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Neurology Flashcards

1
Q

What are clinical signs in the face of myotonic dystrophy? (6)

A

Myopathic facies: long, thin, expressionless
Wasting of facial muscles and SCM
Bilateral ptosis
Frontal balding
Dysarthria due to myotonia of tongue/pharynx
Myotonia of eyes

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2
Q

What are clinical signs in the hands of myotonic dystrophy? (4)

A

Myotonia
Wasting and weakness distally
Areflexia
Percussion myotonia of thenar eminence

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3
Q

What are some systemic associated findings in myotonic dystrophy? (6)

A

Cataracts
Cardiomyopathy
Brady and tachy arrhythmias - PPM
Diabetes
Testicular atrophy
Dysphagia

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4
Q

What is the underlying genetic pathophysiology of myotonic dystrophy?

A

Type 1 or 2 depending on genetic defect
DM1: CTG trinucleotide expansion within DPMK gene chromosome 19
DM2: CCTG repeat sequence ZNF9 gene chromosome 3
Shows genetic anticipation in DM1 so presents earlier 20-40s depending on number of repeats
Autosomal dominant

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5
Q

How is a diagnosis of myotonic dystrophy made? (3)

A

Clinical features
EMG: dive bomber potentials
Genetic testing

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6
Q

What is management of myotonic dystrophy? (12)

A

Mexiletine may help myotonia (caution with arrhythmia)
Modafanil for sleepiness
Advise against general anaesthetic
PPM/ICD for arrhythmias/cardiomyopthay
SLT for dysphagia +/- PEG
NIV +/- cough assist device
Surveillance for cataracts
Management of diabetes
Laxatives etc for bowels
Physio/ OT for aids
Orthotics
Palliative care input

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7
Q

What are causes of bilateral ptosis? (5)

A

Myotonic dystrophy
Myasthenia gravis
Congenital
Oculopharyngeal muscular dystrophy
Mitochondrial disease e.g. Kearns-Sayres

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8
Q

What are causes of unilateral ptosis? (4)

A

Third nerve palsy
Horners syndrome
Posterior Communicating artery aneurysm
Diabetic neuropathy

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9
Q

What are causes of complex ophthalmoplegia? (9)

A

Myaesthenia gravis
Thyroid eye disease
Myopathies e.g. oculopharyngeal muscular dystrophy
Mononeuritis multiplex eg. diabetes causing multiple CN palsies
Mitochondrial disease e.g. Kearn’s Sayres
Miller fisher syndrome
Cavernous sinus pathology
Wernickes encephalopathy
Progressive supranuclear palsy

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10
Q

What are differentials for myasthenia? (4)

A

Lambert eaton myaesthenic syndrome: antibodies to presynaptic voltage gated calcium channels
Botulism
Mitochondrial disease e.g. Kearn’s sayres
Miller fisher variant of guillain barre syndrome

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11
Q

What is the pathophysiology of myasthenia gravis?

A

acquired autoimmune disorder
Antibodies to the postsynaptic AChR of the neuromuscular junction
Thymus is involved in 75% of cases (10-15% have thymoma of which 10% are malignant, 90% have thymic hyperplasia)
50% with thymoma get myasthenia gravis
Occurs in 1 in 10000
20-35 year old females (autoimmune, thymic hyperplasia) or >50 year old men (oculobulbar, thymoma)
Affects extraocular, bulbar, facial, neck, limb and trunk muscles usually in this order
15% have pure ocular MG
20% have pure bulbar
It can be induced by penicillamine

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12
Q

What are investigations of myasthenia gravis? (7)

A

Bloods: AChR Antibodies, antistriatal muscle antibodies, anti-MUSK, TFTs, FBC+CRP, CK (normal), U+E (low K+)
Tensilon test - IV anticholinesterase injection, look for improvement in ptosis, cardiac monitoring needed for bradycardia, conduction block and asystole
Icepack test (ice is applied to the patients eyelid for 2 minutes causing ptosis to improve in MG).
Nerve conduction studies and EMG: repetitive nerve stimulation test (reduced amplitude with repeated stimulation), single fiber EMG (jitter)
Sats, ABG, spirometry (FVC)
CXR
CT/MRI thymus

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13
Q

What is the management of myasthenia gravis? (8)

A

Manage precipitant: infection, drugs, noncompliance with medication, low K+
SALT, NG/PEG feeding
Anticholinesterases (pyridostigmine)
Steroids, gastro, bone protection
Steroid sparing agents (Azathioprine, mycophenolate)
Plasmapheresis
IVIG
Surgical: Thymectomy

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14
Q

What are findings of a 3rd nerve palsy? (5)

A

Complete ptosis: compressive cause
Partial ptosis: medical cause (paralysis of LPS muscle)
Dilated pupil not reactive to direct/consensual light/accommodation (loss of parasympathetic supply)
Eye abducted and depressed (unopposed LR6, SO4)
Diplopia in all directions
Difficulty with medial and superior gaze

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15
Q

What are causes of a 3rd nerve palsy? (9)

A

Midbrain stroke
SOL
Demyelination
Posterior communicating artery aneurysm (painful)
Cavernous sinus pathology: thrombosis, internal carotid artery aneurysm, pituitary tumour
Supraorbital fissure pathology: tumour, thyroid eye disease, fracture
Orbital mass/inflammation/cellulitis
Herniation of uncus through tentorium (false localising sign)
Medical causes (spare the pupil because the parasym fibres are on the outer surface of the nerve and have their own blood supply from nerve sheath vessels): mononeuritis multiplex eg. diabetes, hypertension, vasculitis, giant cell arteritis, myaesthenia gravis, thyroid eye disease, migraine

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16
Q

What are differentials of a dilated pupil? (7)

A

3rd nerve palsy
Holmes Adie
Mydriatic eye drops
Tricyclic antidepressants
Amphetamines
phaeochromocytoma
congenital

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17
Q

What are clinical signs of cerebellar syndrome? (11)

A

Scanning dysarthria /slurred speech
Absence of rebound phenomenon
Finger nose incoordination
Intention tremor
Dysdiadochokinesia
Hypotonia
Hyporeflexia
Nystagmus
Heel shin ataxia
Foot tapping - inability to alternate movements
Wide based gait

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18
Q

What are the clinical finding difference between cerebellar vermis vs lobe lesions?

A

Vermis: ataxic trunk and gait, normal limbs
Lobe: ipsilateral cerebellar signs in limbs

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19
Q

How does direction nystagmus help identify site of lesion?

A

Cerebellar: Fast phase towards lesion
Vestibular nucleus/VIII lesion: fast phase away from lesion

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20
Q

What are causes of cerebellar syndrome? (8)

A

PASTRIES
Paraneoplastic
Alcohol
Sclerosis (MS)
Tumour (posterior fossa)
Rare: friedrichs and ataxia telangectasia
Iatrogenic: phenytoin toxicity
Endocrine: hypothyroidism
Stroke: brainstem

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21
Q

What are possible aetiological clues for causes of cerebellar syndrome on examination? (5)

A

MS: Internuclear ophthalmoplegia, spasticity, female, younger age, optic atrophy
Friedrichs ataxia: optic atrophy, neuropathy
Bronchial carcinoma: clubbing, tar stained fingers, radiotherapy scar
Alcohol: stigmata of liver disease, unkempt, neuropathy
Phenytoin: gingival hyperplasia

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22
Q

What are signs of MS? (11)

A

Wheelchair
Ataxic handshake
INO
Optic atrophy
Reduced VA
Cranial nerve palsy
Spasticity in limbs
Upper motor neurone weakness
Brisk reflexes
Altered sensation
Cerebellar signs

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23
Q

What is internuclear ophthalmoplegia?

A

Medial longitudinal fasciculus lesion
Impaired adduction of the ipsilateral eye with nystagmus of the abducting eye
Eyes can converge normally

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24
Q

What are diagnostic criteria for MS?

A

McDonald criteria
CNS demyelination causing neurological impairment disseminated in time and space

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25
Q

What are causes of MS?

A

Multifactorial
Genetic: HLA DR2, interleukin 2 and 7
Environment: increasing latitude
EBV

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26
Q

What are investigations for MS? (4)

A

Exclude differentials: FBC, inflammatory markers, U&E, LFT, TFT, glucose, HIV serology, calcium and B12 levels
CSF: oligoclonal IgG bands, high protein
MRI: periventricular white matter plaques
Visual evoked potentials: if previous optic neuritis - delayed velocity, normal amplitude
NCS: demyelination in apparently unaffected pathways with characteristic delays

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27
Q

What are some none neurological clinical manifestations of MS? (6)

A

Depression
Urinary retention / incontinence
Impotence
Bowel issues
Uthoffs pnenomenon: worsening of symptoms after hot bath/ exercise
Lhermitte’s sign: lightening pains down spine on neck flexion

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28
Q

What is treatment for MS? (12)

A

MDT: nurse, physio, OT, social worker, neurologist
Methypred for relapse
Relapsing remitting: DMARDs: interferon beta, glatiramer, Peginterferon beta-1a
Monoclonal antibodies: alemtuzumab (CD52), natalizumab (a4 integrin)
Fingolimod
Secondary progressive: Interferon beta 1b, Siponimod
Primary progressive: Interferon beta limited evidence
Vitamin d
Cannabinoids
Anti spasmodics: baclofen
Carbemazepine for neuropathic pain
Laxatives
Intermittent catheterisation
Oxybutynin

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29
Q

What is prognosis for MS?

A

Majority will remain ambulant at 10 years

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30
Q

What are happens to MS in pregnancy?

A

Reduced relapse rate
Safe for foetus - possibly reduced birth weight
Increase risk of relapse in postpartum period

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31
Q

What is the difference between impairment, disability and handicap?

A

Impairment: arm paralysis
Disability: inability to write
Handicap: inability to work as an accountant as a result

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32
Q

What are clinical signs of stroke on inspection? (4)

A

Walking aids
NG tube /PEG
Posturing - flexed upper, extended lower limbs
Wasting or oedema on affected side

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33
Q

What are features of tone in stroke? (3)

A

Spastic rigidity
Clasp knife - resistance to movement then sudden release
Ankles may demonstrate clonus >4 beats

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34
Q

What is the MRC grade for power?

A

0 - none
1- flicker
2- moves with gravity neutralised
3-moves against gravity
4-reduced power against resistance
5-normal

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35
Q

What is the pattern of weakness typically in stroke?

A

Extensors weaker than flexors in upper limbs
Flexors weaker than extensors in lower limbs

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36
Q

What is bamford classification of stroke?

A

TACS: 3/3 of Unilateral weakness (and/or sensory deficit) of face, arm and leg
Homonymous hemianopia
Higher cerebral dysfunction (dysphasia, visuospatial disorder)

PACS: 2/3 of above

POCS: Cranial nerve palsy and a contralateral motor/sensory deficit
Bilateral motor/sensory deficit
Conjugate eye movement disorder (e.g. horizontal gaze palsy)
Cerebellar dysfunction (e.g. vertigo, nystagmus, ataxia)
Isolated homonymous hemianopia

Lacunar: Pure sensory stroke
Pure motor stroke
Sensori-motor stroke
Ataxic hemiparesis

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37
Q

What signs might help to identify cause of stroke? (4)

A

Irregular pulse for AF
BP
Cardiac murmurs
Carotid bruits

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38
Q

What is the difference between TIA and stroke?

A

Deficit <24 hours in TIA

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39
Q

What is the definition of stroke?

A

Rapid onset, focal neurological deficit due to a vascular lesion lasting >24 hours

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40
Q

What investigations should be done for stroke? (8)

A

Bloods: FBC, CRP/ESR (young CVA ?arteritis), glucose, U&Es
ECG: AF or previous infarction
CXR: cardiomegaly or aspiration
CT head: infarction or bleed, territory
Echo: AF, endocarditis, MI, murmur
Carotid Doppler
MRI/A if concern about dissection or CVST
Thrombophilia and vasculitis screen if young

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41
Q

What are causes of stroke? (6)

A

Ischaemic
Haemorrhagic
Carotid artery dissection
CVST
Vasculitis
Thrombophilia

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42
Q

What is the management of acute ischaemic stroke? (9)

A

Thrombolysis with tPA within 4.5 hours
Maintain homeostasis: glucose control, blood pressure, oxygen
Aspirin 300mg for 2 weeks then 75 clopidogrel
Longer term anti coagulation if AF
Longer term statin
MDT - physio, OT, SLT, psychological therapy, stroke rehab
IPCs for VTE prophylaxis
NG/PEG if unsafe swallow
Carotid endarterectomy if good recovery and >70% stenosis

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43
Q

What are risk factors for stroke? (11)

A

Hypertension.
Smoking.
Diabetes mellitus.
Heart disease (valvular, ischaemic, atrial fibrillation).
Peripheral arterial disease.
Post-TIA.
Polycythaemia vera.
Carotid artery occlusion.
Combined oral contraceptive pill.
Hyperlipidaemia.
Excess alcohol.
Clotting disorders.

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44
Q

What are differentials for a stroke like presentation acutely? (6)

A

hypoglycaemia
TIA in the first 24 hours of stroke.
Brain tumour.
Subdural haematoma.
Todd’s palsy.
acute poisoning if the patient is comatose.

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45
Q

What are dominant parietal lobe cortical signs in stroke?

A

Dysphasia - receptive, expressive or global
Gerstmanns syndrome - dysgraphia, dyslexia, dyscalculia, left- right disorientation, finger agnosia

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46
Q

What are non dominant parietal lobe signs in stroke?

A

Dressing and constructional apraxia
Spatial neglect

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47
Q

Which signs can be dominant or non dominant parietal lobe cortical signs in stroke?

A

Sensory and visual inattention
Astereognosis
Graphaesthesia

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48
Q

Where is the lesion if there is bitemporal hemianopia?

A

Optic chiasm

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49
Q

Where is the lesion if there is homonymous hemianopia ?

A

Optic tract - between chiasm and lateral geniculate nucleus

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50
Q

Where is the lesion if there is a homonymous quadrantopia?

A

Optic radiation post LGN
Upper = temporal lobe
Lower = parietal lobe

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51
Q

What is lateral medullary syndrome? (Wallenberg)

A

Posterior inferior cerebellar artery occlusion (PICA)
Cerebellar signs
Nystagmus
Horner syndrome
Palatal paralysis and decreased gag reflex
Loss of Trigeminal pain and temp sensation

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52
Q

What are clinical signs of spasticity of legs? (6)

A

Wheelchair and walking sticks
Disuse atrophy and contractures
Increased tone and ankle clonus
Generalised weakness
Hyper reflexia and extensor plantars
Scissoring gait

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53
Q

What signs may point to a cause of spasticity in legs? (4)

A

Sensory level suggestive of spinal legion
scars on back or spinal deformity
MS features - cerebellar signs, optic atrophy
Bladder symptoms/ catheter/anal tone

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54
Q

What are causes of spasticity in legs? (10)

A

MS
Spinal cord compression /cervical myelopathy
Trauma
Motor neurone disease
Anterior spinal artery thrombosis
Syringomyelia
Hereditary spastic paraplegia
Subacute combined degeneration of cord
Friedreichs ataxia
Parasagittal falx meningioma

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55
Q

What can be causes of cord compression? (4)

A

Disc prolapse above L1/L2
Malignancy
Infection - abscess or TB
Trauma - # vertebra

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56
Q

What movements are done by which lumbo-sacral root levels?

A

L2/3 - hip flexion
L3/4 - knee extension (knee jerk reflex)
L4/5 - foot dorsiflexion
L5/S1 - knee flexion and hip extension
S1/S2- foot plantar flexion (ankle jerk reflex)

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57
Q

Which dermatome covers medial lower leg?

A

L4

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58
Q

Which dermatome covers knee?

A

L3

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59
Q

Which dermatome covers lateral foot and lower leg posteriorly?

A

S1

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60
Q

Which dermatome covers anus?

A

S2-4

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61
Q

What are signs of syringomyelia? (8)

A

Weakness and wasting of small muscles of hand
Loss of reflexes in upper limbs
Dissociated sensory loss in upper limbs and chest - loss of pain and temp with preservation of vibration
Scars from painless burns
Charcot joints - elbow and shoulder
Pyramidal weakness in lower limbs with upgoing plantars
Horners syndrome
If syrinx extends to brain stem - cerebellar/cranial nerve signs

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62
Q

What is syringomyelia?

A

Progressively expanding fluid filled cavity in cervical cord
Typically spans several levels
Associated with Arnold chiari malformation and spina bifida

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63
Q

Which spinal cord tracts are usually affected by syringomyelia? (3)

A

Decussating spinothalamic neurones: pain and temp loss at level of syrinx
Anterior horn cells: LMN weakness at level of syrinx
Corticospinal tract: UMN weakness below syrinx

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64
Q

What is a Charcot joint?

A

Painless deformity and destruction of joint with new bone formation following repeated minor trauma secondary to loss of pain sensation

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65
Q

What are important causes of Charcot joint? (3)

A

Tabes dorsalis: hip and knee
Diabetes: foot and ankle
Syringomyelia: elbow and shoulder

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66
Q

Which cervical/thoracic roots are responsible for movements of upper limbs?

A

C5/6 - elbow flexion and supination (biceps and supinator jerk)
C7/8 - elbow extension (triceps jerk)
T1 - finger adduction

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67
Q

Which dermatome covers thumb?

A

C6

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68
Q

Which dermatome covers middle finger?

A

C7

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69
Q

Which dermatome covers little finger?

A

C8

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70
Q

What are signs of motor neurone disease? (6)

A

Wasting and fasciculation including tongue
Spastic and or flaccid tone
Weakness
Absent and or brisk reflexes
Sensory exam normal
Bulbar or pseudo bulbar speech

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71
Q

What is the difference between bulbar and pseudobulbar speech?

A

Bulbar: LMN lesion, nasal, Donald duck speech, due to palatal weakness
Pseudobulbar: UMN, hot potato speech, due to spastic tongue

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72
Q

What is MND?

A

Progressive neurological condition causing weakness of unknown aetiology
Axonal degeneration of upper and lower motor neurones

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73
Q

What are causes of bulbar palsy? (7)

A

Botulism
MND
MG
Lyme disease
Guillain barre
Poliomyelitis
Acute intermittent porphyria

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74
Q

What are causes of pseudobulbar palsy? (4)

A

MND
CVA of internal capsule or high brainstem
Parkinsons /PSP
MS

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75
Q

What are different types of MND? (3)

A

ALS: affects cortico spinal tracts, predominantly spastic paraparesis
Progressive muscular atrophy: anterior horn cells, predominantly wasting, fasciculations and weakness, best prognosis
Progressive bulbar palsy: lower cranial nerves, suprabulbar nuclei, speech and swallowing issues, bad prognosis

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76
Q

What are investigations for MND? (3)

A

Clinical diagnosis
EMG: fasciculations
MRI brain and spine: exclude other differentials of cord compression, myelopathy and brain stem lesions

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77
Q

What is treatment for MND? (3)

A

Supportive: PEG, NIV
MDT
Riluzole slows progression by 3 months

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78
Q

What is prognosis of MND?

A

Most die within 3 years of diagnosis from pneumonia and resp failure
Worst if elderly, female, bulbar involvement

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79
Q

What are differentials for generalised wasting of hand muscles? (4)

A

Anterior horn cell: MND, Syringomyelia, cervical cord compression, polio
Brachial plexus: cervical rib, Pancoast tumour, trauma
Peripheral nerve: combined median and ulnar nerve lesions, peripheral neuropathy
Muscle: disuse atrophy eg RA

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80
Q

What is fasciculation?

A

Visible muscle twitching at rest
Caused by axonal loss resulting in surviving axons recruiting and innervating more myofibrils than usual resulting in large motor units
Seen in MND and Syringomyelia

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81
Q

What are clinical signs of Parkinson’s? (7)

A

Expressionless face with absence of spontaneous movements
Coarse, pill rolling 3-5Hz tremor, usually asymmetrical
Bradykinesia
Cogwheel rigidity at wrists enhanced by synkinesis
Shuffling and festinant gait
Absent arm swing - asymmetrical
Slow faint and monotonous speech

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82
Q

What are some additional signs in Parkinson’s that might signify Parkinson’s plus syndromes? (4)

A

BP: evidence of MSA, postural hypotension
Cerebellar signs: MSA
Vertical eye movements: PSP lack of upgaze
Dementia: lewy body

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83
Q

What are causes of Parkinson’s? (8)

A

Parkinsons disease: idiopathic
MSA
PSP
Corticobasal degeneration
Drug induced
Anoxic brain damage
Post encephalitis
MPTP toxicity - frozen addict syndrome

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84
Q

What is the pathology of idiopathic Parkinson’s disease?

A

Degeneration of dopamingeric neurones between substantia nigra and basal ganglia

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85
Q

What is treatment for Parkinson’s disease? (7)

A

Levodopa with dopadecarboxylase inhibitor eg co-beneldopa
Dopamine agonist eg pergolide/apomorphine
MAOB inhibitor eg Selegilline
Anti cholinergics
COMT inhibitors eg entacapone
Amantadine
Deep brain stimulation

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86
Q

What are causes of tremor? (3)

A

Resting: Parkinson’s disease
Postural: benign essential tremor, anxiety, Thyrotoxicosis, metabolic encephalopathy, alcohol
Intention: cerebellar disease

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87
Q

What is hereditary sensory motor neuropathy?

A

Charcot Marie tooth disease
Or peroneal muscular atrophy

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88
Q

What are signs of hereditary sensory motor neuropathy? (8)

A

Wasting of distal lower limb muscles with preservation of thigh muscle bulk (inverted champagne bottle)
Pes cavus
Weakness of ankle dorsiflexion and toe extension
Variable degree of stocking distribution sensory loss
High stepping gait due to foot drop
Stamping gait due to absent proprioception
Wasting of hand muscles
Palpable lateral popliteal nerve

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89
Q

What are the types of hereditary sensory motor neuropathies and how are they inherited?

A

HSMN type 1: demyelinating
HSMN type 2: axonal
Autosomal dominant inheritance

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90
Q

What are causes of sensory predominant peripheral neuropathy? (4)

A

Diabetes
Alcohol
Drugs: isoniazid and vincristine
Vitamin B12 and B1 deficiency

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91
Q

What are causes of predominantly motor peripheral neuropathy? (4)

A

Acute: Guillain barre and botulism
Lead toxicity
Porphyria
HSMN

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92
Q

What are causes of mononeuritis multiplex? (5)

A

Diabetes
Connective tissue disease eg SLE/RA
Vasculitis eg PAN/churg-strauss
Infection eg HIV
Malignancy

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93
Q

What are signs of friedrichs ataxia? (9)

A

Young adult in wheelchair
Pes cavus
Bilateral cerebellar ataxia
Leg wasting with absent reflexes and bilateral upgoing plantars
Dorsal column signs: loss of vibration and joint position sense
High arched palate
Optic atrophy
Sensorineural deafness
Murmur of HOCM

94
Q

How is friedrichs ataxia inherited and what is prognosis?

A

Autosomal recessive
Onset during teenage years
Survival rarely exceeds 20 years from diagnosis

95
Q

What are causes of extensor plantars with absent knee jerks? (6)

A

Friedrichs ataxia
Subacute combined degeneration of cord
MND
Tertiary syphillis
Conus medullaris lesions T12-L2
Dual pathology eg cervical spondylosis with peripheral neuropathy

96
Q

What are signs of facial nerve palsy? (4)

A

Unilateral facial droop
Absent nasolabial fold and forehead creases
Inability to raise eyebrows, screw eyes up or smile
Bells phenomenon: eyeball rolls up on attempted eye closure

97
Q

How can you identify level of lesion in facial nerve palsy? (4)

A

Pons: VI palsy and long tract signs
Cerebellar-pontine angle: V, VI, VIII and cerebellar signs
Auditory canal: VIII
Neck and face: scars or parotid mass

98
Q

What are causes of facial nerve palsy? (9)

A

Bells: rapid onset, HSV1 implicated, swelling and compression of nerve in facial canal caused demyelination and conduction block
Herpes zoster: Ramsay hunt syndrome
diabetes
sarcoidosis
Lyme disease
Tumour: cerebellopontine angle, parotid
trauma
MS
Stroke

99
Q

What are causes of bilateral facial palsy? (5)

A

Guillain barre
Myasthenia gravis
Sarcoidosis
Bilateral Bell’s palsy
Lyme disease

100
Q

What are clinical signs of myasthenia gravis? (6)

A

Bilateral ptosis worse on sustained up gaze
Complicated bilateral extra ocular muscle palsies
Myasthenic snarl
Nasal speech, palatal weakness and poor swallow (bulbar involvement)
Proximal muscle weakness with fatiguability
Thymectomy scar

101
Q

What are associations of myasthenia gravis? (2)

A

Other autoimmune conditions: diabetes, RA, Thyrotoxicosis, SLE
Thymoma

102
Q

What is the pathophysiology of myasthenia gravis?

A

Anti nicotinic acetylcholine receptor antibodies affect motor end plate neurotransmission

103
Q

What are investigations for myasthenia? (7)

A

Anti AChR antibodies 90%
Anti MuSK antibodies
EMG: decremented response to train of impulses
Ice pack test
Tensilon test if diagnostic doubt: risk of heart block
CT chest for thymoma
TFTs; graves present in 5%

104
Q

What are treatments for myasthenia? (5)

A

Acute: IV immunoglobulin or plasmapheresis
Chronic: ACh esterase inhibitor - pyridostigmine
Immunosuppression: steroids, azathioprine
Thymectomy
Early ICU involvement if bulbar symptoms /declining FVC or single breath count

105
Q

Which drugs can cause worsening of myasthenia symptoms? (12)

A

Macrolide abx - azithromycin etc
Aminoglycoside abx - gent etc
Fluoroquinolone abx - ciprofloxacin
Steroids - can cause initial worsening
Procainamide
Beta blockers
Botox
Penicillamine
Quinine
Magnesium
Statins
Desferioxamine

106
Q

What is lambert Eaton myasthenic syndrome?

A

Associated with malignancy eg small cell lung cancer
Antibodies to pre synaptic calcium channels

107
Q

How does examination differ in lambert Eaton myasthenia vs MG? (3)

A

Diminished reflexes that become brisker after exercise
Lower limb girdle weakness (rather than upper limb in MG)
EMG shows second wind phenomenon on repetitive stimulation

108
Q

What are causes of bilateral extra ocular palsies? (5)

A

Myasthenia gravis
Graves’ disease
Mitochondrial cytopathies eg kearns sayre syndrome
Miller fisher variant GBS
Cavernous sinus pathology

109
Q

What are causes of bilateral ptosis? (6)

A

Congenital
Senile
Myasthenia gravis
Myotonic dystrophy
Mitochondrial cytopathies eg kearns sayre syndrome
Bilateral Horner’s

110
Q

What is kearns sayre syndrome?

A

Mitochondrial cytopathy
Onset before the age of 20, Chronic progressive external ophthalmoplegia and pigmentary retinopathy

111
Q

What is tuberous sclerosis?

A

multisystem autosomal dominant genetic disease TSC1 and 2.
Hamartomas in brain, kidneys, heart, liver, eyes, lungs and skin
Symptoms; seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung disease, and kidney disease

112
Q

What skin changes are characteristic of tuberous sclerosis? (4)

A

Facial adenoma sebaceum, butterfly distribution (angiofibromata)
Periungual fibromas
Shagreen patch: rough leathery skin over lumbar region
Ash leaf macules: depigmented macules on trunk

113
Q

What respiratory involvement can occur in tuberous sclerosis?

A

Cystic lung disease - Lymphangioleiomyomatosis (LAM)

114
Q

What renal involvement can occur in tuberous sclerosis? (3)

A

Renal enlargement from cysts/angiomyolipomata
Need for renal transplant /dialysis
Renal cell carcinoma can be complication

115
Q

Why is there overlap of ADPKD and tuberous sclerosis?

A

Genes for both are contiguous on chromosome 16 so some mutations cause both conditions

116
Q

What eye involvement occurs in tuberous sclerosis?

A

Retinal phakomas: dense white patches in 50%

117
Q

What CNS involvement occurs in tuberous sclerosis? (5)

A

Developmental delay
Seizures
Giant cell astrocytoma: can lead to hydrocephalus
Cortical tubers
Subependymal nodules: form in the walls of ventricles

118
Q

What signs might a patient have that suggest they are on anti seizure drugs? (4)

A

Gum hypertrophy, hirsuitism, ataxic, tremor if on phenytoin

119
Q

What investigations should be done for tuberous sclerosis? (4)

A

Skull films: railroad track calcification
CT/MRI head: tuberous masses in cerebral cortex, often calcified
Echo: cardiac rhabdomyomas
Abdominal ultrasound: renal cysts/hamartomas

120
Q

What are clinical signs of neurofibromatosis? (8)

A

Cutaneous neurofibromas: 2 or more
Cafe au lait patches: 6 or more >15mm
Axillary freckling
Lisch nodules: melanocytic hamartomas of iris
BP: HTN from RAS and phaeochromocytoma
Chest: fine crackles from fibrosis
Neuropathy with enlarged nerves
Visual acuity: optic glioma/compression

121
Q

What is neurofibromatosis?

A

Autosomal dominant inheritance
Type 1: chromosome 17 peripheral
Type 2: chromosome 22 bilateral acoustic neuromas and sensorineural deafness

122
Q

What conditions are associated with neurofibromatosis? (2)

A

Phaeochromocytoma
Renal artery stenosis

123
Q

What are complications of neurofibromatosis? (4)

A

Epilepsy
Sarcomatous change
Scoliosis
Intellectual disability

124
Q

What are causes of enlarged nerves and peripheral neuropathy? (5)

A

Neurofibromatosis
Leprosy
Amyloidosis
Acromegaly
Refsum’s disease

125
Q

What are causes of enlarged nerves and peripheral neuropathy? (5)

A

Neurofibromatosis
Leprosy
Amyloidosis
Acromegaly
Refsum’s disease

126
Q

What are signs of horners? (5)

A

Partial ptosis (levator palpebrae partially supplied by sympathetic fibres)
Enophthalmos
Anhydrosis
Small pupil (miosis)
Flushed/warm skin ipsilaterally

127
Q

What might you find on examination hinting at cause of Horners?

A

Neck scars - central line, carotid endarterectomy, aneurysms, pancoasts tumour

128
Q

What are causes of Horners? (3)

A

Brain stem: MS, wallenbergs stroke
Spinal cord: syrinx
Neck: aneurysm, pancoasts tumour

129
Q

What is a holmes - adie pupil and what are features? (5)

A

Benign condition, more in females
Moderately dilated pupil
Poor response to light
Sluggish response to accommodation
Absent or diminished ankle and knee jerks

130
Q

What is an Argyll Robertson pupil and what are features? (5)

A

Manifestation of quaternary syphillis or diabetes
Small irregular pupil
Accommodates but doesn’t react to light
Atrophied and depigmented iris
May have sensory ataxia and bilateral ptosis- tabes dorsalis

131
Q

How do you test for syphilis?

A

Treponema Pallidum Hemagglutination Assay (TPHA)
or
fluorescent treponemal antibody absorption (FTA-ABS)

132
Q

What are signs of oculomotor (III) nerve palsy? (3)

A

Complete ptosis
Dilated pupil
Eye down and out due to unopposed lateral rectus (VI) and superior oblique (IV)

133
Q

What will be difference between medical and surgical causes of 3rd nerve palsy?

A

Medical: normal pupil, non traumatic, affecting core of nerve only
Surgical: pupil involved, parasymp fibres run on outside so blocked by compressive lesions

134
Q

What are causes of a medical 3rd nerve palsy? (4)

A

Mononeuritis multiplex: diabetes
Midbrain infarction: webers
Midbrain demyelination: MS
Migraine

135
Q

What are surgical causes of a 3rd nerve palsy? (3)

A

Communicating artery aneurysm (posterior)
Cavernous sinus: thrombosis, tumour, fistula
Cerebral uncus herniation

136
Q

What are clinical signs of optic atrophy? (2)

A

Relative afferent pupillary defect
Disc pallor on fundoscopy

137
Q

Which conditions can cause optic atrophy and what clinical signs hint at it? (8)

A

Glaucoma: cupping of disc
Retinitis pigmentosa: bone spicule pigmentation
Central retinal artery occlusion: pale retina with cherry red spot
Frontal brain tumour: foster Kennedy syndrome - papilloedema in 1 eye to due raised ICP and optic atrophy in other due to compression by tumour
MS: cerebellar signs, INO
Friedrichs ataxia: scoliosis, Pes cavus
Paget’s: large bossed skull, hearing aids
Tertiary syphillis: Argyll Robertson pupil

138
Q

What are causes of PALE DISCS?

A

Pressure: tumour, glaucoma, Paget’s
Ataxia: friedrichs
LEbers: mitochondrial disorder
Dietary: b12 deficiency
Degenerative: retinitis pigmentosa
Ischaemia: CRAO
Syphilis, CMV, toxoplasmosis
Cyanide, alcohol, lead, tobacco
Sclerosis: MS

139
Q

What are signs of AMD?

A

Wet: neovascular and exudative
Dry: non- neovascular, atrophic and non exudative
Macular changes: Drusen, geographical atrophy, fibrosis

140
Q

What are risk factors for AMD? (5)

A

Age
White race
Family history
Smoking
Wet: higher incidence of IHD and stroke

141
Q

What are treatments for AMD?

A

Ophthalmology referral
Wet: intra vitreal injections of anti VEGF

142
Q

What is prognosis for AMD?

A

Majority progress to blindness in affected eye within 2 years of diagnosis

143
Q

What are clinical signs of retinitis pigmentosa? (5)

A

White stick and braille book
Reduced peripheral vision
Fundoscopy: bone spicule pigmentation peripherally, follows veins, spares macula
Optic atrophy
Cataracts

144
Q

Which conditions are associated with retinitis pigmentosa? (5)

A

Ataxia: abetalipoproteinaemia, refsums, kearns sayre
Deafness: refsums, kearns sayre, ushers disease
Ophthalmoplegia/ptosis: kearns sayre
Polydactyly: Laurence-moon-biedl
Icthyosis: refsums

145
Q

What are features of Laurence moon biedl syndrome? (6)

A

Autosomal recessive condition
retinitis pigmentosa
polydactyly
obesity
hypogonadism
Intellectual disability

146
Q

What are features of Refsum’s disease and what causes it? (6)

A

Autosomal recessive
Accumulation of phytanic acid
Ataxia
Icthyosis
Deafness
Retinitis pigmentosa

147
Q

What are features of kearns sayre syndrome and what causes it? (8)

A

Mitochondrial disorder
Chronic progressive external ophthalmoplegia
Ptosis
retinitis pigmentosa
Cardiac conduction abnormalities
Ataxia
Deafness
Diabetes

148
Q

What are features of friedrichs ataxia and what causes it? (7)

A

Autosomal recessive, trinucleotide repeat causing mitrochondrial dysfunction
Ataxia
Hypertrophic cardiomyopathy
Diabetes
Pes cavus
Scoliosis
Optic atrophy

149
Q

What is retinitis pigmentosa?

A

Inherited form of retinal degeneration characterised by loss of photo receptors

150
Q

What are causes of retinitis pigmentosa? (2)

A

Congenital: autosomal recessive, 15% due to rhodopsin pigment mutations
Acquired: post inflammatory retinitis

151
Q

What is prognosis in retinitis pigmentosa?

A

Progressive loss of vision due to retinal degeneration
Initially reduced night vision
Most registered blind at 40
Central vision loss in 7th decade
No treatment although vit A may slow progression

152
Q

What are causes of tunnel vision? (6)

A

Papilloedema
Glaucoma
Choroidoretinitis: CMV/toxo/bechets
Retinitis pigmentosa
Migraine with Aura
Panic attack

153
Q

What are signs of retinal artery occlusion? (2)

A

Pale milky fundus with thread like arterioles
Cherry red macula

154
Q

What are causes of retinal artery occlusion? (2)

A

Embolic: AF, Carotid stenosis
Giant cell arteritis

155
Q

What are signs of retinal vein occlusion? (3)

A

Flame haemorrhages radiating from optic disc
Engorged tortuous veins
Cotton wool spots

156
Q

What are causes of retinal vein occlusion? (4)

A

HTN
Diabetes
Hyperviscocity: waldenstroms or myeloma
Glaucoma

157
Q

Which muscles of the hand are supplied by the median nerve?(4)

A

Lumbricals
Opponens pollicis
Abductor pollicis brevis
Flexor pollicis brevis

158
Q

What is Hoffmans sign?

A

UMN lesion causes reflex flexion-adduction of thumb and fingers when distal phalanx of middle finger forcibly flexed

159
Q

What are differentials for bilateral spastic paraplegia? (12)

A

Hereditary spastic paraplegia
Cerebral palsy
MS
Trauma
Friedrichs ataxia
HIV
Spinal cord tumour
MND
Syringomyelia
Subacute combined degeneration of cord
Transverse myelitis
Anterior spinal artery thrombosis

160
Q

What is an intracranial bilateral cause of spastic paraparesis?

A

Parasagital falx meningioma

161
Q

Where is the lesion in patients with spastic paresis of one leg? How would you differentiate

A

Could be spinal cord or brain
Progression to the arm does not help differentiate, neither does spread to other leg
Need imaging of both to look for lesion

162
Q

What is hereditary spastic paraplegia?

A

Autosomal dominant condition
Spasticity more prominent than weakness
Age of onset variable
Can have vibration sense loss

163
Q

How do you localise a lesion to L2/3? (4)

A

Weakness of hip flexors and quads
Knee jerk affected
Radicular pain: anterior thigh, groin, testicle
Sensory deficit: anterior thigh

164
Q

How do you localise a lesion to L4? (4)

A

Weakness: quads, tibialis anterior and posterior
Knee jerk affected
Radicular pain: anteromedial leg
Sensory deficit: anteromedial leg

165
Q

How do you localise a lesion to L5? (4)

A

Weakness; hamstrings, peroneus longus, extensors of toes
No reflexes involved
Radicular pain: buttock, posterior thigh, anterolateral leg, dorsum of foot
Sensory: dorsum of foot, anterolateral aspect leg

166
Q

How do you localise lesion to S1?(4)

A

Weakness: plantar flexors, extensor digitorum brevis, peroneus longus, hamstrings
Ankle jerk affected
Radicular pain: buttock, back of thigh, calf and lateral foot
Sensory: lateral foot

167
Q

What is important to examine in a patient with hemiplegia? (9)

A

Neuro exam as normal
Homonymous hemianopia
Sensory inattention
Carotid bruits
Speech defects
Pulse for AF
Heart for murmurs
BP
Urine for sugar

168
Q

What are causes of hemiplegia? (8)

A

Stroke
Tumour
Subdural haematoma
Syphilis
MS
Trauma
Connective tissue disorder
Intracranial infection

169
Q

What scoring system is useful in risk stratifying strokes?

A

NIHSS: measure of overall neurological deficit

170
Q

What are important examination features in a patient with ptosis? (7)

A

Complete or incomplete
Unilateral or bilateral
Pupil constricted: Horner’s
Pupil dilated: 3rd nerve palsy
Extraocular muscles involved: 3rd nerve or myasthenia
Eyeball sunken
Light reflex: intact in horners

171
Q

What additional things should you examine in Horner’s? (9)

A

Ptosis
Pupil constriction
Supraclavicular area: percuss for dullness of pancoasts, lymph nodes, scar from sympathectomy
Neck: carotid and aortic aneurysms, tracheal deviation (pancoasts)
Hands: small muscle wasting, clubbing, pain sensation (synringomyelia)
Cerebellar signs
Cranial nerves
Optic discs
Pyramidal signs

172
Q

What additional feature differentiates congenital Horner’s from other causes?

A

Heterochromia of iris: remains grey-blue

173
Q

How can you differentiate level of lesion in horners? (3)

A

Sweating over entire half of head, arm and upper trunk means central lesion
Sweating only on face: proximal to superior cervical ganglion
Sweating not affected: distal to superior cervical ganglion

174
Q

What are causes of unilateral ptosis? (5)

A

Third nerve palsy
Horners syndrome
Myasthenia gravis
Congenital
idiopathic

175
Q

What are causes of bilateral ptosis? (8)

A

Myasthenia gravis
Myotonic dystrophy
Ocular myopathy
Oculopharyngeal dystrophy
Mitochondrial dystrophy
Tabes dorsalis
Congenital
Bilateral Horners (Syringomyelia)

176
Q

What are causes of an Argyll Robertson pupil? (9)

A

Neuro syphilis
Diabetes
Pinealoma
Brainstem encephalitis
MS
Lyme disease
Sarcoidosis
Syringobulbia
Tumours of posterior 3rd ventricle

177
Q

What are causes of aniscoria (unequal pupils)? (7)

A

20% normal individuals
Third nerve palsy
Iritis
Blindness in one eye
Stroke/bleed
Head trauma
Hemianopia due to optic tract involvement

178
Q

What are causes of a dilated pupil? (7)

A

Mydriatic drops
Third nerve lesion
Holmes adie syndrome
Lens implant/iridectomy
Blunt trauma to iris
Drugs: cocaine, amphetamine
Deep coma

179
Q

What are causes of a small pupil? (6)

A

Old age
Pilocarpine drops
Horners syndrome
Argyll Robertson pupil
Pontine lesion
Narcotics

180
Q

What are causes of a central scotoma? (12)

A

MS
Optic nerve compression due to tumour or aneurysm
Glaucoma
Toxins: methanol, lead, arsenic
CRAO
Temporal arteritis
Syphyllis
Friedrichs ataxia
Lebers optic atrophy
Pagets
B12 deficiency
Retinitis pigmentosa

181
Q

What are causes of tunnel vision? (6)

A

Optic atrophy
Retinitis pigmentosa
Choroidoretinitis
Glaucoma
Hysteria
Significant refractive error

182
Q

What are causes of cerebellar syndrome? (9)

A

MS
Brainstem vascular lesion
Phenytoin toxicity
Alcohol
SOL
Hypothyroidism
Paraneoplastic manifestation of bronchogenic carcinoma
Friedrichs ataxia
Congenital malformation at foramen magnum

183
Q

How can you localise cerebellar signs? (3)

A

Gait: anterior lobe
Truncal: flocconodular or posterior lobe
Limb: lateral lobes

184
Q

What are differentials for nystagmus? (9)

A

Labrynthitis
Menieres
Acoustic neuroma
Otitis media
Head injury
MS
Stroke
Brainstem tumour
Wernickes

185
Q

Where are the lesions with expressive or receptive dysphasia?

A

Dysphasia due to lesion in dominant hemisphere
Expressive: Broca’s area in inferior frontal lobe
Receptive: wernickes area in superior temporal lobe

186
Q

What are causes of dysarthria? (6)

A

Stutter
Cranial nerve palsy: bell’s, 9th, 10th, 11th
Cerebellar disease
Parkinsons
Pseudobulbar palsy
Bulbar palsy

187
Q

What are parietal lobe signs? (6)

A

Localisation of touch, position, joint sense, temperature
Loss of two point discrimination
Astereognosis
Dysgraphaesthesia
Sensory inattention
Homonymous hemianopia or lower quadrantic hemianopia

188
Q

Where will the patient have diplopia in a 3rd nerve palsy?

A

In all directions except on lateral gaze to the side of the palsy

189
Q

What are the components of paralysis in 3rd nerve palsy? (4)

A

Unilateral ptosis: levator palpebrae superioris paralysis
Dilated pupil reacting slowly to light: pupil constrictor paralysis
Lack of accommodation: ciliary muscle involvement
Squint and diplopia: superior, inferior, medial recti and inferior oblique paralysis

190
Q

What are important investigations for a 3rd nerve palsy? (7)

A

Blood pressure
Glucose
ESR (exclude temporal arteritis)
Anti AChR and anti musk for MG
TFTs and orbital USS for thyroid eye disease
CT head
Arteriography if painful and pupil involvement

191
Q

What is Webers syndrome?

A

Ipsilateral 3rd nerve palsy with contralateral hemiplegia
Midbrain lesion

192
Q

What are causes of 6th nerve palsy? (8)

A

HTN
Diabetes
Raised ICP (false localising sign)
MS
Basal meningitis
Encephalitis
Acoustic neuroma
Nasopharyngeal carcinoma

193
Q

Where is the nucleus of the 6th nerve located?

A

Pons

194
Q

What structures sit close to the sixth nerve nucleus and fascicles? (4)

A

Facial and Trigeminal nerves
Corticospinal tract
Median longitudinal fasciculus
Parapontine reticular formation

195
Q

How do you differentiate between upper and lower motor neurone lesions of 7th nerve?

A

Lower motor neurone: whole face including forehead
Upper: forehead spared

196
Q

What are the branches of the facial nerve? (4)

A

Greater superficial petrosal nerve: lacrimal, nasal and palatine glands
Stapedius
Chorda tympani: taste to anterior 2/3 tongue, submaxillary and sublingual glands
Motor branches through stylomastoid foramen

197
Q

How do you localise 7th nerve palsy? (3)

A

Pons: ipsilateral 6th
Cerebellopontine angle: 5th and 8th nerve associated
Bony canal: loss of taste, hyperacusis

198
Q

What are causes of chorea? (8)

A

Sydenhams Chorea: rheumatic fever
Huntingtons disease
Polycythemia vera
Chorea gravidarum
Drug induced
SLE
Thyrotoxicosis
If hemi: infarction or tumour

199
Q

What are causes of bilateral high stepping gait? (3)

A

Peripheral neuropathy: CMT, CIDP, leprosy
Neuromuscular: inclusion body myositis, MND, myotonic dystrophy
Cauda equina

200
Q

What are causes of unilateral foot drop/high stepping gait and how do you differentiate between them? (4)

A

Common peroneal nerve palsy: weak ankle dorsiflexion, eversion, inversion, normal ankle jerk, sensory loss outside leg and foot
L4/5 root lesion: as above but also weak hip abduction, sensory loss L4/5
Peripheral neuropathy: weakness of all muscles including plantar flexion, stocking sensory loss
Sciatic nerve lesion: loss of power below knee, loss of knee flexion
loss of ankle jerk and plantar response; not knee jerk, loss of sensation below knee on lateral side

201
Q

What are differentials for tongue atrophy and fasciculations? (3)

A

MND
Tumour
Stroke

202
Q

What are differentials for Optic neuritis/neuropathy causes? (7)

A

Demyelinating: MS
Autoimmune: Neuromyelitis optica, bechets, SLE
Ischaemic: giant cell, non arteritic
Infection: TB, syphilis, Lyme
Nutrition: b12 deficiency
Drugs: methanol, tobacco, ethambutol , isoniazid
Inherited: lebers hereditary ON

203
Q

How can you distinguish clinically between ulnar nerve problem and C8-T1 radiculopathy?

A

abductor and flexor pollicis brevis, opponens pollicis, and lateral two lumbricals involved in C8-T1 (median nerve)
Sensation only in ulnar distribution if ulnar nerve palsy

204
Q

Where can ulnar nerve get trapped? (2)

A

Cubital tunnel syndrome at elbow
Guyon’s canal syndrome at wrist

205
Q

Which muscles are supplied by ulnar nerve?

A

Two muscles of anterior forearm – flexor carpi ulnaris and medial half of flexor digitorum profundus
Intrinsic muscles of hand (apart from thenar muscles and two lateral lumbricals)

206
Q

Which nerve and muscles provide function for wrist extension?

A

Radial nerve
Extensor Carpi Radialis Longus (prime mover), Extensor Carpis Radialis Brevis (prime mover), Extensor Carpi Ulnaris, Extensor Digitorum (assists only)

207
Q

What are drug treatments for neuropathic pain? (4)

A

TCA: Amitriptyline
Anti seizure drugs: Gabapentin/pregabalin
SNRI: Duloxetine
Topical: capsaicin

208
Q

What are causes of peripheral neuropathy? (7)

A

Metabolic: diabetes, hypothyroidism, uraemia, b1/6/12 deficiency
Toxic: chemo, alcohol, amiodarone, isoniazid, nitrofurantoin
Inflammatory: CIDP, sarcoidosis, vasculitis, RA, SLE
Paraneoplastic: lung cancer, myeloma
Inherited: CMT
Infection: HIV, Lyme disease
Idiopathic

209
Q

What useful info can be gained from NCS?

A

Look at axonal vs demyelinating
Length dependent?

210
Q

What is the difference between small and large fibre neuropathy?

A

Large fiber neuropathy: loss of joint position and vibration sense and sensory ataxia
Small fiber neuropathy: impairment of pain, temperature and autonomic functions

211
Q

How do you differentiate between middle cerebral artery and posterior cerebral artery lesion causing homonymous hemianopia?

A

Macular sparing in posterior

212
Q

What additional things might you examine for in stroke to look for cause? (4)

A

Pulse for AF
Carotids for bruit
Heart sounds for murmur
BP

213
Q

What are differentials for bilateral spastic paraparesis and how do you localise? (5)

A

If sensory level: disc prolapse, spinal stroke, transverse myelitis
If dorsal column sensory loss: MS, Friedreich’s ataxia, subacute combined degeneration of cord, cervical myelopathy
If cerebellar signs: MS, Friedrichs
If RAPD/INO: MS
If fasciculations: MND, cervical myelopathy

214
Q

What are causes of cerebellar ataxia? (6)

A

Acute: stroke
Relapsing/remitting: MS
Alcohol related
Genetic: freidrichs, ataxia-telangiectasia
Paraneoplastic
Drugs: phenytoin, lithium, valproate

215
Q

What are causes of fasciculations? (3)

A

MND
Multi focal motor neuropathy
SMA

216
Q

What are differentials for MND? (3)

A

Multi focal motor neuropathy (LMN only)
Kennedys disease (X-linked spinal bulbar muscular atrophy) (LMN)
SMA in young patients

217
Q

What are causes of peripheral neuropathy? (9)

A

Metabolic: diabetes, hypothyroidism, renal failure, vit B1/6/12 def
Drugs: isoniazid, nitrofurantoin, phenytoin, chemo
Toxin: alcohol, lead
Infection: HIV, Lyme, leprosy
Malignancy associated: small cell
Inflammatory: GBS, CIDP
Hereditary: CMT
Immune: RA, SLE, PAN
Traumatic: compression

218
Q

What are pathognomonic findings in Kennedys disease?

A

LMN weakness only
Peri oral fasciculations

219
Q

What is post polio syndrome?

A

disorder of nerves and muscles
many years after they had polio
new muscle weakness, gets worse over time, pain in muscles and joints, and tiredness
often feel exhausted

220
Q

What is polio?

A

LMN syndrome caused by poliovirus
Causes weakness, wasting, pes cavus

221
Q

What is difference between spasticity and rigidity?

A

Spasticity: pyramidal lesions, velocity dependent, clasp knife (initial increased tone then gives way)
Rigidity: extra pyramidal, velocity independent, cogwheel with super imposed tremor or lead pipe uniform (NMS)

222
Q

What are differentials for mixed upper and lower motor neurone signs? (3)

A

MMD
Cervical myelopathy
Syringomyelia

223
Q

What is the sussman protocol for?

A

Initiating treatment of myasthenia gravis with pyridostigmine and steroids (start low and work up to not worsen symptoms unless critically unwell)

224
Q

What is management for GBS acutely? (4)

A

Monitor FVC - if <1.5 - dw ITU
IV immunoglobulins
Plasma exchange
Manage VTE risk

225
Q

What are key examination findings of GBS? (3)

A

LMN weakness
Mixed motor and sensory peripheral neuropathy
Not length dependent

226
Q

What is management of CIDP? (4)

A

MDT
Analgesia
Steroids /immunosuppresion
IV IG/ plasma exchange if any acute component

227
Q

What are non motor features of Parkinson’s? (6)

A

REM sleep disorder
Anosmia
Mood disorder
Pain
Reduced cognition
Constipation

228
Q

What are differences between left and right MCA infarcts?

A

Left: usually contain language centre so aphasia present
Right: usually non dominant, may have neglect

229
Q

What are signs of raised ICP in cranial nerves? (3)

A

Papilloedema
6th nerve palsy
Field defect

230
Q

What are treatments for migraine? (2)

A

Acute: NSAIDs, Triptans
Prevention: propranolol, topiramate, amitriptyline, Botox

231
Q

What are causes of choreiform movements? (7)

A

Immune: Sydenhams (rheumatic fever), SLE
Inherited: huntingtons, Wilson’s
Acute: hyperglycaemia, hyperthyroidism
vascular: stroke
Chorea gravidarum
Paraneoplastic
Drugs: levodopa, amphetamines, lithium

232
Q

What are causes of myelopathy? (9)

A

Degenerative Cervical myelopathy: spinal stenosis, disc prolapse
MS
Autoimmune: RA, SLE
Malignancy
Syringomyelia
Trauma
Spinal stroke
Infection
Subacute combined degeneration

PACES (6 decks)

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