Neurology Flashcards

(196 cards)

1
Q

What gives you a hemiplegic gait?

A

UMN
Stroke, MS, tumour, SOL

See circumduction or drag

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2
Q

What give’s you a bilateral / diplegia gait

A

UMN
Bi-hemispheric = MS or cerebral palsy

Cord = compression, tumour, syringomyelia

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3
Q

What will give you a peripheral motor neuropathy foot drop?

A

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

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4
Q

Peripheral sensory neuropathy features and causes?

A

Broad based, stamping gait with sensory ataxia, rombergs positive

Causes = Diabetes, B12, drugs e.g. vincristine and phenytoin
GBS and CMT

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5
Q

Myopathy features and causes?

A

Waddling, difficulty in rising, Gower’s sign

Causes = muscular dystrophies, thyroid, Cushings and myositis

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6
Q

Motor part of GCS

A
6 = obey commands
5 = Localise to pain
4 = Withdraws to pain
3 = Abnormal flexion to pain
2 = extension to pain
1 = none
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7
Q

Verbal response GCS?

A
5 = orientated
4 = confused
3 = Words
2 = sounds
1 = none
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8
Q

Eyes GCS

A
4 = spontaneous
3 = speech
2 = pain
1 = none
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9
Q

Olfactory nerve palsy causes?

A
Bilateral = URTI, meningioma of olfactory groove
Unilateral = Head trauma, early meningioma
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10
Q

In bitemporal hemianopia what affects superior fields first?

A

Pituitary tumours / temporal one lesions = upper fields

Lower = Craniopharyngeal lesions / parietal lesions

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11
Q

What gives inferior or superior homonymous quadrantopias?

A

Parietal lesion = inferior
Temporal = superior

PITS

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12
Q

What will give you a macular sparing visual loss?

A

Occipital lobe lesion

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13
Q

How does a CN3 lesion present?

A

Down and out pupil as only lateral rectus and superior oblique left

Reduced response of elevator palpable superiors = ptosis

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14
Q

CN3 medical vs surgical?

A

Medical affects vaso vorum causing an ischaemic core = pupillary sparing as not affecting the outer parasympathetic fibres

Cause = Diabetes, MS

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15
Q

Classifying horners lesions?

Investigations?

A

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

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16
Q

What is INO, wheres the lesion and causes?

A

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

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17
Q

Trochlear CN palsy?

A

Paralysis of SO

Diplopia maximal when looking down and in e.g. stars

Affected eye turns up and out when looking laterally

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18
Q

CN6 abducens palsy?

A

Innervates lateral rectus so eye cannot abduct = strabismus

Easily affected due to long course = Tumours, trauma and CVA e.g. Millard Gubler, Wernickes

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19
Q

Trigeminal palsy?

A

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

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20
Q

Afferent and effort pathways of corneal reflex?

A

Afferent = CN5 so get bilateral loss of reflex

If only one side it is due to efferent pathway = facial nerve

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21
Q

Clinical features of Bell’s palsy?

A

Hyperacusis
Loss of motor supply to face
Cold sores if due to HSV

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22
Q

LMN vs UMN facial nerve lesion?

A

LMN affects whole face, UMN lesion is forehead sparing

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23
Q

Management of facial nerve palsy?

A

Eye protection, lubricant and tape eyes shut at night

High dose predinisolone

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24
Q

What is Ramsay hunt sydrome and management?

A

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

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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
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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.
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Spondylosis investigations and management?
MRI is definitive investigation Management = bed rest and NSAIDs Surgery indications = Abnormal neurology, recurrent pain or severe pain.
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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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Clinical features of Friedrich's ataxia?
``` Neurological: Ataxia Absent reflexes Dysarthria Weakness and wasting (relatively late sign) ``` ``` Non-neuro: Pes cavus Scoliosis Cardiac = HOCM DM ```