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
2
Q
Causes of cerebellar syndrome:
A
- Friedreich’s ataxia, ataxic telangiectasia
- neoplastic: cerebellar haemangioma
- stroke
- alcohol
- multiple sclerosis
- hypothyroidism
- drugs: phenytoin, lead
- paraneoplastic
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
4
Q
Total volume of CSF:
A
150ml
5
Q
Where is CSF produced?
A
ependymal cells in choroid plexus or blood vessels
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
7
Q
Composition of CSF:
A
- glucose
- protein
- no RBC
- WBC
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
9
Q
In whom are cluster headaches more common and what can trigger them?
A
- men 3:1
- smokers
- alcohol can trigger attack
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
11
Q
Management of cluster headaches:
A
- acute: 100% oxygen, subcutaneous triptan
- prophylaxis: verapamil
- neurologist advice
12
Q
CNS tumours:
A
- 60%: glioma and metastatic
- 20%: meningioma
- 10%: pituitary lesions
13
Q
What is the most common paediatric CNS tumour?
A
- used to be medulloblastomas
- now astrocytomas
14
Q
CNS tumour diagnosis:
A
MRI scanning
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
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
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
18
Q
Drugs causing peripheral neuropathy:
A
- amiodarone
- isoniazid
- vincristine
- nitrofurantoin
- metronidazole
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
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
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
22
Q
EMG signs in neuropathy:
A
- increased action potential duration
- increased action potential amplitude
23
Q
EMG signs in myopathy:
A
- decreased action potential duration
- decreased action potential amplitude
24
Q
EMG signs in myasthenia gravis:
A
diminished response to repetitive stimulation
25
EMG signs in Lambert-Eaton syndrome:
incremental response to repetitive stimulation
26
EMG signs in Myotonic syndromes:
extended series of repetitive discharges lasting up to 30 seconds
27
Features of encephalitis:
- fever, headache, psych symptoms, seizures, vomiting
- focal features e.g. aphasia
- peripheral lesions (cold sores) have no relation to HSV encephalitis
28
What is encephalitis caused by?
- HSV1 responsible for 95% of cases
| - temporal and inferior frontal lobes
29
Management of encephalitis:
IV acyclovir in all cases of suspected encephalitis
30
What is an essential tremor:
- auotosmal dominant condition
- both upper limbs
- postural tremor worse if arms outstretched
- improved by alcohol and rest
- most common cause of titubation (head tremor)
31
Management of essential tremor:
- propranolol first line
| - primidone
32
What is an extradural heamatoma and what does it most commonly affect:
- 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
33
Lucid interval in head trauma:
- 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
34
What does the facial nerve supply?
- 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
35
Causes of bilateral facial nerve palsy:
- sarcoidosis
- Guillain Barre syndrome
- Lyme disease
- bilateral acoustic neuromas
36
Causes of unilateral facial nerve palsy:
- causes of bilateral
- LMN: Bell's palsy, Ramsay Hunt syndrome (herpes zoster), acoustic neuroma, parotid tumours, HIV, MS, DM
- UMN: stroke, MS
37
How does LMN vs UMN palsy appear:
- UMN: spares upper face (forehead)
| - LMN: all facial muscles
38
What is foot drop?
weak dorsiflexors
39
Causes of foot drop:
- common peroneal nerve lesion
- L5 radiculopathy
- sciatic nerve lesion
- superficial or deep perineal nerve lesion
- other: central nerve lesions
40
What is a common peroneal nerve lesion caused by:
- secondary to compression at neck of fibula
| - certain positions, prolonged confinement, weight loss, Baker's cysts and plaster casts
41
Examination of common peroneal nerve lesion:
- if isolated peroneal neuropathy - weakness of foot dorsiflexion and eversion
- normal reflexes
- wekaness of hip abduction suffusive of L5 radiculopathy
42
Friedreich's ataxia:
- most common of early onset hereditary ataxia
- autosomal recessive
- trinucleotide repeat disorder characterised by GAA repeat in X25 gene on chromosome 9 (frataxin)
43
Features of Friedreich's ataxia:
- 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
44
GCS motor response:
6. obeys commands
5. localises to pain
4. withdraws from pain
3. abnormal flexion to pain (decorticate posture)
2. extending to pain
1. none
45
GCS verbal response:
5. orientated
4. confused
3. words
2. sounds
1. none
46
GCS eye opening:
4. spontaneous
3. to speech
2. to pain
1. none
47
What is Guillain-Barre syndrome?
- 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%
48
Miller Fisher syndrome:
- 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
Guillain Barre initial symptoms:
back/leg pain in early stages
50
Progressive Guillain Barre syndrome:
- 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
Other features of Guillain Barre:
- 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
Investigations of Guillain Barre:
- lumbar puncture: rise in protein with normal WCC
| - nerve conduction studies
53
Migraine:
- 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
Tension headache:
- recurrent, non-disabling, bilateral headache, often described as a tight band
- not aggravated by routine activities
55
Cluster headache:
- 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
Temporal arteritis:
- >60yo
- rapid onset of unilateral headache
- jaw claudication
- tender, palpable temporal artery
- raised ESR
57
Medication overuse headache:
- 15 days or more per month
- developed or worsened whilst taking regular symptomatic medication
- opioids an triptans more at risk
- psychiatric co-morbidity
58
Acute single episode causes of headache:
- meningitis
- encephalitis
- SAH
- head injury
- sinusitis
- glaucoma
- tropical illness e.g. malaria
59
Chronic headache causes:
- chronically raised ICP
- Paget's disease
- psychological
60
Headache red flags:
- 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
Which lobes does HSV typically affect?
temporal lobes (inferior frontal)
62
Features of HSV encephalitis:
- fever, headache, psychiatric, seizures, vomiting
- focal features e.g. aphasia
- perpiheral lesions have no relation to presence of HSV encephalitis
63
Which HSV typically causes encephalitis:
HSV1
64
Investigations for HSV encephalitis:
- CSF: lymphocytosis, protein
- PCR
- CT
- MRI better
- EEG: lateralised periodic discharges at 2Hz
65
Treatment HSV encephalitis:
IV acyclovir
66
What is HSMN
- hereditary sensorimotor neuropathy
- encompasses Charcot marmite Tooth disease
- type I - demyelinating pathology
- type II - axonal pathology
67
HSMN type I
- 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
What is Huntington's disease?
- 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
Features of Huntington's disease:
- after 35yo
- chorea
- personality changes and intellectual impairment
- dystonia
- saccadic eye movements
70
What is idiopathic intracranial hypertension?
- pseudotumour cerebri and benign intracranial hypertension
| - young, overweight females
71
Risk factors for idiopathic intracranial hypertension:
- obesity
- female
- pregnancy
- drugs: oral contraceptive pill, steroids, tetracycline, vitamin A, lithium
72
Features of idiopathic intracranial hypertensions:
- headache
- blurred vision
- papilloedema
- enlarged blind spot
- sixth nerve palsy may be present
73
Management of idiopathic intracranial hypertension:
- 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
What is internuclear ophthalmoplegia?
- 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
Features of internuclear ophthalmoplegia:
- impaired adduction of eye on same side as lesion
- horizontal nystagmus of abducting eye on contralateral side
- causes: multiple sclerosis, vascular disease
76
What can intracranial venous thrombosis cause:
- 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
Sagittal sinus thrombosis presentation:
- seizures and hemiplegia
| - parasagittal biparietal or bifrontal haemorrhagic infarctions sometimes seen
78
Cavernous sinus thrombosis:
- 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
Other causes of cavernous sinus syndrome apart from thrombosis:
- local infection (e.g. sinusitis)
- neoplasia
- trauma
80
Lateral sinus thrombosis symptoms:
6th and 7th cranial nerve palsies
81
What is Lambert Eaton syndrome:
-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
Features of Lambert-Eaton syndrome:
- 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
Investigation of Lambert Eaton syndrome:
EMG: incremental response to repetitive electrical stimulation
84
Management of Lambert Eaton syndrome:
- 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
Lamotrigine (MOA, ADR)
- antiepileptic
- second line variety of generalised and partial seizures
- MOA: sodium channel blocker
- ADR: Steven-Johnson syndrome
86
What is Lateral medullary syndrome and the features:
- 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
Levodopa (MOA, ADR)
- 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
Features of medication overuse headache:
- 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
Management of medication overuse headache:
- 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
Most common cause of meningitis in adults:
- meningococcus
| - pneumococcus
91
Neurological sequelae in meningitis:
- sensorineural hearing loss
- other neurological: epilepsy, paralysis
- infective: sepsis, intracerebral abscess
- pressure: brain herniation, hydrocephalus
92
Triggers for migraine attack:
- tiredness, stress
- alcohol
- COCP
- lack of food or dehydration
- cheese, chocolate, red wines, citrus fruits
- menstruation
- bright lights
93
Formal diagnostic criteria produced by international headache society:
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
Aura symptoms atypical and require further investigation/referral:
- motor weakness
- double vision
- visual symptoms affecting one eye
- poor balance
- decreased level of consciousness
95
Treatment of acute migraine:
- 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
Migraine prophylaxis:
- 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
Migraine during pregnancy:
- paracetamol 1g
- NSAIDs second line in first and second trimester
- avoid aspirin and opioids such as codeine
98
Migraine with COCP:
if migraine with aura, COCP contraindicated
99
What is motor neurone disease?
- unknown cause
- both upper and lower motor neurone signs
- includes amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy
100
Features of motor neurone disease:
- 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
Diagnosis of motor neurone disease:
- 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
Management of motor neuron disease:
- 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
Types of motor neuron disease:
- amyotrophic lateral sclerosis
- primary lateral sclerosis
- progressive muscular atrophy
- progressive bulbar palsy
104
Amyotrophic lateral sclerosis:
- 50%
- typically LMN signs in arms and UMN in legs
- chromosome 21 and codes for superoxide dismutase
105
Primary lateral sclerosis:
UMN signs only
106
Progressive muscular atrophy:
- LMN signs only
- affects distal muscles before proximal
- carries best prognosis
107
Progressive bulbar palsy:
- palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
- worst prognosis
108
2 types of multiple system atrophy:
1) MSA-P - predominant parkinsonism features
| 2) MSA-C - predominant cerebellar features
109
What is Shy Drager syndrome?
a type of multiple system atrophy
110
Features of multiple system atrophy:
- parkinsonism
- autonomic disturbance: erectile dysfunction, postural hypotension, atonic bladder
- cerebellar signs
111
What is myasthenia gravis?
- autoimmune disroder
- antibodies to acetylcholine receptors
- more common in women
112
Features of myasthenia gravis:
- extraocular muscle weakness: diplopa
- proximal muscle weakness: face, neck, limb girdle
- ptosis
- dysphagia
113
Associations of myasthenia gravis:
- thymomas
- autoimmune disorders
- thymic hyperplasia
114
Investigations for myasthenia gravis:
- 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
Management of myasthenia gravis:
- long acting acetylcholinesterase inhibitors (pyridostigmine)
- immunosuppression: predinsolone, azathioprine, cyclosporine, mycophenolate mofetil
- thymectomy
116
Management of myasthenia crisis:
- plasmapheresis
| - IV immunoglobulins
117
Exacerbating factors for myasthenia gravis:
- penicillamine
- quinidine, procainamide
- beta blockers
- lithium
- phenytoin
- antibiotics: gentamicin, macrolides, quinolones, tetracyclines
118
What is myotonic dystrophy:
- 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
Features of myotonic dystrophy:
- 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
