Neurology Flashcards
How do you examine mental status in neuro?
Attention Language Memory Visuospatial function Neglect Frontal lobe function
How do you examine cranial nerve II?
Visual acuity
Visual fields
Pupils
Funds copy
What nerves are extra ocular movements testing?
Cranial nerves III, IV and VI
How do you test the trigeminal nerve?
Facial sensation
What nerve supplies facial muscles?
Facial, CNVII
How do you test cranial nerves IX, X and XII?
Palate and tongue movement
What are the aspects of testing motor function?
Bulk (inspection)
Tone
Power
What is the C5 myotome?
Shoulder abduction
Describe the myotomes of the upper limb
C5 shoulder abduction C5,6 elbow flexion C7 elbow extension C6,7 wrist extension C7,8 wrist flexion C7 finger extension C8 finger flexion T1 thumb/finger abduction
Describe the myotomes of the lower limb
L1,2 hip flexion L5,S1,2 hip extension L5,S1,2 knee flexion L3,4 knee extension L4,5 ankle dorsiflexion S1,2 ankle plantar flexion L5 dorsiflexion of great toe L4,5 ankle inversion L5,S1 ankle ever soon
Describe the MRC grading of muscle power
0 no visible contraction
1 flicker or trace of contraction
2 active movement with gravity eliminated
3 active movement against gravity
4 active movement against some resistance
5 normal power
Describe the innervation of the upper limb reflexes
Biceps C5/6
Brachioradialis C5/6
Triceps C7
Describe the innervation of the lower limb reflexes
Knee L3/4
Ankle S1/2
What is the babinski reflex?
Stroke the lateral sole
Up going plantar reflexes is an UMN sign
I.e. The first movement is up = abnormal
What are the aspects to checking sensation?
Pinprick
Temperature
Vibration
Proprioception
How do you test coordination?
Finger-to-nose or heel-to-shin
Rhythm of movements - rapid alternating movements
What is Romberg’s test?
Steadiness with feet together and eyes closed
Tests for sensory ataxia
What are you looking for in gait in neuro exam?
Stance - narrow or wide base
Shuffling gait and decrease arm swing (PD)
Ataxia
What are the distinguishing features of a brain lesion?
Higher cortical functions disrupted I.e. Speech, personality, memory etc Unilateral signs (contra lateral to affected side)
What are extra pyramidal signs?
Akinesia
Rigidity
Tremor
What are the signs of a brainstem problem?
Cranial nerve signs / abnormalities
What are the cerebellar signs?
Dysdiadokinesia Ataxia Nystagmus Intention tremor Slurred speech Past-pointing
What are the distinguishing features of a spinal cord lesion?
Attributable to a level
Bilateral
Bladder/bowel involvement
How does a nerve root lesion present?
Dermatomal and myotomal distribution
How does a peripheral nerve lesion present?
LMN signs
Glove and stocking sensorineural deficit
Sensory ataxia
Where and what is Broca’s area?
Frontal lobe
Motor area for speech
Damage leads to expressive dysphasia
Where are what is Wernicke’s area?
Parietal lobe
Sensory input for speech
Receptive dysphasia
What are the symptoms and signs of a frontal lobe lesion?
Personality changes
Mono paresis (legs)
Incontinence
Dysphasia (expressive)
What are the signs and symptoms of parietal lobe lesions?
Language difficulties Hemlineglect Visuospatial deficit Leg weakness Homonomous hemianopia Dyspraxia Agnosia
What is the function of the temporal lobe?
Auditory
What is the function of the occipital lobe?
Visual
What is the function of the cerebellum?
Coordination
What are the different parts of the brainstem called?
Midbrain
Pons
Medulla oblongata
What are the functions of the meninges?
Support and mechanically stabilise the contents of the cranium
Organise/divide cranial cavity into anatomical parts
Membranous envelopes that completely cover the brain
What are the meningeal layers?
Dura mater (periosteal + meningeal layers)
Arachnoid mater
Pia mater
What is in the subarachnoid space?
CSF
What are the functions of CSF?
Bathes the brain
Cushions against mechanical agitation
Reservoir for metabolic substrates for the brain
Dissolves and carries away products of metabolism
What is the difference between the meninges of the brain and spinal cord?
Dura mater is a single layer in the spinal cord
At what level is an LP done and why?
L3/4 in adults
Spinal cord terminates at L2
Where is the thalamus?
Wrapped around the 3rd ventricle
What is the function of the hypothalamus?
Regulates visceral functions eg temperature, endocrine, feeding/drinking, emotional states and sexual behaviour
What is the function of the cerebellum?
Integration, regulation and coordination of motor processes
What forms the brainstem?
Midbrain, medulla oblongata and pons
What is the function of the medulla oblongata?
Controls autonomic function
Eg respiration, cardiac centre + baroreceptors, vomiting, coughing, sneezing and swallowing centres
Where do the cranial nerves originate?
3&4 midbrain
5,6,7,8 pons
9,10,11,12 medulla
What is the cauda equina?
Group of nerve roots running in the lumbar cistern
Extend beyond the termination of the cord at L2 level, to reach the remaining vertebral foramina
Where does the spinal cord terminate in adults?
L2
How does cauda equina present?
Bladder, bowel and sexual dysfunction
Sensory changes in saddle and perianal areas
Back pain
Sensory changes or weakness in lower limbs
Reduction or loss of reflexes in lower limbs
What are the common causes of cauda equina syndrome?
Large central IV disc at L4/5 or L5/S1 level
Tumours, trauma, spinal stenosis, inflammatory disease
Which tract is responsible for fine touch and Proprioception?
Dorsal column
Where does the dorsal column decussate?
Medulla (brainstem)
What tract is responsible for pain and temperature sensation?
Lateral spinothalamic
Where does the lateral spinothalamic tract decussate?
Spinal cord (ie straight away)
Which column is responsible for crude touch and pressure sensation?
Anterior spinothalamic
What is the clinical significance of the decussation of the lateral spinothalamic tract?
It decussates straight away in the spinal cord, meaning a lesion always causes CONTRAlateral signs - for pain and temperature
What are pyramidal tracts?
Originating in cerebral cortex
What are extra-pyramidal tracts?
Don’t originate in the cerebral cortex
Eg originating in brainstem eg tectospinal, rubrospinal
Name 2 descending tracts of the pyramidal system
Lateral corticospinal
Ventral corticospinal
What is amyotrophic lateral sclerosis?
Progressive degenerative disease in which corticospinal tracts and central horn cells degenerate
Results in weakness and loss of control to muscles
What is Brown-Sequard syndrome?
Lateral hemisection of the cord
Causes loss of sensation and motor function
How does Brown-Sequard syndrome present?
Spastic paralysis of ipsilateral side
Loss of fine touch and proprioception ipsilaterally
Loss of pain, temp and pressure sensation to CONTRAlateral side due to damage to spinothalamic tract
What is the classic cause of Brown-Sequard syndrome?
Stab wound to back
What is anterior spinal artery syndrome?
Caused by ischaemia of the spinal artery
Affects corticospinal tracts and results in motor paralysis
What is syringomyelia?
Development of cyst/cavity around the central canal
Grows and spreads out over time
Typically affects spinothalamic tract as this decussates just anterior to the central canal
Reduced temp and pain sensation at level of lesion
Can affect motor as it extends
Name the bones of the skull
Frontal Parietal Occipital Ethmoid Sphenoid Temporal
What are the cranial fossae?
Anterior, middle and posterior
Accommodate and support the brain
Name the meninges
Dura mater
Arachnoid mater
Pia mater
What are the falx cerebri and tent oriel cerebelli?
Thick infoldings of the meninges
What are the CSF spaces in the brain?
Sulci
Fissures
Basal cisterns
Ventricles
What are the calcified structures within ventricles?
Choroid plexus
What is the difference between white and grey matter on CT and why?
White matter is darker
It has a high content of myelinated (fatty) axons
So it has a lower density
What are the important grey matter structures in the brain?
Cerebral cortex
Insula
Basal ganglia
Thalamus
What is the insula?
Inner surface of the cortex
Found deep in the Sylvian fissure
What are the internal capsules?
White matter tracts which connect with the corona radiata, white matter of cerebral hemispheres and the brainstem
What is the corpus callosum?
White matter tract located in the midline
Arches over lateral ventricles
How can malignant lesions spread between hemispheres?
Via the corpus callosum
What is found in the posterior cranial fossa?
Cerebellum and brainstem
Which artery supplies the largest area of cortex?
Middle cerebral artery
What calcified structures are found within the brain?
Choroid plexus
Pineal gland
Basal ganglia
Falx cerebri
How can you differentiate between cranial sutures and fractures?
Along the edge of the lesion, if the bone is corticated this means it is a suture
Fractures are also straighter than suture lines
What is hydrocephalus?
Increased production or decreased absorption of CSF
Can result in massive enlargement of ventricles
What changes do you see on CT in Alzheimer’s disease?
Temporal lobes reduced in volume
Enlargement of temporal horns of lateral ventricles
What is the likely diagnosis with generalised reduction in CSF space volume?
Cerebral oedema
What is small vessel disease on CT?
Generalised low density (darkening) of cerebral white matter
Represents chronic ischaemia
Associated with vascular risk factors
What are lacunae infarcts?
Area of brain cell death due to a focal ischaemia event
What are the signs of acute stroke on CT?
Subtle low density
Hyper dense artery sign - thromboembolic material within a cerebral artery
Insular ribbon sign - loss of clarity in the insular ribbon
What are the different types of extra-axial haemorrhage?
Extra dural, su dural and subarachnoid
Where does the blood commonly originate from in an epidural haematoma?
An intracranial artery, most commonly middle meningeal
What does an extra dural haematoma look like on CT?
Lens-shaped collection
What is the underlying pathology of a sub dural haematoma?
Fragile cerebral VEINS
Increased risk in elderly and anticoagulated
May result from minor trauma, or no trauma at all
What does a sub dural haematoma look like on CT?
Crescent-shaped collection (ie all along one side of the brain)
What are subarachnoid haematomas commonly caused by?
Trauma
Spontaneous bleeding from an intracranial aneurysm
What does a subarachnoid haemorrhage look like on CT?
Blood in ventricles or Sulci, fissures
Small foramina connect the subarachnoid space with the CSF spaces
What is the difference between intra- and extra-axial lesions?
Intra axial are within the brain
Extra axial are outside the brain
What are the most common intraaxial lesions?
Neoplastic and malignant
Single - more likely to be primary
Multiple - more likely to be metastatic
What are the most common extra axial lesions seen on head CT?
Meningiomas
What are Meningiomas?
Benign tumours arising from meninges and remaining in contact with them
Can grow very large and surrounded by an area of cerebral oedema
What is mass effect in the cranium?
Intracranial volume can’t change
Any space-occupying intracranial lesion may increase the intracranial pressure and displace the soft tissues of the brain
What pathological process affects mass effect?
Cerebral oedema - worsens mass effect
In infarcts, mass effect comes solely from cerebral oedema
What happens in extreme cases of mass effect?
Coning - extrusion of the posterior fossa structures through the for amen magnum
What is herniation?
Tentorial - structures pass through the incisura tentoria (gap at top of tent normally occupied by brainstem and basal cisterns)
What is meningism?
Acute severe headache
Felt over most of head
Accompanied by neck stiffness
What are the causes of meningism?
Meningitis
Encephalitis
Subarachnoid haemorrhage
How do you manage a patient presenting with meningism?
Admit urgently for CT head
If CT negative, do LP to look for signs of infection or blood products in CSF
What are the features of meningitis?
Fever Photophobia Stiff neck Purpuric rash Coma
What are the features of venous sinus thrombosis?
Subacute or sudden headache
Papilloedema
How does sinusitis present?
Dull constant ache over frontal or maxillary sinuses
Post-nasal drip
Pain worse on bending over
How does acute glaucoma present?
Constant aching pain around 1 eye
Radiates to forehead
Reduced vision & visual haloes, nausea and vomiting
Eye looks red and congested, cornea cloudy
Dilated non-responsive pupil
How do you manage acute glaucoma?
Seek expert help immediately
If delay >1hr start acetazolamide 500mg IV over few mins
What are the common causes of recurrent attacks of headache?
Migraine
Cluster headache
Trigeminal neuralgia
Recurrent meningitis
What are the features of cluster headaches?
Rapid onset excruciating pain around 1 eye
Eye may become watery and bloodshot, lid swelling, lacrimation, facial flushing
Pain always UNIlateral
Lasts 15-60mins, 1-2x per day, often nocturnal
How do you treat cluster headaches?
100% O2 for 15mins
Sumitriptan subcut 6mg at onset
What are the features of trigeminal neuralgia?
Intense, stabbing pain lasting seconds in trigeminal nerve distribution
Unilateral
Face screws up with pain
What are common triggers for trigeminal neuralgia?
Washing affected area Shaving Eating Talking Dental procedures
Give some secondary causes of trigeminal neuralgia
Compression of trigeminal root eg tumour
Chronic meningeal inflammation
MS
Zoster
What drugs are used to treat TN?
Carbamazepine
Lamotrigine
Phenytoin
Gabapentin
How does Giant Cell Arteritis present?
Subacute headache
Tender, thickened, pulseless temporal arteries
Jaw claudication
What investigation is required in suspected GCA?
ESR (>40mm/HR)
How do you treat GCA?
Prednisolone
What are the features of tension headache?
Bilateral
Non-pulsatile
Scalp muscle tenderness
What are the features of raised ICP?
Worse on waking / lying down / bending forward / coughing Vomiting Papilloedema Seizures False localising signs
What is medication overuse headache?
Episodic headaches becoming chronic daily headache
Especially with mixed analgesics (paracetamol + codeine/opiates)
What are the classic symptoms of migraine?
Visual or other aura for 15-30mins
Followed within 1 hour by unilateral throbbing headache
What are the other presentations of migraine?
Isolated aura with no headache
Episodic headaches without aura - often premenstrual
Allodynia - all stimuli produce pain eg brushing hair
What is a migraine prodrome?
Precedes headache by hours or days
Yawning, cravings, mood or sleep changes
What is aura?
Precedes migraine headache by minutes
May continue during it
Visual, somatosensory, motor, speech
What are the criteria for diagnosing migraine (if no aura)?
5 or more headaches lasting 4-72hrs, nausea or photophobia \+ 2 of... Unilateral Pulsating Impairs or worsened by routine activity
What are the common triggers of migraine?
CHOCOLATE Chocolate Hangovers Orgasms Cheese Oral contraceptives Lie-ins Alcohol Tumult Exercise
What are the differential diagnoses for migraine?
Cluster or tension headache Cervical spondylosis Hypertension Intracranial pathology Sinusitis or otitis media TIAs can mimic aura
What are the principles of migraine treatment?
Analgesia
Abortive eg triptans
Prevention eg propanolol, amitriptyline
What medications are used in acute migraine?
NSAIDs eg aspirin
Triptans (5HT agonists) eg sumatriptan
Ergotamine 1mg PO as headache starts
What are the medications used to prevent migraines?
Propanolol
Amitriptyline
Topiramate
What are the non-pharmacological measures to treat migraine?
Warm or cold packs to head
Rebreathing into paper bag
Spinal manipulation
What is the relationship between migraine and the pill?
Slight increased risk of stroke if on the pill and have migraine with aura
Women should stop the pill immediately if they develop migraine with aura, and use alternative or low-dose pill
Define stroke
Syndrome of rapidly developing symptoms or signs of focal loss of CNS function
Symptoms last more than 24hrs or result in death
What are the vascular mechanisms causing stroke?
Infarction - embolisation or thrombotic
Haemorrhage
In what conditions can cardiac emboli arise and cause stroke?
AF
Endocarditis
MI
What are the modifiable risk factors for stroke?
Hypertension Smoking Diabetes Heart disease - valvular, ischaemia, AF PVD Past TIA Carotid bruit Hyperlipidaemia Excess alcohol
What are the different types of stroke according to the Oxford classification?
TACS
PACS
LACS
POCS
What artery is involved in TACS?
Middle or anterior cerebral
What is the commonest cause of TACS?
Cardiac emboli (AF)
What are the signs of TACS?
Higher cortical dysfunction
Hemiparesis or hemisensory
Homonymous hemianopia
What artery is affected in PACS?
Middle or anterior cerebral
What is the commonest cause of PACS?
Carotid disease
What is the difference between TACS and PACS?
PACS has better prognosis
PACS only has 2 out of 3 signs, or higher cortical dysfunction alone
What is the cause of a lacunar stroke?
Thrombus in situ
What are the signs of LACS?
Only motor or only sensory
Arm, face and leg
What are the signs of POCS?
1 of:
A. Cerebellar or brainstem syndromes
B. LOC
C. Isolated homonymous hemianopia
What is the prognosis of posterior cerebral stroke?
Many don’t survive, but if they do then prognosis is good
What is the ROSIER score?
Score greater than 0 raises suspicion of stroke. 1 pt each: Face Arm Leg weakness Speech disturbance Visual field defect Minus 1 for: seizure, LOC or syncope
What is the acute management of suspected stroke?
Airway Pulse, BP, ECG Blood glucose: aim for 4-11 Urgent CT if thrombolysis considered or high-risk of bleed or signs of raised ICP Thrombolysis: if onset within 4-5hr NBM if ?dysphagia Aspirin 300mg AFTER haemorrhage excluded
What is used for thrombolysis?
IV alteplase
What are the contraindications to thrombolysis?
Major infarct or haemorrhage on CT Past CNS bleed Severe liver disease Seizures at presentation Anticoagulants or INR>1.7 Platelets <100 BP >220/130
What is the NIHSS?
National institute of health stroke scale
Used to assess deficits on admission then again at review
Higher score means more severe stroke
What are the differential diagnoses for stroke?
SAH Subdural haemorrhage Venous infarcts Malignancy Abscesses Hemiplegic migraine Functional hemiparesis
What are the factors involved in primary prevention of stroke?
Treat hypertension, diabetes, Hyperlipidaemia, cardiac disease
Exercise
Smoking cessation
Folate supplements
Anticoagulation for AF, prosthetic valves
How do you decide if someone with AF should be anticoagulated?
CHADSVASc
What is secondary prevention for stroke?
Prevent further strokes…
Control risk factors
Antiplatelets after stroke - clopidogrel (as long as no haemorrhage)
Anticoagulation if stroke from AF - warfarin from 2 weeks after stroke
What investigations are done in stroke patients and why?
BP, HbA1c, lipid profile 24hr ECG ?AF CXR Echo ?mural thrombus Carotid Doppler USS (>70% stenosis is significant)
What is the mortality for strokes in the UK?
20% at 1 month
What are the complications of stroke?
Aspiration pneumonia Broncho-pneumonia Pressure sores Subluxation (flaccid paralysis) or contractures (spastic) Constipation Vascular dementia Depression
What 4 areas can you quickly assess for cognitive functions?
Perceptual function: point to a part of body
Spatial ability: copying matchstick patterns
Apraxia: copy a clock face
Agnosia: picking out and naming easy objects
How are TIAs followed up?
Aspirin 300mg for 14 days
Clinic appt within 24hrs if high-risk; 7days if low
No driving for 1 month
Return immediately if they have more symptoms
How do you calculate risk of imminent stroke following TIA?
ABCD2 score... Age >60 BP >140/90 Speech disturbance (1) unilateral weakness (2) Duration of TIA 10-59mins (1) >60mins (2) Diabetes 1-3=Low 4-5=Mod 6-7=High
What is myasthenia gravis?
Autoimmune Antibodies against nACh receptors Causes depletion of post-synaptic sites Interferes with neuromuscular transmission Fluctuating weakness of skeletal muscles
What muscle groups are commonly involved in myasthenia gravis?
Extra ocular
Bulbar: dysphagia, dysphonia, dysarthria
Limbs
Respiratory
What is the characteristic symptom of myasthenia gravis?
Fatiguability of skeletal muscle during activity
Fluctuating, painless weakness
Muscles strong in morning and progressively weaker with activity
What are the effects on extra ocular muscles in MG?
Ptosis - often worse 60-90seconds sustained upgaze. Gradual ptosis with compensatory frontalis contraction. Resting (closing eyes) causes muscle power to return
Pupils are UNaffected
Facial mobility may be impaired
What factors can precipitate myasthenia gravis?
Emotional stress Pregnancy Menses Thyroid dysfunction Trauma Hypokalaemia Drugs eg beta-blockers, CCBs, ACEi Surgery
What are the complications of myasthenia gravis?
Respiratory failure, aspiration or infection
Myasthenic crisis
Cholinergic crisis
What is myasthenic crisis?
Severe exacerbation, can be dramatic... Facial muscles slack, expressionless Slack jaw Unable to support head Limp body Gag reflex lots - risk of aspiration
What is cholinergic crisis?
Miosis + SSLUDGE Salivation Sweating Lacrimation Urinary incontinence Diarrhoea GI upset Emesis
What causes cholinergic crisis?
Excess cholinesterase inhibitors
Excessive ACh stimulation of striated muscle at nicotinic junctions produces flaccid paralysis, clinically hard to distinguish from weakness due to MG
How do you diagnose myasthenia gravis?
AChR antibodies
Tensilon test: IV anticholinesterase (tensilon) - improves muscle contractility
Caution in asthma, MI and bradycardia (cardiac monitoring and have atropine ready)
EMG may show muscle fatigue
How do you diagnose cholinergic crisis?
Tensilon test - IV anticholinesterase agent, makes weakness worse
NB in diagnostic tensilon test for MG, improvement is seen
How do you manage acute myasthenia gravis?
Beside spirometry: monitor FBC
Falling FVC may require ICU for ventilation
What are the drugs used to treat myasthenia gravis?
Anticholinesterase inhibitors e.g. Neostigmine
Steroids: decrease immune response
Azathioprine
What is Lambert-Eaton syndrome?
Destruction of pre synaptic calcium channels to prevent neurotransmitter release
Gives a lot of autonomic symptoms due to similarity between channels
NB no autonomic problems in myasthenia gravis
What is Guillain-Barré syndrome?
Ascending polyneuropathy
What is the commonest form of GBS?
Post-infectious polyneuropathy
Causes ascending polyneuropathic paralysis
Acute, rapidly progressing and potentially life-threatening
What is the Miller Fisher variant of GBS?
Sensory ataxia
Arreflexia
Ophthalmoplegia
What are the required features for GBS diagnosis?
Progressive weakness in both arms and legs
Arreflexia
When does GBS commonly occur?
1-3 weeks after URTI or GI infection
What are the complications of GBS?
Autonomic dysfunction: Cardiac arrhythmias Postural hypotension Hypertension Urinary retention Ileus Resp failure: bedside spirometry!
How do you manage Guillain-Barré syndrome?
Close observation (weakness is progressive) Bedside spirometry Ventilatory support ECG Nutritional support VTE prophylaxis Catheter Laxatives Pain control
What is the prognosis for Guillain-Barré syndrome?
25% require ventilation
70% have complete recovery at 1yr
25-30% have some residual deficit
How do polyneuropathies present?
Distribution usually symmetrical and widespread
Often distal weakness and sensory loss (glove and stocking)
What conditions cause mainly motor polyneuropathy?
Gillian-barre syndrome
Lead poisoning
Charcot-Marie-Tooth syndrome
What conditions cause mostly sensory polyneuropathy?
Diabetes
Renal failure
Leprosy
What are the features of autonomic polyneuropathy?
Postural hypotension Erectile dysfunction Reduced sweating Constipation and nocturnal diarrhoea Urinary retention Horner's syndrome
What are the causes of autonomic polyneuropathy?
Diabetes Guillain-Barre Sjögren's syndrome HIV Leprosy SLE
What is the treatment of choice for neuropathic pain?
Amitriptyline 10-25mg at night
Or gabapentin/pregabalin
What is cervical spondylosis?
Degeneration of the annulus fibrosus of cervical intervertebral discs
Causes narrowing of the spinal canal
Most people have no nerve impairment, but it can cause compression of the cord and nerve roots
What are the features of cervical spondylosis?
Neck stiffness
Limited painful neck movement
Neck flexion may produce tingling down the spine
What is radiculopathy and what are the features?
Root compression
Pain / electrical sensations in arms or fingers at level of compression
Dull reflexes
LMN weakness and eventual muscle wasting of affected root
UMN signs below level of affected root
What is the management of cervical spondylosis?
Firm neck collar restricts to relieve pain
Surgical root decompression: laminectomy or laminoplasty
What are the diagnostic criteria for neurofibromatosis type 1?
6 or more cafe au lait patches 2 or more neurofibromas Freckling in axillary or inguinal regions Optic glioma Lisch nodules - hamartomas on iris 1st degree relative with NF1
How does neurofibromatosis type 2 differ from type 1?
Bilateral vestibular schwannomas (acoustic neuroma)
What is motor neuron disease?
Selective loss of neurones in the motor cortex, cranial nerve nuclei and anterior horn cells
Upper and lower motor neurones affected
What are the important negatives in motor neurone disease?
No sensory loss or sphincter disturbance
What are the 4 clinical patterns of MND?
- ALS (50%)
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
What are the common presenting features of MND?
>40y Stumbling spastic gait Foot drop Proximal myopathy Weak grip Weak shoulder abduction Aspiration pneumonia
What are the UMN signs seen in MND?
Spasticity
Brisk reflexes
Up going plantars
What are the LMN signs seen in MND?
Wasting
Fasciculation
What are the bulbar signs in MND?
Speech or swallowing affected
What makes MND diagnosis definite?
Lower and upper motor neuron signs in 3 regions
What is the treatment for MND?
Antiglutamatergic drugs Drooling: amitriptyline Dysphagia: blend food, NG or PEG Analgesia NIV at home for resp failure
What part of the nervous system does MS affect?
White matter of brain and spinal cord and optic nerves
What is the pathophysiology of MS?
Inflammatory demyelination of CNS tracts
Causes reduction in conduction velocity, as well as distortion and loss of information
Changes not uniformly distributed, foci of inflammation and demyelination = plaques
What is gliosis?
Plaques evolve with time and ultimately result in CNS equivalent of scarring
What is the epidemiology of MS?
Females 3:1
Usually presents age 20 - 40
UK prevalence 1 in 1000
What are the most common presentations of MS?
Visual disturbance
Limb weakness
Sensory disturbance
What is optic neuritis?
Inflammatory demyelination of one optic nerve
Pain around one eye, especially on movement
Rapid decline in central vision (central scotoma)
RAPD
Pink, swollen optic disc
What are the sensory features of MS?
Dysaesthesia
Pins and needles
Decreased vibration sense
Trigeminal neuralgia
What are the motor features of MS?
Spastic weakness
Myelitis
What is the progression of MS?
Symptoms worsen over days/weeks, reach plateau then resolve partially or completely
Relapses
Remissions are incomplete with time, so disability accumulates
What are poor prognostic signs in MS?
Older male
Motor signs at onset
Many relapses early on
Axonal loss
How is MS diagnosed?
Clinically
MRI is sensitive but not specific for plaque detection
Lesions disseminated in time and space, with no other causes - further evidence needed after 1st episode
CSF: oligoclonal bands of IgG on electrophoresis suggest CNS inflammation
What is the treatment for MS?
Steroids: methylprednisolone shortens acute relapse
Interferons
Monoclonal antibodies e.g. Alemtuzumab / Natalizumab
What drugs are used to treat spasticity?
Baclofen
Diazepam
Dantrolene
What is cervical spondylosis?
Degeneration of the annulus fibrosus of cervical intervertebral discs
Causes narrowing of the spinal canal
Most people have no nerve impairment, but it can cause compression of the cord and nerve roots
What are the features of cervical spondylosis?
Neck stiffness
Limited painful neck movement
Neck flexion may produce tingling down the spine
What is radiculopathy and what are the features?
Root compression
Pain / electrical sensations in arms or fingers at level of compression
Dull reflexes
LMN weakness and eventual muscle wasting of affected root
UMN signs below level of affected root
What is the management of cervical spondylosis?
Firm neck collar restricts to relieve pain
Surgical root decompression: laminectomy or laminoplasty
What are the diagnostic criteria for neurofibromatosis type 1?
6 or more cafe au lait patches 2 or more neurofibromas Freckling in axillary or inguinal regions Optic glioma Lisch nodules - hamartomas on iris 1st degree relative with NF1
How does neurofibromatosis type 2 differ from type 1?
Bilateral vestibular schwannomas (acoustic neuroma)
What is motor neuron disease?
Selective loss of neurones in the motor cortex, cranial nerve nuclei and anterior horn cells
Upper and lower motor neurones affected
What are the important negatives in motor neurone disease?
No sensory loss or sphincter disturbance
What are the 4 clinical patterns of MND?
- ALS (50%)
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
What are the common presenting features of MND?
>40y Stumbling spastic gait Foot drop Proximal myopathy Weak grip Weak shoulder abduction Aspiration pneumonia
What are the UMN signs seen in MND?
Spasticity
Brisk reflexes
Up going plantars
What are the LMN signs seen in MND?
Wasting
Fasciculation
What are the bulbar signs in MND?
Speech or swallowing affected
What makes MND diagnosis definite?
Lower and upper motor neuron signs in 3 regions
What is the treatment for MND?
Antiglutamatergic drugs Drooling: amitriptyline Dysphagia: blend food, NG or PEG Analgesia NIV at home for resp failure
What part of the nervous system does MS affect?
White matter of brain and spinal cord and optic nerves
What is the pathophysiology of MS?
Inflammatory demyelination of CNS tracts
Causes reduction in conduction velocity, as well as distortion and loss of information
Changes not uniformly distributed, foci of inflammation and demyelination = plaques
What is gliosis?
Plaques evolve with time and ultimately result in CNS equivalent of scarring
What is the epidemiology of MS?
Females 3:1
Usually presents age 20 - 40
UK prevalence 1 in 1000
What are the most common presentations of MS?
Visual disturbance
Limb weakness
Sensory disturbance
What is optic neuritis?
Inflammatory demyelination of one optic nerve
Pain around one eye, especially on movement
Rapid decline in central vision (central scotoma)
RAPD
Pink, swollen optic disc
What are the sensory features of MS?
Dysaesthesia
Pins and needles
Decreased vibration sense
Trigeminal neuralgia
What are the motor features of MS?
Spastic weakness
Myelitis
What is the progression of MS?
Symptoms worsen over days/weeks, reach plateau then resolve partially or completely
Relapses
Remissions are incomplete with time, so disability accumulates
What are poor prognostic signs in MS?
Older male
Motor signs at onset
Many relapses early on
Axonal loss
How is MS diagnosed?
Clinically
MRI is sensitive but not specific for plaque detection
Lesions disseminated in time and space, with no other causes - further evidence needed after 1st episode
CSF: oligoclonal bands of IgG on electrophoresis suggest CNS inflammation
What is the treatment for MS?
Steroids: methylprednisolone shortens acute relapse
Interferons
Monoclonal antibodies e.g. Alemtuzumab / Natalizumab
What drugs are used to treat spasticity?
Baclofen
Diazepam
Dantrolene
