Neurology + opthal Flashcards

Question

Optic neuritis exam? To finish?

Answer 1

Measure acuity - snellen Fundoscopy RAPD Quick test of colour vision - red in each eye Ishihara plates Visual field - finger counting in 4 quadrants (1 or 2) *State examination with slit lamp + dilating drops Complete and full systematic neuro exam | Acutley Find Rapid Red Fields

Answer 2

In a dimly lit room, have the patient look at a distant point. Shine a light into one eye for about three seconds. Quickly move the light to the other eye and observe the pupils. Repeat the steps a few times to confirm your findings. A normal response is when both pupils constrict equally when light is shined into each eye individually. When the light is moved between the eyes, the pupils should not dilate or escape. If the pupil dilates when the light moves from one eye to the other, a RAPD is present. This indicates that there is a difference in the afferent pathway between the two eyes. [Supported if the good pupil constricts when light goes back to it]

Answer 3

Neuropathy - swollen Atrophy - Pale

Answer 4

Vitamin D ESR and CRP - giant cell Consider aquaporin 4 and MOG antibodies - atypical eg neuromyelitis optica Imaging Orbital MRI - longer section of signal uptate in optic nerve suggestive of atypical [If compressive MRI brain] Sampling Consider LP for oligoclonal bands If history of sarcoid -Consider serum ACE - CT CAP Referral to neuroophthalmologist

Answer 5

Typical - consider steroids -> Plasmapheresis

Answer 6

Gets worse again

Answer 7

about 50% in 15 years

Answer 8

What was going on Motor / sesnsory - How fast, how long - Should be sudden onset but may wear off gradually Speech - Hard to get words out - Difficulty understanding words - dysphasia Vision - Transient amarousis fugax - Loss of visual field Mimics - Headache - GCA / Migrane / Venous sinus thrombosis / meningitis - Seizure like activity / tongue biting / post ictal PMH -Diabetes - blood sugar around event - AF / Palps - Vascular - cardiac / Hypetension / PVD / T2DM / IHD / TIA / stroke Drug history AF anticoagulated? -> Compliance Social - Smokling - Alcohol -> AF - Function at home / work - Drive? Family history - Clotting issues - heart attack / stroke at young age

Answer 9

Stroke / tia -> Loss of function If flashing lights / floaters / tingling etc more likely something else

Answer 10

Score 1-3 (low) 2 day risk = 1.0% 7 day risk = 1.2% Score 4-5 (moderate) 2 day risk = 4.1% 7 day risk = 5.9% Score 6–7 (high) 2 day risk = 8.1% 7 day risk = 11.7%

Answer 11

Basically a cardio exam + extras - examine what ever was the presenting complaint Hands - Pronator drift - Tar staining - Feel pulse ?AF - BP Head and neck -Auscultate carotid bruis - Xantholasma - eyes Dsarthria - say baby hippopotamus Dysphasia - name this (pen / phone) CNs - especially if facial / visual fields Visual acuity Tone / power / Sensation / reflexes Fundoscopy

Answer 12

Migraines - Usually spreading and gradual onset - Headaches - Flashing lights - Pins and needles Seizures - Likely to have amnesia Consider optic neuritis if visual only

Answer 13

Speech disturbance = right sided weakness \UNLESS left handed * All right handed people have speech centre on L side ie if there is speech disturbance and L sided weakness ?migrane / L handed

Answer 14

Bloods - FBC, U&Es -Cholesterol -HBA1C -Glucose ECG +/- 24hr tape depending if not found AF Imaging - CTB - Likely to get an MRI at TIA clinic - Carotid doppler

Answer 15

Thrombophilia screen Vasculitis screen Echo - valvular pathology PFO - will need bubble / TOE

Answer 16

Carotid stenosis > 50% and symptoms related to that side refer to vascular surgery

Answer 17

Vertebral artery dissection -> CT angio

Answer 18

Low salt Exercise Smoking / alcohol Healthy diet Control BP / Diabetes

Answer 19

Aspirin 300mg for 2 weeks then 75mg clopidogrel life long Hypertensive managment Statins if cholesterol high AF

Answer 20

1 month - I would check DVLA before to check HGV - at least 1 year

Answer 21

Diabetes Alcohol B12 deficiency [you have another card with lots more on - but always name these 3 first]

Answer 22

Antibodies to post synaptic Acetyl coline receptors (Not all signals get down) - in first stage can increase ACH in receptors to treat Over time get thickening of receptors and complement deposition - harder to treat which would lead to a fixed weakness

Answer 23

Head droop Ptosis Salivation In paces most will have stable occular myasthenia

Answer 24

[Myasthenia vignette] Smoking, thyroid, breathing, swallowing HPC -When is weakness - Fatigability - Ptosis / double vision that's worse after reading etc - Double vision that is variable - sometimes next to each other / sometimes on top - struggling to finish chewing meal - Proximal muscle weakness Lambert eaton syndrome Cough / weight loss / haemoptysis / dry mouth / sweating / palps **Screen red flags** - ANY DYSPNEA - ANY DYSPHAGIA / CHOKING **PMH** - Coexisting autoimmune - Thyroid - Lung Ca Family history Congenital myasthenia syndrome DH - Previous immune checkpoint - inhibitors for Ca **Social history** Smoking alcohol Driving - diplopia ptosis How affecting function

Answer 25

Black afrocaribbean Anti muscle specific kinase antibodies

Answer 26

Exacerbation of underlying myasthenia which leads to respiratory/ Bulbar compromise

Answer 27

FVC <20mls/kg Progressive decline in FVC Can't complete sentences Struggling with secretions Can't lift head from pillow

Answer 28

Count as far as possible in 1 breath

Answer 29

Surgery Infection - Anaesthetics - non depolarising - Antibiotics - Including surgery antibiotics (penicilliamine rarther than penicillin) Aminoglycosides, macrolides, quinilones - antimalarials - B blockers - Magnesium Non depolarising anesthetics Penicilliamine, Gentamycin / tobramycin Macrolide, Quinolones Quinine EXACERBATE Erythromycin - macrolides Xylocaine/ lignocaine Aminoglycosides Ciprofloxacin - quinolones Electrolytes - magnesium Relaxant - none depolarising Beta blocker and Botox Antimalarial - Quinine Timolol

Answer 30

Rarely get occular / bulbar symptoms Usually more Peripheral muscles Autonomic features predominately Postural hypotension Sweating Dry mouth Smoking haemoptysis weight loss

Answer 31

Rapidly progressive descending Paralysis with diplopia Also lots of autonomic symptoms

Answer 32

Middle age man Hip / knee and wrist/finger flexion weakness

Answer 33

Middle age man Hip / knee and wrist/finger flexion weakness

Answer 34

Lambert eaton Polymyositis / Inclusion body myositis Muscular dystrophies Drug induced MND **Hypothyroidism Cushing** Usually in all these the weakness is progressive rather than variable

Answer 35

Pre synaptic calcium channels -> if you repeatedly stimulate eg a reflex then it slowly releases enough and might potentiate [As the issue is pre-synaptic release essentially]

Answer 36

Down and out Ptosis Myosis

Answer 37

Down and out Ptosis Myosis

Answer 38

End of bed Fvc machine Ptosis / strabismus Myasthenia snarl when smile Thymectomy scar Cranial nerves In addition - make maintain update for 30 seconds see if fatigue Can they stop you opening eyelids Smile for snarl Open lips against them holding shut Ask them to count as far as possible in 1 breath WHILE looking up for 30 seconds Ask for a cough If ptosis -"i would like to do icepack test to see if it reverses ptosis" FVC Neck CVC (plasmapheresis) Trache scar Neck flexion / extension Precus for thymoma. (Found in 10%) Ask to hold arms out for 20 second Check lower limbs reflexes and see if vet worse / better with repeat Hope flexion I would like perform the Ice pack test to assess for fatiguabke ptosis Check **forced vital capacity** Formally asess power and fatiguability with **dynometer** (grip test) Request **SLT** assessment Request optometry assessment

Answer 39

Bedside FVC Swallow Bloods - Test ACH antibodies and MUSK If LES - voltage gated calcium channel abs - Thyroid function and antibodies autoimmune thyroid in about 10% and differential of opthalmoplegia - CK - to help differentiate myotonic dystrophies - ESR - to help differentiate imflammatory eg polymyositis EMG - repetitive stimulaton And for jitter (Jitter involves testing 2 myo fibers at same time and there is slight delay between them) CT for thymoma / hyperplasia [Tension test - old not done]

Answer 40

**Acute** If ICU - High dose steroids - Avoid pyridostigmine as increases secretions - Plasma exchange or IVIG as rescue therapy **Conservative** - Neuro referral - Patient alert card - Refer to MDT inc SLT / optometry / Physio / OT **Medical** - I would treat this patient according to the **Sussman protocol** - Pyridostigmine - Consider buscopan for the GI side effects - Low dose oral steroids -> If they relapsed despite steroids I would consider **azathioprine** or **mycophenolate** **Surgical** If under 45 and positive antibodies should be offered a thymectomy

Answer 41

Parkinsonism is the signs Rigitiy (increased tone) Tremor Bradykinesia Parkinsons is most common differential of this.

Answer 42

Young tremor predominant slow progressive Older Bradykinesia predominant and usually quicker progressive

Answer 43

Degenerative neurological disorder characterised by loss of dopaminergic neurons in the basal ganglia (particualry the substantia nigra) with lewy body deposition

Answer 44

Surrounding - mobility aids Medication - dopa Tremor at rest Hypomimic face Quiet voice when say hello Tremor - Resting and rotational approx 4-6Hz. Make sure relax arm - Usually asymmetrical Tone - Assess all - Usually increased tone especially on 1 side - Cog wheel at wrist rotational Coordination - Bradykinesia - test finger and thumb tapping - Lower limb - ask to stamp feet on floor Eye movements - Upwards gaze palsy Speech - Speed and amplitude saying Baby hippoptamus Writing - micrographia Functional - Doing up buttons "I suspect this is parkinsonism and would like to performa more focused exam - however i can assess" Power / reflexes / sensation usually normal Gait - Reduced arm swing - Shuffling I would like to formally assess for - anosmia (common in parkinsons) - perform a cognitive assessment of memory as decline common in parkinsons as well as a cross over lewy body dementia) - check lying standing blood pressures - MSA / side effects of mediations

Answer 45

People who are cognitively intact and symptoms despite 5x/day dopa therapy Especially on/off symptopms Seen similar site to PPM

Answer 46

Deep brain stimulation Apomorphine infusion sub cut Duodopa - gel preparation of levodopa into jejenum -.. gives continuous therapy

Answer 47

Reduced dopamine in brain -Idiopathic parkinsons (slow progressive usually asymmetric) - mnon scary hallucinations Parkinsons plus = All respond poorly to levodopa - Lew body dementia - forgetfull / early onset cognitive inpairment and scary hallucinations - Progressive supranuclear palsy. Symetrical with tremor and falls predominant. Reduced vertical gaze. Rapid progression - Multi system atrophy - autonomic / cerebellar features predominate - Corticobasilar degeneration - usually rapid with 'alien limb' - loss of use with uncontrolled movements Parkinsonism with NORMAL dopamine levels - Vascular parkinsonism - essentially vascular disease affecting basal ganglia - Drug induced eg metoclopramide / antipsychotics, valproate - Essential tremor - Wilsons - Normal pressure hydrocephalus - tumours in basal ganglia

Answer 48

Conservative - Full history / medication history - L/S BPs - Cognitive assessment - Eg MOCA - Non motor symptom questionnaire Bloods minimal - Consider wilsons Imagining - Strokes - Vascular load in basal ganglia - MRI best - Consider DaTSCAN

Answer 49

Can help differentiate parkinsons from Eg essential tremor - Looks at uptake of dopamine - Only helpful to differentiate parkinsonism with dopamine depletion and with normal dopamine levels

Answer 50

Conservative - Patient education - direct to parkinsons UK - Specialist CNS nurse - Early physio - Speech therapy - As progress may need dieticians Medication - 3 options for first line - Levodopa with peripheral decarboxylase inhibitor (dopamine doesn't cross blood brain barrier, levodopa does) - eg co-careldopa - MAOi - selegiline/rasagiline (can hold off need for dopamine for up to 1 year) - Dopamine agonist - Eg Pramipexole (Mirapex) Rotigotine (Neupro) Ropinirole (Requip) Apomorphine (Apokyn) -> can add together -> COMT inhibitor eg entacapone (reduces breakdown of L dopa) Surgical - Deep brain stimulation

Answer 51

Impulse control issues - Very important to council (+ document) prior to starting

Answer 52

Rotigotine | Ro-stick-it-in

Answer 53

**Non-motor symptom questionnaire** Neuropsych - Mood disorders - REM sleep disorder Anosmia - loss of sense of smell Sleepy Daytime hypersomnolence Autonomic - Urinary retention / over active bladder - Postural hypotension's - Constipation ## Footnote NASA

Answer 54

hoehn yahr

Answer 55

What do you mean by visual loss 1 eye or both Unilateral - cataract / macular degeneration Bilateral - post chiasm / pit / infarcts Pain - Ulcer on cornea, angle closure glaucoma, GCA (pain in temples then painless vision loss) Painless - Macular degeneration / cataracts / Struggle with distance or reading Bumping into things in peripheries PMH - Diabetes - Hypertension Family history - Social history - Smoking very bad - Function - job / drive

Answer 56

Retinal detachment

Answer 57

haemorrhage

Answer 58

People who are far sighted Thier eyes look big with glassess on

Answer 59

usually 1 eye first with blurring / distortion of vision

Answer 60

steroids UV Myotonic dystophy, downs, turners

Answer 61

- Turn off the lights and darken the room - Use an ophthalmoscope with the lens power set to zero and light to max - Hold the ophthalmoscope about 18 inches from the patient's eyes - Shine the light into each eye individually, then both eyes together - Observe the color, brightness, and symmetry of the red reflex What to look for A normal red reflex is symmetrical, bright, and the same color in both eyes An abnormal red reflex may include: -Dark spots or black flecks -An absence of the red reflex -A white reflex (leukocoria) -An asymmetric reflex

Answer 62

Shine light into 1 eye for 2 seconds Both should constrict the same Then switch and both should constrict the same In abnormal eg glaucoma / vein / artery occlusion where partially damaged retina -When shine light into damaged eye only constricts by half. -When light shined into good eye both will constrict completely -> when light switched back to eye with glaucoma it will dilate slightly

Answer 63

Physiological blind spot - corresponding to optic nerve

Answer 64

Superior / inferior arcuate defect The reason these look like arcs and come off the blind spot is that they represent the loss of bundles of nerves as they come out of the optic nerve head. The horizontal border is the horizontal raphe, which is an imaginary line dividing the upper and lower hemispheres of the retina.

Answer 65

Severe constriction with a central island end stage glaucoma.

Answer 66

Raised in glaucoma. >0.7

Answer 67

Magnified - hypermytrophic -> glaucoma Small - myopic -> retinal detachment

Answer 68

Older person with slow progressive loss both distance and reading **Central visual loss** with distortion being a common feature

Answer 69

VEGF inhibitors Eg Sunitinib

Answer 70

Young age with progressive peripheral visual field loss -> bumping into things Much worse at night

Answer 71

Pigmented bone spicules Waxy appearance of disc Arteriola attenuation

Answer 72

Focal seizure is abnormally discharging neurons in a localised area - Either consciousness non-impaired = focal aware seizure - Or consciousness impaired = focal non-aware sezirue -Occipital - flashing blobs of light -Parietal - spreading paraesthesia / pain -Frontal - hyperkinetic high energy motor movements -Temporal - epigastic rising sensation . dejavu, emotion eg fear/panic, olfactory

Answer 73

Occiptal - Short lasting seconds- blobs Migrane - Longer lasting minutes - jagged lines

Answer 74

Can use any of the drugs for generalised seizures. Lamotrigine, carbamazepaine, keppra Valproate usally LESS effective

Answer 75

Inheritance variable based on cause 15% Usher syndrome - recessive [Shhusher - cant hear] - Varying severities of a sensory neural hearing loss - (often born with moderate-severe hearing loss) Bardet-biedel syndrome - Obese / polydactyly - Renal disease [Big Boys] Kearns-sayres - Mitochrondrial (maternal) - Progressibe and complex opthalmoplegia - Cardiac conduction defects - PPM - Ataxia Resfem's disease = recessive [need to Re(f)st as weak and ataxic] - Ataxia - Deafness - Weakness / Polyneuropathy Management -> stop eating foods with phytamic Couple of vitamin deficiencies A and E - Consider if

Answer 76

Can they detect movement - eg moving hand in front of face If not -> check for ability to detect light with pen torch

Answer 77

Hands - polydactyly / scars of fingers removed -> Bardet-biedel Pacemaker - Kearns-sayres Hearing aids - Usher, refsems, Kearns-sayres Acuity Eye movements - consider Kearns-sayres if complex opthalmoplegia Fundoscopy

Answer 78

Referral to opthalmologist Full field electroretinography - Response of retina to light - would be reduced Formal humphrey visual field testing Ischihara plates Retinal photography - better than fundoscopy If suspicion of genetic syndrome -> geneticist If deafness -> audiology assessment

Answer 79

OT hep with visual aids ENT - screening for hearing loss Genetic counciling if specific diagnosis Patient education - explaining no cure often Discuss job / driving status Phsycological support

Answer 80

Clot or blood in the brain

Answer 81

Inspection - no matter if asked to examine CN / Upper / Lower - May have splints - used for preventing fix flexed deformities - Walking aids - Scars on head eg hemicraniectomy - PEG tube Sneaky tip - When introduce self during inspection come from left and offer to shake hands [Assesses left side neglect and also speech - L weakness (R brain) - assoc neglect - R weakness (L brain) - aphasia] Inspect May have flaccid or flexed deformity Tone - Typically flexed high tone - As examine more it will soften - Important to differentiate high tone / contractures Power - Asymmetric pattern of pyramidal weakness Reflexes - consider reinforcement eg gritting teeth / clenching hands Clonus Plantar Sensation - Light touch - Pin prick - Vibration Coordination - Heel shin - dysdiadochokinesia Cortical symptoms - Test inattention -If they have RIGHT sided weakness examine SPEECH - If they have LEFT sided weakness examine NEGLECT [ Visual fields then wiggle both fingers, Sensation L then R then Both] To complete I would examine - Proprioception, temperature A full CN examination paying particular interest in visual field defects such as homonymous hemianopia and a full cortical assessment looking for neglect.

Answer 82

anterior cerebral arteries supply the anteromedial area of the cerebrum. - ACA -> mainly leg The middle cerebral arteries supply the majority of the lateral cerebrum. - MCA -> mainly arm and face The posterior cerebral arteries supply a mixture of the medial and lateral areas of the posterior cerebrum.

Answer 83

Bamford Essentially TACS and PACS involve cortical signs - aphasia if affecting R weakness (L side brain) - neglect if involving L weakness / R brain side. **Total anterior circulation stroke (TACS)** - Usually MCA -> mainly arm and face - ACA -> mainly leg All three of the following need to be present -Unilateral weakness (and/or sensory deficit) of the face, arm and leg -Homonymous hemianopia -Higher cerebral dysfunction (dysphasia, visuospatial disorder) **Partial anterior circulation stroke (PACS)** - a less severe form of TACS, in which only part of the anterior circulation has been compromised. Two of the following need to be present for a diagnosis of a PACS: -Unilateral weakness (and/or sensory deficit) of the face, arm and leg -Homonymous hemianopia -Higher cerebral dysfunction (dysphasia, visuospatial disorder)* -Higher cerebral dysfunction alone is also classified as PACS. **Posterior circulation syndrome (POCS)** involves damage to the area of the brain supplied by the posterior circulation (e.g. cerebellum and brainstem). One of the following need to be present for a diagnosis of a 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 **A lacunar stroke (LACS) ** is a subcortical stroke that occurs secondary to small vessel disease. There is no loss of higher cerebral functions (e.g. dysphasia). -> Weakness usually affects Face arm and Leg as is a more distal vessel One of the following needs to be present for a diagnosis of a LACS: -Pure sensory stroke -Pure motor stroke -Sensori-motor stroke -Ataxic hemiparesis

Answer 84

1 - Lacuna stroke 2 - it is a CN cause Ie. nothing to do with the spine

Answer 85

L weakness - neglect R weakness - Speech [The Right sided Speech Left them with Neglect]

Answer 86

Lateral medullary syndrome - Loss of pain and temperature sensation on one side of the face Loss of pain and temperature sensation on the opposite side of the body

Answer 87

High tone you can overcome Contractures - cannot either too painful or just unable - Definitely means chronic

Answer 88

Flexor posturing in upper limb Extensor posturing in lower limb

Answer 89

0 - no movement 1 - flicker 2 - movement without gravity (eg turn arm so thumb facing up to test wrist extension) 3 - Move against gravity 4 - reduced power 5 - Against resistance

Answer 90

Brain if face Brain if unilateral (usually)

Answer 91

In the Acute stroke with flaccid hemiparesis I would consider - Tods paresis - Migraine However with Chronic stroke - Upper motor neurone signs i would consider - SOL - MS

Answer 92

In the acute setting the investigations would be different from the patient who has more chronic symptoms in front of me Acute - Stabilisation with a focused ABCDE - NIHSS assessment - Organise urgent CT / CTA and call the thrombolysis / thrombectomy team Otherwise **Bedside** - Glucose - ECG **Bloods** FBC U+Es HBA1C Cholesterol and lipids **Imaging** CT + CTA if within thrombolysis window **Special tests later down line** - Carotid dopplers (if you didn't already have the CTA in the acute setting) - Consider 24hr tape - Echo in young patient / embolic stroke

Answer 93

Check if eligible for thrombolysis (4.5hr) or thrombectomy (24hr) Ensure medically stable and perform NIHSS score then discuss with stroke team Ischemic stroke - Asprin 300mg for 2 weeks then clopidogrel - Or anticoag if due to AF Haemorrhagic Manage hypertension to target systolic 140-150 Swallow assessment and consider NG **Chronic** Full assessment from nutrition, physio and OT Cognition assessment with MOCA VTE prophylaxis with intermittent pneumatic compression. [NOT TEDS or enoxaparin] Longer term Manage cardiovascular risk factors - Diabetes, HTN, cholesterol - Management of high tone eg splints / botox - Follow up ECGs and carotid dopplers - Job / driving / visual fields

Answer 94

Unusual thrombosis - Antiphospholipid screen or acquired thrombophilia Vasculitis - Screen ANCA / ANA Bubble echo at 5 weeks MRI

Answer 95

Brain - Swelling eg malignant MCA syndrome - Haemorrhagic transformation - Repeat infarcts - Seizures Bleeds - Hydrocephalus Stroke - Swallowing / aspiration - Incontinence - Spasticity - Pain PEs, DVTs as unable to use clexane Falls Delirium

Answer 96

Inspection Wasting and fasiculation Tone - Usually spastic but may be flaccid Power - Weak, often footdrop is presentation Reflexes - variable Think absent knee jerk with extensor plantar **Sensory - NORMAL** Speech - dysarthria - Bulbar (donald duck) - palate weakness - Pseudo bulbar (hot potato) - spastic tongue Tongue - Wasting and fasiculation - bulbar - Stiff spastic with brisk jaw jerk - pseudobulbar NO sensory, Occular or cerebellar

Answer 97

Progressive disease of unknown aetiology which involves axonal degeneration of the upper and lower motor neurones 3 Ps Painless, progressive paralysis

Answer 98

Often overlap between them Amyotrophic lateral sclerosis (50%) - corticospinal tracts - Spastic paraparesis Progressive muscular atrophy 25% - anterior horn cells - Wasting fasiculation and weakness - BEST prognosis Progressive bulbar palsy 25% - Lower CNs and suprabulbar nuclei -> speech and swallowing issues - WORST prognosis

Answer 99

Clinical diagnosis Bloods Consider b12 / CK Consider genetic testing SOD1 [Poor sod] EMG - motor neurone disfuction you struggle to detect clincially eg fasiculations - Denervation and renervation seen - exclude myaesthenia etc - look at muscle disease MRI brain and spine - Help exclude brain stem lesions, cervical cord compression

Answer 100

Supportive Patient education and support groups as likely progressive PEG feeding / Dietician PT mobility aids Communication aids OT NIV Advance care planning Riluzole - glutamate antagonist

Answer 101

Die by 3 years -> pneumonia / resp failure Poor predictors - Onset in elderly - Female - Bulbar involvement

Answer 102

Anterior horn cell - MND - Syringomyelia - Cervical cord comrpession - Polio Brachial plexus - Cervical rib - Pancoast tumour - trauma Peripheral nerve - peripheral neuropathy Muscle - Disuse atrophy eg rheumatoid

Answer 103

Axonal loss -> remaining axons recruiting and innervating more myofibrils -> large motor units MND Syringomyella

Answer 104

Lower motor neuron disease X linked -> men Tremor predominant gynaecomastia perioral fasiculations symmetrical

Answer 105

Dystrophy has - Myotonia - No upper motoneurone signs - very symmetrical - Rarely significant wasting

Answer 106

Dysarthria - only in MG crisis Ptosis and fatiguability of eye movements

Answer 107

Myotonic dystrophy Myaesthenia - if bulbar Heredetary spastic paraperesis Syringomyela Inclusion body myosisitis - foot drop

Answer 108

The macula is found lateral (temporal) to the optic nerve head. The central part of the macula, the “fovea” is about the same diameter as the optic disc and appears darker than the rest of the macula due to the presence of an additional pigment.

Answer 109

Microaneurysms are localised outpouchings of capillaries Look very similar to dot and blot haemorrhages - bleeding capiliaries

Answer 110

Cotton wool spots

Answer 111

Insufficient retinal perfusion results in the production of vascular endothelial growth factor -> neovascularisation

Answer 112

Lower motorneurone palsy of CN7 Unilateral facial weakness Inability to close eye Hyperacusis Pain behind ear Loss of taste

Answer 113

Bells - onset over hours to days Stroke - sudden onset - Forehead spairing

Answer 114

Forhead gets innervation from both hemispheres but only ipsilateral facial nerve

Answer 115

close eyes agaisnt resistance Elevate eyebrows Show teeth Open mouth against resistnace Formal taste testing

Answer 116

Bilateral - Lime disease - HIV seroconversion - Gillian barre Additional CN involvement - CN8 in cerebellar pontine angle tumours Systemic features - Bullseye rash - lime Sudden onset - Vascular Very slow onset over weeks - ?SOL

Answer 117

Ramsay hunt - Herpes Stroke Lime disease Otitis media Acoustic neuroma Gillian barre - common to invovle CN7 and bilat HIV Autoimmune - Sjogrens - Sarcoid - MS

Answer 118

If typical symptoms can diagnose clinically. If suspicious / not resolving after 6 weeks then consider HIV testing Lyme serology MRI for MS LP if thought to be inflammatory

Answer 119

50mg Pred 10 days Eye protection - lubricating drops [Both due to less lacrimal secretion (reduced parasympathetic) and inability to close eye Aciclovir if evidence of ramsay hunt If prolonged / incomplete recovery can refer to neuro

Answer 120

Dementia - Alz - progressive short term memory loss - Vascular - assoc stroke / vascular disease, mood is big feature - Lewy body - periods reduced consciousness, parkinsonism, vivid dreams - Parkinson - usually parkinsonism before dementia - Fronto temporal- personality / agression / language NPH - gait, incontinence, memory loss SOL - personality change Depression Hypothyroid Alcohol related brain injury Red flags - Left gas on / left house open - got lost and brought back by some body - Feel safe in the car - Loss of inhibition / agression Screen systems - Constipation - Continence - Gait PMH - Cardiovascular risk factors - Previous psych history DH - Do they self manage - dossett box - Antichoninergic - Anti psych / oxybutinin / amitrip Family history - Young memory problems Social - How is affecting - Who is supporting - Driving

Answer 121

Short term - words dates facts Semantic - faces Prospective - furutre eg appointments Working - get to the top of stairs and forget / driving and excecutive Episodic - eg in alz no memory of last 20 years but

Answer 122

Brief systemetic exam Power and coordination in all limbs Ataxia Full set of obs Cognitive screen Eg AMT, MMSE, MOCA

Answer 123

Screening 4AT Tool MMSE MOCA Addenbrookes ACE 3

Answer 124

Formal cognitive assessment Bloods - CAUIT Brain Health Calcium Alcohol / LFTs Uraemia Inflam markers TFTs b12 / folate HIV / sysphilis if any suspicion If young - Autoimmune encephalitis as well Imaging - CTB - rule out SOL, stroke, vessle disease - Likely MRI if need more info

Answer 125

Educate - This is likely to be a slow but progressive condition - Can discuss more later MDT Signposting to info like dementia services Social services - Care / rest bite OT - gas, car Physio Medication - Alz - Ach inhibitors eg donepazil, rivastigmine (improve by less than 1 point on MMSE) - Memantimine - behavioural symptions - Rivastigmine.- reduces hallucinations in lewy body

Answer 126

Consider theraputic LP -> vp shunting if requires regular

Answer 127

**Lower limbs** - Gait - ataxia - Rhombergs negative - Heel-shin If rhombergs positive move to **peripheral neuropathy** exam included - ideally complete this at end **Head** - Brief conversation - Dysarthria - Nystagmus - May have minor head tremor **Upper limbs** - Rebound phenomenon - Finger nose incoordination - Disdiadokinesis - [Hypotonia / Hyporeflexia ] **Pinprick** - peripheral neuropathy (also small fibres which will be lost first ## Footnote DANISH disdiadokinesis Ataxia Nystagmus Intention tremor Scanning dysarthria Hypotonia / Hyporeflexia

Answer 128

Lobe -IPSILATERAL cerebellar signs in limbs Vermis - Ataxic trunk / gait with normal limbs tested on bed Vestibular nucleus - Nystamus from Contralateral side [L nystagmus could be due to a L (ipsilateral) cerebellar lesion or a R contralateral vestibular nucleus lesion]

Answer 129

My HAND VP - MS - Hereditary - Spinocerebella ataxia (esp brisk reflexes), Frederick's / Ataxia telangectasia - Alcohol - Nutritional b1/12 - Drugs - phenytoin - Vascular - stroke - Paraneoplastic / Posterior fossa lesion PASTRIES Phenytoin / Paraneoplastic cerebellar syndrome Alcoholic (thiamine deficiency) cerebellar degeneration Sclerosis - MS Tumor - posterior fossa Rare - friedricks ataxia Infection - viral Endocrone - hypothyroid Stroke - brainstem

Answer 130

Chronic inflammatory demyelinating disorder Relapsing-remitting (85%) Approximately half go on to develop secondary progressive MS Primary progressive or progressive-relapsing (15%)

Answer 131

- Red desaturation - Relative afferent pupillary defect in affected eye Extra-ocular movements - Internuclear ophthalmoplegia – failure of adduction of affected eye, with nystagmus on abduction of the contralateral eye - Document preservation of convergence - Nystagmus Offer fundoscopy to look for pallor of the affected disc May be normal as disease is retrobulbar in the majority of patients

Answer 132

Infective - Lime disease - Tertiary syphilis - HIV - HTLV-1 -> spastic paraperisis Inflamatory - Vasculitis - Acute disseminated encephalomyelitis (following viral illness / vaccination) - Neuromyelitis optica - Transverse myelitis - Systemic inflammatory (SLE/ Sarcoid / sjogren Metabolic - B12 deficiency - Diabetic neuopathy - Hypothyroid Lysosomal storage disease - Eg Fabry Mitochondrial diseases - Myoclonic epilepsy with lactic acidosis and stroke-like episodes (MELAS) Myoclonic epilepsy with ragged red fibres (MERRAF) Heredatory spastic paraperisis

Answer 133

Full blood count (anaemia of chronic disease) Liver function, renal function prior to starting immunosuppression CSF - oligoclonal bands (>80% of patients with MS, but not specific) MRI brain and spine Visual evoked potentials looking for conduction delay (prev optic neuritis)

Answer 134

**Acute attacks** - high dose IV steroids (consider plasma exchange) **Conservative** Patient education + support groups Multidisciplinary team approach Occupational and physiotherapy to preserve and maximize function Walking aids / visual aids as necessary **Treatment of symptoms** - Spasticity: baclofen, gabapentin, tizanidine, dantrolene - Depression: selective serotonin re-uptake inhibitors, tricyclic antidepressants - Fatigue: amantadine - Neuropathic pain: TCAs, pregabalin, gabapentin, carbamazepine - Bladder dysfunction: intermittent self-catheterization, α1-antagoinists, anti-cholinergic agents **Chornic Disease modifying** - Specialist input in rapidly evolving field - Interferon b - Natalizumab - Fingolimod

Answer 135

Reduced relapse rate -> increased post partum

Answer 136

Gerstmann syndrome is a rare neurological disorder that affects the brain's parietal lobe, causing a loss of certain cognitive functions: **Writing disability** Also known as agraphia or dysgraphia, this can include illegible writing, inconsistent letter formation, and unfinished letters **Inability to do math** Also known as acalculia or dyscalculia, this can include difficulty with adding, subtracting, multiplying, and dividing **Inability to identify fingers** Also known as finger agnosia, this can include difficulty identifying one's own or another's fingers Inability to distinguish right from left

Answer 137

Lateral medullary syndrome (wallenberg) - Occlusion of PICA Contralateral - pain and temperature in limbs Ipsilateral - pain and temp face (trigeminal) - Cerebella signs - Nystagmus - vestibular nucleus - horners - descending sympathetic tract - palatal paralysis / loss of gag - - nucleus ambigus

Answer 138

MS Cord compression / cervical myelopathy Trauma MND (if no sensory) Rare - Anterior spinal artery thrombus - sryngomyellia - heredatory spastic paraplegia - Subacute degeneration of spinal cord - freidricks ataxia

Answer 139

L1-2 - Hip flexion L3 - Knee extension L4 - ankle dorsiflexion L5 - big toe extension

Answer 140

Inspection Scars from painless burns Weakness and wasting of the small muscles of the hand Reduced reflexes Sensory loss - Loss of pain and temperature (spinothalamic) - Presevation of joint position and vibration sense (dorsal columns)

Answer 141

Expanding fluid filled cavity within the cervical cord. Compresses spinothalamic neurones first Assoc - arnold chiari malformation Diagnostic mri spine

Answer 142

C5/6 - Biceps - Elbow flexion and suppination C7/8 Triceps - elbow extension T1 - finger abduction

Answer 143

c5 shoulder c6 thumb / lateral forearm c7 middle finger c8 little finger t1 medial forearm

Answer 144

Multisysem atrophy - Hypotension - Cerebellar signs Progressive supranuclear palsy - Vertical eye movements Forgetful - Lewy body dementia Medications

Answer 145

Hereditary sensory motor neuropathy - Pes Cavus (also seen in freidricks ataxia) - Wasting of distal lower limb muscles with preservation of thigh bulk - Wasting of hand muscles - Weakness of ankle dorsiflexion and toe extension - Variable degree stocking sensory loss - High stepping gait (footdrop) and stamping (absent proprioception)

Answer 146

type 1 - demyelinating Autosomal dominant - PMP22 mutation Type 2 - axonal

Answer 147

- Diabetes - Alcohol - Drugs - isoniazid / vincristine - Vit B12 / B1 - Chronic inflammatory demyelinating polyradiculoneuropathy Predominant motor - Guillian barre - Botulism - lead toxicity - Charcot marie tooth Mononeuritis multiplex - Diabetes - Connective tissue - Rheum / SLE - Vasculitis - PAN / eosinophilic granulomatosis - HIV - Malignancy ## Footnote Acute - Guillian barre - Lead toxicity - Diptheria - Porphyria Subacute - Drugs. - vincristine / isoniazid - Toxins - alcohol - Dietary - B12 /B1 Chronic - Diabetes - heredetary eg charcot marie tooth - Connective tissue - Eg rheum A / SLE - Medabolic eg uraemia - Paraproteinaemia - Malignancy

Answer 148

End of bed - Young / wheelchair (or mobility aids) with scoliosis Dysarthria when introduce self - Bilateral cerebellar ataxia -> Ataxiaic hand shake, dysarthria nystagmuis Lower limb - Leg wasting with absent reflexes and bilateral upgoing plantars - Often mild increase in tone - Pes cavus - high arch that doesn't flatten when weight is applied - Loss of vibration and proprioception sense - dorsal columns (may also have some pinprick / soft loss) - Gross bilateral Ataxia If time - Assess speach / nystagmus - Kyphoscoliosis - Upper limb finger nose / disdiadokinesis I would like to assess - Fundoscopy for optic atrophy - Finger prick marks / glucose for diabetes - high arched palate - Full cardiovascular exam HCOM SN hearing loss

Answer 149

Progressive neurodegenerative condition (most common inherited ataxic condition in uk) Frataxin gene on chromosome 9 Autosomal resessive [Trinucleotide repeat with anticipation] -> onset as a teen -> Iron accumulation in mitochondria of; - **cerebellum** -> central ataxia - **dorsal collumns** -> sensory ataxia and loss of tendon reflexes [like b12 deficiency] - **cardiac** myosites -> HCOM (most common cause of death) - **Pancreatic b cells** -> diabetes ## Footnote - - scoliosis - high arched palate - Dysphagia - CN VII, X and XII - Sensory neural deafness - optic atrophy - Pes cavus - Mild dementia

Answer 150

- Friedricks ataxia - subacute degenration of the cord - b12 def / NO2 use - MND - Conus medullaris lesions - Dual patology - Peripheral neuropathy (absent ankle jerk) + stroke on affected side - Tertiary syphilis - Taboparesis

Answer 151

Congenital Senile Myaesthnia myotonic dystrophy Mitochondrial eg kearns-sayre syndrome Bilateral horners Bilateral 3rd nerve palsy

Answer 152

Tuberous sclerosis End of bed / Face - Butterfly distrobution of adenoma sebaceum - Antiepileptic treatment eg gum hyperplasia in phenytoin Hands - Periungal fibromas Trunk - Shagreen patch - rough leathery skin over lumbar region - Ash leaf macules Abdo - Renal enlargement - PKD - Renal transplant / dialysis

Answer 153

Retinal phakoma - dense white spots

Answer 154

Autosomal dominant TSC1 - chrom 9 TSC2 - chrom 16 (PKD 16 too)

Answer 155

Low IQ Seizures Renal manifestations eg PKD / angiomas Hands - Periungal fibromas Face - Adeoma sebaceum - Retinal hamartomas - big white patch Trunk - Shagreen patch - rough leathery skin over lumbar region - Ash leaf macules

Answer 156

Polycystic kidneys RCC angiomyolipomas

Answer 157

Neurofibromatosis Skin - more than 2 cutaneous neurofibromas - Six or more cafe-au lait patches - Axillary frekling Eyes - Lisch nodules - reduced acuity - optic glioma / compression Ears - Hearing loss / tinitus bilateral in type 2 Lowe limbs - Tone sensation power Chest - Lung fibrosis Neuropathy BP - Hypertension (assoc renal artery stenosis / phaeo

Answer 158

Both autosomal dominant Type 1 - chrom 17 - Increased seizures, learning difficulties and malignant peripheral nerve tumours - Large neurofibromas may be in abdo and compress kidneys Type 2 - Chrom 22 - Bilateral acoustic neuromas -> SN deafness - Tumours in spinal cord -> compression - Mostly afects brain and spinal cord -> less skin findings

Answer 159

Phaeo renal artery stenosis Epilepsy sarcomatous change scoliosis Low IQ

Answer 160

Neurofibromatosis Leprosy Amyloidosis Acromegaly Refsums disease

Answer 161

Unilateral Horners Remember to have a look for evidence of CVCs / carotid endarctectomy Pancoast tumour resection Brainstem - MS - Stroke - wallenbergs Spinal cord - Syrinx Neck - pancoast - aneurysm

Answer 162

Holmes-aide pupil Absent / diminished knee / ankle jerk

Answer 163

CN 3 palsy Medical - Mononeuritis multiplex (eg diabetes) - Midbrain infarct - Midbrain demyelination eg MS - Migraine Surgical - Communicating artey aneurysm (posterior) - Cavernous sinus pathology eg tumour / thrombosis - Cerebral uncus herniation

Answer 164

Argyll robertson - spyhilis (may be diabetes) Test for sensory ataxia (also sign of quaternary syphilis)

Answer 165

Positive RAPD

Answer 166

MS Glaucoma B12 Ischemia PALE DDISCS Pressure - Gaucoma / tumour Ataxia - freidricks LEbers optic atrophy Degenerative - retinitis pigmentosia Diet - B12 deficiency Ischemia - CRAO Sphylis / Toxo Cyanide /lead / alcohol poisoning Sclerosis - MS

Answer 167

Ataxia - Freidricks / kearns-sayre / refsum Deafness - refsum / kearn sayre / usher Opthalmoplegia + PPM - kearn sayre Polydactyly - Bardet-Biedl syndrome Ichthyosis - refsum

Answer 168

Full and familty history Urine dip fasting glucose / HBA1C for diabetes ECG / CXR / Echo evidence of HCOM Nerve conduction studies - absent / reduced seonsory action potentials with increased motor velocity MRI brain / spine - atrophic changes / rule out conus medularis or subacute degeneration of cord - Friedricks ataxia - subacute degenration of the cord - b12 def / NO2 use - MND - Conus medullaris lesions - Dual patology - Peripheral neuropathy (absent ankle jerk) + stroke on affected side - Tertiary syphilis - Taboparesis

Answer 169

MDT - Geneticist / genetic counciler - Neurologist - physio - Occupational therapist - Orthotics - Diabetic nurses - Cardiologist - GP for continuity of care No cure Management of complications such as spacicity / diabetes / arrythmias / joint deforment or scoliosis

Answer 170

CN1 (olfactory) - olfactory bulb CN2 (optic) - Retina CN3/4 - Midbrain CN 5-8 - Pons CN 9-12 - Medulla

Answer 171

Superior / inferior / medial / lateral Rectus muscles Superior / inferior obliquie Lateral rectus - CN6 (Abducens) Superior oblique - CN4 (troclear) Remainder - CN 3 (occulomotor)

Answer 172

2 eyes moving in the same direction

Answer 173

Paramedian pontine reticular formation controls horizontal eye movements

Answer 174

L paramedian pontine reticular formation which triggers : L sided abducens (CN6) R sided Occulomotor (CN3) Medial longitudinal fasiculus (heavily myelinated nerve fibres which starts at abducens and travels to occulomotor)

Answer 175

Disorder of horizontal conjugate gaze Impaired **ADduction of ipsilateral eye** With nystagmus of contralateral ABducting eye Caused with a lesion in medial longitudinal fasiculus Assess convergence to see if eye can in fact ADduct

Answer 176

Left medical longitudinal fasiculus lesion **The eye which cannot move (adduct) is the side with the lesion**

Answer 177

Complex opthalmoplegia

Answer 178

My 2 main differentials would be MS or stroke dependent on the patients age and vascular risk factors. MS - (Medial longitudinal fasiculus is highly myelinated) - If young it is likely this Stroke - Small stroke affecting pons / medulla Other - Neoplasm / mets - Inflammatory - cns vasculitis - Infections - syphilis / TB - Trauma - Metabolic - B12 / Wernikes **Differentials** - CN3 palsy, CN4 palsy etc - Mimick - Myaesthenia gravis due to fatiguability [INO+ syndromes - 1 and a half syndrome - 1 eye has complete bilateral horizonal gaze palsy and the other has failure of ADduction (when abducts have nystagmus) - Bilateral wall eyed opthalmoplegia (both eyes face out) ]

Answer 179

MS Stroke would have to involve large area of brainstem to cover both sides -> lots more signs

Answer 180

MRI head looking at brainstem - Infact / haemorrhage / demyelination If young and demyelination - MRI whole spine for cord lesions - MRI with gadalinium (enhances with new lesions) - Oligoclonal bands in CSF If old and evidence of infarct - Stroke bloods - Cholesterol / diabetes - ECG for AF +/- 24hr tape etc Infection LP etc

Answer 181

MS steroids / Disease modifying Stroke - stroke Eye patch etc

Answer 182

Normal Right occulomotor Right Troclear Right abducens

Answer 183

- Superior rectus – moves the eye up. - Inferior rectus – moves the eye down. - Medial rectus – moves the eye in. - Inferior oblique – moves the eye up when the eye is looking in toward the nose. - Levator palpebrae superioris – lifts the upper eyelid. [helps to remeber the function of the inferior oblique as lifting eyelid and eye up happen together] Conditions compromising blood flow to the inner nerve fibers controlling these muscles — without compressing the outer fibers — don’t affect the pupil. **Outer nerve fiber function** Parasympathetic - muscles: - Sphincter pupillae – located in the iris and makes the pupil smaller. - Ciliary muscle – causes the lens to change shape when focusing up close.

Answer 184

Can be pupil sparing

Answer 185

**Surgical - Complete (with pupil involvement)** - Usually a compressive cause eg PICA - This is a neurosurgical emergency until proven otherwise - Others - malignancy, trauma, haemorrhage, uncal herniation - [parasympathetic fibres which run on outside to pupil are suseptible to pressure from external structures] **Medical - Pupil sparing** - microvascular supply to neve damaged - blood flow insufficient to reach inner fibers. - Diabetes, hypertension Other causes would expect more than an isolated CN3 palsy eg infection / vasculitis / cavenous sinus syndrome

Answer 186

If pupil involvement (**surgical**) -> **Urgent CT angio** (or MRA) brain for PICA With urgent neurosurgical referral dependent on result **If Medical** **Bedside** - check BP and sugar **Bloods** - FBC , HBA1C, inflam markers, [TFTs, Ach / MuSK] **Management** - HTN and diabetes and most patients recover before 12 months - No driving / heavy machinery while diplopia - prism glasses / eye patch

Answer 187

- No Driving / work responsibilities - Prism glasses - Eye patch

Answer 188

Right CN4 palsy [Troclear inervating superior oblique] - Get them to ADduct (look to left) and then look down in this position

Answer 189

- Congenital - Microvascular ischemia - DIabetes and hypertension - Head injury - very thin nerve - doesnt require massive trauma [Stroke Inflammation Multiple sclerosis Tumor]

Answer 190

Right CN6 palsy - eye sits slightly medial at rest due to unapposed action of medial recuts

Answer 191

- Congenital - **Raised ICP**( false localising sign also in IIH) - Microvascular ischemia - DIabetes and hypertension - Head injury - very thin nerve - doesnt require massive trauma

Answer 192

Bedside - BP and sugar Bloods - FBC, HBa1c, inflam markers, cholesterol New CN4 / CN6 will get an MRI head to rule out malignancy or clear structural cause Conservative - No driving while symptoms - Eye patch / prism glasses Medical - HTN and diabetes control

Answer 193

- Thyroid eye disease - Myaesthenia - Miller fisher syndrome - CPOE - Chronic progressive external ophthalmoplegia - brainstem lesion eg compressive - mitochondrial issue - Infection classically Lyme disease if multiple cranial mononeuopathies - Meningitis - Inflammatory - MS / vasculitis / sarcoid

Answer 194

Miller fisher

Answer 195

Legs 1. Rhombergs with feet together 2. Heel to toe walking 3. Walk on tip toes and then heels (plantar and dorsiflexion) 4. Hop on 1 foot Arms 1. Pronator drift 2. Keep eyes shut and tough a finger on both hands then ask to use that finger to touch thier nose (middle and ring) 3. Play the piano in mid air 4. Tap back of each hand 5. hold hand and quick tone and cog wheel rigidity while they raise other hand in air Head 1. Temporal visual fields "point at the fingers which move" 2. Eye movements 3. Shut eyes tight (look at pupils when open) 4. Show teeth then stick tongue out On the couch 1. Fundoscopy 2. Tendon reflexes 3. plantars

Answer 196

**Non-proliferative** - Microaneurysms and hard lipid exudates - May progress to dot/blot haemmorhages - May also see cotton wool spots which signify areas of retinal infarction similar to those seen in hypertensive retinopathy Hypertensive - Hard to distinguish in early stages but would be very unlikely to have diabtetic without mucroaneurysm formation

Answer 197

Continued eye screening with opthalmologists Glycaemic control though too rapid control can lead to paradoxical worsening. This is also seen in pregnancy Photocoagulation therapy may be used

Answer 198

Inhibits angiogenic growth factor release

Answer 199

Cataracts more common Retinal artery / vein occulions Vitreous haemorrhages High rates of cranial nerve palsy's affecting eye movement

Answer 200

Grade 3 or 4

Answer 201

Baseline obs especially blood pressure Formal acuity / visual field testing CT imaging of brain with venous phases for sinus thombosis. Consider MR imaging Consider LP for quantification of pressure / theraputic / investigation of raised protein

Answer 202

Women Obesity Steroid use Usually with headaches + visual disturbance Treat with reducing body weight - theraputic LP can be used in interim followed by diruetic eg ACETAZOLAMIDE - carbonic anhydrase inhibitor -If this is not working may reqiure surgery eg VP shunting

Answer 203

Optic neuritis Optic nerve tumour Central retinal vein occlusion

Answer 204

Optic neuritis is commonly retrobulbar

Answer 205

4-20 cmH2O

Answer 206

Transient distortion / loss of vision during manouvers which raise ICP - eg coughing and sneezing or bending forward Suggests there is an immenent threat to sight and reducing pressure should occur

Answer 207

Similar in both eyes Ie occurs after the optic chiasm in brain

Answer 208

Most commonly stroke Trauma Infection Migrane

Answer 209

Cause contralateral... Temporal - Homonymous (congruous) superior quadraniopia Parietal - inferior

Answer 210

Inferior contralateral homonymous quadraniopia Dyscalcia - difficulty calculating Dylexia - reading issues Dysgraphia - writing issues Asterionosia - loss of ability to identify objects by touch

Answer 211

Macula sparing likely lesion in occipital lobe supplied by posterior cerebral artery peripheral vision from posterior cerebreal artery and central from middle cerebral artery

Answer 212

Pituitary adenoma - Sits inferiorly to chiasm - Superior visual fields first Cranoppharyngoma - Sits superiorly - Inferior fields first Meningioma

Answer 213

Anterior - Adrenocorticotrophic hormone (ACTH) - FSH, LH - TSH - Prolactin Posterior - Oxytocin - ADH

Answer 214

remnants of Rathke's pouch,

Answer 215

The speech is dysarthric and there is evidence of nystagmus in all directions. There is evidence of rebound phenomenon and finger nose testing demonstrates past pointing and intention tremor. There is also disdiadokinesis and impaired heel shin testing. The gait is ataxic but rhombergs test was negative. to complete my exam I would like to perform a full cranial nerve and neurological exam

Answer 216

Lesions eg stroke / tumour / demyelination in posterior fossa may affect both.

Answer 217

A tremor that is not present at rest but is present on movement - Gets maximal on reaching target

Answer 218

assessment of rapid alternating movements

Answer 219

overshooting the target

Answer 220

Worsening ataxia when visual stimuli removed Usually much worse with a sensory ataxia rather than a cerebellar ataxia

Answer 221

Ataxia caused by loss of proprioception - Damage to dorsal collum - Peripheral neuropathy

Answer 222

Phenytoin Alcohol Family history

Answer 223

Small cell lung cancer Ovarian Ca Lymphoma Anti-Neuronal antibodied Eg Anti-hu / Anti-yo

Answer 224

Dominant usually present in adulthood - There are a group of approx 15 autosomal chronic degenerative cerebellar ataxias which are grouped by number of the chromosome they are found on. Resessive - usually present in childhood - Friedreich's ataxia - Ataxia telangectasia

Answer 225

Diabetes Optic atrophy Cardiomyopathy Kyphoscoliosis Seonsory neuropathy ## Footnote Dont go walking on the DOCKS if you have ataxia

Answer 226

Cerebellar syndrome - Eg demyelination, infaction or SOL Vestibular issues often associated with N&V / tinnutus and impoved by keeping the head still Nystagmus - the fast phase is towards. Slow phase is away

Answer 227

This middle aged gentleman has features of parkinsons. There is cogwheel rigitidy of his wrists and lead pipe rigidity of his arms with an assymetic pill rolling tremor He had an expressionless face and low volume, slow speech There was a shuffling gait with a stooped posture and lack of arm swing. He demonstrated bradykinesis when asked to perform rapid hand movements and micrographia when asked to write a sentence I would like to complete my exam by performing a full neurological exam, and assessment of L/S blood pressure .

Answer 228

Alpha-synuclein (α-syn) is a protein that plays a role in the development of Parkinson's disease (PD). In PD, α-syn misfolds and clumps together into Lewy bodies (LBs). These clumps may be toxic and spread from neuron to neuron, potentially causing the disease to spread throughout the brain.

Answer 229

Single proton emission CT Used in parkinsons to look at reduced dopamine in substatia nigra. Does not distinguish between parkinsons and parkinsons plus. May use in a patient with a tremor when essential tremor and parkinsons are hard to differentiate

Answer 230

slow onset repetitive involuntary movements. Eg lip smacking and tongue protrusion prolonged muscle contractions causing abnormal postures ## Footnote Both seen with prolonged use of Levodopa

Answer 231

Essential - often symmetrical - More prominet on movements - Often improves with alcohol

Answer 232

- Depression common - Lewy body dementia - Common to get cognitive impairment in corticobasal degeneration and PSP Psychosis associated with medications

Answer 233

Cant Veel PAIN cisplatin cyclosporin Vincristine Phenytoin Amiodarone Isoniazid Nitrofurantoin [Metronidazole ]

Answer 234

Bedside Blood glucose / urine dip Bloods Fasting blood sugar, HBA1C, Urea / renal function Thyroid function ESR autoantibodies B12 Igs If Acute -> LP for raised protein (Gillian barre) Nerve conduction studies to help clarify diagnosis and to determine whether it is axonal or demyelinating EMG - show signs of muscle denervation

Answer 235

Focused A-E Check FVC and blood gas Assess bulbar function - risk of aspitation ECG and BP as risk of. arrythmia and autonomic compromise If diarrhoea - sample for campylobacter as they have worse prognosis Treatment may require IVIg or plasma exchange

Answer 236

Gillian barre Lead toxicity Acute porphyria Diptheria Malignancy ## Footnote Most acute causes will cause predominant motor

Answer 237

Leprosy charcot marie tooth acromegaly amyloid

Answer 238

Demyelinating - Affect velocity of nerve conduction Axonal - affect amplitude of response

Answer 239

Conservative - Minimise complciations eg foot ulcers - Podiatary and supportive footware - Mobility aids Medical - Control of cause Eg diabetes / b12 / alcohol - Pain - eg Gabapentin / Tricyclic antidepressants

Answer 240

Motor, proprioception and vibration sense are all transported by large diameter axons Pain and temperatire - small diameter axons Small fibre damage eg alcohol toxicity is likely to affect small diameter axons first ->pain loss / burning

Answer 241

Mononeuropathy - single nerve palsy In some conditions may develop multiple single nerve neuropathies which mimic a polyneuropathy Causes - Diabetes - Vascultiis - connective tissue diseases - Malignancy - Amyloiid

Answer 242

Varient of gillian barre Triad of Ataxia arreflexia opthalmoplegia Anti-ganglioside antibodies

Answer 243

On inspection I note a wheelchair and indwelling urinary catheter There is dissuse atrophy affecting the muscles of the lower limbs Tone is increased bilaterally There is a symmetrical pryamidal pattern of weakness . There is hyperreflexia Pain and temperature sensation are reduced bilaterally with evidence of a sensory level at Eg T10 Gait examination revealed a spastic gait. I think this is spastic paraparesis secondary to a spinal cord lesion. I would like to perform an MRI scan of the spine and consider an MRI brain, LP, nerve condution studies and EMG following a full history (including family and trauma) neurological examination

Answer 244

Loss of proprioception and vibration predominant - B12 deficiency ## Footnote Any evidence of gastric surgery / crohns [Dude Is Just Feeling Ill Bro]

Answer 245

MS - interthecal immunoglobin production

Answer 246

Extrinsic compression - spondylosis - vertebral disc disease - Tumour - Haematoma Intrinsic - Infective - Transverse myelitis - Inflammatory - Demyelination - Vascular - Spinal cord infarction - Trauma Congential - cerebral palsy - Spina bifida Hereditary spastic parapersis Primary lateral sclerosis ## Footnote In a young person Trauma Demyelinating Hereditary In older Spondylosis Infarction

Answer 247

- The anterior cord has the Spinothalamic and corticospinal tracts which causes.a reduction in power as well as pain and temperature sensation - Often prioprioception and vibration sense are preserved Eg Anterior spinal artery infarct or compression from degenerative vertebral disk / tumour

Answer 248

The dorsal collums relay proprioception and vibration sense - Conditions affecting posterior cord preferentially affect these with preservation of pain and temperature sensation - Eg subacute combined degeneration of the cord in b12 deficiency

Answer 249

Dietary - vegans Decreased absorbtion - must have intrinsic factor eg pernicious anaemia - Ability to absorb from terminal ileum - crohns / surgery - Bacterial overgrowth

Answer 250

Caused by a unilateral spinal cord lesion - Ipsilateral weakness and loss of proprioception and vibration - Contralateral loss of pain and temperature sensation

Answer 251

Acute spinal cord injury -> develop flaccid paralysis and may have loss of sensation / sphincter controll and autonomic dysfunction. Eg Priaprism, hypotension, bradycardia These findings often replaced by spastic parapersis over hours to days

Answer 252

Some lesions in spinal cord can affect different tracts Eg spinothalamic - pain and temp Dorsal collum - prioprioception and vibration This means not all sensory modalities are lost at the same time

Answer 253

Mixed upper and lower signs - MND - Subacute degeneration of the cord - Friedreich's ataxia - Syphilis - toboparesis - Dual pathology - peripheral and cervical neuropathy

Answer 254

* Spinal cord terminates at L1 * lumbar sacral nerve roots make up the cauda equina * Compressive lesions below L1 conpress these -> cauda equina syndrome This manifests as bilateral lower motorneuron weakness of legs and sensory loss in lumbarsacral dermatomes with abnormalities in sphincteric function. Pain may occur

Answer 255

sensory experience that feels like an electrical shock or tingling sensation running down the spine when the patient flexes thier neck

Answer 256

Early phsio and use of orthotics Baclofen / botox can be used

Answer 257

This man presents with a small wringled tongue and evidence of fasiculations at rest. The speech is nasal in character The palate has limited movement on saying Ahh Jaw jerk is absent [To complete my exam I would like to test the gag reflex, examine for peripheral neurology and perform a SLT assesment]

Answer 258

**Bulbar** - Motor neurone disease - Myaesthenia gravis - Syngobulbia - Guillian barre - Poliomyelitis - Neurosyphlis / sarcoid **Psuedobulbar** - Bilateral stroke of internal capsule - MND - MS - Rarely head injuruy

Answer 259

**Bulbar** - Either bilateral lower motor neurone - In the Medulla - Nuromuscular junction Eg myaesthenia. **Pseudobulbar palsy** - Occurs along corticobulbar tracts between cortex and medulla

Answer 260

This lady has a tight small tongue which cannot be protruded There is evidence of hot potato speech Her jaw jerk is increased I would like to examine the peripheral neuro systems for evidence of spaciticy

Answer 261

There is global wasting in all limbs more prominently distally There is presence of both upper and lower motor neuron lesions without sensory invovlment. There is reduced power Lower motor neurone signs include... fasiculations Upper include upgoing plantars, spasticity Coordination is slow but intact. There is no sensory disturbance To complete my exam I would like to perform a full neurological exam espcially focuing on the bulbar cranial nerves with an assessment of swallowing, and forced vital capacity

Answer 262

Upper - lesions occuring before the anterior horn cell of spinal cord - Typically present with increased tone / spaciticity, pyramydal pattern of weakness, and hyperreflexia Lower - Lesions affecting nerve after exit from anterior horn cell of spinal cord - Hypotonia, fasiculations, muscle wasting, hyporeflexia

Answer 263

Chronic debilitating disorder often occuring in middle age, with progressive destruction of both upper and lower motor neurones.

Answer 264

Physiologic - eg post exercise / electrolyte (low mg / potasium) Medications eg salbutamol Benign fasciulation disorder MND Syringomyelia Spinal muscular atrophy (kennedy) Charcot marie tooth cervial spondylarthropathy

Answer 265

Syringomyelia - would expect distal weakness and wasting with dissociated sensory loss cervical myelopathy - would expect dorsal column involvement

Answer 266

Depression - consider antidepressants Pain - using alagesia pain ladder dysphagia - SLT and dietician for consideration of PEG Speech and communication - SLT team Spasticity - PT and orthoritc - baclofen Resp weakness - resp team for consider niv.

Answer 267

On inspection there is bilateral ptsosis and myopathic faces Her speech is slightly dysarthric There is normal tone in the limbs with proximal weakness bilaterally There was evidence of fatiguability in held upwards gaze and proximal mscles of the limbs. Sensation and coordination were normal. Cough and sniff were weak. There was no evidence of myotonia To complete this exam I would complete spirometry and assess the swallow.

Answer 268

My most likely differential is myaesthenia given the involvement of cranial nerves and perhiperal muscles with evidence of fatiguability - Myopathies -poly/dematomyositis I would not expect these to involve the extra ocular muscles - Autoimmune thyroid disease could cause a myopathy and complex opthalmoplegia - Miller fisher syndome could cause complex opthalmoplegia and progressive descending weakness - Inherited muscular dystrophies Would not expect fatiguability - Lamber eaten myaesthenic syndrome Would not expect fatiguabilities

Answer 269

Most cases present with purely occular myaesthenia Remains confined to occular muscles in approx 15% May have chewing / ptosis / issues holding head up Resp weakness - SOBOE / chest infections Limb weakness - diffculty rising from chair ## Footnote If it remains confined to the eyes for 3 years it is likely that it will never progress to generalised myaesthenia

Answer 270

The normal decline in Ach availability in the neuro muscular junction on repeated neve impulses is much more significant when there is reduced Ach receptors on the post synaptic junction.

Answer 271

Repetitive stimulation of muscles may result in a decriment of thier action May help to exclude other mimicks ## Footnote only around 50% sensitive

Answer 272

10% have thymoma 75% thymus hyperplasia Thymectomy is sometimes offered to young people

Answer 273

Opthalmoplegia which isnt limited to a single cranial nerve

Answer 274

Myaesthenia Lambert eaton Botulism

Answer 275

Botulinum toxin prevents release of Ach at NMJ

Answer 276

LEMS - Tends to affect limbs - May improve strength o exercise - Increasing reflexes on repeated tapping / post exercise Myaesthania - predominant CNS - normal reflexes

Answer 277

Diaphragmatic weakness

Answer 278

Calcium + bisphosphonate PPI Regular glucose and BP checks

Answer 279

- Anaesthetics - non depolarising - Antibiotics - Including surgery antibiotics (penicilliamine rarther than penicillin) Aminoglycosides, macrolides, quinilones - antimalarials - B blockers - Magnesium

Answer 280

C1/C2: Neck flexion and extension C3: Lateral neck flexion C4: Shoulder elevation and shrugs C5: Shoulder abduction, external rotation, and elbow flexion C6: Elbow flexion and wrist extension C7: Elbow extension and wrist flexion C8: Thumb extension and finger flexion T1: Finger abduction and adduction L1/L2: Hip flexion L3: Knee extension L4: Ankle dorsiflexion L5: Big toe extension S1: Hip extension, ankle plantarflexion, and ankle eversion S2: Knee flexion S3–S4: Anal wink

Answer 281

Trauma including disc prolapse Spinal haematoma Malignancy - primary or mets (lung breast prostate and others)\ Infective - osteomyelitis, TB and epidural collection Inflammatory

Answer 282

**HyperCa** - Fluids - bisphosphonates **Compression** Urgent - Steroids - Palliative ratiotherpay - Analgesia - Occationally surgery After - May need to involve palliative care team for psychological support - Physio once possible

Answer 283

Dorsal collums - proprioception vibration and light tough - Ipislateral in cord - Decussates in medulla Anterior Spinothalamic - Pain and temp - Deccussates approx 2-3 segments above in cord then contralateral Lateral corticospinal - Motor - Decussates in medulla

Answer 284

Damage to cord above the level of the sympathetic outflow ~T5/6 -> Unbalanced sympathetic reflex Results in. - Hypertension - Loss of bladder and bowel - Sweating and Headaches ## Footnote Below the level of injury impulses from spinothalamic and posterior collums. However inhibitory outflow created by injury is unable to pass below level of obstruction -> So release of neurotransmitters Eg noradrenaline and dopamine -> Increase in blood pressure Vasomotor reflexes attempt to decrease blood pressure though parasympathetic stimulation of heart. -> severe sweating and skin flushing

Answer 285

Good to assess in MSA Many treatments in parkinsons may cause postural symptoms

Answer 286

Not all patients need pharmacological treatment Some may be able to have an acceptable level of indepence and QOL with assistance from **PT, OT** and sometimes **dietitan**. Medical treatments include **Levo-dopa + dopa carboxylase inhibitor** is generally first line. The distadvantage of this is that it will only be effective for a finite number of years Others include **dopamine agonists**, **anticholinergics** which help with tremor and **monoamine oxidase B** **inibitors** which inhibit the break down of dopamine. Also **COMT inhibitors** which prevent breakdown of dopamine Some refractory cases reqiure surgical treatments such as deep brain stimulation

Answer 287

Diagnosis - 2 copies of expanded GAA repeat of frataxin gene Diabetes testing Echocardiography MRI brain / spinal cord

Answer 288

My Progressive Central Pathways **MS** - Auttoimmune to myeline **Progressive multifocal leukoencephalopathy** - Caused by JC virus - think HIV / immunocompromised - Including natalizumab for MS - Predominant speech / visual symptoms (parietal and occupital most affected) - JC virus in CSF **Central pontine myelinolysis** - Caused by rapid changes in osmotic pressures - Chronic hyponatraemia which is too rapidly corrected - Affects CNs 9-11 but not thier nuclei -> **Pseudobulbar** palsy - Can cause locked in sydrome **Post infectious A[DEM] Acute disseminated encephalomyelitis** - Usually seen in children post infection with an acutely pregressive demyelination after infection. - Autoimmune - May look similar to MS on MRI / oligoclonal bands in CSF - Doesn't recur ## Footnote Correction of Na too quick - From high to low your brain will blow, from low to high your pons will die

Answer 289

**Guillian-barre syndrome** - Camplylobacter, mycoplasma, CMV, EBV - Segmental demyelination of peripheral nerves **Charcot-marie-tooth disease** - Most autosomal dominant - causes defective schwann cells (PMP22 gene) or axonal cells - Distal lower weakness and sensory symptoms - Foot drop (common peroneal) and pes cavus (weak muscles of foot) - Scoliosis

Answer 290

- EMG and nerve conduction studies (type 1 demyelinating, type 2 axonal and others) - Genetic testing PMP22

Answer 291

Onion bulb formation - nerve cell has inner layers of damaeged myelin with outer layers of replaced

Answer 292

- Similar to lower but less severe - Generalised wasting more prominent distal - Hyporeflexia - peripheral neuropathy

Answer 293

Neuropathy screen Hiv bbv screen Glucose hba1c Igs | If give IVIg to IgA deficiency -> risk of allegic reaction

Answer 294

Family history (autosomal dominant)

Answer 295

- Neurophysiolocial studies Differentiate demyelinating / axonal - Genetic testing

Answer 296

No disease modifying treatment Conservative - physio inc orthoptics eg foot drop - OT help at home - Consider screening of relatives

Answer 297

Spinobulbar muscular atrophy Progressive muscular atrophy Duchennes muscular dystrophy Bekker muscular dystrophy Fascioscapular humeral muscular dystropy Limb girdle muscular dystrophy

Answer 298

Limb girdle muscular dystrophy

Answer 299

* On inspection this patient has a wheelchair / stick * There is evidence of wasting of the lower limb muscles * His speech is dystharthric and there is which supports evidence of a cerebellar ataxia * However there is also evidence of dorsal collumn dysfuction with impaired proprioception and vibration sense distally * Of note there was areflexic knee jerks but upgoing plantars indicating upper and lower motor neurone signs In the context of someone young this would be compatable with Friedreich's ataxia although other conditions could present similarly

Answer 300

Autosomal ressessive of ATM gene (Ataxia telangectasia mutant) Usually codes for DNA repair - Recurrent infection (T-lymphocyte dysfunction) - Risk of Cancer - Ataxia (Cerebellum) - Telangectasia (unknown pathophys) Diagnosis - ATM gene - Low Immunoglobulins - raised alpha fetoprotein Treatment - Non specific Abx PT/OT/Dietician - Monitoring for Ca

Answer 301

- Wide legged (broad based) - ataxic - High stepping - foot drop = distal weakness - Foot slapping (heavy) = sensory / proprioceptive loss - Waddling (side to side as walk) = Proximal (hip girdle) weakness Painful

Answer 302

DANG THERAPIST Diabetes Alcohol - directly toxic + Nutritional Nutritional b12 / b1 / b6 Guillian barre / CIDP Toxins - Can't Veel PAIN. Hereditary- charcot Marie tooth, Friedrich Environmental - heavy metal eg lead , nitric oxide Relapsing - CIDP Amyloidosis Paraproteinaemia Systemic - renal failure uraemia, hypothyroidism, HIV, syphilis, lime Tumours - paraneoplastic / nerve sheath

Answer 303

Both cause foot drop **L5 nerve** Pain in the buttocks and numbness, and problems with both **foot inversion and eversion** **Common peroneal nerve** Numbness just below the knee, and problems with foot eversion, but intact foot inversion

Answer 304

Crossed adductor reflex - Precus medial thigh -> if other leg moves to adduct it is positive Hoffman in hands

Answer 305

LFTs alcohol Ureamia Hba1c B12 foliate Paraproteins Thyroid function

Answer 306

Amitrip / duloxetine

Answer 307

Macular sparing = in occipital lobe - Could be either posterior / middle cerebral artery as both supply the part of occipital lobe which have macula Middle cerebral atery -> likely to get weakness on the same side as the homonymous hemaniopia

Answer 308

Retinitis pigmentosa Cataracts - if diabetic / steroid Glaucoma Short sighted Diabetic retinopathy Vitamin A/E deficiency if pancreatic / CF patients Pappiloaedema Lebers optic atrophy Toxic eg methanol

Answer 309

Confirm with opthalmology Referral to a geneticist - variable inheritence based on cause No treatements Signpost to charities OT - visual aids Most people are registered blind by middle age

Answer 310

Immune checkpoint inhibitor Tends to -> myasthenia (seropostive) myositis and myocarditis Triple M

Answer 311

- Steroid dip - temporary worsening of symptoms when commenced - Long term - diabetes, HTN, cushing, thinning skin / brusing, adrenal insufficiency, AVN...

Answer 312

Botulism - more rapidly progressive - Involves pupil