Neurology

Question 1

What are the cranial nerve syndromes and what nerves are involved in each?

Answer 1

Cavernous sinus syndrome:
- Unilateral CN3, 4, 5(1,2) and 6

Cerebellar pontine angle syndrome:
- Unilateral CN5, 6, 7, 8 (usually due to a tumour in the cerebellar pontine angle)

Jugluar foramenal lesion:
- Unilateral CN9, 10, 11 (Dif from bulbar plasy because it is unilateral)

Question 2

What causes Cavernous sinus syndrome?

Answer 2

Caused by lesions that affect the cavernous sinus such as:
-> Vascular / thrombotic: VTE cavernous sinus, ICA aneurysm
-> Neoplastic
-> Infection of the cavernous sinus
-> Inflammation from another etiology

Question 3

Presentation of cavernous sinus thrombosis /syndrome?

Answer 3

Presentation (not all must be present):
- Ipsilateral proptosis (only found in thrombosis, due to congestion of the blood and fluid in the orbit from venous obstruction)
- Chemosis secondary to proptosis
- complex opthalmoplegia from LMN lesion or from congestion and phsysical restriction of the EOMs (similar to graves eye disease)
- Horners syndrome
- Trigeminal sensory loss in V1 and 2

Question 4

Presentation of cerebello-pontine angle syndrome?

What is the most common cause of cerebello-pontine angle syndrome and what are some differentials in this aetiology?

Answer 4

Hearing loss, Tinitus, vertigo (CN 8)
Unilateral facial weakness (CN 7)
Loss of facial sensation / facial pain and muscles of mastication (CN5)
Diplopia (CN6)

Most common cause is tumour
Most common tumour is acoustic neuroma (CN8 tumour), but can also be meningioma or other tumours
- Because acoustic neuroma is most common cause, pt will present with vestigular and cochlear symptoms +/- additional CN5,6,7 symptoms

Question 5

What is bruns-Cushing nystagmus and where does it localise to?

Answer 5

Bruns-cushing nystagmus localises to teh cerebelo-pontine angle

It is the combination of a gaze paretic slow beating large amplitude nystagmus, and a contralateral gaze evoked fast beating nystagmus

Question 6

What is gaze paretic and gaze evoke nystagmus?

Answer 6

Gaze paretic nystagmus is a type of gaze evoked nystagmus

Gaze evokes nystagmus is nystagmus that is only present when pt is not in neutral position (ie they are attempting gaze)
- Gaze paretic nystagmus means the pt is unable to hold the gaze position (they have beats of nystagmus to the gaze position but overall the eye tend to the neurtal position)

Question 7

What causes jugular foraemen syndrome? How does it present?

Answer 7

Main cause is vascular :
- Vascular:
-> Internal jugular thrombosis
-> Mass effect from aneurysm of extracranial internal carotid
-> Jugular diverticulum
Other causes include inflammatory, infectious, neoplastic

Presentation is usually hoarsness of voice / dysphonia, often with persistent unilateral periauricular pain and headache
- Hoarse voice and unilateral palate paralysis (uvula dieviation) due to CN 10 Vagus paralysis
- Ipsilateral loss of gag reflex and posterior aspect of tongue due to CN 9 palsy
- Unilateral difficulty with trap and SCM due to CN11 affected

Question 8

Where do the 12 cranial nerves roughly emerge? (relative the the midbrain, pons and medulla)

Answer 8

CN1 and 2 are direct from cerebrum

CN3,4 from midbrain
CN5,6,7,8 from pons
CN9,10,11,12 from medulla

Question 9

What are the localizing forms of nystagmus?

Answer 9

There are localization and non localizing nystagmus.
Non localizing is most common and includes:
- Gaze evoked nystagmus ie horizontal beating gaze evokes, vertical gaze evoked

If you see localizing type of nystagmus you know there must be a lesion at the area the nystagmus localises to:
- Primary position (D)own beating - (C)ervicomedulary junction
- Primary position (U)p beating - (V)ermis of cerebellum
- (C)onvergence retraction nystagmus - (D)osal midbrain
- (S)ee saw nystagmus - (T)hird ventricular or parasella region
- Periodic alternating nystagmus (PAN): spontaneously and periodically direction in primary position - Cerebellum
- Rebound nystagmus (like PAN but alternates with gaze)
- Bruns-Cuhsing - CPA lesion usually tumour

Question 10

What lesion would cause a see saw nystagmus?

Answer 10

This is a localizing nystagmus in hte primary position
It is due to lesion in the sellar and parasellar region ie a tumor
- often associated with bitemporal hemianopia from optic chiasm compression from this lesion

Question 11

What lesion would cause a convergence retraction nystagmus?

Answer 11

Dorsal midbrain syndrome is a collection of signs that include convergence retraction nystagmus.
Other main sign is light, near dissociation due to dirupted afferent pathways at teh dorsal midbrain (efferent is still good because edinger westphal nucleus is not affected)

Dorsal midbrain lesion:
- Neoplastic ie pineal region mass
- Vascular ie stroke
- Demyelination ie MS

Look from side to see retraction. look from front to see convergence in neutral and upward position

Question 12

What lesions would cause PAN?

Answer 12

PAN can be congenital or acquired.
Localities to the cerebellum or posterior fossa, but doesnt localize more then that (ie etiology of issue)

Rx is baclofen for all causes

Question 13

What is the most common lesion that would lead to primary position downbeat nystagmus?

Answer 13

This is a localizing form of nystagmus, localizes to the cervicomedulary junction
It is worse on downward gaze (alexanders law) and especially down and lateral gaze

Causes:
- The most common structural cause is an arnold chiari malformation
- Demyelination ie MS
- Vascular ie infarction

Question 14

What is the most common cause of primary gaze upbeat nystagmus?

Answer 14

This is a localising form of nystagmus and classically localises to the cerebellar vermis.
Can also be due to lesion anywhere in the brain stem (midbrain, pons and medulla)

In other words, the lesion is in the posterior fossa somewhere. Causes include:
- Neoplastic
- Vascular ie stroke, haemorhage
- Demyelination ie MS
- Nutritional or etoh (wernickes encephalopathy mainly)
- Paraneoplastic
- Autoimmunity

Question 15

Signs of CN3 palsy? Causes of CN3 palsy?

Answer 15

• Unilateral complete or partial ptosis - CN3 controls levator palpebrae superioris
• Dilated pupil, imparied reativity
• Down and out eye in primary position (exotropic and hypotropic)

(Note, not all these signs may be present because may have only divisional involvement rather than whole occulomotor nerve involvement. Ie superior division involvement would only cause ptosis and hypotropia)

Causes:
- Isolated third nerve:
-> Vascular: posterior communicating artery aneurysm (PCOM), stroke
-> Neoplastic
-> demyelination
-> infectious
-> inflammatory
- Can also be due to cavernous sinus syndrome if combined with other CN palsies

Question 16

What is the significance of pupiliary involvment in thrid nerve palsy?

Answer 16

The inner most fibers of the occumormotor nerve are the motor fibers. the outermost control the pupil
- Therefore if teh cause is ichemic, it will affect the inner most fibers first causing CN 3 palsy that is pupil sparing
- If cause is compressive ie PCOM aneurysm then then outer most fibers will be affected causing dilation of CN palsy with pupil involvement

Question 17

How is the eye covering test useful in diagnosing opthalmoplegias?

Answer 17

If pt with a opthalmoplegia is asked to focus on a point, they will do so with their non affected eye and try to do so with their affected eye. Because there is a palsy, their affected eye will often not be exactly the same in position as the unaffected eye.
When you cover an eye and ask the patient to look at a certain point, they will focus on the point with the eye that is not covered.

If they have a third nerve palsy, if their affected eye is covered then they will focus on the point with their normal eye and the affected eye will drift into deviated position. Then when their affected eye is uncovered it will try to move back to the gaze position to focus on the point. This movement when uncovered demonstrates the extent of deviation.

Similarly, if unaffected eye is covered then the pt will attempt to focus on point with affected eye. In doing so the unaffected eye will become significantly dieviated such that when it is now uncovered and moves back into position the extent of dieviation is demonatrated.

This meas that the primary dieviation is less than the secondary dieviation which can help indicate which eye is affected.

Question 18

What are the differentials of exotropia in the primary position? What else should you check if you see exotropia?

Answer 18

Thrid nerve palsy
- May also deep slight depression of the eye (exotropia and hypotropia), ptosis and dilated pupil

Other causes will not cause ptosis or cause pupiliary defects. These include:
- INO
- MG
- Decompensated childhood exotropia

Question 19

What are the differentials for ptosis? What else should you check if you see ptosis?

Answer 19

If see ptosis need to check for third nerve palsy by assessing EOM and pupil

Other causes include MG, also need to assess EOM because MG associated opthalmoplegias

Need to check for horners syndrome with contriced pupil, anhydrosis

Other cause is levator muscle dehissence (isolated ptosis)

Question 20

What are the differentials for dilated pupil? What else should you check?

Answer 20

Physiologic aniscoria
- will react and accommodate equally
- Only small difference

Adie’s tonic pupil (idioopathic pathology of teh cilliary ganglion)
- Tonically dilated pupil that reacts slowly to light but accommodates well (light-near disassociation)

Iris pathology
- Test on slit lamp examination

Pharmacological dilation

Need to check for ptosis, and EOM which will be involved to some degree in third nerve palsy. If there is only pupil involved then not going to be third nerve palsy

Question 21

What is light- near dissacociation? what is it caused by?

Answer 21

It is when the eye doesnt contrict to light well, but accomodates normally
- Usually these should be about the same (ie not dissacociated)

It can be due to afferent disease or efferent disease
- Afferent disease
-> bilateral light blindness (cannot be assessed with RAPD because defect is symetrical and bilateral)
-> dorsal midbrain syndrome from mass or syphilus (called argyle ribinson pupil if this is the cause here)
- Eferent disease
-> Idiopathic cilliary ganglion disease is called Adie’s pupil

Question 22

What is the only cranial nerve that crosses before traveling to peripheries? What is the implication of this?

Answer 22

CN4. Exits nucleus and crosses before traveling to peripheries

Therefore trauma or mass efect lesions at dorsal midbrain can impact on both causing bilateral CN4 palsy

Question 23

What is the primary action of CN4?

Answer 23

Only inervates the superior oblique muscles. therefore will always present with blurred vision, eye pain and diplopia as presenting symptoms

Primary action of muscle is intorsion of the eye (in primary position). In some positions it has depression action as well

Question 24

Approach to Dx CN4 palsy on examination?

Answer 24

Inspection:
- Primary position may see slight hypertropia in the affected eye. But also may be normal

Eye movements:
- Grossly may appear normal so have to look closely
- Hypertropia will be worst when affected eye is adducting (look for this with horizontal movements alone)
- Then test depression in adducted position for both eyes. relative hypertropia will be greatest in the affected eye when it attempts to depress in the adducted position

Special tests
- Covering test: will see affected eye hypertropia and exotropia that corrects when affected eye is uncovered (primary dieviation). May see larger secondary deviation in unaffected eye
- Covering test in lateral gaze:
-> the primary and secondary dieviation will be accentuated when the affected eye is made to adduct (ie looking away from the lesion)
- Eye cover test on head tilt test - pt tilts head towards the lesion (this means the pt has to intort the eye which they cant do with a CN4 palsy). Eye covering test in this position will also accentuate the primary and secondary dieviation

Question 25

Causes of isolated and non isolated CN 4 palsy?

Answer 25

Isolated: - Idiopathic - Congenital - Trauma: has the longest intracranial course therefore prone to trauma even mild trauma can affect it - Vascular: microvascular pathology - Neoplastic - dorsal midbrain mass Non isolated - Cavernous sinus syndrome - Briainstem (midbrain lesion) - Large dorsal midbrain mass

Question 26

What are the 6 syndromes of CN6 palsy how they present and what causes them roughly?

Answer 26

The 6 syndromes are based on where the nerve is affected on its journey from the brain stem to the lateral rectus muscle 1. Nucleus affected - Will cause horizontal gaze palsy because CN6 nuc supplies ipsilateral lateral rectus but also contra lateral third nerve nucelus (specifically the contralateral medial rectus) via the median logitudinal fasiculus 2. Fascicle affected - Will have other signs because fasicle is tightly packed in with lots of other structures in the pons so something else is bound to be affected - Because CN6 nuc can still talk to CN 3 nuc, this will behave just like a regular peripheral CN6 palsy (ie wont have horizontal gaze palsy) 3. Subarachnoid space - CN6 plasy is non localising sign of increased or decreased intracranial pressure 4. Clivus - Fractures or neoplasm (usually meningioma but also bone cancers) can affect nerve as it runs along the clivus 5. Cavernous sinus - Can be isolated CN6 in this instance because CN6 sits far away from the rest of teh nerves in teh cavernous sinus 6. Orbit or superior orbital fissue

Question 27

Difference between INO and CN3 palsy?

Answer 27

Both will appear to have exoptropia in primary position INO will have horizontal dissociative abducting nystagmus of unaffected eye. CN 3 will not Therefore lateral gaze testing should distinguish easily. Can reaffirm this with convergance testing in which pt will be able to converge with INO but not with CN3 palsy

Question 28

Name the main components of the speech examination?

Answer 28

General inspection - Movement disorders, cerebellar signs - Syndrome apearances Fluent speech - Ask them to describe the image on the card. Assess for fluent speech and content of speech Verbal comprehension - 1-3 step commands: touch nose. Touch ear then nose Touch nose, then ear then point to sky. - 2 part question: Do you put your shoes on before your socks? Repetition: - Hippopotamus - British constitution - No if and or buts (this is a complex one) Naming: - Point to images on card and ask pt to name them - Listen for difficulty naming Read and write: - Ask them to follow written instructions - Ask them to write a sentence on the page Cranial nerve screening test: - Puh, Tuh and Cuh sounds (CN7, 12, 9/10 respectively) Additional examination: - UL and LL for UMN signs - Cerebellar examination - Visual fields - Lower cranial nerve exam (5 and down)

Question 29

What are the three types of speech problem braodly speaking?

Answer 29

Dysphasia/ aphasia Dysarthria Dysphonia

Question 30

What are the 4 types of distinct dysphasia? What are the hallmarks of each?

Answer 30

Expressive dysphasia (AKA non fluent, brocca's) - Non fluent speech, often visible frustration. Able to comprehend verbal and written. - May have retained automatic speech (ie days of the week or alphabet) - May have retained emotional speech - May have retained singing ability - Due to damage in Broccas area. Dominant hemisphere frontal lobe poterior part third frontal gyrus Receptive dysphasia (AKA fluent dysphasia, wernikes dysphasia) - Verbal and written comprehension is impaired - Fluent and disorganized speech - Due to damage in posterior part of first temporal gyrus Nominal dysphasia - Difficult naming objects but other aspects of speech are normal (Note all types of dysphasia may cause difficulty naming objects but will also have other impariments ie fluency or comprehension) -> May use long sentances to overcome this difficulty (circumlocution) - Due to damage of dominant temporoparital area however is of doubful localising value Conductive aphasia: - Imparied repetition and naming objects - Verbal comprehension intact, able to follow commands - Due to damage of arcuate fasiculus that joins broccas and wernickes

Question 31

What are the main aetiologies of dysarthria and what sort of speech do they produce?

Answer 31

Cerebelar pathology -> Scanning speech Pseudobulbar palsy -> Monotonous, high pitched, slurred, donald duck speech Bulbar palsy -> indistinct (flaccid dysarthria), lacks modulation and has a nasal twang Extrapyramydal movement disorders (ie parkinsons disease) -> soft monotone speech NMJ disease (myesthinia gravis) -> slurred speech. Other lower CN palsies: - Unilateral or bilateral facial nerve palsy > slurred speech. Unable to say Puh - Wallenburg syndrome (PICA stroke)

Question 32

What is dysponia? Describe how it sounds and what causes it?

Answer 32

Dysphonia is alteration in the sound of the voice such as increased horsness and decreased volume It is due to laryngeal disease ie following a viral infection (lost my voice), or due to vocl cord pathology (recurrent laryngeal nerve palsy Fex) - Anatomical disease (Polyps, neoplasms, mass effect) - Neurological conditions (vacal cord palsy, spasmodic dysphonia, paridoxical vocal cord movement disorders - Infectious (viral laryngitis, viral URTI)

Question 33

Suspect Broccas dysphasia OR wernickes dysphasia. What to examine next?

Answer 33

Broccas in 3rd frontal gyrus of dominant (usually left) hemisphere. Wernickes in posterior temporal lobe - Usually due to left MCA stroke or neoplasm other causes inc focal infection, demyelination Need to check: - visual fields - hemineglect in MCA stroke - Gait + UL/LL for hermiparesis (hermiparetic gait, UMN weakness in UL and LL) ->pronator drift - Gerstman syndrome (due to large MCA stroke damaging the left angular gyrus or parietal lobe)

Question 34

What are the 4 features of gerstmann syndrome? How to test for each?

Answer 34

Dysgraphia/agraphia - deficiency in the ability to write - Write a short sentance for me Dyscalculia/acalculia - difficulty in learning or comprehending mathematics - What is 9x5. Please complete this calculation (written 5+7) Finger agnosia: inability to distinguish the fingers on the hand - Which finger is this (Point to index finger.) Now point to you index finger) Left-right disorientation - Take you left hand, and point to your right ear

Question 35

Cerebellar scanning speech. What to examine next and what are the main differentials?

Answer 35

What to examine next: - Nystagmus - Saccades (hypermetric or hypometric) - Pusuits (may have imparied or saccadic pursuit) - Gait (unilateral or bilateral ataxia) -> Ipsilateral cerebealar hemisphere affected - Truncal ataxia (inspect whilst sitting) -> Vermis affected - Cerebellar drift - Finger to nose (past pointing and intention tremour) - DDK (rapid alternating movements) - Heel shin testing - Toe to finger - LL DDK Localizes lesion to cerebellum. Causes of cerebellum pathology inc: - Unilateral lesion = cerebellopontine angle mass, lateral medullar syndrome - Vermis = Etoh / nutritiuonal. Central mass or cancer - Bilateral = SPA (ie friderichs ataxia), MS

Question 36

Suspect Bulbar palsy. What is the most common etiologies?

Answer 36

- MND - Syringobulbia - Infectious: Poliomyelitis, Neurosyphilus, GBS (remember this is LMD disease, not UMN)

Question 37

Suspect pseudobulbar palsy. What is the most common etiologies?

Answer 37

- Most common cause is bilateral CVA affecting the internal capsule - MS - MND

Question 38

Suspect MG speech what should you examine next?

Answer 38

Look for partial ptosis, often asymetrical. EOM Movements - look for opathalmoplegias Lid fatigue on looking up Peek sign: eyes tightly closed then look for lids to open slowly UL fatigue on arm flapping test Inspect for thymectomy scar

Question 39

What are the characteristics of MG speech?

Answer 39

Slurred speech, often monotonous and thick sounding with minimal facial expressions. - Hallmark feature is it gets progressively worse and pt may have to pause. Once pt has had a break it will be a bit better

Question 40

What do the frontal eye feilds do? What does the parietooccipitaltemporal region do?

Answer 40

THese control contralateral horizontal and vertical saccadic gaze. - ie right fronatal eye feild will make you saccade to the left The POT region controls ipsilateral smooth pursuits

Question 41

Pt has some degree of horizontal gaze palsy. How to determine if it is supranuclear vs nuclear / intranuclear / infranuclear?

Answer 41

Dolls head manouver - This utalises the verstibulo-occular reflex to make eyes move and thus avoids supranuclear inputs. - If eye remain fixed looking at you with dolls head then this is a supranuclear palsy

Question 42

What are the affected nerves in pseudobulbar palsy and how does it present?

Answer 42

Psudobulbar palsy is defined as bilateral upper motor neuro palsy of CN9,10,12 in addition to CN5 and 7 Presentation: - CN5 - Increased Jaw jerk - Spastic speech - Gag reflex increased or normal. Nil uvula dieviation but palate not able to move voluntarily well - Tongue spastic - it cannot be protruded, lies on the floor of the mouth and is small and tight - Labile emotions - UMN signs in B/l UL and LL

Question 43

What are causes of pseudobulbar palsy?

Answer 43

- Most common cause is bilateral CVA affecting the internal capsule - MS - MND

Question 44

What are the nerves affected in bulbar palsy? How does this present?

Answer 44

Bulbar palsy is bilateral LMN lesions affecting CN9,10,12. CN5 and 7 are NOT affected unlike in pseudobulbar palsy Presentation: - Gag reflex absent - Nil uvula deviation and palatal movement absent or reduced. - Jaw jerk absent - Speech is nasal and high pitched - Emotions are normal - Signs of underlying cause. Ie limb fasiculations

Question 45

Causes of bulbar palsy?

Answer 45

- MND - Syringobulbia - Infection: GBS/CIDP, poliomyelitis, subacute meningitis, neurosyphilus - Brain stem CVA

Question 46

What sensory modality does the dorsal column carry? Describe the course of the 1st, 2nd and third order neurons?

Answer 46

Vibration and proprioception. Also light touch (carried in both ST and DCML) First order from periphery to doral root ganglion second order from DRG up ipsilateral spinal cord before decusating in medulla in the medial lemiscus THird order from thalamus to primary somatoseonsroy cortex

Question 47

What modality does spinothalamic tract carry? Describe course of 1st and 2nd and t 3rd order neurons?

Answer 47

ST tract carries pain and temperature. Also light touch (carried by ST and DCML) 1st order from peripheries via dorsal root to synapse in posterior horn 2nd order from posterior horn ascends several level on ipsilateral side before decusating adn traveling remainder of way on contralateral side 3rd order from thal to primary senosry cortex

Question 48

WHat does the corticospinal tract carry? Describe the pathway course?

Answer 48

motor fibers from cortex to periphery 2 neuron - UMN and LMN UMN from primary motor cortex via internal capsule. Decusates and splits into anterior and lateral corticospinal tracts at the medullary pyramids. Once crossed, continues down to level of inervation to anterior horn LMN from anterior horn cell body travels out to periphery

Question 48

What are the two tracts of the corticospinal tract. What carries the msot fibers?

Answer 48

anterior and lateral CST - lateral carries 90% of fibers

Question 49

List the main spinal cord syndromes?

Answer 49

Brown sequard - lateral hemicord lesion Subacute combined degeneration of the spinal cord (posterolateral column syndrome) Posterior column syndrome (tabes dorsalis) Anterior cord syndrome Central cord syndrome

Question 50

Causes of brown sequard syndrome? Presentation?

Answer 50

Cause is usually trauma ie stab wound Can also be neoplastic, vascular or demyelination (MS) Presntation: - Ipsilateral UMN weakness from level of injury down - Ipsilateral loss of vibration and prop from level down - Contralateral los of pain and temperature from several level below lesion and down

Question 51

Pt presents with spinal sensory level, with loss of pain and temp contralateral and loss of vibration and proprioception ipsilaterl. Dx?

Answer 51

Brown sequard syndrome

Question 52

What are the causes of subacute combine degeneration of the spinal cord? What tracts are affected and what is teh presentation?

Answer 52

Causes: - B12 def, usually with recreational NO use -> Low intake (Alcoholism, vegan diet) -> Malnutrition/ malabsorption (coeliac disease, pernicious anaemia Causes loss of the dorsal column and corticospinal tract bilaterally Presentation: Progressive from distal to proximal. Often presents with distal parasthesia or anasthesia. When becomes more severe can affect the motor function of legs = ataxia - Gait often appears normal or mildly impaired until eyes are closed which unmasks the seonsory neural ataxia - Positive rhombergs test - Bilateral LL UMN weakness due to LCST involvement -> Normal or exaggerated knee reflexes -> nil ankle reflexes (appears like LMN sign) -> upgoing plantars - Loss of proprioception and vibration with a spinal level and down - often concurrent macrocytic anaemia and pancytopenia

Question 53

Pt presents with loss of light tough and vibration in LL bilaterally distal worse than proximal, and some increased tone in LL bilaterally. Has been unsteady on feet No change in pain or temperature. Diagnosis?

Answer 53

Possible subacute combine degeneration of the spinal cord

Question 54

What tract is affected in Tabes dorsalis? What is the cause? What is the presentation?

Answer 54

Only affects the dorsal columns bilaterally Late stage manifestation of tirtiary neurosyphilus Presentation: Similar to subacute combined degeneration of spinal cord but no weakness or UMN signs - Sensory ataixa, loss of vibration or proprioception - Associated argyl robinson pupil (light near dissasociation)

Question 55

What tract is affected in anterior cord syndrome and what is the presentation? What are the causes

Answer 55

Affects the anterior horn, LCST, ST tract Presentation: - Bilateral UMN findings from level distally - Bilateral Loss of pain and temperature from level and below - Vibration and proprioception with be preserved Causes: - Disc protrusion - ASA occlusion (ischemia) - post AAA repair

Question 56

Pt presents with bilateral loss of pain and temperature and UMN weakness. Prop and vibration intact What is Dx?

Answer 56

Anterior cord syndrome

Question 57

What is syringomyelia? How does it present?

Answer 57

Condition in which the central spinal canal in teh cervical region cystically enlarges and compresses adjacentr structures in the spinal cord Presentation: - Initially bilateral loss of pain and temperature sensation in cape distribution (posterior neck, shoulder and whole arms bilaterally) - May present with atrophy and reduced reflexes in Arms (appears like LMN signs in arms) - When progresses further may see LMN sing sin legs particularly due to compression of medial most part of bilateral LCST - Rare to get dorsal column involvement, therefore nil prop or vibration change - Often presents with scoliosis due to unequal weakness of paravertebral muscles

Question 58

Causes of syringomyelia?

Answer 58

Build up of CSF (ie a blockage that prevents CSF draining into subarachnoid space and being reabsorbed by subarachnoid granulations Usually this is a chiari malformation but anything that obstructs the medial and lateral apetures can cause (ie base of skull cancer

Question 59

Best test for syringomyelia and what does it show?

Answer 59

MRI spine and brain - Fluid filled cyst on MRI in cervical spine - May be complicating syringobulbia

Question 60

What are motor neuron diseases?

Answer 60

These are a collection of diseases that affect the motor neuron, specifically the anterior horn cell, the motor nuclei of the medulla and the descending tracts

Question 61

What is the hallmark features of MND? (what signs would make you immediate think MND)

Answer 61

It causes mixed UMN and LMN signs - this is because it affects the anterior horn cell and the motor nuclei in the medulla (LMN) as well as teh descending tract and intracrnail UMNs) Muscle wasting and atrophy Fasiculations WITHOUT SENSORY CHANGE - If have sensory change then likely not MND. Could be dual path

Question 62

What is the hallmark weakness pattern of UMN lesion?

Answer 62

Pyramidal weakness pattern. This manifests as global weakness however some muscle groups more affected than others - Upper limb extensors weaker - Lower limb flexors weaker This is what is repsonsible for the spastic positioning in hemiplegic stroke. The unopposed action of the stronger muscles pulls the limbs into the contracted position

Question 63

What are UMN signs? What are some pertinent negative signs compared to LMN lesions?

Answer 63

UMN signs: - Increased tone - Hyper-reflexia - Upgoing plantars (in the LLs) - Clonus Pertinent negatives: - Largely preserved muscle mass (atrophy will be from dissue and therefore may be present with long standing UMN lesion) - Nil prominent fasiuculations

Question 64

What are the LMN signs?

Answer 64

LMN: - decreased tone (flacid paralysis). In an elderly or middle aged person, very flacid arms is likely decreased tone. This is because the older we get the more paratonia we get - Muscle wasting (atrophy) - Prominent fasiculations at rest and on flicking - Difficult to elicit reflexes (try once or twice then re-enfornce once then move on)

Question 65

How to rate a reflex?

Answer 65

Absent + decreased but present (likely will need reinforcement) ++ - normal +++ increased/ exagerated ++++ hyper-reflexic

Question 66

What are some of the most common types of MND?

Answer 66

- Amyotrophic lateral sclerosis (ALS) is teh most common and well known - Progressive bulbar palsy - Progressive muscular atrophy - Primary lateral sclerosis

Question 67

What is the typical demographic affected by MND and what is the typical presentation?

Answer 67

Late middle aged man Insidious and progressive weakness of the muscles of the limbs, trunk and speech / face - Often first noticed in ULs with clumsiness or dropping items often - May notice slurred speech or dysarthria

Question 68

What is the treatment of MND?

Answer 68

Nothing slows disease progression or disease course. It is progressive and universally fatal There are symptomatic treatments: - Baclofen for contractures. Botox and surgery also - Anti-muscarinics for excessive salivation (due to not being able to swallow) - Benzos. Breathlessness due to anxiety

Question 69

What are the classifications of neuroapthy? What are some common causes for each?

Answer 69

Sensory - Diabetic peripheral neuropathy - B12 and thiamine Def (severe B12 def or B12 def with NO use can lead to SCD which involves motor) - Para neoplastic - Drug related Motor - AIDP/CIDP - Lead toxicity - Multifocal motor neuropathy Painful - Diabetic (Small fibers affected) - Ethanol - B6 toxicity - Cryoglobulinaemia Mixed motor and sensory - Diabetic Autonomic - Diabetic

Question 70

What is a mononueropathy?

Answer 70

This means one peripheral or cranial nerve is affected - Does not include spinal nerve roots (this is called radiculopathy)

Question 71

What is mononeuritis multiplex?

Answer 71

This is when multiple distinct peripheral or cranial nerves are affected

Question 72

What is a rediculopathy? What if multiple roots affected?

Answer 72

This is pathology affecting one or more spinal nerve roots Polyradiculopathy if multiple

Question 73

What is plexopathy?

Answer 73

This is pathology affecting a plexus (ie brachial or sacral)

Question 74

What is a glove and stocking neuropathy?

Answer 74

Pathology affects nerves based on length resulting in a glove and stocking sensory loss pattern Classically DM

Question 75

What are the two main pathological processes involved in neuropathy? How do these present respectively?

Answer 75

Axonal loss - loss of the number of nerves (axons) - Length dependent (feet affected to knees before hands are involved) - Sensory involved before motor. ie if there is subtle sensory involvement but pronounced motor involvement then it is not axonal -> LMN signs if motor involved Demyelination - demyelination of the nerves without significant loss - Large nerve fibers (motor nerves) affected first resulting in weakness before sensation - Will progress to sensory also

Question 76

What is more common: axonal polyneuropathy or demyelination polyneuropathy?

Answer 76

Axonal Demyelination is an uncommon cause of polyneuropathy. Causes inc: - AIDP/CIDP - Charcot marie tooth disease

Question 77

What are the hallmark features of charcot marie tooth disease?

Answer 77

Distal symetrical sensorimotor polyneuroapthy - Foot drop - Calf muscle masting with inverted champagne bottle appearance Pes cavus Hammer toe

Question 78

What are some pearls of information in the neuroapthy History?

Answer 78

Acute or subacute timecourse (ie started last saturday) - Immune, vasculitic, infectious or neoplastic Step wise progression (ie each week has a new neuroapthy) - THis can indicate mulitple mononeuropathies which is usually diverticulitis in aetiology) Relapsing remitting - due to intermitent exposure or CIPD Positive symptoms (shooting pains, sensation of mass etc) - more common in inherited rather than aquired

Question 79

What are the sings of a typical diabetic peripheral neuropathy?

Answer 79

Most commonly these are length predominatley sensory (but can be mixed) neuroapthies - Affect small fibers the large fibers, therefore often painful - Distal motor involvement later

Question 80

What is diabetic lumbosacral radiculoplexopathy (AKA diabetic amyotrophy)? How does it present?

Answer 80

As the name suggests this is a radiculoplexopathy that affects the femoral and lumbar areas. Usually in T2DM, well controlled (ie not associated with duration or poor control like other neuroapthies). Associated with LOW (ie new lifestyle intervention) Presentation: - Begins in one limb as a painful neuroapthy, often in the thigh or quad region - Spreads to the contralateral limb but remains very asymetrical - hyperalgesia - Then pain subsides and progresses to motor neuropathy with predominant weakness - Often femoral or sacral predoiminant (ie proiximal weakness vs foot drop distal weakness)

Question 81

What paraprotein is most associated with peripheral neropathy?

Answer 81

IgM

Question 82

Are hereditary neuroapthies more likely to cause sensory change or motor change?

Answer 82

Motor - Therefore they present late because weakness usually has to get bad enough to affect functioning for pt to present

Question 83

What are some common medications that cause peripheral neuropathy?

Answer 83

Anti infectious: - Metronidazole Chemotherapy: -cisplatinin - Tacrolimus - Vincristine - Thalidomide/bortezimib Cardiovascular: - Hydralazine - Amiodarone Other: - B6

Question 84

What is the main diagnostic information from of NCS?

Answer 84

Axonal or demyelinating

Question 85

What is the most common infection preceeding GBS? How long after infection do Sx onset?

Answer 85

Campylobactor jejuni infection, usually GI Onset usually 7-10 days, but can be variable

Question 86

What is the typical sings of AIDP?

Answer 86

Bilateral LMN signs in the legs (flaccid paralysis). Usually less so in the arms because the typical course involves progression from legs to arms - Can have sensory loss but this is variable - Cranial nerves can be affected - Can have back pain from radicular inflamation - Syphincter not affected unlike transverse myelitis

Question 87

Can MS cause LMN signs?

Answer 87

No, only UMN signs

Question 88

What are the main signs in an MS patient?

Answer 88

UMN signs (spastic paresis) - due to lesion in the brain stem or the spinal cord Posterior column sensory loss - loss of vib and prop Cerebellar signs (including ataxia, vertigo etc) Visual changes - Optic neuritis with painful EOM ROM and red desaturation - INO (rare but almost Diagnostic on MS in young pts. In elderly can be due to stroke too). Can be uni or bilateral -> need to do convergence test to see if CN 3 ok (this means its internuclear)

Question 89

Ask pt to look laterally left. Right eye cannot adduct and left eye has fast beating dissacociative nystagmus. What is teh lesion and which side is affected?

Answer 89

INO Right side medial longitudinal fasiculus

Question 90

What is the typical weakness pattern in a myopathy, and how does this differ from the typical weakness from motor neuropathy?

Answer 90

Myopathy is proximal weakness - Symmetrical - Appears like LMN lesion but nil fasiculations and relativly preserved muscle bulk - Distal myopathy is much less common, almost always genetic. Distal weakness can be due to MND, or IBM Nueropathy is usually distal rather than proximal - Not always the case ofc

Question 91

What is the hallmark weakness pattern of IBM?

Answer 91

Distal symmetrical weakness of UL bilaterally with muscle wasting Proximal weakness of legs bilaterally

Question 92

What are the causes of myopathy?

Answer 92

Inherited - structural and metabolic: - Structural - hereditary muscular dystrophy - Metabolic Inflammatory: - poly or dermatomyositis - Inclusion body myositis - Necrotising autoimmune myositis Mitochondrial disease Other: - Alcohol, Aids (HIV) - Drugs (steroids, statins, Chloroquine) - Carcinoma - Endocrine causes - Periodic paralysis - Osteomalaecia - Saracoidosis Note alcohol, hypothyroidism and connective tissue diseases cause proximal myopathy with a peripheral neuropathy too

Question 93

What are some common muscular dystrophies?

Answer 93

Dystrophica myotonica Duchennes muscular dystrophy Bekkers muscular distroipthy Limb girdle muscular dystroiphy Fasicuscapulohumeral muscular dystrophy

Question 94

What are the signs of myotonica dystrophica?

Answer 94

This has an associated syndromic appearance which in conjunction with myotonia and proximal and diustal UL and LL weakness suggests myotonica dystrophica Appearance: - Frontal balding (prominent forehead) - Expressionless face with partical ptosis of both eyes - Thin triangular face - Temperalis wasting - May have thick glassess - Thin neck due to SCM wasting Percussive myotonia - percuss over thenar eminence and look for myotonia Ask pt to clench fists to rapidly open - Will have significatn difficulty opening hands UL and LL distal and proximal weakness and atrophy without fasiculations

Question 95

What are the main signs of Dermatomyositis? What about polymyositis?

Answer 95

Skin signs: - Gottrons papules - Violaceous rash over the posterior aspects of the hands and fingers. It is not confluent, more discrette - Shawl sign - across chest and back - Heliotrope rash over face (similar to SLE) Neurological signs: - Symetrical proximal muscle weakness in UL and LL bilaterally. Not fatiguable like MG or LEMS Polymyositis is the same but with out skin changes

Question 96

Pt has symetrical distal upper limb weakness and proximal lower limb weakness. Dx?

Answer 96

IBM

Question 97

What are the signs of inclusion body myositis?

Answer 97

Syemtrical distal upper limbs and hands, and symetrical proximal LL weakness with prominent muscle wasting - Cant sit to stand - Dropping things, clumbsy, can peel fruits or use utensils

Question 98

What are the hallmark features of MG? What are the typical signs and symptoms?

Answer 98

Fatigue muscle weakness, with minimal muscle wasting and nil fasiculations, and nil sensory change Proximal muscle weakness without sensory change or muscle wasting Often concurrent eye symptoms (partial ptosis or asymetrical complex opthalmoplegias Often have slurred speech

Question 99

What are the basic tests on examination if suspect MG?

Answer 99

Sustain upgaze - positive if see progressive partial ptosis Peek test - squeeze eyes shut tightly and look for progressive parting of lids (peeking) Repeated movments in ULs - flapping arms to see decreasing amplitude Look for thymectomy scar

Question 100

What is involved in a full cerebellar examination?

Answer 100

- Nystagmus testing - horizontal or vertical - Saccades - Hypo or hypermetric saccades - Smooth pursuits - lag or saccadic pursuits - Speech - scanning speech - Arm drift - upward drift (this is dif from pronator drift) - Finger to nose test - past pointing, intention tremour - Rapid alternating movments - DDK - Rebound testing - overshoot - Heel shin testing - toe finger - Alternatign movements - Gait - Truncal ataxia

Question 101

Manifestations of unilateral cerebellar signs? What are the differentials for ulilateral cerebellar sign?

Answer 101

Ipsilateral limb ataxia manifesting as falling only to one side Differentials: - Cerebellar pontine angle mass. Need to examine CN 5,7,8 which will also be affected in this case - Lateral medullary syndrome

Question 102

How will a midline cerebellar lesion manifest and what are the causes?

Answer 102

This will manifest as truncal ataxia, abnormal heel to toe walking and abnormal speech - Central mass / cancer - Paraneoplastic syndrome

Question 103

Manifestations of bilateral cerebellar signs? What are the differentials for bilateral cerebellar sign?

Answer 103

Limb ataxia in both limbs, manifesting as falling to both sides Differentials - MS - Friedrichs ataxia -> Look for pes cavus as the best initial screening exam - Hypothyroidism (very rare)

Question 104

What are the manifestations of isolated rostral vermis affected?

Answer 104

This is present with bilateral leg only ataxia, nil upper limb signs - Alcohol classically presents like this

Question 105

What are the main signs of freiderichs ataxia?

Answer 105

Usually affects young pts, therefore if see an old pt don’t immediately thing this. It is a hereditary mitochondrial disorder If you see a young pt with bilateral cerebellar signs (ie UL and LL bilaterally) with pes cavus, the it is Friederichs ataxia. Other signs include: - Bilateral cerebellar signs - Pes Cavus - UMN signs in limbs BUT reflexes are absent - Loss of Dorsal columns (ie prop and vib loss in limbs) - Cardiomyopathy - Diabetes

Question 106

Explain the examination findings of parkinsons disease?

Answer 106

Inspection: - Mask facies - typically flexed posture with minimal spont movments and slow movements Tremor: - any tremour involving thumb is pill rolling - unilateral in early disease, bilateral but remains asymetrical in late disease worse when resting but better when performing tasks usually 5-10hz Gait: -Slow shuffling gait, hardly raises feet from ground - difficulty initiating. then fenestrating gait - lack of normal arm swing Bradykinesia: - Finger taping test - Twiddling test Tone: - Cogwheeling, superimposed tremour on increased tone Glabellar tap test - pt continue to blink with tapping of teh forehead Occular movements - Test vertical gaze to screen for SNP Writing - micrographia - progressive decreae in amplitude of writing

Question 107

Causes of parkinsonism?

Answer 107

Idiopathic: - parkinsons disease Parkinsons plus syndromes: - PSP, CBD, MSA, LBD Drugs: - methyldopa, phenothiazine Other: - Toxins, wilsons disease

Question 108

Features of PSP?

Answer 108

Main feature on examination is impaired vertical eye movements. Therefore if find parkinsonism and imparied vertical eye movments then PSP is likely differential

Question 109

Main feature of MSA?

Answer 109

○ Parkinsons subtype § Presents basically like Parkinson's disease ○ Cerebellar subtype Ataxia / apendicular ataxia