Neurology

Question 1

Parkinson’s disease definition

Answer 1

Neurodegenerative disease caused by the disruption of dopaminergic neurotransmission in the basal ganglia. Histological loss of melanin containing dopaminergic neurones in the substantia nigra and cytoplasmic inclusions known as lewy bodiers in surviving neurones. 60-80% neurone loss before clinical symptoms.

Question 2

Dementia in parkinsons disease

Answer 2

Late, 15-30% patients. Earlier occurance of cognitive symptoms (i.e. within 1 year of motor symptoms may suggest lewy body dementia)

Question 3

Parkinson’s vs Parkinsonism

Answer 3

Parkinson’s asymmetrical distribution of signs, good reponse to levodopa, absent parkinsonian plus signs, progressive progression.
Parkinsonism more symmetrical, poor response to levodopa, may have addditional signs to suggest PP syndromes

Question 4

Parkinsonian plus syndromes

Answer 4

MSA
PSP
CBD

Question 5

MSA

Answer 5

Parkinsonism, autonomic failure, cerebellar dysfunction and pyramidal signs.
Progressive loss of neuronal/oligodendronal cells at numerous sites in CNS, with relative sparing of globus pallidus, caudate nucleus, CST and anterior horn cells.

Question 6

PSP

Answer 6

Most common PP+. Multiple neurotransmitter pathways (cholinergic, adrenergic, dopaminergic).
Abnormal vertical then horizontal saccades. Difficulty opening eyes, early and prominent falls, speach/swallow, frontal symptoms of depression, apathy, cognitive impairment.
Symmetrical parkinsonian features.

Question 7

CBD

Answer 7

Frontoparietal cortical atrophy + extrapyramidal degeneration. 
Limb aprexia (&alien limb syndrome) + cortical sensory loss.

Question 8

What is parkinsonism?

Answer 8

Movement disorder characterised by bradykinesia and at least one of rigidity, tremor or postural instability

Question 9

Carpel tunnel syndrome

Answer 9

Look for: weakness of flexion, abduction and opposition of thumb. Decreased sensation over lateral three and a half fingers. Tinel’s sign (tingling in median nerve distribution on percussion of median nerve). Phalen’s sign (hyperextension of wrist for 1 minute reproduces symptoms. Look for a scar of previous median nerve decompression.
Associations: Acromegaly, myxoedema, rheumatoid arthritis, occupational trauma, pregnancy.

Question 10

Chorea

Answer 10

Sydenham’s chorea, Huntington’s disease, polycythemia vera, chorea gravidarum, drug induced, systemic disease (SLE, thyrotoxicosis, etc). If hemichorea, consider infarction or tumour.

Question 11

Ankylosing spondylitis

Answer 11

Features shown: ‘Question mark’ posture due to loss of lumbar lordosis, fixed kyphoscoliosis of thoracic spine and hyperextension at cervical spine and some protuberance of the abdomen.
Look for: whole body turning when patient tries to rotate neck, inability of the occiput to make contact with the wall when the heels and back are against the wall (occiput-to-wall distance), limited spinal movement (increased finger floor distance and Schober’s test), reduced chest expansion, eyes (iritis and anterior uveitis), aortic regurgitation and apical lung fibrosis.

Question 12

Homonymous hemianopia

Answer 12

Lesion behind the optic chiasm.

Question 13

Bitemporal hemianopia

Answer 13

Lesion at the optic chiasm
DDx pituitary tumour, craniopharyngioma, suprasellar meningioma, glioma, metastasis
Pituitary tumours compress from below (upper temporal first), craniopharyngiomas compress from above (lowe temporal field first)
Look for: acromegaly, cushings, hypopituitarism, gynaecomastia, sarcoid, tuberculosis, malignancy

Question 14

Homonymous upper quadrantopia

Answer 14

Temporal cortex

Question 15

Homonymous lower quadranopia

Answer 15

Parietal cortex

PITS

Question 16

What do you understand by homonymous

Answer 16

Identical pattern of visual field defects in each eye

Question 17

Kennedy-Foster syndrome

Answer 17

Ipsilateral optic atrophy 2* to compression of optic nerve

Contralateral paplilloedema secondary to raised ICP

Question 18

Myotonic Dystrophy Examination

Answer 18

Face - myopathic pacies, thin expressionless, wasting of facial and SCN muscles, bilateral ptosis, frontal balding, dysarthria from myotonia of tongue and pharynx
Hands - myotonia (grip my hand, not let go), wasting and weakness distally with areflexia
Percussion myotonia - percus thenar eminence and watch for involuntary thumb movement
Additional - catracts, CM, PPM from brady/tachy arrythmias, diabetes, testicular atrophy, dysphagea

Question 19

Myotonic dystrophy genetics

Answer 19

AD, with genetic anticipation
DM1 - CTG trinucleotide repeat DMPK gene Chr19
DM2 - CCTG trinucleotide repeat within ZNF9 gene on chr 3
DM1 in 20s and 30s, DM2 later (but variable)

Question 20

Diagnosing myotonic dystrophy

Answer 20

Clinical examination
EMG - dive bomber potentials
Genetic testing

Question 21

Management of myotonic dystrophy

Answer 21

Respiratory and cardiac complications leading causes of mortality
Weakness - no treatment
Phenytoin may help myotonia
High risk of complications from anaesthesia

Question 22

DDx ptosis

Answer 22

Bilateral - MG, myotonic dystrophy, congenital

Unilateral - 3rd nerve palsy, horners syndrome

Question 23

Interpreting cerebellar lesion

Answer 23

Ipsilateral cerebellar signs in the limbs

Nystagmus - maximal fast paced direction towards (when looking at) the lesions

But** in vestibular/VIII lesions maximum away from the lesion

Question 24

Causes of cerebellar syndrome

Answer 24

PASTRIES
Paraneoplastic 
Alcoholic 
Sclerosis (MS)
Tumour
Rare (Freidrich, ataxia telangectasia)
Iatrogenic (phenytoin)
Endocrine (hypothyroidism)
Stroke

Question 25

Investigating MS

Answer 25

GCS - oligoclonal bands MRI - periventricular white matter changes Visual Evoked Potentials - dleayed velocity but normal amplitude to suggest optic neuritis

Question 26

MS treatment

Answer 26

MDT - nurses, PT, OT, social worker, physician Treatments: INF-b reduces relapse rate but does not affect progression. mAb - natalizumab (anti a4 integrin, blocks T cell trafficking) and alemtuzumab (anti-CD-52, lymphocyte depletion) Symptomatic - methylprednisolone will shorten duration of attack Anti-spasmodics like baclofen Neuropathic pain - carbamazepine Bladder and bowel regimes

Question 27

Pyramidal weakness

Answer 27

Extensors weaker than flexors in upper limbs | Flexors weaker than extensore in lower limbs

Question 28

Define stroke and TIA

Answer 28

Stroke - rapid onset focal neurological deficit secondary to vascular lesion lasting >24 hours TIA - rapid onset focal neurological deficit secondary to vascular lesion lasting <24 hours

Question 29

Bamford classification of stroke

Answer 29

TACS - HHH (hemianopia, higher dysfunction, hemiplegia) PACS - 2/3 TACS LACS - pure hemi motor or sensory loss, ataxic hemiplegia POCS - Cranial nerve palsy and a contralateral motor/sensory deficit Bilateral motor/sensory deficit Conjugate eye movement disorder (e.g. horizontal gaze palsy) Cerebellar dysfunction (e.g. vertigo, nystagmus, ataxia) Isolated homonymous hemianopia

Question 30

Lateral Medullary Syndrome (Wallenberg)

Answer 30

PICA occlusion Ipsilateral cerebellar, horner, palatal paralysis, loss of V sensation Contralateral loss of pain and temperature sensation

Question 31

Subacute combined degeneration of the cord

Answer 31

Absent reflexes with upgoing plantars

Question 32

Spastic legs - examination

Answer 32

Observation - wheelchair, sticks, disuse atrophy and contractures Increased tone, ankle clonus Weakness Hyperreflexia and extensor plantats Scissoring gait Other - look at back for surgical scars, syringomyelia (upper limb signs), anterior spinal artery thrombosis, hereditary spastic parapalegia (+ve FH), SCDC, Friedrich ataxia, cord compression

Question 33

Cord compression

Answer 33

``` Disc prolapse above L2/L1 Malignancy Traumatic Infection - abscess of TB investigation - spinal MRI Management - surgical decompression, streroids / Rx for malignant choice ```

Question 34

Examining syringomyelia

Answer 34

Weakness/wasting small musles of hand, areflexia, dissociated sensory loss in upper limbs and chest, loss of pain (STT) but preserved vibration and proprioception (dorsal column) Scars from burns Charcot joints Pyramidal weakness in lower limbs with upgoing plantars Kyphscoliosis and horners If syrinx extends into brainstem (syringobulbia) may be cerebellar and lower cranial signs

Question 35

What is syringomyelia

Answer 35

Progressively expanding fluid filled cavity (syrinx) within cervical cord, typically spanning several levels Expands ventrally: 1. STT = pain and loss of temp at level of syrinx 2. Anterior horn cells = segmental LMN weakness at level 3. CST = UMN weakenss below level of syrinx 4. Dorsal columns spared (intact proprioception and vibration sense) Signs may be asymmetrical MRI scan

Question 36

What are charcot joints

Answer 36

``` Painless deformity and destruction of a joint with new bone formation following repeated minor trauma secondary to loss of pain sensation Tabes dorsalis - hip and knee Diabetes - foot and ankle Syringomyelia - elbow and shoulder Bisphosphonates can help ```

Question 37

MND on examination

Answer 37

``` Inspection: Wasting and fascilculations Tone: spastic / flaccid Power: reduced Reflexes: absent or brisk Sensory: normal Speech: dysarthria (bulbar - donald duck, nasal, palatal weakness); or pseudobulbar (hot potato, spastic tongue) Tongue: wasting and fasciculations No sensory, extra-ocular, cerebellar, or extramyramidal involement ```

Question 38

MND definition and investigation

Answer 38

Progressive neurodgenerative disease of unknown aetiology resulting in axonal degeneration of upper and lower motor neurones. 3 main subtypes: 1. Amyotrophic Lateral Sclerosis (50%, ALS) - CST predominant = spastic paraparesis or tetraparesis 2. Progressive muscular atrophy (25%) = Anterior horn cells prdominantly = wasting fasciculations and weakness, best prognosis 3. Progressive bulbar palsy (25%) = lower cranial nerves and suprabulbar nuclei, speech and swallow problems, worst prognosis

Question 39

MND investigation treatment and prognosticaiton

Answer 39

Clinical diagnosis, EMG, MRI brain/spine to r/u cervical cord compression, myelopathy and brainstam lesions Supportive: PEG feeding and NIPPV MDT Rilozule - slow disease progression 3/12 but not affecting function or QoL Most die within 3 years, usually resp complications Wost if elderly, female and bulbar onser

Question 40

DDx wasting of hand muscles

Answer 40

Anterior horn cell - MND, syringomyelia, cervical cord compression, polio Brachial plexus - cervical rib, pancoast tumour, trauma Peripheral nerve - combined medial & ulnar lesions Muscle - disure atrophy (e.g. RA)

Question 41

What is a fasciculation?

Answer 41

Visible muscle twitching at rest Due to axonal loss results in surviving neurones recruiting and innervaintg more myofibrils than ususal resulting in large motor units Commonly in MND or syringomyelia

Question 42

Treating parkinsons disease

Answer 42

levodopa with peripheral dopa-decarboxylase inhibitor (co-careldopa, co-beneldopa). SE of nausea nad dyskinesia. Effects wear off after few years so best to pick moment. on/off motor fluctuations can be decreases by MR preperations Dopamine agonists - younger patients, less SE, saves L dopa until necessary. Apomorphine also agonist which can be used as S/C or infusion for pts with severe off periods MAO-B inhibitors (selegiline) inhibit breakdown of dopamine Anticholiergics - reduced tremor COMT inhibitors (e.g. entercapone) - inhibit peripheral breakdown of L dopa thus reducing motor flucutations Amantadine - increases dopamine release Surgery / DBS

Question 43

Causes of tremor

Answer 43

Resting - PD Postural - benign essential (50% familial, improves with etoh) Anxiety, thyrotoxicosis, CO2 and HE, alcohli intention - cerebellar disease

Question 44

Hereditary sensory motor neuropathy - examination

Answer 44

Charcot-Marie-Tooth disease Wasting distal lower limb muscle with preservation of thigh bulk (inverted champagne appearance), pes cavus, weakness ankle dorsiflexion and toe extension Variable degree stocking distribution of sensory loss (usuallly mild) High stepping gait (foot drop) + stamping (loss of proprioception) Wasting hand muscles Palpable lateral popliteal nerve

Question 45

Genetics of CMT

Answer 45

``` Hereditary sensory motor neuropathy I (demyelinating) II (axonal) AD - PMP 22 in type 1 Also known as peroneal muscular atrophy ```

Question 46

Causes of peripheral neuropathy

Answer 46

Sensory --> DM, Alcohol, Drugs (isoniazid, vincristine), Vitamin B1 b12 Motor --> Guillain Barre, lead, porphyria, HSMN Mononeuritis multiplex --> DM, CTD (SLE, RA), vasculitis (PAN, CS), malignancy

Question 47

Friedrich Ataxia - examination

Answer 47

Young Adult, wheelchair Pes cavus Bilateral cerebellar ataxia Leg wasting with absent reflexes and bilateral upgoing plantars Posterior column signs (loss of vibration and propriocepiton) ``` Kyphosclolisos Optic atrophy High arched palate SN deafness HOCM DM ```

Question 48

Friedrich ataxia - genetics

Answer 48

AR ONset teenage Survival 20 years from diagnosis Association with HOCM and DM

Question 49

Extensor plantars + absent knee jerks

Answer 49

``` Friedrich ataxia SCDC MND Taboparesis Conus medullaris lesion Combined lower and upper pathology (e.g. cervical spondylosis with peripheral neuropahy ```

Question 50

Facial nerve palsy

Answer 50

U/L facial droop, involvement of forehead VI+VII palsy - pons (MS/Stroke) V+VI+VIII+cerebellar - cerebropontine lesion VII + VIII - auditory/facial canal Scars/parotic mass - tumour / trauma

Question 51

Commonest VII palsy

Answer 51

Bells - LMN Rapid onset 1-2 days, HSV implicated Swelling and compression within facial canal causes demyelination and temporary condution block Prednisolone if started within 72h improves outcomes, acyclovir EYE proteciton (tape, viscotears) 70-80% full recovery, remainder varying degrees of weakenss More common in pregnancy Other - VZV (ramsay hunt), mononeuropathy (DM, sarcoid, lyme), tumour/trauma, MS

Question 52

Bilateral facial palsy

Answer 52

``` Guillain barre Sarcoidosis Lyme disease MG B/L bells palsy ```

Question 53

Examining myaesthenia gravis

Answer 53

Bilateral ptosis worse on sustained upwards gaze Complex bilateral extraocular palsies Myaesthenic snarl Bulbar - nasal speech, palatal weakness, poor swallow Fatiguability Sternotomy (thymectomy) FVC

Question 54

Investigating myaesthenia gravis

Answer 54

Anti-AChR +ve in 90% Anti-MuSK (muscle specific kinase) if Anti-AChr -ve EMG - decremented response Tensilon tests - an acetylcholine esterase inhibitor increases ACH at motor endplate to improve weakness (risks heart block or asystole) CT - thymoma TFTs (graves in 5%

Question 55

Treating myaesthenia gravis

Answer 55

Acute - IVIg or plasmapheresis is severe Chronic - acetylcholine esterase inhibitors (pyridostigmine) Immunusupression - steroids, azathiopine Thymectomy - beneficial even if no thymoma LEMS - diminished reflexes become brisker after exercise, lower limb girdle weakenss, SCLC association, Ab block voltage gated calcium channels, EMG

Question 56

Causes of bilateral extra-ocular palsies

Answer 56

``` MG Graves disease Mitochondrial disease (e.g. Kearns-Sayre) Miller Fisher Cavernous sinus pathology ```

Question 57

Cause of bilateral ptosis

Answer 57

``` Congenital Senile MG Myotonic Dystrophy Bilateral Horners ```

Question 58

Horners syndrome

Answer 58

Ptosis, miosis, anhydrosis, enothalmos Look ipsilateral side of neck for scars - trauma, central lines, carotid endarterectomy, aneurysms, pancoast tumour Brainstem - MS, stroke Spinal cord - syrinx Neck - aneurysm, Pancoast tumour

Question 59

Holmes Adie pupil

Answer 59

Myotonic pupil Moderately dilated pupil with poor light response and a sluggish reaction to accomodation Absent or diminished ankle and knee jerks Benign condition

Question 60

III palsy

Answer 60

Down and out Ptosis (usually complete) Pilated pupil Medical - pupil normal - Mononeutritis multiplex (DM), midbrain infarct (webers) or demyelination, migraine) Surgical - pupil blown - posterior communicating artery aneurysm, cavernous sinus thrombosis, tumour or fistula, cerebral uncal herniation

Question 61

Compression of the cord

Answer 61

Myelopathy

Question 62

Compression of the nerve roots

Answer 62

Radiculopathy

Question 63

Ddx myelopathy

Answer 63

Sudden - vascular (spinal infarcts), disc prolapse | Inflammatory - MS, transverse myelitis

Question 64

Transverse myelitis

Answer 64

Immune - MS, post infectious, paraneoplastic Viral - VZV, EBV, CMV, HSV Bacterial - TB

Question 65

Assessing double vision

Answer 65

Does the double vision disappear when covering either eye = biocular diplopia Is the double vision horizontal or vertical? Horizontal diplopia: lateral / medial rectus (unilateral / bilateral VI or INO) Vertical diplopia: superior / inferior rectus, superior / inferior oblique (III or IV) Is the double vision worse on looking in any particular direction? Diplopia is maximal in the direction of action of the paretic muscle

Question 66

Surgical Sieve

Answer 66

INVITED MD ``` Infection Neoplasia Vascular Inflammatory/autoimmune Trauma Endocrine Degenerative Metabolic Drugs Congenital ```

Question 67

Sturge Weber Syndrome

Answer 67

Port wine stain - classically V1 and v2 facial distribution Leptomeningioma Seizures Glaucoma