Neurological Conditions Flashcards
Define Epilepsy
Chronic neurological condition characterised by recurrent seizures
Define Seizure
Transient neurological change due to synchronous hyperexcited neuronal activity
Give 5 causes of unprovoked seizures
Genetic
Structural (eg congenital malformation)
Metabolic
Immune
Infection (HIV)
Describe the pathophysiology of Epilepsy
Due to imbalance of excitatory and inhibitory signals in the brain
High frequency excitable Action Potentials lead to synchronous hyperexcitable activity and propagation to other neurones
Transformed long lasting structural/biochemical changes
Name three risk factors for Epilepsy
Cerebral infections
Family history
Prematurity
How can you classify seizure type?
Area of onset
Awareness
Clinical Features
Describe the subtypes of ‘area of onset’ in epilepsy
Focal (networks of neurones in one hemisphere)
Generalised (affecting both hemispheres)
Focal to Bilateral (Secondary Generalised Seizure)
Describe the subtypes of ‘awareness’ in epilepsy
Fully aware
Impaired Awareness
Only relevant for Partial
Describe the subtypes of ‘Clinical Features’ in epilepsy
Motor (Tonic, Clonic, Myoclonic, Atonic, Spasms)
Non Motor (any sensory/cognitive/emotional dysfunction or generalised absence)
Name four epilepsy classifications
Focal
Generalised
Generalised and Focal
Unknown
Name two Epilepsy Syndromes
West Syndrome
Lennox Gastout Syndrome
What are the four periods of different clinical features in Epilepsy?
Prodromal
Early Ictal (ie Aura - normally seen in general)
Ictal
Post Ictal
How is Epilepsy diagnosed?
One of:
-More than two unprovoked seizures occurring more than 24h apart
- 1 unprovoked seizure with probability of further
- Diagnosed epilepsy syndrome
Give three differentials for Epilepsy
Anoxic Seizures
Sleep Associated
Migraine Associated
How is an EEG used in suspected Epilepsy?
To support diagnosis, assess recurrence risk and determine seizure type
When is an MRI required in Epilepsy?
<2 years old
Refractory to AEDs
Focal
Name four education/safety points in terms of Epilepsy
Avoid taking baths
Avoid operating heavy machinery
Always use contraception while on AEDs
Inform DVLA
What are the DVLAs rules on driving after seizures?
First seizure - 6m
Epileptic Seizure - 12m
No LOC - ask DVLA
First Seizure + Lorry - 5y
Epileptic Seizure + Lorry - 10y
What is done at the ‘First Fit Clinic’
Formal Assessment and relevant EEG/MRI
If diagnosis is made and risk of another is felt to be high - AED started
Name three SE of Sodium Valproate
Teratogenic
DILI
Pancreatitis
Name two SE of Lamotrigine
Slightly teratogenic
Skin reactions (life threatening)
Name two SE of Levetiracetam
CNS and Neuropsych disturbance
Name three SE of Phenytoin
Arrhythmia
Teratogenic
Gum Hypertrophy
What are the AEDs of choice for Focal Seizures?
1) Lamotrigine
2) Sodium Valproate
What are the AEDs of choice for General Seizures?
Sodium Valproate or Lamotrigine
What are the AEDs of choice for Absence Seizures?
Ethosuxamide or Sodium Valproate
What are the AEDs of choice for Myoclonic Seizures?
Sodium Valproate
Define Migraine
A primary headache characterised by recurrent moderate to severe headaches and associated with nausea/vomiting/photophobia/phonophobia
Migraines can be episodic or chronic. How does a patient qualify for ‘Chronic Migraines’?
Headaches >15d a month, at least 8 of which have migraine features
Describe the diagnostic criteria for Migraines without Aura
Atleast 5 fulfilling:
-Headaches lasting 4-72 hours
-Atleast 2 of - unilateral/pulsating/mod to severe/aggravated by physical activity
-Not accounted for
Describe the diagnostic criteria for Migraines with Aura
Atleast two attacks fulfilling:
-One or more reversible aura (scintillating scotoma most commonly)
- Atleast 3 of - one>5 mins/two or more in succession/one unilateral/one positive/one followed by a headache within 60 mins
Migraines without Aura are commonly associated with the Menstrual Cycle. If first line Migraine management fails in these women, what can be used?
Frovatriptan
Name 5 Migraine Red Flags
Motor Weakness
Double Vision
Aura without headache
Severe Sudden Onset
Worse on Standing (CSF leak)
How is an Acute Migraine managed?
Simple Analgesia (eg rectal diclofenac) and Antiemetic (buccal prochlorperazine)
Severe - Sumatriptans (5HT1 agonist)
Come off COCP
How can Migraines be prevented?
Propanolol
Topirimate
Amitryptyline
How can inretractable Migraines be prevented?
I.E more than 3 medications tried
Galcenezumab
Botox
Name three complications of Migraines
Status Migrainosus (>72h)
Migrainal Infarction
Ischaemic Stroke
How can Tension Headaches be classified?
Infrequent Episodic - 10 episodes occuring on <12d per year
Frequent - 1 to 14d a month for >3m
Chronic - >15d a month for >3m
How are chronic Tension Headaches managed?
Accupuncture
Low dose Amitryptyline
Lifestyle
Define Trigeminal Neuralgia
Episodes of acute severe facial pain in the distribution of the trigeminal nerve (commonly affecting maxillary and mandibular)
Trigeminal Neuralgia can be primary or secondary. Describe the Primary pathophysiology
Vascular compression at the nerve root entry zone leading to demyelination and abnormal electrical activity
Trigeminal Neuralgia can be primary or secondary. Describe the Secondary pathophysiology
Disease occuring due to another condition
eg Vestibular Shwannoma, Meningioma, Cysts, MS
Describe the diagnostic criteria for Trigeminal Neuralgia
Pain lasting up to two minutes of severe electric shock like intensity
Precipitated by innocuous stimuli within the region (eg cold)
Not accounted for by alternate diagnosis
Describe the medical management options for Trigeminal Neuralgia
Carbemazepine (titrate up to remission and then back down to lowest possible maintenance)
Second Line - Gabapentin, Lamotrigine
Describe the surgical management for Trigeminal Neuralgia
Microvascular Decompression
Gamma Knife Radiosurgery
Cluster Headaches can be chronic or episodic, what is the difference?
Episodic - in periods lasting between 7d and 1y with pain free episodes in between
Chronic - Occurring for one year without/short lived remissions
What hormone do Cluster Headaches have a link to?
Melatonin
Describe the nature of Cluster Headaches
Rapid Onset over 10 minutes, often at night, excruciating around one eye lasting up to 90 mins
Associated lacrimation and rhinorrhoea
Name three triggers for Cluster Headaches
Alcohol
Histamine
Disrupted Sleep
Describe the diagnostic criteria for Cluster Headaches
5 attacks qualifying:
-Severe unilateral pain lasting 15-180 minutes untreated
-Accompanied by lacrimation/congestion/rhinorrhoea
- Attacks occurring from one every other day to 8 times a day
How can an Acute Cluster Headache be treated?
Sumatriptans and 100% O2
Could use Indometacin/Lidocaine
How can a Cluster Headache be prevented?
Stop Smoking, Stop Alcohol, Maintain good sleep hygiene
Verapamil, Prednisolone, Lithium
Define Parkinson’s
Chronic progressive neurodegenerative condition occurring secondary to loss of dopaminergic neurones in substantia nigra
Define Parkinsonism
Bradykinesia plus one of resting tremor/rigidity/postural instability
Name three roles of the Basal Ganglia
Inhibit muscle tone
Initiation of movement
Suppression of useless movement
Describe the direct Basal Ganglia pathway
Stimulatory pathway associated with D1 receptors
Activation leads to neural connections and movement initiation (via dopamine from substantia nigra)
Describe the indirect Basal Ganglia pathway
Inhibitory pathway associated with D2 receptors. Activation leads to inhibition of muscular tone, inhibition of movement initiation and inhibition of direct pathway
How does Bradykinesia of Parkinsons present?
Slowing of voluntary movements
Reduced arm swing
Reduced amplitude with repetitive movements
How does Tremor of Parkinsons present?
At rest
Described as pill rolling
4-6Hz
Can be induced by distraction
How does Rigidity of Parkinsons present?
Increased resistance to passive movement
Cogwheel rigidity due to superimposed tremor
Other than the classic triad, give three other clinical features of Parkinsons
Expressionless face
Micrographia
Shuffling Gait
Name three exclusion criteria for Parkinsons
Cerebellar Abnormalities
Restricted to lower limbs for >3y
Suspected Frontotemporal Dementia
Parkinsonism is an umbrella term. Other than Idiopathic Parkinsons Disease, name three other causes
Parkinson Plus Syndromes
Drug Induced (Antipsychotics, Antiemetics, Lithium)
Other (Post Encephalitis, Trauma)
Parkinson Plus Syndromes affect a wider area of nervous system causing more complex disease and increased survival. Name four causes
Multiple System Atrophy
Progressive Supranuclear Palsy
DLB
Corticobasal Degeneration
How does Multiple System Atrophy present?
Rapidly progressive disease characterised by profound autonomic dysfunction leading to severe postural hypotension and urogenital dysfunction
How does Progressive Supranuclear Palsy present?
Neurodegenerative disorder characterised by vertical gaze dysfunction, dysarthria and cognitive decline
How does Corticobasal Degeneration present?
Progressive dementia, Parkinsonism and Limb Apraxia
Severe - Alien Limb Syndrome
Idiopathic Parkinsons Disease is largely a clinical diagnosis. Name three possible investigations
CT/MRI - if strong suspicion of underlying cause/failed response to treatment
PET with Fluorodopa - can localise dopamine deficiency in Basal Ganglia
DaTscan - differentiates Parkinsons from Essential Tremor
Parkinsons Disease requires early initiation of treatment. What are the three mainstay treatment options?
Levodopa
Ropinorole (Dopamine Agonists)
Rasigiline (MAO-B Inhibitors)
Describe the use of Levodopa in Parkinsons
-Effective, for mostly 5-6y
-Can cross BBB and be converted to Dopamine by Dopa-Decarboxylase
-Some peripheral conversion
-Treats motor symptoms well but also causes dyskinesia/on off fluctuations
Describe the use of Ropinorole in Parkinsons
-Dopamine Agonist
-Less motor effects but can cause problems with impulse control and sedation
Eventually, all Parkinsons patients will require more than monotherapy. Name four adjuvant options
-COMT Inhibitors (Entacapone- given with Levodopa)
-Antimuscarinics (Procyclidine)
-Amantadine
-Apomorphine (Dopamine Agonist for advanced disease)
Another treatment option for Parkinsons is Deep Brain Stimulation. Name two advantages and two disdvantages to this method
Advantages - Reduces rigidity and tremor
Disadvantages - Haemorrhage and Confusion
Name two motor and two non motor complications of Parkinsons Disease
Motor - Freezing of Gait, Falls
Non Motor - Aspiration Pneumonia, Pressure Sores
Name four causes of Neuromuscular Weakness
Guillaine Barre Syndrome
Myasthenia Gravis
Motor Neurone Disease
Muscular Dystrophy
Define Guillaine Barre Syndrome (GBS)
Acute inflammatory polyneuropathy characterised by progressive ascending neuropathy with weakness and reduced reflexes
Often triggered by infections such as Campylobacter
Describe the pathophysiology of GBS
Immune mediated damage to peripheral nerves (proposed antigenic mimicry)
What are the four subtypes of GBS?
Acute Inflammatory Demyelinating Polyneuropathy (90%)
Acute Motor Axonal Neuropathy
Acute Motor Sensory Neuropathy
Miller Fisher Syndrome
How does the AIDP subtype of GBS present?
Progressive symmetrical weakness (often ascending from distal muscles)
Reduced/absent reflexes
Reduced sensation
What is the destructive target in Acute Motor Axonal Neuropathy (GBS)?
The Axons
How does the Miller Fisher subtype of GBS present?
Ataxia
Areflexia
Opthalmoplegia
Name five investigations for suspected GBS
-Viral Screen
-CXR (Rule out Sarcoidosis)
-Electrophysiology (confirms diagnosis and differentiates axonal and demyelinating)
- LP (increased protein and normal WCC)
- AB for Miller Fisher
The management for GBS is normally supportive, if required, what management can be given?
FVC<20ml/kg - ITU and intubation
IVIG/Plasma Exchange
What is Hughes Disability Score?
Severity scoring for GBS
0 - Healthy
4 - Bedridden
5 - Assisted Ventilation
6 - Death
Define Myasthenia Gravis
Antibody mediaed blockage of Neuromuscular Transmission due to ACh receptor AB
Describe the pathophysiology of Myasthenia Gravis
-Type II Hypersensitivity reaction
-Cross links receptors causing destruction and activates –Membrane Attack Complexes to destroy membrane
- Disease fluctuates as NMJ can repair itself and create more receptors easily
What are the three stages of Myasthenia Gravis?
1 - Fluctuating muscle weakness with most severe symptoms happening here
2 - Persistent but stable symptoms
3 - Remission
What is the relation of Myasthenia Gravis to the Thymus?
10-15% have a Thymoma
Up to 85% have Thymic Hyperplasia
There are myoid cells in the thymus which may start the target for autoimmunity
What are the two clinical subtypes of Myasthenia Gravis?
Ocular
Generalised
Name three antibodies associated with Myasthenia Gravis
AChR - Ab
Anti MuSK
Anti - LRP4
Name four ocular features of Myasthenia Gravis
Diplopia (improved on occluding one eye)
Ptosis
Weak eye movements
Pupillary sparing
Name four features of Generalised Myasthenia Gravis
-Bulbar - Fatiguable chewing
-Facial - Expressionless
-Neck - Dropped head towards EOD
-Proximal limb weakness more than distal
What is the Ice Pack Test for Myasthenia Gravis?
Improvement of ptosis after ice applied to closed eye for one minute
Neuromuscular transmission is better at lower temperatures
Other than the Ice Pack Test, name four investigations for Myasthenia Gravis
-Tensilon Test (improvement after AChesterase inhibitors)
-Serological Antibodies
-Repetitive Nerve Stimulation (showing decline)
-CT/MRI thymus
Name three options for treating Myasthenia Gravis
Pyridostigmine
Prednisolone
Azathioprine
Could also do a Thymectomy
What is a Myasthenic Crisis?
Worsening of weakness requiring respiratory support
Precipitants include warmth/surgery/stress/infection
How are Myasthenic Crises managed?
IVIG
Plasma Exchange
Steroids
Define Motor Neurone Disease
Umbrella term for progressive and ultimately fatal condition where motor neurones stop functioning
What are the four subtypes of Motor Neurone Disease?
Amyotrophic Lateral Sclerosis
Progressive Bulbar Palsy
Progressive Muscular Atrophy
Primary Lateral Sclerosis
How does the Amyotrophic Lateral Sclerosis subtype of MND present?
Mid to Late 50s
Asymmetrical weakness (spreading sequentially)
Wrist or Foot Drop
Can have UMN or LMN signs
Can have Bulbar involvement
Amyotrophic Lateral Sclerosis is more of a clinical diagnosis. What investigations can be done?
EMG (sensory intact)
Genetics
MRI?CT
Antibodies to exclude MG/Lambert Eaton
Amyotrophic Lateral Sclerosis is incurable. Name four managements to ease symptoms and slow progression
Riluzole/Edavarone - slows progression and increases survival by a few months
Baclofen - muscle cramps
Opioids - Breathlessness
Physiotherapy
Define Muscular Dystrophy
Umbrella term that causes gradual wasting and weakness of muscles
Describe Duchennes Muscular Dystrophy
-X linked recessive inherited abnormal gene
-If mother is a carrier, son has 50% chance of being affected
- Boys present at 3-5y with pelvic muscular weakness
-Life expectancy of 25-35y
How is Duchennes MD managed?
Steroids to slow progression and creatine supplements
Describe Beckers MD
Dystrophin gene is less severely affected than in Duchennes
Symptoms start around 8-12y and may require a wheelchair by 20-30y
Describe Myotonic Muscular Dystrophy
-Genetic disorder presenting in adulthood
-Progressive muscle weakness, prolonged contractions, cataracts, cardiac arrhythmia
Describe Facioscapulohumeral MD
In childhood, weakness around face progressing to shoulders and arms
Sleep with eyes open, can’t purse lips, can’t blow out cheeks
Describe Oculopharyngeal MD
Bilateral Ptosis
Restricted eye movements
Swallowing problems
Describe Emery Dreifuss MD
-Contractures (usually at elbow and ankles)
-Progressive weakness and muscle wasting
Define Multiple Sclerosis
Chronic immune mediated inflammatory disease of the CNS (demyelinating)
Type 4 hypersensitivity
What causes Multiple Sclerosis?
Abnormal immune reaction to an unknown trigger in genetically predisposed individual
More than 200 polymorphisms of genetic succeptibility
Risks - EBV, Low sunlight, adolescent obesity, smoking
Describe the pathophysiology of Multiple Sclerosis
Oligodendrocytes are destroyed which leads to demyelination and axonal loss
B lymphocytes, macrophages and microglia create MS plaques (optic nerves, spinal cord, brainstem)
What are the classifications of Multiple Sclerosis?
-Relapsing/Remitting (getting residual damage each time)
-Primary Progressive
-Secondary Progressive (after 15y of Relapse/Remit)
-Clinically Isolated (clinical episode but no evidence on neuroimaging)
What are the possible visual features of Multiple Sclerosis?
Optic Neuritis (visual loss, poor colour, pain, scotoma)
Internuclear Opthalmoplegia (disconjugate lateral gaze - abducens moves fine, oculomotor doesn’t)
Abducens Palsy
What Motor Symptoms are classical of Multiple Sclerosis?
Weakness
Ataxia
UMN signs
What specific Motor Syndromes can you get within Multiple Sclerosis?
Transverse Myelitis - focal inflammation within the spine and sensorimotor symptoms below with bladder/bowel involvement
Cerebellar Syndrome - DANISH
What sensory features can you get in Multiple Sclerosis?
Paraesthesia and Pain
L’hermitte Phenomenon - Electric shock feeling triggered by neck flexion
Uhthoff Phenomenon - Transient worsening of neuro symptoms with heat
What is the criteria for diagnosing MS based on MRI called?
McDonald Criteria
Define an MS attack
Episode of neurological symptoms lasting >24h with or without recovery (but with at least 30 days between attacks)
Name four differentials for Multiple Sclerosis
Transverse Myelitis
Lyme Disease
B12 Deficiency
Diabetic Neuropathy
Name three supportive investigations for Multiple Sclerosis
CSF Oligoclonal Bands (not identified in serum)
Visual Evoked Response (measures electrical activity over occipital cortex in response to light)
AB Testing
How is an acute MS attack managed?
After ruling out any infection
Oral/IV Methylprednisolone + PPI
Name two disease modifying therapies for MS
Interferon Beta
Glatiramir Acetate
What other general care managements do you need to consider in MS?
Bladder dysfunction (Oxybutinin)
Depression
Fatigue
Neuropathic Pain
Spasticity (Baclofen)
Define Benign Essential Tremor
Relatively common condition characterised by fine trmor affecting voluntary muscles, most noticable in hands but can affect other areas
Name four features of Benign Essential Tremor
Fine Tremor
Symmetrical
Improved by alcohol
More prominent on voluntary (eg outstretched)
How is Benign Essential Tremor managed?
No definitive management
Propranolol or Primidone can be tried
What is Bell’s Palsy?
Idiopathic facial nerve palsy
Name four symptoms of Bell’s Palsy
Difficulty chewing
Tingling
Ear Pain
Hyperacusis
Name four signs of Bell’s Palsy
Loss of nasolabial folds
Drooping corner of mouth
Drooping eyebrow
Asymmetric smile
How can Bell’s Palsy be differentiated from a stroke?
A stroke affected UMN so is forehead sparing
What is the grading system for Bell’s Palsy?
House Brackmann Grading System
When would you consider an alternative diagnosis to Bell’s Palsy?
Overt pain
Systemic Illness
Hearing abnormalities
How is Bell’s Palsy managed?
Prednisolone if within 72 hours
Eye drops and tape for protection
May take several months to recover
Name two complications of Bell’s Palsy
Corneal Abrasion
Aberrant Reinnervation (voluntary contraction of one muscle leads to involuntary contraction of another muscle)
Name five presenting features of Cervical Spondylosis
- Cervical pain worsened by movement
- Cervical stiffness
- Vague numbness/tingling/weakness of upper limbs
-Radiculopathy
-Retro orbital/Temporal pain
How can you demonstrate dural irritation in Cervical Spondylosis?
Lateral flexion and pressure on top of head
Name three read flags for Cervical Spondylosis
-Age of onset <20 ir >55
-Weakness/ Sensory loss in more than one Dermatome/Myotome
-Constitutional symptoms
How would you manage Cervical Spondylosis initially?
First 3-4 weeks reassure patient that it is common and will resolve
Stay active
One firm pillow at night
After four weeks how should Cervical Spondylosis be managed?
Physiotherapy, Occupational health referral, Pain Clinic, Surgery
What is a Radiculopathy?
Sensory/Motor symptoms in response to nerve root damage by any cause
How does Posterior Sciatica present?
Pain along posterior thigh and posterolateral leg due to L5/S1 radiculopathy
How does Anterior Sciatica present?
Pain along anterior leg/thigh due to L3/L4 radiculopathy
Name the nerve roots for upper limb reflexes
Biceps - C5
Triceps - C7
Wrist - C8
Name the nerve roots for lower limb reflexes
Knee - L3/L4
Ankle - L5/S1
What is Carpal Tunnel Syndrome?
Compression/Entrapment/Irritation of Median Nerve
Carpal tunnel syndrome is normally idiopathic, give three associations
Pregnancy
Obesity
Hypothyroid
How does Carpal Tunnel Syndrome present?
Tingling/numbness/pain in median nerve distribution
Often worse at night (patient may hang arm out of bed to revive)
Weakness if hand grip
Severe- Thenar wasting
What are the two clinical tests for Carpal Tunnel Syndrome
Tinnel’s
Phalen’s
Carpal Tunnel Syndrome is normally a clinical diagnosis . What investigations could be done?
Electroneurography
Ultrasound
How is Carpal Tunnel Syndrome managed?
Splint at night and NSAIDs
Corticoteroid injections, Accupuncture
Surgical cutting the transverse carpal ligament
Name three causes of Ulnar Nerve Palsy
Dislocation/Fracture of elbow
Ulnar artery aneurysm
Synovial inflammation
How does Ulnar Nerve palsy at the elbow present?
Wasting along medial forearm
Weakness in hand muscles and finger flexion
Sensory Loss
Ulnar Claw
How does Ulnar Nerve palsy at the wrist present?
Cutaneous often spared
How can Ulnar Nerve Palsy be investigated?
USS Cubital tunnel
EMG
CXR (pancoast)
Describe the conservative management for Ulnar Nerve Palsy
Anterior elbow splinting
Ergonomic correction
NSAIDs
If not then surgery
Diabetic Neuropathy is the most common cause of Peripheral Neuropathy. Give three risk factors
Smoker
>40
Periods of poor glycaemic control
Patients with Diabetic Neuropathy may not notice themselves. What are the five different types?
Peripheral Sensorimotor
Acute Diffuse Painful
Autonomic
Mononeuropathy
Diabetic Amyotrophy
How does Peripheral Sensorimotor Diabetic Neuropathy present?
Sensory nerves affected more (glove and stocking)
Loss of ankle jerks and later knee
Hands only in long standing
How does Acute Diffuse Painful Diabetic Neuropathy present?
Abrupt onset but can resolve completely
Burning foot pain (worse at night)
Associated with poor glycaemic control
How does Autonomic Diabetic Neuropathy present?
Cardiac abnormalities
Exercise intolerance
Reduced respiratory drive
Reduced baroreceptor sensitivity
How does Mononeuropathic Diabetic Neuropathy present?
E.G Carpal Tunnel
How does Diabetic Amyotrophy present?
Pain and paraesthesia in upper legs with weakness and wasting of muscle
How is Diabetic Neuropathy investigated?
Diabetic Control
Measuring BP
Nerve conduction studies
Electromyography
How is Diabetic Neuropathy managed?
Regular surveillance and foot care
Good diabetic control
bed foot cradles
Neuropathic pain relief if required
What are the two types of ADLs?
Personal - Washing/Dressing/Toileting/Continence
Domestic - Cooking/Cleaning/Shopping
Define Alzheimer’s
Progressive neurodegenerative disorder that causes reduced mental performance and impairment in social and occupational function
What causes Alzheimer’s?
A combination of factors
Older Age, Genetics (Sporadic/APP/Presenilin), CVS Disease
What are the two main pathological features associated with Alzheimer’s Disease?
Senile Plaques (Beta Amyloid deposits extracellularly, they are seen in normal ageing)
Neurofibrillary Tangles (Hyperphosphorylated Tau Proteins in regions involved in memory, promoting cell death)
Name two cognitive and two non cognitive symptoms of Alzheimer’s disease
Cognitive - Poor Memory, Language
Non Cognitive - Agitation, Emotional Lability
Name four cognitive assessments
-Mini - Cog (three item word memory and clock drawing)
-AMT (ten item tool)
-MMSE (eleven item tool)
-MoCA (several domains including executive function, attention, language, memory and visuospatial)
How is Alzheimer’s Disease diagnosed?
Functional Inability (and decline from previous)
Cognitive (impairment in >2 domains)
Differentials excluded (via neuroimaging etc)
Name three non pharmacological managements of Alzheimer’s Disease
Advanced Care Planning
Inform DVLA
Encourage groups and activities
What are the pharmacological options for Alzheimer’s management?
Mild to Mod - AChesterase inhibitors (Donepazil)
Mod to Severe - NMDA Antagonists (Memantine)
Frontotemporal dementia has prominent disturbances in social behaviour, language and personality. What are the subtypes?
Behavioural Variant - Progressive personality and behavioural change
Primary Progressive Aphasia (Non Fluent or Semantic)
Describe the pathophysiology of Frontotemporal Dementia
Phosphorylated Tau Proteins/Pick Bodies
Some familial cases with AD Inheritance
Atrophy of frontal and temporal lobes
Frontotemporal Dementia can present in many different ways. How does the Behavioural Variant present?
Disinhibition, Loss of empathy, Apathy
Frontotemporal Dementia can present in many different ways. How does the Primary Progressive Aphasia present?
Effortful/Halting Speech
Speech Apraxia
Difficulty finding words
Frontotemporal Dementia can present in many different ways. How do the Motor Syndromes present?
-MND
-Corticobasal Syndrome (Dystonia, Asymmetrical Akinesia, Alien Limb)
-Progressive Supranuclear Palsy (difficulty looking up)
What would the MRI of Frontotemporal Dementia show?
Frontal and temporal lobe atrophy
How would you manage Frontotemporal Dementia?
Financial advice, SALT input, Supervision
SSRIs (decrease impulsivity)/Atypical Antipsychotics
State the five subtypes of Vascular Dementia
-Subcortical
-Stroke Related (Large Cortical)
-Single/Multiple Infarct
-Mixed (with Alzheimers)
-Autosomal Dominant
How does Vascular Dementia present?
Stepwise cognitive decline
Can have focal neurological symptoms
How is Vascular Dementia managed?
Donepazil/Memantine
Control CVS factors
What is Horner’s Syndrome?
Classic triad of Miosis/Ptosis and anhidrosis/enopthalmos due to a lesion along oculosympathetic pathway
What is the Oculosympathetic Pathway?
First Order - Hypothalamus to Spinal Cord
Second Order - Preganglionic (spinal cord to sympathetic chain)
Third Order - Post Ganglionic (within adventitia of IC, travelling to dilator pupillae and muller’s muscle)
What are the causes of Horner’s Syndrome if first order neurones are affected? (4S’s)
Stroke
multiple Sclerosis
SOL
Syringomyelia
Anhidrosis on one half of body
What are the causes of Horner’s Syndrome if second order neurones are affected? (4T’s)
Tumour
Trauma
Thyroidectomy
Top Rib
What are the causes of Horner’s Syndrome if third order neurones are affected? (3C’s)
Carotid Aneurysms
Carotid Artery Dissection (no other sx)
Cavernous Sinus Thrombosis
No Anhidrosis
How can Horner’s Syndrome be investigated?
CT Angiography (exclude dissection)
MRI (if brainstem features)
CXR (Pancoast)
How can Horner’s Syndrome be confirmed?
Cocaine Eye Drops - blocks NA reuptake, only unaffected eye constricts
Apraclonidine - Alpha agonist causing affected pupil to dilate and normal pupil to constrict
Hydroxyamphetamine - all dilate except post ganglionics
Name two cerebellopontine angle lesions
Acoustic Neuroma
Meningioma
What are Acoustic Neuromas?
Tumours of the vestibulocochlear nerve arising from schwann cells
Typically benign and slow growing but causes pressure on surrounding tissues
Name two risk factors for Acoustic Neuromas
Neurofibromatoses
High dose ionising radiation
What initial symptoms can Acoustic Neuromas present with?
Unilateral/Asymmetric hearing loss/tinnitus
Impaired facial sensation
Balance problems without explanation
As an Acoustic Neuroma grows, what further symptoms can it cause?
Facial Pain/Numbness
Earache
Ataxia
Hydrocephalus
What investigations are required for Acoustic Neuromas?
Audiology
MRI
If the Acoustic Neuroma is small, you can just manage with serial scans. What other management options are there?
Microsurgery (can cause CSF leak or stroke)
Radiotherapy (sterotactic, fractionated, proton beam)
What are Meningiomas?
Slow growing benign tumours arising from Dura Mate
Normally well circumscribed
How are Meningiomas graded?
I - Generally Benign
II - Higher rate of recurrence post surgery
III - Anaplastic
How do Meningiomas present?
Seizures
Raised ICP
Changes in personality
Nerve palsies
Can be spinal - Brown Sequard
How do Meningiomas appear on MRI?
Well defines
Central Cystic Degeneration
Oedema of nearby white matter
What are the management options for Meningiomas?
Endovascular Embolisation (Coil or Glue)
Surgical Removal
Stereotactic Radiotherapy
What is Subacute Combined Degeneration of the Cord?
Degeneration of the posterior and lateral columns as a result of B12/Vitamin E/Copper deficiency
Name three underlying causes of Subacute Combined Degeneration of the Cord
Dietary Deficiency
Lack of IF
Low Gastric pH
What would be the underlying investigative abnormality in Subacute Degeneration of the Spinal Cord?
B12 and folate deficiency leading to megaloblastic anaemia
How does Subacute Degeneration of the Spinal Cord present?
Weakness of leg/arms/trunk
Progressive tingling and numbness
Posterior Column - reduced vibration and proprioception
Lateral Column - UMN signs, Ataxia
How is Subacute Degeneration of the Spinal Cord managed?
Replace B12
If folate also deficient, treat B12 first
What is Cavernous Sinus Syndrome?
Any pathology involving Cavernous Sinus which may present as a combination of unilateral opthalmoplegia, autonomic dysfunction, or sensory loss
Give four causes of Cavernous Sinus Syndrome
Meningioma
Sarcoidosis
Basal Skull Fracture
Cavernous Sinus Thrombosis
How does Cavernous Sinus Syndrome present?
Can compress any nerves leading to isolated palsies or post ganglionic Horners
May get Proptosis and Chemosis secondary to pressure
How is Cavernous Sinus Syndrome investigated?
Bloods
MRI
CT
How is Cavernous Sinus Syndrome managed?
Tumour - Surgery or Radio
Traumatic - Orbital Decompression
Inflammatory - Steroids
Vascular - Embolisation/Clipping
What is Wernicke’s Encephalopathy?
Spectrum of diseases resulting from Thiamine deficiency (usually related to alcohol abuse although can be secondary to Bariatric Surgery/Hyperemesis Gravidarum etc)
Describe the pathophysiology of Wernicke’s Encephalopathy
Inadequate nutritional thiamine/decreased absorption from GI tract/impaired thiamine utilisation
Thiamine is a cofactor required by three enzymes for carbohydrate synthesis so causes metabolic disruption
What is the classic triad of Wernicke’s Encephalopathy? Name two other symptoms
Ataxia, Opthalmoplegia, Confusion
Unexplained hypotension/hypothermia
Hallucinations
What is required to diagnose Wernicke’s Encephalopathy?
At least 2 of:
Dietary Deficiency
Oculomotor Abnormalities
Cerebellar Dysfunction
Altered Mental State/Impairment
How is Wernicke’s Encephalopathy managed?
IM/IV Pabrinex
Can then switch to PO thiamine when stable
Alcoholics should have prophylactic thiamine as long as they are malnourished/have decompensated liver disease
May have to use Mental Health Act
Hypomagnesaemia may be a cause - used in thiamine production - so check urgently
One of the complications of Wernicke’s Encephalopathy is Korsakoff Syndrome. Name three features of this
Confabulation
Anterograde and Retrograde Amnesia
Psychosis
What is an Argyll Robertson Pupil?
Small irregular pupils that have little/no constriction to light but do accommodate
Classically caused by neurosyphilis
Describe the pathophysiology behind an Argyll Robertson Pupil
Damage to dorsal aspect of EWN disrupts inhibitory neurones causing constant constriction
What is Creutzfeld-Jakob Disease?
Best known Human Prion Disease
Accumulation of small infectious pathogens, containing protein but lacking nucleic acids
Name the four main variants of CJD
-Sporadic (85% of cases)
-Hereditary (Familial clusters with dominant inheritance)
-Iatrogenic (Neurosurgery, tissue grafts)
-New Variant (linked to eating BSE infected cattle products)
CJD presents very non specifically and can’t be reliably diagnosed until death. Give some presentations
-Myoclonus
-Progressive Ataxia/Choreiform
-Visual Disturbance
-Rapidly progressing cognitive and functional impairment
How is CJD investigated?
-Brain Biopsy (can use tonsil biopsy if new variant)
-CSF markers
-EEG (Periodic wave complexes in sporadic)
-MRI (increased intensity in certain brain regions depending on subtype)
Define Syringomyelia
Fluid filled tubular cyst in central spinal cord that can elongate/enlarge and expand into grey/white matter, compressing tracts
Define Syringobulbia
Where Syringomyelia has extended into brainstem
Can cause nerve palsies
Describe the aetiology of Syringomyelia
Blockage of CSF (normally secondary to Chiari malformation)
SOL, Arachnoiditis, Post Traumatic
How does Syringomyelia present in terms of sensory features?
Spinothalamic lost in a shawl like distribution
Extends into Dorsal Column
Dysaesthesia - Pain when skin is touched
How does Syringomyelia present in terms of motor features?
As t extends and damages UMN of anterior horn
Muscle wasting and weakness beginning in hand
Reduced tendon reflexes
Claw hand
How does Syringomyelia present in terms of autonomic features?
Can affect bowel/bladder/sexual organs
Horners
How can Syringomyelia be investigated?
MRI - shows soft tissue causes
CT - shows bony causes
How can Syringomyelia be managed?
Physio and rehab
Taught to avoid damage which may occur in the absence of pain
Surgery - Shunt/Laminectomy/Syringotomy
What is Hypoxic Ischaemic Encephalopathy?
Entire brain is derived of adequate oxygen supply but loss is not total. Normally associated with neonates but can occur in adults (eg post cardiac arrest)
How does HIE present?
Cyanosis as blood is redirected
Fainting/Coma/Seizures/Brain Death
What would be seen on CT of HIE?
Diffuse oedema
Reduced cortical grey matter
What Syndrome can Pituitary Tumours be associated with?
MEN1
Name four types of Pituitary Tumour
Non functioning adenoma
Prolactinoma
GH secreting
ACTH secreting
Describe the local effects of a Pituitary Tumour
Headache (retro-orbital or bitemporal, worse on waking)
Visual Field Defects
Facial Pain
If extending into hypothalamus - Diabetes Insipidus etc
How can the cause of Bitemporal Hemianopia be distinguished?
If initially lower quadrants affected - Craniopharyngoma
If upper - Pituitary Adenoma
What is the order of hormones affected in Hypopituitarism
LH, GH, TSH, ACTH, FSH
The management for Pituitary Adenomas is often Trans-sphenoidal surgery. Name four complications
SIADH
DI
Addisons
CSF leak
How can you manage Pituitary Adenomas medically?
Acromegaly - Octreotide
Prolactinoma - Bromacriptine
When would you use Radiotherapy for Pituitary Adenoma?
Incomplete resection
Name three causes of Olfactory Nerve Damage
Trauma
Frontal Lobe Tumour
Meningitis
Name three causes of Oculomotor Nerve Damage
DM
GCA
PCA
Name two causes of Trochlear Nerve Damage
Rare -Orbital trauma, Diabetes
Name three causes of Abducens Nerve Damage
MS
Pontine CVA
Raised ICP
Name two causes of Facial Nerve Damage
Upper - Stroke
Lower - Bells
Name three causes of Vestibulocochlear Nerve Damage
Loud Noises
Pagets
Acoustic Neuroma
Name three causes of Glossopharyngeal Nerve Damage
Trauma
GBS
Polio
Name two causes of Vagus and Accessory Nerve Damage
Trauma
Brainstem pathology
Name three causes of Hypoglossal Nerve Damage
Polio
Syringomyelia
TB
What AED is tolerated really well?
Levetiracetam (Keppra)
Name three stroke mimics
Migraines
Todd’s Paresis (post seizure)
Bells Palsy
Name three Chameleons (atypical strokes)
Bilateral thalami stroke
Bilateral occipital stroke
Limb shaking TIA
What can affect the seizure threshold?
Medications
Drugs
Sleep
Flashing Lights
What is the pregnancy prevention programme for epilepsy?
At least 1 form of contraception (ideally 2)
Should be started Atleast one month before starting the medication
MCA infarcts make up 2/3 of Ischaemic strokes. How would ACA strokes present differently?
Contralateral Lower limb > upper limb
Disorder of executive function
What is characteristic on an epileptic EEG?
Spike and wave discharge
Hz is how many spikes and waves in a second
What are the four recognised stages of Parkinson’s
Early
Maintenance
Advanced
Palliative
What is Restless Leg Syndrome?
Urge to move legs (aka this is) associated with par aesthetics (crawling,throbbing), and night time movements (often noticed by partner)
Give 5 associations of Restless Leg Syndrome
Family History
Uraemia
Iron Deficiency Anaemia (check ferritin)
Pregnancy
Diabetes Mellitus
How is Restless Leg Syndrome managed?
Simple exercises such as walking and stretching
Dopamine agonists such as Ropinorole first line
Name two scoring systems for Parkinson’s
Heohn Yahr
UPDRS
Describe the Heohn Yahr Scale for Parkinson’s Disease
I - Unilateral Disease
II - Bilateral Disease with intact posture
III - Mod Bilateral disease with postural instability
IV - Severe Bilateral Disease but can still walk independently
V - Wheelchair only or bed bound
Describe the UPDRS
Measured on 4 categories (Non Motor Symptoms of ADL, Motor Symptoms of ADL, Motor Assessment, Motor Complications), rated out of 5
Rashes in meningitis can be petechial (<2mm) or purpuric (>2mm). How can these be tested in PoC
Still via glass tumbler test but look for paler areas of body, such as palms/soles/roof of mouth
