Neurology Flashcards
What is the location of a Lumbar Puncture?
Between L3 and L4
What is the function of the Cerebellum?
Control of motor function and maintenance of muscle tone
What are the 3 functional and anatomical divisions of the cerebellum?
Cerebrocerebellum - Planning movements, motor learning and coordination of muscle activation
Spinocerebellum (vermis + intermediate zone) - Body movements and receives proprioceptive information
Vestibulocerebellum (flocculonodular) - Balance control and ocular reflexes e.g. fixation on a target. Receives input from the vestibular system.
What is the blood supply to the cerebellum?
Superior Cerebellar Artery (SCA) and Anterior Inferior Cerebral Artery (AICA) from BASILAR ARTERY
Posterior Inferior Cerebellar Artery (PICA) from VERTEBRAL ARTERY
What are the causes of cerebellar dysfunction? (PASTRIES)
Posterior fossa tumour Alcohol misuse Stroke Trauma Rare causes Inherited - Friedrich’s Ataxia Epilepsy medications Sclerosis (MS)
What are the symptoms of cerebellar dysfunction? (DANISH)
Dysdiadochokinesia and Dysmetria
Ataxic gait
Nystagmus
Intention tremor
Slurred speech
Hypotonia
What is dysdiadochokinesia and what is dysmetria?
Dysdiadochokinesia = impaired rapidly alternating movements
Dysmetria = past pointing
Which part of the cerebellum is damaged to give dysdiadochokinesia, dysmetria, ataxic gait and intention tremor?
Spinocerebellum
Basilar infarct/ SCA/ AICA
Which part of the cerebellum is damaged to give Nystagmus?
Vestibulocerebellum
PICA infarct
Are cerebellar signs contralateral or ipsilateral and why?
Ipsilateral
What is the function of the basal ganglia?
Regulation of movement by providing a feedback mechanism to the cerebral cortex
Where is the basal ganglia located?
Forebrain and midbrain
Part of the extrapyramidal system
How does the extrapyramidal system differ to the corticospinal tracts?
Involuntary actions whereas corticospinal is voluntary
What is the direct pathway?
Excitatory
Excitation of the striatum = ↑ inhibition of the Globus Pallidus Interna and Substantia Nigra
↓ inhibition of the Thalamus (as GPi and SNR inhibit thalamus) = ↑ excitation of the motor cortex
Movement!
What is the indirect pathway?
Inhibitory
Excitation of the striatum = ↑ inhibition of the Globus Pallidus Externa
↓ inhibition of the Subthalamic nucleus (as GPe inhibits STN)
↑ excitation of the Substantia Nigra (as STN excites)
↑ inhibition of the Thalamus (as SNR inhibits) = ↓ excitation of the motor cortex
↓ movement
What is the function of the direct and indirect pathways?
Regulation between the two pathways is what allows control and coordination of movement
What is the pathophysiology behind Parkinson’s disease?
The degradation of dopaminergic neurones in the Substantia Nigra Pars Compacta
Lewy bodies are also present in the basal ganglia, brain stem and cortex
Leads to dysregulation between the direct and indirect pathways
What is the Parkinsonism triad?
Resting tremor - pill rolling
Hypertonia - Cogwheel Rigidity (hypertonia + tremor)
No muscle weakness
Bradykinesia
How does bradykinesia present in PD?
Slow to start moving
Slow actions that decrease in amplitude upon repetition e.g. Micrographia
Festinant gait (shuffling, forward leaning)
Might stop in doorways
Reduced facial expressions
What are the autonomic effects of PD?
Postural hypotension
Constipation
Dribbling
What are the neuropsychiatric effects of PD?
Depression
Psychosis
How is PD mainly diagnosed?
Clinical diagnosis
Triad is progressive and signs are usually worse on one side
Which drugs must be excluded when diagnosing PD?
Psychiatric drugs - typical antipsychotics/ methyldopa/ memantine
Anti-emetics e.g. metoclopramide
Which other conditions must be excluded when diagnosing PD?
Wilson’s disease
Non-Parkinson’s dementias e.g. Dementia with Lewy Bodies, Frontotemporal dementia
Other tremor causes e.g. essential Tremor
What is the conservative management of PD?
Refer to neurologist/stop the drug causing the Parkinsonism
Provide patient and family with written info and support sources e.g. a leaflet and Parkinson’s UK
Inform DVLA
MDT involvement
Manage other symptoms as and when e.g. constipation, sleep disturbance
What is the medical management of PD?
1) Levodopa + Co-Beneldopa - Dopamine precursor + Dopa Decarboxylase Inhibitor.
2) MAO-B inhibitor e,g. Selegiline
3) Oral Dopamine Agonists e.g. Pramiprexole
What is the MOA of Levodopa + Co-Beneldopa
Dopamine precursor + Dopa Decarboxylase Inhibitor.
Dopamine can cross through the blood brain barrier but DCI cannot.
This prevents the synthesis of dopamine in the peripheries therefore reducing adverse side effects!
What are the side effects of Levodopa + Co-Beneldopa
dyskinesia painful dystonia psychosis visual hallucinations N&V
What are the side effects of MAO-B inhibitor
Don’t worry about tyrosine rich food (MAO-A).
Side effects = atrial fibrillation and postural hypotension
What are the adjuvant medical treatments for PD?
COMT - catechol-O-methyl transferase (COMT) inhibitors. Prevents methylation of dopamine and levodopa (LFT monitoring)
Amantadine - actually an antiviral! ↑ exogenous dopamine production (not much evidence)
What must be considered with the medical management of PD?
Efficacy reduces over time
Can’t withdraw suddenly due to risk of neuroleptic malignant syndrome and acute akinesia (careful if vomiting or something)
What is the surgical management of PD? When would this be considered?
Deep brain stimulation
Motor complications that are refractory to medical treatment
dopamine responsive
no co-morbid mental health conditions
What is Multiple Sclerosis?
Chronic and progressive demyelination of nerves found in the central nervous system and spinal cord.
Never affects peripheral nerves.
What is the pathophysiology of MS? (big card)
T cell (CD4 helper and CD8 cytotoxic) mediated degradation of the myelin sheaths
Myelin activates T cells then BBB expresses specific receptors to allow more T cells in and cause further damage
TNF-a and Interleukins also increase permeability of the BBB and allow more T cells in
This attracts B cells and macrophages which attack the Oligodendrocytes
- Prevents further production of myelin - Scar tissue formation = plaques on oligodendrocytes
Remyelination initially occurs but eventually stops - relapses?
What type of hypersensitivity reaction is MS?
Type 4!
ABCDDDDD - takes days
What are the 4 types of MS?
Relapsing and remitting - most common + periods of attacks
Primary Progressive - 1 constant attack
Secondary Progressive - Starts as relapsing and remitting but becomes progressive
Progressive Relapsing - Bouts of attacks but getting worse overall
What are the non-modifiable risk factors for MS?
Female
Genetics - HLA-DR2 gene
What are the modifiable risk factors for MS?
Infections (+genetic vulnerability) - EBV and Herpes 6 (Roseola Infantum)
Vitamin D Deficiency
Smoking
How does MS commonly present?
Optic nerve affected = Optic Neuritis
brainstem affected = nystagmus and diplopia
other motor deficits e.g. spasticity/ataxia/dysarthria/intention tremor
What is optic neuritis?
Sudden blurred vision and unilateral eye pain
loss of visual acuity (hole in the middle) and contrast sensitivity (red in particular)
Get a relative afferent pupillary defect (see opthalmology)
Swelling of optic disc margins but not papilloedema
How can MS present as sensory symptoms?
Pain and loss of sensation
Lhermitte’s sign (sudden sensation resembling an electric shock that passes down the back of your neck and into your spine and may then radiate out into your arms and legs)
What are other symptoms of MS?
Autonomic System
Bladder, bowel and sexual dysfunction
Cognitive System
Later
What are 2x Ddx for MS?
Systemic Inflammatory Disease e.g. SLE, Sarcoidosis (won’t have Antibody in CSF)
Space Occupying Lesions e.g. vascular
How is MS diagnosed?
MRI of brain and spinal cord is definitive
Lumbar Puncture
Nerve conduction studies (lower amplitude)
+ absence of other treatable causes
What is the conservative treatment for MS?
R&R
Physical and psychological support, occupational therapy
What is the medical management for MS?
Corticosteroids (IM Methylprednisolone)
IVIG
Plasmapheresis
Immunosuppressants
Which immunosuppressants are used for MS?
Immunomodulating - B-Interferons to reduced inflammation and maintain BBB
Glativamer Acetate - Similar antigens to myelin so T cells compete
Monoclonal antibodies - lots of side effects e.g. increased risk of progressive multifocal leucoencphalopathy due to JC virus. PML = lytic infection of oligodendrocytes AND cancer
What is Guillan Barre Syndrome?
Inflammatory, demyelinating polyneuritis causing muscle weakness and loss of sensation
How does GBS normally present?
Elderly
Sudden onset paralysis
Starts at feet and ascends with a variable rate of progression
Max. Weakness usually at 2-3 weeks
Bilateral
Back, shoulder and thigh pain
Associated parasthesia, numbness and absent deep tendon reflexes
What are the required features to diagnose GBS?
Bilateral progressive weakness in the upper and lower limbs
Hyporeflexia
What are the 2 most serious complications of GBS?
Weakness eventually reaches the respiratory muscles = respiratory failure type 2
Autonomic dysfunction so urinary retention etc
What are some other complications of GBS? (5)
Pain VTE SIADH Renal Failure (following IVIG) Immobility = VTE and Hypercalcaemia
What is the natural progression of GBS?
Often following an infection. Usually 1-3 weeks after
Campylobacter Jejuni (gastroenteritis)
Cytomegalovirus
Epstein-Barr Virus
Mycoplasma Pneumoniae
Flu vaccine may be associated (???)
What are the 4 sub types of GBS?
Acute Inflammatory Demyelinating Polyradiculoneuropathy (most common)
Acute Motor Axonal Neuropathy
Acute Motor and Sensory Axonal Neuropathy
Acute Pandysautonomia
What are the main investigations for GBS?
Mainly a clinical diagnosis. Do bedside spirometry to monitor FVC!!!
BUT can do a lumbar puncture and look for proteins in the CSF to exclude Polio and Lymphoma
Nerve conduction studies to exclude other causes of muscle weakness
Campylobacter Serology if GI upset - AMAN = anti GM1
What is the treatment of GBS?
General Supportive Management
IVIG
Plasmapheresis within the first 2 weeks of onset
Not steroids as they may worsen symptoms!
What is the neuromuscular junction?
The synapse between a motor neurone and its corresponding muscle fibre
The muscular end plate has lots of junctional folds in which ligand gated (Acetylcholine) ion channels sit
Which ion channels are located on the muscular end plates?
Nicotinic (ionotropic so have integral ion channel. For short and rapid responses.)
or Muscarinic (metabotropic so GPCR and longer response)
Which enzyme is present on the muscular end plate and why?
Acetylcholinesterase is also present on the end plate to terminate the effect following contraction.
What is myasthenia gravis?
Autoimmune condition
B cells make an antibody that blocks the Nicotinic Acetylcholine Receptor on the muscular end plate
Deficient/reduced Acetylcholine means that the muscle can’t contract = weakness
What type of hypersensitivity reaction is myasthenia gravis?
ABBBBBBCD = B
Type 2! B cells involved
What are the main symptoms of myasthenia gravis?
Muscle weakness e.g. partial and bilateral ptosis, diplopia, dysphagia
Fatiguability - symptoms are worse after repetitive movements and at end of the day
What is the main complication of myasthenia gravis?
Breathing difficulties (crisis/later on). Especially in water for example. Get type 1 rest failure
What are the bedside tests for MG?
Ice cube test - Put ice cubes/pack on eyelids and see if ptosis resolves
What are the blood tests for MG?
ntibodies - Anti-nAchR and Anti-MUSK (if anti-nAchR is -ve)
What imaging should be done for MG?
CT CAP - Thymoma is associated
What special tests should be done for MG?
Nerve Stimulation tests - decrease in amplitude following repeated stimulation
What is the medical management of MG for SYMPTOM RELIEF?
Acetylcholinesterase Inhibitors e.g. PO Pyridostigmine.
↑ availability of Acetylcholine.
Short half life so have to give several doses over the course of the day.
What is the medical management of MG for IMMUNE REGULATION?
Steroids e.g. IM methylprednisolone.
Preventative for relapse.
What is the medical management of MG for PREVENTION OF CRISIS OR BEFORE SURGERY?
IVIG. Destroys autoantibodies and prevents production of new autoantibodies
Plasmapheresis
What is the surgical management of MG?
Thymectomy - Thymic hyperplasia is a common association
What is an important Ddx for MG and how does it differ?
Lambert Eaton Myasthenic Syndrome (LEMS)
Paraneoplastic syndrome from small cell lung cancer
HOWEVER it is the pre-synaptic voltage gated calcium channels that are affected
SO you get the opposite whereby muscle weakness improves upon repetitive movements
What is the driving restriction following a seizure?
First unprovoked/isolated seizure: 6 months off if there are no relevant structural abnormalities on brain imaging and no definite epileptiform activity on EEG.
If either abnormality is present then the time increases to 12 months
What is the driving restriction following a syncopal episode? (4)
simple faint: no restriction
single episode, explained and treated: 4 weeks off
single episode, unexplained: 6 months off
two or more episodes: 12 months off
What is the driving restriction following a TIA or stroke?
4 weeks
What are the stages of raised intracranial pressure according to the Monro-Keillie Theory?
1 = compensated so CSF and venous loss to allow for expanding areas
2 = decompensated so no more volume loss and ICP rises but is still normal
3 = positive feedback = ICP greater than BP so oedema
What clinical signs are seen when the midbrain is compressed and why?
Reduced consciousness and GCS due to compression of the reticular formation
What clinical signs are seen when the pons is compressed and why?
Diplopia on horizontal gaze due to compression of CN VI
What clinical signs are seen when the medulla is compressed?
Nausea and vomiting
Usually happens late on
What clinical signs are seen when the brain and therefore optic nerve are compressed?
Papilloedema and reduced visual acuity
leakage of cellular components into optic disc = swelling
What clinical signs are seen when the brain and therefore arterioles are compressed?
Stimulation of Cushing’s response due to a reduction in cerebral perfusion pressure
What is Cushing’s Reflex?
Bradycardia
Hypertension
Respiratory Depression
Why does RICP cause a headache? What are the features?
Due to pressure on the pain receptors in vessels and meninges
Worse on straining/bending over and when waking in the morning
Why do the RICP headache features occur?
Coughing etc = raised intra-abdominal pressure reduces venous return form SVC so increases volume pressing on meninges
Sleep = physiological hypoventilation leads to vasodilation of cerebral vessels (CO2 retention)
What are the observations seen on fundoscopy of RICP?
Blurred disc margins and hyperaemia
Flame haemorrhages
Loss of venous pulsations
What are the causes of RICP? (5)
Focal oedema (Brain haemorrhage, trauma, infarction)
Neoplasms (mets, glioma, meningioma)
Infection (abscess, meningitis, encephalitis)
CSF disturbance
Idiopathic Intracranial Hypertension
What is the first stage of acute RICP treatment after confirming with a CT head?
Elevate head, admit to ITU and sedate
Anaglesia, IV fluids + inotropes
Other bits e.g ventriculostomy I don’t think you need to know them
What is the second stage of acute RICP after confirming with a CT head?
IV mannitol - osmotic diuretic so draws fluid back into the intravascular space
Hypertonic saline
What is the third stage of acute RICP treatment after confirming with a CT head?
Decompressive craniotomy
What are the layers of the scalp?
SCALP
S kin C onnective tissue (dense) A poneurosis L oose connective tissue P eriosteum
What is the clinical significance of the loose connective tissue in the scalp?
Emissary veins connect scalp to dural venous sinuses
no bony insertion for the aponeurosis = bleeding can occur peri-orbitally
What are the 3 layers of the meninges?
Dura mater
Arachnoid Mater
Pia Mater
Describe the Dura Mater. What is its significance
2 layers: periosteal and meningeal
Dural venous sinuses exist between layers along with lots of other vessels
Describe the Arachnoid Mater. What is its significance
Loosely surrounds the brain
CSF exists in here which is for nutrition, protection and creation of a weightless environment
How is CSF produced?
Produced by the choroid plexus in the lateral ventricles
Gravity allows drainage into 3rd ventricle, cerebral aqueduct and 4th ventricle
4th ventricle then allow drainage into SA space
Describe the Pia Mater. What is its significance
Vacuum packs the brain for pathogen protection
v v thin
Why do subarachnoid haemorrhages normally occur?
Spontaneous rupture of berry aneurysm
How does encephalitis normally present?
A triad of fever, headache and a change in mental status
What are the most common causes of encephalitis?
Viral - HSV, CMV or all childhood viruses
Bacterial - TB, listeria, legionella
Fungal and parasitic
Which investigations should be done to diagnose encephalitis?
Bed - stool culture + throat swab
Bloods - All the bloods + blood film
Imaging - CT head to rule out RICP causes
MRI to detect demyelination
Special - LP + viral PCR
Where does the spinal cord terminate?
Lower level of L1 so between L1 and L2
What is the aetiology of cauda equine syndrome?
Compression or damage of the nerve roots below the level of the spinal cord
Iatrogenic following Spinal/epidural/LP
Trauma
Neoplasia
Lumbar stensosis
How does cauda equina normally present symptom wise?
Pain - lower back +/- radiation down lower limbs
Saddle anaesthesia
Urinary retention + constipation/incontinence
LMN signs - hypotonia, weakness
Sexual dysfunction
How does cauda equina normally present examination wise?
LMN signs + sensory impairment esp in perineum
Reduction in anal tone when both resting and contracting
palpable bladder
Which investigations should be done to diagnose cauda equina?
All the bloods
MRI!
Or ct myelogram if not possible
What is the management of cauda equina?
Urgent surgical decompression
What is central cord syndrome? How does it normally present?
Extension injury to an osteoarthritic spine leads to cord injury in the central grey matter
Lower limb fibres are more lateral so are less affected
Compromised motor function and pain/temperature in upper limbs
What is Horner’s syndrome?
Miosis
Partial ptosis (usually unilateral)
Anhidrosis on one side
Due to damage to the sympathetic trunk therefore reducing sympathetic innervation to eye and face
Describe a cluster headache
Episodic eye pain (usually unilateral)
lacrimation
nasal stuffiness
occurring daily and lasts around 15 minutes
What is the management of a cluster headache?
acute: 100% oxygen subcutaneous triptan
Prophylaxis: Verapamil
How can the symptoms of a focal temporal epilepsy help to localise it?
HEAD
Hallucinations
Emotional/epigastric rising
Automatisms e.g. lip smacking/ pinching
Deja vu
How can the symptoms of a focal parietal epilepsy help to localise it?
Sensory!
Parasthesia etc
How can the symptoms of a focal frontal epilepsy help to localise it?
MOTOR
Head/leg movements
posturing
post-ictal weakness
Jacksonian march
How can the symptoms of a focal occipital epilepsy help to localise it?
Visual
Floaters/flashes
Give 3 drugs that may exacerbate a myasthenia crisis?
Bisoprolol/B blockers
Abx e.g. tetracycline/gentamicin
Lithium
What are the 3 most common causes of foot drop?
Common peroneal nerve lesion
L5 radiculopathy
Sciatic nerve lesion
How does a common peroneal nerve palsy normally present?
Weak dorsiflexion
Weak eversion
Normal reflexes
How does a L5 radicuolpathy normally present?
Weak hip abduction
Weak dorsiflexion
Weak eversion and inversion
Parasthesia between the webbing of first and second toe
What is the function of upper motor neurones? How would a lesion affect function?
A net inhibitory effect on LOWER motor neurones
Lesion = LOSS of inhibition of LMN = ↑ excitation
↑ number of action potentials = ↑ muscle contractions = ↑ tone
What are the 4 main signs of an UMN lesion?
Hypertonia
Spasticity
Changes in reflexes
Clonus
What is spasticity and how can it be elicited in an UMN lesion?
Continuous contraction of a muscle and paralysis of its antagonist
Pronator Drift - indicates spasticity as limbs trying to return to pathological position
UL = flexion dependent LL = extension dependent
How do reflexes change following an UMN lesion?
Hyperreflexia
Babinski reflex
Clasp-knife reflex (quick ↓ in resistance following force)
What is Babinski’s reflex?
Rubbing lateral side of the sole of the foot causing extension
Big toe dorsiflexes and the other toes fan out.
Normal in children up to 12 months old.
What is clonus?
Series of involuntary and rhythmic muscular contractions and relaxations.
What is the function of Lower Motor Neurones?
Link UMNs to skeletal muscle
Constantly firing so have to be inhibited by UMNs
What are the 4 signs of a LMN lesion?
Hypotonia + reduced power
Hyporeflexia
Muscle wasting
Fasciculations
What are fasciculations?
Uncoordinated contractions and relaxations of muscle due to ectopic Ach receptor expression
How does an UMN lesion affect the facial muscles and why?
Paralysis of contralateral lower face. Cannot smile but can raise eyebrows
Upper half of face receives innervation from both contralateral and ipsilateral sides
How does a LMN lesion affect the facial muscles and why?
Paralysis of ipsilateral upper and lower half of face. Cannot move one side
Lower half of face receives innervation from only the contralateral side
How does a migraine normally present?
Headache - unilateral, throbbing +/- parasthesia
Prodrome - precedes by days/hours. mood/sleep changes
Aura - precedes headache by ~15-30mins. Visual changes/hemianopia
N&V
Light sensitivity
Mechanophobia
Motor features e.g. ataxia or hemiparesis
What are the triggers of a migraine?
CHOCOLATE covers ~50% cases C hocolate H angers O rgasms C heese/caffeine O ral contraceptive pill L ie ins A lcohol T ravel E xercise
Realistically triggers are
- changes to sleep pattern
- stress
- missing meals
- dehydration
What is the conservative management of a migraine?
Trigger avoidance - keep a headache diary
Pain management - warm/cool pack, rebreathing into a paper bag, acupuncture
What is the acute medical management for a migraine?
NSAIDS/simple analgesia
Triptans at START OF HEADACHE
Anti-emetic e.g. metoclopramide/prochlorperazine
What is the mechanism of action of triptans?
Serotonin 5-HT agonist = vasoconstriction
What is the follow up management for a migraine?
Review in 2-8 weeks and see if preventative management is needed
When would follow up management be needed for a patient with migraine?
Triptans and analgesia = treatment overuse headache
Affecting QOL
What is the preventative management for migraine?
Monoclonal antibodies
propanolol
amitriptyline
Topiramate
Should there be special consideration for females suffering with migraine?
YES
COCP is absolutely contraindicated if patient has migraine with aura
Give POP and advise to stop using immediately if they develop MWA.
How do migraines change during pregnancy?
Often improves in pregnancy
If worsens then is associated with a greater risk of pre-eclampsia
What is a bulbar palsy and which nerves are affected?
Disease affecting CNs due to LMN pathology so get LMN signs.
IX (glossopharyngeal)
X (Vagus)
XI (Accessory)
XII (Hypoglossal)
What is the swallow reflex?
Soft Palate Stimulated and afferent nerve is CN IX (glossopharyngeal)
Goes to Trigeminal Nerve Nucleus then…
CN X = pharyngeal constriction
CN V = Jaw Opens
CN XII = Tongue thrusts
How do the symptoms of bulbar palsy relate to the anatomy?
IX (glossopharyngeal) - Dysphagia = Unsafe swallow
X (Vagus) - Dysphagia and Dysphonia
XI (Accessory) - can’t shrug shoulders or turn head
XII (Hypoglossal) - Atrophic tongue, dysarthria, nasal voice
What are 4 causes of bulbar palsy?
Acute = GBS (Rare)
Chronic = MND, MG, GBS, Syringobulbia
Which investigations should be done for bulbar palsy?
CN Focussed Exam = Swallow? Speech? Shrug Shoulders? Turn Head? Eye movements? Look at tongue?
What is a corticobulbar palsy?
UMN lesion affecting swallowing and speech muscles
Bilateral lesions (PD, Stroke, MS, MND) to corticobulbar tracts above the mid pons
UMN signs
Hyperreflexia of gag reflex
Slow and deliberate speech
Pseudobulbar affect - incongruent crying/laughing
Define Brown Sequard syndrome
A complete hemisection of the spinal cord leading to both sensory and motor loss
What are the CONTRALATERAL signs of Brown Sequard?
Contralateral loss of Spinothalamic Tracts (crude touch, pain, temperature) 2-3 levels below the level of the lesion
What are the IPSILATERAL signs of Brown Sequard
Ipsilateral loss or Dorsal Columns (fine touch, proprioception and vibration) at the level of the lesion and below
Motor
Ipsilateral hemiplegia due to loss of Dorsal Horns (UMN signs) below the level of the lesion
Ipsilateral flaccid paralysis due to loss of Ventral Horns (LMN signs) at the level of the lesion
What could cause a brown sequard syndrome?
Penetrating trauma e.g. stab wound
Vertebral fracture
V artery dissection
What is the definition of cauda equina syndrome?
A collection of symptoms and signs that result from severe compression of the descending lumbar and sacral nerve roots.
What are the symptoms and signs of cauda equina syndrome?
Symptoms
Saddle + anal anaesthesia
Pain (Lower back, Sciatic)
Signs
Loss of autonomic control e.g. bladder or bowel incontinence, sexual dysfunction
Give 6 causes of cauda equina syndrome
Neoplasia - schwannoma, mets from prostate
Vascular - aortic dissection
Degenerative - spinal stenosis, lumbar disc herniation (L2-S2)
Inflammatory - Ank Stond
Define spondylosis
A spinal condition resulting from degeneration and flattening of the intervertebral discs leading to pressure on the nerve roots
What is the pathophysiology behind spondylosis?
Happens because of degeneration of the annulus fibrosis and osteophyte formation on the adjacent vertebra
= narrowing of canal and movement rubs the nerve over osteophytes
How does spondylosis present?
Pain +/- Lhermitte’s Sign
Reduced movement and crepitus
Parasthesia
Muscle Weakness
Sensory loss
(depending on where the nerve root is)
What is Lhermitte’s sign?
Electrical pain down the spine upon neck flexion
What are the investigations to do for spondylosis?
XR = narrowing of disc space and osteophytes
URGENT MRI
How should spondylosis be managed?
Conservative (Physio, Neck Brace/Lumbosacral corset)
Medical (Analgesia - NSAIDS/follow WHO ladder)
Surgical - Fusion of vertebrae
What is Syringomyelia?
A cavity (syrinx) forms within the brainstem (bulbia) or spinal cord (myelia).
The syrinx is filled with CSF and expands outwards from the central canal AT THE SEGMENTAL LEVEL
Which tracts are affected in Syringomyelia and why?
Spinothalamic Tracts - Bilateral loss = cape like distribution
Nerves cross AT THE SEGMENTAL LEVEL and so damaged area
Which tracts are NOT affected in Syringomyelia and why?
Dorsal Columns
Nerves DO NOT CROSS AT SEGMENTAL LEVEL as ascend on ipsilateral side so not affected
Get DISSOCIAL sensory loss
What are the myotomes for:
C4 C5 C6 C7 C8
C4 = shoulder shrugs
C5 = Elbow flexion
C6 = Wrist extension
C7 = Elbow extension and wrist flexion
C8 = Finger flexion and thumb extension
What are the myotomes for
T1 L2 L3 L4 L5
T1 = Finger abduction
L2 = Hip flexion
L3 = Knee extension
L4 = Ankle dorsiflexion
L5 = Big to extension
What are the myotomes for
S1
S1 = Ankle plantarflexion
What is the definition of vitamin B12 deficiency
A common condition that can manifest with neurological, psychiatric and haem symptoms
How does a B12 deficiency lead to anaemia?
B12 is a cofactor for enzymatic reactions in DNA synthesis (methionine synthesis) and FA metabolism
↓DNA synthesis = macrocytic anaemia + pancytopenia
Also ↓ conversion of homocysteine to methionine = neuropathy, glossitis and CVS disease
FA not metabolised = demyelination of nerves
How does inadequate absorption of B12 occur?
↓ in Intrinsic Factor (Drugs - PPI or Metformin use, Atrophic gastritis (H. Pylori, Gastrectomy)
Terminal Ileum pathology (Ulcerative colitis/Crohns +/- a resection, Coeliac Disease, Pancreatic insufficiency)
Give 2 other mechanisms for b12 deficiency?
Lack of dietary intake (found in meat and dairy) - Strict veganism, chronic alcohol use
Increased demand for B12 - Leukaemia, Pregnancy, Breastfeeding
What is subacute degeneration of the spinal cord?
Loss of vibration and proprioception (Dorsal Columns) @ hands and feet
Progresses to sensory loss @ all modalities
Gait ataxia
Distal Muscle Weakness (esp legs)
+/- dementia features
Which blood tests are for B12 deficiency?
FBC
Peripheral Blood
Smear
Serum B12
Reticulocyte Count
Autoantibodies - Anti-IF and Anti-Parietal Cell (pernicious anaemia)
What is the management of B12 deficiency?
Replace via IM injections
Neuro symptoms might be irreversible 🤷🏽♀️
What is the definition of diabetic neuropathy?
A highly prevalent complication of diabetes (type 1 or type 2) and is characterised by the presence of symptoms and/or signs of peripheral nerve dysfunction and/or autonomic nerve dysfunction.
What is the pathophysiology behind Diabetic neuropathy?
Hyperglycaemia causes:
Ischaemia to nerves supplying small vessels
Inflammation to nerves
SO,
metabolic and vascular dysfunction leads to mitochondrial dysfunction
Leads to progressive and accumulative damage to nerves
How would an EARLY diffuse diabetic neuropathy present?
Distal, symmetrical pattern of sensory loss
Glove and sto
cking distribution
Pain (prickling, burning, worse @ night)
Ulcers
How would a late diffuse diabetic neuropathy present?
Autonomic dysfunction
Orthostatic Hypotension
Bladder/Bowel/Sexual dysfunction
GI - bloating, early satiety
How would a diabetic mononeuropathy present?
Isolated nerve palsies e.g. CN, PN - foot drop, carpal tunnel
What are the risk factors for diabetic neuropathy?
Non-Modifiable - Age, disease duration, Tall height
Modifiable - Lifestyle (smoking, obesity, poorly controlled hyperglycaemia), CVS (HTN, Hyperlipidaemia)
What are 4 differentials for diabetic neuropathy?
B12 deficiency
Chronic Inflammatory Demyelinating Polyneuropathy (more motor)
GBS
Drug induced Neuropathy
What are 3 complications of diabetic neuropathy?
Ulcers!!! (infection +/- gangrene, amputation, Charcot foot)
Depression
Silent MI
What should you look for on examination when diabetic neuropathy is suspected?
Symmetrical, distal SENSORY loss
Reduced or absent ankle reflexes
? Ulcers
? Ataxia
Which bedside tests should be done in diabetic neuropathy?
Baseline obs - BP for postural drop???
Which blood tests should be done in diabetic neuropathy?
HbA1c, fasting blood glucose - is the diabetes well controlled?
B12 - exclude deficiency
FBC - exclude anaemia and other inflammatory disorders
TFTs, lipids?
What is the conservative management of diabetic neuropathy?
Annual review
Supportive - help to manage glycaemic control
Self care education about feet (grim)
What is the medical management of diabetic neuropathy?
Pain management
Pregablin/Gabapentin +/- duloxetine
Antidepressant
Opioid
Alpha 1 Agonist for autonomic dysfunction
Define carpal tunnel syndrome
A clinical syndrome that occurs due to impingement of the median nerve (C7) as it passes underneath the flexor retinaculum
What are the symptoms/signs of carpal tunnel?
Sensory changes in the C7 dermatome
Numbness
Parasthesia
Pain
Wasting of the thenar eminence
Clumsy
What are the causes of carpal tunnel
Median Trap
Myxoedema (hypothyroidism) Edema Diabetes Mellitus Idiopathic Acromegaly Neoplasia Trauma Rheumatoid Arthritis, Amyloidosis Pregnancy
What are the risk factors for carpal tunnel?
Non-Modifiable
- Female - Age (40-60) - Hx wrist fracture - Rheumatoid arthritis
Modifiable
- Lifestyle - smoking - Occupation involving lots of wrist work - Walking aids
Give 2 investigations for carpal tunnel?
Tinel’s Test - Tap on median nerve at wrist + parasthesia = POSITIVE
Phalen’s Manoeuvre - Flex hands 30-60 degrees + parasthesia = POSITIVE
What is the management of carpal tunnel?
Splint
NSAIDs
Cortiocsteroids
Surgical release
What is the definition of MND?
A cluster of neurodegenerative diseases characterised by selective loss of neurones in the:
Motor Cortex Frontotemporal Cranial Nerve Nuclei Bulbar (pons, medulla) Anterior Horn Cells
usually due to cytotoxicity by glutamate excess
Where is most affected by motor neurone disease?
Both UMN and LMN
BUT ABSOLUTELY NOT
sensory neurones (polyneuropathy and MS), sphincters and eye muscles (=movements like in myasthenia)
What are the 3 types of MND?
Amyotrophic Lateral Sclerosis (UMN and LMN)
Primary Lateral Sclerosis (UMN)
Progressive Muscular Atrophy (LMN)
What is the usual presentation of ALS? (5)
Proximal myopathy, foot drop, spastic gait = common
Progressive disability with no periods of remission or stability
Asymmetrical!!!
UMN and LMN signs
> 40 (usually)
What is the management of MND?
Conservative (MDT involvement - Physio, OT, SALT, Education on end of life care and ways to improve QOL)
Medical (Riluzole - reduces Glutamate.
Anti-cholinergics (to stop dribbling ‘sialorrhoea’
Annual pneumococcal and flu vaccines)
Surgical
PEG can improve QOL
What is the definition of muscular dystrophy?
A progressive, generalised diseases of muscle, most often caused by defective or specifically absent glycoproteins (e.g., dystrophin) in the muscle membrane.
Characterised by ongoing degeneration and re-generation of muscle fibres.
What is the pattern of inheritance in both Duchenne and Beckers muscular dystrophy?
X-linked recessive
What is the usual clinical presentation of muscular dystrophy?
Delayed motor milestones* e.g. head control by 4 months, walking by 18 months, running by 3 years
Waddling gait/tiptoe walking
Calf pseudohypertrophe (large due to fibrosis and fat)
Episodes of unexplained myoglobulinaemia
Gower’s Sign
What is Gower’s sign?
Slowly try to get up from lying on tummy by using their arms
Due to symmetrical, proximal muscle weakness
What are 3 later signs of muscular dystrophy?
Respiratory failure
Scoliosis
Dilated cardiomyopathy
How do Duchenne and Becker’s MD differ?
D = Deficit of dystrophin. Symptoms are more severe and earlier onset (~5 years old). ID = common
B = Misshapen dystrophin. Symptoms are less severe and later onset (10-20)
What is the conservative management of Muscular Dystrophy?
Physio and conditioning
Genetic counselling for parents
What is the medical management of muscular dystrophy?
Glucocorticoids can prolong independent walking and improve muscle strength
However, also cause weight gain and behavioural abnormalities so have to weigh up choices
What is the definition of Wernicke’s encephalopathy?
A neurological emergency resulting from thiamine deficiency with varied neurocognitive manifestations, typically involving mental status changes and gait and oculomotor dysfunction.
How does chronic alcohol consumption lead to B1 deficiency?:
Conversion of Thiamine to its active form
Absorption of Thiamine @ the Duodenum
Storage of Thiamine in the liver due to cirrhosis
(and also causes malnutrition)
What is the triad of symptoms seen in Wernicke’s?
Ataxia
Autonomic Instabiity
Nystagmus
(+ confusion)
What is the management for Wernicke’s?
Pabrinex/IV B1 - Levels need to be corrected before giving glucose otherwise there won’t be a glycolysis mechanism = lactic acidosis
What is the definition of Korsakoff’s syndrome?
A residual syndrome in patients who suffered from a Wernicke encephalopathy and did not receive appropriate treatment at that time. It is chronic and irreversible.
What are the symptoms of Korsakoff’s?
Retrograde and anterograde amnesia
Confabulation
What is the definition of Horner’s syndrome?
Compression of the sympathetic chain leads to reduced sympathetic innervation to head and neck.
What is the triad of symptoms seen with Horner’s?
Partial ptosis (Reduced innervation to Superior Tarsal Muscle so can’t elevate eyelid)
Unilateral anhidrosis
Miosis (Reduced innervation to Dilator Pupillae so can’t dilate)
Give 4 causes of Horner’s syndrome
Vascular - ICA dissection, Cavernous sinus thrombosis, lateral medullary syndrome
Inflammatory - Syringomylia
Trauma - Atlas fracture
Neoplasia - Pancoast Tumour
Give 3 ddx for ptosis and how they differentiate from each other
CN III lesion - Ptosis is unilateral and complete
Sympathetic Lesions e.g. Horners - Ptosis is unilateral and partial
Myopathy - Ptosis is bilateral and partial e.g. MG
Define temporal arteritis
Granulomatous vasculitis of large and medium-sized arteries. It primarily affects branches of the external carotid artery, and it is the most common form of systemic vasculitis in adults.
What are the branches of the external carotid artery
Some Angry Ladies Figured Out PMS
Superior Thyroid Ascending Pharyngeal Lingual Facial Occipital Posterior Auricular Maxillary Superficial Temporal
How does temporal arteritis normally present?
Headache over the temporal region (HEADACHES IN >50 ARE GCA UNTIL PROVEN OTHERWISE AS CAN HAVE PERMANENT VISUAL LOSS)
Optic neuropathy/visual disturbances?
Monocular sudden loss of vision/Amaurosis Fugax
Weight Loss
Fatigue, malaise
Sweats
Jaw claudication
Vessels can become tender, thick, nodular and pulsatile = Occlusion
What are the non-modifiable risk factors for temporal arteritis?
Age >50
Female > Male
Caucasian > other ethnicities
Hx of Polymyalgia Rheumatica
What are the modifiable risk factors for temporal arteritis?
Smoking
Other CVS risk factors e.g. atherosclerosis
Investigations
Which blood tests should be done in temporal arteritis?
ESR - Raised in GCA. If ESR is not raised then it isn’t GCA.
LFTs - Raised ALP
IgG - Raised
Creatine Kinase is not raised (no muscle damage)
What diagnostic investigation should be done in temporal arteritis?
Temporal Artery biopsy is gold standard
Have to do serial sectioning and large segments incase a bit is missed
Shows mononuclear cells that have joined together to form giant cells
What is the management of temporal arteritis?
PO prednisolone
What are the typical features of a post-lumbar puncture headache?
develops within 24-48 hours following LP but may occur up to one week later
may last several days
worsens with upright position
improves with recumbent position
What is the management of a post-lumbar puncture headache?
Supportive - analgesia, fluid, rest
IV caffeine, blood patch, epidural saline if more than 72 hours
Define Bell’s palsy
An acute, unilateral, idiopathic, facial nerve paralysis.
What are the features of Bell’s palsy?
lower motor neuron facial nerve palsy - forehead affected i.e. forehead not spared
patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis
What is the triad seen in normal pressure hydrocephalus?
urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson’s disease)
Define degenerative cervical myelopathy
Spinal cord dysfunction that occurs when age-related osteoarthritic changes cause narrowing of the cervical spinal canal, leading to chronic spinal cord compression and neurologic disability.
How does degenerative myelopathy present?
Pain
Loss of motor function
Loss of sensory function causing numbness
Loss of autonomic function (urinary or faecal incontinence and/or impotence
Hoffman’s sign:
What is Hoffman’s sign?
A reflex test
gently flick one finger on patient’s hand.
A positive test = in reflex twitching of the other fingers on the same hand in response to the flick.
What is the gold standard test for degenerative myelopathy?
An MRI of the [cervical] spine
It may reveal disc degeneration and ligament hypertrophy, with accompanying cord signal change.
What is the treatment for degenerative myelopathy?
Decompressive surgery
Should the DVLA be informed after a provoked seizure?
Yes
The patient has to inform them and it is their decision whether they can drive again
(probably ok if they’re provoked)
