Managing Acute NeurologyMemo to myself 17.11.17 – several videos didn’t play ?trimmed clips. Test on laptop first.

BACK TO BASICS1. PROCESS?

VINDIE

VASCULAR Arterial stroke, Bleeds, Venous (thrombosis, hypertension)

INFECTIVE Bacterial, Viral, Atypical, Prion

NEOPLASTIC Primary, Secondary, Paraneoplastic

DEGENERATIVE Brain, Spinal, Disc disease, Genetic

INFLAMMATORY Demyelination, Vasculitis

EPISODIC Epilepsy, Migraine, Metabolic, Functional, TIA

BACK TO BASICS2. LOCALISATION?

NEUROAXIS

BRAIN – BRAINSTEM

SPINAL CORD

ANTERIOR HORN CELL

NERVE ROOT – PLEXUS – PERIPHERAL NERVE

NEUROMUSCULAR JUNCTION (NMJ)

ENDORGAN (MUSCLE, EYE, SKIN)

V – Strokes / Venous sinus thrombosisI – Meningitis/Encephalitis/CJDN – Tumours/ParaneoplasticD – Alzheimer’sI – MS / SarcoidE – Epilepsy, Mitochondrial

V – StrokesI – Listeria, Bickerstaff’sN –TumoursD –I – MS / Sarcoid E –

V – StrokesI – Abscess, TBN – TumoursD – Disc diseaseI – MS / NMOE -

V –I – PolioN – ParaneoplasticD – MNDI –E –

V –I –N – NeoplasticD –I – GBSE –

V –I –N – ParaneoplasticD – Pressure palsiesI – VasculitisE – Metabolic (toxins)

V –I – BotulismN – Paraneoplasia (LEMS)D –I – MyastheniaE –

V –I –N – DermatomyositisD – Genetic (Dystrophies )I – Polymyositis, VasculitisE – Mitochondrial

UMN signsPseudobulbar palsyFrontalis sparingTone: spastic, clonusReflex: Babinski,Hyper-reflexia

LMN signsBulbar speechBell’s signAnt Horn: wasting, fascics, no sensoryRoot: areflexia, proximal weaknessNerve: areflexia, distal weaknessNMJ: fatigueable weakness, no sensoryMuscle: proximal weakness, no sensory

Functional SignsMismatch of signs v functionDistractabilityEntrainmentHypo / hyper-reflexiaGlobal / patternless weaknessTypical patterns (gait, seizures)

CASE VIGNETTES

All of these cases are real, or else the case is real and the pictures / video demonstrates the relevant findings

Occasionally I have combined 2 or more cases for convenience

I have tried to cover the most typical neurological presenting symptoms, but will assume you are reasonably confident with acute headaches due to bleeds or meningitis, and standard strokes

MOST TYPICAL ACUTE PRESENTATIONS

Weakness

Pain

Vision and eye movements

Breathing

Altered consciousness or behaviour

Involuntary movements and seizures

PART 1

PART 2

CASE 145FCleaner

3/7 difficulty walking Woke up and struggled to get down the stairs

By end of day was walking with support

Next morning was unable to get out of bed and had some mild UL weakness B3

3/7 back pain requiring codeine

No hx of spinal injury

2 most likely differentials?

2 discriminatory questions?

2 most useful examination clues?

GBS vs Functional

Discriminatory Questions Recent (4 wks) infective illness?

Early tingling in fingers (or toes)?

Did it Peak at onset or Progress?

Useful Examination Clues Reflexes

Pattern of weakness

GBS? Lost reflexes

Distal, proximal or ‘pyramidal’ weakness

Functional? Intact reflexes

Global weakness

Mismatch between power when tested vs observed gait

?Typical functional gait leg drag, buckling/bouncing, flapping, walking on ice

She has absent LL reflexes, reduced UL reflexesPyramidal weakness 2/5 legs, 3/5 arms

She can’t weight bear so you can’t assess gait

Next day she develops dysarthria and you re-examine her

Video

Why is she dysarthric? How could you make her speak normally?!

What eye sign is demonstrated?

The chap on the left was referred to the Acute Neurology clinic ?GBS with 2/7 LL weakness, falls and gait difficulty – note the functional buckling at the knees

Reminiscent of ‘shellshock’ gait disturbances -https://www.youtube.com/watch?v=IWHbF5jGJY0 (below clip is at 1.19)

The chap below has a more bouncy, tremulous functional gait -https://www.youtube.com/watch?v=nIDc8cU6znM

CASE 249FBeauty Therapist

3/7 progressive severe new neckache

Intermittent tingling in both arms

Denies headache, vomiting, photophobia or neck stiffness

Diabetic

T 37.4

What is the first thing to exclude?

What 2 signs would suggest it?

Cervical spine abscess and cord compression

The 2 signs Upper motor neurone signs in the UL

Neck tenderness

What does this MRI show?

In her case, neurological exam is normal

Urgent MRI cervical cord is normal

Subtle suggestion of new dysarthria

What else do you need to exclude?

Vertebral artery dissection

Further history to ask about? Antecedent neck hyper-extension / trauma

Posterior circulation symptoms

vertigo, dysphagia, dysarthria, hemianopia

Admits to recently having expensive salon haircut

MRI / MRA shows signal dropout in left vertebral suggestive of dissection - but no infarcts seen

How would you treat?

No clear evidence to guide us

Rule of thumb for carotid or vertebral dissections - Pain w/o neurology antiplatelets

Pain with neurology anticoagulants (esp if evolving symptoms)

Because she’s had tingling in her arms which could indicate brainstem embolic TIAs, I anticoagulate:

Low risk strategy as no infarct to bleed from

Minimises risk of evolving to basilar thrombosis with >80% mortality

Short-term treatment (3-6months)

CASE 330MIndian extraction

Sudden onset blurred/double vision and mild headache

No other symptoms

Known migraines

Your SHO examines and says he has bilateral 6ths and slightly blurred disc margins

He has an urgent CT brain to r/o raised ICP - normal

MSMRI brain

You examine him

What does he have?

Where’s the lesion?

What’s the most likely differential?

What test does he need?

Right

CASE 422FMansfield

1/52 loss of vision in left eye

Optician found L VA 6:60 and queried ipsilateral papilloedema

Right eye unaffected

2 commonest differentials?

2 discriminatory questions?

3 most valuable bedside eyes tests in this situation?

Optic neuritis vs functional

Discriminatory questions Pain on moving eye?

Did it Peak at onset or Progress?

Any neurological disturbances in the Past?

Essential bedside visual tests Colour vision (Ishihara – many free apps)

Pupillary light reflexes

Swinging torchlight test

Room needs to be dark for proper dilatation reflex in good eye

Visual fields for functional patterns

Functional visual field patterns

1. Tubular fields (tunnel vision)

2. Binocular extinction Both eyes open =

homonymous hemianopia

Bad eye only – hemianopia

Good eye only – full fields!

She had a left RAPD and normal visual fields

She developed 1/5 weakness and numbness in arms overnight

This was her scan – what does it show? A very long cord lesion (>3 vertebrae)

What is the diagnosis? Neuromyelitis optica (NMO) – variant of MS

?commonest causative antibody Aquaporin 4

Needs urgent IV treatment with steroids

CASE 571MPig farmer

1/12 progressive breathing difficulties

Worse in bed or after meals

No known heart or lung disease

GP mentions he looks more drowsy than normal but patient says he feels wide awake

Not weak but has recently had a few falls

How would you decide if his breathing problems were neurological at the bedside?

What is the neurological differential?

Look for evidence of respiratory muscle weakness – egs? Sniff (insp)

Cough (exp)

Abdominal paradox (diaphragm)

Neck flexion power

Single breath count test (equates to FVC)

Neurological differential for resp musc weakness? High cervical lesion

Anterior horn cell – usually MND

Nerve root - usually GBS

NMJ – usually Myasthenia or LEMS

Muscle – eg acid maltase myopathy (Pompe’s dis)

Which features of the examination help distinguish these?

Inspection Ptosis / fatigueable ptosis

Tongue

Wasting (LMN – bulbar)

Fasciculations (LMN – bulbar)

Speed (slow – pseudobulbar)

Range (restricted – pseudobulbar)

Limbs

Muscle wasting

Fasciculations

Speech bulbar vs pseudobulbar dysarthria

Pseudobulbar = MND as only cause of mixed UMN + LMN

Power Fatigueable weakness – Myasthenia / LEMS

Reflexes Absent - GBS Normal – Myasthenia Augmented - LEMS Hyper-reflexia / Babinski – Cord compression, MND

Sensation Abnormal – rules out MND, NMJ, Muscle

CASE 649MPublican

Admitted to ENT with acute onset of dysphonia

Also c/o of being unsteady and slightly blurred vision

Drinks 40-50 units a week, but alcohol levels <50 on admission

Nasendoscopy is normal

ENT ask you to assess him ?stroke

He tells you an additional hx of dysphagia to liquids

O/E

Nasal sounding speech, but no slurring. No confusion - MMSE 30/30

Difficulty with tandem gait, but no limb weakness

What kind of speech problem is this?

What 2 signs should you check for that might give the diagnosis?

Play Video 1 comments?

Nasal speech suggests a bulbar dysarthria

Nasal regurgitation is classic for neurological dysphagia

And he has a bipalatal palsy explaining both

His symptoms of blurred vision and unsteadiness mean you should also check for Areflexia and Ophthalmoplegia Video 2

Clinically confirms Miller Fisher syndrome (GBS variant) Ophthalmoplegia (supranuclear gaze palsy)

Ataxia

Areflexia But can also have other features such as lower cranial nerve palsies

GQ1b antibody +ve

Main differentials are: Wernicke’s – Ophthalmoplegia, Ataxia, Confusion (+/- toxic areflexia)

Myasthenia

CASE 749M

Brought in off legs

Has had increasingly unsteady gait for the past 2 weeks

Becoming increasingly confused according to family

In the past 5 days has a tremor of his arms and legs

O/E

Confused in time and place but able to obey simple commands

Brisk reflexes and upgoing plantars

Jerky limb tremor Video

What kind of movement disorder is this?

Continues to rapidly deteriorate

Becomes off legs

Then mute and unable to communicate within 1 week

Fully dependent on nurses for all care needs

He has to have MRI and LP under GA

What do you need to warn the lab about his CSF samples?

What’s the diagnosis?

Will might his MRI show?

What might his EEG show?

What should his CSF show?

• Sporadic CJD

• Basal ganglia hyperintensity

• Periodic complexes

• The usual CSF tests will all be N

• CNS protein 14-3-3 will be +

IS THIS ABOUT HALFWAY?

CASE 848MTurkish business man

Brought in with GCS 3

Found in bed

No evidence of trauma or fever

PMH: depression

Pupils normal, no meningism, no fever

Slightly blurred disc margins

WCC 14 but CRP <5

ABGs: pH 7.22, lactate 5.1, bicarb 19, normal CO2

What is the immediate differential?

Overdose vs seizure

CT brain reported normal

GCS picks up to 6 over 2 hours, pH/lactate normalise

Collateral history emerges of a severe headache for the past week

Toxicology screen negative, no tablets missing to suggest overdose

You conclude it was a seizure

What is the concern?

What test next?

Persistently low GCS suggests a secondary cause vs simple epilepsy

Who would LP? Unlikely to be coning, as GCS slowly recovering BUT

Any significantly low GCS plus ?papilloedema would suggest avoid LP

You do blood cultures, treat for viral and bacterial infections

And you do MRI brain which shows?

Venous sinus thrombosis Pathergy testing suggested Behcet’s disease

Note VST easily missed on non-contrast CT

CASE 918F Boston schoolgirl

Dad brought her in Very irritable with family, normally very placid Has had lots of nightmares in the last few weeks

Mum says she’s very stressed due to exams coming up “Just behaving like a typical teenager”

Ward staff say she yelled at a HCA

Nursing staff want rid of her

Patient tells you she feels fine and wants to go home

O/E: Alert, orientated, but with a slight ‘attitude’

Neuro physical examination is 100% normal

MMSE scores 29/30 (loses 1 pt for phrase repetition)

Bloods come back normal except for a borderline low Na 131

What are the 2 main possibilities?

Stress vs an ‘organic’ psychiatric disorder

Determining which it is partly rests on:

1. Is the -1 on MMSE significant?

2. How could the Na 131 be relevant?

Phrase repetition requires adequate attention Said to be sensitive to acute brain dysfunction (if native speaker)

No explanation for lowish Na - but can it explain this picture?

You keep her in overnight for observation and repeat bloods

The next morning, the nurses can’t wake her up

All obs are fine and she can’t tolerate an airway

They suspect she’s “messing around”’ and indeed she’s wakes up fine in time for lunch, with an even worse attitude

But the same thing happens late evening, again unrousable

She’s then awake half of the night

What could her unrousable episodes be?

1. ‘Sleep attacks’ – seen in various neurological disorders

2. Non-convulsive status epilepticus (complex partial status)

Limbic encephalitis

You request MRI but it fails as she becomes very agitated in scanner

Needing 1:1 nursing, tries to bite a member of staff so is taken to ITU and sedated

Subsequent CT is normal

LP shows no cells, protein of 900, normal glucose

Woken up, agitated spells alternating with unrousable episodes

Ward EEG during an episode shows normal sleep and nil epileptic

Na falls to 125 and then 121 and she is fluid restricted

Osmolalities are 230 (serum) and 255 (urine)

MRI under GA is planned but she then has a tonic clonic seizure

Diagnosis?

MRI shows temporal lobe signal changes

What other tests would we ask you to do?

Antibodies for VGKC-complex (LGI1 and CASPR2)

Full body examination

CT or MRI body looking for occult tumour

If negative, PET scan for occult tumour A proportion are paraneoplastic

Treatment IV Steroids PEX IVIG

If paraneoplastic, need to remove the tumour

CASE 1061FRetired widow

Admitted to B3 with chest infection and mild confusion

Symptoms settle with antibiotics

Later that day she mentions double vision to the nurses

Vague history - unclear if acute and if ever had before

O/E

Double vision looking left or right

No visible ophthalmoplegia

No ptosis or fatigueable ptosis

No dysarthria

Saying a few odd things, admits she’s very lonely and depressed

S/B Neurology ?MG, but best to get an MRI brain scan

• Gets sent for an MRI, but ?panics and scan has to be abandoned

• You are asked to see her to see if she is willing to try again and whether she might need sedation

• It isn’t easy getting a clear account of what happened

• Video 1 What kind of problem is she demonstrating? • Video 2

• Admitted to drinking 30 units a week since losing husband• Still c/o of some diplopia, so treated with Pabrinex• Diplopia resolved within 24 hours

• Dx: Wernicke-Korsakoff’s dementia

CASE 11 72M

Admitted with D & V - treated with fluids and antiemetics

On day 2 develops confusion and says he sees animals on the ward

Unable to self care or stand due to stiffness

PMH of tremor thought to be related to his antidepressant (Prozac)

Not on any other meds at homes

O/E

Afebrile, pulse and BP normal, not sweaty

Confused in time and place

Globally rigid

Rest and postural tremor

What is the differential?

Which drug needs to be stopped?

Lewy body dementia

Neuroleptic malignant syndrome (NMS)

Serotonin syndrome (SS)

Drug?

He’s probably received an antiemetic with dopamine antagonist properties like metoclopramide or prochlorperazine

NMS and SS are unlikely given no fever or autonomic features

CK comes back normal and he recovers on stopping metoclopramide

In FU clinic he describes occasional visual hallucinations

His family report variable confusion day to day

He’s walking again but it isn’t normal Video

Parkinsonian gait – no arm swing, emergent R arm tremor

All in keeping with Lewy body dementia

SEIZURES CASES 12 – 14

DISCUSSION

IDEALLY SPLIT INTO SMALL GROUPS BASED ON WHETHER YOU HAVE CASE 12, 13 or 14

DISCUSS YOUR CASE WITH THE OTHERS IN YOUR GROUP

WRITE DOWN YOUR ANSWERS TO THE QUESTIONS

ABOUT 5 MINUTES

CASE 1260M

Admitted with an unwitnessed blackout

Hot day, standing, came round on floor. Confused after for ~5 minutes

No tongue biting or incontinence, no head injury

Further hx

Gets an odd feeling every 1-2 weeks since he lost his job 2 yrs ago

It occurs suddenly, mainly when stressed “It’s hard to describe doc - like seeing a familiar movie reel in my head”

He gets a feeling like an epiphany

But afterwards can’t quite recall what that feeling was

Fully aware and able to communicate throughout

Lasts <60 secs - stops abruptly

CT and EEG are normal

List 4 salient diagnostic features

Are his regular events seizures, anxiety or something else?

What might his blackout have been?

CASE 1260M

Admitted with an unwitnessed blackout

Hot day, standing, came round on floor. Confused after for ~5 minutes

No tongue biting or incontinence, no head injury

Further hx

Gets an odd feeling every 1-2 weeks since he lost his job 2 yrs ago

It occurs suddenly, mainly when stressed “It’s hard to describe doc - like seeing a familiar movie reel in my head”

He gets a feeling like an epiphany

But afterwards can’t quite recall what that feeling was

Fully aware and able to communicate throughout

Lasts <60 secs - stops abruptly

CT and EEG are normal

Salient features in red, unhelpful features in grey

Focal seizures (auras)

Likely tonic clonic seizure given postictal confusion

TEMPORAL LOBE SEIZURE videos

subtle, easily missed, easily mistaken for anxiety

CASE 1319F

Presents with her first episode of shaking

Boyfriend woken by her screaming, “like she was having a nightmare”

Thought she was pointing at something with her left hand

Then twitched briefly all over

Her body then relaxed and she immediately began snoring loudly

It lasted <30 secs in total, then she went back to sleep

1y hx of similar episodes only ever at night

Boyf usually awake and notices her pointing with her left hand

Eyes are always open

He prods her and she stops pointing and tells him to stop prodding her, but is not usually confused

List 3 salient diagnostic features

Are her regular events seizures, nightmares or something else?

What was her shaking episode?

CASE 1319F

Presents with her first episode of shaking

Boyfriend woken by her screaming, “like she was having a nightmare”

Thought she was pointing at something with her left hand

Then twitched briefly all over

Her body then relaxed and she immediately began snoring loudly

It lasted <30 secs in total, then she went back to sleep

1y hx of similar episodes only ever at night

Boyf usually awake and notices her pointing with her left hand

Eyes are always open

He prods her and she stops pointing and tells him to stop prodding her, but is not usually confused

Salient features in red, unhelpful features in grey

Focal seizures. Posturing + nocturnal only frontal lobe

Shaking very likely to have been brief tonic clonic seizure

FRONTAL LOBE SEIZUREVideos

brief, motor, loud, often bizarre with rapid recovery -most likely to be mistaken for functional

CASE 1421M

1/12 history of new onset convulsive seizures

Occurring nearly every day – all the same except +/- warning

If warning – lasts 5-10 min – c/o nausea, headache, palpitations

Sometimes no warning, just falls like a “sack of potatoes”

Wife reports violent shaking with numerous falls injuries

Shaking stops and starts 3-4 times over 15 minutes, never <5 mins

Afterwards is dazed, can answer his name, but just wants to sleep

Has bitten his tongue and/or been incontinent

Several have been triggered by flashing ambulance lights

Presents to ED for 4th time this month

Wife concerned as in this one he stopped breathing at the end of shaking, before gasping awake and asking “who are you?”

List 5 salient diagnostic features

Is this epilepsy, cardiac or something else?

CASE 1421M

1/12 history of new onset convulsive seizures

Occurring nearly every day – all the same except +/- warning

If warning – lasts 5-10 min – c/o nausea, headache, palpitations

Sometimes no warning, just falls like a “sack of potatoes”

Wife reports violent shaking with numerous falls injuries

Shaking stops and starts 3-4 times over 15 minutes, never <5 mins

Afterwards is dazed, can answer his name, but just wants to sleep

Has bitten his tongue and/or been incontinent

Several have been triggered by flashing ambulance lights

Presents to ED for 4th time this month

Wife concerned as in this one he stopped breathing at the end of shaking, before gasping awake and asking “who are you?”

Salient features in red, unhelpful features in grey

Is this epilepsy, cardiac or something else?

DISSOCIATIVE SEIZURES / NON-EPILEPTIC ATTACKSVideos

Note the fast & big shaking (unlike tonic clonic sz)

CASE 1520FStudent

Presents with ‘first fit’ in Fresher’s week

Looked a bit blank, twitched and then dropped “like a felled tree”

Initially motionless and quiet, then let out a groan

Then started shaking, at first rapid, then slower but stronger

Lasted 45-60 secs

Started snoring straight after

Came round ~5 mins, tried to strip, fought paramedics

5 year history of clumsiness in the morning

Got scholarship to secondary school but failed most of GCSEs, as she constantly daydreamed in class

What is the diagnosis? What are the seizure types?

CASE 1520FStudent

Presents with ‘first fit’ in Fresher’s week

Looked a bit blank, twitched and then dropped “like a felled tree”

Initially motionless and quiet, then let out a groan

Then started shaking, at first rapid, then slower but stronger

Lasted 45-60 secs

Started snoring straight after

Came round ~5 mins, tried to strip, fought paramedics

5 year history of clumsiness in the morning

Got scholarship to secondary school but failed most of GCSEs, as she constantly daydreamed in class

Juvenile myoclonic epilepsy

Likely worsened by late nights and alcohol

TONIC CLONIC SEIZUREvideos

Note the tonic groan and tonic limbs, & the evolution of shaking from fast & small slow & big

https://www.youtube.com/watch?v=gWZGMABBfYo – note the postictal stertor (snorting)

https://www.youtube.com/watch?v=aZYgwLlAKAQ - it’s rare to see a tonic clonic from the start like this. The yelps are typical, also a few attempts at stertor. Note however some atypical factors – eg gradual onset and recovery of awareness is very quick.