Neurology

Question 1

Which modality leaves the spinal cord at the following region:

Dorsal root ganglion

Anterior horn

Answer 1

• Dorsal root ganglion - Sensory cell bodies
• Anterior horn – Motor cell bodies

Question 2

What is an upper motor neurone (UMN)?

Answer 2

All CNS structures

• Brain
• Spinal cord

Question 3

What are the UMN signs?

Answer 3

• Hypertonia - Rigidity + Spasticity
• Clonus
• Brisk reflexes
• Weakness
• Babinski reflex - Up going plantars

Question 4

What is a Lower motor neurone (LMN)?

Answer 4

All PNS structures

• Nerve root (Anterior horn cell)
• Peripheral nerves
• Neuromuscular junction

Question 5

What are the LMN signs?

Answer 5

• Weakness + Wasting in motor unit
• Reduced tone (Flaccid)
• Fasciculation
• Reduced/Absent reflexes

Question 6

What do the following control?

Corticospinal tract

Spinothalamic tract

Dorsal columns (Fasciculus cuneatus & Gracilis)

Answer 6

• Corticospinal tract – Movement
• Spinothalamic tract - Pain & Temperature
• Dorsal columns (Fasciculus cuneatus & Gracilis) – Vibration & Proprioception

Question 7

Where do the corticospinal tracts decussate?

Answer 7

Medulla

Question 8

What is Brown Sequard syndrome?

Answer 8

Hemisection of the spinal cord (Other side remains in tact)

• UMN signs
• Loss of sensation ipsilateral and at level of lesion
• Loss of vibration & proprioception ipsilateral to lesion
• Loss of temperature & Pinprick contralateral to the lesion

Question 9

Where do the dorsal columns decussate?

Where does the spinothalamic tract decussate?

Answer 9

• Dorsal columns – Medulla
• Spinothalamic tract – Spinal cord, at level of entry of neuron

Question 10

What is Dysarthria?

What is Dysphasia?

Answer 10

Dysarthria – Slurred speech due to motor deficit (Broca’s area)

Dysphasia – Disturbance of language leading to either speech problems or understanding (Wernickes)

• Expressive - Difficulty finding words
• Receptive - Difficulty understanding words spoken

Question 11

What are the causes of dysarthria?

Answer 11

• Lesions of tongue/lips/mouth
• Bulbar palsy - Bilateral LMN lesion
• Cerebellar dysfunction
• Myasthenia gravis
• Parkinsonism

Question 12

Damage to which part of the brain would lead you to expect dysphasia/dysarthria?

Answer 12

• Left side
• Most common in R + L handed people, in L handed people it may be the R side.

Question 13

What are the following, where are they found?

Broca’s area

Wernicke’s area

Answer 13

Broca’s area – Inferior frontal region

• Word production & language expression

Wernicke’s area – Superior posterior temporal lobe

• Comprehension & Spoken language

Question 14

What are the functions of the frontal lobe?

Answer 14

• Personality
• Emotional response
• Social behaviour
• Smell
• Posterior part contains the pre-central gyrus (Motor strip) – Controls voluntary movement

Question 15

What are the functions of the parietal lobe?

Answer 15

• Calculation
• Language
• Planned movement
• Spatial awareness
• Anterior part contains the post-central gyrus – Sensory strip (Conscious sensation)

Question 16

What are the functions of the occipital lobe?

Answer 16

• Vision
• Vision interpretation

Question 17

What are the functions of the temporal lobe?

Answer 17

• Auditory perception
• Speech and language
• Non-verbal memory

Question 18

You are asked to do a cranial nerve examination on a patient, what are the steps?

Answer 18

[Introduction]

• Wash hands & Introduce self
• Ask Patient name & DOB & Age
• Today I am going to be doing a neurological examination, to identify if there is anything going on with your brain or your nerves.
  
  • Is that what you were expecting?
• For the examination I will need access to your upper limbs and lower limbs, so it is best if you undress to your underwear and put a robe on.
  
  • The examiner will act as a chaperone for both of us
• To start the examination for the cranial nerves which supply your face I will need you sat facing me on a chair
  
  • Do you have any pain at the moment?

[General Inspection]

• Assess the surrounding area
  
  • Walking aids
• Assess the patient - SWIFT
  
  • Do they look well at rest?
  • Body habitus
  • Scars
  • Wasting
  • Involuntary movements
  • Fasciculations
  • Tremor

[CN 1 - Olfactory nerve]

• Sense of smell (CN1)
• Not useful clinically unless pt reports loss of sense of smell in Hx

[CN 2, 3, 4, 6 - Optic/Occulomotor/Trochlear/Abducens]

AFRO - Acuity/Fields/Reflexes/Opthalmoscope

Visual acuity:

• If patient reports changes to their vision in Hx
• Measure visual acuity with a Snellen chart

Visual fields:

• Can you please cover your left eye with your left hand, and I will cover my right eye
• Can you keep looking at my face, and tell me when you notice my fingers/this red pin coming into your view
• Move the red pin inside slowly from all 4 corners of the visual field towards the centre of vision
• Ensure red pin is equal distant between myself and the patient
• Compare my visual field with the patient to determine if abnormal
• Repeat for R eye - You cover your R eye with your R hand, and I will cover my L eye

Pupillary reflexes:

• I’m just going to have a look at your pupils in your eyes briefly, so if you could keep them open and stare over my shoulder at something on the wall
• Inspect both pupils closely for discrepancy in size or shape
• Ok now I will be shining a bright light into your eye, it should be painful but let me know if it is too much
• Bring in the light from the side - avoids accommodation reflex
  
  • Check both eyes with the light
• 1. Direct reflex - Shine torch in patients eye SAME pupil should constrict
• 2. Consensual reflex - Shine torch into pts eye and the OTHER pupil should constrict
• 3. Swinging light test - Move torch from eye to eye, back and forth If relevant Afferent Pupillary Defect (RAPD) -> Paradoxical pupil dilation
  
  • Defect in the direct response - When the light reaches a pupil there should be a normal direct and consensual response, when the light is shone in the AFFECTED pupil it remains dilated after light being shone in the healthy pupil

Eye movements:

• Now I am going to be checking your eye movements
• What I would like you to do, is to follow my finger with your eyes, but keep your head still
  
  • Hold finger approx 50cm away b/w me and patient
  • Let me know if you get pain or double vision whilst looking at my finger
• Move finger in H pattern, assess for:
  
  • Nystagmus
  • Opthalmoplegia
• If patient notes double vision, identify if it is:
  
  • Maximum/Vertical/Horizontal/Tilted
• Does closing one eye make the double vision better?

Saccades:

• Hold palm to one side of patient and fist to the other side of the patient
• Can you keep your head still and look at my fist then look at my palm and alternative between them
  
  • Rapid eye movements - b/w the two -> Saccades

Fundoscopy:

• Darken the room
• Take the fundoscope and tell patient: I’m going to be having a look into the back of your eye.
  
  • I’ll have to get very close to your face and will put my hand on your shoulder to steady myself.
  • Is that ok?
• Examine the eye:
  
  • Look for red reflex from afar then move in closer
  • Disc
  • Cup
  • Colour
  • Contour
  • Papilloedema (Sign of Raised ICP)
  • Pale disc (Sign of previous optic neuropathy)

​[CN 5 - Trigeminal nerve]

• Assess if a patient reports numbness in the face - check sensation in the face
  
  • Ophthalmic branch - V1
  • Maxillary branch - V2
  • Mandibular branch - V3
• Test muscles of mastication - Inspect for muscle wasting
  
  • Temporalis
  • Masseter
  • Medial & Lateral Pterygoids
• Jaw power
  
  • Place hand under jaw as resistance, ask patient to open jaw -> Note deviation
• Corneal reflex test - Offer. lightly touching a wisp of cotton wool to the patients cornea, causing them to blink
  
  • Assess Ophthalmic branch -> Sensation, Facial nerve -> Blink the eye itself
• Jaw jerk reflex

[CN 7 - Facial nerve]

• Now Im going to be having a look at the nerve that supplies your face
• Observe for signs of
  
  • Weakness
• I’m going to ask you to do a range of facial expressions to me, so if you could just copy me that would be great
  
  • Can you raise your eyebrows
    
    • Does the forehead wrinkle on both sides?
  • Can you close your eyes really tight?
    
    • Assess for asymmetry
  • Keep your eyes closed and I want you to stop me from opening them - resistance
    
    • Note signs of weakness
  • Can you keep your lips tightly shut for me, and don’t let me open them
    
    • Note signs of weakness
  • Can you show me your teeth?
    
    • Note any asymmetry
  • Can you puff out your cheeks?
  • Stop me from pushing them in
• Anterior 2/3 taste on tongue
  
  • Have you noticed any change to your taste in the front of your tongue?
  • Offer to test taste
• Any change to your hearing?

[CN 8 - Vestibulococchlear]

• If patient reports problems with hearing or balance (CN8)
• Check hearing
  
  • Briefly rustling your finger and thumb in front of each ear to see if they can perceive
• Rinne - Not usually performed in neurology
• Webers test - Not usually performed in neurology

[CN 9 & 10 - Glossopharyngeal + Vagus nerve]

• Pharynx
• Next i’m going to look inside your mouth - Ill just get a tongue depressor
  
  • Get tongue depressor & pen torch
  • Inspect the palate and the uvula Does the uvula deviate to one side?
• Can you please say aaahhhh
  
  • Palate and uvula should move UP
• Testing:
  
  • CN 9 -> Sensation
  • CN 10 -> Motor function
• Extra tests
  
  • If the patient reported problems with swallowing:
    
    • Ask patient to swallow and observe this -> CN 9 & 10 (Rarely tested)
    • Ask patient to cough
• If you suspect problems with the patients speech - formally assess speech
  
  • Ask them to repeat “yellow lorry” or “Baby hippopotamus”
• Can you blow out your cheeks and keep the air in there
  
  • Listen for air escaping through nose, as for cheeks to puff out the palate must elevate to block the nasopharynx -> Air escaping shows weak palate
• Offer Gag reflex

[CN 11 - Accessory nerve]

• If you suspect weakness in shoulders and neck
• Ask pt to shrug shoulders - then do it against resistance
• Ask patient to turn their head against resistance

[CN 12 - Hypoglossal Nerve]

• Next i’m just going to have a look at your tongue
• Assess the tongue for:
  
  • Fasciculations
  • Muscle wasting
• Can you please stick your tongue out, assess for:
  
  • Deviation
  • Can you wiggle your tongue side to side
• Check for speed of tongue movement

Question 19

You are asked to do a Upper limb neurological examination on a patient, what are the steps?

Answer 19

[Introduction]

• Wash hands & Introduce self
• Ask Patient name & DOB & Age
• Today I am going to be doing a neurological examination, to identify if there is anything going on with your brain or your nerves. Is that what you were expecting?
• For the examination I will need access to your upper limbs and lower limbs, so it is best if you undress to your underwear and put a robe on. The examiner will act as a chaperone for both of us
• Do you have any pain at the moment?

[General Inspection]

• Assess the surrounding area
  
  • Walking aids
• Assess the patient - SWIFT
  
  • Do they look well at rest?
  • Body habitus
  • Scars
  • Wasting
  • Involuntary movements
  • Fasciculations
  • Tremor
  • Posture

Limb exam

The next stage of the exam is to have a look at your limbs, and how they are functioning

• Are you right or left handed?
• I will need you to sit on the bed for this part of the exam
  
  • Upper limbs - sitting
  • Lower limbs - lie down

[Inspection]

Assess for muscle wasting in the

• Upper limb
• Fasciculation – Assess for irregular ripples or twitches which are associated with muscle wasting

Look at:

• Tap over muscles as this may elicit them
• Tremor Assess the UL/LL for any signs of a tremor
  
  • Describe the tremor - Is it fast/slow or Fine/course
  • Examine UL at rest -> on posture -> and during coordination testing
• Myoclonic jerks Assess for sudden shock like contractions
  
  • Are the focal/diffuse/singular/repetitive
• Deformity & Posture
  
  • Typical UMN problems can cause adduction of the shoulder, flexed elbow, flexed wrist
• Pronator drift
  
  • Can you stick your arms straight out please with your palms UP
  • Can you close your eyes
• Assess for
  
  • Normal - Arms remain stationary
  • Arms drifting down and pronation
    
    • Suggest lesion in the pyramidal tracts -> UMN lesion
  • Ask patient to stick arms straight out please with palms DOWN
    
    • Checking pseudoathetosis & Tremor

[Tone]

• Hold pts hand as if shaking it and support at the elbow with the other hand
  
  • Can you please relax your arm completely - keep talking to the patient
  • Rotate forearm - Pronation/Supination
  • Flexion/Extension of the wrist
  • Flexion/Extension of the elbow
• Rigidity -> Stiffness that remains throughout the entire movement, regardless of speed of movement.
  
  • NON velocity dependent (Lead pipe/Cog wheel)
  • Evidence of extrapyramidal lesion -> Parkinson’s disease
• Spasticity -> Stiffness that is exacerbated when there is an attempt to move the limb quickly.
  
  • IS velocity dependent (Clasp knife)
  • Evidence of pyramidal lesion

[Power]

• Assess UL power Shoulder abduction - Don’t let me push your shoulders down
  
  • C5
• Elbow flexion - Dont let me pull your elbows away from your body
  
  • C5/6
• Elbow extension - Don’t let me push your elbows into your body
  
  • C7
• Wrist flexion - Dont let me pull your wrist away from your body
  
  • C7
• Wrist extension - Dont let me push your wrist away from your body
  
  • C6
• Finger extension - Dont let me push your fingers away from your body
  
  • C7
• Finger flexion - Dont let me pull your fingers away from your body
  
  • C8
• Finger abduction - Dont let me push your fingers back together
  
  • T1
• Thumb abduction - Dont let me push your thumb back towards your index finger
  
  • T1

MRC Muscle strength reporting:

• Grade 0 -> No power
• Grade 1 -> Twitching but no movement
• Grade 2 -> Movement, but cannot overcome gravity
• Grade 3 -> Can overcome gravity
• Grade 4 -> Movement against gravity and resistance
• Grade 5 -> Normal muscle strength

[Reflexes]

• Now I’m going to check your reflexes, can you please relax for me
• Assess for muscle contraction - not limb movement Try 2 times - then try reinforcement
  
  • UL -> Clench your teeth
• Grade the reflex
  
  • Reduced/absent -> LMN lesion
  • Normal
  • Increased/brisk -> UMN lesion
• Biceps jerk C5
  
  • Fingers on biceps tendon
• Supinator jerk C5/6
  
  • Fingers on tendon (Brachioradialis)
• Triceps jerk C7
  
  • Hold patients arm out with elbow flexed and hand facing down
• Strike tendon directly

[Coordination]

• Finger-nose test
  
  • Hold finger at arms length in front of the patients nose
  • Ask patient to repeatedly touch b/w their nose and the tip of my finger
  • Cerebellar lesions -> finger to under or over shoot (Past pointing)
  • Cerebellar lesions -> Intention tremor - fine tremor just before touching your finger
• Ask patient to close eyes Repeat the test to touch b/w nose and tip of finger in same position
  
  • If unable to tell position of finger w/o vision -> Sensory ataxia
• Dysdocokinesia
  
  • Can you put your hands palm up on your other palm, then alternate
  • Repeat this
  • Look for:
    
    • Fatigue
    • Lack of coordination

[Sensation]

• Use neurotip - pin (Superficial pain)
• I’m going to test the sensation if your UL now with this neurotip/cotton wool
• I’m just going to show you what it feels like now on your forehead.
  
  • I will then ask you to close your eyes, and tell me when you get the same sensation again somewhere on your body
• Test in dermatomal pattern
• Compare one arm to the other
  
  • C4 -> Above shoulder tip
  • C5 -> Regimental badge area
  • C6 -> tip of thumb
  • C7 -> Tip of middle finger
  • C8 -> Tip of little finger
  • T1 -> Medial forearm
  • T2 -> Medial upper arm
  • T3 -> Axilla
• if there is a deficit - try to map out the region affected
• Sensory inattention
  
  • Ask patient to close their eyes
  • Touch their arms/legs in turn - which side has been touched?
    
    • Touch both sides at the same time - ask whether the left/right/both sides were touched?

[Proprioception]

• If Hx suggests impaired proprioception, hold IP joint of thumb and ask patient to close eyes
• Ask them to identify whether you are holding their toe UP or DOWN

[Vibration]

• Next I’m going to be testing your ability to sense vibrations in your UL
  
  • Use 128Hz tuning fork
• I’m just going to show you what the vibrations are like on your forehead.
  
  • I will then ask you to close your eyes and tell me when you get the same sensation again somewhere on your body
• I want you to tell me when you feel the buzz, not the cold metal
• Place tuning fork on bony prominences:
  
  • Finger - can you feel this?
  • Close your eyes now and tell me when the vibration stops
• If deficit -> try more proximal body prominences

[Complete examination]

• Thank patient & Wash hands
• “This is patient x who is a x year old Male/Female with the following findings”
• I would take a full Hx + examine any other relevant systems
• I would consider the differentials
• I would order relevant investigations
  
  • Observations
  • Bloods
  • Imaging
• I would initiate management of the most likely differential

Question 20

You are asked to do a lower limb neurological examination on a patient, what are the steps?

Answer 20

[Introduction]

• Wash hands & Introduce self
• Ask Patient name & DOB & Age
• Today I am going to be doing a neurological examination, to identify if there is anything going on with your brain or your nerves. Is that what you were expecting?
• For the examination I will need access to your upper limbs and lower limbs, so it is best if you undress to your underwear and put a robe on. The examiner will act as a chaperone for both of us
• Do you have any pain at the moment?

[General Inspection]

• Assess the surrounding area
  
  • Walking aids
• Assess the patient - SWIFT
  
  • Do they look well at rest?
  • Body habitus
  • Scars
  • Wasting
  • Involuntary movements
  • Fasciculations
  • Tremor

[Other tests]

Gait

• So now I would like to assess your walking – Can you walk from point A to B for me. Assess for:
  
  • Stride length
  • Symmetry
  • Ease of turning

If normal -> What I would like you to do next is to walk heel-toe like your are walking on a tight rope please

• Emphasise any gait ataxia

Rombergs

• Can you stand straight in front of me, hands by your sides, feet together and close your eyes
• Ready to catch them if they become unsteady
• Normal -> Maintain position w/o losing balance
• Eyes open -> visual feedback aiding balance
• If swaying/lurching/unable to stand -> Cerebellar ataxia
• Eyes closed -> Reliance on proprioception for balance If impaired - pt cannot tell position in space w/o visual feedback, so will lose balance. -> Sensory ataxia (Dorsal column damage)

[Inspection]

• Wasting
  
  • Assess for muscle wasting
  • Fasciculation – Assess for irregular ripples or twitches which are associated with muscle wasting
• Look at:
• Tap over muscles as this may elicit them

Tremor Assess the LL for any signs of a tremor

• Describe the tremor - Is it fast/slow or Fine/course
• Examine UL at rest -> on posture -> and during coordination testing

Myoclonic jerks Assess for sudden shock like contractions

• Are the focal/diffuse/singular/repetitive

[Tone]

• Test LL tone
  
  • Can you please relax your legs completely - keep talking to the patient
  • Roll leg from side to side
    
    • Look for normal movement of the foot
• Lift knee slowly
  
  • Then lift knee quickly of bed
  • Heel should stay on bed - If foot lifts off and catches -> increased tone, UMN lesion
• If this happens with rapid movements -> Spasticity (Velocity dependent increase in tone)
• Rigidity -> increased tone = through all speeds of passive movement

Clonus

• Rapidly dorsiflex the foot and hold
• Look for clonus -> Sign of spasticity
• >4 beats -> Pathological, UMN pathology

[Power]

• Hip flexion - Dont let me push your leg into the bed
  
  • L1/2
• Knee extension - Dont let me push your shin into the bed
  
  • L3/L4
• Ankle dorsiflexion - Don’t let me push your foot away from your body
  
  • L4
• Great toe dorsiflexion - Don’t let me push your toe away from your body
  
  • L5
• Ankle plantar flexion - Don’t let me push your foot towards the floor
  
  • S1
• Knee flexion - Dont let me lift your shin off the bed
  
  • L5/S1
• Hip extension - Dont let me lift your leg off the bed
  
  • L5/S1

MRC Muscle strength reporting:

• Grade 0 -> No power
• Grade 1 -> Twitching but no movement
• Grade 2 -> Movement, but cannot overcome gravity
• Grade 3 -> Can overcome gravity
• Grade 4 -> Movement against gravity and resistance
• Grade 5 -> Normal muscle strength

[Reflexes]

• Now I’m going to check your reflexes, can you please relax for me
  
  • Use the tendon hammer - strike tendon not the muscle
• Assess for muscle contraction - not limb movement Try 2 times - then try reinforcement
• LL -> Grasp your hands and pull

Grade the reflex

• Reduced/absent -> LMN lesion
• Normal
• Increased/brisk -> UMN lesion

LL reflexes

• Knee L3/4
  
  • Knee relaxed and slightly flexed + external rotation
  • Watch quadriceps muscle for contraction
• Ankle S1/2
  
  • Hip abducted/externally rotated - flex knee
• Dorsiflex the ankle - to stretch tendon
  
  • Strike the achilles tendon -> Warch for calf muscle contraction/ankle plantar flexion
• Plantar reflex
  
  • Run orange stick up the lateral border of the foot from the heel to the little toe, then run it medially towards the big toe
  • Look for INITIAL reaction of the big toe
  • Big toe DOWN -> Normal reflex
  • Big toe UP -> abnormal Babinksi reflex (Upgoing) -> UMN lesion

[Coordination]

• Heel-shin test
  
  • Ask patient to bring heel to shin and lift off at knee and repeat
  • Perform this with R & L leg

[Sensation]

• I’m going to test the sensation if your UL/LL now with this neurotip (Superficial pain)
• I’m just going to show you what it feels like now on your chest. I will then ask you to close your eyes, and tell me when you get the same sensation again somewhere on your body
• Compare one arm/leg to the other
  
  • L2 -> Antero-medial mid thigh
  • L3 -> Medial thigh just above the knee
  • L4 -> Medial malleolus
  • L5 - Dorsal 1st web space
  • S1 -> Lateral little toe + Lateral heel
• if there is a deficit - try to map out the region affected
• Sensory inattention
  
  • Ask patient to close their eyes
  • Touch their arms/legs in turn - which side has been touched?
  • Touch both sides at the same time - ask whether the left/right/both sides were touched?

[Proprioception]

• If Hx suggests impaired proprioception, hold IP joint of big toe/thumb and ask patient to close eyes
• Ask them to identify whether you are holding their toe UP or DOWN

[Vibration]

• Next I’m going to be testing your ability to sense vibrations in your UL/LL
• Use 128Hz tuning fork
• I’m just going to show you what the vibrations are like on your chest. I will then ask you to close your eyes and tell me when you get the same sensation again somewhere on your body
• I want you to tell me when you feel the buzz, not the cold metal
• Place tuning fork on bony prominences:
  
  • Great toe - bony prominence
  • Close your eyes now and tell me when the vibration stops
• If deficit -> try more proximal body prominences
• Impaired vibration sense -> Dorsal column pathology (Vibration/Proprioception)

[Complete examination]

• Thank patient & Wash hands
• “This is patient x who is a x year old Male/Female with the following findings”
• I would take a full Hx + examine any other relevant systems
• I would consider the differentials
• I would order relevant investigations
  
  • Observations
  • Bloods
  • Imaging
• I would initiate management of the most likely differential

Question 21

You are asked to assess the cerebellar function, as part of a neurological examination. What are the steps?

Answer 21

[Introduction]

• Wash hands & Introduce self
• Ask Patient name & DOB & Age
• Today I am going to be doing a neurological examination, to identify if there is anything going on with your brain or your nerves. Is that what you were expecting?
• For the examination I will need access to your upper limbs and lower limbs, so it is best if you undress to your underwear and put a robe on. The examiner will act as a chaperone for both of us
• To start the examination for the cranial nerves which supply your face I will need you sat facing me on a chair, but for the rest of the examination its best if you are sat on a couch.
• Do you have any pain at the moment?

[Focused questions]

• What happened when you first presented with his condition?
• How is it affecting you?
• When is your tremor worse?

[General Inspection]

• Assess the surrounding area
  
  • Walking aids
• Assess the patient - SWIFT
  
  • Do they look well at rest?
  • Body habitus
  • Scars
  • Wasting
  • Involuntary movements
  • Fasciculations
  • Tremor

[Gait]

• Can you walk from A - B, I will be nearby in case you feel unsteady
• Can you walk heel to toe if you can?
  
  • Assessing for ataxic gait
• Can you sit in a chair with your arms folded
  
  • Assessing for truncal ataxia

[Posture]

• Can you stand with your feet together for me?
  
  • Can you close your eyes (Rombergs test)
    
    • Assess stability, for sensory ataxia

[Face]

• H test for extraoccular muscle function
  
  • pause at lateral gaze
• Assessing for:
  
  • Nystagmus
  • Saccades
  • Can you look from one target to another
  • Hypometric saccades

[Speech]

• Say “Baby hippopotamus” and “British constitution”
• Assessing for slurring/Staccato speech

[Upper limbs]

• Wasting – Assess for muscle wasting in the
  
  • Upper limb
  • Fasciculation – Assess for irregular ripples or twitches which are associated with muscle wasting
• Tap over muscles as this may elicit them

Tremor Assess the UL/LL for any signs of a tremor

• Describe the tremor - Is it fast/slow or Fine/course
• Examine UL at rest -> on posture -> and during coordination testing

Myoclonic jerks Assess for sudden shock like contractions

• Are the focal/diffuse/singular/repetitive

Deformity & Posture

• Typical UMN problems can cause adduction of the shoulder, flexed elbow, flexed wrist

Pronator drift

• Can you stick your arms straight out please with your palms UP
• Can you close your eyes

Assess for

• Normal - Arms remain stationary
• Arms drifting down and pronation
• Suggest lesion in the pyramidal tracts -> UMN lesion

Ask patient to stick arms straight out please with palms DOWN

• Checking pseudoathetosis & Tremor

[Tone]

Test UL tone

• Hold pts hand as if shaking it and support at the elbow with the other hand
• Can you please relax your arm completely - keep talking to the patient
  
  • Rotate forearm - Pronation/Supination
  • Flexion/Extension of the wrist
  • Flexion/Extension of the elbow
• Rigidity -> Stiffness that remains throughout the entire movement, regardless of speed of movement.
  
  • NON velocity dependent (Lead pipe/Cog wheel)
  • Evidence of extrapyramidal lesion -> Parkinson’s disease
• Spasticity -> Stiffness that is exacerbated when there is an attempt to move the limb quickly.
  
  • IS velocity dependent (Clasp knife)
  • Evidence of pyramidal lesion

[Coordination]

• Finger-nose test
  
  • Hold finger at arms length in front of the patients nose
  • Ask patient to repeatedly touch b/w their nose and the tip of my finger
    
    • Cerebellar lesions -> finger to under or over shoot (Past pointing)
    • Cerebellar lesions -> Intention tremor - fine tremor just before touching your finger
• Ask patient to close eyes Repeat the test to touch b/w nose and tip of finger in same position
  
  • If unable to tell position of finger w/o vision -> Sensory ataxia

[Lower limbs - Tone]

• Can you please relax your legs completely - keep talking to the patient
  
  • Roll leg from side to side
  • Look for normal movement of the foot
• Lift knee slowly
  
  • Then lift knee quickly of bed
  • Heel should stay on bed - If foot lifts off and catches -> increased tone, UMN lesion
• If this happens with rapid movements -> Spasticity (Velocity dependent increase in tone)
• Rigidity -> increased tone = through all speeds of passive movement

Clonus

• Rapidly dorsiflex the foot and hold
  
  • Look for clonus -> Sign of spasticity
  • >4 beats -> Pathological, UMN pathology

[Coordination]

• Heel-shin test
  
  • Ask patient to bring heel to shin and lift off at knee and repeat
• Perform this with R & L leg

[Complete examination]

• Thank patient & Wash hands
• “This is patient x who is a x year old Male/Female with the following findings”
• I would take a full Hx + examine any other relevant systems
  
  • CN exam with fundoscopy
  • Full neurological exam
• I would consider the differentials
• I would order relevant investigations
  
  • Observations
  • Bloods
  • Imaging
• I would initiate management of the most likely differential

Question 22

What are the signs of cerebellar dysfunction?

Answer 22

DANISH

• D - Dysdiadokinesia + Dysmetria (Past pointing)
• A - Ataxia
• N - Nystagmus
• I - Intention tremor
• S - Slurred/Staccato speech
• H - Hypotonia

Question 23

What are the causes of Cerebellar disease?

Answer 23

MAVIS

• M - MS
• A - Alcohol
• V - Vascular
• I - Inherited
• S - SoL

Question 24

You are asked to interpret a CT brain scan, what are the steps to do this?

Answer 24

• Isodense – Same as brain
• Hyperdense – Brighter than brain
• Hypodense – Darker than brain

[Identification]

• Name
• Date of birth/Age

[Scan details]

• Date
• Time
• Orientation
• Contrast
• Windowing

[Describe lesion]

• Site – Where is the lesion?
• Shape – Diffuse or well-circumscribed? Smooth or irregular?
• Homogeneity – Homogenous or inhomogeneous?
• Enhancement – Enhancing or non-enhancing?
• Associated features – oedema, dural origin, calcification?

[Specific features]

• Midline – Midline shift
• Ventricles – Blood & mass effect
• Cisterns – Blood and pus
• Parenchyma – Ischaemia & bleeding
• Sulci – Blood & prominence
• Sinuses – Blood and pus
• Bones – Fracture
• Soft tissues - Haematoma

Question 25

What is MS?

Answer 25

• Cell mediated autoimmune condition - with repeated episodes of inflammation of the central nervous system
• Leads to loss of the myelin sheath - both grey + white matter
• Requires separate lesions in both time & space

Question 26

What causes MS?

Answer 26

• Inflammation leads to – Multiple areas of scar tissue (Sclerosis) along neurons
• Slowing ± blocking transmission of signals to and from brain – Spinal cord
• Genetic & Environmental causes
• Linked to EBV – Molecular mimicry

Question 27

What are the different patterns of MS?

Answer 27

Relapsing remitting – Most common

• Relapse of symptoms - new lesion formation.
• Followed by remission for some time (Re-myelination)

Primary progressive

• From beginning
• Symptoms gradually develop and worsen over time, no remissions.

Secondary progressive

• Follows on from relapsing remitting MS
• Gradually more/worse symptoms with fewer remissions

Question 28

Who is most likely to develop MS?

Answer 28

• Caucasians
• Women
• 20-40yrs peak incidence
• FHx of MS

Question 29

What are the classical symptoms of MS?

Answer 29

• UMN Signs
• Limb weakness
• Optic neuritis - Acute/painful reduction or loss of vision in one eye
• Diplopia
• Facial weakness
• Deafness
• Vestibular dysfunction
• Sensory deficits
• Autonomic system dysfunction - Bladder & Bowel symptoms

Question 30

If you suspect a patient may have MS, what investigations should be done?

Answer 30

• Clinical diagnosis + MRI to confirm
• MRI brain & Spinal cord (Focal demyelination in brain or spinal cord)
  
  • At >1 site + >1 month b/w attacks
• LP
  
  • Increased protein
  • Increased IgG concentration - Oligoclonal bands
• Delayed evoked potentials (Nerve stimulation studies)

Question 31

If you have confirmed a diagnosis of MS, how is it managed?

Answer 31

• Patient education
• Inform DVLA

Relapse:

• IV Methylprednisolone (High dose steroids)
• Gastric protection - Ranitidine/Omeprazole
• Bone protection - Bisphosphonates

Disease modifying drugs:

• Monoclonal antibodies
• Dimethyl Fumarate
• Plasma exchange

Question 32

A patient presents with the following Hx, what are the differentials to be considered?

A 28-year-old white woman who has smoked 1 pack per day for the last 10 years presents with subacute onset of cloudy vision in 1 eye, with pain on movement of that eye. She also notes difficulty with colour discrimination, particularly of reds. She was treated for a sinus infection 2 weeks ago and on further history recalls that she had a 3-week history of unilateral hemibody paraesthesias during examination week in university 6 years ago. She occasionally has some tingling on that side if she is overly tired, stressed, or hot.

Answer 32

MS******

Myelopathy due to cervical spondylosis

Stroke

GBS

B12/Folate deficiency

Peripheral neuropathy

Question 33

A patient presents with the following Hx, what are the differentials to be considered?

A 31-year-old woman with strong family history of autoimmune disease is 6 months postnatal and develops ascending numbness and weakness in both feet, slightly asymmetrically, over a period of 2 weeks. She gradually develops difficulty walking to the point where she presents to an emergency department and is also found to have a urinary tract infection.

Answer 33

MS******

Myelopathy due to cervical spondylosis

Stroke

GBS

B12/Folate deficiency

Peripheral neuropathy

Question 34

If a patient presents with weakness, what could be the cause of the problem:

CNS

PNS

Answer 34

CNS

• Motor cortex
• Brainstem
• Spinal cord (Myelopathy)
• Anterior horn cell

PNS:

• Nerve root (Radiculopathy)
• Nerve plexus (Plexopathy)
• Peripheral nerve (Peripheral neuropathy)
• Neuromuscular junction
• Muscle (Myopathy)

Question 35

A patient presents with symptoms in the following time frame, what are the likely differentials?

Sudden onset

Acute onset (Minutes to hrs)

Subacute (Days to weeks)

Chronic (Months to years)

Answer 35

Sudden onset

• Traumatic
• Vascular (Infarction/ICH/SAH)

Acute onset (Minutes to hrs)

• Vascular (Central Venous Sinus Thrombosis)
• Infections (Meningitis/Encephalitis)
• Drug reactions

Subacute (Days to weeks)

• Infections (TB Meningitis)
• Cerebral abscess
• Autoimmune (MS)

Chronic (Months to years)

• Genetic (DMD/Neurodegenerative Parkinsons)
• Metabolic (Wilsons)
• Neoplastic (Glioblastoma)

Question 36

Where do the CN’s leave the brain?

Answer 36

• CN 1 + 2 – Leave from the cerebrum
• CN 3 + 4 – Leave from the midbrain
• CN 5-8 – Leave from the Pons
• CN 9 - 12 – Leave from the Medulla (Bulbar)

Question 37

What is meant by the terms:

Pyramidal

Extra-pyramidal

Answer 37

Pyramidal – Motor tracts that originate in the cerebral cortex, carrying motor fibres to the spinal cord and brain stem. They are responsible for the voluntary control of the musculature of the body and face.

• Corticospinal tracts – supplies the musculature of the body.
• Corticobulbar tracts – supplies the musculature of the head and neck

Extra-pyramidal – Motor tracts originate in the brain stem, carrying motor fibres to the spinal cord. They are responsible for the involuntary and automatic control of all musculature, such as muscle tone, balance, posture and locomotion

• The vestibulospinal and reticulospinal tracts do not decussate, providing ipsilateral innervation
• The rubrospinal and tectospinal tracts do decussate, and therefore provide contralateral innervation

Question 38

You are asked to examine the UL dermatomes as part of a neurological exam, which nerve supplies which part of the upper limb?

Answer 38

• C4 – Shoulder tip
• C5 – Regimental badge
• C6 – Tip of thumb
• C7 – Tip of middle finger
• C8 – Tip of little finger
• T1 – Medial forearm
• T2 – Medial upper arm
• T3 – Axilla

Question 39

You are asked to examine the UL myotomes as part of a neurological exam, which nerve supplies which part of the upper limb?

Answer 39

• C4 – Shoulder Adduction
• C5 – Shoulder abduction
• C5/6 – Elbow flexion
• C7 – Elbow extension
• C6 – Wrist extension
• C7 – Finger extension
• C8 – Finger flexion + Thumb extension
• T1 – Thumb abduction + Finger Abduction

Question 40

You are asked to examine the UL reflexes, which nerves supply the reflex arc?

Answer 40

• Suppinator jerk:
  
  • Brachioradialis muscle – C5/6
• Biceps – C5
• Triceps – C7

Question 41

You are asked to examine the LL dermatomes as part of a neurological exam, which nerve supplies which part of the upper limb?

Answer 41

• L2 – Antero medial mid thigh
• L3 – Medial thigh just above knee
• L4 – Medial malleolus
• L5 – Dorsal 1st web space
• S1 – Lateral little toe + Lateral heel

Question 42

You are asked to examine the LL myotomes as part of a neurological exam, which nerve supplies which part of the upper limb?

Answer 42

• L2 – Hip flexion
• L3+4 – Knee extension
• L4 – Ankle dorsiflexion
• L5 – Great toe dorsiflexion
• S1 – Ankle plantarflexion
• S1 – Knee flexion
• S4 – Bladder + Rectum motor supply

Question 43

You are asked to examine the LL reflexes, which nerves supply the reflex arc?

Answer 43

• Knee reflex – L3/L4
• Ankle – S1/S2

Question 44

What is the significance of the following signs on visual field testing?

Homonymous Hemaniopia

Bitemporal hemaniopia

Monocular blindness

Answer 44

• Homonymous Hemaniopia – Occipital cortex/Optic radiation lesion
• Bitemporal hemaniopia – Optic chiasm lesion
• Monocular blindness – Optic nerve

Question 45

What is Guillain Barre syndrome?

Answer 45

• Acute inflammatory neuropathy
• Causes demyelination & Axonal degeneration (Peripheral neuropathy)
• Usually preceeded by infection

Question 46

What are the characteristics of GBS?

Answer 46

Acute Ascending + Progressive neuropathy

• Weakness
• Hyporeflexia
• Parasthesiae
• Polyradiculoneuropathy

Question 47

What usually causes GBS?

Answer 47

Usually Hx of preceeding infection (URTI or GI infection) within 6 weeks of neurological symptoms

Commonly caused by infection from:

• Campylobacter Jejuni
• EBV
• CMV

Question 48

Who classically gets GBS?

Answer 48

• Males > Females
• 15-35yrs or 50-75yrs
• Past Hx of GI or Upper respiratory tract infection
• Recent vaccinations
• Malignancy – Lymphoma
• Pregnancy/Post partum

Question 49

How does GBS classically present (Symptoms/Signs)?

Answer 49

• Hx of viral illness
• Weakness:
  
  • Ascending pattern (Starts in LL)
  • Progressive + Symmetrical
  • Weakness stops progressing
• May have facial weakness
• Neuropathic pain
• LMN signs
• Bulbar dysfunction
• Autonomic symptoms

Question 50

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 20-year-old woman with no significant past medical history presents with lower back pain and bilateral foot and hand tingling. Her symptoms rapidly progress over 4 days to include lower extremity weakness to the point that she is unable to mobilise her lower extremities. She reports coryzal symptoms 2 weeks ago. On examination, she has 0/5 power in her lower extremity with areflexia, but despite the paraesthesias she does not have sensory deficits. Her aminotransferases are elevated, and lumbar puncture reveals mildly elevated protein with no cells and normal glucose. She weighs 70 kg and her admission vital capacity is 1300 mL, maximum inspiratory pressure is -30 cmH₂O, and maximum expiratory pressure is 35 cmH₂O.

Answer 50

• Guillain Barre syndrome
• Stroke/Brainstem compression
• Cord compression
• Transverse myelitis
• Vasculitis
• Encephalitis
• Myasthenia gravis

Question 51

A patient presents with the following Hx, suggestive of GBS. What investigations should be ordered?

A 20-year-old woman with no significant past medical history presents with lower back pain and bilateral foot and hand tingling. Her symptoms rapidly progress over 4 days to include lower extremity weakness to the point that she is unable to mobilise her lower extremities. She reports coryzal symptoms 2 weeks ago. On examination, she has 0/5 power in her lower extremity with areflexia, but despite the paraesthesias she does not have sensory deficits. Her aminotransferases are elevated, and lumbar puncture reveals mildly elevated protein with no cells and normal glucose.

Answer 51

Clinical diagnosis:

• Hx
• Neurological examination
  
  • Progressive weakness in UL + LL
  • LMN signs

Bloods

• FBC – Normal.
• U&E – Normal

LP:

• Increased protein
• Normal cell count

Nerve conduction studies:

• Abnormal – Slowing of nerve conduction velocities (Repeat after 2wks)

LFTs: Increased AST + ALT

Question 52

How is a patient with confirmed GBS managed?

Answer 52

• Plasma exchange
• IV Ig
• DVT Prophylaxis:
  
  • S/C Heparin/Enoxaparin + TED Stockings
• Intubation ± ventilation if respiratory distress
• Rehabilitation
• Pain relief for neuropathic pain

Question 53

What are the complications of GBS?

Answer 53

• Persistent paralysis
• Hypotension/HTN
• Respiratory failure
• Aspiration pneumonia

Question 54

What is transverse myelitis?

Answer 54

Focal inflammation of the spinal cord

Leads to:

• Motor weakness
• Sensory impairment
• Autonomic dysfunction

Question 55

What can cause transverse myelitis?

Answer 55

• MS
• Infection – HSV/HZV/EBV/TB/Syphillis
• Post vaccination
• SLE/Sjogrens syndrome
• Saracoidosis
• Paraneoplastic syndrome

Question 56

What are the different types of transverse myelitis?

Answer 56

Acute partial transverse myelitis

• Usually post infection due to acute spread of infection
• Affects 1-2 vertebral segments
• Asymmetric spinal cord lesions
• Increased risk of developing MS

Longitudinally extensive TM:

• Asymmetric or Symmetric spinal cord lesions
• Span >3 contiguous vertebrae
• Typically bilateral symptoms
• Increased risk of Neuromyelitis Optica

Question 57

Who typically gets transverse myelitis?

Answer 57

• Peak age 10-20yrs and 30-40yrs
• Typically heralds or accompanies MS
• Female

People with:

• Recent infection/vaccination

Question 58

What are the classical symptoms/signs of Transverse myelitis?

Answer 58

• Motor weakness:
  
  • Progressive weakness affecting LL or All extremities
  • Asymmetrical or Symmetrical
  • Pyramidal pattern
• Parasthesia or sensory loss
• Urinary & bowel dysfunction
• Back pain
• UMN signs

Question 59

A patient presents with the following symptoms, what is the most likely diagnosis? What are the differentials?

A 26-year-old woman with no significant medical history presents with a 5-day history of left lower extremity paraesthesias that subsequently ascended the left trunk to the level of the umbilicus. She also reports new-onset urinary frequency and that her right leg drags during ambulation. On examination, she has a partial Brown-Sequard’s syndrome with mild pyramidal weakness and impairment of vibration and proprioceptive sensation involving the right lower extremity, and reduced appreciation for pain throughout the left lower extremity and trunk below T11. The right plantar response is extensor.

Answer 59

• Transverse myelitis
• MS
• GBS
• Anterior/Posterior spinal artery occlusion
• Myasthenia gravis
• B12/Folate deficiency

Question 60

A patient presents with the following symptoms, what is the most likely diagnosis? What are the differentials?

A 45-year-old woman with a history of Hashimoto’s thyroiditis presents with a 2-day history of progressive bilateral upper and lower extremity numbness, paraparesis, bladder incontinence, mid-thoracic back pain, and L’hermitte’s sign (limb paresthesias upon neck flexion). On examination, she has 4/5 power in the distal upper extremities, 1/5 power in her lower extremities with symmetric hyper-reflexia, clonus, and Babinski’s signs. There is a bilateral sensory level at T4 and loss of vibratory and proprioceptive sensation in the feet, and she is unable to walk.

Answer 60

• Transverse myelitis
• MS
• GBS
• Anterior/Posterior spinal artery occlusion
• Myasthenia gravis
• B12/Folate deficiency

Question 61

A patient with the following symptoms suggestive of acute transverse myelitis presents. What investigations should be carried out?

A 45-year-old woman with a history of Hashimoto’s thyroiditis presents with a 2-day history of progressive bilateral upper and lower extremity numbness, paraparesis, bladder incontinence, mid-thoracic back pain, and L’hermitte’s sign (limb paresthesias upon neck flexion). On examination, she has 4/5 power in the distal upper extremities, 1/5 power in her lower extremities with symmetric hyper-reflexia, clonus, and Babinski’s signs. There is a bilateral sensory level at T4 and loss of vibratory and proprioceptive sensation in the feet, and she is unable to walk.

Answer 61

MRI Spinal cord:

• Exclude compressive SC lesion
• Intrinsic cord lesion - Diagnostic

MRI brain

• No lesions, Assess for MS or Neuromyelitis Optica

LP:

• Increased WCC
• Increased protein
• Abnormal Oligoclonal bands
• If normal – Recheck in 2 weeks to confirm

Question 62

How is Transverse myelitis managed?

Answer 62

• Corticosteroids – Methylprednisolone for 3-5 days
• Neuropathic pain management
• Catheter if acute urinary retention
• DVT Prophylaxis

Question 63

You are asked to interpret the lumbar puncture results of a patient presenting with a number of symptoms. What do the results suggest?

• Clear and colourless appearance.
• Protein level - 0.2-0.4 g/L (neonate <1.7 g/L).
• Glucose level - 60-80% of plasma glucose.
• WCC <5 per mm3
• Opening pressure 10-20 cm H2O

Answer 63

Normal

Question 64

You are asked to interpret the lumbar puncture results of a patient presenting with a number of symptoms. What do the results suggest?

• Cloudy and turbid CSF (if severe).
• Raised protein >1.5 g/L.
• Glucose level is <50% of the plasma level.
• Cell count is high (>1,000 per mm3) and mostly neutrophils.
• Opening pressure is high.

Answer 64

Bacterial meningitis

Question 65

You are asked to interpret the lumbar puncture results of a patient presenting with a number of symptoms. What do the results suggest?

• Clear CSF
• Protein is raised or normal
• Glucose level is usually within normal limits
• Cell count is high and mostly lymphocytes
• Opening pressure may or may not be raised

Answer 65

Viral Meningitis

Question 66

You are asked to interpret the lumbar puncture results of a patient presenting with a number of symptoms. What do the results suggest?

• May be blood stained/Xanthochromia (Yellow)
• Protein is raised or at the high end of normal
• Glucose level is usually low
• High number of RBCs, Normal WCC count
• Opening pressure is usually high if excessive RBCs are present

Answer 66

Subarachnoid Haemorrhage

Question 67

Which location is a LP done?

Answer 67

• L4/L5 disc space
• Line from ASIS - marks the L5 vertebrae level

Question 68

What are the contraindications for a LP?

Answer 68

• Patient is too unstable
• Signs of Raised ICP:
  
  • Change to GCS
  • HTN
  • Bradycardia
  • Focal neurological signs
  • Papilloedema
• Suspected SoL
• Bleeding disorder
• Local cellulitis at site of LP

Question 69

What are the signs of raised ICP?

Answer 69

• Change to GCS
• HTN
• Bradycardia
• Focal neurological signs
• Headache
• Diplopia
• Papilloedema
• Kernigs sign – Patient supine, thigh flexed to 90deg, try to straighten leg – Resistance
• Brudzinki’s sign – Flexion of neck leads to involuntary flexion of knees + hips

Question 70

What are the signs of Meningococcal meningitis/Sepsis?

Answer 70

• Non-blanching rash
• Unwell looking patient
• Purpura
• Capillary refill >2s
• Neck stiffness

Signs of raised ICP:

• Change to GCS
• HTN
• Bradycardia
• Focal neurological signs
• Papilloedema

Question 71

What is Meningitis?

What causes viral/bacterial meningitis?

Answer 71

Inflammation of the meninges due to pathogen

Bacteria –

• Strep Pneumoniae (Pneumococcal)
• Nesseria meningitis (Meningococcal)
• Haemophilus Influenza (Hib)

Virus

• HSV1/2
• EBV
• Coxsackie virus

Question 72

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 1-month-old girl presents to her general practitioner with a high fever, feeding difficulties, and irritability for the past 24 hours. Examination reveals altered mental status and a bulging fontanelle.

Answer 72

• Bacterial Meningitis
• Viral meningitis
• Encephalitis
• Drug induced meningitis

Question 73

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

An 18-year-old male student presents with severe headache and fever that he has had for 3 days. Examination reveals fever, photophobia, and neck stiffness.

Answer 73

• Bacterial meningitis
• Viral meningitis
• Encephalitis
• Drug induced meningitis

Question 74

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 19-year-old man presents with a 2-day history of headache and associated nausea. He says that bright light hurts his eyes. He has no significant past medical history, is not currently taking any medicine, and reports no drug allergies. He works as a librarian and has not travelled overseas for the past year. He lives with his girlfriend whom he has been seeing for 2 years. They have a pet hamster.

Answer 74

• Viral meningitis
• Bacterial meningitis
• Encephalitis
• Drug induced meningitis

Question 75

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

Parents bring their 2-year-old child who has been ill for 1 day with irritability, vomiting, and fever. The child has a widespread maculopapular rash.

Answer 75

• Viral meningitis
• Bacterial meningitis
• Encephalitis
• Drug induced meningitis

Question 76

What are the classical Hx of meningitis?

Answer 76

• Fever
• Severe headache
• Neck stiffness
• Photophobia
• Altered mental state
• Vomiting
• Increased ICP signs
• Petechial/Purpuric non-blanching rash – Meningococcal meningitis

Question 77

A patient presents with the following Hx, suggestive of bacterial meningitis. What investigations should be ordered?

An 18-year-old male student presents with severe headache and fever that he has had for 3 days. Examination reveals fever, photophobia, and neck stiffness.

Answer 77

• LP
  
  • Increased WCC - Neutrophil predominant
  • Decreased glucose
  • Increased protein
• CSF Culture + Gram stain – Positive
• Blood culture
• FBC – Raised WCC, Thrombocytopenia
• CRP – Increased
• U&E – Normal. May have metabolic abnormalities
• ABG – May have metabolic acidosis

Question 78

How is a patient with bacterial meningitis managed?

Answer 78

• ABx therapy – Ceftriaxone
• O2
• Prevent hypoglycaemia
• If rash:
  
  • SEPSIS 6
  • Take bloods + Blood culture + LP
  • Take urine output
  • Take Lactate
  • Give O2 if required
  • Give Fluids
  • Give ABx

Question 79

When a patient presents with symptoms and signs of meningitis, what are the key aspects of the Hx that need to be identified?

Answer 79

• Contact with others
• Travel Hx
• Sexual Hx
• Vaccination Hx

Question 80

A patient presents with symptoms suggestive of viral meningitis. What investigations should be done?

A 19-year-old man presents with a 2-day history of headache and associated nausea. He says that bright light hurts his eyes. He has no significant past medical history, is not currently taking any medicine, and reports no drug allergies. He works as a librarian and has not travelled overseas for the past year. He lives with his girlfriend whom he has been seeing for 2 years. They have a pet hamster.

Answer 80

• LP:
  
  • Raised WCC count - Lymphocyte predominant
  • Gram stain – Negative
  • Normal or Raised protein
  • Normal glucose
  • PCR – Positive
• Blood culture
• FBC – Raised WCC, Thrombocytopenia
• CRP – Increased
• U&E – Normal. May have metabolic abnormalities
• ABG – May have metabolic acidosis

Question 81

How is viral meningitis managed?

Answer 81

• Supportive care:
  
  • Hydration
  • Antipyretics for fever (Paracetamol)
  • Anti-emetics
  • Analgesia
• If immunosuppressed or HSV or VZV positive – Aciclovir for 10 days.

Question 82

What is Encephalitis?

Answer 82

Inflammation of the brain parenchyma causing neurological dysfunction

Characteristic features:

• Altered consciousness level
• Seizures
• Personality changes
• Cranial nerve palsy
• Motor/Sensory deficit

Question 83

What typically causes Encephalitis?

Answer 83

Viruses are most common

• HSV1/HSV2
• VZV
• EBV
• Coxsackievirus

Can also be caused by bacteria – Neisseria/TB/Syphillis

Question 84

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 56-year-old man presents to the emergency department with headache, fever, blurred vision, and somnolence followed shortly by unresponsiveness to verbal commands. For the last 2 weeks he had been feeling ill and had decreased appetite and myalgias. Three days prior to presentation he experienced intermittent confusion, severe headache, and fever. Examination was limited by a generalised tonic-clonic seizure, for which he received lorazepam.

Answer 84

• Encephalitis
• Bacterial meningitis
• Seizure
• Status epilepticus

Question 85

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 19-year-old man presents to the emergency department with a witnessed generalised tonic-clonic seizure episode. One month previously he had an upper respiratory tract infection. Over the last 2 weeks he developed headaches, blurred vision, generalised weakness, and progressive difficulty in walking. Examination revealed pain on eye movement as well as limb and gait ataxia.

Answer 85

• Encephalitis
• Bacterial meningitis
• Seizure
• Status epilepticus

Question 86

What are the classical symptoms & signs seen with encephalitis?

Answer 86

• Altered mental state
• Focal neurological signs – Hemipareisis/UMN signs
• Seizures
• Cognitive behavioural changes
• If concurrent meningitis – Headahce/Photophobia/Neck stiffness

Question 87

A patient presents with the following Hx, suggestive of Encephalitis. What investigations should be carried out?

A 19-year-old man presents to the emergency department with a witnessed generalised tonic-clonic seizure episode. One month previously he had an upper respiratory tract infection. Over the last 2 weeks he developed headaches, blurred vision, generalised weakness, and progressive difficulty in walking. Examination revealed pain on eye movement as well as limb and gait ataxia.

Answer 87

• FBC – Increased WCC
• U&Es – Hyponatramia
• LFTs – Normal or elevated
• Blood cultures
• Throat/Sputum cultures
• CT brain – May be normal
  
  • MRI brain – Diagnostic
• LP
  
  • Increased WCC
  • Normal/Increased protein
  • Normal/Low glucose
  • CSF Gram stain ± PCR

Question 88

How is Encephalitis managed?

Answer 88

Viral cause:

• Aciclovir
• May require shunting/Surgical decompression (Raised ICP)
• Supportive care:
  
  • Endotracheal intubation if required

Non-viral cause

• ABx – Benzylpenicillin

Question 89

What is a Febrile seizure/convulsion?

Answer 89

• Seizure activity in childhood associated with fever with NO evidence of intracranial infection
• Commonly caused by viral infections - RSV
• Patients are slightly more likely to develop epilepsy in adulthood

Question 90

What is meant by the following:

Simple febrile seizure

Complex febrile seizure

Febrile Status Epilepticus

Answer 90

Simple febrile seizure – Any of

• Generalised
• Lasts <15mins
• not repeated in 24hr period

Complex febrile seizure – Any of:

• Focal
• Lasts >15 mins
• Repeated within 24hr period

Febrile Status Epilepticus

• Seizure activity >5mins
• Short series of seizures w/o regaining consciousness in post-ictal state

Question 91

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A previously healthy and developmentally normal 18-month-old boy presents to the emergency department by ambulance after his parents witnessed a seizure. The parents report the boy had a febrile illness with mild upper respiratory symptoms and they treated him with paracetamol and ibuprofen at home. The child then began to have frequent jerking movements of all limbs. The rectal temperature was 39.5°C (103.1°F). The parents called the emergency services, and a paracetamol suppository was administered during transport to the emergency department. The jerking stopped after approximately 5 minutes. Afterwards, the child was sleepy but responsive to verbal stimulation. Examination revealed a diffuse erythematous maculopapular rash and a normal mental and neurological status.

Answer 91

• Febrile convulsions
• Bacterial meningitis
• Viral meningitis
• Viral encephalitis
• Epilepsy

Question 92

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 10-month-old girl is brought to the emergency department with a history of recurrent right arm and leg jerking followed by prolonged sleepiness. The parents report a 2-day history of fever with chest congestion and irritability. The child is admitted to hospital for neurological evaluation.

Answer 92

• Febrile convulsions
• Bacterial meningitis
• Viral meningitis
• Viral encephalitis
• Epilepsy

Question 93

What is the classic presentation (Symptoms & Signs) of Febrile seizures?

Answer 93

• Young age
• Fever
• Followed by Loss of consciousness
  
  • Generalised clonic + tonic movements
  • Short duration w/quick post ictal recovery
• FHx of febrile seizures
• Signs of infection

Question 94

A patient presents with the following symptoms suggestive of febrile convulsions. What investigations should be carried out?

A 10-month-old girl is brought to the emergency department with a history of recurrent right arm and leg jerking followed by prolonged sleepiness. The parents report a 2-day history of fever with chest congestion and irritability. The child is admitted to hospital for neurological evaluation.

Answer 94

• Clinical diagnosis
• LP:
  
  • Normal WCC
  • Normal protein
  • Normal Glucose
  • Gram stain/PCR – Negative
• Blood culture – Negative

Question 95

How are febrile seizures managed?

Answer 95

1st simple/complex febrile seizure:

• Antipyretic – Ibuprofen ± Paracetamol
• Place child in recovery position (Prevent aspiration + tongue biting)

>1 febrile seizure:

• Consider referral to neurology

Febrile Status epilepticus:

• Consult with Paeds Neurologist/ICU
• Epilepsy Status epilepticus protocol

Question 96

How is power graded, when assessing it as part of the neurological exam?

Answer 96

MRC grading

• 5 is normal
• 4 is weakened against resistance
• 3 is able to move against gravity
• 2 is moves with support
• 1 is muscle twitches
• Nothing/absent

Question 97

What is a Seizure?

Answer 97

• Abnormal & excessive discharge of electricity in the brain - causing the brain to shut down
• Leads to disturbance in:
  
  • Consciousness
  • Behaviour
  • Emotion
  • Motor/Sensory function
• Can be provoked or unprovoked

Question 98

What is Epilepsy?

Answer 98

At least 2 unprovoked seizures >24hrs apart

Question 99

What are the different types of seizure?

Answer 99

Provoked v Unprovoked

• Provoked – Caused by reversible stimulus
  
  • Seizure within 7 days of acute condition
• Unprovoked – Not caused by reversible stimulus
  
  • Epilepsy

Generalised tonic-clonic:

• Tonic - Stiffness, Clonic - Jerking

Focal:

• From 1 hemisphere
  
  • Simple focal seizure – Retained awareness
  • Complex focal seizure – With impaired awareness

Absence seizure

Question 100

What are the classical symptoms/signs of seizures?

Answer 100

• FHx of epilepsy
• Tonic-clonic movements
• Post ictal phase – Unconsciousness/Disorientation following the episode
  
  • Disturbance in consciousness
• Incontinence
• Tongue biting
• Photosensitivity
• Absence seizure – Brief period of impaired consciousness, with motor arrest/stereotyped movement or automatisms
  
  • Precipitated by hyperventilation or lights
• Myoclonic – Brief arrythmic muscular jerking movements
• Clonic – Rhythmic muscular jerking movements
• Atonic – Drop attacks, patient falls to ground
• Normal neurological examination

Question 101

A patient with the following Hx suggestive of seizure, What investigations would you do?

Answer 101

• EEG – Diagnostic if seizure is present
• Blood glucose – Normal.
• FBC – Normal. WCC may be increased if Provoked seizure due to infection
• U&E – Normal. Electrolyte imbalance can cause provoked seizure
• ECG – Normal. Exclude cardiac cause of seizure like episode
• LFTs – Normal
• Drug screen – Normal. May have evidence of cocaine/amphetamine/heroin use
• MRI Brain – If secondary cause is suspected

Question 102

How are seizures managed?

How is Epilepsy managed?

Answer 102

1st Acute Seizure:

• Referral to Neurology within 2 weeks
• First aid – Place in recovery position
• If >2/3mins seizure activity: begin treatment
  
  • Rectal Diazepam/Buccal Midazolam
  • IV Phenytoin/Lorazepam

Epilepsy prophylaxis (ONLY if unprovoked):

• Sodium Valproate/Lamotrigine/Levetiracetam
• Absence seizures – Ethosuximide
• Focal seizure – Carbamazepine

Inform DVLA:

• If 1st seizure – 6mo no driving (Seizure free)
• If epilepsy – 12mo no driving (Seizure free)

Question 103

What is Status Epilepticus?

How is it managed?

Answer 103

Seizure activity for >5mins OR Repetitive seizures with no recovery of consciousness

Management:

• Airway + High flow O2
• Check Blood glucose
• Place in recovery position + Ensure head is protected

If vascular access:

• IM Midazolam/Buccal/Intranasal or Rectal Diazepam
• If no response after 10 mins – Repeat Midazolam
• IV Diazepam/Lorazepam
• If no response after 5 mins – Repeat above (Max 2 doses)
• If no response after 5 mins - Phenytoin/Phenobarbital
• If no response after 20mins – ICU for rapid induction with Thiopentone/Propofol

If NO vascular access:

• IM Midazolam/Buccal/Intranasal or Rectal Diazepam
• If no response after 10 mins – Repeat Midazolam
• If no response after 10 mins – Paraldehyde PR
• If no response after 10 mins – ICU for rapid induction with Thiopentone/Propofol

Question 104

You are asked to explain a prescription of Lamotrigine to a patient, how would you do this?

Answer 104

Layout:

• Check what the patient knows
• Brief history
• Do they know what the drug is?
• Indication & Action of the drug
• Side effects
• How to take it
• Monitoring requirements

[Indication & Action of the drug]

Indication:

• Seizure prophylaxis in epilepsy
• Focal seizures
• Generalised tonic-clonic seizures
• Absence seizures
• Bipolar depression - not mania/hypomania

Action:

• Binds to voltage sensitive Na+ channels in neurones
  
  • Interferes with Na+ influx into the neutron
• Impedes repetitive neurone firing - characteristic of seizure activity
  
  • Inhibits postsynaptic glutamate receptor

[Side effects]

• Headache
• Drowsiness
• Irritability
• Blurred vision
• Dizziness
• GI symptoms
• Skin rash - within weeks of starting
• Usually mild, but may also be sign of severe hypersensitivity reaction
• Antiepileptic hypersensitivity syndrome - usually within 2 months of starting treatment
• Severe skin reaction - SJS/TEN
• Fever
• Lymphadenopathy with systemic involvement (Haematological/hepatic/renal)

Complications & CI

• Avoid in patients with Hx of hypersensitivity to other epileptic drugs
• Avoid in patients with hepatic impairment
• May be a reasonable choice in women of child-bearing age

[How to take it]

• Start with low dose, then increase over 2 weeks to reach maintenance dose
• Tablet taken orally - swallowed whole with water
  
  • Aim of treatment is to reduce seizure frequency not cure epilepsy
• May take up to 2 weeks to reach levels in blood which would be therapeutic
• If patient misses a dose they should take it as soon as they remember, unless the next dose is imminent then wait and take that one
  
  • Treatment should not be stopped abruptly - risk of rebound seizures

[Monitoring requirements]

• Signpost patients to signs of severe hypersensitivity - seek urgent medical attention
• Haematological toxicity
• Skin rashes
• Bruising
• Bleeding
• High temperature
• Mouth ulcers
• Liver toxicity
• Reduced appetite
• Abdominal pain

Monitor treatment efficacy

• Compare seizure frequency before and after starting treatment

Signpost patient:

• Must not drive unless seizure free for 12 months
• 6 months after changing or stopping treatment
• Must inform DVLA

Question 105

You are asked to explain a prescription of Valproate to a patient, how would you do this?

Answer 105

Layout:

• Check what the patient knows
• Brief history
• Do they know what the drug is?
• Indication & Action of the drug
• Side effects
• How to take it
• Monitoring requirements

[Indication & Action]

Indications

• Seizure prophylaxis in epilepsy
• Status epilepticus not responding to benzodiazepines
• Bipolar disorder - acute treatment of manic episodes and prophylaxis against recurrence

Action

• Weak inhibitor of neuronal Na channels - stabilising resting membrane potentials & reducing neuronal excitability
• Increases brain content of GABA - inhibitory NT (Involved in regulation of neuronal excitability

[How to take it]

• Take in 1-3 divided doses
• Comes as tablet/granules/oral solutions/IV infusion
• Reduce GI upset by taking it with food

[Important SE]

• GI Upset
• Nausea/Gastric irritation/diarrhoea
• Neurological & psychiatric effects
• Tremor/ataxia/behavioural disturbances
• Thrombocytopenia + platelet dysfunction
• Transient increase in liver enzymes

Severe rare SE:

• Severe liver injury - chemical hepatitis
• Pancreatitis
• Bone marrow failure
• Anti-epileptic hypersensitivity syndrome

Complications/Contraindications

• Avoid in:Women of childbearing age - conception & 1st trimester
• Increased risk of foetal abnormalities - NT defects/Craniofacial/Cardiac & limb abnormalities
  
  • Also risk of developmental disorders - ADHD/ASD
  • Note: If pregnancy can not be avoided - Folic acid 5mg daily
• Hepatic impairment
• Severe renal impairment

Special info

• Signpost to seek urgent medical advice, for unexpected symptoms
• Lethargy/Loss of appetite/vomiting/abdominal pain - Signs of liver poisoning
• Bruising/high temperature/mouth ulcers - Indicates blood abnormalities

[Monitoring requirements]

• Monitor by comparing seizure frequency before + after starting treatment
• Monitor safety
• Measure LFTs + PT time
• Pregnancy test
• Before starting
  
  • During first 6 mo of treatment
• Measure Plasma valproate levels
  
  • Check for adherence/toxicity

Question 106

You are asked to explain Carbamazepine to a patient, how would you go about doing this?

Answer 106

Layout:

• Check what the patient knows
• Brief history
• Do they know what the drug is?
• Indication & Action of the drug
• Side effects
• How to take it
• Monitoring requirements

[Indication & Action of the drug]

Indication:

• Seizure prophylaxis in epilepsy
• Generalised Tonic clonic seizures
• Focal seizures
• Trigeminal neuralgia - control pain and reduce frequency and severity of attacks

Action:

• Inhibits neuronal Na+ channels
• Stabilises resting membrane potential
• Reduced neuronal excitability
• Inhibits spread of seizure activity in epilepsy & controls neuralgic pain by blocking synaptic transmission in the trigeminal nucleus

[Side effects]

• Dose dependent
• GI Upset - N & V
• Neurological effects
• Dizziness
• Ataxia
• Hypersensitivity
• Mild Maculopapular skin rash
• Antiepileptic hypersensitivity syndrome - usually within 2 months of starting treatment
• Severe skin reaction - SJS/TEN
• Fever
• Lymphadenopathy with systemic involvement (Haematological/hepatic/renal)
• ADH like effect
• Hyponatraemia
• Oedema

Complications & CI

• Causes neural tube defects/cardiac/urinary tract abnormalities - in pregnancy
• Give 5mg Folic acid if pregnancy is not avoidable
• Prescribe with caution in
• Hepatic/renal/cardiac disease - risk of toxicity

CI:

• Prior anti epileptic hypersensitivity syndrome

[How to take it]

• Oral or rectal administration
  
  • Immediate/Modified release
• Start at low dose - to reduce SE
• Tolerance develops so dose is increased gradually
• Should NOT be stopped suddenly, requires gradual withdrawal
• Aim of treatment is to reduce seizure frequency not cure epilepsy
• May take up to 2 weeks to reach levels in blood which would be therapeutic

[Monitoring requirements]

• Signpost patients to signs of severe hypersensitivity - seek urgent medical attention
• Haematological toxicity
• Skin rashes
• Bruising
• Bleeding
• High temperature
• Mouth ulcers
• Liver toxicity
• Reduced appetite
• Abdominal pain
• Monitor treatment efficacy

Compare seizure frequency before and after starting treatment

Signpost patient:

• Must not drive unless seizure free for 12 months
• 6 months after changing or stopping treatment
• Must inform DVLA

Question 107

You are asked to explain Levetiracetam to a patient, how would you go about doing this?

Answer 107

Layout:

• Check what the patient knows
• Brief history
• Do they know what the drug is?
• Indication & Action of the drug
• Side effects
• How to take it
• Monitoring requirements

[Indication & Action of the drug]

Indication:

• Seizure prophylaxis in epilepsy
• Focal seizures - if Carbamazepine/Lamotrigine are not suitable/tolerated
• Myoclonic seizures
• Generalised tonic clonic seizures
• Established convulsive Status Epilepticus - not responsive to benzodiazepines

Action:

• Levetiracetam interferes with synaptic vesicle function - modulates neuronal excitability
  
  • reduces risk of seizures
• Mechanism is poorly understood

[Side effects]

• Generally well tolerated
• Mild adverse effects or none at all
• Drowsiness
• Weakness
• Dizziness
• Headache
• Mood disturbance
• Suicidal ideation (Rare)
• Serious hypersensitivity reaction (Rare) Antiepileptic hypersensitivity syndrome - usually within 2 months of starting treatment
• Severe skin reaction - SJS/TEN
• Fever
• Lymphadenopathy with systemic involvement (Haematological/hepatic/renal)

Complications & CI

• Dose reduction may be required in renal impairment
• Difficult to exclude effect on congenital defects, but not known to cause any
• Caution in pregnancy

[How to take it]

• Start on low dose initially then titrate to increased dose gradually based on clinical response
• IV dose if Status epilepticus
• Tablets should be swallowed whole
• Aim of treatment is to reduce seizure frequency not cure epilepsy
• May take up to 2 weeks to reach levels in blood which would be therapeutic
• If patient misses a dose they should take it as soon as they remember, unless the next dose is imminent then wait and take that one
• Treatment should not be stopped abruptly - risk of rebound seizures

[Monitoring requirements]

• If patient develops mood changes - depression -> Seek attention
• Monitor treatment efficacy
• Compare seizure frequency before and after starting treatment

Signpost patient:

• Must not drive unless seizure free for 12 months
• 6 months after changing or stopping treatment
• Must inform DVLA

Question 108

What is Cerebral Palsy?

Answer 108

Non-progressive disease of the brain caused by brain injury before or shortly after birth - causing motor and coordination dysfunction

Types:

• Spastic – Most common, Muscles are stiff and tight. (Motor cortex damage)
• Dyskinetic – Involuntary movements (Basal Ganglia damage)
• Ataxic – Shaky movements, balance and positional sense affected (Cerebellum damage)

Question 109

Who classically gets Cerebral Palsy?

Answer 109

• Premature babies
• Babies where there was maternal illness (TORCH)
• Birth asphyxia
• Neonatal sepsis/Hyperbilirubinaemia/Periventricular haemorrhage
• FHx of CP

Question 110

What are the classical symptoms/signs of Cerebral Palsy?

Answer 110

• Delayed motor milestones
• Delay in speech development
• Muscle weakness

Spastic:

• Spastic tone
• Hyper-reflexia
• Clonus
• Dyskinetic:
• Athetosis/Chorea/Dystonia
• Involuntary recurring sterotyped movements

Ataxic:

• Loss of muscular coordination
• Gait + Truncal ataxia
• DANISH signs

Question 111

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

An 18-month-old child with a history of prematurity (28 weeks’ gestation, 1200 grams) presents with failure to meet developmental milestones. The child sat independently at 1 year, has few words vocally, does not pull to stand, and exhibits increased deep tendon reflexes in the lower extremities and sustained clonus at both ankles. There is good upper-extremity function. A magnetic resonance imaging scan of the brain reveals periventricular leukomalacia.

Answer 111

• Cerebral palsy - Spastic type
• Spinal muscular atrophy
• Muscular dystrophy
• Familial dystonia

Question 112

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 2-year-old boy, born after a normal pregnancy and delivery, presents with an asymmetric gait. Examination reveals mild spasticity of the left upper and lower extremity, hyperactive left knee and ankle deep tendon reflexes, and decreased dorsiflexion of the left ankle compared with the right. When walking, the patient walks on his left toes, and his left arm is held mildly flexed at the elbow with the palm facing the floor (pronated forearm). The left calf is smaller in girth than the right.

Answer 112

• Cerebral palsy
• Spinal muscular atrophy
• Muscular dystrophy
• Familial dystonia

Question 113

A patient presents with the following Hx, suggestive of Cerebral Palsy, what investigations would you order?

An 18-month-old child with a history of prematurity (28 weeks’ gestation, 1200 grams) presents with failure to meet developmental milestones. The child sat independently at 1 year, has few words vocally, does not pull to stand, and exhibits increased deep tendon reflexes in the lower extremities and sustained clonus at both ankles. There is good upper-extremity function. A magnetic resonance imaging scan of the brain reveals periventricular leukomalacia. The child is diagnosed with spastic diplegic CP.

Answer 113

MRI brain:

• Periventricular leukomalacia
• Congenital malformation
• Stroke/Haemorrhage

Question 114

How is Cerebral Palsy managed?

Answer 114

• OT/PT/SLT
• Orthoses – Splinting to maintain correction
• Adaptive equipment – Walking aids

If spastic:

• Botulinum toxin A

Dyskinesia:

• Carbidopa/Levadopa trial

Ataxia:

• Reduce tremor (Propranolol/Clonazepam)

Question 115

What is Duchenne Muscular Dystrophy?

What are the symptoms/Signs?

How is it investigated and managed?

Answer 115

• Progressive generalised diseases of muscle - due to absent glycoproteins (Dystrophin) in muscle membrane
• On-going degeneration and re-generation of muscle fibers

Symptoms/Signs:

• FHx of DMD
• Boy > Girls
• Delayed motor milestones
• Imbalance of strength, weak Hip/knee extensors, Ankle dorsiflexors
• Gowers sign – Proximal weakness
• Deep tendon reflexes & tone reduced in all muscle groups
• Sensation – normal

Investigations:

• Serum Creatinine Kinase – Increased
• Genetic testing

Management:

• Prednisolone
• Physio
• Exercise
• Patient education

Question 116

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 4-year-old boy presents with a history of ambulation delayed until 18 months of age, toe walking, calf hypertrophy, and proximal hip girdle muscle weakness. His paediatrician, considering a mild static encephalopathy, did not request screening for myopathy but referred him to an orthopaedic surgeon, who found that his creatine kinase levels were greatly elevated, indicating need for referral to a neurologist. His siblings, a boy of 6 years and a girl of 7 years, are apparently well

Answer 116

• Duchenne Muscular Dystrophy
• Becker muscular dystrophy – Later age of onset + mild clinical involvement
• Cerebral palsy
• Polymyositis

Question 117

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 16-year-old boy presents to the emergency department with a first-time seizure event after attending an all-night party and consuming alcohol. Witnesses described the seizure as beginning abruptly with bilateral limb stiffening, followed by jerking movements in all limbs; the patient has no memory of warning symptoms prior to the seizure. The event seemed to last about 1 minute, and the patient was quite somnolent afterwards. Further review of the history reveals that the patient has been experiencing ‘jerks’ in the morning after awakening, usually involving the arms and shoulders and occasionally causing him to drop things. These ‘jerks’ do not seem to present a problem during the rest of the day.

Answer 117

• Provoked seizure
• Epilepsy
• NEAD
• Convulsive syncope
• TIA
• Cardiac arrythmia

Question 118

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 55-year-old woman recently diagnosed with a brain tumour in the left hemisphere has a witnessed seizure event. The seizure is initially recognised when the patient begins staring and is unresponsive to those around her. She seems to be picking at her clothes with her left hand, but the right arm and leg are not moving. After 20 seconds, she displays rapid head-turning and eye deviation to the right, with tonic extension of the right arm and flexion of the left arm. This is quickly followed by tonic extension of the left arm as well, then clonic jerking occurring in both arms synchronously. This jerking gradually slows and stops after about 30 seconds. The patient then becomes quite somnolent, and she appears to be using her arm and leg less on the right than the left.

Answer 118

• Provoked seizure
• Epilepsy
• NEAD
• Convulsive syncope
• TIA
• Cardiac arrythmia

Question 119

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 6-year-old female without a significant past medical history presents for evaluation of frequent unusual episodes for the past 3 months. The unusual episodes consist of sudden activity arrest with staring and minimal eyelid flutter for 10 to 20 seconds occurring 5 to 10 times per day. The patient is unresponsive to voice or tactile stimulation during the episodes. She is able to immediately resume activities without any recollection of the event once the episode finishes. Her teachers have noted that she stares off in class repeatedly and does not seem to be remembering instructions and classroom material. The diagnosis of attention-deficit/hyperactivity disorder had been suggested. One such unusual episode is induced in front of medical staff with hyperventilation.

Answer 119

• Absence seizure
• Daydreaming
• ADHD
• Epilepsy
• NEAD

Question 120

A patient presents to A&E and you are asked to do their GCS score, how would you go about doing this?

Answer 120

E4, V5, M6

Eyes:

• Open before stimulus – 4
• Open after spoken/shouted request – 3
• Open after finger-tip stimulus (Pressure) – 2
• Not opening at any time – 1

Verbal:

• Correctly gives name, place, date (Oriented) – 5
• Not oriented but can communicate coherently (Confused) – 4
• Intelligible single words (Words) – 3
• Moans/Groans (Sounds) – 2
• No audible response – 1

Movement:

• Obeys 2 part request – 6
• Brings hand to clavicle to supraorbital notch pressure (Localising) – 5
• Bends arm at elbow rapidly away from body (Normal flexion) – 4
• Beds arm at elbow to chest, rotates arm, extension of leg (Abnormal flexion) – 3
• Extends arm at elbow – 2
• No movement in arms/legs – 1

Question 121

How else can consciousness be measured if in a rush?

Answer 121

AVPU

• A - Alert
• V - Responds to Verbal
• P - Pain
• U - Unconscious

Question 122

If a patient presents with a low GCS/Reduced consciousness. How should they be investigated & managed?

Answer 122

• Investigations:
  
  • Capillary blood glucose
  • ABG + Lactate
  • U&E – Electrolyte dysfunction
  • LFTs
  • FBC
  • Blood culture – if signs of infection
  • Urinalysis
  • LP
  • CT head

Question 123

What can cause a provoked seizure?

Answer 123

• Genetic
• Structural lesion
• Metabolic lesion
• Alcohol
• Drugs
• Metabolic imablance - electrolytes etc.
• Head injury

Question 124

What is Non-Epileptic Attack Disorder?

Answer 124

Sudden events which can be mistaken for epilepsy

Either Physiological or Psychogenic

Physiological:

• Syncope
• Delirium
• Sleep disorders
• TIA

Psychogenic:

• Dissociative seizure - involuntary and unconscious
• Factitious seizure - Fabricated/induced illness (Munchhausens)

Question 125

Who classically gets NEAD?

What are the signs/symptoms?

Answer 125

• Females > Males
• Usually preceeds psychological stress

Symptoms/Signs:

• Sudden without warning
• Loss of awareness/unresponsive
• Strange, repeated movements resembling convulsions - violent thrashing
• May cause injury + Urinary incontinence
• Gradual onset
• Asynchronous movements
• Eyes closed
• Recall period of unresponsiveness

Question 126

How is NEAD investigated and managed?

Answer 126

• Video EEG
• Full psychiatric assesment
• FBC – Normal.
• Glucose – Normal
• U&E – Normal
• LFTs – Normal
• Drug screen – Rule out drug use

Management:

• Refer to psychological/psychiatric services for assesment

Question 127

What is a Sub-Arachnoid Haemorrhage?

Answer 127

Bleeding in the sub-arachnoid space (b/w arachnoid & pia mater)

Can be caused:

• Berry aneurysm
• Head injury

Question 128

Who classically gets SAH?

What are the symptoms/signs?

Answer 128

• Usually age >50yrs
• Women > Men
• HTN/Smoking
• Cocaine use/Excess alcohol consumption
• FHx of SAH

Symptoms/Signs:

• Sudden explosive headache - Diffuse
• Lasts 1-2 weeks
• May have nausea & vomiting
• May have seizures
• May have decreased GCS score
• HTN

Question 129

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 53-year-old black woman complains of a sudden, excruciating headache while sitting at work. The headache is diffuse, intense, and accompanied by nausea and vomiting. She describes the headache as the worst headache of her life. She loses consciousness following onset of the headache and is on the floor for less than 1 minute. She is being treated for hypertension and is a smoker. On examination she has a normal mental state, meningismus, bilateral subhyaloid haemorrhages, and right third cranial nerve palsy. There are no sensory deficits or weakness. Brain computed tomography (CT) reveals diffuse subarachnoid blood in basal cisterns and sulci.

Answer 129

• SAH
• Stroke
• Trauma
• Cerebral venous sinus thrombosis
• Hypertensive emergency

Question 130

How would you manage (Investigations + Management) a patient with the following Hx suggestive of SAH?

A 53-year-old black woman complains of a sudden, excruciating headache while sitting at work. The headache is diffuse, intense, and accompanied by nausea and vomiting. She describes the headache as the worst headache of her life. She loses consciousness following onset of the headache and is on the floor for less than 1 minute. She is being treated for hypertension and is a smoker. On examination she has a normal mental state, meningismus, bilateral subhyaloid haemorrhages, and right third cranial nerve palsy. There are no sensory deficits or weakness. Brain computed tomography (CT) reveals diffuse subarachnoid blood in basal cisterns and sulci.

Answer 130

Investigations:

• CT Head – Shows hyperdense appearance of blood
  
  • Immediately
• FBC – May have Increased WCC
• Clotting profile – May have coagulopathy (Increased INR + PTT)
• U&E – Hyponatraemia
• If CT Scan is negative, wait 12 hrs then LP:
  
  • Xanthochromia
• ECG – May show changes

Management:

• Neurosurgical referral
• Catheter coiling/clipping via neuroradiology referral
• Oral Nimodipine - prevent ischaemia via vasospasm
• Analgesia + Antiemetics
• If depressed GCS – Intubation + Ventilation
  
  • ICU admission

Secondary prevention:

• HTN management
• Smoking cessation
• Rehabilitation

Question 131

What is a migraine?

What are the different types of migraine?

Answer 131

• Episodic + chronic recurrent headache that occurs with or without aura, lasts >2hrs
• Due to neurogenic inflammation of the 1st division of the trigeminal sensory neurons - change to brain processing
• Due to cortical spreading depression also

Types:

• Migraine with aura
• Migrain without aura
• Hemiplegic migraine
• Chronic migraine

Question 132

Who classically gets migraines?

What are the symptoms/signs?

Answer 132

• Women > Men
• FHx
• Children + Adults
• Menstruation/obesity/lack of sleep

Symptoms/Signs:

• Paroxysmal headache - Pulsating character
• Severe pain
• Unilateral but may be bilateral
• May be preceeded by visual/auditory aura
• Photophobia
• If menstrual - Up to 2 days of onset of menstruation – due to low estrogen levels
• Normal fundoscopy
• BP – May be normal or High/Low
• Head + Neck exam - normal
• Neurological exam - Normal

Question 133

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 32-year-old woman presents with a 13-year history of 1 to 3 attacks per month of disabling pounding pain over one temple, with nausea and sensitivity to light. She says that her headaches can be triggered by lack of sleep and made worse by physical exertion, and are more common during menstrual bleeding. Untreated, they last for 2 days. On 4 occasions, headaches were preceded by the gradual appearance of a shimmering, zigzag line that enlarged, moved to the peripheral visual field, and then faded away over 45 minutes. Examination is normal.

Answer 133

• Migraine
• Cluster headache
• Tension headache
• Meningitis
• Sinusitis
• SoL
• GCA if >50yrs

Question 134

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 9-year-old boy presents with a 6-month history of recurrent headaches. He does not experience aura. Each headache builds up gradually over 20 minutes and is usually unilateral in nature, pulsating in quality, of moderate intensity, and aggravated by exercise. Nausea, vomiting, photophobia, and phonophobia are common accompanying symptoms. During attacks he favours going to sleep in a dark room, and will often go to bed earlier than usual and wake fully recovered the next day. Attacks can be of variable duration (2 to 48 hours) but generally last around 16 hours. Neurological examination during an attack is entirely normal. He is normotensive. He is completely well in between attacks and is making good academic progress. There is a strong family history of migraine with aura.

Answer 134

• Migraine
• Cluster headache
• Tension headache
• Meningitis
• Sinusitis
• SoL

Question 135

A patient presents with the following Hx suggestive of migraine, how would you investigate and manage the patient?

A 32-year-old woman presents with a 13-year history of 1 to 3 attacks per month of disabling pounding pain over one temple, with nausea and sensitivity to light. She says that her headaches can be triggered by lack of sleep and made worse by physical exertion, and are more common during menstrual bleeding. Untreated, they last for 2 days. On 4 occasions, headaches were preceded by the gradual appearance of a shimmering, zigzag line that enlarged, moved to the peripheral visual field, and then faded away over 45 minutes. Examination is normal.

Answer 135

Investigations:

• Clinical diagnosis
• Unless red flags, then exclude other DD:
• CT/MRI
• FBC + ESR
• Referral to neurology if not resolving w/treatment or red flags present

Management:

• Patient education
• Lifestyle modification
• Headache diary to identify triggers

Acute management:

• Paracetamol/Ibuprofen
• If N+V – Cyclizine (Children) + Prochlorperazine
• Triptans - Sumatriptan (Avoid if HTN)

Prophylaxis:

• Beta blocker – Propranolol/Bispoprolol
• Amitryptyline
• Topimarate/Sodium Valproate
• Pizotifen
• Botox if refractory

Question 136

What are the red flags in a patient presenting with headache?

Answer 136

• Papilloedema
• New seizure
• PMHx of cancer
• Confusion/Ataxia/LoC
• Age <10yrs + 50yrs
• Change to pattern of headache

Question 137

You are asked to explain Triptans to a patient (Sumatriptan), how would you go about doing this?

Answer 137

Layout:

• Check what the patient knows
• Brief history
• Do they know what the drug is?
• Indication & Action of the drug
• Side effects
• How to take it
• Monitoring requirements

[Indication & Action of the drug]

Indication:

• Acute migraine with or without aura

Action:

• Relieve symptoms of acute migraine
• Migraine is thought to be caused by dilatation of cranial blood vessels
• Triptans constrict cranial blood vessels
• Inhibit NT in the peripheral trigeminal nerve & trigeminocervical complex

[Side effects]

• Pain/Discomfort in the chest and throat
• N & V
• Tiredness
• Dizziness
• Transient high BP
• Very rarely - MI

Complications & CI:

• Avoid in:
  
  • CAD - Angina/MI
  • Cerebrovascular disease - Stroke
  • Risk of acute vascular events
  • Hemiplegic migraines
  • Basilar migraines

[How to take it]

• Oral/Intranasal/SC
• Tablets should be swallowed whole with water
• During headache + if it recurs
• Can be taken in combination with Ibuprofen + Paracetamol

[Monitoring requirements]

• Treatment is to reduce severity of their migraines - should shorten the duration and intensity of the headache
• Take treatment as soon as they feel the migraine coming on, within 6hrs of onset of a moderate to severe headache
• Will not prevent an attack
• Schedule follow up appointment for all patients

Question 138

Who classically gets tension headaches?

What are the symptoms/Signs?

Answer 138

• most common type of chronic recurring head pain
• Women > Men
• most common in young adults

Symptoms/Signs:

• featureless, often generalised headache – Mild/moderate
• pressure or tightness, like a vice or tight band around the head - Frontal Occipital pain
• relationship to the neck, with pain into or from the neck
• disabling for a few hours
• Gradual onset, short duration
• Not aggravated by routine physical activity
• Often present at, or soon after, getting up in the morning
• Related to Anxiety/Depression/Poor posture/Poor sleep/Stress/Muscular tightness
• may be some tenderness in the scalp or neck
• Normal neurological examination

Question 139

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 37-year-old woman presents with a 12-year history of episodic headaches. She experiences these 4 times a week, typically beginning at the end of a working day. The pain is generalised and described as similar to wearing a tight band around her head. The headaches are bothersome, but not disabling, and she denies any nausea or vomiting. She is slightly sensitive to noise but has no photophobia. Pain during her attacks typically responds to ibuprofen. Examination reveals tenderness of her scalp and both trapezius muscles.

Answer 139

• Tension headache
• Migraine.
• Giant cell arteritis (temporal arteritis).
• Trigeminal neuralgia.
• Temporomandibular joint dysfunction.
• Subarachnoid haemorrhage.
• Sinusitis.
• Encephalitis.
• Cervical spondylosis.

Question 140

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 56-year-old man presents with a 25-year history of constant headache. The onset was insidious and he is certain that the only time he is headache-free is when he sleeps. He states the headache is generalised and his neck and shoulders are always ‘tight’. He denies any associated autonomic symptoms including eye tearing, nasal congestion, light and sound sensitivity, nausea, or vomiting.

Answer 140

• Tension headache
• Migraine.
• Giant cell arteritis (temporal arteritis).
• Trigeminal neuralgia.
• Temporomandibular joint dysfunction.
• Subarachnoid haemorrhage.
• Sinusitis
• Encephalitis.
• Cervical spondylosis.

Question 141

A patient presents with the following Hx, suggestive of tension headache. How would you investigate and manage the patient?

A 56-year-old man presents with a 25-year history of constant headache. The onset was insidious and he is certain that the only time he is headache-free is when he sleeps. He states the headache is generalised and his neck and shoulders are always ‘tight’. He denies any associated autonomic symptoms including eye tearing, nasal congestion, light and sound sensitivity, nausea, or vomiting.

Answer 141

Investigations:

• Clinical diagnosis

Management:

• Reassurance through a positive diagnosis, based on the features of the headache
• Patient education
• ibuprofen 400 mg
• Amitriptyline is the treatment of choice for frequently recurring episodic TTH or chronic TTH

Question 142

Who classically gets cluster headaches?

What are the symptoms/signs?

Answer 142

• lifelong duration
• Men > Women
• usually begins between the ages of 20 and 40 years

Symptoms/Signs:

• Periodic short lived Headaches last 6-12 weeks, once a year or two years, often at the same time each year
• severe unilateral pain, localised in or around the eye (Trigeminal distribution)
• sharp and penetrating
• ipsilateral Autonomic features:
  
  • include ptosis, conjunctival injection, lacrimation, rhinorrhoea, nasal stuffiness, eyelid and facial swelling, aural fullness, facial sweating, and redness
• very restless or agitated during an acute attack
• Made worse or triggered by alcohol
• Normal neurological exam

Question 143

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 44-year-old male smoker presents with a 9-year history of recurrent headaches. Headaches occurred twice-monthly initially, always in the early hours of the morning (2 a.m. to 3 a.m.). The headaches have increased to an average of 2 episodes per day. The acute episodes can occur at any time, and last between 2 and 4 hours. He always has a nocturnal event. Attacks are triggered immediately after drinking alcohol or with the smell of strong aftershave or petrol. The pain is excruciating and focused around his right eye. The right eye reddens and tears, the right eyelid droops, and the right nostril runs. He becomes severely agitated during attacks, often pacing the room or rocking back and forth. Physical examinations, lumbar puncture, brain magnetic resonance imaging (including pituitary views), and pituitary function blood tests are normal.

Answer 143

• Cluster headache
• Paroxysmal hemicrania
• Migraine
• Tension headache
• GCA
• Trigeminal neuralgia

Question 144

A patient presents with the following Hx suggestive of cluster headahces. How would you investigate and manage the patient?

A 44-year-old male smoker presents with a 9-year history of recurrent headaches. Headaches occurred twice-monthly initially, always in the early hours of the morning (2 a.m. to 3 a.m.). The headaches have increased to an average of 2 episodes per day. The acute episodes can occur at any time, and last between 2 and 4 hours. He always has a nocturnal event. Attacks are triggered immediately after drinking alcohol or with the smell of strong aftershave or petrol. The pain is excruciating and focused around his right eye. The right eye reddens and tears, the right eyelid droops, and the right nostril runs. He becomes severely agitated during attacks, often pacing the room or rocking back and forth. Physical examinations, lumbar puncture, brain magnetic resonance imaging (including pituitary views), and pituitary function blood tests are normal.

Answer 144

Investigations

• Clinical diagnosis
• Urgent referral is recommended for all people with suspected CH - for confirmation of the diagnosis, investigation of secondary causes of CH, and initiation of preventative treatment
• Brain CT/MRI - normal in primary cluster headache
• ESR – Normal

Management:

• Be prepared for attacks. Patients should be encouraged to have both acute and preventative treatments available
• Stop smoking
• Abstain from alcohol during attacks
• Sumatriptan - 6 mg subcutaneously is effective/Sumatriptan nasal spray - works less well for most patients
• Oxygen - 100% oxygen, given for 15 minutes up to five times per day

Prophylaxis:

• Verapamil
• Prednisolone
• Lithium

Question 145

What are the symptoms/signs of increased ICP?

What are the causes?

How should it be investigated & managed?

Answer 145

Causes:

• SoL
• Disorders of CSF circulation – Obstructive hydrocephalus/Communicating hydrocephalus
• Traumatic haematoma – Subdural/extradural
• Abscess
• Focal oedema from trauma/infarction/tumour
• Idiopathic intracranial HTN

Symptoms/Signs:

• Headache – Worse on coughing/moving head
• Papilloedema
• Pupillary changes - irregular + dilated in 1 eye
• Vomiting
• Altered mental state – lethergy + irritability + confusion to late signs of Coma/Stupor
• Unilateral ptosis
• 3rd/6th nerve palsy

Late signs:

• Hemipareisis
• Increased BP
• Wide pulse pressure
• Low HR

Investigations:

• ABCDE assesment
• CT/MRI – determine if underlying lesion
• Blood glucose
• U&E – assess for metabolic imbalance
• Monitor ICP

Management:

• Maintain O2 + BP
• Normal glucose
• Avoid pyrexia
• Manage seizures
• Drain CSF if required
• Elevate head of bed
• Analgesia + Sedation
• If continued:
  
  • Barbituate induced coma
  • Hypothermia
  • Depressive craniotomy

Question 146

What is idiopathic intracranial HTN?

Answer 146

• Raised ICP in the basence of a mass lesion or hydrocephalus
• Due to impaired CSF absorption in subarachnoid space via arachnoid villi into dural sinuses
• Emergency – Risk of partial or total blindness (Papilloedema)

Associated with:

• Endocrine conditions (Adrenal insufficiency/Cushings syndrome/Hypothyroidism)
• Medication (Corticosteroids/Levothyroxine/Lithium)
• Other conditions (Polycythemia vera/IDA/CKD/SLE)

Question 147

Who classically gets Idopathic intracranial HTN?

What are the symptoms/Signs?

Answer 147

• Common in obese women of childbearing age

Symptoms/Signs:

• Headache - Generalised throbbing + Worse in morning + last thing at night
• Relieved by standing
• Aggravated by valsalva maneouvre + change in position
• Gradual vision loss
• N&V
• May have diplopia due to CN6 palsy

Question 148

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 29-year-old woman presents with a 3-month history of worsening headaches and increasing visual loss. She describes occasional episodes of bilateral visual greyouts lasting 20 seconds that may be precipitated by bending forwards or standing. Over the last 2 weeks she has often heard a ‘whooshing’ sound, synchronous with her pulse, that is more noticeable when she is about to go to sleep. Her visual acuity is 20/30 (6/9 metres) in each eye. Fundus examination shows bilateral optic disc swelling, and Humphrey automated perimetry shows enlargement of the blind spot and scattered abnormal test locations. Magnetic resonance imaging shows a partially empty sella, and a magnetic resonance venogram shows no evidence of a thrombosis but does demonstrate bilateral transverse sinus venous stenoses. Lumbar puncture opening pressure is 280 mm H2O.

Answer 148

• Idiopathic Intracranial HTN
• Headache – Migraine/Cluster/Tension
• SoL
• Malignant HTN

Question 149

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 42-year-old obese man presents with a 2-month history of increasing headaches that are worse in the morning and associated with nausea. He has occasional episodes of transient visual loss in both eyes that lasts 30 seconds before recovering. At night he admits to hearing a ‘whooshing’ sound in his head in time with his pulse. His wife reports that he snores in his sleep and occasionally seems to stop breathing.

Answer 149

• Idiopathic Intracranial HTN
• Headache – Migraine/Cluster/Tension
• SoL
• Malignant HTN

Question 150

A patient presents with the following Hx suggestive of idiopathic intracranial HTN, how would you investigate and manage the patient?

A 42-year-old obese man presents with a 2-month history of increasing headaches that are worse in the morning and associated with nausea. He has occasional episodes of transient visual loss in both eyes that lasts 30 seconds before recovering. At night he admits to hearing a ‘whooshing’ sound in his head in time with his pulse. His wife reports that he snores in his sleep and occasionally seems to stop breathing.

Answer 150

Investigations:

• Clinical diagnosis
• Rule out other DD:
  
  • Fundoscopy - normal/may have papilloedema
  • FBC
  • ESR
  • CT/MRI – Ventricles normal or decreased in size
  • Visual field charting – enlarged blind spot + peripheral visual loss

Management:

• Weight reduction
• CSF diversion surgery if visual loss
• Diuretics

Acute treatment:

• Prednisolone – relieve headache + papilloedema

Question 151

You are asked to explain to a patient how a LP will be performed. How will you go about this?

Answer 151

Before the procedure

• Explain to the patient what you want to do and why you need to do it.
• Obtain explicit consent and use the appropriate consent form.
• Tell the patient that co-operation is important and that they can talk to you at any time.
• Explain that you will numb the area with local anaesthetic, which will involve a sharp scratch and then some stinging which will quickly settle
• Advise that, subsequently, there should be no pain. However, they may feel sensations down a lower limb and there could be a pulling/pressure sensation and mild discomfort.

Procedure:

• Place the patient on their left side with their back exactly vertical, aligned with the edge of the bed, with their spine fully flexed - knees up to chin
• Mark the intervertebral space L4/L5 or L3/L4 (at the same height as the iliac crest) with a gentle skin indentation using a thumbnail or an object like a pen top.
• Wash your hands thoroughly and put on a mask and sterile gloves.
• Sterilise the area with iodine-based antiseptic unless the patient is allergic.
• Anaesthetise the skin with 1% lidocaine
• After one minute, insert a 22G spinal needle with stylet in place
• Withdraw the stylet and wait for CSF to appear at the needle cuff.
• Measure CSF pressure with the manometer
• Usually 5-10 drops for each sample are sufficient.
• Three bottles go to microbiology, one to virology, one to biochemistry.
• Reinsert the stylet to halt CSF flow
• Clean, then dress, the site.

After:

• Prescribe simple analgesia as required in case of headache.
• Document the procedure

Complications:

• Post-LP headache – Treatment with pain relief + fluids
• Infection.
• Bleeding (approximately 2%).
• Cerebral herniation (rare but potentially fatal)

Question 152

What is an extradural haematoma?

Answer 152

• collection of blood in the potential space between the dura and the bone
• Usually that bone is the skull but extradural haemorrhage can occur in the spinal column
• most often due to a fractured temporal or parietal bone damaging the middle meningeal artery or vein, with blood collecting between the dura and the skull

Question 153

Who classically gets EDH?

What are the symptoms/signs?

Answer 153

• Head injury
• Males > Females

Symptoms/Signs:

• Hx of trauma ± head injury that causes loss of consciousness.
• Followed by a lucid interval after which the patient deteriorates
  
  • EDH in the posterior fossa can produce a very rapid deterioration to death, measured in minutes.
• Headache.
• Nausea or vomiting.
• Seizures.
• Bradycardia with or without hypertension, indicates raised intracranial pressure.
• Evidence of skull fractures, haematomas, or lacerations.
• Cerebrospinal fluid (CSF) otorrhoea or rhinorrhoea resulting from skull fracture with a tear of the dura.
• Unequal pupils.
• Facial nerve injury.
• Weakness of limbs
• radicular symptoms or a complete cord compression

Question 154

How would you investigate a patient with EDH and manage them?

Answer 154

Investigations:

• Baseline FBC and U&E
• If there is any suspicion of abnormality of coagulation – platelets and coagulation studies are required
• CT scanning gives much more information. It may show a haematoma or air pockets
• Avoid LP if possible raised ICP

Management:

• ABCDE
• Maintain airway
• C-spine protection
• O2 + IV fluids
• alert patient with a small haematoma may be treated conservatively but must be observed in case of sudden deterioration
• If intracranial pressure is raised, it may be treated with osmotic diuretics, such as IV mannitol
• Burr holes may be required to evacuate a haematoma

Question 155

What is a subdural haematoma?

Answer 155

• subdural space is the space between the dura mater and the arachnoid mater
• collection of clotting blood that forms in the subdural space

caused by either:

• Tearing of bridging veins from the cortex to one of the draining venous sinuses – typically occurring when bridging veins are sheared during rapid acceleration-deceleration of the head.
• Bleeding from a damaged cortical artery
• Blunt head trauma is the usual mechanism of injury

Question 156

Who classically gets SDH?

What are the symptoms/signs?

Answer 156

• physical abuse in infants and children
• Elderly due to cerebral atrophy (Tension on veins)
• Alcohol misuse leads to a risk of thrombocytopenia, prolonged bleeding times and blunt head trauma and is a risk factor for SDH
• Anticoagulation use

Symptoms/Signs:

• recent trauma
• coagulopathy or anticoagulant use/Alcohol use
• Usually presents shortly after a moderate-to-severe head injury.
• Loss of consciousness may occur but not always.
• There may be a ‘lucid interval’ of a few hours after the injury
• Symptoms tend to be gradually progressive.
• There is often a history of anorexia, nausea and/or vomiting.
• There may be a gradually evolving neurological deficit such as focal limb weakness, speech difficulties, increasing drowsiness/confusion or personality changes.
• If there is accompanying and progressive headache
• bradycardia and hypertension associated with raised intracranial pressure.

Question 157

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A young man is brought to the emergency department after being involved in a high-speed motor vehicle accident. He was an unrestrained driver, and no airbags were deployed. He has multiple areas of abrasions, lacerations, and ecchymosis on his scalp and face. On neurological examination, he does not open his eyes to painful stimuli; he is intubated, and he withdraws his left side to pain. His right side is plegic. His right pupil is 3 mm and reactive to light and his left pupil is 8 mm and non-reactive.

Answer 157

• Subdural haematoma
• Epidural haematoma.
• Subarachnoid haemorrhage.
• Intracerebral haemorrhage or infarction.
• Meningitis or encephalitis.
• Cerebral tumour
• Stroke

Question 158

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

An older man with a longstanding history of atrial fibrillation on anticoagulation with warfarin is brought into the emergency department by his carer, who states his concern about the patient’s confusion at home. The carer describes frequent falls over the last several months and says that the patient is dropping utensils from his right hand. On neurological examination, his pupils are equal, round, and reactive to light. He has a right-sided pronator drift and is weaker on his right side than on his left. His mental status testing reveals poor concentration and attention, and impaired short- and long-term recall and registration.

Answer 158

• Subdural haematoma
• Epidural haematoma.
• Subarachnoid haemorrhage.
• Intracerebral haemorrhage or infarction.
• Meningitis or encephalitis.
• Cerebral tumour
• Stroke

Question 159

A patient with the following Hx suggestive of SDH presents. How would you investigate and manage the patient?

An older man with a longstanding history of atrial fibrillation on anticoagulation with warfarin is brought into the emergency department by his carer, who states his concern about the patient’s confusion at home. The carer describes frequent falls over the last several months and says that the patient is dropping utensils from his right hand. On neurological examination, his pupils are equal, round, and reactive to light. He has a right-sided pronator drift and is weaker on his right side than on his left. His mental status testing reveals poor concentration and attention, and impaired short- and long-term recall and registration.

Answer 159

Investigations:

• FBC
• U&Es
• LFTs
• blood for group and save/cross-match
• CT Head

Management:

• ABCDE assesment
• C-spine immobilisation
• Obtain senior A&E, anaesthetic or neurosurgical advice
• If the condition is strongly suspected or confirmed by investigation, refer urgently to the neurosurgical team
• Hypertonic saline or mannitol may be considered if there is raised intracranial pressure.
• Burr holes may be considered if there is rapid deterioration
• SDH is treated by emergency craniotomy and clot evacuation

Question 160

What is Bells palsy?

Answer 160

• Damage to the facial nerve, causing weak muscles of facial expression
• UMN Upper facial muscles partially spared (CAN wrinkle forehead)
  
  • Due to the function of the opposite nerve still functioning, Note: if bilateral lesion this would not happen
• LMN
  
  • CANNOT wrinkle their forehead -> b/c final common pathway is damaged
  • Lesion is either in Pons or outside brainstem

Cause:

• reactivation of HSV 1 within the Geniculate ganglion, leads to the destruction of the ganglion cells and infection of the schwann cells -> Demylination & neural inflammation

Question 161

Who classically gets Bells palsy?

What are the symptoms/signs?

Answer 161

• Most common cause of unilateral facial palsy in >2yr olds
• Most prevalent age 15-45yrs
• Male = Females

Symptoms/Signs:

• Single episode - can recur but highly unlikely
• Unilateral Weakness of ALL muscles of facial expression & eye closure
• Patient may be unable to voluntarily close their eyes -> damage to conjunctiva & cornea

More severe cases:

• Loss of taste over anterior 2/3 of tongue
• Intolerance to high pitched/loud noises
• Mild dysarthria & difficulty eating
• No systemic symptoms
• No fever/malaise/myalgia/headache/rash
• Face sags and is drawn across opposite side on smiling
• Patient may be unable to voluntarily close their eyes -> damage to conjunctiva & cornea
• Keratoconjunctivitis Sicca -> Dry eye

Question 162

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 40 yr old woman awakens with left-sided facial fullness and a subjective feeling of facial and tongue ‘numbness’ , but it is not visible

She also notes left-sided dysgeusia (change to sense of taste)

Later that day she develops left-sided otalgia, hyperacusis, post-auricular pain, and facial discomfort.

Left-sided facial palsy ensues, with associated oral incompetence, facial weakness, and asymmetry progressing to complete flaccid paralysis by the next morning.

O/E: the resting appearance of the left face demonstrates brow ptosis, a widened palpebral fissure, effacement of the left nasolabial fold, and inferior malposition of the left oral commissure.

There is complete absence of brow movement, incomplete eye closure with full effort, and loss of smile, snarl, and lip pucker on the affected side.

The remainder of the history and physical examination are unremarkable.

Answer 162

• Bells palsy
• Herpes zoster oticus (Ramsey Hunt syndrome)
• Lyme disease
• Facial nerve schwannoma (Benign nerve tumour)
• Malignant facial nerve tumour
• Blunt force trauma to face or temporal bone
• Stroke

Question 163

A patient presents with the following Hx, suggestive of Bells palsy. How would you investigate and manage the patient?

A 40 yr old woman awakens with left-sided facial fullness and a subjective feeling of facial and tongue ‘numbness’ , but it is not visible

She also notes left-sided dysgeusia (change to sense of taste)

Later that day she develops left-sided otalgia, hyperacusis, post-auricular pain, and facial discomfort.

Left-sided facial palsy ensues, with associated oral incompetence, facial weakness, and asymmetry progressing to complete flaccid paralysis by the next morning.

O/E: the resting appearance of the left face demonstrates brow ptosis, a widened palpebral fissure, effacement of the left nasolabial fold, and inferior malposition of the left oral commissure.

There is complete absence of brow movement, incomplete eye closure with full effort, and loss of smile, snarl, and lip pucker on the affected side.

The remainder of the history and physical examination are unremarkable.

Answer 163

Investigations

• Clinical diagnosis - based on Hx & examination
• Serology - rule out Lyme disease/HSV

Management:

• Reassurance that it most cases resolve spontaneously within 3wks
• If no recovery - Steroids required
• Prednisolone within 72hrs
• Eye careTo prevent irreversible blindness from corneal exposure
• Lubricating eye drops ± eye patch

Question 164

What are the complications of Bells palsy?

Answer 164

• Facial asymmetry
• gustatory lacrimation
• inadequate lid closure
• brow ptosis
• drooling
• hemifascial spasms

Question 165

What is Ramsey Hunt syndrome?

Answer 165

• Herpes zoster oticus/auricular herpes zoster
• When VZV virus becomes reactivated in the genuculate ganglion of CN 7
• causing
  
  • Facial paralysis
  • Loss of taste
  • Vestibulococchlear dysfunction & pain
  • Shingles -> Disease of sensory nerves, Ramsey Hunt -> Disease of the motor nerves also

Question 166

Who classically gets Ramsey Hunt syndrome?

What are the symptoms/Signs?

Answer 166

• HZV is a disease usually seen in older people, but can affect all ages
• Incidence and severity increase with age
• More common in patients who are immunocompromised

Symptoms/Signs:

• Can present without a rash -> Herpes Zoster sine herpete
• Deep pain within the ear
• Usually paroxysmal at first, then radiates out to the Pinna where it is more constant - diffuse & dull background pain
• Pt may also have - Vertigo/ipsilateral hearing loss/hyperacusis/tinnitus
• Facial weakness or droop
• Rash/blisters - in distribution of nerves. particularly in skin of ear canal/auricle/both

Question 167

How would you investigate + Manage a patient with suspected Ramsey Hunt syndrome?

Answer 167

Investigations:

• Clinical diagnosis
• Serology may be obtained - not necessary
• MRI - can identify the areas of inlammation along CN 7
• Audiometry

Management:

• Shingles Management - within 72hrs of symptom onset Antivirals
• Aciclovir 5x a day for 7 days
• High dose steroids
• Prednisolone
• Analgesia
• NSAIDs/Paracetamol with or w/o codeine
• If there is a problem closing the eyes with the facial palsy
• Pad to protect the cornea & eye lubricants

Question 168

How does a common peroneal nerve palsy typically present?

What are the causes?

Answer 168

Causes

• Some cause of external pressure on the nerve - Habitual leg crossing/prolonged squatting or kneeling/confinement to bed/or use of leg supports
• Pt may have recent weight loss - Slimmers palsy
• Pt may have Hx of trauma to the fibular head - # or surgery

Symptoms/Signs:

• High stepping gait - to prevent foot from dragging
  
  • Due to weakness of the foot dorsiflexion muscles
• Difficulty walking on heel of the affected leg -> if peroneal neuropathy
• May have swelling & erythema if linked to trauma/injury
• Fasciculations/muscle wasting - More extensive neurological problem (MND)
• Sensory abnormality in 1st web space (Deep peroneal nerve lesion)
• Sensory abnormality in anterolateral aspect of lower leg & foot dorsum (Superficial peroneal nerve lesion)
• If weak hip abduction -> Lumbar L5 radiculopathy
• NO weak hip abduction -> peroneal neuropathy
• Reflexes should be normal

Question 169

How should a common peroneal nerve palsy be investigated and managed?

Answer 169

Investigations:

• Clinical diagnosis
• Nerve conduction studies - confirm diagnosis & inform prognosis
• If Demyelinating (Most common) -> Full recovery is expected in a few weeks
  
  • If severe compression -> Axonal loss, recovery will take longer & may be incomplete
• Pt would have abnormalities on EMG - Denervation changes

Management:

• Advise patient to avoid similar activities in the future
• Recommend foot drop splint
• There will be gradual improvement, but recommend referral to neurology clinic -> Confirm diagnosis
• Symptoms typically improve in 2-3months

Question 170

What happens with a radial nerve palsy?

What causes it?

Answer 170

• Causes a wrist drop
• Sensory loss in the dorsal aspect of the root of the thumb
• Due to compression of the radial nerve, against the mid shaft of the humerus - Susceptible to compression
• Classic description
  
  • Sleeping on hard chair - with arm hanging over the back or arm, perhaps after drinking alcohol

Question 171

What is Charcot Marie tooth disease?

What are the symptoms/Signs?

Answer 171

• inherited peripheral neuropathies
• Autosomal dominant inheritance

Symptoms/Signs:

• Onset is insidious and slowly progressive
• Onset is usually by the age of 10 years
• Muscle weakness and wasting starting from the intrinsic muscles of the feet, and gradually affecting the lower leg and lower thigh + Hands and forearms may also be involved
• Muscle wasting
• Sensory loss - ascending + progressive
• Proprioceptive sensory loss can cause sensory ataxia and a steppage gait
• Pain
• Generalised tendon areflexia

Question 172

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 45-year-old man presents with a bilateral steppage gait (lifting legs up excessively to clear the toes), foot numbness, and difficulty with buttons. His birth history was normal, and early motor milestones were achieved on time. He began walking at 13 months, and was noted to be a ‘toe-walker’. As a child he ran towards the middle to the back of his peers and was never able to ice skate because of weak ankles. He first noted problems with walking in his early twenties, tripping often and falling once a month, and has started having problems with his hands in the last 5 years. Nerve conduction studies show symmetrical nerve slowing to 23 m/second (normal >50 m/second) with mildly reduced amplitudes and prolonged distal latencies. Sensory responses are absent. His father was noted to have the same symptoms.

Answer 172

• Charcot Marie Tooth disease
• Other genetic neuropathies.
• Friedreich’s ataxia.
• Acquired neuropathies - eg, related to alcohol, vitamin B12 deficiency, thyroid disease, diabetes mellitus, and infection (HIV, leprosy, syphilis).
• Vasculitis.
• Amyloidosis.

Question 173

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnosis?

A 30-year-old woman presents with severe leg weakness and distal arm weakness. Her birth history was normal and early motor milestones were achieved on time. She began to have trouble with foot-drop and falling in her pre-school and school-age years. The foot-drop and weakness in the proximal muscles progressed through her teens, so that walking up stairs became very difficult. She required a wheelchair for primary ambulation at 20 years of age. On examination, her hands are atrophied and contractures are present, causing an ‘en griffe’ appearance, and weakness extends proximally. The sensory examination is mostly normal. Nerve conduction studies show no motor or sensory responses. However, she has brought studies from childhood showing normal conduction velocities and severely reduced amplitudes, indicative of axonal degeneration. She has no family history of weakness or neuropathy.

Answer 173

• Charcot Marie Tooth disease
• Other genetic neuropathies.
• Friedreich’s ataxia.
• Acquired neuropathies - eg, related to alcohol, vitamin B12 deficiency, thyroid disease, diabetes mellitus, and infection (HIV, leprosy, syphilis).
• Vasculitis.
• Amyloidosis.

Question 174

How would you investigate a patient with CMT disease?

How is it managed?

Answer 174

Investigations:

• Exclude other causes of neuropathy with tests that may include:
• FBC.
• TFTs.
• LFTs.
• VDRL.
• Vitamin B12.
• Folate.
• Antinuclear antibodies.
• Creatine kinase.
• Serum and urine protein electrophoresis.
• Muscle biopsy.
• Cerebrospinal fluid (CSF) examination.
• MRI of the brain and spinal cord.
• Genetic studies - family history may be falsely negative due to the variable expression of mutations
• nerve conduction studies show – Low conduction velocities

Management:

• no effective treatments to reverse or slow the underlying disease process.
• Supportive treatment is offered based on rehabilitation and surgical corrections of skeletal deformities

Question 175

What does the following CT scan show?

Answer 175

Subarachnoid haemorrhage

• hyperattenuating material is seen filling the subarachnoid space
• Starfish appearance

Question 176

What does the following CT scan show?

Answer 176

Extradural haemorrhage

• B/w the skull and the dura mater.
• Limited by cranial sutures
• Lemon shape

Question 177

What does the following CT scan show?

Answer 177

Subdural haemorrhage

• Mass effect (Midline shift)
• Haemorrhage crosses suture lines
• Banana shaped

Question 178

What does the following CT scan show?

Answer 178

Chronic subdural haematoma

• Banana shape
• Haemorrhage crosses suture lines

Question 179

What is Myasthenia gravis?

Answer 179

• Autoimmune condition - B & T cells
• Antibodies to nicotinic ACh receptors on post synaptic NMJ
• muscular weakness with easy ‘fatiguability’, which is worse on exercise and improves with rest

Question 180

Who classically gets Myasthenia gravis?

What are the symptoms/signs?

Answer 180

• Men > Women
• Age at onset: child, or aged less than or more than 50 years.
• Type of course: ocular or generalised
• Common with thymic atrophy/tumour

Symptoms/Signs:

• Slowly increasesing/relapsing muscular fatigue
• Proximal weakness
• Affecting - Extraocular (May be in isolation)/Bulbar/Face/Neck/Limb/Girdle/trunk
• Ptosis
• Diplopia
• Orbicularis fatigability (Begin to seperate after manual opposition to closure)
• Dysphonia on counting
• No wasting/fasciculation
• Normal tone
• Sensation normal
• Normal tendon reflexes

Question 181

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 25-year-old woman presents with recurrent slurring of speech that worsens when she continues to talk. She has trouble swallowing, which deteriorates when she continues to eat, and has double vision that gets worse when sewing, reading, or watching TV. She reports that her head is heavy and hard to hold up. Her symptoms have progressively deteriorated over the past 6 months. She has intermittent weakness in her legs and arms. She is fearful of falling due to her legs giving way and she has trouble combing her hair or putting on deodorant. She reports a feeling of generalised fatigue and is occasionally short of breath.

Answer 181

• Myasthenia gravis
• Polymyositis
• Myopathy
• SLE
• Takayashu’s arteritis

Question 182

A patient presents with the following Hx suggestive of Myasthenia gravis, how would you investigate & manage them?

Answer 182

Investigations:

• Anti ACh antibodies – Increased
• MuSK antibodies – present if Anti-ACh negative
• EMG – Decremental muscle respose to repetitive stimulation
• CT – Exclude thymoma
• TFTs – Normal

Management:

• Pyridostigmine (Anticholinesterase)

Relapse:

• Prednisolone
• Azathioprine/Ciclosporin/Mycophenilate mofetil
• Thymectomy

Question 183

What is a Myasthenic crisis?

Answer 183

• Life threatening weakness of respiratory muscles during myasthenia gravis relapse
• Monitor FVC

Management:

• Ventilatory support
• Plasmapheresis (Remove AChR antibodies from circulation)
• OR
• IV Ig
• Identify trigger for relapse - Infection/medications

Question 184

What is Lambert Eaton syndrome?

What are the symptoms/signs?

Investigations?

Management

Answer 184

• Similar to Myasthenia gravis, but antibodies are to Ca channels on the presynaptic membrane

Causes

• Paraneoplastic (SCLC)
• Autoimmune

Symptoms/Signs:

• Gait difficulty before eye signs
• Autonomic involvement
• Hyporeflexia + weakness – improve after exercise

Investigations:

• Symptoms improve after exercise
• EMG – Amplitude increases after exercise, otherwise same as myasthenia

Management:

• Pyridostigmine
• IV Ig
• Regular CXR/HRCT - as may preceede cancer