Download - Managing Acute Neurology - RCP London
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.