal veins haemorrhages around optic disc Paton's lines - creases or fold in retina around optic disc

Question 132

What are meningiomas?

Answer 132

tumours growing from cells of meninges usually benign take up space, 'mass effect' can lead to raised ICP and neuro symptoms

Question 133

What are vestibular schwannomas?

Answer 133

acoustic neuromas - benign tumours of Schwann cells that surround auditory nerve that innervates inner ear account for approx 5% of intracranial tumours and 90% of cerebellopontine angle tumours tumours often slow growing and benign

Question 134

Vestibular schwannoma presentation

Answer 134

combo of vertigo, hearing loss, tinnitus and absent corneal reflex (CN 5, 7 and 8) usually unilateral

Question 135

Vestibular schwannoma investigations and management

Answer 135

MRI of cerebellopontine angle, audiometry Management = surgery, radiotherapy or observation

Question 136

Pituitary tumours

Answer 136

tend to be benign can press optic chiasm and cause bitemporal hemianopia (loss of outer visual fields)

Question 137

What can pituitary tumours cause?

Answer 137

hormone deficiencies or excessive hormone release: acromegaly hyperprolactinaemia Cushing's disease thyrotoxicosis

Question 138

Pituitary tumour management

Answer 138

trans-sphenoidal surgery radiotherapy bromocriptine - block excess proalctin somatostatin anaglogues - block excess GH

Question 139

What is bulbar palsy?

Answer 139

impaired function of lower cranial nerves, those that arise from brainstem (9,10,11,12)

Question 140

Bulbar palsy symptoms

Answer 140

dysphasia absent gag reflex slurred speech aspirations dysphonia drooling issues chewing weak jaw / facial muscles

Question 141

Bulbar palsy causes

Answer 141

brainstem tumours and strokes degenerative diseases - amytrophic lateral sclerosis autoimmune - Guillain-Barre syndrome genetic diseases eg Kennedy disease

Question 142

Treatment of bulbar palsy

Answer 142

no known treatment manage symptoms : medication for drooling feeding tube SLT

Question 143

Cerebellar disease

Answer 143

DANISH Dysdiadochokinesia, dysmetria Ataxia Nystagmus Intention tremor Slurred staccato speech, scanning dysarthria Hypotonia

Question 144

Cerebellar syndrome causes

Answer 144

Friedreich's ataxia, ataxic telangiectasia neoplastic - cerebellar haemangioma stroke alcohol multiple sclerosis hypothyroidism drugs - phenytoin, lead poisioning paraneoplastic eg 2nd to lung cancer

Question 145

What is cerebral palsy?

Answer 145

movement and posture disorder due to non-progressive lesion of motor pathways in developing brain 2/1000 live births most common cause of major motor impairment

Question 146

Causes of cerebral palsy

Answer 146

antenatal (80%) - cerebral malformation and congenital infection intrapartum (10%) - birth asphyxia / trauma postnatal (10%) - intraventricular haemorrhage, meningitis, head-trauma

Question 147

Cerebral palsy manifestations

Answer 147

abnormal tone early infancy delayed motor milestones abnormal gait feeding difficulties

Question 148

Associated non-motor problems in cerebral palsy pts

Answer 148

learning difficulties (60%) epilepsy (30%) squints (30%) hearing impairment (20%)

Question 149

Cerebral palsy classification

Answer 149

- spastic - hemiplegia, diplegia or quadriplegia, increased tone from UMN damage - dyskinetic - basal ganglia and substantia nigra damage, athetoid movements and oro-motor problems - ataxic - cerebellum damage - mixed

Question 150

Cerebral palsy manamgent

Answer 150

MDT approach spasticity - oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy anticonvulsants analgesia as required

Question 151

What is Bell's palsy?

Answer 151

acute, unilateral, idiopathic, facial nerve paralysis peak incidence = 20-40yrs more common in pregnant women

Question 152

Features of Bell's palsy

Answer 152

lower motor neuron facial nerve palsy - forehead affected post-auricular pain altered taste dry eyes hyperacusis

Question 153

Bell's palsy management

Answer 153

oral prednisolone w/in 72 hrs of onset eye care - prevent exposure keratopathy: artificial tears or eye lubricant

Question 154

Bell's palsy prognosis

Answer 154

most make full recovery w/in 3-4 months if untreated around 15% have permanent moderate - severe weakness

Question 155

How is epilepsy classified?

Answer 155

1. where seizure begins in brain 2. lvl of awareness during seizure 3. other features of seizures

Question 156

What are focal seizures?

Answer 156

start in specific area, on one side of brain lvl of awareness varies: focal aware, focal impaired and awareness unknown classified as motor (eg Jacksonian march), non-motor or having other features like aura

Question 157

What are generalised seizures?

Answer 157

engage both sides of brain at onset lose consciousness immediately motor (tonic-clonic) and non-motor (absence) tonic, clonic, atonic, tonic-clonic and absence

Question 158

What is a focal to bilateral seizure?

Answer 158

starts on one side of brain in specific area before spreading to both lobes (previously 2ndary generalised seizures)

Question 159

What conditions have an association w/ epilepsy?

Answer 159

cerebral palsy - around 30% tuberous sclerosis mitochondrial diseases

Question 160

Temporal lobe typical seizure type

Answer 160

W/ or w/out impairment of consciousness or awareness Aura: rising epigastric sensation, psychic or experiental phenomena eg deja vu, less commonly hallucinations typically last around 1 min, automatisms are common

Question 161

Frontal lobe typical seizure type

Answer 161

head / leg movements posturing post-ictal weakness Jacksonian march

Question 162

Parietal lobe typical seizure type

Answer 162

sensory paraesthesia

Question 163

Occipital lobe typical seizure type

Answer 163

visual floaters flashes

Question 164

Other causes of recurrent seizures

Answer 164

febrile convulsions alcohol withdrawal seizures psychogenic non-epileptic seizures

Question 165

Forms of epilepsy in children

Answer 165

infantile spasms Lennox-Gastaut syndrome Benign rolandic epilepsy Juvenile myoclonic epilepsy

Question 166

Generalised seizures signs

Answer 166

bitten tongue urine incontinence

Question 167

Syncope episodes v seizures

Answer 167

syncopal episodes associated w/ short ictal period and rapid recovery seizures usually have long post-ictal period

Question 168

When are epileptic drugs considered?

Answer 168

after second seizure

Question 169

What groups of patients should be considered during epilepsy management?

Answer 169

pts who drive (can't drive 6 months following) pts taking other medications women wishing to get pregnant women taking contraception

Question 170

Sodium valporate

Answer 170

used for generalised seizures in males increases GABA activity should not be used in females of reproductive age

Question 171

Carbamezapine

Answer 171

used second line for focal seizures binds to sodium channels increasing refractory period

Question 172

Lamotrigine

Answer 172

used for variety of generalised and focal seizures sodium channel blocker can be used in females

Question 173

Phenytoin

Answer 173

no longer used 1st line due to side effects binds to sodium channels increasing refractory period SE: osteopenia, osteoporosis, drowsiness, headaches

Question 174

Acute management of seizures

Answer 174

benzodiazepines eg diazepam administered if seizure doesn't terminate after 5-10 mins

Question 175

What is status epilepticus?

Answer 175

single seizure lasting >5mins or >= 2 seizures w/in 5min period w/out person returning to normal between

Question 176

Status epilepticus management

Answer 176

ABC = airway adjunct, oxygen, blood glucose 1st line = benzodiazepines, IV lorazepam if in hospital. Repeated once after 5-10 mins If ongoing, 2nd line may be used eg levetiracetam If no response w/in 45 mins, induce general anaesthesia or phenobarbital

Question 177

Sodium valproate contraindications

Answer 177

maternal use associated w/ significant risk of neurodevelopmental delay in children

Question 178

Generalised tonic-clonic seizure treatment

Answer 178

males = sodium valproate females = lamotrigine or levetiracetam

Question 179

Focal seizures treatment

Answer 179

1st = lamotrigine or levetiracetam 2nd = carbamezapine, oxcarbazepine or zonisamide

Question 180

Absence seizures treatment

Answer 180

1st = ethosuximide 2nd = male: sodium valproate, female: lamotrigine or levetiracetam

Question 181

Myocolonic seizures treatment

Answer 181

males = sodium valproate females = levetiracetam

Question 182

Tonic or atonic seizures treatment

Answer 182

males = sodium valproate females = lamotrigine

Question 183

What should be considered in women with epilepsy taking contraception?

Answer 183

- effect of contraceptive on effectiveness of anti-epileptic medication - effect of anti-epileptic on effectiveness of contraceptive - potential teratogenic effects of anti-epileptic if woman becomes pregnant

Question 184

Clinical features of febrile convulsions

Answer 184

usually occur early in viral infection as temp rises seizures usually brief, <5mins most commonly tonic-clonic

Question 185

Types of febrile convulsions

Answer 185

Simple = <15mins, generalised, typically no recurrence w/in 24hrs, should recover in an hr Complex = 15-30mins, focal, may have repeat in 24hrs Febrile status epilepticus = >30mins

Question 186

Common causes of febrile convulsions

Answer 186

resp tract infections otitis media urinary tract infections influenza HHV-6

Question 187

Ongoing management of febrile convulsions

Answer 187

phone ambulance if >5mins if recurrent, benzodiazepine rescue meds considered children who have had a first seizure or any features of a complex seizure should be admitted to paeds

Question 188

Anterior cerebral artery stroke

Answer 188

contralateral hemiparesis and sensory loss lower extremity > upper

Question 189

Middle cerebral artery stroke

Answer 189

contralateral hemiparesis and sensory loss upper extremity > lower contralateral homonymous hemianopia aphasia

Question 190

Posterior artery stroke

Answer 190

contralateral homonymous hemianopia w/ macular sparing visual agnosia

Question 191

Weber's syndrome

Answer 191

branches of pos. cerebral artery that supply midbrain ipsilateral CN3 palsy contralateral weakness of upper and lower extremity

Question 192

Anterior inferior cerebellar artery stroke

Answer 192

Similar to Wallenberg's but ipsilateral facial paralysis and deafness

Question 193

Posterior inferior cerebellar artery stroke (Wallenberg syndrome)

Answer 193

ipsilateral facial pain and temp loss contralateral limb/torso pain and temp loss ataxia, nystagmus

Question 194

Lacunar strokes

Answer 194

present w/ isolated hemiparesis, hemisensory loss or hemiparesis w/ limb ataxia strong association w/ HTN common sites = basal ganglia, thalamus and internal capsule

Question 195

Other issues during stroke management

Answer 195

fluids glycaemic control BP management feeding assessment and management disability scales

Question 196

ROSIER score

Answer 196

Stroke assessment exclude hypogylcaemia first, then assess: loss of consciousness or syncope (-1) seizure activity (-1) New, acute onset of: (+1) - asymmetric facial weakness - asymmetric arm weakness asymmetric leg weakness - speech disturbance - visual field defect stroke likely if >0

Question 197

Acute ischaemic stroke CT

Answer 197

areas of low density in grey and white matter of territory hyperdense artery sign corresponding w/ responsible arterial clot - tends to be visible immediately

Question 198

Acute haemorrhagic stroke CT

Answer 198

areas of hyperdense material (blood) surrounded by low density (oedema)

Question 199

Features of a TIA

Answer 199

unilateral weakness or sensory loss aphasia or dysarthria ataxia, vertigo or balance loss visual problems (loss of vision in one eye, diplopia, homonymous hemianopia)

Question 200

Examples of TIAs that require exclusion

Answer 200

hypoglycaemia intracranial haemorrhage (all pts on anticoags or w/ similar risk factors should be admitted for urgent imaging to exclude haemorrhage)

Question 201

How to assess territory of ischaemia in TIAs

Answer 201

MRI including diffusion-weighted and blood-sensitive sequences done on same day as specialist assessment

Question 202

TIA management

Answer 202

immediate anti-thrombotic therapy provided no contraindications or high bleeding risk

Question 203

When is aspirin given as antithrombotic medication?

Answer 203

resolved TIA symptoms, awaiting specialist review w/in 24hrs

Question 204

When is aspirin and clopidogrel given as antithrombotic therapy?

Answer 204

reviewed by specialist, initial 21 days when at high risk of further events

Question 205

When is clopidogrel given as antithrombotic medication?

Answer 205

long-term secondary prevention after 21 days

Question 206

Further investigations in a TIA

Answer 206

carotid imaging - carotid duplex ultrasound carotid endarterectomy recommended if pt has suffered stroke or TIA in carotid territory and is not severely disabled (considered if stenosis >50% according to NASCET criteria)

Question 207

What is a subarachnoid haemorrhage?

Answer 207

intracranial haemorrhage presence of blood w/in subarachnoid space - deep to subarachnoid layer of meninges

Question 208

Most common cause of subarachnoid haemorrhage

Answer 208

traumatic - head injury

Question 209

Causes of spontaneous subarachnoid haemorrhage

Answer 209

intracranial aneurysm (saccular 'berry' aneurysm) (~85%) arteriovenous malformation pituitary apoplexy mycotic (infective) aneuryms

Question 210

Presenting features of subarachnoid haemorrhage

Answer 210

headache - thunderclap, sudden onset. Severe, occipital nausea and vomiting meningism coma seizures ECG change - ST elevation

Question 211

Subarachnoid haemorrhage investigations

Answer 211

non-contrast CT head - blood in basal cisterns, sulci and sometimes ventricular system IF CT head done w/in 6hrs of symptoms onset and is normal = no LP IF CT head done >6hrs of symptom onset and is normal = LP (LP should be performed at least 12hrs following symptom onset) If CT shows SAH, refer to neurosurgery

Question 212

Lumbar puncture findings in subarachnoid haemorrhage

Answer 212

xanthochromia (RBC breakdown) normal or raised opening pressure

Question 213

Further investigations of subarachnoid haemorrhages

Answer 213

after spontaneous SAH confirmed, try to find causative pathology that needs treatment CT intracranial angiogram - vasc lesion +/- digital subtraction angiogram

Question 214

Management of confirmed aneurysmal subarachnoid haemorrhage

Answer 214

- supportive - bed rest, analgesia, venous thromboembolism prophylaxis, discontinuation of antithrombotics - vasospasm prevention - nimodipine -intracranial aneurysms need prompt intervention, preferably w/in 24hrs

Question 215

Intracranial aneurysm treatment

Answer 215

coil by interventional neuroradiologists, minority require craniotomy and clipping by neurosurgeon

Question 216

Complications of aneurysmal SAH

Answer 216

rebleeding hydrocephalus vasospasm hyponatraemia due to SIADH seizures

Question 217

Important predictive factors in SAH

Answer 217

conscious level on admission age amount of blood visible on CT head

Question 218

What is a subdural haemorrhage?

Answer 218

bleeding in space between dura mater and arachnoid layers of meninges typically result of significant head trauma leading to rupture of bridging veins, can also present spontaneously or after minor injuries

Question 219

Subdural haemorrhage risk factors

Answer 219

age alcoholism anticoagulant therapy trauma vasc malformations coagulopathies

Question 220

How are subdural haematomas classified?

Answer 220

acute - fresh blood, usually trauma subacute chronic - blood collection present for wks to months (presentation usually several wk to month progressive history of either confusion, reduced consciousness or neuro deficit)

Question 221

Investigations of acute subdural haematoma

Answer 221

CT imaging crescentic collection, not limited by suture lines hypERdense in comparison to brain large subdural haematomas will push on brain and cause midline shift or herniation

Question 222

Investigations of chronic subdural haematoma

Answer 222

CT crescentic shape, not restricted by suture lines and compress brain hypOdense compared to substance of brain

Question 223

Acute subdural haematoma management

Answer 223

observed conservatively surgical = monitor ICP, decrompressive craniectomy

Question 224

Chronic subdural haematoma management

Answer 224

if incidental finding or small w/ no neuro deficit = conservative if neuro deficit or severe imaging findings = surgical decompression w/ burr holes

Question 225

Subdural haemorrhage complications

Answer 225

raised ICP cerebral oedema herniation syndromes infection hydrocephalus venous infarction epilepsy

Question 226

What is an extradural haematoma?

Answer 226

collection of blood between skull and dura almost always caused by trauma, most typically by 'low-impact' trauma

Question 227

Where do extradural haematomas usually occur?

Answer 227

temporal region - thin skull at pterion overlies middle meningeal artery - vulnerable

Question 228

Presentation of an extradural haematoma

Answer 228

pt who initially loses, briefly regains and then loses consciousness again after low-impact head injury 'lucid interval' - lost eventually due to expanding haematoma and brain herniation As haematoma expands, uncus of temporal lobe herniates around tentorium cerebelli and pt develops fixed and dilated pupil due to compression of parasympathetic fibres of 3rd CN

Question 229

Extradural haematoma investigations

Answer 229

imaging - appears biconcave, hyperdense collection around surface of brain limited by suture lines of skull

Question 230

Extradural haematoma management

Answer 230

no neuro deficit = cautious clinical and radiological observation otherwise, craniotomy and evacuation of haematoma

Question 231

What is temporal arteritis?

Answer 231

giant cell arteritis vasculitis predominantly affecting medium and large arteries, particularly branches of carotid artery

Question 232

Temporal arteritis contributing factors

Answer 232

genetic predisposition - HLA alleles environmental factors - seasonal and geographical clustering age - 70-80 sex - female ethnicity - N. Europe

Question 233

Temporal arteritis pathology

Answer 233

antigenic trigger T-cell activation - CD4+ T-cells, initiate inflammatory cascade macrophage and giant cell formation vasc inflammation and damage - results in intimal thickening, narrowing and ischaemia

Question 234

Temporal arteritis presentation

Answer 234

rapid onset headache jaw claudication scalp tenderness visual disturbances temporal artery abnormality - tenderness, thickening or reduced pulsation

Question 235

Temporal arteritis investigations

Answer 235

raised inflam markers: ESR>50mm/hr, raised CRP temporal artery biopsy - skip lesions may be present creatine kinase and EMG normal

Question 236

Temporal arteritis investigations guidelines

Answer 236

low clinical probability (<20%) = temporal and axillary US medium probability (20-50%) = US may be prior to biopsy high probability (>50%) = +ve US makes diagnosis

Question 238

Temporal arteritis classification

Answer 238

American College of Rheumatology 3 or more = highly suspected: age >50 new onset headache temporal artery abnormality ESR > 50mm/hr abnormal artery biopsy

Question 239

Temporal arteritis differentials

Answer 239

migraine central retinal artery occlusion acute glaucoma trigeminal neuralgia multiple sclerosis

Question 240

Temporal arteritis management

Answer 240

high dose corticosteroids- prednisolone - vision loss adjunctive therapy w/ low-dose aspirin - ischaemic complications temporal artery biopdy w/in 14 days of starting steroids to confirm diagnosis immunosuppressants can be used if can't tolerate steroids eg methotrexate

Question 241

Temporal arteritis complications

Answer 241

vision loss ocular complications stroke aortic aneurysm and dissection large vessel involvement polymyalgia rheumatica

Question 242

Glasgow coma scale eye opening

Answer 242

4 - spontaneous 3 - to sound 2 - to pressure 1 - none

Question 243

Glasgow coma scale verbal response

Answer 243

5 - orientated 4 - confused 3 - words 2 - sounds 1 - none

Question 244

Glasgow coma scale - motor response

Answer 244

6 - obeys commands 5 - localising 4 - normal flexion 3 - abnormal flexion 2 - extension 1 - none

Question 245

Glasgow coma scale scores

Answer 245

13-15 = mild 9-12 = moderate 3-8 = severe

Question 246

Causes of migraines

Answer 246

genetics environment - stress, hormonal changes, sleep disturbances, dietary factors, sensory stimuli, medications

Question 247

Migraine pathology - cortical spreading depression

Answer 247

characterised by wave of transient neuronal depolarisation followed by prolonged period of suppressed neuronal activity implicated in initiation of migraine aura and subsequent headache phase

Question 248

Migraine pathology - neurovascular changes

Answer 248

changes in cerebral blood flow and vascular function during early phase of attack, there's decrease in cerebral blood flow, followed by vasodilation and increased blood flow process may contribute to activation of nociceptive trigeminal nerve fibres, leading to release of vasoactive neuropeptides and inflammation

Question 249

Migraine pathology - neuropeptides and neurotransmitters

Answer 249

- CGRP - vasodilater, activates trigeminal nerve fibres, promotes inflammation and pain transmission - Serotonin (5-HT) - vasoactive, modulates pain pathways - Glutamate - excitatory neurotransmitter, initiates and maintains CSD and sensitisation of trigeminal nociceptive neurons

Question 250

How can migraines be classified?

Answer 250

with or without aura chronic probable migraine episodic syndromes associated w/ migraine

Question 251

Migraine w/out aura

Answer 251

most common moderate to severe pulsating headache lasting between 4 and 72 hrs, usually unilateral other symptoms include photophobia, phonophobia, nausea and vomiting, aggravation by routine physical activity

Question 252

Migraine w/ aura

Answer 252

1/4 of migraines - typical aura - visual, sensory, speech or language, motor, brainstem or retinal symptoms preceding headache phase - hemiplegic migraine - reversible motor weakness as aura - retinal migraine - transient monocular visual loss or blindness

Question 253

What is a chronic migraine?

Answer 253

headache on 15 or more days per month for at least 3 months migraines on at least 8 days per month

Question 254

What are complicated migraines?

Answer 254

migraines appear to directly cause neuro damage or other complications - migrainous infarction - ischaemic stroke that occurs during typical attack of migraine w/ aura - migraine aura-triggered seizure - persistent aura w/out infarction - aura symptoms for 1wk or more, w/out evidence of stroke

Question 255

Episodic syndromes that may be associated w/ migraine

Answer 255

cyclic vomiting syndrome abdominal migraine benign paroxysmal vertigo of childhood

Question 256

Clinical features of migraines

Answer 256

severe, unilateral, throbbing headache associated w/ nausea, photophobia and phonophobia last up to 72hrs precipitated by aura

Question 257

What is aura?

Answer 257

1/3 of migraine pts visual, progressive last 5-60 mins transient hemianopic disturbance or spreading scintillating scotoma

Question 258

Migraine investigations

Answer 258

usually diagnosed by history and physical exam neuroimaging to rule out other blood tests - rule out systemic conditions MRI lumbar puncture invasice angiography

Question 259

International headache society's migraine w/out aura diagnostic criteria

Answer 259

A - at least 5 attacks fulfilling criteria B-D B - headache lasts 4-72hrs C - characteristics: 1.unilateral, 2. pulsating quality, 3. moderate or severe pain intensity, 4. aggravation by routine physical activity D - at least one of: 1, nausea or vomiting, 2. photophobia and phonophobia E - not attributed to another disorder

Question 260

Migraine differentials

Answer 260

tension-type headache cluster headache temporal artertitis

Question 261

Tension type headache vs migraine

Answer 261

TTH presents as bilateral, pressing or tightening sensation of mild to moderate intensity (band around head) Migraine is unilateral, pulsating quality of pain TTH no accompanies by nausea or vomiting Migraine usually exacerbated by routine physical activity

Question 262

Cluster headache vs migraine

Answer 262

Cluster headache - unilateral pain usually centred around eye or temple, piercing or burning, more intense than typical migraine attacks Cluster headache has shorter duration (15mins - 3hrs) but occur more frequently Cluster headache associated w/ ipsilateral autonomic symptoms eg lacrimation, nasal congestion or rhinorrhoea

Question 263

Temporal arteritis vs migraine

Answer 263

TA typically affects individuals over 50 TA is new-onset, persistent and localised to temples or occiput, no pulsating TA may have systemic symptoms ESR and CRP typically elevated in TA

Question 264

Migraine management

Answer 264

1st: combo therapy of oral triptan and NSAID or oral triptan and paracetamol 12-17yrs = nasal triptan If above not effective, consider metoclopramide or prochlorperazine and add non-oral NSAID or triptan

Question 265

Migraine prophylaxis

Answer 265

propanolol topiramate - avoid in women of child bearing age acupuncture course riboflavin menstrual migraines = frovatriptan or zolmitriptan

Question 266

Migraine management general rule

Answer 266

5-HT receptor agonists used in acute treatment 5-HT receptor antagonists used in prophylaxis

Question 267

Migraine acute complications

Answer 267

- status migrainosus - debilitating migrain for >72hrs, lead to dehydration and stroke - migrainous infarction - ischaemic stroke during migraine w/aura - persistent aura w/out infarction - visual or sensory symptoms of aura last longer than 1wk after headache resolved

Question 268

Chronic complications of migraine

Answer 268

- chronic migraine - >15days per month for at least 3months - medication overuse headache - transformed migraine - chronic daily headache, progression from episodic to pattern of daily or near daily headaches - psychiatric comorbidities - cardiovascular complications

Question 269

Features of a tension-type headache

Answer 269

most prevalent primary headache disorder bilateral non-pulsatile mild to moderate pain w/ pressing or tightening quality tight-band around head not associated w/ aura, nausea/vomiting or aggravated by routine physical activity

Question 270

Tension-type headache classification

Answer 270

ICHD-3 criteria: Infrequent episodic = <1 day of headache per month Frequent episodic = 10+ episode of headache occurring on <15 days per month average, for >3months Chronic = >15 days of headache per month, for more than 3 months in absence of medication overuse

Question 271

Acute treatment for tension-type headahe

Answer 271

aspirin, paracetamol or NSAID

Question 272

Prophylaxis for tension-type headache

Answer 272

up to 10 session of acupuncture over 5-8wks low-dose amitriptyline non-pharmacological = relaxation training, biofeedback and CBT avoid triggers

Question 273

Cluster headache risk factors

Answer 273

male gender (3:1) smoking alcohol may trigger attack

Question 274

Features of cluster headache

Answer 274

pain - intense, stabbing pain around one eye typical occurs once or twice a day, episode lasting 15mins - 2hrs clusters typically last 4-12wks restlessness and agitation redness, nasal stuffiness, lacrimation, lid swelling minority may have miosis and ptosis

Question 275

Cluster headache investigations

Answer 275

neuroimaging, MRI w/ gadolinium contrast - brain lesion may be found

Question 276

Cluster headache classification

Answer 276

ICHD-3: A = at least 5 attacks fulfilling B-D B = severe or v. severe unilateral orbital, supraorbital and/or temporal pain 15-180mins C = either one or both 1. ipsilateral: conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhoea, eyelid oedema, forehead and facial sweating, miosis and/or ptosis. 2. sense of restlessness or agitation D = occurring w/ frequency between one every other day and 8 per day E = not better accounted by for another ICHD-3 diagnosis

Question 277

Cluster headache management

Answer 277

Acute = 100% oxygen, subcutaneous triptan Prophylaxis = verapamil seek specialist if pt develops headaches w/ respect to neuroimaging

Question 279

What is trigeminal autonomic cephalgia?

Answer 279

grp of conditions including cluster headache, paroxysmal hemicrania and short-lived unilateral neuralgiform headache w/ conjunctival injection and tearing (SUNCT) refer pts for specialist assessment

Question 280

Features of trigeminal neuralgia

Answer 280

unilateral disorder characterised by brief electric shock-like pains, abrupt in onset and termination, limited to 1 or more divisions of trigeminal nerve pain commonly evoked by light touch, occurs spontaneously small areas in nasolabial fold or chin may be particularly susceptible to precipitation of pain pains usually remit for variable periods

Question 281

Red flag symptoms suggesting serious underlying cause of trigeminal neuralgia

Answer 281

sensory changes deafness or other ear problems history of skin or oral lesions that could spread perineurally pain only in ophthalmic division of trigeminal nerve (eye socket, forehead and nose), or bilaterally optic neuritis FH of multiple sclerosis onset before <40

Question 282

Trigeminal neuralgia management

Answer 282

1st = carbamazepine failure to respond to treatment or atypical features eg <50 = refer to neuro

Question 283

What is Horner's syndrome?

Answer 283

rare sympathetic nervous system condition underlying cause varies eg tumours, injuries or neuro disorders treat depending on underlying cause

Question 284

Clinical features of Horner's syndrome

Answer 284

Ptosis - weakness of Muller's muscle Miosis on affected side Anihydrosis ipsilateral over forehead, face and neck regions May see heterochromia iridium rarely - enophthalmos

Question 285

Horner's syndrome diagnosis

Answer 285

thorough physical exam - pupil size assessment and response to light imaging tests - identify underlying cause

Question 286

Traumatic causes of peripheral nerve injuries / palsies

Answer 286

fractures and dislocations penetrating injuries stretch injuries

Question 287

Compressive causes of peripheral nerve injuries / palsies

Answer 287

carpal tunnel syndrome cubital tunnel syndrome

Question 288

Inflammatory causes of peripheral nerve injuries / palsies

Answer 288

vasculitis sarcoidosis

Question 289

Metabolic causes of peripheral nerve injuries / palsies

Answer 289

diabetes mellitus

Question 290

Infectious causes of peripheral nerve injuries / palsies

Answer 290

Lyme disease HIV

Question 291

Neoplastic causes of peripheral nerve injuries / palsies

Answer 291

direct invasion paraneoplastic syndromes

Question 292

Iatrogenic causes of peripheral nerve injuries / palsies

Answer 292

surgical procedures

Question 293

Risk factors of peripheral nerve injuries

Answer 293

advanced age presence of comorbid conditions eg diabetes or rheumatoid arthritis occupations involving repetitive movements high-risk sports substance use

Question 294

Peripheral nerve injury / palsy pathophysiology

Answer 294

- Initial mechanical insult that disrupts normal architecture of nerve - Wallerian degeneration distal to lesion site - breakdown of axon and myelin sheath, macrophage recruitment for debris clearance and Schwann cell proliferation forming Bangs of Bungner - Axonal regeneration and remyelination - Reinnervation - regenerated axons make functional connections w/ target tissues, but due to slow rate of regeneration, muscles may undergo atrophy or target organs may be lost before innervation occurs, leading to incomplete recovery of function

Question 295

What are Bands of Büngner?

Answer 295

formed when uninnervated Schwann cells proliferate and the remaining connective tissue basement membrane forms endoneurial tubes - act as guides for regenerating axons

Question 296

How does axonal regeneration and remyelination occur?

Answer 296

Regeneration initiated by changes in gene expression w/in injured neurons growth cones form at tips of regenerating axons, guided by chemical cues towards their target tissues Remyelination occurs due to Schwann cells enveloping new axon sprouts in layers to form a new myelin sheath

Question 297

Pathological changes in peripheral nerve injuries / palsies

Answer 297

nerve regeneration may lead to non-functional outcomes eg- Neuromas - disorganised growths of nerve tissue that can cause pain or sensory dysfunction Synkinesis - misdirection of regenerating axons towards incorrect end organs, resulting in simultaneous and uncoordinated movements

Question 298

What are the Seddon's 3 categories of peripheral nerve injury / palsy classification?

Answer 298

neuropraxia axonotmesis neurotmesis

Question 299

Peripheral nerve injuries / palsies: neuropraxia

Answer 299

mildest form of nerve injury - temporary blockage of nerve conduction w/out any anatomical disruption to nerve or its surrounding structure - transient motor and sensory loss - no Wallerian degeneration observed - rapid recovery w/in days to wks

Question 300

Peripheral nerve injuries / palsies: axonotmesis

Answer 300

damage to axon w/ preservation of endometrium, perineurium and perineurium typically caused by crush or stretch injuries - motor and sensory loss below inury lvl - Wallerian degeneration distal to injury site - potential for regeneration at rate of 1mm/day w/ variable recovery outcomes depending on severity and location of injury

Question 301

Peripheral nerve injuries / palsies: neurotmesis

Answer 301

most severe form, complete transection or disruption of nerve fibre including all its encapsulating structures - total motor and sensory loss below injury lvl - complete Wallerian degeneration - no spontaneous recovery, surgical intervention required for potential functional return

Question 302

What is Sunderland's classification of peripheral nerve injuries / palsies?

Answer 302

1st degree = neuropraxia 2nd degree = axonotmesis w/ preservation of endoneurium 3rd degree = axon and endometrium damage, intact perineurium and epineurium 4th degree = only epineurium intact, surgical intervention often required due to neuromas 5th degree = neurotmesis - complete transection requiring surgical repair or grafting

Question 303

Clinical features of peripheral nerve injuries / palsies

Answer 303

- Sensory deficits: loss of sensation or abnormalities - tingling, burning in distribution of affected nerve - Motor weakness: muscle weakness, fine motor skills compromised, deep tendon reflexes diminished / absent - Autonomic dysfunction: changes in skin temp, sweating abnormalities and trophic changes

Question 304

Peripheral nerve injury / palsy sensory deficit on examination

Answer 304

reduced or absent: tactile discrimination temp perception proprioception vibratory sensation pattern can help determine mononeuropathy or polyneuropathy

Question 305

Additional features of peripheral nerve injuries/ palsies

Answer 305

- may experience neuropathic pain described as burning, shooting or stabbing - spontaneous firing of damaged nerve fibres - Tinnel's - tingling sensation elicited by tapping injured nerve - Phalen's - wrist flexion causing parasthesia in median nerve distribution - long-standing peripheral nerve injury pts may develop compensatory changes eg modified gait or posture

Question 306

Peripheral nerve injury / palsies investigations

Answer 306

- Electrodiagnostic studies - nerve conduction studies (assess speed and degree of myelination) and electromyography (evaluates muscle activity to determine whether there's denervation) - MRI - identify lesions and tumours - High-resolution ultrasound - Bloods - DM, B12 deficiency, thyorid issues, connective tissues disorders

Question 307

Peripheral nerve injuries / palsies management

Answer 307

Non-surgical = pain - NSAIDs, opioids, gabapentin or pregabalin physiotherapy Surgical - for severe injuries eg grade 4 and 5 lesion, failed conservative treatment or presence of sharp penetrating trauma. Primary repair, nerve grafting or nerve transfers Follow-up and rehabilitation

Question 308

Causes of peripheral neuropathy

Answer 308

diabetes alcohol misuse vitamin deficiencies eg B12 and E hereditary disorders - Charcot-Marie-Tooth autoimmune eg Guillain-Barre syndrome infections eg Varicella zoster, HIV toxins malignancies idiopathic

Question 309

Risk factors of diabetic neuropathy

Answer 309

duration of diabetes glycaemic control age HTN dyslipidaemia smoking and alcohol use

Question 310

Causes of diabetic neuropathy

Answer 310

- hyperglycaemia-induced metabolic changes - chronic hyperglycaemia triggers series of metabolic changes - microvascular insufficiency - nerve hypoxia and ischaemia, damaging vasa nervorum - autoimmune response - lead to demyelination and axonal regeneration - inflammation - oxidative stress - directly damages, promotes inflammation, AGE formation and PKC activation

Question 311

Chronic hyperglycaemia leads to...

Answer 311

increased intracellular glucose w/in peripheral nerves, activating polyol pathway results in accumulation of sorbital and fructose, causing osmotic stress and reduced nerve conduction velocity Also causes advanced glycation end-products (AGEs) to form

Question 312

What do AGEs (advanced glycation end-products) do?

Answer 312

bind to their specific receptors (RAGE) on neuronal cells and evoke oxidative stress, inflammatory responses and apoptosis formed due to persistent hyperglycamia

Question 313

How does oxidative stress exacerbate nerve damage?

Answer 313

promotes... Mitochondrial dysfunction = mitochondrial electron transport chain becomes impaired leading to energy deficit in neurons DNA damage = reactive oxygen species (ROS) generated from oxidative stress cause direct DNA damage, including cell death pathways Lipid peroxidation = ROS also attack polyunsaturated fatty acids in cellular membranes, results in lipid perioxidation which alters membrane fluidity and function therefore contributing to neuronal injury

Question 314

What does chronic hyperglycaemia induce?

Answer 314

activation of protein kinase C, particularly PKCβ isoforms implicated in vasc abnormalities and neuronal dysfunction in diabetic neuropathy

Question 315

What happens to nerve growth factor in diabetic neuropathy?

Answer 315

production and utilisation of NGF is compromised deficiency leads to impaired nerve regeneration and contributes to degeneration of peripheral nerves

Question 316

Typical presentation of diabetic neuropathy

Answer 316

'stocking and glove' distribution of symptoms, characterised by bilateral numbness, tingling or burning sensations in hands and feet progression generally from distal to proximal

Question 317

Presenting features of diabetic neuropathy

Answer 317

Sensory - paraesthesia, dysesthesia, hypoesthesia or anaesthesia Motor - muscle weakness and atrophy Autonomic - gastroparesis, erectile dysfunction, neurogenic bladder, orthostatic hypotension, sudomotor dysfunction

Question 318

Initial investigations of diabetic neuropathy

Answer 318

Blood tests - FBC, renal function, liver function, TFT, B12, serum protein electorphoresis, HbA1c Urinalysis - microalbuminaemia

Question 319

Focused neurological investigations of diabetic neuropathy

Answer 319

Nerve conduction studies - speed of conduction of impulse through nerve Quantitative sensory testing - tests large and small fibre function Skin biopsy

Question 320

Diabetic neuropathy management

Answer 320

Glycaemic control Pain managment - tricyclic antidepressant or gabapentinoid Treat complications - check for ulcers, complete foot exams regularly Lifestyle modifications - diet, physical activity, stop smoking Referral to specialist

Question 321

Diabetic neuropathy complications

Answer 321

peripheral vascular disease gastroparesis cardiovascular autonomic neuropathy hypoglycaemia unawareness erectile dysfunction and urinary incontinence severity of complications usually correlates w/ degree of glucose control

Question 322

Mechanical causes of radiculopathies

Answer 322

disc herniation spinal stenosis spondylolisthesis trauma or injury

Question 323

Systemic conditions contributing to radiculopathies

Answer 323

diabetes mellitus - chronic hyperglycaemia infections - herpes zoster (shingles) malignancies

Question 324

Risk factors of radiculopathies

Answer 324

obesity tobacco use aging

Question 325

What are radiculopathies?

Answer 325

disorders affecting spinal nerves or nerve roots, leading to pain, numbness, weakness or difficulty controlling specific muscles

Question 326

Most common causes of radiculopathies in different parts of the spine

Answer 326

Cervical = degenerative changes that narrow space where nerve roots exit spine Lumbar = herniated disc Thoracic = variety of conditions eg herpes zoster

Question 327

Describe the initial pathogenesis of radiculpathies

Answer 327

Mechanical compression leads to decreased blood flow and ischaemia. Impairs axonal transport, leading to build-up of neurotoxic waste products and causing neuronal injury. Subsequent reperfusion leads to oxidative stress exacerbating injury Inflammatory mediators released from degenerated disc material or 2ndary to systemic conditions can incite infammatory response in nerve root. Mediators eg cytokines (IL-1) and TNF-alpha, causes vasodialtion, increased vasc permeability and recruitment of inflammatory cells Combo leads to oedema and further increases pressure on root

Question 328

Pathophysiology of radiculopathies

Answer 328

- Damage to nerve roots elicits electrochemical response: altered ion channel, ectopic discharges from demyelinated regions of neurons - Demyelination allows for abnormal cross-talk between sensory fibres, Ephaptic transmission - Neuronal injury and subsequent inflammation leads to nociceptor activation, Peripheral sensitisation - Central sensitisation results in heightened pain sensitivity (hyperalgesia) or pain response from non-painful stimuli (allodynia) - Chronic radiculopathies may result in structural changes in NS, may contribute to persistent pain even after resolution of initial causative pathology

Question 329

What is Ephaptic transmission?

Answer 329

cross-talk between sensory fibres that normally carry non-painful stimuli (large myelinated A-beta fibres) and those that carry painful stimuli (small unmyelinated C fibres and thinly myelinated A-delta fibres) contributes to sensation of pain in radiculopathies

Question 330

What are nociceptors?

Answer 330

sensory receptors that respond to potentially damaging stimuli by sending 'possible threat' signals to spinal cord and brain

Question 331

What is central sensitisation?

Answer 331

continued nociceptive input to CNS induces changes in dorsal horn neurons leads to increased response to peripheral stimuli results in hyperalgesia or allodynia

Question 332

What structural changes may occur in the nervous system due to chronic radiculopathies?

Answer 332

neuronal loss alterations in synaptic connectivity neurochemical changes

Question 333

Sensory symptoms of radiculopathies

Answer 333

pain - sharp, shooting, electric-like, follows dermatomal distribution paresthesia - tingling or prickling numbness hypersensitivity

Question 334

Motor symptoms of radiculopathies

Answer 334

muscle weakness muscle atrophy fasciculations / twitching

Question 335

Radiculopathies secondary symptoms

Answer 335

decreased reflexes gait abnormalities

Question 336

Radiculopathies investigation

Answer 336

Imaging: MRI, CT, x-ray Nerve conduction studies and electromyography Serological testing CSF analysis

Question 337

Radiculopathies differentials

Answer 337

- Peripheral neuropathies - usually symmetrical, no accompanying localised spinal pain, use NCS and EMG to differentiate - Myelopathies - signs more diffuse, UMN signs, MRI to distinguish - MSK conditions - pain usually exacerbated by specific movements or positions, present w/ localised pain and dysfunction

Question 338

Radiculopathies management

Answer 338

Pharmacological - NSAIDs for pain relief, corticosteroids Physiotherapy and pt education Lifestyle modifications Surgical interventions eg disectomy, laminectomy and spinal fusion

Question 339

Spinal cord injury risk factors

Answer 339

age gender alcohol and substance abuse osteoporosis

Question 340

Underlying causes of spinal cord injury

Answer 340

- trauma - diseases - cancer, arthritis, osteoporosis and inflammation - surgical complications - non-traumatic spinal cord ischaemia - aortic anyeursms/dissections, severe htn or aorta complications

Question 341

Primary mechanism of spinal cord injury

Answer 341

compression, contusion, laceration or transection severity - force and direction of impact physical disruption leads to immediate neural cell death in grey matter and axonal damage in white matter tracts vascular damage leading to haemorrhage and disruption of BBB can lead to 2ndary injury from biochemical cascades

Question 342

Secondary mechanism of spinal cord injury explained

Answer 342

- Inflammation - activation of resident microglia and astrocytes along infiltration by neutrophils and macrophages. Release cytokines and TNF-a, and reactive oxygen species causing further tissue damage - Excitotoxicity results from excessive release and impaired re-uptake of glutamate, causes persistent activation of NMDA receptors. Leads to intracellular calcium overload resulting in mitochondrial dysfunction and free radical generation - Apoptosis triggered by activation of caspases, increased by cytochrome C release from damaged mitochondria - Free radicals and perioxidation cause oxidative stress leading to damage to lipids, protein and nucleic acids in neural cells. This exacerbates inflammation and apoptosis causing further injury

Question 343

Secondary mechanism of spinal cord injury stages

Answer 343

Inflammation Excitotoxicity Apoptosis Free radical formation Lipid perioxidation

Question 344

Spinal cord injury classification based on level of injury

Answer 344

- Cervical: C1-C7, upper = C1-4, lower = C5-7, quadriplegia - Thoracic: T1-T12, paraplegia - Lumbar: L1-L5, loss of function in hips and legs - Sacral: S1-S5, loss of bowel and bladder function, sexual dysfunction

Question 345

Spinal cord injury classification based on severity

Answer 345

Complete = total loss of sensory and motor function below injury lvl Incomplete = some sensory or motor function remains below injury lvl. Degree of function depends on extent and location of damage

Question 346

Spinal cord injury classification based on clinical syndromes

Answer 346

Central cord syndrome Anterior cord syndrome Brown-Sequard syndrome Conus medullaris syndrome Cauda equina syndrome

Question 347

What is central cord syndrome?

Answer 347

greater motor impairment in upper limbs than lower limbs bladder dysfunction varying degree of sensory loss below injury lvl

Question 348

What is anterior cord syndrome?

Answer 348

damage to anterior part of spinal cord, results in loss of motor function and pain and temp sensation, but preservation of proprioception and touch

Question 349

What is Brown-Sequard syndrome?

Answer 349

damage to one half of spinal cord results in loss of motor function, proprioception and vibration sense ipsilaterally and loss of pain and temp sensation contralaterally

Question 350

What is conus medullaris syndrome?

Answer 350

injury at terminal part of spinal cord, leading to areflexic bowel, bladder and lower limbs

Question 351

What is cauda equina syndrome?

Answer 351

injury to lumbar and sacral nerve roots resulting in areflexic bowel, bladder, variable motor/sensory loss in lower limbs

Question 352

Typical presentation of spinal cord injury

Answer 352

sudden onset of neurological deficits, primarily motor and sensory dysfunction, which are often associated w/ pain or discomfort in back, neck or head

Question 353

Clinical features of spinal cord inury

Answer 353

Motor dysfunction - weakness or paralysis below lesion lvl, spasticity, hyperreflexia, Babinski sign Sensory dysfunction - sensation, pain, temp and proprioception below lesion lvl Pain Autonomic dysregulation - cardiovasc instability, resp compromise, bladder dysfunction, sexual dysfunction Bulbar symptoms - dysphagia and dysarthria

Question 354

What is spinal shock?

Answer 354

in acute phase after injury, characterised by flaccid paralysis, reflexes loss, sensation loss below injury lvl temporary, lasts from several hrs - several wks

Question 355

Spinal cord injury investigations

Answer 355

radiographs - anterior-posterior and lateral cervical spine CT MRI SSEP - somatosensory evoked potentials - integrity of sensory pathways MEP - motor evoked potentials - motor pathway function Electromyography (EMG) and nerve conduction studies

Question 356

Spinal cord injury management

Answer 356

Acute resuscitation Steroid therapy - high-dose methylprednisolone w/in 8hrs of injury Surgical management - decompression and stabilisation Rehabilitation Long term care of chronic complications

Question 357

Spinal cord injury complications

Answer 357

Neuro: spinal. shock, autonomic dysreflexia, syringomyelia Pulmonary: ventilatory impairment, pneumonia GI: gastric dilatation and ileus, bowel dysfunction MSK and skin: osteoporosis, fractures, pressure ulcers GU: neurogenic bladder Pain and psychological: chronic pain, depression, anxiety, PTSD

Question 358

What is spinal stenosis?

Answer 358

narrowing of part of spinal canal, resulting in compression of spinal cord or nerve roots usually affects cervical or lumbar spine common in pts over 60

Question 359

Where is spinal stenosis most commonly found?

Answer 359

Lumbar

Question 360

What are the types of spinal stenosis?

Answer 360

Central - narrowing central spinal canal Lateral - narrowing nerve root canals Foramina - narrowing intervertebral foramina

Question 361

Causes of spinal stenosis

Answer 361

- congenital - degenerative changes - facet joint changes, disc disease and bone spurs - herniated discs - thickening of ligamenta flava or pos. longitudinal ligament - spinal fractures - spondylolisthesis - tumours

Question 362

Symptoms of central spinal stenosis

Answer 362

gradual onset intermittent neurogenic claudication - lower back pain - buttock and leg pain - leg weakness occur w/ standing and walking bending forward improves, standing straight worsens

Question 363

Symptoms of lateral and foramina stenosis

Answer 363

sciatica

Question 364

Spinal stenosis investigations

Answer 364

MRI investigations to exclude peripheral arterial disease - ankle-brachial pressure index, CT angiogram

Question 365

Spinal stenosis management

Answer 365

exercise and weight loss analgesia physiotherapy decompression surgery where conservative treatment fails laminectomy

Question 366

What is a laminectomy?

Answer 366

removal of part or all of lamina from affected vertebra laminae = bony parts that form posterior part of vertebral foramen, attaches to spinous process

Question 367

What is cauda equina syndrome?

Answer 367

rare neurosurgical/orthopaedic emergency compression of cauda equina leads to bladder, bowel and/or sexual dysfunction w/ peri-anal sensory loss

Question 368

Common causes of cauda equina syndrome

Answer 368

lumbar disc herniation degenerative lumbar canal stenosis neoplastic space occupying lesion spinal trauma

Question 369

Uncommon causes of cauda equina syndrome

Answer 369

infection haematoma spina bifida late-stage ankylosing spondylitis neurosarcoidosis inferior vena cava thrombosis spinal haemorrhage

Question 370

Cauda equina syndrome pathology

Answer 370

distal to conus medullaris, lower lumbar nerve roots, sacral nerve roots and coccygeal nerve root continue to travel through vertebral canal to respective exit foramina as part of nerve bundle - cauda equina cauda equina stops growing at age 4, vertebral column continues to lengthen

Question 371

What are the neurones w/in the causa equina responsible for?

Answer 371

sensory and motor innervation to lower limbs sensory innervation to saddle area motor innervation to anal sphincters parasympathetic innervation to bladder

Question 372

Why are the nerve roots in the cauda equina highly susceptible to injury?

Answer 372

compared to peripheral nerves, they have a poorly developed epineurium surrounding their sheaths and don't have a segmental blood supply

Question 373

Damage to the cauda equina nerve roots may result in...

Answer 373

reduces sensation or sensory loss lower motor neurone signs and symptoms - hyporeflexia or areflexia, hypotonia or atonia, flaccid weakness or paralysis, local muscle atrophy, fasciculations

Question 374

What are the patterns of onset in cauda equina syndrome?

Answer 374

Acute = sudden onset, rapidly progressing symptoms which worsen over several hrs or days Chronic = insidious onset w/ slow progression of symptoms

Question 375

What are the two groups of cauda equina syndrome symptoms?

Answer 375

Core diagnostic: urinary, bowel, sexual dysfunction, saddle anaesthesia Accompanying symptoms: lower back pain w/ or w/out sciatica, lower limb sensory loss, lower limb lower motor neurone signs (can be unilateral or bilateral)

Question 376

Cauda equina syndrome classification

Answer 376

Cauda equina syndrome w/ retention Incomplete cauda equina syndrome

Question 377

Cauda equina syndrome investigations

Answer 377

Diagnosis suspected after identification of saddle anaesthesia, bladder, bowel and sexual dysfunction urgent MRI once confirmed diagnosis, further investigations of blood tests and lumbar puncture

Question 378

Cauda equina syndrome differentials

Answer 378

- conus medullaris syndrome - herniated lumbar disc - degenerative lower back pain +/- sciatica - spinal cord compression - neoplastic spinal lesions - lumbar radiculopathy

Question 379

Cauda equina syndrome management

Answer 379

if cause is suspected to be traumatic, pts spine should be immobilised surgical decompression if primary or metastatic malignancy = IV dexamethasone prior to surgical intervention to reduce oedema

Question 380

Who is suitable for spinal decompressive surgery?

Answer 380

- cauda equina syndrome - spinal trauma and fractures - haematomas - space occupying lesions w/ radiological imaging indicating likely surgical removal - spinal stenosis

Question 381

Who is not suitable for surgical decompression surgery?

Answer 381

inflammory disease eg late stage ankylosing spondylitis infection spinal neoplastic disease

Question 382

Cauda equina syndrome prognosis

Answer 382

late diagnosis and delayed treatment increases risk of permanent neuro deficit in pts may be left w/ paralysis of lower limbs or permanent bladder, bowel and sexual dysfunction

Question 383

What is Meniere's disease?

Answer 383

disorder of inner ear of unknown cause characterised by excessive pressure and progressive dilation of endolymphatic system more common in middle-aged adults but may be any age

Question 384

Risk factors of Meniere's disease

Answer 384

age: 20-50 gender: some studies suggest higher prevalence in women family history: genetic predisposition

Question 385

Clinical features of Meniere's disease

Answer 385

recurrent episodes of vertigo, tinnitus and hearing loss sensation of aural fullness or pressure nystagmus +ve Romberg test episodes lasting mins - hrs

Question 386

Meniere's disease management

Answer 386

ENT assessment to confirm inform DVLA acute attacks = buccal or IM prochlorperazine prevention = betahistine and vestibular rehab exercises

Question 387

Meniere's disease prognosis

Answer 387

symptoms resolve in majority of pts after 5-10yrs majority of pts left w/ degree of hearing loss psychological distress is common

Question 388

What is chronic fatigue syndrome?

Answer 388

persistent fatigue that's not relieved by rest, often accompanied by other symptoms eg muscle pain, headaches and cognitive impairment

Question 389

Chronic fatigue syndrome diagnosis criteria

Answer 389

1. Substantial reduction or impairment in ability to engage in pre-illness lvls of occupational, educational, social or personal activities that persists for >6 months and is accompanied by fatigue 2. Post-exertional malaise 3. Unrefreshing sleep despite adequate hrs spent asleep 4. Cognitive impairment affecting attention, memory, information processing speed or executive function 5. orthostatic intolerance manifested by lightheadedness, dizziness or fainting upon standing up

Question 390

Clinical features of chronic fatigue syndrome

Answer 390

Immune = recurrent sore throat, tender lymph nodes, muscle pain, joint pain w/out swelling, flu-like symptoms Neuro = headaches, photophobia, phonophobia, blurred vision, balance and coordination issues Autonomic = neurally mediated htn, POTS, palpitations and GI disturbances Cognitive = issues w/ concentration, memory, information processing speed, attention Sleep disturbances Pain syndromes = fibromyalgia, temporomandibular joint disorder, interstitial cystitis, chronic pelvic pain

Question 391

NICE recommend we suspect CFS if:

Answer 391

- person has all of persistent symptoms for min. 6wks in adults and 4wks in children - person's ability to engage in occupational, educational, social or personal activities is significantly reduced from pre-illness levels - symptoms not explained by another condition

Question 392

Chronic fatigue syndrome management

Answer 392

- refer to CFS specialist - energy management: self-management strategy - physical activity and exercise based on a specific programme - cognitive behavioural therapy - supportive

Question 393

What is Wernicke's encephalopathy?

Answer 393

condition of acute thiamine (B1) deficiency most commonly associated w/ chronic alcoholics, can occur secondary to other causes of malnutrition

Question 394

Causes of Wernicke's encephalopathy

Answer 394

Alcohol abuse: high carb load leads to high thiamine requirement Reduced thiamine absorption eg fasting, severe diarrhoea, bariatric surgery, hyperemesis increased metabolic requirements

Question 395

Wernicke's encephalopathy paathophyisiology

Answer 395

thiamine is essential in metabolisis of carbohydrates and lipids - Krebs cycle coenzyme Insufficent glucose metabolism causes oxidative stress and mitochonrial dysfunction - localised area of low pH due to lactic acidosis 2nd to metabolic dysfunction further damage neural tissue - thiamine deficiency also associated w/ deranged glutamate transport and resultant neural excitotoxicity Neurotoxic effects are maximal in areas of brain that require highest glucose turnover, have highest thiamine demands

Question 396

Where does Wernicke's encephalopathy lead to neural damage?

Answer 396

mamillary body areas of brainstem associated w/ CN3, 4, 6, 8 and 10 nuclei

Question 397

How does the damage in Wernicke's encephalopathy lead to the triad of symptoms?

Answer 397

- damage to cranial nerve nuclei controlling ocular muscles = ophthalmoplegia - damage to cerebellar vermis and motor cortical tracts = ataxia diffuse nature of damage = variable changes in mental state

Question 398

Symptoms of Wernicke's encephalopathy

Answer 398

Ophthalmoplegia Nystagmus Ataxia Cognitive: confusion, memory deficits, apathy or agitation hypothermia altered consciousness GI symptoms tachycardia

Question 399

Wernicke's encephalopathy investigations

Answer 399

diagnosed clincally on presence of 1 or more of triad (due to risk of harm through delayed treatment) Serum thiamine available, but normal result doesn't rule out WE

Question 400

Wernicke's encephalopathy differential diagnosis

Answer 400

delirium tremens hepatic encephalopathy stroke normal pressure hydrocephalus

Question 401

Wernicke's encephalopathy management

Answer 401

urgent administration of parenteral (not oral) thiamine for minimum 5 days thiamine must be administered before or concurrently w/ any glucose administration

Question 402

What is myasthenia gravis?

Answer 402

chronic autoimmune neuromuscular disease characterised by fluctuating muscle weakness and fatigue, increases during period of activity and improves after rest

Question 403

Myasthenia gravis causes

Answer 403

Genetic Environmental - infections (EPV, H.pylori), hormones, drugs Thymomas Autoimmunity Autoimmune disorders

Question 404

Myasthenia gravis genetics

Answer 404

HLA-B8, DR3 haplotype = early onset HLA-B7, DR15/16 = late-onset non-HLA = PTPN22 and CTLA4

Question 405

What drugs may exacerbate myasthenia gravis?

Answer 405

penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics - gentamicin, macrolides, quinolones, tetracyclines

Question 406

Myasthenia gravis autoantibodies

Answer 406

autoantibodies target nicotinic AChR at post-synaptic neuromuscular junction autoantibodies produced by B cells under T-helper cells' influence, leading to complement-mediated destruction of AChR and neuromuscular transmission impairment MuSK antibodies may be identified, which is involved in clustering ACRs at NMJs. LRP4 also sometimes found in MG pts

Question 407

Myasthenia gravis pathophysiology

Answer 407

Production of autoantibodies against components of NMJ, particularly nicotinic acetylcholine receptor Binding to nAChRs initiate: - complement activation leading to membrane attack complex formation - cross-linking and subsequent endocytosis of nAChRs - functional blockage of acetylcholine binding Results in reduction in no and function of nAChRs at post-synaptic membrane Diminished nAChR density leads to decreased safety factor for NM transmission, which manifests as muscle weakness

Question 408

What are the autoantibodies usually produced against NMJ in myasthenia gravis?

Answer 408

IgG1 and IgG3 subclasses produced by B cells under direction of T-helper cells

Question 409

Clinical features of myasthenia gravis

Answer 409

fluctuating muscle weakness that worsens w/ repetitive activity ocular, bulbar and limb muscles - ptosis - diplopia - dysarthria - dysphagia - proximal limb weakness can vary from generalised weakness to resp muscle involvement

Question 410

Myasthenia gravis investigations

Answer 410

- Electrophysiological studies: repetitive nerve stimulation (RNS), single-fiber electromyography (SFEMG) - Serological testing: AChR, MuSK, LRP4 - imaging: CT or MRI (look for thymoma) - edrophonium test

Question 411

Myasthenia gravis differentials

Answer 411

Lambert-Eaton syndrome Guillain-Barre syndrome Botulism

Question 412

Myasthenia gravis pharmacological management

Answer 412

Pyridostigmine - ACh inihibitor Immunosuppresive agents - corticosteroids (pred, azathioprine) IVIg - severe cases or during crisis consider monoclonal antibodies eg rituximab in refractory cases

Question 413

Other management of myasthenia gravis

Answer 413

Surgical - thymectomy In pregnancy, close monitoring needed due to potential exacerbations Pts should receive vaccinations as per guidelines, live vaccines used w/ caution

Question 414

Myasthenic crisis management

Answer 414

immediate hospitalisation intensive resp support - intubation and mechanical ventilation rapid short term control by plasma exchange or IVIg

Question 415

What is myasthenic crisis?

Answer 415

acute exacerbation of muscle weakness, leads to resp failure. Requires immediate intervention w/ vent support and plasmapheresis or IVIg

Question 416

Myasthenia gravis complications

Answer 416

myasthenic crisis resp infections aspiration pneumonia thymoma cortcosteroid complications cholinergic crisis mood disorders

Question 417

What is cholinergic crisis?

Answer 417

overmedication w/ anticholinesterase drugs can cause muscle fasciculations, increased salivation, diarrhoea and bradycardia

Question 418

What are the cranial nerve palsies?

Answer 418

3rd = at rest eye points 'down and out' 4th = upwards and inwards, worse looking up and down 6th = medially deviated, worse looking side to side

Question 419

Cranial nerve palsies investigations

Answer 419

CT / MRI to look for compressive leisons Bloods LP Nerve conduction studies

Question 420

Cranial nerve palsy treatment

Answer 420

treat underlying cause blood sugar control in diabetic neuropathy immunosuppression in MS

Question 421

Spinal cord compression

Answer 421

back pain weakness below lvl urinary / faecal incontinence constipation hypertonia hyperreflexia clonus sensory loss UMN symptoms

Question 422

Spinal cord compression investigation and management

Answer 422

MRI whole spine Tx = major trauma centre referral, immobilise pt If metastatic, give high dose steroids, PPI and surgical decompression

Question 423

What is neurofibromatosis?

Answer 423

grp of genetic disorders characterised by growth of neurofibromas - benign tumours that affect nerve tissue

Question 424

Typical presentation of neurofibromatosis

Answer 424

cafe-au-lait macules (pigmented patches appearing at birth or early childhood) and neurofibromas (benign tumours that develop from nerve sheath, may not be apparent until later in life)

Question 425

Clinical features of neurofibromatosis

Answer 425

Cutaneous: cafe-au-lait macules, neurofibromas, freckling Ocular: Lisch nodules, optic pathway gliomas Skeletal: scoliosis, pseudarthrosis Neuro: learning disability, epilepsy, malignant nerve sheath tumours

Question 426

Diagnostic criteria for neurofibromatosis type 1

Answer 426

2 or more of: - 6+ cafe-au-lait macules >5mm in diameter in prepubertals and >15mm in postpubertals - 2+ neurofibromas - optic glioma - 2+ lisch nodules - distinctive osseous lesion - 1st degree relative w/ NF1

Question 427

Diagnostic criteria for neurofibromatosis type 2

Answer 427

less common than NF1, either: - bilateral vestibular schwannomas, diagnosed using MRI - 1st degree relative w/ NF2 and either unilateral vestibular schwannoma before 30, or any 2 of: meningioma, glioma, schwannoma, cataract / juvenile posterior subscapular lenticular opacity

Question 428

Neurofibromatosis medical management

Answer 428

analgesia - NSAIDs or opioids antihypertensives - ACE inhibitors or CCBs for HTN 2nd to renal artery stenosis malignant peripheral nerve sheath tumours - require surgery, radiation and chemo. MEK inhibitor selumetinib also effective

Question 429

Neurofibromatosis surgical management

Answer 429

plexiform neurofibromas - surgical debulking, attempt complete resection (may not be possible due to infiltrative nature) optic pathway gliomas - surgery, but can have risk due to proximity to critical structures scoliosis - orthopaedic intervention, spinal fusion

Question 430

Other management of neurofibromatosis

Answer 430

ophthalmological = lisch nodules - regular assessments psychological and educational support - increased risk of learning disabilities, ADHD and autism Genetic counselling - autosomal dominant inheritance pattern

Question 431

Neurofibromatosis complications

Answer 431

cutaneous and subcutaneous neurofibromas plexiform neurofibromas optic pathway gliomas skeletal abnormalities cognitive impairments CV issues aqueductal stenosis and hydrocephalus meningiomas and astrocytomas GISTs

Question 432

What is the triad of normal pressure hydrocephalus?

Answer 432

gait disturbance cognitive impairment urinary incontinence

Question 433

Causes of normal pressure hydrocephalus

Answer 433

idiopathic 2ndary: - subarachnoid haemorrhage - meningitis - traumatic brain injury - intracranial tumours - ventricular shunting or lumbar puncture

Question 434

Normal pressure hydrocephalus pathophysiology

Answer 434

- imbalance between production and absorption of CSF, leads to progressive ventricular dilation - ventricular enlargement exerts mechanical stress on surrounding periventricular white matter tracts. Leads to stretching and compression of fibres, particularly frontal white matter tracts - mechanical stress also disrupts BBB integrity w/in periventricular regions, induces localised inflammation - inflammatory response further exacerbates white matter damage, leading to gliosis and demyelination. Chronic inflammation may impair glymphatic clearance of interstitial solutes - ventricular dilation and subsequent brain parencyma compression can lead to alterations in cerebral perfusion. Reduced cerebral blood flow and impaired autoregulation

Question 435

Normal pressure hydrocephalus pathophysiology summarised

Answer 435

ventricular dilation periventricular stress disruption of blood-brain barrier inflammatory response cerebral perfusion changes

Question 436

How do the pathophysiological changes in normal pressure hydrocephalus correlate w/ clinical manifestations

Answer 436

gait disturbance - damage to motor pathways, especially corticospinal tract cognitive impairment - disruption of fronto-striatal circuits urinary incontinence - dysfunction of frontal lobes and periventricular white matter tracts involved in incontinence

Question 437

Clinical features of normal pressure hydrocephalus

Answer 437

gait disturbance - unsteady, wide based gait, 'magnetic' cognitive impairment - deficits in attention, concentration and executive functions urinary incontinence - urgency and frequency also headaches, changes in personality or behaviour

Question 438

Normal pressure hydrocephalus investigations

Answer 438

imaging - hydrocephalus w/ and enlarged 4th ventricle, in addition to ventriculomegaly there's absence of substantial sulcal atrophy

Question 439

Normal pressure hydrocephalus differential diagnosis

Answer 439

alzheimer's parkinson's vascular dementia

Question 440

Normal pressure hydrocephalus management

Answer 440

- ID suitable candidates for intervention via cranial imaging and invasive testing eg LP - shunt surgery - ventriculoperitoneal shunt w/ adjustable valve systems - post-op follow up and regular monitoring - non-surgical management = lifestyle modifications eg avoid medications that exacerbate, physiotherapy

Question 441

What medications can exacerbate normal pressure hydrocephalus?

Answer 441

sedatives anticholinergics