Neurology Flashcards

Question

Disease modifying treatments for Multiple Sclerosis

Answer 1

1. Interferon‐beta and Glatiramer reduce relapse rate but don’t affect progression. 2. Monoclonal antibody therapy potentially offers greater benefits; reducing disease progression and accumulated disability, e.g. ===> Alemtuzumab (anti‐CD52) – lymphocyte depletion, ===> Natalizumab (anti‐α4 integrin) – blocks T‐cell trafficking. >>>> Toxicity may limit their use.

Answer 2

1. Methyl‐prednisolone during the acute phase may shorten the duration of the ‘attack’ but does not affect the prognosis. 2. Anti‐spasmodics, e.g. Baclofen. 3. Carbamazepine (for neuropathic pain). 4. Laxatives and intermittent catheterization/oxybutynin for bowel and bladder disturbance.

Answer 3

Variable: The majority will remain ambulant at 10 years.

Answer 4

1. Reduced relapse rate during pregnancy 2. Increased risk of relapse in postpartum period 3. Safe for foetus (possibly reduced birth weight)

Answer 5

1. Arm paralysis is the impairment 2. Inability to write is the disability 3. Subsequent inability to work as an accountant is the handicap Occupational therapy aims to help minimize the disability and abolish the handicap of arm paresis.

Answer 6

Examine this patient’s limbs neurologically and then proceed to examine anything else that you feel is important.

Answer 7

• Inspection: walking aids, nasogastric tube or PEG tube, posture (flexed upper limbs and extended lower limbs), wasted or oedematous on affected side. • Tone: spastic rigidity, ‘clasp knife’ (resistance to movement, then sudden release). Ankles may demonstrate clonus (>4 beats). • Power: reduced. • Coordination: sometimes reduced. Usually impaired due to weakness but may reflect cerebellar involvement in posterior circulation stroke. • Reflexes: brisk with extensor plantars Offer to • Walk the patient if they are able to, to demonstrate the flexed posture of the upper limb and ‘tip toeing’ of the lower limb. • Test sensation (this is tricky and should be avoided if possible!). Proprioception is important for rehabilitation. Other signs • Upper motor neurone unilateral facial weakness (spares frontalis due to its dual innervation). • Gag reflex and swallow to minimize aspiration. • Visual fields and higher cortical functions, e.g. neglect helps determine a Bamford classification. • Signs of the Cause: irregular pulse (AF), blood pressure, cardiac murmurs or carotid bruits (anterior circulation stroke).

Answer 8

``` 0, none 1, flicker 2, moves with gravity neutralized 3, moves against gravity 4, reduced power against resistance 5, normal Extensors are usually weaker than flexors in the upper limbs and vice versa in the lower limbs. ```

Answer 9

• Stroke: rapid onset, focal neurological deficit due to a vascular lesion lasting > 24 hours.

Answer 10

• TIA: focal neurological deficit lasting < 24 hours.

Answer 11

1. Bloods: FBC, CRP/ESR (young CVA may be due to arteritis), glucose and renal function 2. ECG: AF or previous infarction 3. CXR: cardiomegaly or aspiration 4. CT head: infarct or bleed, territory 5. Consider echocardiogram, carotid Doppler, MRI/A/V (dissection or venous sinus thrombosis in young patient), clotting screen (thrombophilia), vasulitis screen in young CVA

Answer 12

1. Thrombolysis with tPA (within 4.5 hours of acute ischaemic stroke) 2. Clopidogrel (or aspirin + dipyridamole) 3. Referral to a specialist stroke unit: multidisciplinary approach: physiotherapy, occupational therapy, speech and language therapy and specialist stroke rehabilitation nurses 4. DVT prophylaxis

Answer 13

1. Carotid endarterectomy in patients who have made a good recovery, e.g. in PACS (if >70% stenosis of the ipsilateral internal carotid artery) 2. Anticoagulation for cardiac thromboembolism 3. Address cardiovascular risk factors 4. Nursing +/− social care.

Answer 14

1. Total anterior circulation stroke (TACS) • Hemiplegia (contra‐lateral to the lesion) • Homonomous hemianopia (contra‐lateral to the lesion) • Higher cortical dysfunction, e.g. dysphasia, dyspraxia and neglect 2. Partial anterior circulation (PACS) • 2/3 of the above 3. Lacunar (LACS) • Pure hemi‐motor or sensory loss

Answer 15

Dead 60% Dependent 35% Independent 5%

Answer 16

Dead 15% Dependent 30% Independent 55%

Answer 17

Dead 10% Dependent 30% Independent 60%

Answer 18

1. Dysphasia: receptive, expressive or global 2. Gerstmann’s syndrome ⚬⚬ Dysgraphia, dyslexia and dyscalculia ⚬⚬ L‐R disorientation ⚬⚬ Finger agnosia

Answer 19

1. Dressing and constructional apraxia | 2. Spatial neglect

Answer 20

1. Sensory and visual inattention 2. Astereognosis 3. Graphaesthesia

Answer 21

Visual field defects 1. Unilateral field defect 2. Bitemporal Hemianopia 3. Homonymous Hemianopia 4. Superior Homonymous Quadrantinopia 5. Inferior Homonymous Quadrantinopia

Answer 22

* Most common brainstem vascular syndrome * Due to occlusion of posterior inferior cerebellar artery (PICA) * Often variable in its presentation

Answer 23

1. Vestibular Nucleus 2. Inferior Cerebellar Peduncle 3. Descending Sympathetic Tract 4. CN 5 Descending Spinal Tract 5. CN 5 Spinal Nucleus 6. Spinothalamic Tract 7. Nucleus Tractus Solitarius (CN 7, 9, 10) 8. Nucleus Ambiguus (CN 9, 10)==> Specific to PICA

Answer 24

1. Cerebellar signs => Inferior cerebellar peduncle 2. Nystagmus (Present with vertigo and vomiting) => Vestibular nucleus 3. Horner syndrome => Descending sympathetic tract 4. Palatal paralysis and decreased gag reflex => Nucleus ambiguus (CN IX and X) 5. Loss of trigeminal pain and temperature sensation => Trigeminal nerve (CN V) spinal nucleus and tract

Answer 25

Loss of pain and temperature sensation => Spinothalamic tract

Answer 26

Examine this man’s lower limbs neurologically. He has had difficulty in walking.

Answer 27

1. Wheelchair and walking sticks (disuse atrophy and contractures may be present if chronic) 2. Increased tone and ankle clonus 3. Generalized weakness 4. Hyper‐reflexia and extensor plantars 5. Gait: ‘scissoring’

Answer 28

1. Examine for a sensory level suggestive of a spinal lesion 2. Look at the back for scars or spinal deformity 3. Search for features of multiple sclerosis, e.g. cerebellar signs, fundoscopy for optic atrophy 4. Ask about bladder symptoms and note the presence or absence of urinary catheter. 5. Offer to test anal tone

Answer 29

1. Multiple sclerosis 2. Spinal cord compression/cervical myelopathy 3. Trauma 4. Motor neurone disease (no sensory signs)

Answer 30

1. Anterior spinal artery thrombosis: dissociated sensory loss with preservation of dorsal columns 2. Syringomyelia: with typical upper limb signs 3. Hereditary spastic paraplegia: stiffness exceeds weakness, positive family history 4. Subacute combined degeneration of the cord: absent reflexes with upgoing plantars 5. Friedreich’s ataxia 6. Parasagittal falx meningioma

Answer 31

• Medical emergency

Answer 32

1. Disc prolapse (above L1/2) 2. Malignancy 3. Infection: abscess or TB 4. Trauma: # vertebra

Answer 33

spinal MRI

Answer 34

1. Urgent surgical decompression | 2. Consider steroids and radiotherapy (for a malignant cause)

Answer 35

- L 2/3 Hip flexion - L 3/4 Knee extension => Knee jerk L 3/4 - L 4/5 Foot dorsi‐flexion - L 5/S 1 Knee flexion & Hip extension - S 1/2 Foot plantar‐flexion => Ankle jerk S 1/2

Answer 36

Hints: L3 (knee) L4 (to the oor medially) S2, 3, 4 (keeps the faeces off the floor!)

Answer 37

This man complains of gradually increasing weakness. Please examine him neurologically.

Answer 38

1. Inspection: wasting and fasciculation 2. Tone: usually spastic but can be flaccid 3. Power: weak 4. Reflexes: absent and/or brisk. (Absent knee jerk with extensor plantar reflexes.) 5. Sensory examination is normal 6. Speech: dysarthria may be bulbar (nasal, ‘Donald Duck’ speech, due to palatal weakness) or pseudo bulbar (‘hot potato’ speech, due to a spastic tongue). 7. Tongue: wasting and fasciculation (bulbar) or a stiff spastic tongue with brisk jaw jerk (pseudo‐bulbar). 8. There is no sensory, extra‐ocular muscle, cerebellar or extra‐pyramidal involvement. Sphincter and cognitive disturbance occasionally seen.

Answer 39

1. MND is a progressive disease of unknown aetiology | 2. There is axonal degeneration of upper and lower motor neurones

Answer 40

1. Amyotrophic lateral sclerosis (50%): affecting the cortico‐spinal tracts predominantly producing spastic paraparesis or tetraparesis. 2. Progressive muscular atrophy (25%): affecting anterior horn cells predominantly producing wasting, fasciculation and weakness. Best prognosis. 3. Progressive bulbar palsy (25%): affecting lower cranial nerves and suprabulbar nuclei producing speech and swallow problems. Worst prognosis.

Answer 41

1. Clinical diagnosis 2. EMG: fasciculation 3. MRI (brain and spine): excludes the main differential diagnoses of cervical cord compression and myelopathy and brain stem lesions

Answer 42

1. Supportive, e.g. PEG feeding and NIPPV 2. Multidisciplinary approach to care 3. Riluzole (glutamate antagonist): slows disease progression by an average of 3 months but does not improve function or quality of life and is costly

Answer 43

1. Most die within 3 years of diagnosis from bronchopneumonia and respiratory failure. Some disease variants may survive longer. 2. Worst if elderly at onset, female and with bulbar involvement.

Answer 44

A. Anterior horn cell 1. MND 2. Syringomyelia 3. Cervical cord compression 4. Polio B.Brachial plexus 1. Cervical rib 2. Pancoast’s tumour 3. Trauma C. Peripheral nerve 1. Combined median and ulnar nerve lesions 2. Peripheral neuropathy D. Muscle 1. Disuse atrophy, e.g. rheumatoid arthritis

Answer 45

* Visible muscle twitching at rest * Cause: axonal loss results in the surviving axons recruiting and innervating more myofibrils than usual resulting in large motor units * Seen commonly in MND and syringomyelia

Answer 46

This man complains of a persistent tremor. Examine him neurologically.

Answer 47

* Expressionless face with an absence of spontaneous movements. * Coarse, pill‐rolling, 3–5 Hz tremor. Characteristically asymmetrical. * Bradykinesia (demonstrated by asking patient to repeatedly oppose each digit onto thumb in quick succession). * Cogwheel rigidity at wrists (enhanced by synkinesis – simultaneous movement of the other limb (tap opposite hand on knee, or wave arm up and down) ). * Gait is shuffling and festinant. Absence of arm swinging – often asymmetrical. * Speech is slow, faint and monotonous. In addition • BP looking for evidence of multisystem atrophy: Parkinsonism with postural hypotension, cerebellar and pyramidal signs. • Test vertical eye movements (up and down) for evidence of progressive supranuclear palsy. • Dementia and Parkinsonism: Lewy‐body dementia. • Ask for a medication history.

Answer 48

Parkinson’s disease (idiopathic) Parkinson plus syndromes: Multisystem atrophy (Shy–Drager) Progressive supranuclear palsy (Steele–Richardson–Olszewski) Corticobasal degeneration; unilateral Parkinsonian signs Drug‐induced, particularly phenothiazines Anoxic brain damage Post‐encephalitis MPTP toxicity (‘frozen addict syndrome’)

Answer 49

• Degeneration of the dopaminergic neurones between the substantia nigra and basal ganglia.

Answer 50

• l‐Dopa with a peripheral Dopa‐decarboxylase inhibitor, e.g. Madopar/co‐beneldopa: ⚬⚬ Problems with nausea and dyskinesia ⚬⚬ Effects wear off after a few years so generally delay treatment as long as possible ⚬⚬ End‐of‐dose effect and on/off motor fluctuation may be reduced by modified release preparations • Dopamine agonists, e.g. Pergolide: ⚬⚬ Use in younger patients: less side effects (nausea and hallucinations) and save l‐Dopa until necessary ⚬⚬ Apomorpine (also dopamine agonist) given as an SC injection or infusion; rescue therapy for patients with severe ‘off’ periods • MAO‐B inhibitor, e.g. Selegiline, inhibit the breakdown of dopamine • Anti‐cholinergics, can reduce tremor, particularly drug induced • COMT inhibitors, e.g. Entacapone, inhibit peripheral breakdown of l‐Dopa thus reducing motor fluctuations • Amantadine, increases dopamine release • Surgery; deep‐brain stimulation (to either the subthalamic nucleus or globus pallidus) helps symptoms

Answer 51

• Resting tremor: Parkinson’s disease • Postural tremor (worse with arms outstretched): ⚬⚬ Benign essential tremor (50% familial) improves with EtOH ⚬⚬ Anxiety ⚬⚬ Thyrotoxicosis ⚬⚬ Metabolic: CO2 and hepatic encephalopathy ⚬⚬ Alcohol • Intention tremor: seen in cerebellar disease

Answer 52

This man complains of progressive weakness and a change in the appearance of his legs. Please examine him neurologically.

Answer 53

* Wasting of distal lower limb muscles with preservation of the thigh muscle bulk (inverted champagne bottle appearance) * Pes cavus (seen also in Friedreich’s ataxia) * Weakness of ankle dorsi‐flexion and toe extension * Variable degree of stocking distribution sensory loss (usually mild) * Gait is high stepping (due to foot drop) and stamping (absent proprioception) * Wasting of hand muscles * Palpable lateral popliteal nerve

Answer 54

* The commonest HSMN types are I (demyelinating) and II (axonal). * Autosomal dominant inheritance (test for PMP22 mutations in HSMN I). * HSMN is also known as Charcot–Marie–Tooth disease and peroneal muscular atrophy.

Answer 55

1. Diabetes mellitus 2. Alcohol 3. Drugs, e.g. isoniazid and vincristine 4. Vitamin deficiency, e.g. B12 and B1

Answer 56

1. Guillain–Barré and botulism present acutely 2. Lead toxicity 3. Porphyria 4. HSMN

Answer 57

1. Diabetes mellitus 2. Connective tissue disease, e.g. SLE and rheumatoid arthritis 3. Vasculitis, e.g. polyarteritis nodosa and Churg –Strauss 4. Infection, e.g. HIV 5. Malignancy

Answer 58

Examine this young man’s neurological system.

Answer 59

1. Young adult, wheelchair (or ataxic gait) 2. Pes cavus 3. Bilateral cerebellar ataxia (ataxic hand shake + other arm signs, dysarthria, nystagmus) 4. Leg wasting with absent reflexes and bilateral upgoing plantars 5. Posterior column signs (loss of vibration and joint position sense)

Answer 60

1. Kyphoscoliosis 2. Optic atrophy (30%) 3. High‐arched palate 4. Sensorineural deafness (10%) 5. Listen for murmur of HOCM 6. Ask to dip urine (10% develop diabetes)

Answer 61

1. Inheritance is usually autosomal recessive 2. Onset is during teenage years 3. Survival rarely exceeds 20 years from diagnosis 4. There is an association with HOCM and a mild dementia

Answer 62

1. Friedreich’s ataxia 2. Subacute combined degeneration of the cord 3. Motor neurone disease 4. Taboparesis 5. Conus medullaris lesions 6. Combined upper and lower pathology, e.g. cervical spondylosis with peripheral neuropathy

Answer 63

Examine this patient’s cranial nerves. What is wrong?

Answer 64

1. Unilateral facial droop, absent nasolabial fold and forehead creases 2. Inability to raise the eyebrows (frontalis), screw the eyes up (orbicularis oculi) or smile (orbicularis oris) Bell’s phenomenon: eyeball rolls upwards on attempted eye closure.

Answer 65

Bell’s palsy 1. Rapid onset (1–2 days) 2. HSV‐1 has been implicated 3. Induced swelling and compression of the nerve within the facial canal causes demyelination and temporary conduction block

Answer 66

1. prednisolone commenced within 72 hours of onset improves outcomes, plus aciclovir if severe 2. Remember eye protection (artificial tears, tape eye closed at night)

Answer 67

70–80% make a full recovery; substantial minority have persistent facial weakness

Answer 68

Bell’s palsy is more common in pregnancy, and outcome may be worse

Answer 69

1. Herpes zoster (Ramsay–Hunt syndrome) 2. Mononeuropathy due to diabetes, sarcoidosis or Lyme disease 3. Tumour/trauma 4. MS/stroke

Answer 70

1. Guillain–Barré 2. Myasthenia gravis 3. Sarcoidosis 4. Bilateral Bell’s palsy 5. Lyme disease

Answer 71

Examine this patient’s cranial nerves. She has been suffering with double vision.

Answer 72

1. Bilateral ptosis (worse on sustained upward gaze) 2. Complicated bilateral extra‐ocular muscle palsies 3. Myasthenic snarl (on attempting to smile) 4. Nasal speech, palatal weakness and poor swallow (bulbar involvement) 5. Demonstrate proximal muscle weakness in the upper limbs and fatiguability. The reflexes are normal 6. Look for sternotomy scars (thymectomy) 7. State that you would like to assess respiratory muscle function (FVC)

Answer 73

Other autoimmune diseases, e.g. diabetes mellitus, rheumatoid arthritis, thyrotoxicosis, SLE and thymomas

Answer 74

Anti‐nicotinic acetylcholine receptor (anti‐AChR) antibodies affect motor end‐plate neurotransmission

Answer 75

Diagnostic tests: 1. Anti‐AChR antibodies positive in 90% of cases 2. Anti‐MuSK (muscle‐specific kinase) antibodies often positive if anti‐AChR negative 3. EMG: decremented response to a titanic train of impulses 4. Edrophonium (Tensilon) test: an acetylcholine esterase inhibitor increases the concentration of ACh at the motor end plate and hence improves the muscle weakness. Can cause heart block and even asystole. Other tests 1. CT or MRI of the mediastinum (thymoma in 10%) 2. TFTs (Grave’s present in 5%)

Answer 76

Acute • IV immunoglobulin or plasmapheresis (if severe) Chronic 1. Acetylcholine esterase inhibitor, e.g. pyridostigmine 2. Immunosuppression: steroids and azathioprine 3. Thymectomy is beneficial even if the patient does not have a thymoma (usually young females)

Answer 77

1. Diminished reflexes that become brisker after exercise 2. Lower limb girdle weakness (unlike myasthenia gravis) 3. Associated with malignancy, e.g. small‐cell lung cancer 4. Antibodies block pre‐synaptic calcium channels 5. EMG shows a ‘second wind’ phenomenon on repetitive stimulation

Answer 78

1. Myasthenia gravis 2. Graves’ disease 3. Mitochondrial cytopathies, e.g. Kearns–Sayre syndrome 4. Miller–Fisher variant of Guillain–Barré syndrome 5. Cavernous sinus pathology

Answer 79

1. Congenital 2. Senile 3. Myasthenia gravis 4. Myotonic dystrophy 5. Mitochondrial cytopathies, e.g. Kearns–Sayre syndrome 6. Bilateral Horner’s syndrome

Answer 80

This patient has had a first seizure recently. Please examine them as you wish. What is the diagnosis?

Answer 81

Skin changes 1. Facial (perinasal: butterfly distribution) adenoma sebaceum (angiofibromata) 2. Periungual fibromas (hands and feet) 3. Shagreen patch: roughened, leathery skin over the lumbar region 4. Ash leaf macules: depigmented macules on trunk (fluoresce with UV/Wood’s light) Respiratory 1. Cystic lung disease Abdominal 1. Renal enlargement caused by polycystic kidneys and/or renal angiomyolipomata 2. Transplanted kidney 3. Dialysis fistulae Eyes 1. Retinal phakomas (dense white patches) in 50% CNS 1. Mental retardation may occur 2. Seizures 3. Signs of anti‐epileptic treatment, e.g. phenytoin: gum hypertrophy and hirsuitism

Answer 82

* Autosomal dominant (TSC1 on chromosome 9, TSC2 on chromosome 16) with variable penetrance * 80% have epilepsy (majority present in childhood; but adult presentation also seen) * Cognitive defects in 50%

Answer 83

• Include 1. Renal angiomyolipomas, renal cysts and renal cell carcinoma 2. The genes for tuberous sclerosis and ADPKD are contiguous on chromosome 16, hence some mutations lead to both conditions 3. Renal failure may result from cystic disease, or parenchymal destruction by massive angiomyolipomas

Answer 84

1. Skull films: ‘railroad track’ calcification 2. CT/MRI head: tuberous masses in cerebral cortex (often calcify) 3. Echo and abdominal ultrasound: hamartomas and renal cysts

Answer 85

Previously known as EPILOIA (EPIlepsy, LOw Intelligence, Adenoma sebaceum)

Answer 86

``` HAMARTOMAS: Hamartomas in CNS and skin; Angiofibromas ; Mitral regurgitation; Ash-leaf spots; cardiac Rhabdomyoma; (Tuberous sclerosis); autosomal dOminant; Mental retardation (intellectual disability); renal Angiomyolipoma; Seizures, Shagreen patches. ``` incidence of subependymal astrocytomas and ungual fibromas.

Answer 87

Examine this patient’s skin.

Answer 88

1. Cutaneous neurofibromas: two or more 2. Café au lait patches: six or more, >15 mm diameter in adults 3. Axillary freckling 4. Lisch nodules: melanocytic hamartomas of the iris 5. Blood pressure: hypertension (associated with renal artery stenosis and phaeochromocytoma) 6. Examine the chest: fine crackles (honeycomb lung and fibrosis) 7. Neuropathy with enlarged palpable nerves 8. Visual acuity: optic glioma/compression

Answer 89

* Inheritance is autosomal dominant * Type I (chromosome 17) is the classical peripheral form * Type II (chromosome 22) is central and presents with bilateral acoustic neuromas and sensi‐neural deafness rather than skin lesions

Answer 90

1. Phaeochromocytoma (2%) | 2. Renal artery stenosis (2%)

Answer 91

1. Epilepsy 2. Sarcomatous change (5%) 3. Scoliosis (5%) 4. Mental retardation (10%)

Answer 92

1. Neurofibromatosis 2. Leprosy 3. Amyloidosis 4. Acromegaly 5. Refsum’s disease

Answer 93

``` Examine this patient’s eyes. Horner’s pupil Holmes–Adie (myotonic) pupil Argyll Robertson pupil Oculomotor (III) nerve palsy ```

Answer 94

'PEAS' 1. Ptosis (levator palpebrae is partially supplied by sympathetic fibres) 2. Enophthalmos (sunken eye) 3. Anhydrosis (sympathetic fibres control sweating) 4. Small pupil (miosis) May also have flushed/warm skin ipsilaterally to the Horner's pupil due to loss of vasomotor sympathetic tone to the face. Extra points • Look at the ipsilateral side of the neck for 1. Scars (trauma, e.g. central lines, carotid endarterectomy surgery or aneurysms) and 2. Tumours (Pancoast’s).

Answer 95

Brain stem >>> MS & Stroke (Wallenberg’s) Spinal cord >>> Syrinx Neck >>> Aneurysm & Trauma Pancoast’s

Answer 96

Moderately dilated pupil that has a poor response to light and a sluggish response to accommodation (you may have to wait!) Extra points • Absent or diminished ankle and knee jerks

Answer 97

A benign condition that is more common in females. Reassure the patient that nothing is wrong.

Answer 98

Small irregular pupil Accommodates but doesn't react to light Atrophied and depigmented iris Extra points • Offer to look for sensory ataxia (tabes dorsalis)

Answer 99

1. Usually a manifestation of quaternary syphilis, but it may also be caused by diabetes mellitus 2. Test for quaternary syphilis using TPHA or FTA, which remain positive for the duration of the illness 3. Treat with penicillin

Answer 100

Ptosis usually complete Dilated pupil The eye points 'down and out' due to the unopposed action of lateral III nerve palsy rectus (VI) and superior oblique (IV) Test for the trochlear (IV) nerve On looking nasally the eye will intort (rotate towards the nose) indicating that the trochlear nerve is working Extra points • If the pupil is normal consider medical causes of III palsy • Surgical causes often impinge on the superficially located papillary fibres running in the III nerve

Answer 101

1. Mononeuritis multiplex, e.g. DM 2. Midbrain infarction: Weber’s 3. Midbrain demyelination (MS) 4. Migraine

Answer 102

1. Communicating artery aneurysm (posterior) 2. Cavernous sinus pathology: thrombosis, tumour or fistula (IV, V and VI may also be affected) 3. Cerebral uncus herniation

Answer 103

Examine this woman’s eyes.

Answer 104

• Relative afferent pupillary defect (RAPD): dilatation of the pupil on moving the light source from the normal eye (consensual reflex) to the abnormal eye (direct reflex): Swinging light test >>> Marcus- Gunn pupil • Fundoscopy: disc pallor Extra points Look for the cause.

Answer 105

1. Glaucoma (cupping of the disc) 2. Retinitis pigmentosa 3. Central retinal artery occlusion 4. Frontal brain tumour: Foster–Kennedy syndrome (papilloedema in one eye due to raised intercranial pressure and optic atrophy in the other due to direct compression by the tumour)

Answer 106

1. Cerebellar signs, e.g. nystagmus: >>> multiple sclerosis (internuclear ophthalmoplegia), >>> Friedreich’s ataxia (scoliosis and pes cavus) 2. Large bossed skull: Paget’s disease (hearing aid) 3. Argyll–Robertson pupil: Tertiary syphilis

Answer 107

PALE DISCS 1. Pressure*: tumour, glaucoma and Paget’s 2. Ataxia: Friedreich’s ataxia 3. LEber's optic atrophy 4. Dietary: ↓B12, 5. Degenerative: retinitis pigmentosa 6. Ischaemia: central retinal artery occlusion 7. Syphilis and other infections, e.g. CMV and toxoplasmosis 8. Cyanide and other toxins, e.g. alcohol, lead and tobacco 9. Sclerosis*: MS (* denotes commonest cause)

Answer 108

Examine this elderly patient’s fundi. She complains of recent loss of vision.

Answer 109

a. Wet (neovascular and exudative) or dry (non neovascular, atrophic and non‐exudative) b. Macular changes: 1. Drusen (extracellular material) 2. Geographic atrophy 3. Fibrosis 4. Neovascularization (wet)

Answer 110

1. Age, 2. white race, 3. family history and 4. smoking • Wet AMD have a higher incidence of coronary heart disease and stroke

Answer 111

1. Ophthalmology referral 2. Wet AMD may be treated by intravitreal injections of anti‐VEGF (though can increase cerebrovascular and cardiovascular risk)

Answer 112

• Majority of patients progress to blindness in the affected eye within 2 years of diagnosis

Answer 113

This man has been complaining of difficulty seeing at night. Please examine his eyes.

Answer 114

1. White stick and braille book (registered blind) 2. Reduced peripheral field of vision (tunnel vision) 3. Fundoscopy a. Peripheral retina 'bone spicule pigmentation', which follows the veins and spares the macula. b. Optic atrophy due to neuronal loss (consecutive). Association: cataract (absent red reflex).

Answer 115

1. Ataxic: Friedreich’s ataxia, abetalipoproteinaemia, Refsum’s disease, Kearns–Sayre syndrome 2. Deafness (hearing‐aid/white stick with red stripes): Refsum’s disease, Kearns– Sayre syndrome, Usher’s disease 3. Ophthalmoplegia/ptosis and permanent pacemaker: Kearns–Sayre syndrome 4. Polydactyly: Laurence–Moon–Biedl syndrome 5. Icthyosis: Refsum’s disease

Answer 116

>> Inherited form of retinal degeneration characterized by loss of photo receptors

Answer 117

a. Congenital: often autosomal recessive inheritance, 15% due to rhodopsin pigment mutations b. Acquired: post‐inflammatory retinitis

Answer 118

1. Progressive loss of vision due to retinal degeneration. Begins with reduced night vision. Most are registered blind at 40 years, with central visual loss in the seventh decade 2. No treatment although vitamin A may slow disease progression

Answer 119

1. Papilloedema 2. Glaucoma 3. Choroidoretinitis 4. Migraine 5. Hysteria

Answer 120

Examine this man’s fundi.

Answer 121

1. Pale, milky fundus with thread‐like arterioles 2. ± Cherry red macula (choroidal blood supply) 3. Cause: AF (irregular pulse) or carotid stenosis (bruit) 4. Effect: optic atrophy and blind (white stick) Note that branch retinal artery occlusion will have a field defect opposite to the quadrant of affected retina

Answer 122

1. Embolic: carotid plaque rupture or cardiac mural thrombus >> Treatment: aspirin, anti‐coagulation and endarterectomy 2. Giant cell arteritis: tender scalp and pulseless temporal arteries >> Treatment: high‐dose steroid urgently, check ESR and arrange temporal artery biopsy to confirm diagnosis

Answer 123

Examine this patient’s fundi.

Answer 124

1. Flame haemorrhages +++ radiating out from a swollen disc 2. Engorged tortuous veins 3. Cotton wool spots 4. Cause: look for diabetic or hypertensive changes (visible in branch retinal vein occlusion). 5. Effect: Rubeosis iridis causes secondary glaucoma (in central retinal vein occlusion), visual loss or field defect.

Answer 125

1. Hypertension 2. Hyperglycaemia: diabetes mellitus 3. Hyperviscocity: Waldenström’s macroglobulinaemia or myeloma 4. High intraocular pressure: glaucoma