Neurology COPY COPY Flashcards
What is a cerebrovascular accident?
Ischaemia or infarction of brain tissue secondary to inadequate blood supply
OR
Intracranial haemorrhage
What are the different types of cerebrovascular accidents?
Transient Ischaemic Attack(TIA)
Stroke:
Haemorrhagic
Ischaemic
What is a TIA?
Sudden onset focal neurological deficit with complete clinical recovery secondary to ischaemia without infarction.
Sx Last <24 hrs
Caused by Reduced blood flow, ischaemia, embolism or disease of blood vessels or blood.
What is a crescendo TIA?
where there are two or more TIAs within a week. This carries a high risk of developing in to a stroke.
What is the epidemiology of a TIA?
15% of first strokes are preceded by TIA
M > F
Black ethnicity is at greater risk due to their hypertension and atherosclerosis predisposition
20, 000 people have a TIA
What are the risk factors for a TIA?
Increasing age
Hypertension
Smoking
Diabetes
Hypercholesterolaemia
Atrial fibrillation
HTN
VSD
Carotid stenosis
What are the causes of a TIA?
Thrombus formation or embolus (for example in patients with atrial fibrillation)
Atherosclerosis + embolism from carotid
Shock
Vasculitis
Hyper viscosity - polycythaemia, sickle cell, myeloma
What artery is commonly the route of a TIA?
90% = ICA
10% = Vertebral
What are the Clinical features of a TIA?
Depends on the site of the TIA:
- Sudden Loss of function with complete recovery
- Stroke Sx - Slurred Speech Facial Droop
- 90% of TIAs affect anterior circulation (ICA)
- 10% affect Posterior Circulation (vertebral Arteries)
What would be the signs of a TIA in the Anterior Cerebral artery?
Weak/numb contralateral leg
What would be the signs of a TIA in the Middle Cerebral Artery?
weak/Numb contralateral side of body
Face drooping w/ forehead spared
Dysphasia
What would be the signs of a TIA in the Posterior Cerebral Artery?
Vision loss:
Contralateral homonymous hemianopia w/ macula sparing = occipital cortex affected.
What would be the signs of a TIA in the Vertebral Artery?
Cerebellar Syndrome: DANISH w/ +tve romberg test
Dysdiadokinesia
Ataxia
Nystagmus
Intention tremor
Slurred staccato speech
Hypotonia
What is Amaurosis Fugax?
a painless temporary loss of vision, usually in one eye
Due to occlusion/reduced blood flow to the retina through the ophthalmic, retinal or ciliary artery. (often due to emboli)
This is a bad sign as it often signals stroke is impending
What is the Primary investigation for a TIA?
Diagnosis is Clinically made: Usually TIA/Stroke is obvious
ABCD^2 assessment
FAST test
MRI - First line for confirmation
Carotid Doppler - Look for Stenosis
CT angiography - Look for Stenosis
ECG
What is the FAST acronym?
FACE
ARMS
SPEECH
TIME
What is the ABCD^2 scoring system for TIA/Stroke?
No longer recommended by NICE
Age >60
BP >140/90
Clinical Sx - Unilateral Weakness (+2). Speech Disturbance w/o weakness (+1)
Duration >1hr (+2) / <1hr (+1)
DM - Type 2
What would be a high or low risk score following ACBD^2 assessment for TIA/stroke?
High risk:
ABCD2 score of 4 or more
AF
More than TIA in one week
TIA whilst on anti-coagulation
Low risk:
None of the above
Present more than a week after their last symptoms have resolved
How can you distinguish between a TIA and a Stroke?
You cant until after recovery
TIA Sx resolve usually within/<24 hours
Stroke Sx last more than 24 hours
What is the management of a High and low Risk TIA?
Lower RFs - stop smoking, reduce alcohol, exercise and diet
Immediate Aspirin 300mg
Start secondary prevention of CVD:
Clopidogrel 75mg
Atorvastatin 80mg
Treat BP - Ramipril
High Risk - Refer for specialist assessment within 24 hours of onset of symptoms
Low Risk - Refer for specialist assessment within 7 days of onset of symptoms
What are the main complications of a TIA?
Increased risk of stroke
Increased risk of underlying CVD
What are the two types of stroke?
Ischaemic (85% of cases)
Haemorrhagic (15% of cases)
Define a stroke?
Rapid onset neurological deficits caused by focal, cerebral, spinal or retinal infarction lasting more than 24 hrs
What is the epidemiology of a stroke?
1.2 million people living with stroke in the UK
110, 000 people have a first or recurrent stroke per year
Uncommon in those under 40
More common in males than females
Incidence is falling due to more vigorous approach to risk factors in primary care e.g. statin use and BP control
What is an Ischaemic Stroke?
85% of strokes
An episode of neurological dysfunction caused by focal cerebral, spinal or retinal infarction secondary to the occlusion of a blood vessel
What are the categories of Ischaemic Stroke?
Large Vessel Disease (50%) - Atherosclerosis
Small Vessel Disease (25%) - Lacunar
Cardioembolic (20%) - AF, IE, MI, Dissection
Cryptogenic (<5%)
Rare causes (<5%) - vasculitis
What are the main causes of Ischaemic stroke?
Large artery stenosis
Cardiac emboli from AF, MI or infective endocarditis 🡪 blood stasis
Atherothromboembolism e.g. from carotid artery
Dissection of aorta/carotid
Shock – reduced blow flow throughout body
Vasculitis
What is a Haemorrhagic Stroke?
15% of strokes:
Rapidly developing clinical signs of neurological dysfunction attributable to a focal collection of blood within the brain parenchyma or ventricular system not caused by trauma.
What are the subtypes of haemorrhagic stroke?
Primary Intracerebral: bleeding within the brain parenchyma
Subarachnoid Haemorrhage: bleeding into the subarachnoid space
Secondary haemorrhage (these are NOT strokes) - due to trauma, anti-coagulation (warfarin), Bleeding due to tumour
What are the main causes of a haemorrhagic stroke?
Hypertension
Aneurysm rupture 🡪 SAH
Arteriovenous Malformation (AVM)
Amyloidosis
What is the 3rd leading cause of mortality in the US and UK?
Stroke
What are the risk factors for stroke?
Hypertension - greatest RF
Increased age - 68-75 MC
Male
Smoking
Diabetes
Hypercholesterolaemia
AF
Heart disease
FHx
Medication
What are the symptoms of stroke?
Focal neurological deficit based on site of the infarct.
What would be an extra finding in haemorrhagic stroke?
Increased intracranial pressure (ICP)
Causes midline shift
What is a Lacunar Stroke?
Very common type of ischaemic stroke of the lenticulostriate arteries.
These supply the deep brain structures (BG, IC, Thalamus, pons)
What are the clinical manifestations of a stroke in the anterior cerebral artery?
Contralateral weakness of lower limb
Contralateral sensory loss of lower limb
Truncal ataxia
Incontinence
Drowsiness
Dysphasia (if dominant hemisphere)
Logical thinking
Personality
What are the clinical manifestations of a stroke in the Middle cerebral artery?
Contralateral motor weakness – arms and legs
Hemiplegia – paralysis of one side of the body
Contralateral sensory loss
Aphasia – inability to understand (Wernicke’s) or produce speech (Broca’s)
Dysphasia (speech problems) – only if stroke is in dominant hemisphere (Broca’s)
Facial droop
What are the clinical manifestations of a stroke in the Posterior cerebral artery?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
Disorders of Perception
What are the clinical manifestations of a stroke in the Vertebrobasilar artery?
Cerebellar signs
Reduced consciousness
Disorders of balance
Coordination disorders
Quadriplegia or hemiplegia
What are the clinical manifestations of a stroke in Weber’s syndrome (midbrain infarct; branches of posterior cerebral artery)
Oculomotor palsy and contralateral hemiplegia
What are the clinical manifestations of a stroke in Lateral medullary syndrome (posterior inferior cerebellar artery occlusion)
Ipsilateral facial loss of pain and temperature
Ipsilateral Horner’s syndrome
Ipsilateral cerebellar signs
Contralateral loss of pain and temperature
What is horner’s Syndrome?
Caused by a Lesion within the Sympathetic Pathway
Presents as a unilateral triad of:
Miosis: small pupil
Ptosis: drooping eyelid
Anhidrosis: lack of sweat
What are the clinical manifestations of a stroke in the Basilar Artery?
Locked in syndrome;
Paralysed except for muscles that control eye movements.
Px are consciously aware, can think and reason but cannot speak or move
What are the extra symptoms of a haemorragic stroke?
headache
AMS (altered Mental Status)
Seizures
N+V
What are the primary investigations for a stroke?
CT Head:
Ischaemic - mostly normal
Haemorrhagic - Hyperdense blood
Ix over first few days after stroke:
MRI - with diffusion weighted imaging
CT Angiography
Carotid Doppler USS
ECHO - for those with cardioembolic stroke
ECG - For AF
Bloods - rule out hypoglycaemia
What is the very first step in the acute management of a suspected stroke?
Determine if the stroke is haemorrhagic or ischaemic from the initial CT.
What is the management for an Ischaemic stroke?
immediate CT to exclude primary intracerebral haemorrhage
If presents within 4.5hrs:
Clot buster (thrombolysis) - IV Alteplase
Presenting After 4.5 hrs
Aspirin 300mg for 2 weeks
Prophylaxis - Lifelong clopidogrel (75mg)
What is the function of Alteplase?
What are the contraindications?
Tissue plasminogen activator that rapidly breaks down clots and can reverse the effects of a stroke if given in time.
CIs: Haemorrhage, Intracranial bleed, clotting disorders, aneurysms
What is the management of a Haemorrhagic Stroke?
Neurosurgery referral for larger bleeds
Control BP - BB/ARB to <150 systolic
Iv mannitol for increased ICP
Vitamin K if bleeding is warfarin related
rehabilitation - Physio/OT
What type of stroke should be suspected if the patient is on oral anticoagulants?
Suspect Haemorrhagic until proven otherwise
What are some key differential diagnoses for strokes?
Hypoglycaemia
Space occupying Lesions - tumour, AVM
Syncope due to arrythmia
Migraines
Functional Neurological Disorders
Infection - particularly in elderly
What is the prophylactic prevention of secondary strokes?
Clopidogrel 75mg once daily (alternatively dipyridamole 200mg twice daily)
Atorvastatin 80mg should be started but not immediately
Carotid endarterectomy or stenting in patients with carotid artery disease
Treat modifiable risk factors such as hypertension and diabetes
What is a haematoma?
Describes a bleed that has mostly clotted and hardened
What is a Haemorrhage?
Describes an active ongoing bleed
Where can intracranial haemorrhages occur?
Extradural
Subdural
Subarachnoid
Intracerebral
What are the main blood vessels within the meninges?
Extradural – middle meningeal artery
Subdural – bridging veins
Subarachnoid – circle of Willis
Pia – no vessels as it forms part of the blood-brain barrier
What is an Extradural (Epidural) haemorrhage (EHD)?
Bleeding into the potential space between the skull and the dura mater.
The blood then collects in this space and is referred to as an extradural haematoma (EDH).
What are the causes of Extradural haemorrhage?
Usually Trauma (blunt trauma):
Arterial bleed - often middle meningeal artery
Venous bleed - often due to dural venous sinus
What is the most common blood vessel damaged to cause an extradural bleed?
Middle meningeal artery due to damage to the pterygoid bone
What are some rarer causes of an EDH?
Non-traumatic:
Haemorrhagic tumour
Coagulopathy
Infection
Vascular Malformation
What are the risk factors for an EDH?
Younger age (20-30)
Male
Anticoagulant usage
Why is an EDH less likely to occur in the elderly?
The Dura matter is more firmly adhered to the skull so blood is less likely to accumulate in this region.
What is the typical presentation of an Extradural bleed?
Characteristic history:
Head injury
Loss of consciousness following trauma or initial drowsiness
Lucid interval – period of time between traumatic brain injury and decrease in consciousness
Signs of raised ICP – headache, vomiting, nausea, seizure
± hemiparesis with brisk reflexes
Cushing’s Triad - Bradycardia and raised BP and Respiratory Irregularity
Ispilateral pupil dilation
Decreased Glasgow coma scale
Coning of brain through foramen magnum
Death due to respiratory arrest
What are the cushings triad signs of an extradural haemorrhage?
Wide Pulse Pressure
Bradycardia
Respiratory irregularity
Why is there an increased ICP in an EDH?
Haemorrhage - increased volume decreased pressure
old blood clots which can then take up water (osmotically active)
Will increase in volume and raise ICP
What are the primary investigations of an EDH?
CT head:
Lens shaped hyperdense bleed/haematoma
Confined within suture lines
Midline shift
Skull XR - may show fracture
What is the management on an EDH?
Urgent Neurosurgery referral:
- Burr Hole craniotomy
Ligation of bleeding vessel
Stabilise Px - ABCDE management
IV Mannitol to reduce ICP
What are the complications of an EDH?
Cerebral oedema
Raised intracranial pressure and herniation
Ischameia: can occur due to mass effect, herniation, hypoperfusion, vasospasm
Seizures
Infection
What is the Typical History of an EDH?
The typical history is a young patient with a traumatic head injury that has an ongoing headache.
They have a period of improved neurological symptoms and consciousness followed by a rapid decline over hours as the haematoma gets large enough to compress the intracranial contents.
What are some Key differential Diagnoses of an EDH?
Subdural haematoma
Subarachnoid haematoma
Epilepsy
Meningitis
Carotid dissection
Carbon monoxide poisoning – fit with lucid period
What is a Subdural Haemorrhage?
When blood accumulates in between the dura and arachnoid matter often due to rupture of the bridging veins
What are the causes of a Subdural haemorrhage?
Often due to the rupture of a bridging vein caused by trauma:
Shearing deceleration injury
Abused children (shaken baby sydrome)
What is the Epidemiology of a Subdural Haemorrhage?
Often in Px who have small brains:
Alcoholics, Dementia, Children
Occurs in Abused Children (Shaken Baby Syndrome)
What are the risk factors for a subdural haemorrhage?
Increased age
alcoholics
Trauma
Cortical atrophy (due to age/dementia)
Child abuse
What is the pathology of a SDH?
- Trauma either due to deceleration due to violent injury or due to dural metastases results in bleeding from bridging veins between cortex and venous sinuses
- Bridging veins bleed and form a haematoma (solid swelling of clotted blood) between the dura and arachnoid
- This reduces pressure 🡪 bleeding stops
- Days/weeks later the haematoma starts to autolyse due to the massive increase in oncotic and osmotic pressure 🡪 water is sucked into the haematoma 🡪 haematoma enlargement 🡪 gradual rise in intra-cranial pressure (ICP) over many weeks
- This shifts midline structures away from the side of the clot and can lead to tectorial herniation and coning (brain herniates through foramen magnum) if left untreated
What are the symptoms of a subdural haemorrhage?
Often gradual symptom onset with a latent period (can be days, weeks, months)
Progressive confusion and cognitive deficit
Headaches and vomiting
Focal neurological deficit, e.g. weakness or fixed dilated pupil
Fluctuating consciousness
What are the clinical signs of a subdural haemorrhage?
Signs of Raised ICP - headache, N+V, Seizure
Cushing Triad - Wide PP, Bradycardia,
Resp irregularity
Fluctuating GSC
Herniation - Supratentorial and infratentorial
What are the primary investigations of a subdural haemorrhage?
CT Head:
Banana/Crescent shaped dense region.
Not confined to suture lines.
May have midline shift
If acute - Hyperdense (bright)
If Subacute - Isodense
If Chronic - Hypodense (darker than brain
What is the treatment for a subdural haemorrhage?
Referral To Neurosurgeon
IV mannitol - reduce ICP
Burr hole / Craniotomy to relieve pressure
What is a subarachnoid haemorrhage (SAH)?
Subarachnoid haemorrhage involves bleeding in to the subarachnoid space, where the cerebrospinal fluid is located, between the pia mater and the arachnoid membrane.
This is usually the result of a ruptured cerebral aneurysm.
What is the Epidemiology of a Sub Arachnoid Haemorrhage?
Typical age 35-65 – mean age 50
Account for ~5% of strokes
8-12 per 100, 000/year
What are the possible causes of an SAH?
Trauma
Non-Trauma: (spontaneous)
Berry Aneurysm from circle of willis- MC of spontaneous SAH (70%)
AVM
Where do berry aneurysms often occur?
Usually at junctions of arteries within the circle of Willis.
Often communicating junctions
What is the most common artery affected in an SAH?
Circle of Willis:
Anterior communicating artery
ACA junction
What are the risk factors for a SAH?
What are some strong associations with SAH?
Increased age - >50yrs
HTN
Known Aneurysm
PKD
Trauma
FHx
Smoking
Alcohol excess
Strong associations with Cocaine Use and Sickle Cell
What is the Pathology of an SAH from a Berry Aneurysm?
Rupture of the arteries forming the circle of Willis
Rupture of the junction of the anterior communicating artery and the anterior cerebral artery or the posterior communicating artery and the internal carotid artery
Leads to tissue ischaemia (since less blood can reach the tissue) as well as rapid raised ICP as the blood acts like a space-occupying lesion and puts pressure on the brain 🡪 neurological deficits
What is the Pathology of an SAH from an AVM?
Atriovenous malformations (AVM)
Vascular development malformation often with a fistula between arterial and venous systems causing high flow through the AVM and high-pressure arterialisation of draining veins 🡪 rupture
What are the symptoms of a SAH?
Occipital Thunderclap Headache
Seizures
Vomiting
Loss of Consciousness - Coma/Drowsiness
Meningism (mimics meningitis)
Reduced GSC
Nerve palsies - 3rd / 6th
What are the signs of an SAH?
Meningeal irritation:
- Kernig’s sign – unable to extend patient’s leg at the knee when the thigh is flexed
- Neck stiffness
- Brudzinski’s sign – when patient’s neck is flexed, patient will flex their hips and knees
Subhyaloid haemorrhages (bleeding between retina and vitreous membrane) ± papilloedema
What are the characteristics of the headache experienced in an SAH?
Occipital Thunderclap Headache
Sudden onset
Worst headache of their life (0-10 instantly)
May have a sentinel headache preceding this
Why may you get nerve palsies in SAH?
3rd nerve palsy:
An aneurysm arising from the posterior communicating artery will press on the 3rd nerve, causing a palsy with a fixed dilated pupil
6th nerve palsy:
A non-specific sign which indicates raised intracranial pressure
What are the primary investigations in a SAH?
CT Head - diagnostic with 100% sensitivity if performed within 6 hrs.
If Negative but SAH still suspected:
Lumbar Puncture (performed after 12hrs)
will show xanthochromia / yellowish CSF due to RBC haemolysis
Angiography (CT or MRI) can be used once a subarachnoid haemorrhage is confirmed to locate the source of the bleeding.
ABG - Rule out hypoxia
Who do SAHs more commonly affect?
Black patients
Female patients
Age 45-70
What is the Glasgow Coma Scale (GCS)?
Tool for assessing consciousness:
Based on Eyes, verbal and motor response.
Normal - 15/15
Comatose - 8/15
Unresponsive - 3/15
Eyes:
Spontaneous = 4
Speech = 3
Pain = 2
None = 1
Verbal response:
Orientated = 5
Confused conversation = 4
Inappropriate words = 3
Incomprehensible sounds = 2
None = 1
Motor response:
Obeys commands = 6
Localises pain = 5
Normal flexion = 4
Abnormal flexion = 3
Extends = 2
None = 1
What are important differential diagnoses of SAH?
Meningitis - no thunderclap headache in this
Migraine - no meningism/thundercap headache in this
What is the treatment for an SAH?
1st line:
Neurosurgery Referral:
1st - Endovascular coiling
2nd - Surgical clipping
Give immediate Nimodipine (CCB) 60mg (every 4 hrs for 21 days)- prevents vasospasm
Resuscitation
Monitoring for complications
What are some complications of an SAH?
Re-bleeding
Cerebral ischaemia - due to vasospasm
Hydrocephalus – due to blockage of arachnoid granulations
Hyponatraemia - due to urinary salt loss
What are some Differential Diagnoses of an SAH?
Migraine
Meningitis
Corticle vein thrombosis
What is an Intracerebral haemorrhage?
Intracerebral haemorrhage involves bleeding into the brain tissue. It presents similarly to an ischaemic stroke.
Often has a headache/come and signs of raised ICP
Define Meningitis?
Inflammation of the meninges
This is a notifiable condition to PHE
What are the different causes of Meningitis?
Viral:
Enterovirus (coxsackie)
HSV2
VZV
Bacterial:
N. Meningitidis
S. pneumonia
What is the most common cause of meningitis?
Viral cause
What organism is the most common cause of viral and bacterial meningitis?
Viral - Enterovirus (coxsackie)
Bacterial - S. pneumoniae
Which is a more severe form of meningitis?
Bacterial is more severe
What is the pathology of Meningitis?
- Brain and the CSF should be sterile – no bacteria
- Bacteria can get in through different routes:
- Neurosurgical complications: post-op, infected shunts, trauma
- Extracranial infection: ear – otitis media, nasopharynx, sinuses – sinusitis
- Via the blood stream: i.e. bacteraemic seeding
- Once the CSF is infected the pathogen can multiply and replicate because there are no immune cells (as the BBB prevents the movement from the blood)
- Eventually the WBCs enter into the CSF, meninges and brain due to the blood vessels becoming leaky
- This causes meningeal inflammation +/- brain swelling
What are the main risk factors for meningitis?
Extremes of age (Infant/elderly)
Immunocompromised
Pregnancy
Travel
Crowded environment - barracks/uni
Non-vaccinated
What is the most common bacterial cause of meningitis in neonates (0-3 months)?
Group B Strep (S. agalactiae)
Due to the colonisation of mothers vagina which can cause infection in neonate at birth.
Also E.coli and Listeria
What is the most common bacterial cause of Meningitis in Infants?
N. meningitidis
S. pneumoniae
H. Influenzae (now rare due to vaccine)
Why is Haemophilus influenzae now a less common cause of meningitis?
Due to vaccination
What are the common bacterial causes of meningitis in adults?
S. pneumoniae
N. Meningitidis
What are the most common bacterial causes of meningitis in the elderly?
S. pneumoniae
N. meningitidis
Listeria
What are the most common bacterial causes of meningitis in the immunocompromised?
Listeria monocytogenes
M. Tuberculosis
What vaccines are available for meningitis coverage?
N. Meningitidis - Men B + Men C + Men ACWY
S. pneumoniae - PCV Vaccine
What is meningococcal Septicaemia?
N. meningitidis infection in the blood.
This causes a non-blanching purpuric rash
This is due to Disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages
What are the symptoms of Meningitis?
Meningism:
Headache, Photophobia, Neck stiffness
Fever
Nausea + Vomiting
Seizures
Purpuric Rash - Bacterial Meningitis
Non-Blanching Purulent Rash - Meningococcal Septicaemia
What are the clinical signs of meningitis?
Kernig’s Sign:
When the hip is flexed and the knee is at 90°, extension of the knee results in pain
Brudzinski Sign:
severe neck stiffness causes the hips and knees to flex when the neck is flexed
What are the primary investigations in meningitis?
Bloods:
FBC - raised WCC
CRP - raised
Blood glucose - compared with CSF
Blood culture - to determine viral/bacterial
CT Head - Look for Brain Lesions/Abscesses/CIs for LP
GS DIAGNOSTIC = Lumbar Puncture (LP) + CSF Analysis
What are some contraindications for a lumbar puncture?
Raised ICP
GCS <9
Focal Neurological signs
Where is a lumbar puncture usually taken from?
Between L3/L4
Since spinal cord ends L1/2
What would the results of an LP CSF sample analysis look like in bacterial meningitis?
CSF:
Appearance - Cloudy
WCC - High neutrophils
Protein High
Glucose - Low
Culture - bacterial organism
bacteria swimming in the CSF (cloudy) will release proteins (high) and use up the glucose (low). Immune response to bacteria is neutrophils
What would the results of an LP CSF sample analysis look like in Viral meningitis?
CSF:
Appearance - Clear
WCC - High Lymphocytes
Protein - Normal/Mildly raised
Glucose - Normal
Culture - Negative
Viruses cant be seen (clear) don’t use glucose (normal) but may release a small amount of protein (normal/mild inc). Immune response to viruses are lymphocytes
What is the treatment for viral Meningitis?
Usually milder and so Supportive Tx
If HSV/VZV infection then Acyclovir
What is the treatment for bacterial meningitis in a hospital?
Broad Spec Abx - Cover All Likely Organisms:
1st Line - Ceftriaxone or Cefotaxime (as they get through the BBB)
WTIH OR AFTER
IV Dexamethasone - Prevent neurological sequelae
2nd Line: Chloramphenicol
Once Blood cultures have been done then can tailor Abx:
Eg. IV Benzylpenicillin for N.Meningitidis
What is the treatment for suspected meningitis w/ non-blanching rash present in the community?
Suspected Meningococcal infection:
Urgent/immediate IM Benzylpenicillin
Prior to immediate transfer to a hospital (unless it will cause a delay)
What are some complications of meningitis?
Hearing loss is a key complication
Seizures and epilepsy
Cognitive impairment and learning disability
Memory loss
Cerebral palsy, with focal neurological deficits such as limb weakness or spasticity
What are some Differential Diagnoses of Meningitis?
‘Worst headache ever’ - subarachnoid haemorrhage (especially if trauma, ‘thunderclap onset’)
migraine
Encephalitis
flu and other viral illnesses
sinusitis
brain abscess
malaria
What are some specific complications related to meningococcal meningitis?
Risk of DIC
Risk of Waterhouse Friedrichsen Syndrome
What is Waterhouse Friedrichsen Syndrome?
Adrenal insufficiency caused by intra-adrenal haemorrhage as a result of meningococcal DIC
What is the most common cause of fungal meningitis?
Cryptococcus Neoformans
Candida
Only really affects immunocompromised Px
What can be given to close contacts of a Px who has Meningitis?
Rifampicin
Ciprofloxacin
What is Encephalitis?
infection of the brain leading to inflammation of the brain parenchyma
What are the causes of Encephalitis?
Viral (most common)
Also bacterial, fungal, parasitic, paraneoplastic
What is the most common cause of encephalitis?
HSV-1
Accounts for 95% of the cases
Also CMV, EBV, HIV, VZV
What are the risk factors of encephalitis?
Immunocompromised
Extremes of age
Transfusion/Transplantation
Why can close contact with cats be a risk factor for encephalitis?
Risk of toxoplasmosis infection
What are the symptoms of encephalitis?
Px often has preceding flu like Sx
Triad Of:
Fever
Headache
Altered GCS: Behavioural changes/Psychotic behaviour/mood changes
Confusion
Focal Neurological deficit
Memory loss
Seizures
What are the clinical signs of encephalitis?
Pyrexia
Reduced GCS
Focal neurological deficit:
Aphasia
Hemiparesis
Cerebellar signs
What is the most common place to be affected by encephalitis?
Temporal and inferior frontal lobe
What are the primary investigations for encephalitis?
Bloods - FBC, CRP, U&Es
MRI head – to show swelling/ inflammation ± midline shifting due to raised ICP
EEG – shows periodic sharp and slow waves
Lumbar puncture (after): raised lymphocytic CSF, viral PCR
HIV TEST – acute phase viraemia can cause encephalitis
What is the treatment of encephalitis?
Mostly Supportive
IV Acyclovir - for HSV/VZV
What is multiple sclerosis?
Chronic progressive autoimmune, T-cell mediated inflammatory disorder of the CNS against the myelin basic protein of oligodendrocytes causing demyelination of CNS neurons.
It occurs sporadically over years
What type of reaction is MS?
Type 4 Hypersensitivity Reaction:
T-Cell mediated
What is the Epidemiology of MS?
Women to Men – 2:1
Usually diagnosed between 20-40 = disease of the young
More common the further from the equator you go (possible a link to Vitamin D) – explains why it is exacerbated by heat
What are the risk factors for MS?
Females
20-40
Autoimmune disease
FHx - Associated with HLA-DR2
EBV exposure in childhood
What is the pathophysiology of MS?
Type IV Hypersensitivity Rxn:
T-cell mediated – T cells activate B cells to produce auto-antibodies against Basic myelin protein of oligodendrocytes
T Lymphocytes manage to cross the BBB, they can cause a cascade of destruction to the neuronal cells in the brain by recruiting other immune cells
This results in plaques of demyelination and inflammation and therefore conduction disruption along axons
Although the myelin sheath does regenerate, the new myelin is less efficient and temperature dependent
Therefore Symptoms are exacerbated by heat
Where do the plaques of Demyelination occur in MS?
Often Perivenular (but can occur anywhere in the CNS)
Commonly at:
Optic nerves - blurred vision
Ventricles of the brain
Corpus Callosum
Brainstem and cerebellum connections
Cervical Spinal Cord
What are the features of pathological lesions in Active MS?
- Demyelination – breakdown products present
- Variable oligodendrocytes
- Hypercellular plaque edge due to infiltration of tissue with inflammatory cells
- Perivenous inflammatory infiltrate (mainly macrophages and T-lymphocytes)
- Extensive BBB disruption
- Older active plaques may have central gliosis
What are the features of pathological lesions in Inactive MS?
- Demyelination – breakdown products absent
- Variable oligodendrocytes loss
- Hypocellular plaque
- Variable inflammatory infiltrate
- Moderate to minor BBB disruption
- Plaques gliosed
What are the different Classifications of MS?
- Pattern 1 – macrophage mediated
- Pattern 2 – antibody mediated
Lots of inflammation, those patients respond to certain therapies such as plasma exchange - Pattern 3 – distal oligodendrogliopathy and apoptosis (ischaemic/toxic, virus induced)
- Pattern 4 – primary oligodendroglia degeneration (metabolic defect)
What are the types of MS Progression?
Relapse - Remitting:
The most common pattern (85% of cases)
Episodic flare-ups (may last days, weeks or months), separated by periods of remission
Secondary Progressive:
Starts with relapse remitting
Gets progressively worse w/o remission
Primary Progressive:
Symptoms get progressively worse from disease onset with no periods of remission
Progressive/relapsing:
Progressive disease from onset, with clear acute relapses, with or without full recovery, with periods between relapses characterised by continuing progression
What is Uhtohoff’s Phenomenon?
worsening of neurological symptoms in MS when body gets overheated from hot weather, exercise, saunas and hot tubs, showers
What percentage of MS patients progress from relapse remitting to secondary progressive MS?
60-75% progress within 15 years
What are the causes of MS?
Unclear cause for demyelination but:
Multiple genes
Epstein–Barr virus (EBV)
Low vitamin D
Smoking
Obesity
combination of these have an influence
What is the presentation of MS?
Variable presentation depending on region affected
Sx progress over 24 hrs and can last days-weeks and then Improve
What are the General symptoms of MS?
Charcot’s Triad:
- Dysarthria - Plaques in the brainstem
- Intention Tremor - Plaques along motor pathways
- Nystagmus - Plaques in nerves of eyes
- Spinal cord
Weakness
Paraplegia
Spasticity
Tingling
Numbness
- Optic nerves
Impaired vision
Eye pain
Medulla and pons
Dysarthria
Double vision
Vertigo
Nystagmus
- Cerebellar white matter
Dysarthria
Nystagmus
Intention tremor
Ataxia
What are the clinical signs of MS?
- Typical – LOSS NB
L - Lhermitte’s sign - electric shock runs down back and radiates to limbs
O - Optic neuritis – impaired vision and eye pain
S - Spasticity and other pyramidal signs
S - Sensory symptoms and signs
N - Nystagmus, double vision and vertigo - 6th nerve palsy
B - Bladder and sexual dysfunction
- Exacerbated by heat – showers, hot weather, saunas (Uhthoff’s phenomenon)
- Improved by cool temperatures
What are the primary investigations for MS?
Clinical Dx (using McDonald criteria) and Sx progression/remission
Contrast MRI of Brain and spinal cord:
Used to support Dx by identification of demyelinating plaques
LP w/ CSF Electrophoresis - may show oligoclonal IgG bands in CSF
Evoked Potentials - Test length of time of impulse travel
What is the McDonald Criteria?
Criteria used to diagnose MS:
- 2 or more CNS lesions disseminated in time and space
- Need to be sure it affects two parts of nervous system e.g. brain and spinal cord, optic nerve and brain
- More than one attack spaced out in time e.g. optic neuritis last year, now present with weakness in both legs
What would the results of the McDonald Criteria be to diagnose MS?
2 or more relapses
AND EITHER
Objective clinical evidence of 2 or more lesions
OR
Objective clinical evidence of one lesion WITH a reasonable history of a previous relapse
(Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potential)
Who manages MS?
MDT:
including neurologists, specialist nurses, physiotherapy, occupational therapy and others.
How can acute relapses of MS be treated (Relapse-Remitting MS)?
Steroids:
IV Methylprednisolone
+ Sx treatment of Complications:
Depression - SSRIs
Neuropathic pain - Gabapentin
Exercise - maintain strength
How can Chronic MS be treated long term?
1st line (frequent relapse):
– SC beta interferon and glatiramer acetate
2nd line (disease modifying):
- IV alemtuzumab – CD52 monoclonal antibody that targets T cells
- IV 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 are the side effects to Beta Interferon injections used to treat MS?
Injection site reactions
Flu-like symptoms
Mild intermittent lymphopenia
Mild to moderate rises in liver enzymes
Most common S/E (e.g. flu-like symptoms) tend to decrease after the first few months of treatment
What is Guillain Barre Syndrome?
Guillain-Barré syndrome (GBS) is an autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system, often triggered by infection (URTI/GI)
“Equivalent to MS for the PNS”
What is the Epidemiology of Guillain Barre Syndrome?
More common in males
Peak ages 15-35 and 50-75
Most common acute polyneuropathy
What are the causes of Guillain Barre Syndrome?
Usually triggered post infection (6 weeks) and strongly associated with:
Campylobacter jejuni (most common)
CMV
EBV
What is the most common cause of Guillain Barre Syndrome?
Post C. jejuni infection
What is the most common type of Guillain Barre Syndrome?
What are the 3 classical symptoms of this type?
Acute demyelinating inflammatory polyneuropathy (ADIP) in 90% of cases
Presents with:
Progressive symmetrical weakness in limbs
Reduced or absent tendon reflexes
Reduced sensation
What is the pathophysiology of Guillain Barre Syndrome?
Molecular Mimicry:
A pathogenic antigen (e.g. Campylobacter jejuni) resembles myelin gangliosides in the peripheral nervous system
The immune system targets the antigen of pathogen and subsequently and attacks the similar antigen of the myelin sheath of sensory and motor nerves Schwann Cells
Nerve cell damage consists of damage to the Schwann cells and therefore segmental demyelination 🡪 reduction in peripheral nerve conduction 🡪 an acute polyneuropathy
This autoimmune process involves the production of anti-ganglioside antibodies (anti-GMI is positive in 25% of patients)
The demyelination causes polyneuropathy
What are the risk factors for Guillain Barre Syndrome?
History of respiratory or GI infections 1-3 weeks prior to onset
Vaccinations have been implicated e.g. flu vaccine
Post pregnancy – incidence decreases during pregnancy but increases in months after delivery
What are the symptoms of Guillain Barre syndrome?
Recent Hx of Infection (GI/URTI)
Paraesthesia and numbness
Muscle weakness
Back and limb pain
CN involvement – diplopia and dysarthria
Respiratory – affects diaphragm 🡪 death
- Autonomic features
Sweating
Raised pulse
Postural hypotension
Arrhythmias
What are the clinical signs of Guillain Barre Syndrome?
Reduced sensation in limbs
Ascending Symmetrical weakness - lower extremities first (proximal then distal)
Loss of deep tendon reflexes (hyporeflexia)
Autonomic dysfunction - Tachycardia, HTN, postural hypotension etc.
Respiratory distress - RF in 35% of patients
What are the primary investigations for Guillain Barre syndrome?
Lumbar Puncture (L4) - Raised protein + normal WCC = inflammation but no infection
Nerve conduction studies:
Conduction velocity reduced/blocked
Lung Function Tests (spirometry) - Decreased FVC
What investigations can aid to confirm the diagnosis of Guillain Barre?
Ix to exclude other causes:
TFTs - Hypothyroidism for cause of weakness
U&Es - Electrolytes for neuropathic issues
Spirometry - measure lung function
What is the management of Guillain Barre syndrone?
First line:
IV Ig (immunoglobulin) for 5 days - reduces duration and severity of paralysis
+ Plasmapheresis - to remove autoantibodies
LMWH (enoxaparin) reduce risk of venous thrombosis
What is the prognosis of Guillain Barre?
Good prognosis
85% make a full recovery
When may Ig treatment for Guillain Barre be contraindicated?
If patient is IgE deficient
May cause an allergic reaction
What are some Differential Diagnoses of Guillain Barre Syndrome?
Other causes of NM paralysis
Hypokalaemia
Polymyositis
Myasthenia gravis
Botulism
Poliomyelitis
What is Huntington’s Chorea (HD)?
A Neurodegenerative Autosomal dominant genetic condition causing chorea characterised by the lack of the main inhibitory neurotransmitter GABA
What is the penetrance of HD?
Full penetrance:
All genotypes of Huntington’s will express the phenotype
Also shows Anticipation
What are the genetics related to HD?
Huntington’s chorea is a “trinucleotide repeat disorder” that involves a genetic mutation in the Huntingtin (HTT) gene on chromosome 4.
Need >36 Triplet CAG repeats affecting HTT gene to be diagnostic of HD
What is a common feature of tri-nucleotide repeat disorders in terms of prevalence?
They Show anticipation:
where successive generations have more repeats in the gene, resulting in:
Earlier age of onset
Increased severity of disease
What is the Pathology of Huntington’s Chorea?
Repeated CAG sequence leads to translation of an expanded polyglutamine repeat sequence in the huntingtin gene
The more CAG repeats present, the earlier symptoms present
Faulty Huntingtin protein builds up in the striatum causing cell death and loss of cholinergic and GABA-nergic neurons 🡪 decreased ACH and GABA synthesis in striatum
Less GABA causes less regulation of dopamine to striatum causing increased dopamine levels resulting in excessive thalamic stimulation and subsequently increased movement (chorea)
When do symptoms of HD usually present?
Often ASx in patients under 30 yrs
Sx present between 30-50 yrs initially with a prodromal phase of mild Sx
People with HD will have more severe symptoms typically around 60+ yrs
What are the symptoms of HD?
Insidious progressive worsening:
Starts with cognitive, psychiatric and mood problems (around middle age)
Then can progress to:
Chorea (involuntary, abnormal movements)
Eye movement disorders
dysarthria
dysphagia
How is a Diagnosis of HD made?
Clinical diagnosis with FHx HD and subsequent generations showing anticipation.
Genetic testing - >36 CAG repeats on chromosome 4
MRI/CT - Shows atrophy of striatum
What is the treatment of HD?
No Tx to stop or progress HD
Provide Extensive counselling and support
Can provide Sx treatment for chorea:
Chorea:
Antipsychotics (olanzapine) - Dopamine Receptor antagonists
Benzodiazepines (diazepam)
Dopamine-depleting agents (tetrabenazine)
Depression - SSRI (sertraline)
What is the prognosis of HD?
Progressive uncurable condition
Life expectancy of 15-20 years post diagnosis
Death in HD most commonly caused by aspiration pneumonia or Suicide
What are some differential diagnoses for chorea such as in HD?
HD
Sydenham’s chorea (rheumatic fever)
SLE
Basal ganglia stroke
Wilson’s disease
What are the causes of depression?
Psychiatric:
Anxiety
Alcohol abuse
Substance misuse
Grief
Physical:
Parkinson’s
MS
Endocrine
Medication – Beta-blockers, combined OCP
CV accident (stroke)
Learning difficulties
What are the symptoms of Depression?
Low mood – loss of interest in activities they used to enjoy
Appetite and weight change
Sleep pattern changes – sleeping too much/difficult sleeping
Fatigue
Lack of concentration or making decisions
Feelings of worthlessness or excessive/inappropriate guilt
Self-harm and suicide
How can Depression be treated/managed?
Mental health team – crisis team - IAPT
CBT
Counselling
Medication
Selective Serotonin Reuptake Inhibitors e.g. fluoxetine, sertraline
Prevent the presynaptic neuron from reabsorbing serotonin so more remains in synaptic cleft and can stimulate receptors on post synaptic neuron (in pre-frontal cortex)
Tricyclic antidepressants e.g. amitriptyline and amoxapine
Act on muscarinic receptors
Information/education
What is Parkinson’s Disease (PD)?
A progressive neurodegenerative movement disorder characterised by loss of dopaminergic neurones within the substantia nigra pars compacta (SNPC) of the basal ganglia (nigrostriatal pathway).
What is the Epidemiology of PD?
The second most common neurodegenerative disorder after dementia
Prevalence increases with age (peak onset 55-65)
More common in males
What are the risk factors for PD?
Increased age
Male
FHx
What is the peak age of onset for PD?
55-65 yrs
What are the causes of PD?
Idiopathic condition but potentially related to genetics
There are secondary causes of parkinsonism:
Drugs/Toxins - CO
Wilsons disease
Infections - Encephalitis, Creutzfeldt-Jakob Disease
What are the genetic factors related to PD?
Mutation in Parkin Gene
Mutation in alpha-Synuclein gene
What is the pathogenesis of PD?
- Neurodegenerative loss of dopamine secreting cells from the pars compacta of the substantia nigra that project to the striatum
- Reduced striatal dopamine levels
- Less dopamine means the thalamus will be inhibited resulting in a decrease in movement
- symptoms of Parkinson’s
- Under the microscope you will see the presence of intracytoplasmic intrusion bodies in the remaining neurones: Lewy bodies
What are the typical Parkinsonism Symptoms?
Bradykinesia
Resting Tremor
Rigidity
What are the symptoms of PD?
Parkinsonism (bradykinesia, Resting Tremor, Rigidity)
Postural Instability
Anosmia - early sign
Constipation
Sleep disturbances
Cognitive/memory problems
Depression
What are the clinical signs of PD?
Signs are often Asymmetrical (unilateral)
Pin rolling tremor of thumb
Lead pipe arm (reduced arm swing)
Shuffling gait
Cogwheel movement
What makes up a diagnosis of PD?
Clinical Dx based on Sx and Examination and Hx
- Bradykinesia + 1 or more other Cardinal Sx
DaTSCAN - Imaging to dopaminergic terminals in Str
MRI/Head CT - may show SNpc Atrophy
What is the treatment for PD?
No cure so Sx management
- Levodopa (synthetic Dopamine) to increase levels (as dopamine cannot cross BBB)
+ Benserazide or Carbidopa - Dopa Decarboxylase inhibitors
(eg. Carledopa (Levodopa + Carbidopa)) - Dopamine Agonists - Bromocriptine
- COMT/MAO-B Inhibitors - Rasagiline (COMT) / Selegiline (MAO-B)
Surgical - Deep Brain Stimulation
What is the issue with using levodopa to treat PD?
Initially works well but soon the Px becomes resistant to it and the effects wear off.
Therefore want to only use it when Sx are bad enough to prevent early resistance.
What is an important differential diagnosis to PD?
Lewy body dementia (often associated with parkinsonism)
Parkinson Sx then Dementia = Parkinson dementia
Dementia then Parkinson Sx = Lewy body dementia w/ Parkinsonsim.
What is dementia?
A syndrome caused by neurodegeneration of various causes resulting in progressive reduction of cognition and difficulty with ADLs
What is the epidemiology of dementia?
Rare under 55 years
Prevalence rises with age
Alzheimer’s most common – more common in females
Vascular and mixed dementias are more common in males
Autosomal dominant – 10-20% of developing it
What is the commonest cause of dementia?
Alzheimer’s Disease (AD)
Accounts for 60-75% of all dementias
What is the epidemiology of AD?
Rarely affects under 65yrs
(otherwise EOAD which accounts for 3% of cases)
Affects more females than males
Prevalence significantly rises with age
What is the pathophysiology of AD?
Accumulation of Beta amyloid plaques (Ab plaques) and Tau Neurofibrillary tangles (NFT) in the cerebral cortex.
This leads to cortical scarring and brain atrophy
Additionally, damage to ACh neurons leading to poor neurotransmission
What is the APP protein?
Amyloid Precursor protein that is pathologically cleaved into Beta Amyloid plaques that accumulate in the brain (AD) and other tissues (in amyloidosis)
What are the risk factors for AD?
Increasing age
Downs Syndrome
APP gene mutation
APOE4 allele for familial AD (linked to EOAD)
What are the symptoms of AD?
Agnosia - cant recognise things
Apraxia - Cant do basic motor skills
Aphasia - Speech difficulties
Amnesia - Memory impairment
What is the progression of cognitive decline like in AD?
Steady progressive decline
What is the second most common cause of dementia?
Vascular Dementia
Accounts for roughly 20% of cases
What is Vascular Dementia?
Cognitive decline that occurs in a stepwise manner due to a Hx of cerebrovascular events.
What is the pathophysiology of Vascular dementia?
Hx of cerebrovascular events leads to infarction and loss of brain cells/damaged tissue.
Neurons cannot regenerate so the neurodegeneration is permanent.
Increased risk of secondary cerebrovascular events can lead to further brain damage and cognitive decline.
What are the symptoms of vascular dementia?
Signs of vascular pathology:
Raised BP
Hx of cerebrovascular events
Focal CNS signs
General cognitive decline in a stepwise manner
What is Lewy Body Dementia?
3rd most common form of dementia
Alpha-Synuclein and Ubiquiting aggregates accumulate in the cerebral cortex
Results in cognitive decline.
What are the symptoms of Lewy Body Dementia?
Cognitive decline
+ Parkinsonism
What is Frontotemporal dementia?
An uncommon form of dementia (5% of cases)
Specific degeneration of frontotemporal lobes often causing speech and language deterioration and Thinking/memory problems
What are the risk factors for Frontotemporal dementia?
FHx
Autosomal dominant mutation in Tau protein (chromosome 17)
FHx of MND (TDP-43)
What is the Diagnostic test for dementia?
Mini Mental State Exam (MMSE) /30
>25 = normal
18-25 = impaired
<17 = severely impaired
Brain MRI - May show cortical atrophy
What is the treatment for dementia?
Mostly Conservative:
Social stimulation/interaction
Exercise and healthier lifestyle
What pharmacological treatment can be given to aid Alzheimer’s Disease?
Sx management:
First line: AChE inhibitors (rivastigmine, neostigmine)
NMDA antagonist - memantine
SSRIs - treat BPSD (behaviour and psychological Sx of dementia)
What Pharmacological therapy can be given to aid vascular dementia?
Antihypertensives - Ramipril
To reduce risk of subsequent cerebrovascular events
Control CVD risk factors
What are the primary headaches?
No underlying cause relevant to headache:
Migraine
Cluster
Tension
(Trigeminal Neuralgia)
What are the secondary Headaches?
Due to underlying conditions:
GCA
Infection
SAH
Trauma
Cerebrovascular disease
Eye, Ear Sinus pathology
Drug overdose
Define a Migraine?
Recurrent throbbing headache often preceded by an aura and associated
with nausea, vomiting and visual changes
What is the epidemiology of a Migraine?
Most common cause of episodic (recurrent) headaches
More common in females
90% onset in under 40 yrs
(if onset > 50 yrs then think pathological)
What happens to Migraine severity with age?
Severity of Migraine typically decreases with advancing age
What are the potential triggers of a migraine?
CHOCOLATE:
- Chocolate
- Hangovers
- Orgasms
- Cheese
- Oral contraceptives
- Lie-ins
- Alcohol
- Tumult - loud noise
- Exercise
What are the different Types of migraine?
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
What is the typical progression of migraines?
Occur in attacks that tend to follow a typical pattern:
Prodrome
Aura
Throbbing headache
Resolution
Post-dromal/recovery
Explain the progression of a Migraine?
Prodrome: Days prior to attack
Mood changes
Aura: last for 60mins
Vision changes minutes before attack (attack follows soon after)
ZigZag lines
Throbbing Headache: 4-72hrs
(Hemiplegic in some cases)
Resolution:
Headache fades away slowly
Recovery
What is the Clinical Dx of a migraine?
2 or more of:
Unilateral pain
Throbbing
Motion Sickness
Mood severely intense
PLUS
1 or more of:
Nausea
Vomiting
Photophobia/Phonophobia
W/ A NORMAL NEURO EXAM
What is the diagnostic criteria for a Migraine without Aura?
4 diagnostic criteria (ABCD):
A – 5 attacks fulfilling B-D
B - attacks last 4-72 hours
C – two of the following
Unilateral
Pulsing
Moderate/severe
Aggravated by routine physical activity
D – during headache at least one of
Nausea and/or vomiting
Photophobia (light sensitive) and phonophobia (sound sensitive)
What is the Diagnostic Criteria for a Migraine with an Aura?
3 diagnostic criteria (ABC):
A – at least 2 attacks fulfilling B and C
B - > 1 reversible aura symptom
Visual – zigzags, spots
Unilateral sensory – tingling, numbness
Speech – aphasia
Motor weakness – known as “hemiplegic migraine” so rule out stroke and TIA
C > 2 of the following 4
> 1 aura symptoms spread gradually over 5 minutes and/or > 2 aura symptoms occurring in successions
Each aura symptom lasts 5-60 minutes
> 1 aura symptom is unilateral
Aura accompanied/followed within 60 minutes by headache
How are migraines diagnosed?
Clinical Dx unless pathology is suspected where you do tests to exclude DDx:
Neuro imaging
LP
What are the treatments for Migraines?
Conservative - Avoid Triggers
Acute Mx:
Mild - Paracetamol/NSAIDs - ibuprofen
Severe - Triptans - sumatriptan
Anti-emetics - metoclopramide
Prophylaxis:
1st line – Propranolol (beta-blocker) or Topiramate (anti-convulsant)
2nd line – acupuncture
3rd line – Amitriptyline (tricyclic anti-depressant)
4th line – Botulinum toxin type A (only if not responded to 3 prior Tx)
What is the mechanism of action of Triptans?
Why are they used for migraines?
5-HT receptor agonists (serotonin) that are used to abort migraines when they start to develop
What are cluster headaches?
Severe unilateral headaches often periorbital that come in clusters of attacks
How long can a cluster headache last for?
Clusters of attacks may last for weeks/months before a pain free period follows which may last for years
What is the primary feature of a cluster headache?
Periorbital unilateral pain
Extremely painful (most disabling of primary headaches)
Who typically presents with Cluster headaches?
Males
30-50 yrs old
What are the risk factors for cluster headaches?
Male
Smokers
30-50yrs old
Genetics (autosomal dominant link)
What are the types of Cluster headaches?
Episodic - > 2 cluster periods lasting 7 days to 1 year separated by pain free periods lasting > 1 month
Chronic – attack occur for > 1 year without remission or with remission lasting <1 month
What is the presentation of cluster headaches?
- Cluster headache – can take 3 years to get a diagnosis
- A – At least 5 headache attacks fulfilling B-D
- B – Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes if untreated and rises to crescendo
- C – headache is accompanied by ipsilateral cranial autonomic features and/or sense of restlessness or agitation
- D – attacks have a frequency from 1 every other day to 8 per day
Deemed to have a boring/hot poker characteristic - Associated with ipsilateral eye lacrimation and redness, rhinorrhoea (blocked nose), miosis (excessive pupil constriction) and/or ptosis (drooping or falling of upper eyelid)
- Usually occur in middle of the night or morning hours
What is the diagnosis of cluster headaches?
Clinical Dx
5 or more similar attacks fulfilling criteria
What is the treatment of cluster headaches?
Acute Mx:
Triptans - SC Sumatriptan or IM Zolmitriptan
High flow 100% oxygen
Prophylaxis:
Verapamil (CCB)
What are Tension Headaches?
The most common form of a primary headache which has a characteristic rubber band feeling around the head.
What is the Epidemiology of Tension headaches?
Most common primary headache
Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)
What are the causes of Tension Headaches?
Neurovascular irritation which refers to scalp muscles and soft tissues
MC SCOLD:
Missed meals
Conflict
Stress
Clenched jaw
Overexertion
Lack of sleep
Depression
What is the Presentation of a Tension headache?
- A - > 10 attacks occurring <1 day/month (<12 days/year) and fulfilling B-D
- B - Headache lasting from 30 minutes to 7 days
- C – headache has two of the following
Bilateral
Pressing/tightening (non-pulsatile) quality
Mild or moderate intensity/pain
Not aggravated by routine physical activity (e.g. walking or climbing stairs) - D – both of the following
No nausea or vomiting (anorexia may occur)
No more than one of photophobia and phonophobia - E – not attributed to another disorder
What is the diagnosis for a tension headache?
Clinical Diagnosis from Hx
What is the treatment for a Tension headache?
Stress relief
Avoid triggers
Simple analgesia:
Aspirin/Paracetamol
What is the main risk factor/trigger for a tension headache?
STRESS
What is Trigeminal Neuralgia?
A chronic, debilitating condition resulting in intense and extreme episodes of pain
What are the risk factors for Trigeminal Neuralgia?
Increased age
MS
Female
HTN
What is the Epidemiology of Trigeminal Neuralgia?
Females
50-60 yrs
Increases with age
Almost always Unilateral
What are the causes of Trigeminal Neuralgia?
Compression of the trigeminal Nerve that leads to excitation and erratic pain:
- Vein or artery compressing CN5
- Local Pathology pressing on CN5 (tumours/Aneurysms)
- CN5 nerve lesion
What is the headache like in Trigeminal Neuralgia?
Electric Shock Pain that lasts for seconds to minutes across the face
90% unilateral
10% bilateral
What is the diagnostic criteria for trigeminal neuralgia?
Clinical Dx
3 or more attacks with characteristic unilateral facial pain and Symptoms
MRI - exclude secondary causes/other pathology
What are the treatment options for trigeminal neuralgia?
First Line:
Carbamazepine (anticonvulsant)
Second Line:
Phenytoin/Gabapentin (analgesic targeted for neuropathic pain)
Surgery possible to decompress/intentionally damage nerve
Overview of Primary Headache Features:
Duration
Site
Character
Severity
Acute Tx
Duration:
MH - 4-72 hrs
TH - Minutes to dates
CH - 15-180 mins
TGN - Few seconds
Site:
MH - Unilateral
TH - Bilateral
CH - Retro-orbital/unilateral
TGN - unilateral V1/2/3 distribution
Character:
MH - Throbbing
TH - Pressing/tight band
CH - Hot poker/boring
TGN - Electric shock/stabbing
Severity:
MH - Moderate-severe
TH - Mild to moderate
CH - Severe to very severe
TGN - Severe to very severe
Acute Tx:
MH - Triptan
TH - Paracetamol
CH - Triptan/100% O2
TGN - Carbamazepine
Define a Seizure?
A paroxysmal alteration of neurological function as a result of excessive, hypersynchronous discharge of neurons within the brain
Define Epilepsy?
A neurological disorder characterised by an increased tendency to have recurrent seizures that are idiopathic and unprovoked.
(>2 episodes more than 24hrs apart)
What are the different causes of seizures?
VITAMIN DE:
Vascular
Infection
Trauma
Autoimmune - SLE
Metabolic - Hypocalcaemia
Idiopathic - Epilepsy
Neoplasms
Dementia + Drugs (cocaine)
Eclampsia + everything else
What are the risk factors for developing epilepsy?
Familial inherited
Dementia (10x more likely)
Drug use
Cerebrovascular events
What is the epidemiology of epilepsy?
One of the most common neurological disorders
2% of UK population will have 2 or more seizures
Highest prevalence at extremes of life (<20yr or >60yrs)
What is the pathophysiology of seizures?
Disrupted neurological function resulting in an imbalance between inhibition and excitation of neurons.
Balance of GABA and Glutamate shifts towards glutamate
Therefore more excitatory stimulation
What are the different types of seizures?
Epileptic vs Non-Epileptic:
Generalised
Focal
Define an Epileptic Seizure
Paroxysmal event in which changes of behaviour, sensation or cognitive processes are caused by excessive (too much voltage), hypersynchronous neuronal discharges in the brain
What are the characteristics of an epileptic seizure?
Duration – 30-120 seconds
Positive ictal symptoms – excess of something
Seeing/hearing something that isn’t there
Feeling touch when you aren’t being touched
Negative postictal symptoms
May occur from sleep
May be associated with other brain dysfunction – bleeds, stroke, tumours etc.
Typical seizure phenomena – lateral tongue bite, déjà v
Define a Non-Epileptic Seizure?
Paroxysmal event in which changes in behaviour, sensation and cognitive function caused by mental processes associated with psychosocial distress
What are the characteristics of a Non-epileptic Seizure?
Situational
Duration 1 – 20 mins
Dramatic motor phenomena or prolonged atonia
Eyes closed
Ictal crying and speaking
Surprisingly rapid or slow postictal recovery
History of psychiatric illness, other somatoform disorders
What are the subtypes of generalised seizures?
Tonic Clonic
Absence
Toni
Myoclonic
Atonic
What are the subtypes of Focal Seizures?
Simple focal
complex focal
How are Seizures classified?
Where the seizures began
Level of awareness during the seizure
Other features of the seizure e.g. motor
What are generalised seizures?
A seizure that starts within both hemispheres of the brain at onset.
They are bilateral
ALWAYS a loss of consciousness
What are Focal Seizures?
Seizures that originate within one side of the brain and are usually confined to one region.
They may progress to secondary lobes (Focal-bilateral seizures)
What is the clinical progression of an epileptic seizure?
Prodrome: (days before)
Mood changes
Pre-Ictal: (minutes before)
Aura
Seizure trigger
Automatisms - lip smacking/rapid blinking
Ictal Event: The Seizure
Post-Ictal Period: (30 mins after)
Headaches
Confusion / reduced GCS
Todd’s Paralysis
Amnesia
Sore tongue
What are the features of a Tonic Clonic seizure?
No aura
Loss of consciousness
Tonic phase - rigidity/muscle tensing (fall to floor)
Clonic phase - Jerking of limbs/muscles
Upgazing open eyes
Incontinence
Tongue bitten
What is the management of a Tonic Clonic Seizure?
First line: sodium valproate
Second line: lamotrigine or carbamazepine
What is an Absence Seizure?
Typically in children:
Px stares blankly into space (seconds to mins)
Unaware of surrounds and will not respond.
Will then carry on as normal after the seizure.
What is a tonic seizure?
A seizure where there is just rigidity/muscle tensing
What is a Myoclonic seizure?
A seizure where there is just jerking limbs
What is an Atonic Seizure?
Sudden floppy limbs and muscles
They may be indicative of Lennox-Gastaut syndrome
What is a febrile convulsion?
Seizures that occur in children whilst they have a fever.
They are not caused by epilepsy or underlying pathology.
What parts of the brain are affected in a generalised Seizure?
All the cortex and deep brain structures bilaterally affected.
What parts of the brain are affected in a simple focal seizure?
The Focal region of cortex
NO basal ganglia/thalamus involvement
What parts of the brain are affected in a complex focal seizure?
The focal region of cortex
Basal ganglia and thalamus are involved.
What is a Simple Focal Seizure?
No Loss of consciousness
The patient is awake and aware
Will have uncontrollable muscle jerking and may be unable to speak
What is a Complex Focal Seizure?
There is loss of Consciousness
Patient is unaware
What are the symptoms of Focal seizures?
Dependent on where the focal deficit is located
What is the most common region affected in a focal seizure?
Temporal lobe
What are the features of a Temporal focal seizure?
Oral automatism: lip smacking, chewing, swallowing
Manual automatisms: picking, fumbling
Automatic behaviour: running, walking
Auras: déjà vu, feeling of fear, unpleasant smells
Auditory: buzzing, ringing, vertigo
What are the features of a Frontal focal seizure?
Predominantly motor symptoms: pelvic thrusting, bicycling, tonic posturing
Sexual automatisms
Bizarre behaviour
Vocalisations
What are the features of a Parietal focal seizure?
Parasthesias
Visual hallucinations
Visual illusions
More subjective and difficult to diagnose than other areas
What are the features of a Occipital focal seizure?
Visual hallucinations
Transient blindness
Rapid and forced blinking
Movement of head or eyes to the opposite side
What is required for a diagnosis of Epilepsy?
Must have had 2 or more seizures MORE THAN 24 hrs apart to be considered:
CT/MRI head - Examine HIPPOCAMPUS, check for bleeds and trauma
EEG: 3H2 wave in absence
Bloods: rule out metabolic/infection
What is the Treatment for epilepsy and seizures?
Sodium Valporate for generalised epilepsy
Carbamazepine for focal seizures
Surgical resection possible
What is a contraindication for using Sodium Valporate?
All females of childbearing age (15-45)
Sodium Valporate is Teratogenic
Instead use Lamotrigine
What is a complication of Epilepsy?
Status Epilepticus (NEURO EMERGENCY)
Epileptic seizures without a break back to back
OR
Seizure lasting >5 minutes
What are the symptoms of STATUS EPILEPTICUS?
Post ictal confusion
Todd’s palsy – paralysis in arms or leg (usually isolated to one side of the body
Can last ~15 hours and usually subsides in around ~2 days
Temporary and severe suppression of seizure-affected area
What is the treatment for Status Epilepticus?
Benzodiazepines:
Lorazepam 4mg IV
If not worked then Lorazepam again.
Then Phenytoin if second dose doesnt work.
What are the differences between an epileptic and non-epileptic seizure?
Epileptic Seizures have:
Eyes OPEN
Synchronous Movements
Can occur in sleep
What must you do if you are diagnosed with Seizures?
Inform DVLA
Cannot drive until seizure free for one year
What is the length of the spinal cord?
From C1-L1/2
Where is the conus medullaris and cauda equina?
At L1/2
Define Hemiplegia?
Paralysis to one side of the body
(usually due to a brain lesion)
Define Paraplegia?
Paralysis of both legs/lower body
(usually due to a spinal cord lesion)
What does the DCML tract convey information for?
Ascending tract for fine touch, 2pt discrimination and proprioception.
What is the pathway for the DCML
Travels in dorsal route
(Fasciculus Gracilis/Cuneatus)
Decussates in the medulla
What does the spinothalamic tract convey information for?
Ventral: Crude Touch and pressure
Lateral: Pain and temperature
What is the pathway for the spinothalamic tract?
Ascending:
Enters at spinal level of nerve
ascends 1-2 spinal levels and then decussates
What information does the corticospinal tract convey?
Upper motor neurons for movement.
Decussates at the medulla
What are the nerve routes for the Knee jerk reflex?
L3/4
What are the nerve routes for the Big toe reflex?
L5
What are the nerve routes for the ankle Jerk reflex?
S1/2
What are the commonest causes of spinal cord injury?
Trauma
Vertebral compression fractures
Intervertebral disc disease - prolapse/herniation
Tumours
Infection
What are the common vertebral body neoplasms that can cause spinal compression?
Mets from:
Lungs
Breast
RCC
Melanoma
What are the different types of spinal cord injury?
Complete SC injury
Anterior SC injury
Posterior SC injury
Central SC injury
Brown-Sequard Syndrome
What is Spinal Cord Compression?
Compression of the spinal cord resulting in upper neurone signs and specific symptoms dependent on where compression is
What is the commonest cause of acute spinal cord compression?
Vertebral body neoplasms
What are the main causes of Spinal Cord Compression (myelopathy)?
Tumour (slow onset)
Osteophytes
Disc prolapse (slower onset)
Nucleus pulposus moves and presses against the annulus but it doesn’t escape outside the annulus
Can produce a bulge in the disc which, sometimes, can result in pressure (less pressure than herniation) on nerve root resulting in pain
Disc herniation - centre of disc (nucleus pulposus) has moved out through the annulus (outer part of disc) resulting in pressure on nerve root and pain
What are the symptoms of spinal cord compression?
Sx depend on level of cord compression:
UMN signs below level of lesion – everything goes up
Sensory loss below level of lesion
LMN signs AT level
Bladder sphincter involvement – hesitancy, frequency, painless retention
What is the concern if there is sphincter involvement in spinal cord compression
This is a late and bad sign signalling a poorer prognosis
What would be the features of a complete spinal cord compression?
All motor and sensory function below the SCI level
What would be the features of an anterior spinal cord compression?
Disruption of anterior spinal cord or anterior spinal artery
Loss of motor function below the level
Loss of pain and temperature sensation (anterior column)
Preservation of fine touch and proprioception (posterior column)
What would be the features of a posterior spinal cord compression?
Disruption of posterior spinal cord or posterior spinal artery (rare)
Loss of fine touch and proprioception (posterior column)
Preservation of pain and temperature sensation (anterior column)
What are some differential Diagnoses for Spinal Cord Compression?
Transverse myelitis
MS
Cord vasculitis
Trauma
Dissecting aneurysm
What is the primary investigation if suspecting a Spinal cord compression?
MRI Spinal cord ASAP:
(risk of permanent damage)
CT CAP - if malignancy suspected + biopsy to identify nature
What is the treatment for spinal cord compression?
Neurosurgery to decompress cord
Dexamethasone to reduce inflammation
What is Brown Sequard Syndrome?
Hemi-section of the spinal cord and therefore loss of sensations of pain temperature and touch and motor movement
What are the causes of Brown-Sequard Syndrome?
Space occupying lesions
Intervertebral disc prolapses
Vertebral bone fractures
Trauma – gunshot wounds, knife wounds
Infectious – HIV
MS
What would be the clinical features of brown-Sequard syndrome?
- (Ipsilateral Corticospinal) Ipsilateral weakness and loss of motor function below the lesion.
- (Ipsilateral DCML) Ipsilateral loss of proprioception, 2-point discrimination and fine touch.
- (Contralateral Spinothalamic) Contralateral loss of pain and temperature sensation 1-2 spinal segments below the lesion.
What is the Investigation and Treatment for Brown Sequard Syndrome?
Ix - MRI Spine
Tx - Supportive (physical/occupational therapy) and Steroids (Dexamethasone)
What is Sciatica?
Sciatica refers to the symptoms associated with irritation of the sciatic nerve.
What spinal nerves form the sciatic nerve?
Where is the most common region of compression to cause Sciatica?
L4-S3 spinal roots
Most common region of compression at L5/S1
What is the innervation of the sciatic nerve?
The sciatic nerve supplies sensation to the lateral lower leg and the foot.
It supplies motor function to the posterior thigh, lower leg and foot.
What nerves are branches of the sciatic nerve?
Common peroneal nerve
Tibial nerve
What are the symptoms of Sciatica?
- Unilateral pain from the buttock radiating down the back of the thigh to below the knee or feet
- Paraesthesia (pins and needles), numbness and motor weakness
- Reflexes may be affected depending on spinal root affected
Signs:
Unilateral
Weak plantar flexion
Absent right ankle jerk
Decreased sensation over lateral edge and sole of right foot
What are the main causes of Sciatica?
Intervertebral Herniated/prolapsed disc
Tumours
Piriformis Syndrome
Spondylolisthesis
Spinal stenosis
What is Bilateral sciatica a red flag for?
Cauda Equina syndrome
What is the Socrates mnemonic for?
Assessing pain
S – Site
O – Onset
C – Character
R – Radiation
A – Associations
T – Timing
E – Exacerbating and relieving factors
S – Severity
What are the diagnostic investigations for sciatica?
Clinical Dx generally:
Can’t Do straight leg raise test without pain
May have:
XR
CT
MRI - if cauda equina suspected
What is the main treatment for sciatica?
Physiotherapy + Analgesia:
Amitriptyline
Duloxetine
What is Cauda Equina Syndrome (CES)?
Surgical emergency where the nerve roots of the cauda equina at the bottom of the spine are compressed below the conus medullaris (L2)
What do the nerves of the cauda equina supply?
Sensation to the lower limbs, perineum, bladder and rectum
Motor innervation to the lower limbs and the anal and urethral sphincters
Parasympathetic innervation of the bladder and rectum
What are the main causes of CES?
Large Central Herniated disc (the most common cause)
Tumours, particularly metastasis
Spondylolisthesis (anterior displacement of a
vertebra out of line with the one below)
Abscess (infection)
Trauma
What is the most common cause of CES?
Lumber Herniation L4/5 or L5/S1
What are the main symptoms of CES?
Leg weakness w/ LMN signs (ipsilateral hypotonia, fasciculations, hyporeflexia)
Saddle anaesthesia - perianal numbmess
Bladder/Bowel dysfunction + sphincter involvement
Erectile Dysfunction
What are the main Signs of CES?
Areflexia
Fasciculations
Loss of bowel/bladder control
Urinary retention
What is the diagnostic investigation for CES?
MRI spinal cord (diagnostic)
Testing nerve roots/reflexes
What is the treatment for CES?
Refer to neurosurgeon ASAP to relive pressure
Surgical decompression
High dose dexamethasone
Corticosteroids
What are the red flag signs in CES?
Bilateral sciatica
Bilateral flaccid leg weakness
Saddle anaesthesia
Bladder and bowel dysfunction
Erectile dysfunction
Areflexia
What is the main difference between CES and spinal cord lesions above the CE?
Major difference between cauda equina and lesions higher up in the cord is that leg weakness is flaccid and areflexic (LMN lesion) and not spastic and hyperreflexic (UMN lesion)
Where do the cranial Nerves arise from?
CN1 - Olfactory Bulb
CN2 - Optic Chiasm
CN3 - Interpeduncular fossa
CN4 - Dorsal aspect of Midbrain
CN5 - Lateral Pons
CN6 - Pontomedullary Junction
CN7 - Pontomedullary Junction
CN8 - Pontomedullary Junction
CN9 - Medulla
CN10 - Medulla
CN11 - Medulla (below CN12)
CN12 - Medulla (Above CN11)
What is the causes of Cranial nerve lesions?
Damage to the brainstem:
- Tumour
- MS
- Trauma
- Aneurysm
- Vertebral artery dissection resulting in infarction
- Infection - cerebellar abscess from ear
What are the Symptoms of a CN1 Palsy?
Anosmia - Cant smell
What are the Symptoms of a CN2 Palsy?
Loss of vision in the eye of optic nerve lesion
What are the symptoms of a CN3 palsy?
Ptosis
Down and out eye
Fixed and dilated pupil
Why do you get a down and out eye in a CN3 palsy?
Unopposed action of the Trochlear (CN4) and Abducens (CN6) cranial nerves
Why do you get a fixed and dilated pupil in a CN3 palsy?
Loss of parasympathetic outflow from the Edinger-Westphal nucleus supplying the pupillary sphincter and ciliary bodies.
What are the causes of a CN3 palsy?
- Raised ICP
- Diabetes
- Hypertension
- Giant cell arteritis
What are the symptoms of a CN4 palsy?
Diplopia (double vision) when looking down
“walking down stairs”
What are the causes of a CN4 palsy?
Rare:
Due to trauma of the orbit
What are the symptoms of a CN5 palsy?
- Jaw deviates to side of lesion
- Loss of corneal reflex
What are the causes of a CN5 Palsy?
Trigeminal Neuralgia (pain but no sensory loss)
VZV
nasopharyngeal cancer
What are the symptoms of a CN6 palsy?
Adducted eye
What are the causes of a CN6 palsy?
Raised ICP
MS
Wernicke’s Encephalopathy
Pontine Stroke
What are the symptoms of a combined CN3,4,6 palsy?
Non-Functional Eye
What are the causes of a combined CN3,4,6 palsy?
- Pontine Stroke
- Tumours
- Wernicke’s encephalopathy
What are the signs of a CN7 palsy?
unilateral facial weakness (motor component), altered taste (sensory component), and a dry mouth (parasympathetic component).
What are the causes of a CN7 palsy?
Bells Palsy
Fractures to pteroid bone
Parotic inflammation
Otitis Media
What is Bells Palsy?
Neurological condition that presents with a rapid onset of unilateral facial paralysis
What is clinically relevant about the control of the facial muscles?
The lower half of the faces only has contralateral innervation
Top half has bilateral. forehead sparing
How can you tell if a bells palsy is an UMN or LMN lesion?
UMN injured, lower half on contralateral side is weak but forehead is not as it has contralateral and ipsilateral innervation (bilateral)
LMN - weakness of all the muscles on the ipsilateral side of the face
How do you determine the level of CN7 lesion?
Proximal to pons - loss of all function (lacrimation, salivation, taste, facial weakness)
Lesion above chorda tympani (same as previous but does have lacrimation)
Distal Lesion to the stylomastoid foramen (facial weakness only)
How do you determine the level of CN7 lesion?
Proximal to pons - loss of all function (lacrimation, salivation, taste, facial weakness)
Lesion above chorda tympani (same as previous but does have lacrimation)
Distal Lesion to the stylomastoid foramen (facial weakness only)
What are the symptoms of a CN8 palsy?
Hearing impairment
Vertigo
Loss of balance
What are the causes of a CN8 palsy?
Skull fracture
Otitis Media
Tumours
Compression
What are the symptoms of a CN9 and CN10 palsy?
- Gag reflex issues
- Swallowing issues
- Vocal issues - hoarse voce
What are the causes of a CN9/CN10 palsy?
Jugular foramen lesion
What are the symptoms of a CN11 palsy?
Can’t shrug shoulders/turn head against resistance
What are the symptoms of a CN12 palsy?
Tongue deviation towards the side of the lesion
Define Weakness/paresis
impaired ability to move a body part in response to will
Define Paralysis?
ability to move a body part in response to will is completely lost
Define Ataxia/
willed movements are clumsy, ill-directioned or uncontrolled (usually due to an issue with the cerebellum)
Define Apraxia?
disorder of consciously organised patterns of movement or impaired ability to recall acquired motor skills
What is a Motor Unit?
Basic functional unit of muscle activity
Alpha motor neuron (LMN) + axon + skeletal muscle fibres it innervates
Number of muscles fibres depends on sophistication of the movement e.g. hands and eyes (finely controlled), each motor neuron will only control a few muscle fibres whereas in bulky muscles, each motor neuron will supply more muscle fibres
What is the organisation of Movement?
Idea of movement – association areas of cortex
Activation of upper motor neurons in the pre-central gyrus
Impulses travel to lower motor neurons and their motor units via the corticospinal (pyramidal) tracts
Modulating activity of the cerebellum and basal ganglia
Hence why individuals with cerebellar and basal ganglia disorders can’t control movements properly
Further modification of movement depending on sensory feedback
How is Muscle Tone Regulated?
Stretch receptors in muscle (muscle spindles) innervated by gamma motor neurons
Muscle stretched 🡪 afferent impulses from muscle spindles which 🡪 reflex partial contraction of muscle
Disease states e.g. spasticity and extrapyramidal rigidity alter muscle tone by altering the sensitivity of this reflex
What is Motor Neuron Disease (MND)
Progressive neurodegenerative disease where both upper and lower motor neurons stop functioning but there is no effect on the sensory neurons.
What is the most common form of MND?
Amyotrophic Lateral Sclerosis (ALS)
Accounts for 50% of cases
What are the risk factors of MND?
Increased age >60 yrs
Male
FHx
Smoking
RUGBY
What genetic mutation is linked with MND?
SOD-1 mutation
What is the main motor tract in the body?
Corticospinal tract
UMN from precentral gyrus
Anterior (10% - no decussation)
Lateral (90% - Decussation in Medullary pyramids)
What are the different types of MND?
Amyotrophic Lateral Sclerosis (ALS)
Progressive Muscular Atrophy (PMA)
Primary Lateral Sclerosis (PLS)
Progressive Bulbar Palsy (PBP)
What is the Epidemiology of MND?
More common in men
Most commonly affects people in middle age
ALS - most common type of MND
Most die within 3 years of diagnosis – usually from respiratory failure as a result of bulbar palsy and pneumonia
What are the signs of ALS?
UMN and LMN signs
What are the signs of PMA?
LMN only
Has best prognosis
What are the signs of PLS?
UMN signs only
What are the signs of PBP?
CN9-12 affected
Speech and swallowing issues
Worst prognosis
What is the pathology of MND?
Degenerative condition affecting motor neurons – mainly the anterior horn cells
There is relentless and UNEXPLAINED destruction of UMN and anterior horn cells in the brain and spinal cord
Causes both UMN and LMN dysfunction
UMN and LMN affected but no sensory or sphincter loss – distinguishes from MS
Never affects eye movements – distinguishable from myasthenia gravis
What are the signs of an Upper motor neuron (UMN) lesion?
Hypertonia
Rigidity + spasticity
Hyperreflexia
Babinski Reflex Positive - Big toe goes up when stroking foot
Power:
Arms - Flexors > Extensors
Legs - Flexors < Extensors
What are the signs of a Lower Motor Neuron (LMN) Lesion?
Hypotonia
Flaccidity + muscle wasting
Hyporeflexia
Fasciculations
Babinski Reflex Negative - big toe goes down when stroking foot
Generally loss of power
What should you think if you see a patient with mixed Upper and lower motor neuron signs?
MND!!
Where is an UMN lesion?
Anywhere on a motor nerve between the pre-central gyrus to the anterior spinal cord
Where is a LMN lesion?
Anywhere on a motor nerve between the anterior spinal cord and the innervated muscle
What is NEVER affected in MND?
Eye muscles:
Affected in MS and MG
Sensory Function and Sphincters:
Affected in MS and Polyneuropathies
What are the symptoms of MND?
Progressive weakness
Falls
Speech and swallowing issues (in PBP)
What is split hand sign?
Disproportionate wasting of the thenar muscles compared to the hypothenar muscles
What are the clinical signs of MND?
Mixed UMN and LMN signs
Dysarthria / Dysphagia
Fasciculations
Split Hand Sign
What is the primary investigation for MND?
Clinical Dx due to mixed UMN/LMN picture
- LMN/UMN signs in 3 regions
EMG - fibrillation potentials
May carry out MRI / CSF examinations for exclusion of differentials
What are some good diagnostic tips to exclude DDx of MND?
No sensory loss = rules out MS or myelopathy
No disturbances in eye movements = rules out Myasthenia Gravis or MS
No sphincter disturbances = rules out MS
What is the management of MND?
No cure - progressive
Supportive therapy:
Physiotherapy
Breathing support if needed
Pharmacological Tx:
Riluzole - anti-glutaminergic
What are the complications of MND?
Aspiration pneumonia
Respiratory failure
What is Myasthenia Gravis (MG)?
Autoimmune condition characterised by Autoantibodies against the nicotinic Acetylcholine receptors (nAChR) at the neuromuscular Junction.
What type of Hypersensitivity reaction is Myasthenia Gravis?
Type 2
What are the risk factors for MG?
Females
FHx
Autoimmunity
Thymoma/ Thymic Hyperplasia
What is the epidemiology of MG?
Generally more common in Females
Female peak (20-30yrs) - associated with autoimmune disease
Male peak (50-60yrs) - associated with Thymoma
What is the pathophysiology of MG?
85% Anti-nACh Receptors:
- Bind to post synaptic receptor and competitively inhibit ACh binding.
- ACh cannot bind during exertion and therefore there is progressive weakness of muscles
- Auto-ABs Will also bind to complement factors and cause NMJ destruction
15% Anti MuSK:
Inhibit MuSK from synthesising ACh Receptors so there is reduced expression on post synaptic membrane.
What are the symptoms of MG?
Lethargy
Muscle weakness that starts at head/neck and moves downwards
Weak eye muscles - diplopia
Ptosis
Jaw fatiguability - slurred speech/ chewing difficulties
Swallowing difficult
What is the progression of weakness like in MG?
Worse during the day/with exertion
Better with rest
Due to more AChRs being required in exertion and therefore greater inhibiton
What are the clinical signs of MG?
ptosis
Head drop
Myasthenic Snarl - difficulty smiling
What is the primary investigation for MG?
Serology - Ab testing:
Anti-nACh-R Abs (85%)
Anti-MuSK Abs (15%)
Bedside tests - Count to 50 and as they reach higher numbers their voice becomes less audible
Nerve Stimulation Tests - decrement in evoked potential after motor nerve stimulation
Edrophonium Test
CT/MRI - look for Thymoma
What is the Edrophonium Test?
Give Edrophonium (neostigmine) and AChE inhibitor to increase ACh
Will give brief/temporary relief of Sx and increase muscle power
What is the first line treatment for MG?
1st Line: Reversible AChE inhibitors:
Rivastigmine, Neostigmine Pyridostigmine
2nd Line: Immunosuppressants
Prednisolone/Azathioprine
Potentially Monoclonal Abs (Rituximab)
What is the main complication of MG?
Myasthenic Crisis:
Severe acute worsening of Sx
Often Triggered by another illness (URTI)
Severe Respiratory weakness
What is the Treatment for Myasthenic Crisis?
IV Ig (immunoglobulin) and Plasmapheresis
Non-invasive ventilation:
BiPAP for Resp failure
What is a differential Diagnosis of Myasthenia Gravis?
Lambert Eaton Syndrome
What is Lambert Eaton Syndrome?
A NMJ syndrome which has similar Sx to MG.
Autoimmunity against VG-Ca channels thereby reducing ACh release at the NMJ causing muscle weakness.
What is the cause of Lambert Eaton Syndrome?
Unclear but likely a paraneoplastic syndrome:
Typically occurs in Px with Small Cell Lung Cancer (SCLC)
What is the presentation of Lambert Eaton Syndrome?
Proximal muscle weakness that develops more slowly
Sx start at extremities and progress towards the head
Shares most of same Sx with MG
What is the difference between MG and Lambert Eaton Syndrome clinically?
Lambert Eaton Syndrome symptoms tend to improve following a period of strong muscle contraction.
Post Tetanic Potentiation
What is the Treatment for Lambert Eaton Syndrome?
Dx and Tx underlying condition (often SCLC)
Amifampridine - blocks K+ channels and increases ACh release
+ Steroids and Immunosuppressants
What is Syncope?
Transient loss of consciousness due to transient global reduction in cerebral perfusion with complete and rapid recovery
What are the classifications of the causes of syncope?
Neurally mediated: Vasovagal
Orthostatic (postural) syncope
Cardiac syncope
Structural
Arrythmias
What is vasovagal syncope?
Vagus nerve receives a strong signal:
Emotion, pain, temperature change
Activates Parasympathetic NS
Dilates blood vessels reducing cerebral perfusion
causing hypoperfusion of the brain leading to collapse
What is the prodrome of syncope (presyncope Symptoms)?
Hot or clammy
Sweaty
Heavy
Dizzy or lightheaded
Vision going blurry or dark
Headache
What are the causes of primary syncope?
Dehydration
Missed meals
Extended standing in a warm environment, such as a school assembly
A vasovagal response to a stimuli, such as sudden surprise, pain or the sight of blood
What are the causes of secondary syncope?
Hypoglycaemia
Dehydration
Anaemia
Infection
Anaphylaxis
Arrhythmias
Valvular heart disease
Hypertrophic obstructive cardiomyopathy
What is Orthostatic syncope?
Syncope due to postural change (standing from sitting)
Occurs due to:
Upon standing blood pools in the lower limbs/lower abdomen → venous return decreases → transient ↓cardiac output
Sympathetic activation increases SV. reduction of Parasympathetic stimulation further increases HR and CO.
Failure of these mechanisms leads to orthostatic syncope
What are the classical characteristics of syncope?
Situational
Typically, from sitting or standing
Rarely from sleep
Presyncopal symptoms
Occipital – see stars, dizzy and light headed, vision goes black
Temporal – noises may sound distorted
Back of brain most sensitive to oxygen
Duration – 5-30 seconds
Recovery within 30 seconds
Cardiogenic syncope – less warning, history of heart disease
What are the primary investigations for syncope?
Clinical Hx and examination
Ix to rule out pathological causes:
Bloods - infection
FBC - anaemia
ECG- arrythmia
Glucose - Hypoglycaemia
B-hCG - ectopic pregnancy
What is the management of Syncope?
Confirm Dx to exclude underlying pathology
Avoid Dehydration
Avoid missing meals
Avoid standing still
What are the different functions of the different nerve fibre types?
A-alpha – proprioception
A-beta – light touch, pressure, vibration
A – delta – dull pain and cold
C – fibres – sharp pain and warmth
What is a mononeuropathy?
A process of nerve damage that affects a single nerve
What is polyneuropathy?
Disorders of peripheral or cranial nerves, whose distribution is
usually symmetrical and widespread (multi-systemic)
Often due to:
Diabetes
MS
Guillain Barre
What is Mononeuritis Multiplex?
A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes.
What are the causes of Mononeuritis Multiplex?
WARDS PLC:
- Wegener’s granulomatosis
- Aids/Amyloid
- Rheumatoid arthritis
- Diabetes mellitus
- Sarcoidosis
- Polyarteritis nodosa
- Leprosy
- Carcinoma
What is Peripheral Neuropathy?
What are the types of peripheral nerve disease?
Nerve pathology outside of the CNS that affects the peripheral nerves
Mononeuropathy
Polyneuropathy
What are some causes of peripheral nerve disease?
DAVIDE:
Diabetes
Alcohol
Vitamin B12 Deficiency
Infective/malignant
Drugs - isoniazid
Every vasculitis
What are the mechanisms of Peripheral Neuropathy?
Demyelination
Axonal Damage - Nerve cut
Nerve Compression
Vasa Nervorum Infarction
Wallerian Degeneration (Nerve lesion and distal end dies)
What are the 6 mechanisms of peripheral neuropathy and how do they occur?
- Demyelination
o Schwann cell damage leads to myelin sheath disruption
o Results in marked slowing of conduction e.g. in Guillain Barre syndrome
- Axonal degeneration
o Axonal damage causes the nerve fibre to die back from periphery
o Conduction velocity initially remains mortal because axonal continuity is maintained in surviving fibres
o Axonal degeneration occurs in toxic neuropathies
- Compression
o Focal demyelination at the point of compression causes disruption of conduction
o Typically occurs in entrapment neuropathies e.g. carpal tunnel syndrome
- Infarction
o Micro-infarction of vasa nervorum occurs in diabetes and arteritis such as polyarteritis nodosa and eosinophilic granulomatosis with polyangiitis (GPA)
- Infiltration
o Infiltration occurs by inflammatory cells in leprosy and granulomas such as sarcoid and by neoplastic cells (cancer)
- Wallerian degeneration
o Process that results when a nerve fibre is cut or crash and the distal part of the axon that is separated from the neuron’s body degenerates
Give some examples of Mononeuropathies
- Carpal tunnel syndrome (medial nerve) – most common
- Ulnar neuropathy (entrapment at the cubital tunnel)
- Peroneal neuropathy (entrapment at the fibular head)
- Cranial mononeuropathies (III or VII cranial nerve palsy)
Idiopathic
Immune mediated
Ischaemic
What are the investigations for a peripheral neuropathy?
- History
- Clinical examination
Reduced or absent tendon reflexes
Sensory deficit
Weakness – muscle atrophies
- Neurophysiological examination i.e. Nerve conduction studies/QST
Demyelinating 🡪 Slow conduction velocities
Axonal 🡪 Reduced amplitudes of the potential
What is the management for peripheral neuropathy?
Symptomatic Tx:
Pain – give anti-neuralgic (GAP) - Gabapentin, Amitriptyline, Pregabalin
Cramps - Quinine
Balance - Physiotherapy/walking aids
Aim is to identify any reversible cause and stop disease progression if possible
What is the most common Mononeuropathy?
Carpal Tunnel Syndrome
What is the pathophysiology of Carpal Tunnel Syndrome?
Compression/pressure of the Median Nerve as it passes through the Flexor Retinaculum “carpal tunnel”
What are the causes of Carpal Tunnel Syndrome?
Mostly Idiopathic
Also: (HODPARAR)
Hypothyroidism
Obesity
Diabetes
Pregnancy
Acromegaly
Rheumatoid Arthritis
Amyloidosis
Repetitive Strain Injury
Who is more likely affected by Carpal Tunnel Syndrome?
Females due to narrower wrists so more likely to have compression
Over 30s
What is the presentation of Carpal Tunnel Syndrome?
Gradual Onset of Sx
Start off as intermittent Sx
Worse at night
What are the Symptoms of Carpal Tunnel Syndrome?
Sensory Symptoms: Palmer aspects and full fingertips of:
Thumb
Index and middle finger
The lateral half of ring finger
Motor Symptoms: Thenar Muscles
Flexor Pollicis Brevis
Abductor Pollicis
Opponens Pollicis
What are the sensory symptoms of Carpal Tunnel Syndrome?
Numbness
Paraesthesia (pins and needles or tingling)
Burning sensation
Pain
What are the motor symptoms of Carpal Tunnel Syndrome?
Weakness of thumb movements
Weakness of grip strength
Difficulty with fine movements involving the thumb
Wasting of the thenar muscles (muscle atrophy)
What nerve is affected in Carpal Tunnel Syndrome?
Which branch is involved for the sensory symptoms?
MEDIAN Nerve
Sensory Sx: Palmer Digital Cutaneous Branch
What are the tests to diagnose Carpal Tunnel Syndrome?
Phalen Test
Tinel Test
What is the Phalen Test?
Put the backs of their hands together in front of them with the wrists bent inwards at 90 degrees.
The test is positive when this position triggers the sensory symptoms of carpal tunnel, with numbness and paraesthesia in the median nerve distribution.
What is the Tinel Test?
Tapping Median nerve in carpal tunnel causes sensory Sx
(Tinels = Tapping)
What is the management for Carpal Tunnel Syndrome?
Wrist splints that maintain a neutral position
Steroid injections (hydrocortisone)
Surgery - decompression (last resort)
What are the nerve roots for the Median Nerve?
C6-T1
What can lead to compression of the Median Nerve to cause Carpal Tunnel Syndrome?
Swelling of contents within Carpal Tunnel
Narrowing of the Carpal Tunnel
What is the nerve roots of the radial nerve?
C5-T1
What is the classical presentation of a radial nerve palsy?
Wrist drop
(with elbow flexed and arm pronated
What muscles are innervated by the Radial Nerve?
BEST:
Brachioradialis
Extensors of forearm
Supinator
Triceps
What muscles are innervated by the Median Nerve?
2LOAF:
- 2 Lumbricals
- Opponens pollicis
- Abductor pollicis brevis
- Flexor pollicis brevis
What can cause wrist drop?
Damage to the radial nerve
Compression of the radial nerve at the humerus
What is the nerve roots of the Ulnar Nerve?
C8-T1
What is the classical presentation of an ulnar nerve palsy?
Claw hand (4th/5th fingers claw up)
What is the treatment of Wrist drop and Claw hand?
Splint
Analgesia
What are the nerve roots for the common peroneal nerve?
L4-S1
(Branch off the Sciatic nerve)
What is the sign of a Sciatic/Common peroneal nerve Palsy?
Foot drop
What Drugs can cause peripheral Neuropathy?
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
What are the main symptoms of peripheral neuropathy?
numbness and tingling in the feet or hands
burning, stabbing or shooting pain in affected areas
loss of balance and co-ordination
muscle weakness, especially in the feet
What is Charcot-Marie-Tooth Syndrome?
Inherited sensory and motor peripheral neuropathy disease caused by an autosomal dominant mutation in PUP22 gene on chromosome17
What are the genetics of Charcot-Marie-Tooth Syndrome?
Mutation in PUP22 gene
Chromosome 17
+ multiple others
What is the pathophysiology of Charcot-Marie-Tooth Syndrome?
Generally mutations cause dysfunction of the myelin or axons leading to neuropathy
Different mutations in different genes have different pathophysiology’s.
When do symptoms onset in Charcot-Marie-Tooth Syndrome?
Usually before the age of 10.
May appear after 40yrs
What are the classical features of Charcot-Marie-Tooth Syndrome?
Foot Drop (common peroneal palsy)
Distal muscle wasting (stork legs)
Hammer Toes (toes always curled up)
Pes Cavus (high foot arches)
Peripheral sensory loss
Reduce tendon reflexes
What is the Diagnostic investigation for Charcot-Marie-Tooth Syndrome?
Clinical Dx
Nerve biopsy
Genetic Testing
What is the treatment for Charcot-Marie-Tooth Syndrome?
Supportive Tx:
Physiotherapists
Orthopaedics
Occupational Therapists
What is Duchenne Muscular Dystrophy (DMD)?
X-Linked recessive condition caused by a mutation in the Dystrophin Gene
Who presents with DMD?
Boys Exclusively:
Age of onset is around 3-5 years old
Explain the genetics of DMD?
X linked Recessive condition.
Therefore mother with 1 faulty gene:
Daughters - 50% chance of being carrier
Sons - 50% chance of being affected
What is the pathophysiology of DMD?
Lack of Dystrophin gene (vital part of muscle fibre) means that the muscles are not protected from being broken down by enzymes
Therefore in DMD you get progressive wasting and weakness of muscle as they are broken down.
The muscle tissue is then replaced by fibrofatty tissue
Most Px in wheelchair by teenage years
What cardiovascular condition is associated with DMD?
DCM
Dystrophin gene in heart muscle not present which is normally involved in membrane stability.
Therefore in DMD there is damage to the cellular mechanisms causing dilation of
ventricles due to wasting of the cardiac muscle causing cardiomyopathy
What are the symptoms of DMD?
Child struggles to get up from lying down
(GOWER’S sign)
Skeletal deformities - scoliosis
What are the diagnostic tests for DMD?
Prenatal tests and DNA genetic testing
What is Gower’s Sign?
Children with proximal muscle wasting stand up in a specific way:
They get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose.
They then shift their weight backwards and transfer their hands to their knees.
Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect
What is the treatment for DMD?
Purely Supportive: No cure
Physiotherapy
Occupational Therapy
Medical appliances - Wheelchairs
Tx of complications - Scoliosis/DCM
What is the life expectancy of a child with DMD?
25-35 years
What may be used to slow DMD muscular progression?
Oral steroids have been shown to delay progression by 2 years.
Creatine Supplements may improve some strength
What is Wernicke’s Encephalopathy?
Reversible severe cognitive decline due to severe Vitamin B1 (thiamine) deficiency.
What is the cause of Wernicke’s Encephalopathy?
Alcohol abuse
Alcohol massively decreases Thiamine levels leading to deficiency
What is the classic triad of Wernicke’s Encephalopathy?
Ataxia
Confusion
Ophthalmoplegia
How is Wernicke’s Encephalopathy Diagnosed?
Clinically Recognised
Macrocytic Anaemia
Deranged LFTs
What is the treatment of Wernicke’s Encephalopathy?
Parenteral PABRNEX (Vit B1) for 5 days.
Oral thiamine Prophylactically
What is the main complication of Wernicke’s Encephalopathy?
Korsakoff Syndrome:
Caused when WE left for too long without Tx
Leads to Sx with disproportionate memory loss and irreversible damage.
What bacteria causes Tetanus?
Clostridium Tetani (Gram +tve Bacillus anaerobe)
Inoculation from dirty soil
What is the Pathophysiology of Tetanus?
Tetanospasmin Toxin produced.
Travels up axons
Blocks the release of the inhibitory neurotransmitters GABA and glycine
resulting in continuous motor neuron activity → continuous muscle contraction →
lockjaw and respiratory paralysis
What is the Treatment for Tetanus?
Primary Vaccine (prevention better than cure)
Sx treatment:
Muscle relaxants
Analgesics - paracetamol
Metronidazole - remove residual bacteria
What is Creutzfeldt-Jakub disease?
Prion disease “Mad cow Disease”
Idiopathic misfolded prion proteins deposited in the cerebrum and cerebellum
This leads to severe cerebellar Dysfunction.
There is NO Tx
How does Creutzfeldt-Jakub Disease Present?
Ataxia
Poor memory
Behavioural changes
Muscle weakness
Myoclonus
Dementia
What is the affect of Botulinum toxin?
Blocks acetylcholine (ACh) release leading to flaccid paralysis
What is the epidemiology of brain tumours?
10,000+ new cases per year in the UK
55% are malignant
9th most common cancer (3% of all cancers)
What are the main Primary Brian tumours?
Gliomas - Tumour of brain intrinsic tissue (subdivided into tissue type; Astrocytoma, Oligodendroglioma, Ependymoma)
Meningioma - tumour of brain lining (meninges)
Schwannoma (CN tumours)
What is the most common Primary brain tumour?
Astrocytoma (90% of Primary brain tumours)
2nd Most common Paediatric cancer
What are the common causes of secondary brain tumours?
Metastases from:
Non-Small Cell Lung Cancer (NSCLC)
Breast
Small Cell Lung Cancer (SCLC)
Melanoma
RCC
Gastric Cancer
What is the most common cause of a brain tumour?
Secondary brain tumour from a NSCLC
What is the treatment for Secondary brain tumours?
Often Palliative Care (due to being mets from high grade tumours)
Chemotherapy
What is the WHO grading for an astrocytoma (and other brain tumours)?
What is the prognosis for each grade?
Graded 1-4
1 = Benign Pilocytic astrocytoma - Good prognosis
2 = Diffuse Astrocytoma - >5 yrs (10 years average)
3 = Anaplastic Astrocytoma - 2-5 yrs
4 = Glioblastoma - <1 yr
How are Brain tumours classified into the WHO grade?
Characterised histologically
- Cellularity
- Mitotic activity
- Vascular proliferation
- Necrosis
What is a Glioma?
A tumour made arising from the Glial cells:
Astrocytoma (glioblastoma multiforme is the most common)
Oligodendroglioma
Ependymoma
What are the symptoms of a brain tumour?
Often ASx when small - progress to varied presentations depending on type and location of tumour.
Focal neurological Sx as they develop
Will often RAISE ICP causing Headaches:
Woken by headache
Worse in morning
Worse Lying down
Associated with N+V
Exacerbated by coughing, sneezing and drowsiness
Papilledema (due to raised ICP)
Cushing Triad (Increased PP, Bradycardia, Irregular Breathing)
Epileptic Seizures
Cancer Sx:
Lethargy
Malaise
Weight Loss
What are the red flag signs of a brain tumour?
Headaches:
With features of raised ICP (including papilloedema)
With focal neurology, check for field defect
Other urgent referrals:
- New onset focal seizure
- Rapidly progressive focal neurology (without headache)
- Past history of other cancer
What is papilloedema?
Swelling of the optic disc that occurs secondary to a raised ICP.
This is often picked up on Fundoscopy
What is a Glioblastoma multiforme?
Glioblastoma (GBM), also referred to as a grade IV astrocytoma, is a fast-growing and aggressive brain tumour
Often fatal within 1 year of Dx
What are the investigations for a brain tumour?
MRI head - locate tumour
Biopsy - determine grade
Fundoscopy - Papilloedema due to raised ICP
NO LP as this is CI in raised ICP
What is the management of brain tumours?
Depends on Type, Grade and Site:
Tx is non-curative except for Grade I tumours
Surgical removal if possible/reduce ICP (dexamethasone to reduce oedema)
Chemotherapy/Radiotherapy Before/during/after surgery.
(Radiotherapy is mainstay of treatment)
Palliative Care
What are the differential Diagnoses of a brain tumour?
Aneurysm
Abscess
Cyst
Haemorrhage
Idiopathic intracranial hypertension
What are Pituitary Tumours?
- Pituitary gland – sits in pituitary fossa (behind nose and below base of brain)
- Tumours are almost always benign and usually curable
- Excessive effects of tumour
- Local effects of tumour – bitemporal hemianopia
- Inadequate hormone production by the remaining pituitary gland
- Treated with Transsphenoidal surgical resection
- What does Cn1 innervate?
- What are it’s functions?
What is its test?
Olfactory
- Innervates: olfactory epithelium.
- Function: olfaction.
- Test each nostril for smell sensation
- What does Cn2 innervate?
- What are it’s functions?
What are its tests?
Optic:
- Innervates: retina.
- Function: vision.
pupillary light reflex
Visual Acuity
Visual Fields
- What does Cn3 innervate?
- What are it’s functions?
What are its tests?
Occulomotor:
- Innervates: medial, superior and inferior rectus muscles and inferior oblique and levator palpebrae superioris.
- Motor function: movement of eyeball.
- Parasympathetic function: constriction and accommodation.
pupillary light reflex
lateral deviation
- What does Cn4 innervate?
- What are it’s functions?
What are its tests?
Trochlear:
- Innervates: superior oblique.
- Functions: movement of eyeball.
Elevation of the eye
- What does Cn5 innervate?
- What are it’s functions?
What are its tests?
Trigeminal:
- Sensory innervation: face, scalp, cornea, nasal and oral cavities, anterior 2/3 of tongue, dura mater.
- Motor innervation: muscles of mastication and tensor tympani.
- Sensory function: general sensation.
- Motor functions: open and close the mouth. Tenses tympanic membrane.
Corneal Reflex
Open and close mouth
- What does Cn6 innervate?
- What are it’s functions?
What are its tests?
Abducens:
- Innervates: lateral rectus.
- Function: eye movement, abduction.
Medial deviation
- What does Cn7 innervate?
What are its tests?
Facial:
- Special sensory innervation: anterior 2/3 of tongue - taste.
- Motor innervation: muscles of facial expression and stapedius.
- Parasympathetic innervation: submandibular and sublingual and lacrimal glands.
lacrimation
Facial Weakness? UMN or LMN
Hearing - hyperacusis
Corneal Reflex
- What does Cn8 innervate?
- What are it’s functions?
What are its tests?
Vestibulocochlear:
- Innervation: cochlea and vestibular apparatus.
- Functions: hearing and proprioception of head and balance.
Cover ear and whisper into the opposite one
caloric test
- What does Cn9 innervate?
What are its tests?
Glossopharyngeal:
- Sensory innervation: posterior 1/3 of tongue, middle ear, pharynx, carotid bodies.
- Motor innervation: stylopharyngeus.
- Parasympathetic innervation: parotid gland.
examined with CNX
- What does Cn10 innervate?
What are its tests?
Vagus:
- Sensory innervation: pharynx, larynx, oesophagus, external ear, aortic bodies, thoracic and abdominal viscera.
- Motor innervation: soft palate, larynx, pharynx.
- Parasympathetic innervation: CV, respiratory and GI systems.
hoarseness of voice / nasal sound
Gag reflex (afferent CNIX and efferent CNX)
contraction of the palate and deviation away from lesion
- What are the functions of Cn10?
- Sensory functions: general sensation.
- Motor functions: speech and swallowing.
- Parasympathetic functions: control over CV, respiratory and GI systems.
- What are the functions of Cn9?
- Sensory functions: general sensation, taste, chemo/baroreception.
- Motor functions: Swallowing (larynx and pharynx are elevated).
- Parasympathetic function: salivation.
- What are the functions of Cn7?
- Sensory function: taste.
- Motor function: facial movement and tension of ossicles.
- Parasympathetic function: salivation and lacrimation.
- What does Cn11 innervate?
- What are it’s functions?
What are its tests?
Accessory:
- Innervation: trapezius and sternocleidomastoid.
- Functions: movement of head and shoulders.
turn head against resistance
shrug shoulders
- What does Cn12 innervate?
- What are it’s functions?
What are its tests?
Hypoglossal
- Innervation: intrinsic and extrinsic muscles of the tongue.
- Function: movement of the tongue.
stick tongue out
look for signs of deviation.
ipsilateral side is paralysed
