Neuro Flashcards
What is status epilepticus?
When seizures last > 5 mins without stopping in between
MEDICAL EMERGENCY
Tx Status Epilepticus
IV Lorazepam
What is a Transient Ischaemic Attack (TIA)?
Acute loss of cerebral/ocular function
Lasting < 24 hours
COMPLETE CLINICAL RECOVERY
Ischaemia without infarction
RF TIA
Other than atherosclerotic RFs
Male
AF
VSD
COCP
How many of 1st strokes are preceded by TIA?
15%
Causes TIA
Atherothromboembolism! from Carotid artery - MC!!
Small vessel occlusion
Cardioembolism - emboli from MI, AF etc or valve disease or prosthetic valve
Hyperviscosity - polycythaemia, sickle cell anaemia
Hypoperfusion (important to look at in younger people) - cardiac dysrhythmia, postural hypotension
Where do the majority of TIAs affect?
How do these type of TIAs present?
90% affect CAROTID ARTERY
Weak, numb contralateral leg (+/- similar but less intense in arm)
Hemiparesis
Dysphasia
Hemi-sensory disturbances
AMOUROSIS FUGAX
What is amourosis fugax?
Sudden painless transient loss of vision in one eye
Described as “curtain descending over field of vision”
Pathophysiology Amourosis Fugax
Emboli passes into retinal, opthalmic or ciliary artery
Ix TIA
Until recovery, NO WAY TO DIFFERENTIATE FROM A STROKE
So i assume urgent CT? because you would act like it’s a stroke
But i guess once recovered,
GS = Diffusion weighted MRI
Carotid dopple
CT angiography
ECG - AF
Also, ABCD^2 score but NICE don’t recommend this anymore
Where do the lesser proportion of TIAs affect?
How do they present?
Posterior circulation (Vertebrobasilar artery)
Diplopia
Vertigo
Vomiting
Ataxia
Choking
Dysarthria
Hemisensory loss
Transient global amnesia
Tetraparesis
Tx TIA
Urgent 300mg aspirin
Refer to specialist within 7 days
Then will be put on statin or clopidogrel
What are the different classes in the Bamford Stroke Classification?
TACI - total anterior circulation infarct
PACI - partial anterior circulation infarct
LACI - lacunar infart
POCI - posterior circular infarct
How does a TACI present?
MUST have all 3 of the following :
- Unilateral weakness +/- sensory deficit of face, arms and legs
- Homonymous hemianopia
- Higher cerebral dysfunction (dysphasia, visuospatial disorder)
How does a PACI present?
MUST have 2 of the following :
- Unilateral weakness +/- sensory deficit of face, arms and legs
- Homonymous hemianopia
- Higher cerebral dysfunction (dysphasia, visuospatial disorder)
How does a LACI present?
MUST have 1 of the following :
Pure sensory stroke
Pure motor stroke
Sensori-motor stroke
Ataxic hemiparesis
What is Charcot’s neurological triad?
- Dysarthria
- Nystagmus
- Intention tremor
What is Uhthoff’s phenomenon?
Sx exacerbated by heat
What type of HS R is MS?
Type 4
Define MS
Chronic, autoimmune T-cell mediated inflammation disorder of CNS
Multiple plaques of demyelination
RF MS
More common the further from equator (link to Vit D?)
FHx
Exposure to EBV in childhood
Female!
Some genetic link (HLA-DR2) but def environment affects
Typical MS Patient
F 20-40yrs
Types of MS Progression
Relapsing/Remitting MC!!
Clear relapses w partial/full recovery, no progression in between
1º Progressive
Linear progression, no relapse
2º Progressive
Starts at RR then 1º
Progressive/Relapsing
Same as RR but progression in between relapses
Signs / Symptoms MS
LOSS NB
Lhermitte’s sign - tingling when neck flexion
Optic neuritis - also struggle to see red
Sensory Sx + Sx
Spasticity
Nystagmus
Bladder/sexual dysfunction
–
UHTHOFF’S PHENOMENON!!!
–
Charcot’s neuro triad
UMN signs (not LMN)
Paraesthesia
Ix MS
MACDONALD’S CRITERIA!!
At least 2 events disseminated by time and space
–
GS!! (w above) - MRI W CONTRAST
Lesions appear white
Lumbar puncture w CSF electrophoresis
(inflam proteins only in CSF bc nervous system disease)
Evoked potentials - how long impulses travel
the more demyelination, the slower the conduction
Tx MS
Acute
Acute attacks = IV methylprednisolone
Tx MS
CHRONIC
NO CURE!
1st Line - beta interferon and glatiramer acetate
Sx Tx :
Tremor - BB
Muscle spasticity -
Mild-Mod : Oral diazeam, baclofen
Focal disabling : periph nerve blocks e.g. botulinum, alcohol, phenol
SEVERE :
Reversible invasive procedures e.g. intrathecal baclofen
Irreversible e.g. functional neurosurgery
S/E beta interferon
Injection site reactions
Flu Sx (will decrease after first few months)
Mild lymphopenia
Mild-mod rise in liver enzymes
Mechanism Alemtuzumab
CD52 monoclonal antibody that targets T cells
Mechanism Natalizumab
acts against VLA-4 receptors that allow immune cells
to cross the BBB and therefore reduces number of immune cells that
can enter the CNS and cause damage
What is Myasthenia Gravis?
Autoimmune disease against nicotinic acetylcholine receptors in the neuromuscular junction
Patho MS
What is Guillain-Barre Syndrome?
Acute, inflamm demyelination polyneuropathy
Affects PNS - Schwann cells
Following URTI or GI tract infection
Signs / Symptoms GB syndrome
Within 4 weeks of URTI or GI tract infection
Symmetrical, ascending muscle weakness
Proximal muscles more commonly affects - trunk, resp and Cranial nerve esp CN7
REFLEXES LOST (deep tendon refleces)
RESP INVOLVEMENT - affects diaphragm = death!!
Paraesthesia, numbness
Back/limb pain
Cranial nerve involvement - Diplopia, dysarthria
Autonomic fts - Sweating, ↑Pulse, postural hypotension, arrhythmias
Comp GB syndrome
Clots - PE!!
Resp system - if affects diaphragm = death
Causes GB syndrome
Triggered by infection! - usually GI tract or Upper resp tract
Campylobacter jejuni MC
Cytomegalovirus
EBV
Pathophysiology GB
MOLECULAR MIMICRY
Infection occurs
∴ immune response
Body produces antibodies against antigens
But these antigens similar to those on Schwann cells
∴ attacks Schwann cell
∴ Demyelination + Acute polyneuropathy
Monitoring GB
MONITOR BREATHING BC RESP INVOLVEMENT!!
Ix GB syndrome
Clinical diagnosis, no GS
Nerve conduction studies
Lumbar puncture (L3/4) to test CSF - ↑protein, normal WCC, normal glucose
Tx GB
IV Ig for 5 days
Plasma exchange - remove Abs
(CI in IgA def patients!! will cause allergic reaction)
Supportive care
VTW prophylaxis (LMWH) e.g. SC enoxaparin and compression stockings - bc clots are common
Spirometry!
If FVC < 0.8, consider intubation and ITU
Prognosis GB
80% recover completely - bc myelin sheath recovers
15% lasting weakness
5% die
Quick!
Symmetrical limb weakness, Absent tendon reflexes and ↓ Sensation
What is it?
GB syndrome
In whom does Myasthenia Gravis usually present in?
Female < 40
Males > 60
How can you distinguish from Motor Neurone disease and Myasthenia Gravis?
MND never affects eye movements!
What type of HS reaction is Myasthenia Gravis?
Type 2
Signs / Symptoms Myasthenia Gravis
MUSCLE WEAKNESS
Worse at end of day/with exertion
Better with rest
Starts w/ head and neck! then progresses to lower body
Eye muscle weakness - diplopia
Ptosis
Myasthenic snarl - hard to smile, looks weird
Jaw fatigue
Dysphagia - bulbar weakness
Dysphasia
Pathophysiology Myasthenia Gravis
Normally, acetylcholine crosses synaptic gap and attaches to receptors
MC!! - Immune system produces Nicotinic Ach-R antibodies, bind to receptors and blocks!
∴ ↓ muscle contraction
As muscles are used, more receptors are blocked!
∴ ↑ ACTIVITY = ↑ WEAKNESS
ALSO : Muscle Specific Kinase (MuSK) and low-density lipoprotein receptor related protein 4 (LRP4)
Ix Myasthenia Gravis
Serology - Anti-AchR and Anti-MuSK
Tensilon/Edrophonium test!
Administer Edrophonium, POS test = ↑↑muscle power for a few seconds
Also, repeatedly blink causes ptosis
Look up for a while causes diplopia
Repeatedly abduct arm causes unilateral muscle weakness when compare arms
Count to 50 - as higher numbers, voice get less audible
ALSO : FVC! to test resp muscles
Tx Myasthenia Gravis
1st Line = ACh inhibitors!
NEOSTIGMINE, PYRIDOSTIGMINE
2nd Line = Immunosuppression - steroids (prednisolone/azathioprine)
Monoclonal Abs - Rituximab, eculizumab
Thymectomy
Myasthenia Gravis assoc disease
Lambert-Eaton Myasthenic Syndrome!
Paraneoplastic condition, SCLC
Weakness will improve AFTER exercise
RF Myasthenia Gravis
Other than F 20-40
Thymoma! / Thymic hyperplasia
Autoimmune disease - RA, SLE, pernicious anaemia
Tx Myasthenia Crisis
IV Ig
Plasmapheresis
Pathophysiology Lambert-Eaton Syndrome
Autoantibodies against calcium channels in SCLC cells
Also target calcium channels in presynaptic terminals
∴ ↓ ACh released
Signs / Symptoms Lambert-Eaton Syndrome
Same as Myasthenia Gravis
But start w extremities rather than head and neck
Ix Lambert-Eaton Syndrome
Rule Myasthenia Gravis out !! (aka if ACh-R antibodies = Myasthenia Gravis)
MRI - SCLC
Tx Lambert-Eaton Syndrome
Treat any malignancy
ACh inhibitors - pyridostigmine, neostigmine
Amifampridine - improves muscle strength
If v severe - immunosuppression/IV Ig/Plasmapheresis
Pathophysiology Parkinson’s disease
SIMPLE
Progressive reduction of dopamine in the basal ganglia
Pathophysiology Parkinson’s disease
MORE DETAIL
Neurodegen loss of substantia nigra in pars compacta
∴ ↓ Dopamine levels
∴ Thalamus is inhibited
∴ ↓ Movement
∴ Symptoms of Parkinson’s
Triad of Parkinson’s
- Resting tremor
- Bradykinesia
- Rigidity
Parkinson’s : TRAP
Tremor - pin-rolling
Rigidity - cogwheel
Akinesia
Postural instability
Causes ParkinsonISM
Parkinson’s disease
Vascular parkinsonism
Infections - encephalitis, creutxfeldt-Jakob disease
Toxin induced - carbon monoxide, drugs
Why is Parkinson’s thought to double in the next gen?
People are getting older
Population is increasing
Parts of basal ganglia
Striatum - putamen and caudate nucleus
Globus pallidus - external and internal
Substantia nigra - produces dopamine
Subthalamic nucleus
Signs / Symptoms Parkinson’s disease
TRAP - ASYMM tremor, rigidity, akinesia, postural instability
Small shuffling steps
Reduced arm swing
Difficulty initiating movement - struggle to start walking
Hypomimia - ↓ facial expressions, mask-like face
Depression
Memory problems
Anosmia
Constipation
↑ Urinary freq, NOT incontinence
What should NOT be present at the beginning of Parkinson’s disease?
If these symptoms ARE present, what are you thinking of instead?
TIDES
Tremor in action
Incontinence
Dementia
Early falls
Symmetry
–
Normal pressure hydrocephalus
Essential tremor
Signs / Symptoms Essential tremor
Better with alcohol
Action tremor
Tx Essential tremor
Deep brain stimulation
BB
Primidone
Tx Normal Pressure hydrocephalus
Surgery
Ix Parkinson’s disease
Usually from history and exams
DaTSCAN
Head CT/MRI
Microscopy - Lewy bodies
RF Parkinson’s disease
FHx
Male
↑ Age
Smoking seems to be protective!
Parkinsonism Cardinal signs
Resting tremor
Bradykinesia
Rigidity
Postural instability
Tx Parkinson’s disease
LEVODOPA + Carbidopa
Can cross BBB, dopamine can’t
NOT GIVEN INITIALLY bc effect will wear off after a while ∴ saved for when symptoms are rlly bad
Has on/off fluctuations!! - Sx can be well controlled, then suddenly not controlled
–
Dopamine agonists - Bromocriptine, Ropinirole
COMT inhibitors - entacapone, rasagiline
MAO-B inhibitors - selegiline
Why can’t you just give dopamine to treat Parkinson’s disease?
silly
you can’t do that bc dopamine can’t cross the BBB
and will cause arrhythmias etc
Why MUST you give Carbidopa with Levodopa when treating Parkinson’s disease?
Carbidopa ensures Levodopa is not metabolised peripherally, otherwise can cause arrhythmias
Parkinson Sx THEN dementia = ?
Parkinson’s dementia
Dementia THEN Parkinson’s Sx = ?
Lewy Body dementia w Parkinsonism
Mechanism of COMT/MAO-B inhibitors
Inhibit the inhibition of dopamine
DDx TIA
Obvs stroke
Hypoglycaemia
Migrainous aura
Mass lesions
Causes Ischaemic Stroke
Atherosclerotic rupture
Thrombus, embolus
Shock
Cardiac emboli - AF, MI, IE (blood stasis)
Atherothromboembolism - from carotid artery
Vasculitis
RF Ischaemic stroke
Atherosclerosis RF
Ethnicity - black or asian
HTN
Smoking
DM
Age
Alcohol
Past TIA
Heart disease
AF
Describe the ABCD^2 score
Age > 60 (1)
BP > 140/90 (1)
Clinical Sx - unilateral weakness (2), slurred speech, no weakness (1)
Duration Sx - 1 hour (2), 1 hour (1)
DMT2 (1)
> 6 REFER TO NEUROLOGY ASAP
Describe Glasgow Come Scale
Signs / Symptoms Ischaemic Stroke
Anterior cerebral artery
D CLIT
Drowsiness
Contralateral leg weakness +/- sensory loss (arms probs not affected too but could happen)
Logical thinking and personality affected
Incontinence
Truncal/Gait ataxia
Signs / Symptoms Ischaemic Stroke
Middle cerebral artery
MOST COMMON PRESENTATION!!
CASH
Contralateral motor weakness/sensory loss - both arms and legs
Aphasia - Wernicke’s or Broca’s
Smile droop (facial droop)
Hemiplagia
Signs / Symptoms Ischaemic Stroke
Posterior cerebral artery - Occipital lobe
VIP
Visual agnosia - can’t interpret visual info
Isolated homonymous hemianopia or cortical blindness
Propagnosia - can’t recog faces
Signs / Symptoms Ischaemic Stroke
Posterior circulation - vertebrobasilar artery
Balance disorder
Coordination disorder
If the posterior circulation (Vertebrobasilar artery) is affected in ischaemic stroke, why is this more concerning?
Larger region supplied by this artery
∴ More catastrophic effects
Signs / Symptoms Lateral Medullary Syndrome
Sudden vomiting
Vertigo
Ipsilateral Horner’s syndrome
Facial numbness
Limb ataxia
Dysphagia
Dysarthria
Tx Ischaemic Stroke
Urgent NCCT!!!!! EXCLUDE HAEMORRHAGE
Immediate 300mg loading dose Aspirin
Then continue this for 2 weeks
Thrombolysis w IV Alteplase - WITHIN 4.5 HOURS OF SX ONSET !!!!
Lifelong Clopidogrel after 2 weeks of aspirin (75mg)
–
Thrombectomy - within 6 hours of symptom onset!!
Only indicated if severe - large artery has been affected
CI Thrombolysis w IV alteplase
History of stroke in DM patients
Severe stroke
Stroke in last 3 months
Active malignancy
A patient on anticoags has a stroke and presents to the hospital. Thoughts?
if Px on anticoags, always suspect to be haemorrhagic stroke until proven otherwise
Causes Haemorrhagic strokes
Anything that increases risk of vessel rupture
HTN
2º to ischaemic stroke! - causes stiff, brittle vessels prone to rupture
Head trauma
AV malformations
Vasculitis
Vascular/Brain tumours
Cerebral amyloid angiopathy
Carotid artery dissection
Presentation of ↑ICP
Virtually indistinguishable from Ischaemic infarct
LoC and headache more likely
Papilloedema
Extradural haemorrhage
Rupture of?
Middle meningeal artery
Subdural haemorrhage
Rupture of?
Bridging cranial veins
Subarachnoid haemorrhage
Rupture of?
Berry aneurysm in circle of Willis
go do haemmorhages from table
Causes Dementia
Alzheimer’s - MC!
Vascular
Lewy-Body
Fronto-Temporal (Pick’s)
QUICK!
Headache, photophobia and neck stiffness
What is it?
MENINGITIS!
What must you make sure you do if a patient has meningitis?
Notify Public Health!!
RF Meningitis
Elderly or very young children
Immunocompromised
Non-vaccinated
Crowded environments
Meningism Triad
- Headaches
- Photophobia
- Neck stiffness
Non-Infective Causes Meningitis
Paraneoplastic
Drug S/E
Autoimmune e.g. RA, SLE
Causes Bacterial Meningitis
Neisseria Meningitis
Strep. Pneumoniae
Listeria spp
Group B strep
Haemophilus influenzae B
E. Coli
Causes Viral Meningitis
Herpes Simplex Virus
Varicella zoster virus
Enterovirus - Coxsackie
Causes Meningitis
Chronic presentation
Mycobacterium Tuberculosis
Syphilis
If Neisseria Meningitidis is found in the bloodstream, what does it indicate?
MENINGOCOCCAL SEPTICAEMIA !!
How does Meningococcal Septicaemia present?
Non-Blanching Purpuric Rash
Disseminated Intravascular clotting - v easy bleeding
How does ↑ICP present?
Papilloedema
GCS < 12
Focal neuro signs - weakness, paralysis, headaches, ataxia, visual impairment etc
Continuous or uncontrolled seizures
Go through bacterial causes of Meningitis and in what groups you are likely to find them in
Neisseria Meningitidis - Kids/Teenagers
Step Pneumoniae - Young children OR adults
Listeria spp - Immunocomp, pregnancy
Group B Strep - neonates
bc colonises maternal vagina
Shape and gram stain
Neisseria Meningitidis
Gram Neg
Diplococcus
Shape and gram stain
Strep Pneumoniae
Gram Pos
Diplococcus
Shape and gram stain
Group B Strep
Gram Pos
Coccus in chains
Shape and gram stain
Listeria monocytogenes
Gram Pos
Bacillus
Signs / Symptoms Meningitis
HEADACHE, NECK STIFFNESS, PHOTOPHOBIA
Fever
Kernig’s sign
Brudzinski’s sign
If patient presents to GP with Meningism, what should they do immediately?
Give IM Benzylpenicillin + admit to hospital!!!
Where can Listeria monocytogenes be found?
CHEESE
∴ pregnant women advised to try and avoid
Describe the steps taken once a patient arrives at hospital with suspected meningitis
- ABCDE + GCS score
- Blood cultures!
- Broad spec Abx (don’t wait for results) - IV ceftriaxone or Cefotaxime
If immunocomp - ADD high dose amoxicillin
If recent travel - ADD IV Vancomycin
If pencillin allergy (severe - anaphylaxis) - Give Chloramphenicol - Steroids - IV Dexamethasone (↓Neuro comps)
- LUMBAR PUNCTURE!
When treating Meningitis with broad spec Abc (aka with cephalosporins), why would you give Chloramphenicol if a patient has a severe penicillin allergy?
BC there is a 10% chance that if they react to penicillins, they will react to cephalosporins
Why do you give IV Vancomycin if recent travel when treating Meningitis?
Bc recent travel increases chance of penicillin resistance
CI Lumbar Puncture
Abnormal clotting
If on anti-coags
Petechial rash (Indicates meningococcal sepsis - DIC)
Infection at LP site (e.g. shingles)
↑ ICP - bc risk of coning
Ix Meningitis
LP TABLE !!!!!
What should you do after treating a meningitis patient?
Inform Public health
And identify close contacts! (Living together esp in first 7 days of infection!)
Give prophylaxis Abx - Ciprofloxacin or Rifampicin if CI !!
DDx Meningitis
SAH! - thunderclap
Migraine
Flu
Sinusitis - facial pain
Brain abscess
Malaria
Big diff between syncope and epilepsy
Epilepsy usually has amnesia - forget what happened straight after episode
Causes Encephalitis
HERPES SIMPLEX (MC by far!!)
Varicella zoster (chicken pox)
Measles, Mumps
Rubella
EBV
HIV
CMV
Coxsackie
ALSO : TB, Malaria, Rabies
Non-Infective : Autoimmune, paraneoplastic
Presentation of Encephalitis
Hours to Days Preceding : Flu-like illness
THEN, triad of :
1. Altered GCS - confusion, drowsiness, coma
2. Fever
3. Headache
Also : Seizures, Memory loss +/- history of meningism (may have progressed from meningitis)
Ix Encephalitis
MRI head - shows swelling/inflamm + midline shift (bc ↑ICP)
ECG - periodic sharp and slow waves
Lumbar puncture - done after, ↑lymphocytes
HIV test
Tx Encephalitis
Mostly supportive
IV Aciclovir - if HSV or VZV
If seizures - carbamazepine
If meningitis sus - IM benzylpenicillin
Triggers of a Migraine
CHOCOLATE
Chocolate
Hangovers
Orgasms
Cheese
Oral contraception
Lie-ins
Alcohol
Tumult - loud noises
Exercise
Describe a migraine
Unilateral, recurrent throbbing headache +/- aura
Mod - Severe pain
Often w vision changes
Usually bc of specific trigger
MC cause of recurrent headache
Describe a typical patient of migraine
Women under 40
If over 50, should be sus of underlying cause
Describe the difference between 1º and 2ºheadaches
1º - syndrome is based on Sx
2º - symptoms bc of underlying cause e.g. SAH
Describe the diagnostic criteria for migraines WITHOUT aura
A - 5 attacks of
B - 4 - 72 hours
C - 2 of :
Unilateral, pulsing, mod/severe, aggravated by routine physical activity
D - Headache comes w/ N+V OR Photophobia + Phonophobia
Describe the diagnostic criteria for migraines WITH aura
A - At least 2 attacks fulfilling B + C :
B - At LEAST 1 reversible aura Sx :
Visual - zigzags, spots
Unilateral sensory - tingling, numbness
Speech - aphasia
Motor weakness - hemiplegic migraine (rule out stroke/TIA)
C - AT LEAST 2 of following :
* ≥ 1 aura Sx spread gradually over 5 mins +/- ≥ 2 aura Sx in succession
* Each aura Sx = 5 - 60 mins
* ≥ 1 aura Sx is unilateral
* Aura accompanied/followed within 1hr of headache
Ix Migraine
Clinical diagnosis
Diagnostic criteria w/ normal neuro exam!
Exclude other causes e.g. SAH
If a patient presents with a migraine, what are the indications for a lumbar puncture?
Worst headache of life - thunderclap
Rapid onset
Progressive headaches
Unresponsive headaches
Tx Migraines
Conservative - avoid triggers
ACUTE :
Mild - NSAIDs, paracetamol
Severe - oral triptan e.g. sumatriptan
Avoid opioids if possible! bc risk of dependence and worsens nausea
Prophylaxis Migraines
1st Line - propanolol or topiramate (anti-convulsant)
2nd Line - acupuncture
3rd Line - Amitriptyline
4th Line - Botulinum toxin Type A
What’s the most common headache?
Tension headache
Trigger Tension headache
Stress !!
Describe a tension headache
Bilateral generalised headache, radiates to neck / trapezius
Like a rubber band / clamp around head
NO motion sickness / N+V / aura
Tx Tension headache
SImple analgesia - aspirin, paracetamol
Avoid opioids! (dependence!!)
Causes Tension Headache
MC SCOLD
Missed meals
Conflict
Stress
Clenched jaw
Overexertion
Lack of sleep
Depression
Which is the most disabling headache?
Cluster
Describe a cluster headache
Unilateral periorbital pain w/ autonomic features (15 - 160 mins)
Rises in severity
Excruciating pain - hot/boring poker characteristic
Usually in middle of night/morning hours
Rare-ish
Describe some autonomic features found in a cluster headache
Conjunctival infection + lacrimation (watery bloodshot eyes)
Ptosis
Miosis (dilated unilateral pupil)
Rhinorrhoea
Ix Cluster headaches
At least 5 similar attacks confirms diagnosis
Tx Cluster headaches
Acute :
SC sumatriptan
IM Zolmitriptan
100% O2 therapy! (unless COPD)
Prophylaxis Cluster headaches
Verapamil
Lithium
Corticosteroids
Describe Trigeminal Neuralgia
Chronic, debilitating condition
Causes intense and extreme episodes of pain
Almost always unilateral
Age of Trigeminal Neuralgia normally
50 - 60 years
RF Trigeminal Neuralgia
MS!!!!!!!!!
↑ Age
Female
Triggers Trigeminal Neuralgia
Eating
Shaving
Talking
Brushing teeth
Presentation Trigeminal Neuralgia
Electric shock pain (seconds-mins)
Diagnostic criteria Trigeminal Neuralgia
At least 3 attacks
Tx Trigeminal Neuralgia
Carbamazepine
Surgery if nothing else works
Name types of Generalised seizures
Tonic-Clonic
Myoclonic
Absence
Tonic
Atonic
Define Epilepsy
Recurrent tendency to have spontaneous, intermittent, abnormal electrical activity that manifests as seizures
**AT LEAST 2 SEIZURES TO BE EPILEPTIC **
Define an Epileptic Seizure
Spontaneous, intermittent, uncontrolled electrical brain activity
What is Ictus?
The epilepsy attack
What is Prodrome?
Non-specific symptoms that precede an epileptic attack
What is Aura?
Sensory disturbances that precede an epileptic attack, usually by minutes
More specific than Prodrome
What age group is Epilepsy common?
Can happen at age
Highest < 20 and > 60
RF Epilepsy
FHx
Premature birth
Developmental abnormalities
Trauma
Drugs e.g. cocaine
Space occupying lesion - tumour, infection, haematoma
Alzheimer’s /Dementia
Types of Epileptic Seizures
Briefly describe
-
Primary Generalised
Originates in midbrain/brainstem
Electrical discharge in both hemispheres
Assoc w/ LOC -
Partial/Focal - MC
Electrical discharge restricted to one hemisphere
May develop into generalised (2º)
Types of Partial/Focal Seizures
Partial WITHOUT impairment of conciousness e.g. déjà vu, Jacksonian seizures
Partial WITH impairment of consciousness e.g. psychomotor seizures
Describe how a Tonic-Clonic seizure presents
Often no aura
Tonic : stiffening of the limbs, tongue biting, incontinence, no breathing (10-60s)
Clonic : Convulsions, limb jerking, eye rolling, uncoordinated breathing (secs-mins)
Potential physical injuries of a Tonic-Clonic seizure
Common!
Drowsy
Confusion
Headache
Describe the presentation of Absence seizures
Childhood
Conscious but unresponsive
Can occur many times a day
Usually very short, normal function resolves quickly
Describe a Myoclonic seizure
Sudden isolated jerk of limb, face or trunk
May fall suddenly to the ground
Describe a Tonic seizure
Sudden increased tone, RIGID
No jerking!
Describe an Atonic seizure
Sudden loss of muscle tone + movement
RESULTS IN A FALL
Describe a Simple Partial/Focal seizure
No LOC or loss of memory
Isolated limb jerking
Head turning! (away from side of seizure)
Isolated paraesthesia
TODD’s PARALYSIS - temporary paralysis/weakness
Describe a Complex Partial/Focal seizure
Usually from temporal lobe
Can affect awareness +/- memory - before, during or after!
Visual/auditory halluciations
LIP SMACKING
Automatism
Post-ictal confusion/drowsiness
Sx depend on lobe involved!
Describe a 2º Generalised seixure
Partial seizure that spread to lower brain area
∴ Initial a generalised seizure
usually tonic-clonic
What is Status Epilepticus?
Seizure lasting longer than 5 minutes or more than 1 within 5 minutes without returning to normal consciousness in between
Types of Status Epilepticus
Brief description
Convulsive :
Jerking, grunting, drooling, nystagmus
Non-Convulsive :
NO jerking, confusion, unable to speak, behavioural changes
Tx Status Epilepticus
IV benzodiazepine - Lorazepam, diazepam
Rectal benzodiazepine - Buccal midazolam
Phenobarbital - 2nd Line
Phenytoin - 3rd Line
Comp of Phenobarbital
Can cause circulatory depression
Comp of Phenytoin
Can cause severe cardiac arrythmia
Ix Epilepsy
Clinical diagnosis - AT LEAST 2 or more unprovoked seizures > 24 hours apart !!
EEG - Not diagnostic
Can support diagnosis but might show false negatives
MRI/CT - can show focal lesions to identify cause
Bloods - FBC, U&E, LFTs, BM
(to look for potential cause)
Tx Generalised Tonic-Clonic
Sodium Valproate
UNLESS female of childbearing age - Lamotrogine
Tx Tonic
Sodium Valproate
UNLESS female of childbearing age - Lamotrogine
Tx Atonic
Sodium Valproate
UNLESS female of childbearing age - Lamotrogine
Tx Myoclonic
Sodium Valproate
UNLESS female of childbearing age - Levetiracetam or Topiramate
Tx Absence
- Ethosuximide
- Sodium Valproate
UNLESS female of childbearing age - Ethosuximide
Tx Partial/Focal Seizures
Lamotrigine or Carbamazepine
Types of Motor Neuron Disease
Brief description of area affected
1. Amyotrophic Lateral Sclerosis (ALS)
Loss of MN in motor cortex + ant. horn
UMN + LMN SIGNS
2. Progressive Bulbar Palsy
Affects brainstem motor nuclei, CN nerves 9-12 !! UMN + LMN
3. Progressive Muscular Atrophy
LMN only!
Distal muscles before proximal
4. Primary Lateral Sclerosis
UMN signs only!
Types of MND and how they present ish
1. ALS
Progressive focal wasting, weakness and fasciculations spreading to other limbs!
Cramps
Spasticity
Brisk reflexes
2. Progressive Bulbar Palsy
CN 9-12 affected ∴ PALSY of the tongue, chewing/swallowing and facial muscles
3. Progressive Muscular Atrophy
Weakness + Fasciulations starts in one limb, progresses to adjacent spinal segments
Distal before proximal!!
LMN only!
4. Primary Lateral Sclerosis
Slow progressive tetraparesis and pseudobulbar palsy
UMN only!
UMN lesion signs
↑Muscle tone = spasticity
Hyperreflexia (overrative/overresponsive reflexes)
NO Fasciculations!
++POS BABINSKI SIGN - Extensor plantar response
Pyramidal/Pronator drift
CORTICAL SENSORY LOSS
Maybe emotional lability
Upper limb extensor weaker than flexor!
Lower limb flexors weaker than extensor!
Fine movements impaired
LMN lesion signs
Everything goes DOWN
↓Muscle tone (Hypotonia) - Flaccid (can be normal)
HYPOreflexia (reduction or absence of reflex)
FASCICULATIONS !
Muscle wasting! Can be Bulbar (speech/swallowing muscles), Upper limb (hand), Lower limb
RESPIRATORY! - weakness of breathing muscles, most patients will die bc of this
Diaphragm higher up!!
What is a Positive Babinski sign?
When you stroke sole of foot, normally big toe will go down
If positive = will go up = UMN lesion
Describe Pyramidal/Pronator drift
Hold arms out w palms up, eyes closed
Arm will “drift” away (or turn so palm down? idk need to check this)
What can lower limb wasting look like?
Esp with LMN lesions
Wasting of tibialis anterior muscle = BILATERAL FOOT DROP
& Prominent tibial bones
DDx MND
Cervical spine lesion - can present with UMN + LMN in arms and legs
Idiopathic multifocal motor neuropathy - weakness in hands and profuse fasciculations
Ix MND
MRI of brain/spine
Nerve conduction studies
Electromyography - can confirm LMN
By itself, are fasciculations enough to diagnose LMN lesions?
NO
NEED WEAKNESS TOO!!!!!
Median age on onset MND?
60 years
Prognosis MND
Usually fatal within 2-4 years
RF MND
Usually sporadic, no known RF
MIGHT be linked to a mutation in SOD-1 (scavenging enzyme superoxide)
Diagnostic tips for differentating DDx and MND!
- No sensory loss = RULE OUT MS or MYELOPATHY
- No disturbances in eye movements = RULE OUT MYASTHENIA GRAVIA OR MS
- No sphincter disturbances = RULE OUT MS
Tx MND
NO CURE!
Oral Riluzole
Palliative :(
Symptom Tx for MND
NG/PEG tube - Dysphagia
Oral amitriptyline - Drooling (bulbar palsy)
Baclofen - spasticity
Analgesic ladder - Joint pain
MOA Riluzole
Na+ channel blocker
Inhibits Glutamate release
Big difference in Bell’s Palsy vs Stroke
Bell’s = Lesion in CN 7
NOT FOREHEAD SPARING - forehead and lip droop
Stroke = Central lesion
FOREHEAD SPARING - ONLY lip droop
Causes Peripheral Neuropathy
ABCDE
Alcohol
B12 deficiency
Cancer and CKD
DM and Drugs - isoniazid, amiodarone, cisplatin
Every vasculitis
Signs / Symptoms Peripheral Neuropathy
2 Patterns - Axonal Loss and Demyelination
- Axonal loss
Sensory changes - symmetrical numbness, burning or pins and needles
THEN progresses to motor symptoms - Demyelination
Motor changes - Weakness
THEN progresses to motor symptoms
–
Slow healing
Sensitivity to touch
Persistent injury
Lack of sensation
Burning pain
Ulcers
Ix Peripheral Neuropathy
Nerve conduction studies
Rule out DDx
CN 1
What is it?
How does CN1 Palsy present?
Olfactory
Anosmia - Can’t smell
CN 2
What is it?
How does CN2 Palsy present?
Optic
Visual defect - depends on location of lesion!
LEARN
CN 3
What is it?
How does CN3 Palsy present?
Occulomotor
Ptosis - drooping eyelid
Fixed dilated pupil
Eye down and out
What might cause a CN3 palsy?
↑ICP
DM
HTN
Giant cell arteritis
CN 4
What is it?
How does CN4 Palsy present?
Trochlea
Diplopia when looking down (e.g. walking downstairs)
CN 5
What is it?
How does CN5 Palsy present?
Trigeminal
Jaw deviates to side of lesion
Loss of corneal relex
CN 7
What is it?
How does CN7 Palsy present?
Facial
Facial droop and weakness (BELL’S)
Dribbling at side of mouth
CN 8
What is it?
How does CN8 Palsy present?
Vestibulocochlear
Hearing impairment, Vertigo, Lack of balance
Causes of CN8 Palsy?
Local tumours - if tumour in internal acoustic meatus, will press on CN7+8
Skull fracture - CN8 close to bone
Ear infections
CN 9 + 10
What is it?
How does CN 9 + 10 Palsy present?
Glossopharyngeal + Vagus
Gag reflex issues
Dysphagia - Uvula deviates AWAY from side of lesion
Vocal issues
Which might cause a CN 9 +/- 10 lesion?
Jugular foramen lesion
CN 11
What is it?
How does CN11 Palsy present?
Accessory
Sternocleidomastoid and Trapezius palsy
Can’t shrug shoulders or shake head
CN 12
What is it?
How does CN12 Palsy present?
Hypoglossal
Tongue deviates towards side of lesion
Types of Dementia
Alzheimer’s - MC !! (50%)
Vascular
Lewy-Body
Fronto-Temporal
Can be mixed - more than one cause
Epilepsy
What must we do once a patient has been diagnosed?
Must inform DVLA!!!
Can’t drive until seizure free for one year!!
What’s another name for Fronto-Temporal Dementia?
Pick’s
RF Alzheimer’s
↑ Age - usually develops after 60
FHx
Mutations in amyloid precursor protein
Apoprotein E allele E4 - encodes a cholesterol transport protein
Caucasian
Down’s
Pathophysiology Alzheimer’s
Beta amyloid plaques and neurofibrillary tangles accumulate causing neuronal death
Also, loss of ACh
Signs / Symptoms Alzheimer’s
4 A’s
Amnesia - recent mems lost first
Aphasia - speech muddled/disjointed
Agnosia - recog problems
Apraxia - can’t carry out fine/skilled tasks despite normal motor function
–
gradual onset!
Ix Alzheimer’s
MRI !!
Degen of cerebral cortex w/ Cortical atrophy
Tx Alzheimer’s
Acetylcholine inhibitor e.g. Donepezil, Rivastigmine
Memantine
Social support
Onset of Alzheimer’s
Gradual
What is Fronto-Temporal Dementia?
Atrophy to frontal and temporal lobes!
Presentation Fronto-Temporal Dementia
Onset BEFORE 65
Memory + Visuospatial skills are preserved
Personality changes and social conduct problems !!
Onset of Fronto-Temporal Dementia
Insidious onset
Rapid progress
Tx Fronto-Temporal Dementia
Supportive
What is Vascular dementia?
Brain damage from a cerebroVASCULAR disease
RF Vascular Dementia
Hx of Stroke/TIA
HTN
DM
Smoking
Obesity
Coronary hear disease
FHx of Stroke
Signs / Symptoms Vascular Dementia
STEPWISE DETERIORATION OF COGNITION - w short periods of stability
Attention deficits
Loss of exec function (kinda like organising, scheduling, memory - stuff for work)
Ix Vascular Dementia
MRI - infarcts
Tx Vascular Dementia
↓ RF
Pathophysiology Lewy-Body Dementia
ALPHA-SYNUCLEIN AGGREGATES
form Lewy body deposits within neurons
Found in substantia nigra and cortex!!
Signs / Symptoms Lewy-Body Dementia
After onset of dementia, PARKINSONISM follows ! :(
Cog fluctuations
Visuospatial abilities impaired
REM sleep affected
Visual hallucinations
↑↑Exec dysfunction!
Ix Lewy-Body Dementia
Usually clinical diagnosis
Onset of Vascular Dementia
Abrupt OR Gradual
Onset of Lewy-Body Dementia
Insiduous
Tx Lewy-Body Dementia
Acetylcholinesterase inhibitors - rivastigmine, donepexil
Memantine
Typical age of onset Lewy-Body Dementia
50+
Vascular Dementia typical age of onset
65+
What is Huntingtons Disease?
Autosomal dominant neurodegen movement disorder
FULL PENETRANCE - all gene carriers WILL get the disease
Pathophysiology Huntington’s
↑ CAG repeats !
< 28 = normal
28 - 35 - high risk
> 36 = DIAGNOSTIC !
–
CAG repeats form faulty protein
This protein builds up in striatum
∴ cell death + loss of cholinergic + GABA-nergic neurons
∴ ↓ACH and ↓ GABA synthesis in striatum
↓ GABA = less regulation of dopamine
∴ XS dopamine
∴ chorea
What is Chorea?
Continuous flow of involuntary, jerky, semi-purposeful movements
Cease during sleep
Huntingtons - If early onset i.e. from childhood, how many CAG repeats would we expect to see?
60+
Define Anticipation in terms of Huntington’s
More repeats each generation
If ONE parent has Huntington’s, what’s the chance of the child getting it?
50%
ONLY IF THEY HAVE THE GENE THEY WILL GET IT
Presentation Huntington’s
1st Phase
Depression
Incoordination
Personality change
2nd Phase
Chorea
Abnormal eye movement
Loss of coord
Dysarthria
Dementia
Depression
Rigidity
Ix Huntington’s
PCR testing - shows CAG repeats
MRI/CT - shows atrophy of striatum (caudate and putamen)
Tx Huntington’s
No cure.
Symptoms management =
FOR CHOREA - Antipsychotics e.g. Risperidone or Tetrabenazine
FOR DEPRESSION - SSRI e.g. Sertraline
FOR AGGRESSIVE BEHAVIOUR - Antipsychotics e.g. Risperidone
Most common death of Huntington’s :(
Suicide :((( rlly rlly sad :(
Aspiration pneumonia also common cause
MOA Risperidone
Dopamine receptor antagonists
MOA Tetrabenazine
Depletes dopamine