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Neurology > Neurology Important Parts > Flashcards

Neurology Important Parts Flashcards

1
Q

Symptoms of cerebellar disease:

A

D - dysdiadochokinesia, dysmetria, ‘drunk’
A - ataxia
N - nystagmus
I - intention tremor
S - slurred staccato speech, scanning dysarthria
H - hypotonia

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2
Q

Causes of cerebellar syndrome:

A
  • Friedreich’s ataxia, ataxic telangiectasia
  • neoplastic: cerebellar haemangioma
  • stroke
  • alcohol
  • multiple sclerosis
  • hypothyroidism
  • drugs: phenytoin, lead
  • paraneoplastic
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3
Q

What is cerebral perfusion pressure?

A

net pressure gradient causing blood to flow to the brain
-sharp rise may result in rising ICP
-fall may result in cerebral ischaemia
CPP = mean arterial pressure - ICP

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4
Q

Total volume of CSF:

A

150ml

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5
Q

Where is CSF produced?

A

ependymal cells in choroid plexus or blood vessels

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6
Q

Circulation of CSF:

A
  • lateral ventricles (foramen of Munro)
  • 3rd ventricle
  • cerebral aqueduct (aqueduct of Sylvius)
  • 4th ventricle (via foramina of Magendie and Luschka)
  • Subarachnoid space
  • reabsorbed into venous system via arachnoid granulations into superior sagittal sinus
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7
Q

Composition of CSF:

A
  • glucose
  • protein
  • no RBC
  • WBC
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8
Q

Charcot-Marie-Tooth disease

A
  • most common hereditary peripheral neuropathy
  • no cure
  • Hx of sprained ankles
  • foot drop
  • high arched feet
  • hammer toes
  • distal muscle weakness
  • distal muscle atrophy
  • hyporeflexia
  • stork leg deformity
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9
Q

In whom are cluster headaches more common and what can trigger them?

A
  • men 3:1
  • smokers
  • alcohol can trigger attack
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10
Q

Features of cluster headaches:

A
  • pain one or twice a day with episodes 15 min - 2 hours
  • clusters 4-12 weeks
  • pain around eye
  • always on same side
  • redness, lacrimation, lid swelling
  • nasal stuffiness
  • some miosis and ptosis
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11
Q

Management of cluster headaches:

A
  • acute: 100% oxygen, subcutaneous triptan
  • prophylaxis: verapamil
  • neurologist advice
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12
Q

CNS tumours:

A
  • 60%: glioma and metastatic
  • 20%: meningioma
  • 10%: pituitary lesions
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13
Q

What is the most common paediatric CNS tumour?

A
  • used to be medulloblastomas

- now astrocytomas

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14
Q

CNS tumour diagnosis:

A

MRI scanning

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15
Q

Features of a common peroneal nerve lesion:

A
  • weakness of foot dorsiflexion
  • weakness of foot eversion
  • weakness extensor hallucinate longus
  • sensory loss over dorsum of foot and lower lateral part of leg
  • wasting of anterior tibial and peroneal muscles
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16
Q

What is Creutzfeldt-Jakob disease?

A
  • rapidly progressive neurological condition caused by prion proteins
  • induce formation of amyloid folds - tightly packed beta pleated sheets resistant to proteases
  • rapid dementia and myoclonus
  • normal CSF, EEG biphasic high amplitude sharp waves, MRI hyper intense signals in basal ganglia and thalamus
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17
Q

Features of degenerative cervical myelopathy:

A
  • pain (neck, upper or lower limbs)
  • loss of motor and sensory function
  • loss of autonomic function
  • Hoffman’s sign - reflex
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18
Q

Drugs causing peripheral neuropathy:

A
  • amiodarone
  • isoniazid
  • vincristine
  • nitrofurantoin
  • metronidazole
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19
Q

Dystrophinopathies:

A
  • x-linked recessive
  • mutation in gene encoding dystrophin, dystrophin gene on Xp21
  • dystrophin is part of large membrane associated with protein in muscle which connects muscle membrane to actin
  • Duchenne and Becker
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20
Q

Duchenne muscular dystrophy:

A
  • frameshift mutation with one or both binding sites so severe from
  • progressive proximal muscle weakness from 5 years
  • calf pseudohypertrophy
  • Gower’s sign: child uses arms to stand up from squat
  • 30% intellectual impairment
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21
Q

Becker muscular dystrophy:

A
  • non-frameshift insertion in dystrophin gene so both binding sites preserved and milder form
  • develops after the age of 10 years
  • intellectual impairment much less common
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22
Q

EMG signs in neuropathy:

A
  • increased action potential duration

- increased action potential amplitude

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23
Q

EMG signs in myopathy:

A
  • decreased action potential duration

- decreased action potential amplitude

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24
Q

EMG signs in myasthenia gravis:

A

diminished response to repetitive stimulation

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25
Q

EMG signs in Lambert-Eaton syndrome:

A

incremental response to repetitive stimulation

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26
Q

EMG signs in Myotonic syndromes:

A

extended series of repetitive discharges lasting up to 30 seconds

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27
Q

Features of encephalitis:

A
  • fever, headache, psych symptoms, seizures, vomiting
  • focal features e.g. aphasia
  • peripheral lesions (cold sores) have no relation to HSV encephalitis
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28
Q

What is encephalitis caused by?

A
  • HSV1 responsible for 95% of cases

- temporal and inferior frontal lobes

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29
Q

Management of encephalitis:

A

IV acyclovir in all cases of suspected encephalitis

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30
Q

What is an essential tremor:

A
  • auotosmal dominant condition
  • both upper limbs
  • postural tremor worse if arms outstretched
  • improved by alcohol and rest
  • most common cause of titubation (head tremor)
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31
Q

Management of essential tremor:

A
  • propranolol first line

- primidone

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32
Q

What is an extradural heamatoma and what does it most commonly affect:

A
  • between skull and dura
  • typically low impact trauma
  • temporal region
  • pterion overlies middle meningeal artery
  • biconvex (lentiform)
  • no neurological deficit: clinical and radiological observation
  • craniotomy and evacuation of heamatoma
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33
Q

Lucid interval in head trauma:

A
  • initial brief loss of consciousness, regains in lucid interval and lost again due to expanding haematoma and brain herniation
  • temporal lobe herniates around tentorium cerebella -fixed and dilated pupil due to compression of parasympathetic fibres of third cranial nerve
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34
Q

What does the facial nerve supply?

A
  • structures of second embryonic branchial arch
  • efferent nerve to muscles of facial expression, digastric and glandular structure (salivary glands and lacrimal glands)
  • few afferent fibres originating in cells of vehicular ganglion (taste)
  • ear: nerve to stapedius
  • anterior 2/3 of tongue
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35
Q

Causes of bilateral facial nerve palsy:

A
  • sarcoidosis
  • Guillain Barre syndrome
  • Lyme disease
  • bilateral acoustic neuromas
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36
Q

Causes of unilateral facial nerve palsy:

A
  • causes of bilateral
  • LMN: Bell’s palsy, Ramsay Hunt syndrome (herpes zoster), acoustic neuroma, parotid tumours, HIV, MS, DM
  • UMN: stroke, MS
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37
Q

How does LMN vs UMN palsy appear:

A
  • UMN: spares upper face (forehead)

- LMN: all facial muscles

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38
Q

What is foot drop?

A

weak dorsiflexors

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39
Q

Causes of foot drop:

A
  • common peroneal nerve lesion
  • L5 radiculopathy
  • sciatic nerve lesion
  • superficial or deep perineal nerve lesion
  • other: central nerve lesions
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40
Q

What is a common peroneal nerve lesion caused by:

A
  • secondary to compression at neck of fibula

- certain positions, prolonged confinement, weight loss, Baker’s cysts and plaster casts

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41
Q

Examination of common peroneal nerve lesion:

A
  • if isolated peroneal neuropathy - weakness of foot dorsiflexion and eversion
  • normal reflexes
  • wekaness of hip abduction suffusive of L5 radiculopathy
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42
Q

Friedreich’s ataxia:

A
  • most common of early onset hereditary ataxia
  • autosomal recessive
  • trinucleotide repeat disorder characterised by GAA repeat in X25 gene on chromosome 9 (frataxin)
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43
Q

Features of Friedreich’s ataxia:

A
  • typical age of onset 10-15yo
  • gait ataxia and kyphoscoliosis most common presenting features
  • neurological: absent ankle jerks/extensor plantars, cerebellar ataxia, optic atrophy, spinocerebellar tract degeneration
  • HOCM (most common cause of death)
  • diabetes mellitus
  • high arched palate
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44
Q

GCS motor response:

A
  1. obeys commands
  2. localises to pain
  3. withdraws from pain
  4. abnormal flexion to pain (decorticate posture)
  5. extending to pain
  6. none
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45
Q

GCS verbal response:

A
  1. orientated
  2. confused
  3. words
  4. sounds
  5. none
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46
Q

GCS eye opening:

A
  1. spontaneous
  2. to speech
  3. to pain
  4. none
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47
Q

What is Guillain-Barre syndrome?

A
  • immune mediated demyelination of peripheral nervous system
  • often triggered by infection (Campylobacter jejuni)
  • cross reaction of Ab with gangliosides in peripheral nervous sytem
  • correlation between anti-ganglioside Ab (e.g. anti-GM1) and clinical features
  • anti-GM1 Ab in 25%
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48
Q

Miller Fisher syndrome:

A
  • variant of Guillain-Barre
  • opthalmoplegia, areflexia and ataxia
  • eye muscles affected first
  • descending paralysis rather than normal ascending
  • anti-GQ1b Ab present in 90%
49
Q

Guillain Barre initial symptoms:

A

back/leg pain in early stages

50
Q

Progressive Guillain Barre syndrome:

A
  • progressive symmetrical weakness of all limbs
  • ascending
  • proximal muscles usually affected before distal
  • reflexes are reduced or absent
  • sensory symptoms mild (e.g. distal paraesthesia)
51
Q

Other features of Guillain Barre:

A
  • history of gastroenteritis
  • respiratory muscle weakness
  • cranial nerve involvement: diplopia, bilateral facial nerve palsy, oropharyngeal weakness
  • autonomic involvement: urinary retention, diarrhoea
  • less common: papilloedema
52
Q

Investigations of Guillain Barre:

A
  • lumbar puncture: rise in protein with normal WCC

- nerve conduction studies

53
Q

Migraine:

A
  • recurrent, severe, unilateral throbbing
  • may have aura, nausea and photosensitivity, phonophobia
  • aggravated by routine activities
  • women - menstruation
  • attacks up to 72 hours
  • precipitated by aura (visual, progressive, 5-60 minutes, transient hemianopia disturbance or spreading scintillating Scotoma)
54
Q

Tension headache:

A
  • recurrent, non-disabling, bilateral headache, often described as a tight band
  • not aggravated by routine activities
55
Q

Cluster headache:

A
  • pain once or twice per day
  • intense pain around one eye (always same side)
  • restless during attack)
  • redness, lacrimation, lid swelling
  • more common in men and smokers
56
Q

Temporal arteritis:

A
  • > 60yo
  • rapid onset of unilateral headache
  • jaw claudication
  • tender, palpable temporal artery
  • raised ESR
57
Q

Medication overuse headache:

A
  • 15 days or more per month
  • developed or worsened whilst taking regular symptomatic medication
  • opioids an triptans more at risk
  • psychiatric co-morbidity
58
Q

Acute single episode causes of headache:

A
  • meningitis
  • encephalitis
  • SAH
  • head injury
  • sinusitis
  • glaucoma
  • tropical illness e.g. malaria
59
Q

Chronic headache causes:

A
  • chronically raised ICP
  • Paget’s disease
  • psychological
60
Q

Headache red flags:

A
  • compromised immunity
  • under 20 or Hx malignancy
  • vomiting without obvious cause
  • worsening headache with fever
  • thunderclap
  • new onset neuro deficit
  • new onset cognitive dysfunction
  • change in personality
  • impaired consciousness
  • recent head trauma (3mo)
  • headache triggered by cough, valsalva, sneeze or exercise
  • orthostatic headache
  • giant cell arteritis or acute narrow angle glaucoma symptoms
  • substantial change in headache characteristics
61
Q

Which lobes does HSV typically affect?

A

temporal lobes (inferior frontal)

62
Q

Features of HSV encephalitis:

A
  • fever, headache, psychiatric, seizures, vomiting
  • focal features e.g. aphasia
  • perpiheral lesions have no relation to presence of HSV encephalitis
63
Q

Which HSV typically causes encephalitis:

A

HSV1

64
Q

Investigations for HSV encephalitis:

A
  • CSF: lymphocytosis, protein
  • PCR
  • CT
  • MRI better
  • EEG: lateralised periodic discharges at 2Hz
65
Q

Treatment HSV encephalitis:

A

IV acyclovir

66
Q

What is HSMN

A
  • hereditary sensorimotor neuropathy
  • encompasses Charcot marmite Tooth disease
  • type I - demyelinating pathology
  • type II - axonal pathology
67
Q

HSMN type I

A
  • autosomal dominant
  • due to defect in PMP-22 gene (myelin)
  • features at puberty
  • motor symptoms
  • distal muscle wasting, pes cavus, clawed toes
  • foot drop, leg weakness often first features
68
Q

What is Huntington’s disease?

A
  • inherited neurodegenerative condition
  • progressive and incurable
  • death 20 years after symptoms develop
  • autosomal dominant
  • trinucleotide repeat disorder (CAG)
  • degeneration of cholinergic and GABA ethic neurons in striatum of basal ganglia
  • defect in Huntington gene on chromosome 4
69
Q

Features of Huntington’s disease:

A
  • after 35yo
  • chorea
  • personality changes and intellectual impairment
  • dystonia
  • saccadic eye movements
70
Q

What is idiopathic intracranial hypertension?

A
  • pseudotumour cerebri and benign intracranial hypertension

- young, overweight females

71
Q

Risk factors for idiopathic intracranial hypertension:

A
  • obesity
  • female
  • pregnancy
  • drugs: oral contraceptive pill, steroids, tetracycline, vitamin A, lithium
72
Q

Features of idiopathic intracranial hypertensions:

A
  • headache
  • blurred vision
  • papilloedema
  • enlarged blind spot
  • sixth nerve palsy may be present
73
Q

Management of idiopathic intracranial hypertension:

A
  • weight loss
  • diuretics e.g. acetazolamide
  • topiramate also - benefit of weight loss
  • repeated lumbar puncture
  • surgery: optic nerve sheath decompression and fenestration to prevent damage to optic nerve, lumboperitoneal or ventriculoperitoneal shunt
74
Q

What is internuclear ophthalmoplegia?

A
  • cause of horizontal disconjugate eye movement
  • lesion in medial longitudinal fasciculus - controls horizontal eye movements by interconnecting the 3rd, 4th and 6th cranial nuclei, located in paramedic area of midbrain and pons
75
Q

Features of internuclear ophthalmoplegia:

A
  • impaired adduction of eye on same side as lesion
  • horizontal nystagmus of abducting eye on contralateral side
  • causes: multiple sclerosis, vascular disease
76
Q

What can intracranial venous thrombosis cause:

A
  • can cause cerebral infarction but much less common than arterial causes
  • 50% have isolated sagittal sinus thromboses
  • others have coexistent lateral sinus thromboses and cavernous sinus thromboses
  • headache, n&v
77
Q

Sagittal sinus thrombosis presentation:

A
  • seizures and hemiplegia

- parasagittal biparietal or bifrontal haemorrhagic infarctions sometimes seen

78
Q

Cavernous sinus thrombosis:

A
  • periorbital oedema
  • opthalmoplegia: 6th nerve damage typically occurs before 3rd and 4th
  • trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
  • central retinal vein thrombosis
79
Q

Other causes of cavernous sinus syndrome apart from thrombosis:

A
  • local infection (e.g. sinusitis)
  • neoplasia
  • trauma
80
Q

Lateral sinus thrombosis symptoms:

A

6th and 7th cranial nerve palsies

81
Q

What is Lambert Eaton syndrome:

A

-associated with small cell lung cancer
-sometimes breast and ovarian cancer
-independently as autoimmune disorder
myasthenia syndrome caused by antibody directed against presynaptic voltage gated calcium channel in peripheral nervous system

82
Q

Features of Lambert-Eaton syndrome:

A
  • repeated muscle contractions lead to increased muscle strength (contrast to myasthenia gravis)
  • limb girdle weakness (lower limbs first)
  • hyporeflexia
  • autonomic symptoms: dry mouth, impotence, difficulty micturating
  • opthalmoplegia and ptosis not commonly (unlike myasthenia gravis)
83
Q

Investigation of Lambert Eaton syndrome:

A

EMG: incremental response to repetitive electrical stimulation

84
Q

Management of Lambert Eaton syndrome:

A
  • treatment of underlying cancer
  • immunosuppression e.g. prednisolone and/or azathioprine
  • 3,4-diaminopyridine (blocks potassium channel efflux so ap increased, ca channels open longer to allow greater Ach release)
  • IV immunoglobulin therapy and plasma exchange
85
Q

Lamotrigine (MOA, ADR)

A
  • antiepileptic
  • second line variety of generalised and partial seizures
  • MOA: sodium channel blocker
  • ADR: Steven-Johnson syndrome
86
Q

What is Lateral medullary syndrome and the features:

A
  • Wallenberg’s syndrome
  • following occlusion of posterior inferior cerebellar artery
  • cerebellar features: ataxia, nystagmus
  • brainstem features: ipsilateral dysphagia, facial numbness, cranial nerve palsy e.g. Horner’s
    contralateral: limb sensory loss
87
Q

Levodopa (MOA, ADR)

A
  • combined with decarboxylase inhibitors (carbidopa or benserzide) to prevent peripheral metabolism of L-dopa to dopamine
  • reduced effectiveness with time
  • no use in neuroleptic induced parkinsonism
  • ADR: dyskinesia, on-off effect, postural hypotension, cardiac arrhythmias, n&v, psychosis, reddish discolouration of urine upon standing
88
Q

Features of medication overuse headache:

A
  • 15 days or more per month
  • develop or worsened whilst taking regular symptomatic medication
  • using opioids and triptans at most risk
  • psychiatric co-morbidity
89
Q

Management of medication overuse headache:

A
  • analgesics and triptans withdraw abruptly which may at first worsen headache
  • opioid analgesics gradually withdraw
  • withdrawal symptoms: vomiting, hypotension, tachycardia, restlessness, sleep disturbance, anxiety
90
Q

Most common cause of meningitis in adults:

A
  • meningococcus

- pneumococcus

91
Q

Neurological sequelae in meningitis:

A
  • sensorineural hearing loss
  • other neurological: epilepsy, paralysis
  • infective: sepsis, intracerebral abscess
  • pressure: brain herniation, hydrocephalus
92
Q

Triggers for migraine attack:

A
  • tiredness, stress
  • alcohol
  • COCP
  • lack of food or dehydration
  • cheese, chocolate, red wines, citrus fruits
  • menstruation
  • bright lights
93
Q

Formal diagnostic criteria produced by international headache society:

A

A - at least 5 attacks fulfilling criteria B-D
B - lasting 4-72 hours
C - headache with at least 2 of: unilateral, pulsating, moderate or severe, aggravated by routine activity
D - during headache: nausea and/or vomiting or photophobia and phonophobia
E - not attribute to another disorder

94
Q

Aura symptoms atypical and require further investigation/referral:

A
  • motor weakness
  • double vision
  • visual symptoms affecting one eye
  • poor balance
  • decreased level of consciousness
95
Q

Treatment of acute migraine:

A
  • first line: oral triptan and NSAID or paracetamol
  • 12-17yo nasal triptan
  • if not effective: metoclopramide or prochlorperazine (consider adding non-oral NSAID or triptan)
96
Q

Migraine prophylaxis:

A
  • if 2 or more attacks per month
  • 60% effective
  • topiramate or propanolol
  • propranolol for women of childbreaing age
  • riboflavin
  • menstrual migraine: meganamic acid, aspirin paracetamol and caffeine or frovatriptan or zolmitriptan
97
Q

Migraine during pregnancy:

A
  • paracetamol 1g
  • NSAIDs second line in first and second trimester
  • avoid aspirin and opioids such as codeine
98
Q

Migraine with COCP:

A

if migraine with aura, COCP contraindicated

99
Q

What is motor neurone disease?

A
  • unknown cause
  • both upper and lower motor neurone signs
  • includes amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy
100
Q

Features of motor neurone disease:

A
  • fasciculations
  • absence of sensory signs/symptoms
  • mixture of lower motor neurone and upper motor neurone signs
  • wasting of small hand muscles/tibialis anterior common
  • doesn’t affect external ocular muscles
  • no cerebellar signs
  • abdominal reflexes preserved and sphincter dysfunction if present as late features
101
Q

Diagnosis of motor neurone disease:

A
  • nerve conduction studies: normal motor conduction to exclude neuropathy
  • electromyography shows reduced number of action potentials with increased amplitude
  • MRI to exclude the differential diagnosis of cervical cord compression and myelopathy
102
Q

Management of motor neuron disease:

A
  • riluzole (prevents stimulation of glutamate receptors, mainly in amyotrophic lateral sclerosis, prolongs life by 3 months)
  • respiratory care (NIV at night, 7 month survival benefit
  • prognosis poor (50% die in 3 years)
103
Q

Types of motor neuron disease:

A
  • amyotrophic lateral sclerosis
  • primary lateral sclerosis
  • progressive muscular atrophy
  • progressive bulbar palsy
104
Q

Amyotrophic lateral sclerosis:

A
  • 50%
  • typically LMN signs in arms and UMN in legs
  • chromosome 21 and codes for superoxide dismutase
105
Q

Primary lateral sclerosis:

A

UMN signs only

106
Q

Progressive muscular atrophy:

A
  • LMN signs only
  • affects distal muscles before proximal
  • carries best prognosis
107
Q

Progressive bulbar palsy:

A
  • palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
  • worst prognosis
108
Q

2 types of multiple system atrophy:

A

1) MSA-P - predominant parkinsonism features

2) MSA-C - predominant cerebellar features

109
Q

What is Shy Drager syndrome?

A

a type of multiple system atrophy

110
Q

Features of multiple system atrophy:

A
  • parkinsonism
  • autonomic disturbance: erectile dysfunction, postural hypotension, atonic bladder
  • cerebellar signs
111
Q

What is myasthenia gravis?

A
  • autoimmune disroder
  • antibodies to acetylcholine receptors
  • more common in women
112
Q

Features of myasthenia gravis:

A
  • extraocular muscle weakness: diplopa
  • proximal muscle weakness: face, neck, limb girdle
  • ptosis
  • dysphagia
113
Q

Associations of myasthenia gravis:

A
  • thymomas
  • autoimmune disorders
  • thymic hyperplasia
114
Q

Investigations for myasthenia gravis:

A
  • single fibre electromyography: high sensitivity
  • CT thorax to exclude thymoma
  • CK normal
  • autoantibodies (to acetylcholine receptors or anti-muscle specific tyrosine kinase antibodies)
  • tensilon test: IV edrophonium reduces muscle weakness temporarily (risk of cardiac arrhythmia)
115
Q

Management of myasthenia gravis:

A
  • long acting acetylcholinesterase inhibitors (pyridostigmine)
  • immunosuppression: predinsolone, azathioprine, cyclosporine, mycophenolate mofetil
  • thymectomy
116
Q

Management of myasthenia crisis:

A
  • plasmapheresis

- IV immunoglobulins

117
Q

Exacerbating factors for myasthenia gravis:

A
  • penicillamine
  • quinidine, procainamide
  • beta blockers
  • lithium
  • phenytoin
  • antibiotics: gentamicin, macrolides, quinolones, tetracyclines
118
Q

What is myotonic dystrophy:

A
  • autosomal dominant
  • trinucleotide repeat disorder
  • DM1 caused by CTG repeat at end of DMPK gene on chromosome 19, distal weakness more prominent
  • DM2 caused by repeat expansion of ZNF9 gene on chromosome 3, proximal weakness more prominent
119
Q

Features of myotonic dystrophy:

A
  • myotonic facies
  • frontal balding
  • bilateral ptosis
  • cataracts
  • dysarthria
  • myotonia
  • weakness of arms and legs
  • mild mental impairment
  • diabetes mellitus
  • testicular atrophy
  • cardiac involvement: heart block, cardiomyopathy
  • dysphagia

Neurology (8 decks)

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