Flashcards in Neurology Deck (196)
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1
What gives you a hemiplegic gait?
UMN
Stroke, MS, tumour, SOL
See circumduction or drag
2
What give's you a bilateral / diplegia gait
UMN
Bi-hemispheric = MS or cerebral palsy
Cord = compression, tumour, syringomyelia
3
What will give you a peripheral motor neuropathy foot drop?
High stepping gait
Anterior horn = Polio
Radicular = L5 weak dorsiflexion (can't stand on heels), S1 weak plantar flexion (can't stand on toes)
Sciatic or common peroneal = foot drop
Bilateral = GBS or Charcot Marie Tooth
4
Peripheral sensory neuropathy features and causes?
Broad based, stamping gait with sensory ataxia, rombergs positive
Causes = Diabetes, B12, drugs e.g. vincristine and phenytoin
GBS and CMT
5
Myopathy features and causes?
Waddling, difficulty in rising, Gower's sign
Causes = muscular dystrophies, thyroid, Cushings and myositis
6
Motor part of GCS
6 = obey commands
5 = Localise to pain
4 = Withdraws to pain
3 = Abnormal flexion to pain
2 = extension to pain
1 = none
7
Verbal response GCS?
5 = orientated
4 = confused
3 = Words
2 = sounds
1 = none
8
Eyes GCS
4 = spontaneous
3 = speech
2 = pain
1 = none
9
Olfactory nerve palsy causes?
Bilateral = URTI, meningioma of olfactory groove
Unilateral = Head trauma, early meningioma
10
In bitemporal hemianopia what affects superior fields first?
Pituitary tumours / temporal one lesions = upper fields
Lower = Craniopharyngeal lesions / parietal lesions
11
What gives inferior or superior homonymous quadrantopias?
Parietal lesion = inferior
Temporal = superior
PITS
12
What will give you a macular sparing visual loss?
Occipital lobe lesion
13
How does a CN3 lesion present?
Down and out pupil as only lateral rectus and superior oblique left
Reduced response of elevator palpable superiors = ptosis
14
CN3 medical vs surgical?
Medical affects vaso vorum causing an ischaemic core = pupillary sparing as not affecting the outer parasympathetic fibres
Cause = Diabetes, MS
15
Classifying horners lesions?
Investigations?
1st order = central = MS / stroke / brainstem lesion
- Trunk, arms and face
2nd order = pre-ganglionic = pan coasts, apical TB, cervical rib, previous chest drain, thoracic/neck surgery
-Face
3rd order = Post-ganglionic = herpes zoster, carotid pathology
-Sweating unaffected
Investigations = CXR, MRA if brain and neck
16
What is INO, wheres the lesion and causes?
Lesion to the medial longitudinal fasciculus between midbrain and pons
Imapired adduction of ipsilateral, nystagmus on contralateral abduction
Causes = MS, vascular brainstem lesion, pontine glioma and encephalitis
17
Trochlear CN palsy?
Paralysis of SO
Diplopia maximal when looking down and in e.g. stars
Affected eye turns up and out when looking laterally
18
CN6 abducens palsy?
Innervates lateral rectus so eye cannot abduct = strabismus
Easily affected due to long course = Tumours, trauma and CVA e.g. Millard Gubler, Wernickes
19
Trigeminal palsy?
Lose sensation in ophthalmic, maxillary and mandibular regions.
Lose motor function of masseter and pterygoids
No jaw jerk
Corneal reflex = Afferent is CN5 ophthalmic branch, so if both eyes don't close it is CN5
Causes: Midbrain lesions, trigeminal ganglion lesion e.g. acoustic neuroma. Lesion in cavernous sinus
20
Afferent and effort pathways of corneal reflex?
Afferent = CN5 so get bilateral loss of reflex
If only one side it is due to efferent pathway = facial nerve
21
Clinical features of Bell's palsy?
Hyperacusis
Loss of motor supply to face
Cold sores if due to HSV
22
LMN vs UMN facial nerve lesion?
LMN affects whole face, UMN lesion is forehead sparing
23
Management of facial nerve palsy?
Eye protection, lubricant and tape eyes shut at night
High dose predinisolone
24
What is Ramsay hunt sydrome and management?
Reactivation of VZV in geniculate ganglion of CN8
PC = ear pain and neck stiffness
Vesicular rash in auditory canal
Ipsilateral facial weakness
Management = Aciclovir and steroids
25
Bilateral facial palsy causes?
Sarcoidosis, GBS and limes disease
Can also see bilateral acoustic neuromas in NFT2
26
Webers lateralises to the right side, this means?
Ipsilateral conductive hearing loss = right conductive
Or contralateral sensorineural
27
Taste to anterior 2/3rds of the tongue?
Facial
28
Taste to Posterior 2/3rds of tongue?
Glossopharyngeal
29
What do you see in a vagus nerve palsy?
Uvula deviates away from the lesion
Loss of gag reflex
30
Hypoglossal palsy?
Controls motor component of tongue
So will deviate towards the side of the lesion
May see wasting / fasciculations
Like facial tongue has bilateral UMN innervation so only lost of LMN
31
Resting tremor features and causes?
Increase with distraction, abolished on voluntary movement
Seen in Parkinson's, treat with dopamine agonists
32
Action / postural tremor features and causes?
Absent at rest, worse on movement
Causes = BEAT
Benign essential tremor = alcohol improves
endocrine e.g. thyrotoxicosis
Alcohol withdrawal
Toxins e.g. B agonists
33
What is acute dystonia, causes and management?
Prolonged muscle contractor causing unusual joint posture / repetitive movements
Torticollis, trismus, oculogyric crisis
Often a drug reaction e.g. neuroleptics, L-DOPA
Management = procyclidine
34
Whats athetosis?
Slow sinuous writhing movements
Seen in cerebral palsy
35
Syncope causes?
CRASH
Cardiac = Stoke Adams attacks / CV syncope
Reflex = vagal overactivity e.g. vasovagal syncope, carotid sinuous hypersensitivity
OR
Sympathetic under activity e.g. postural hypotension
Arterial = Vertebrobasillar insufficiency
Systemic = hypoglycaemic
Head = epilepsy
36
Causes and features of cardiac syncope?
Bradycardia e.g. Heart block, long QT
Tachycardia e.g. SVT or VT
Structural e.g. LVF, tamponade
Before = palpitation, pain, SOB
During = Short LOC, pale and pulseless
After = quick recovery and flushed
37
Features of reflex syncope?
Before = slow onset, sweaty, clammy and tunnel vision
During = pale, grey, bradycardia, may have clonic tonic jerks but NO TONGUE BITING
38
Investigations for syncope?
Postural BP, cardio and neuro exam
ECG ± 24 hour tape
U&E's, FBC
Echo
CT
39
Differential for vertigo?
IMBALANCE
Infection = labrynthitis
-associated URTI
-Acute and short lived
-otorrhoea
Menieres
-Episodic vertigo with roaring tinnitus
-Lasts minutes to hours
-sensation of pressure discomfort
BPPV
-Sudden, <30 seconds, head movements precipitate
Aminoglycosides and furosemide
Lymphatic fistula = tulles phenomenon where vertigo induced by nosie
Arterial = stroke, TIA
Nerves = Acoustic neuroma
Central = MS, tumour
E = epilepsy
40
Management of labrynthitis?
Vestibular suppressants = Promethazine
Prednisolone
If bacterial = Topical ofloxacin
41
management of menieres?
Low salt diet
Vestibular suppressant e.g. Promethazine, and corticosteroids
Menniet device TDS = delivers intermittent pulse pressures through ear
If ongoing can have surgery
42
BPPV management?
Educate and reassure not subside within 6 months
Epley manœuvre for treatment
If this fails Semont repositioning
43
What is a seizure?
Clinical manifestation of presumed / proven abnormal electrical activity in the brain
44
Different seizure presentations maintaining consciousness?
Myoclonus = irregular jerk caused buy involuntary muscle contraction
Aura = Simple partial seizure only lasting seconds
Simple partial motor = clonic (regular shaking), tonic (stiffness) or dystonic (spasm) lasting seconds
45
Different types of seizures losing consciousness?
Absences
Complex partial / focal awareness impaired
Tonic clonic / generalised
46
Features of an absence seizure?
management?
Last seconds, occurring multiple times in one day
3-10 years
Stimulated by hyperventilation
EEG = characteristic bilateral symmetrical 3Hz spike and wave pattern
90% seizure free by adulthood
Ethosuximide or sodium valproate
Second line = Lamotrigine
47
Complex partial features and management?
Impaired awareness / memory
Automatisms
Involve one side of the brain
Rapid recovery, no sleepiness
Carbamazepine or lamotrigine
2nd line = Levetiracetam or sodium valproate
48
Simple partial features and management?
Emotional disturbance and automatisms
Post-ictal phase
Carbamazepine or lamotrigine
2nd line = Levetiracetam or sodium valproate
49
Generalised seizure features and management?
No warning if generalised, aura if focal with secondary generalisation
Lateral tongue biting, incontinent, cyanosed
Can last 1-2 minutes
Post-ictal up to ten
Sodium valproate
Second line = Lamotrigine
50
Management of myoclonic seizures?
Sodium valproate
Second line = lamotrigine
51
Common side effects of anti-epileptics?
Lamotrigine = skin hypersensitivity
Valproate = teratogenic and weight gain
Carbamazepine = Skin hypersensitivity, vision, and SIADH
Ethosuximide = GI effects, insomnia and psychotic episodes
52
Driving regulations epilepsy?
First seizures reported to DVLA
Can drive once 12 month seizure free
If bus / lorry driver = ten years free
53
status epilepticus definition?
Continuous seizure lasting > 5 minutes, or repeated seizures lasting > 5 minutes with no regain of full consciousness in between
54
Status epileptics management?
ABC, 100% O2 and suction
IV access and bloods
1. Reverse potential causes
2. IV lorazepam 2-4mg / Rectal diazepam 10mg if no IV access. Second dose if no response after 10 minutes
3. 2nd line = phenytoin IV 20mg/kg at a rate not exceeding 50mg/minute
OR
Phenobarbitol IV 10mg/kg at 100mg/minute
Call anaesthetist
4. RSI
55
What is West syndrome and its features?
<1 year
Salaam attacks = Flexion of head, trunk and limbs. then extension of arms
Last 1-2 seconds, repeated 50 times
Progressiv mental handicap
EEG = hypsarrhythmia
56
Lennox-Gastaut?
Onset 1-5 years
Atypical absences, jerks and falls
90% moderate to severe metal handicap
EEG = slow spike
Ketogenic diet may help
57
What is benign rolandic epilepsy?
Paraesthesia e.g. unilateral face on waking up
58
Juvenil myoclonic epilepsy features?
Onset in teens
Female
Infrequent generalised seizure, often in morning
Daytime absences
Sudden shock like myotonics
Responds well to sodium valproate
59
Migraine without aura criteria
At least 5 attacks, lasting 4-72 hours
Headache is 2 of:
Unilateral
Pulsating
Moderate to severe
Aggravated by exercise
During the headache 1 of:
N&V, photophobia, phonophobia
60
Migraine with aura criteria?
Same as without but aura must fulfil...
1 of:
Fully reversible +ve / -ve symptoms
Dysphasic speech disturbance
2 of:
Homonymous visual or unilateral sensory symptoms
One aura symptom develops over at least 5 minutes
Each last 5-60 minutes
61
Chronic migraine criteria?
>15 days a month, for at least 3 months +
Patient has had > 5 migraine attacks ± aura
On >8 days a month for 3 months fulfilling a migraine
62
Migraine management?
Acute reliever = Paracetamol / NSAIDs with an oral triptan
If under 17 = nasal triptan
Prophylaxis:
Avoid triggers
Topiramate or propranolol if > 2 attacks per month
2nd line = gabapentin
63
What is absolutely contraindicated in migraines?
COC due to increased risk of TIA
64
Cluster headache criteria?
5 attacks fulfilling below:
Severe unilateral orbital, supraorbital or temporal pain, lasting 15 minutes to 3 hours
Accompanied by one of:
Lacrimation
Rhinorrhoea
Facial oedema
Miosis/ptosis
Attacks happen at least every other day, up to 8/day
65
Management of cluster headaches?
Acutely 1005 O2
Sumitriptan 6mg subcut
Prophylaxis = Verapamil
66
What is trigeminal neuralgia, causes and management
Paroxysmal unilateral stabbing pain
Cause = Vessel compressing it commonly superior cerebellar, MS, varicella zoster
1st line = carbamazepine 100mg BD
67
GCA features and management?
Associated with PMR in 50%
ESR > 60
Temporal artery biopsy is gold standard
Management = Oral prednisone 60mg one of if visual symptoms, then refer to ophthalmology
If no visual = 40-60mg daily
Assess steroid response in 48 hours
68
Features of raised ICP?
Headache
Vomiting + seizures
Papilloedema
GCS reduced
Cushings reflex = Triad of increased BP, bradycardia and irregular breathing
69
Causes of raised ICP?
Vascular = haemorrhage, haematoma, AVM
Infection = Abscess, cyst, meningitis
Malignancy
TB granuloma
Hydrocephalus
70
Management of raised ICP?
ABC and 100% O2
Tilt bed to 45 degrees
Consider mannitol if head injury / bleed 0.25g/kg IV stat
IV dexamethasone if SOL 0.25mg/kg daily IV
71
IIH features and management?
As for ICP
1st line = weight loss
Acetazolamide 500mg BD
72
Kernigs and Brudzinskis sign?
Kernigs = Flex thigh, and straighten knee slowly. =pain
B sign = Patient supine and examiner flexes neck = involuntary knee and hip flexion
73
Lumbar puncture signs in bacterial meningitis?
Cloudy
Low glucose
high protein (0.5-3)
Raised WCC - neutrophils
74
LP in viral meningitis?
Clear / cloudy
raised leucocytes
Raised protein,
normal / high glucose
75
TB LP in meningitis?
Clear / cloudy. Fibrin web
Raised monocytes
Protein raised
Glucose normal
76
Empirical meningitis management?
Empirical = Cefotaxime 2g IV BD (+ ampicillin if <3 month, >50 or immuncomp)
77
Management for meningococcal meningitis?
IV BenPen 2.4g IV 4 hourly
78
Management for pneumococcal / haemophilia meningitis ?
IV cefotaxime
79
Management for listeria meningitis?
IV gentamicin and ampicillin
80
Fungal meningitis management?
Cryptococcal and candididal = Amphotericin B 5mg/kg/day IV
Aspergillus = Voriconazole 6mg/kg IV BD
81
What is encephalitis?
Causes?
Inflammation of the brain and meninges
Viruses e.g HSV!/2, CMV, EBV, West nile
Also any bacterial meningitis
82
Clinical features of encephalitis and management?
Prodromal fever and rash
Altered mental state, meningism and Neuro signs
Seizures
Empirical = acyclovir 10mg/kg IV 8 hourly for three weeks
83
Investigations for encephalitis?
Bloods = cultures, smears and FBC
Viral PCR
CT brain = late changes = hypodense regions
LP
84
Stroke definition?
Rapidly developing clinical signs of focal disturbance of cerebral function, > 24 hours.
85
Stroke causes?
80% = ischaemia e.g. atheroma, embolism
20% = haemorrhage e.g. HTN, aneurysm, anticoagulation
86
Oxford classifications of strokes 4 types?
Total anterior
-highest mortality
Large infarct of carotid/MCA or ACA
-All 3 of higher dysfunction, contralateral sensory / motor deficit and contralateral homonymous hemianopia
Partial anterior
-same as above but only 2/3
Posterior circulation
-Vertebrobasillar territory
- Any of cerebellar syndrome, brainstem syndrome, LOC and contralateral homonymous hemianopia
Lacunar strokes
-Small infarcts around basal ganglia, internal capsule
-Pure motor
-Pure sensory
-Mixed
-Ataxic hemiparesis
87
What vessel causes dysphasia?
MCA
88
What is Wernickes and where is affected?
Receptive dysphasia = speech fluent but cannot understand
Sentences make no sense
Good repetition
Superior temporal gyrus
89
What is brooks?
Expressive dysphasia = understand things, just cannot express fluently
Speech is laboured, non-fluent and halting
Inferior frontal gyrus
90
Conduction dysphasia?
Associative dysphasia, fluent but with abnormal comprehension + abnormal repetition
Arcuate nucleus = connection between Broca's and wernickes
91
What will ACA stroke show?
Contralateral motor / sensory. legs > arms. Face spared
92
MCA stroke?
Contralateral motor / sensory. Face and arms > leg
93
PCA stroke?
Contralateral homonymous hemianopia, macula sparing
94
Webers stroke?
Branches of the posterior artery that supply midbrain
Ipsilateral CN3 and contralateral hemiparesis
95
Wallenberg stroke?
Posterior inferior cerebellar artery
Ipsilateral facial pain and temperature loss
Contralateral limb/torso pain and temperature loss
A-HAND:
Ataxia
Horners
Anaesthesia (as above)
Nystagmus and vertigo
Dysphagia
96
Lateral pontine syndrome?
Anterior inferior cerebellar artery.
Similar to Wallenberg but get ipsilateral facial paralysis and deafness
97
Millard Gubler Syndrome
Lesion in the pons
CN6/7 and corticospinal tracts
Diplopia
LMN lesion facial loss and loss of corneal reflex
Contralateral hemiplegia
98
Locked in syndrome
Ventral pons infarct = basilar artery
Patient is aware, but completely paralysed except for vertical gaze and upper eyelid movement
Due to sparing of the mid-brain tectum
99
Cerebelopontine angle syndrome causes and features
Causes = Acoustic neuromas, meningioma, metastasis
Ipsilateral CN5/6/7/8 + cerebellar signs
100
Subclavian steal syndrome
Subclavian artery stenosis proximal to origin of vertebral artery = blood is stolen from here due to retrograde blood flow
Syncope and neurology on use of arm
101
Stroke investigations?
Bloods = exclude hypoglycaemia, U&E's, cardiac enzymes + clotting
ECG
CT head
102
Management of acute ischaemic stroke?
No venous sinus thrombosis (VST) within 4.5 hours:
-Alteplase 0.9mg/kg IV 10% dose as bolus, rest 90% over 1 hour
Max 90mg
300mg aspirin for 2 weeks
If no VST, >4.5 hours = 2 weeks aspiring 300mg OD. no rTPA
If VST = Heparin full dose, then warfarin until INR 2-3
103
Management of acute haemorrhage stroke?
ITU, airway
BP control = labetalol 10mg IV over 2 minutes
Correct any coagulopathy:
Warfarin = Stop, phytomenadione and FFP + PT complex
Heparin = stop and protamine sulphate
Dagibatran = Idarucizumab (Praxbind)
If deteriorating = External ventricular drainage of CSF / haematoma removal surgically
104
Long term management of stroke?
Clopidogrel lifelong
RF control = BP, statins
Physio, salt, OT and Neuro-rehabilitation
If ischaemic = lifelong anticoagulation
105
What is a TIA?
Stroke symptoms which resolve with 24 hours
106
TIA management?
Clopidogrel 75mg immediato and lifelong
RF control
Assess stroke risk with ABCD2
TIA clinic referral
Cant drive for 1 month, or three if multiple over a short period of time
107
What is a SAH and its causes?
Bleeding into the SA space between the arachnoid membrane and Pia mater
Cause = Rupture of berry aneurysms (80%) and AVMs
108
Where do berry aneurysms occur?
Branch points
Junction of posterior communicating artery with internal carotid
Junction of ACA and anterior cerebral artery
109
Clinical features of SAH?
Sudden onset headache
Collapse
LP = bloodstained CSF
CT = Well defined round hyper attenuated lesion
110
SAH management?
Unruptured <7mm can be observed
Surgical:
Clipping = more suitable if large parenchymal haematomas
- craniotomy and clip
Endovascular coil via femoral:
- Better suited for old / frail
Medical:
Nimodipine 60mg four hourly for three weeks = vasospasm prophylaxis
Stool softeners
111
What is a subdural haemorrhage?
Features?
Haematoma between the dura and the arachnoid
Caused by damage to the riding veins between cortex and sinus
Old alcoholic, lots of falls
Headache and slowly progressive mental/physical decline
112
Subdural investigations and management?
CT = crescenteric, midline shift
Management:
<10mm or midline shift <5mm:
Prophylactic anti epileptics e.g. phenytoin
Correct any coagulopathy
ICP management = tilt, ventilate and mannitol
>10mm or >5mm midline shift or significant neuro:
Same as above
Burr hole craniotomy and irrigate clot with saline and suction
113
What is an extradural?
Clinical features?
Collection of blood between inner layer of the skull and outer layer of the dura
Middle meningeal artery bleed, with parietal/temporal fracture
Lucid interval, can be hours/days
Raised ICP symptoms
Brainstem compression = Cushings triad
114
Extradural investigations and management?
CT = lens shaped haematoma + skull fracture
Neuroprotective ventialtion, mannitol and craniotomy for clot evacuation and vessel ligation
115
Two types of intracranial venous thrombosis?
Causes?
Dural venous, Cortical Vein
Pregnancy, OCP, head injury, dehydration and thrombophilia
116
Types of Dural venous sinus thrombosis type and PC?
Sagittal = 45% of IVT. Headache, vomiting, seizures and visual
Transverse = 35% of IVT
Headache ± mastoid pain, focal neuro signs, seizures
Cavernous sinus
Spread from facial pustules / folliculitis
Often involves CN3-6
117
Investigations and management of intracranial venous thrombosis?
Exclude SAH and meningitis with CT/MRI contrast = convex bowing of lateral walls and increased dural enhancement
LP = increased opening pressure
LMWH then warfarin INR 2-3
Thrombolysis
For cavernous = Vancomycin and ceftriaxone
118
Delirium definition?
Acute fluctuating confusional state, with clouding of consciousness affecting the sleep wake cycle
119
Clinical features and management of neuroleptic malignant syndrome?
Young male, within 10 days of starting anti-psychotics
Pyrexia
Rigidity
Tachycardia
Rasied CK
Stop anti-psychotic
Fluids
Bromocriptine or Dantrolene
120
What is dementia?
Progressive mental decline interfering with ADLs, insidious in onset with clear consciousness
121
Pathology of Alzheimers?
Altered APP metabolism = amyloid B deposition causing:
Extracellular plaques
NFT's
Cerebral amyloid angiopathy
Cerebral atrophy
122
Classification of Alzheimers severity?
MMSE:
mild = 21-26
moderate = 10-20
(Moderately severe = 10-14)
Severe = <10
123
Alzheimers management?
Structured cognitive stimulation
Mild to moderate = AChE inhibitors e.g. Donepezil, Galantamine and rivastigmine
Severe = Memantine
124
Clinical features and management of vascular dementia?
Step wise progression, with vascular risk factors
Manage the predisposing RF's
125
Lewy body dementia pathology, PC and management?
Lewy bodies in occipital-parietal cortex
PC = fluctuating cognition, visual hallucinations and Parkinsonism
Management = AChE inhibitors
126
Fronto-temporal dementia pathology, PC and scan findings?
Fronto-temporal atrophy, marked gloss and neuronal loss, balloon neutrons + tau+ve pick bodues
PC = disinhibition, personality change, early memory preservation
Scan = fronto-temporal atrophy
127
Parkinsons pathology?
Loss of dopaminergic neutrons that originate in substantial migration and project to the striatum = Lewy body inclusion, +ve for alpha-synuclein
NFT's = hyper-phosphorylated tau
128
Parkinsons features?
Tremor, rigidity and akinesia / bradykinesia
Minimal facies
Postural instability
50% get dementia
90% = sleeping disorders
129
Management of Parkinson's?
1st line = L-DOPA, most effective for motor signs.
Can give with decarboxylase inhibitor e.g. Sinemet / Madopar
2nd line = Pramipexol, Rotigotine
MAO-B inhibitors = Rasagiline
COMT inhibitors = entacapone
130
SE's of L-DOPA?
On-off motor fluctuations
End of dose deterioration
Permanently induce dyskinesia
131
What class is pramipexol / rotigotine and SE's?
Dopamine agonist
Impulse behaviour
Oedema
Fibrosis
132
How do MAO inhibitors and COMT inhibitors work?
Both inhibit the breakdown of dopamine
133
What do you use to treat L-DOPA induced dyskinesia?
Stop drug
Amantidine
SE's = ataxia, slurred speech and lived reticularis
134
What three conditions make ups MSA?
Olivopontocerebellar atrophy
Shy-drager syndrome
Striatonigral degeneration
Generally now classed as MSA-P or MSA-C dependant on the predominant cerebella or Parkinsonism symptoms
135
Clinical features of MSA?
Autonomic dysfunction = postural hypotension and bladder dysfunction
Cerebellar signs
Rigidity > tremor
136
PC of corticobasal degeneration?
Parkinsonism
Speech disturbances
Alien limb
Tau +ve
137
PSP clinical features?
Supranuclear gaze and postural instability = can't look down
Rocket sign = when asked to get out of a chair they fly up
138
What is multiple sclerosis?
Chronic inflammatory demyelinating disease of the CNS = CD4 mediated destruction of oligodendrocytes
139
MS classification?
Relapsing remitting = 80%
Secondary progressive (following R-R)
Primary progressive = 20%
Progressive relapsing
140
MS relapses vs progression
Relapses = acute onset with complete / partial recovery. Last > 24 hours
Progression = Insidious, relentless and irreversible . For at least 1 year
141
Clinical features of MS?
Most common at onset = optic neuritis, motor weakness and sensory disturbances
UMN ONLY
Optic neuritis = pain on eye movement and reduced vision. Uhthoffs = vision reduced with heat
Spinal cord lesion = spastic paraparesis, Lhermittes sign where electric shock triggered by neck flexion, urinary incontinence
Brainstem lesion = INO, vertigo, nystagmus
Cerebellar = ataxic
142
MS investigations?
Lesions must be separated in time and space
4 typical MRI lesions = SIPS
Spinal cord
Infratentorial
Subcortical
Periventricular
CSF = Increased production of IgG oligoclonal bands
143
Management of MS?
Acute attack = 1g prednisolone for 3 days
Preventing relapses in R-R: IFN-B 30ug once weekly OR Glatiramer 20mg subcut. OD
2nd line = Alemtuzumab 12mg for 3 days, then head later 12mg for 5 days
Secondary progressive = Methylprednisolone 1g IV monthly
Primary progressive MS = Ocrelizumab 300mg initial dose
144
Management of specific MS problems?
Spasticity = baclofen
Bladder dysfunction = may require regular self catheterisation and anti-cholinesterase
145
Clinical features of cord compression?
Spinal pain
Numbness / paraesthesia below level
Weakness
Bladder and bowel dysfunction
146
Management of cord compression?
Trauma = immobilise, surgery and methylpred.
Malignancy = Methylpred. ± surgery ± radiation
Abscess = Vancomycin + metronidazole + cefotaxime
147
Cauda equina signs and management?
Saddle anaesthesia, back pain and poor anal tone
bilateral flaccid and areflexic lower limbs
Incontinence / retention
Mx = Laminectomy
148
What is spondylosis?
Degenerative change of intervertebral discs
149
Cervical spondylosis symptoms?
C5 = Deltoid, supraspinatus and reduced supinator jerk
+ numb elbow
C6 = Biceps, brachioradialis, reduced biceps reflex
+ Numb index
C7 = Triceps, finger extension and triceps reflex
+ numb middle finger
C8 = Finger flexors and intrinsic hand muscles
+ numb ring and little
150
Lumbosacral spondylosis symptoms?
L4 = Quad wasting / weakness. Impaired knee jerk. Sensory impairment over medial calf.
+ve femoral stretch test
L5 = Wasting and weakness of dorsiflexors - some degree of foot drop. Sensory over dorsum of foot.
INTACT REFLEXES
+ve sciatic nerve test
S1 = Impaired ankle jerk. Sensory impairment over lateral aspect of foot.
+ve sciatic nerve test.
151
Spondylosis investigations and management?
MRI is definitive investigation
Management = bed rest and NSAIDs
Surgery indications = Abnormal neurology, recurrent pain or severe pain.
152
Cervical myelopathy causes, clinical features and management
Causes = spondylosis, trauma and congenital
PC = Pain affecting neck and limbs, loss of motor function is reduced dexterity, loss of outonomic function = incontinence / retention.
MRI = gold standard
Management = Urgent surgical referral for spinal decompression
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PC of musculocutaneous lesion?
Weak flexion at elbow, absent biceps reflex.
Numbness over lateral forearm
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PC of axillary nerve damage?
Weak shoulder abduction and external rotation. Sensory loss over regimental patch
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PC of radial nerve damage?
Wrist drop as cannot extend wrist, weak elbow extension.
Cannot abduct thumb
Absent triceps and supinator reflex.
Sensory loss over dorsal aspect of dorsal aspect of thumb and lateral two fingers
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PC of median nerve lesion?
Cannot flex wrist, weak flexion of fingers BUT can still flex at DIPJ of ring finger and little finger. All PIPs gone as lose FDS.
Lose sensation over palmar aspect of thumb and medial two fingers.
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PC of ulnar nerve lesion?
Can't abduct or adduct fingers = paper test.
Weak wrist flexion, weak flexion of ring and little fingers.
Numbness over hypothenar eminence and lateral 1.5 fingers.
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Causes of polyneuropathies?
Metabolic = Diabetes, B12
Vasculitis
Inflammatory = GBS, sarcoidosis
Inherited = CMT
Infection = HIV, syphilis
Drugs = Isoniazid, vincristine, phenytoin
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Investigations for polyneuropathy?
B12 levels.
MRI may show you subacute combined degeneration
Diabetes = HbA1c
Alcohol = AST:ALT ratio, GGT
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What is GBS?
Causes?
An acute inflammatory post-infectious neuropathy, antibodies to gangliosides
Viral e.g. SMV, EBV (HHV4),
Bacterial = Campylobacter
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Clinical features of GBS?
Prodromal = malaise, vomtiing, headache, limb pain
Followed by ascending paralysis = reduced reflexes, weakness, respiratory distress
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Types of GBS?
Acute inflammatory demyelinating polyradiculopathy = most frequent in western world
Acute motor axonal neuropathy = purely motor
Acute motor and sensory = very rare
Miller-Fisher Syndrome = Ophthalmoplegia, ataxia and areflexia.
Descending paralysis
Anti-GQ1b antibodies
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GBS management?
Plasma exchange or IVIG 400mg/kg/day for 5 days
- Plasma exchange if IgA deficiency or renal failure
85% full recovery
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What is CMT and the two types?
Hereditary sensory and motor neuropathy. Autosomal dominant
CMT1 = commonest, autosomal dominant, demyelination
CMT2 = second commonest, autosomal dominant, axonal degeneration
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Clinical features of CMT?
FHx is key = pes cavus, neuropathy or abnormal gait
Walking difficult with foot drop / high stepping gait
Pes cavus
Reduced reflexes, sensation,
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Management of CMT?
Physiotherapy with low impact exercise and stretching
Bracing
Orthopaedic surgery to correct foot deformities
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Types of MND?
UMN AND LMN
Amyotrophic lateral sclerosis
Primary lateral sclerosis
Progressive muscular atrophy
Bulbar or pseudo bulbar palsy
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Features of ALS?
Most common MND
Lesion to corticospinal tracts
UMN in legs, LMN in arms
progressive and unrelenting with 3-5 year survival
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features of PLS
Isolated UMN with progressive weakness and spasticity
Slower disease progression vs ALS
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Features of progressive muscular atrophy
Isolated LMN = lesions of anterior horn cell
Asymmetrical limb wasting and weakness
Affects distal then proximal
Median survival = 4.6 years = BEST PROGNOSIS
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Features of pseudobulbar palsy
Degeneration of corticobulbar tracts to CN5/7/10/11/12
Difficulty in mastication, facial expressions, weak swallowing and weak tongue
Labile emotions
Brisk jaw jerk
UMN signs
SPARES 3/4/5 = EYES
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Bulbar palsy
Impairment of CN9/10/12
Get dysarthria, dysphagia and poor swallow
Nasal speech
LMN signs
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MND management?
Riluzole 50mg PO BD =
Prevents stimulation of glutamate receptors
Can prolong life by 3 months
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Myopathy vs neuropathy?
Myopathy = muscle dysfunction. Proximal weakness with no sensory loss.
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Different causes of myopathies?
Muscular myopathies e.g. Duchennes, Beckers, Facioscapulohumeral, myotonic
Metabolic e.g. thyroid, graves
Drug induced e.g. alcohol, statins and steroids
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clinical features and management of Duchennes
X-linked, non-functional dystrophin
PC = delayed walking, falls, waddling gait and Gower's.
Calf pseudohypertrophy
Management = prednisollone, creatine and supportive
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Clinical features and management of Becker's?
X-linked, partially functioning dystrophin.
Same as Duchennes
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features of fascioscapulohumeral dystrophy?
Weakness in second or third decade.
Mainly facial, periscapular and humeral.
Characteristic unlined facies, pouting lips and transverse smile.
foetal myoglobin and sarcolemma are increased
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Features of myotonic dystrophy?
PC = Cataracts, hypogonadism, frontal balding and cardiac disorders
Wasting of sternocleidomastoids = Swan necked appearance.
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What is myasthenia gravis?
Chronic autoimmune disorder of post-synaptic membrane at the NMJ in skeletal muscle
Antibodies vs. the Ach receptor
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Myasthenia associations in men vs women?
Men = thyme atrophy / tumour
Women = autoimmune disease e.g. RA, DM
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Clinical feature of Myasthenia?
Muscle fatigueablilty = bilateral ptosis, diplopia. Myasthenia snarl.
Proximal limb weakness
Normal reflexes
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What can precipitate Myasthenia?
BB's, lithium, phenytoin, antibiotics e.g. gentamicin
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Investigations and Management of Myasthenia?
Single fibre EMG, serum AchR antibodies, muscle specific tyrosine kinase antibodies
Mx:
Cholinetserase inhibitors = Pyridostigmine
Prednisolone
Consdier thymectomy
refractory = IVIG
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What is LEMs?
Causes?
Auto-immune disorder of the NMJ with antibodies vs. the voltage gated calcium channel = no presynaptic fusion and therefore no excretion of Ach
Cause = paraneoplastic pulse with small cell carcinoma or autoimmune.
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Clinical features of LEMs and management?
Leg weakness before eyes.
Autonomic symptoms e.g. dry mouth
movement IMPROVES symptoms
Mx:
Treat underlying cause
Amifampridine
Refractory = IVIG
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Clinical features of NFT1?
CAFE-NOIR
Cafe au lait spots >6 15mm in diameter
Axillary freckling
Fibromas = subcutaneous or plexiform (nerve trunk)
Eye = Lisch nodules
Neoplasia e.g. CNS meningioma, phaeo, CML/AML, GI tumours
Orthopaedic = scoliosis
IQ reduced
Renal = RAS = HTN
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Clinical features NFT2?
Bilateral acoustic neuromas = SNHL, vertigo
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NFT management?
MDT
Genetic counselling
Surgery for any underlying malignancies
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What is tuberous sclerosis?
Autosomal dominant condition causing tumours throughout he body.
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Clinical features of tuberous sclerosis?
Cutaneous
Neurological
Other
Cutaneous:
Depigmented ash leaf spots. Roughened shagreen patches over lumbar region
Adenoma sebaceum
Subungal fibratoma
Neuro:
Developmental delay
Epilepsy
Intellectual impairment
Other features = retinal hamartomas (NFT HAS IRIS HAMARTOMAS)
Gliomatous changes in brain lesions
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What is syringomyelia?
Slowly progressive syndrome, I which cavitation (called a syrinx) occurs in central segments of the spinal cord, often cervical
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Causes of syringomyelia?
Spina bifida
Arnold-Chiari malformation in which cerebellum extends through foramen magnum into first part of cervical spine.
Secondary to cord trauma / tumours
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Clinical features and management of syringomyelia?
Sensory loss due to spinothalamic damage = temperature and pain, often in arms and shoulders
Anterior horn cell damage = LMN signs = wasting and weakness
Management = surgery to promote free flow of CSF
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What is Friedrich's ataxia?
Hereditary progressive ataxia, due to degeneration of spinocerebellar and corticospinal tracts + dorsal root ganglion and cerebellar cells
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