MRCP PACES MANUAL

Louise Pealing MA Hons (Cantab) MBBS MSc MRCP MRCGP General Practitioner and Clinical Research Fellow, Nuffield Department of Primary Care Health Sciences, University of Oxford

Benjamin Mullish MB BChir MA (Cantab) MRCPAFHEA Specialty Registrar/Academic Clinical Fellow, and , St Mary’s Hospital, London

Philip J Smith BMedSci (Hons) BMBS (Hons) MRCP MSc (Nutrition) Gastroenterology Specialist Registrar and MRC Clinical Research Training Fellow Gastroenterology Department, University College London Hospital, London

Edited by Douglas C Macdonald BM (Hons) BSc (Hons) MRCP PhD Consultant Hepatologist, Royal Free London NHS Foundation Trust Royal Free Hospital, London Contents

Preface vi

Introduction vii

STATION 1 ^ Respiratory and Abdominal Examinations 1 The abdominal examination 3 Abdominal scenarios 7 The respiratory examination 47 Respiratory scenarios 52

STATION 2 ^ History-Taking Examination 71 History-taking scenarios 77

STATION 3 ^ Cardiovascular and Neurological Examinations 169 The cardiovascular examination 170 Cardiovascular scenarios 175 The neurological examination 220 Neurological scenarios 241

STATION 4 ^ Communication skills and Ethics’ Examination 353 Approach to the communication skills and ethics station 355 Communication skills and ethics scenarios 359

STATION 5 ^ Integrated Clinical Assessment 393 Approach to Station 5 398 Integrated clinical assessment scenarios 407

Abbreviations 599

Index 605

v Station 3 Neurological scenarios

1. 17. Median palsy 2. Parkinson’s disease 18. palsy 3. Motor neuron disease 19. palsy 4. Hemiparesis 20. Common peroneal nerve 5. Spastic paraparesis palsy and L4–5 root lesions 6. Cervical myelopathy 21. Nystagmus 7. / 22. Ophthalmoplegia 8. Myotonic dystrophy 23. Visual field defect 9. 24. Dysphasia 10. Muscular dystrophy 25. Bulbar and pseudobulbar 11. Charcot–Marie–Tooth palsy disease 26. Brain-stem syndromes 12. Friedreich’s ataxia 27. Cerebellopontine angle 13. Cerebellar syndrome lesion 14. 28. 15. palsy 29. Old 16. Wasting of the small 30. Involuntary movements and muscles of the hand Huntington’s disease 306 MRCP PACES Manual

SCENARIO 17. PALSY

Identifying clinical signs profundus I and II and flexor digitorum super- ficialis, and unopposed action of the radial The median nerve serves the intrinsic small mus- nerve-innervated finger extensors. Some call this cles of the hand not served by the ulnar nerve. the ‘papal sign’. Look for surgical or traumatic The median nerve also serves muscles of the scars at the wrist, palmar crease, or volar surface of the forearm, which includes the elbow. main wrist flexors. Motor function: there is weak thumb abduction The most common appearing (abductor pollicis brevis), tested for by asking the in the PACES examination is a patient to place the hand palm up and point the syndrome. Indeed this is the most common per- thumb up to the sky perpendicular to the plane ipheral mononeuropathy. However, there can be of the palm and test against resistance of your more proximal lesions of the median nerve that thumb. There is weakness of thumb opposition , offer more objective clinical signs. tested for by asking the patient to oppose the thumb with the little finger and stop you pulling Distal median nerve lesions (carpal them apart. There may be normal flexion at the tunnel syndrome) thumb MCP joint due to intact ulnar nerve in- Inspection: the hand shows thenar wasting (a nervation of flexor pollicis brevis. There is nor- late sign) with hypothenar and first dorsal inter- mal flexion of the thumb at the IP joint due to osseous sparing. The thumb is externally rotated intact innervation of flexor pollicis longus in the and adducted into the plain of the palm (Figure forearm. Arm pronation and wrist flexion are 3.18) due to weakness of opponens pollicis and also normal if the median nerve lesion is distal. abductor pollicis, and some refer to this as ‘ape- hand’ deformity (but it is best to describe the Sensory loss : there is sensory loss in the palmar position of the thumb). The index (first) and aspect of the thumb and lateral two and a half middle (second) fingers may also be held in fingers but normal sensation over the thenar extension due to weakness of flexor digitorum eminence (supplied by the palmar cutaneous branch of the median nerve which does not pass through the carpal tunnel).

Additional tests: you can ask to perform Tinel’s test – tapping over the median nerve at the wrist may cause paraesthesia in the distal median dis- tribution. Phalen’s test involves flexing the wrist to 90 8 for at least 60 seconds, which may cause paraesthesia in the distal median distribution. The median nerve compression test involves pressing on the palmar aspect of the wrist for up to 60 seconds, which may cause paraesthesia in the distal median distribution.

Figure 3.17 Median nerve cutaneous dis- Additional signs tribution Examine for signs of other diseases associated with such as: The Cardiovascular and Neurological Examinations 307

Median nerve

Pronator teres Flexor carpi radialis Palmaris longus Anterior interosseous nerve Flexor digitorum profundus I & II Flexor digitorum Flexor pollicis longus superficialis Pronator quadratus Abductor pollicis brevis

Flexor pollicis brevis Opponens pollicis Second lumbrical First lumbrical

Figure 3.18 Route and muscles innervated by the median nerve

• Hypothyroidism: goitre, slowly relaxing Anterior interosseous nerve palsy reflexes, pretibial myxoedema This nerve has branches to flexor digitorum pro- • Acromegaly: supraorbital ridge, prognathism, fundus I and II, flexor pollicis longus and prona- interdental separation, bitemporal tor quadratus. It is typically affected by a hemianopia, large doughy hands, midshaft fracture of the radius, excessive exercise hypertension or penetrating injuries of the forearm. There is • Rheumatoid arthritis : deforming arthropathy, weakness of the thumb and index finger flexion , elbow nodules, steroid skin changes, best shown with the ‘okay’ sign, ie flattened due episcleritis/scleritis, interstitial lung disease to failure of distal flexion (see Figure 3.17). The • mellitus: finger-prick testing, muscles are spared. There is no peripheral neuropathy, retinopathy sensory loss . • Gout: deforming arthropathy and gouty tophi on hands, ears and feet. Di¡erential diagnosis Proximal median nerve lesions Pronator syndrome: compression of the median • Elbow lesions: nerve can occur as it passes between the two • Fracture: supracondylar fractures are most heads of the pronator teres, high in the volar common aspect of the forearm. It can present with purely • Dislocation sensory symptoms of over the volar surface • Compression: ligament of Struthers of the forearm at rest or with forearm pronation. • Forearm lesions: There will be sensory loss within the median • Fracture: midshaft radial fracture causing distribution including the thenar eminence (un- anterior osseous nerve palsy like carpal tunnel syndrome). • Injury: penetrating injuries of the forearm 308 MRCP PACES Manual

• Compression: pothyroidism, diabetes, acromegaly, rheumatoid • Wrist lesions: arthritis, gout, renal failure, multiple myeloma • Fracture/trauma and amyloidosis. • Carpal tunnel syndrome (CTS). Investigations Electrophysiology: nerve conduction studies and Clinical judgement and EMG. maintaining patient welfare Imaging: seldom needed, but MRI might be con- CTS is caused by compression of the median sidered. nerve within the carpal tunnel, which is bound by the carpal bones below, flexor retinaculum Management above, radially by the scaphoid and trapezium, • Treat any underlying associated conditions and medially by the pisiform and hamate. • Physiotherapy and splint the wrists with a degree of dorsiflexion It is most commonly idiopathic, but associated • Steroid injections into the carpal tunnel area conditions include: pregnancy, menopause, hy- • Surgical decompression of the carpal tunnel. The Cardiovascular and Neurological Examinations 309

SCENARIO 18. ULNAR NERVE PALSY

Identifying clinical signs the radial nerve). With the hands held supine, marked hypothenar wasting with sparing of the The ulnar nerve serves most of the intrinsic small thenar eminence can be seen, and no wasting of muscles of the hand. However, the ulnar nerve the forearm muscles. Look for scars or deformity also supplies two important extrinsic muscles of from trauma, or arthritis around the fore- the hand: flexor carpi ulnaris and flexor digitor- arm and wrist. um profundus 4 and 5; it is important to remem- ber this when considering a claw hand deformity. Motor function: there is weak abduction (dorsal Clues pointing towards an ulnar nerve lesion interossei) and weak adduction (palmar inter- include unilateral signs, clawing of the hand and ossei) of the fingers. There is weak thumb adduc- wasting of the hypothenar eminence with sparing tion (adductor pollicis) as demonstrated by of the thenar eminence. attempting to grip a piece of paper held between the borders of the index finger and extended Distal ulnar nerve lesion (distal to the thumb. Grip can be maintained only using thumb elbow) flexion (intact flexor pollicis longus served by the Inspection: with the hands held prone there is median nerve), known as Froment’s sign . There is dorsal guttering and marked wasting of the first intact flexion of the fourth and fifth DIP joints , interosseous between the thumb and index fin- as shown by the marked appearance ger. The hand shows a claw deformity (you might (it is difficult to test actively when the fingers are want to say a clawed appearance ) with hyperex- held in fixed flexion). There is intact medial/ tension at the fourth and fifth MCP joints and ulnar flexion of the wrist. flexion at the fourth and fifth PIP and DIP joints, due to paralysis of the medial lumbricals. There Sensory loss : in the ulnar distribution over the is slight ulnar deviation of the fifth finger (known fifth finger (see Figure 3.19), adjacent ulnar side as Wartenburg’s sign) from unopposed action of of the fourth finger, ulnar side of the palm and extensor digiti minimi (which inserts into the dorsal aspect of the hand. (In distal ulnar nerve ulnar side of the little finger and is innervated by lesions this sensory loss may be variable or present only in the fifth finger.) Test along the radial side of the fourth finger and up the ulnar border of the wrist and forearm to check that this does not represent a C8–T1 lesion instead (mus- cle wasting would also be different, see Scenario 16).

Additional tests: you can ask to perform Tinel’s test tapping along the course of the ulnar nerve from the wrist along the ulnar border of the forearm and up to the elbow, which may elicit tingling from irritation of the nerve.

Proximal ulnar nerve lesion (at the Figure 3.19 Ulnar nerve cutaneous distri- elbow) bution Inspection: with the hands held prone there is dorsal guttering and marked wasting of the first 310 MRCP PACES Manual interosseous between the thumb and index fin- Di¡erential diagnosis ger. The hand shows a mild/no clawed appear- ance with hyperextension at the fourth and fifth Elbow lesions MCP joints but only mild flexion at the fourth • Fractures: and fifth PIP joints and no flexion at the DIP • Supracondylar fractures are most common joints. This is known as the ulnar paradox , with and late complications of fracture/surgery proximal lesions resulting in less clawing. There can include cubitus valgus deformity of is slight ulnar deviation of the fifth finger known the elbow joint. as Wartenburg’s sign (see above). With the palms • Dislocation: facing up, there is marked hypothenar wasting , • Arthritis: bony spurs and narrowing of the with sparing of the thenar eminence. In addition, ulnar groove there is wasting of the ulnar border of the fore- • Compression: cubital tunnel syndrome arm. Look for scars or deformity from trauma, describes constriction under the fibrous surgery or arthritis around the elbow. arch of the two points of insertion of flexor carpi ulnaris. Certain occupations are more at wrist such as secretaries (from Motor function: there is weak abduction (dorsal leaning on elbows) and decorators (from interossei) and weak adduction (palmar interos- repeated elbow flexion and extension). sei) of the fingers. There is weak thumb adduc- tion (adductor pollicis) with Froment’s sign (see Wrist lesions above). There is loss of flexion of the fourth and • Fractures fifth DIP joints (loss of flexor digitorum profun- • Ganglion dus with high lesions) as shown by holding the • Tumour fourth and fifth MCP and PIP joints extended and • Mononeuritis multiplex. asking the patient to try to flex the fourth and fifth DIP joints (this takes practice to demonstrate effectively!). There is weakness of medial/ulnar Clinical judgement and flexion of the wrist (flexor carpi ulnaris) not maintaining patient welfare found in distal lesions (see above). Investigations Sensory loss : this occurs in the ulnar distribution Imaging: plain radiograph of elbow joint, ultra- over the fifth finger, ulnar side of the fourth finger, sonography of the cubital tunnel or MRI. ulnar side of the palm and dorsal aspect of the hand (see Figure 3.19). Nerve conduction studies localise the site of the lesion Additional tests: you can ask to perform Tinel’s Blood tests: FBC, CRP, ESR, anti-nuclear antibodies test, tapping along the course of the ulnar nerve (ANAs), anti-DNA, pANCA (perinuclear anti- from the wrist up the ulnar border of the forearm neutrophil cytoplasmic antibody or ANCA) , cAN- towards the elbow, which may elicit tingling from CA (cytoplasmic ANCA), rheumatoid factor, hepa- nerve irritation. titis B and C serology (mononeuritis multiplex).

The elbow flexion test can be used to test for Management ulnar nerve compression at the elbow, particu- Conservative : avoidance of aggravating factors, larly in cubital tunnel syndrome. The elbow is physiotherapy, splinting, NSAIDs. flexed fully with the forearm supinated, and with- in 60 seconds the patient starts to feel pain or Surgical: transposition of the ulnar nerve and/or tingling in the fourth and fifth fingers. decompression of the cubital tunnel. The Cardiovascular and Neurological Examinations 311

SCENARIO 19. RADIAL NERVE PALSY

Identifying clinical signs Radial nerve lesion at the axilla Inspection: there is and slight finger The radial nerve serves the extensors of the flexion but with no wasting of the hand muscles. elbow, wrist and fingers. With knowledge of the Look for surgical or traumatic scars anywhere of radial nerve innervation it is possible along the route of the radial nerve from the axilla to describe the level of the lesion giving rise to to the wrist. clinical signs (see Figure 3.20).

Deltoid

Teres minor Triceps, long head

Triceps, lateral head Triceps, medial head

Radial nerve

Extensor carpi radialis longus Extensor carpi radialis brevis Supinator Posterior interosseous Extensor carpi ulnaris nerve Extensor digitorum Abductor pollicis longus Extensor pollicis longus Extensor pollicis brevis Extensor indicis

Figure 3.20 Route and muscles supplied by the radial nerve 312 MRCP PACES Manual

Motor function : there is weakness of all radially osseous nerve dips beneath the fibrous arcade of innervated muscles: Frohse (‘supinator arch’) which is the common entrapment area). There is intact elbow flexion • Weakness of elbow extension and flexion (midway between supination and pronation) and (midway between supination and pronation) intact elbow extension and forearm supination. • Forearm supination (tested with arm by the There is weakness of wrist extension and finger side and attempted supination of forearm extension at the MCP joints. Triceps and bra- against resistance applied to the patient’s chioradialis deep tendon reflexes are intact. hand) • Wrist extension and finger extension at the Sensory loss : there is no sensory loss because the MCP joints. posterior interosseous branch is a purely motor Both triceps and brachioradialis (biceps) deep nerve. tendon reflexes are absent. Radial nerve lesion at the wrist Sensory loss : this occurs over the triceps, poster- Inspection: the arm and hand appear normal. ior forearm and first dorsal interosseous. Look for surgical or traumatic scars around the wrist. Radial nerve lesion in the spiral groove of the humerus Motor function: there is no motor weakness Inspection: there is wrist drop and slight finger (because the radial nerve serves only extrinsic flexion but with no wasting of the hand muscles. extensor muscles of the hand from above the Look for surgical or traumatic scars. wrist).

Motor function : there is weakness of all radially Sensory loss : there is sensory loss in the first innervated muscles below the triceps. There is dorsal interosseous only (as the sensory branch weakness of elbow flexion (midway between becomes superficial at the wrist). supination and pronation) but elbow extension is intact (triceps innervation above the spiral grove). There is weakness of forearm supination, wrist extension and finger extension at the MCP joints. Di¡erential diagnosis The triceps reflex is preserved but the brachio- radialis (biceps) deep tendon reflex is absent. • Axillary lesions : • Fracture/dislocation of humeral head Sensory loss : there is sensory loss over the poster- • Compression: use of shoulder crutch or ior forearm and first dorsal interosseous. There ‘Saturday night palsy’ from prolonged may be variable loss of sensation over the tri- hanging of the arm over the back of a ceps. chair (when intoxicated) • Spiral groove lesions : Radial nerve lesion con¢ned to the • Fracture: mid-shaft fracture of the humerus posterior interosseous nerve • Compression: wheelchair users resting Inspection: there is wrist drop and slight finger back of their arm against the chair flexion but with no wasting of the hand muscles. • Posterior lesions : Look for surgical or traumatic scars. • Compression: from the arcade of Frohse/ supinator arch Motor function : there is weakness of radially • Interosseous lesions : innervated muscles below the supinator (the su- • Tumours/lipomas or ganglia near the pinator nerve comes off before the posterior inter- elbow The Cardiovascular and Neurological Examinations 313

• Wrist lesions: Imaging : ultrasonography/MRI may rarely be con- • Fracture: at the distal radius sidered. • Compression: tight bracelets/handcuffs/ plaster casts. Management • Conservative management of ‘Saturday night palsy’ with spontaneous improvement Clinical judgement and • Physiotherapy and splinting for mild maintaining patient welfare compressive lesions • Surgical correction of fracture/dislocations. Investigations Electrophysiology: nerve conduction studies and EMG. 314 MRCP PACES Manual

SCENARIO 20. COMMON PERONEAL NERVE PALSY AND L4^5 ROOT LESION

Identifying clinical signs flexion and inversion. The common peroneal nerve (Figure 3.21) serves the anterior part of the Common peroneal nerve palsy is the most com- lower leg, winding around the neck of the fibula mon cause of foot drop. However, a lesion in and dividing into the superficial peroneal nerve any of the areas, including the motor cortex, (foot eversion and sensation to lateral lower leg , lumbar nerve roots L4–5, lumbo- and dorsum of foot) and the deep peroneal nerve sacral plexus and , and peripheral (foot and toe dorsiflexion and sensation to the neuropathies or myopathies, can cause foot drop dorsal web space between the hallux and second with different associated clinical signs. Know- toe). ledge of the anatomy of the common peroneal nerve will help determine the level of the lesion. Common peroneal nerve palsy Inspection: there is foot drop with a high- The sciatic nerve (L4, L5, S1–3) divides into its stepping gait . There is wasting of the antero- terminal branches, the tibial nerve and the com- lateral compartment of the lower leg . Look for mon peroneal nerve, two-thirds down the poster- surgical or traumatic scars near the knee and ior thigh. The tibial nerve serves the posterior neck of the fibula. Look for any ankle supports/ compartment of the lower leg, producing plantar- adapted footwear.

Lateral cutaneous n. of calf Common peroneal n. Cutaneous distribution Deep peroneal n. (cut)

Common peroneal nerve Superficial peroneal n.

Peroneus longus Superficial peroneal nerve

Peroneus brevis

Medial cutaneous Deep peroneal nerve branch serving web space Lateral Anterior Lateral cutaneous branch

Figure 3.21 Route and muscles supplied by the common peroneal nerve and cutaneous distribution The Cardiovascular and Neurological Examinations 315

Motor function: there is weakness of ankle dorsi- Sensory loss : only in the dorsal web space be- flexion, and hallux extension (deep peroneal tween the hallux and second toe. nerve) and eversion (superficial peroneal nerve). Test for eversion in a passively dorsiflexed foot Di¡erential diagnosis because the everters cannot exert their action if Myopathy : tends to give rise to proximal weak- the foot is in plantarflexion. The ankle jerk is ness but there are distal variants. Signs will be intact and plantar reflex is downwards. There is bilateral. All foot movements will be weak in- normal plantarflexion and inversion. In mild cluding plantarflexion and hallux flexion. The cases, weakness may be seen only when asking ankle jerk may be reduced. There will be no the patient to walk on the heels. sensory loss. There will be myopathic signs in the upper limbs and possibly the face. Sensory loss : over the lateral calf and dorsum of the foot, but sparing the little toe. The little toe Sensorimotor peripheral neuropathy : there is has sensation from the sural nerve, a branch of also weak plantarflexion and a stocking pattern the tibial nerve. of sensory loss including the little toe. Signs will be bilateral. There may be dorsal column signs and a positive Romberg’s test and sensorimotor Super¢cial peroneal nerve palsy signs in the upper limbs. Inspection: there is wasting of the lateral com- partment of the lower leg but no obvious high- Neuromuscular junction disorder: signs tend to stepping gait . Look for surgical or traumatic be bilateral with and facial involve- scars near the knee and neck of the fibula. Look ment. Weakness will affect plantarflexion and be for any ankle supports/adapted footwear. fatigable. Reflexes and sensation will be normal.

Motor function: there is slight weakness of ankle MND: signs are bilateral, although in early dis- dorsiflexion which might be seen only when the ease there can be asymmetry. There will be a patient is asked to walk on the heels. There is no mixture of UMN and LMN signs with increased weakness in hallux extension , but there is weak- tone, brisk ankle jerks, possibly upgoing plantar ness of eversion . There is normal plantarflexion reflexes, marked wasting and . and inversion. There tend to be signs in the upper limbs and face. There are no sensory findings. Sensory loss : over the lower lateral calf and Sciatic nerve lesion : a peripheral nerve lesion dorsum of the foot, but sparing the little toe . affecting the sciatic nerve can cause foot drop, but also weak plantarflexion and inversion, weak Deep peroneal nerve palsy knee flexion (with preserved knee extension) and Inspection: there is foot drop with a high- hip extension. The ankle jerk is absent with a stepping gait . There is wasting of the anterior preserved knee jerk. Sensory loss will be along compartment of the lower leg . Look for surgical the posterior thigh, lower leg and foot, sparing or traumatic scars near the knee and neck of the the medial side of the lower leg. Injury to the fibula, anterior lower leg. Look for any ankle sciatic nerve can occur through hip surgery, mis- supports/adapted footwear. placed gluteal injections and pelvic , such as trauma, haematoma, abscess or tumours. Motor function: there is weakness of ankle dorsi- flexion (deep peroneal nerve) and hallux exten- Lumbosacral plexus lesion : presents similarly to a sion but intact eversion (superficial peroneal sciatic nerve lesion but with femoral nerve invol- nerve). vement. This will cause additional weakness in 316 MRCP PACES Manual hip flexion, abduction and adduction, and knee Cortical lesion : a tumour or affecting the extension. The knee and ankle jerks are lost. motor cortex, such as a lacunar infarct or para- There will be more extensive sensory loss includ- sagittal meningioma, can rarely cause localised ing the anterior thigh and medial lower leg. lower limb weakness and foot drop. There will be UMN signs.

L4–5 : leads to similar findings as a Causes of common peroneal nerve palsy common peroneal nerve lesion. In addition there • Trauma will be weak hip abduction and adduction and • Fibular fracture variable effects on knee flexion and extension. • Knee surgery The knee jerk (L3–4) may be lost but ankle jerks • Compression: plaster cast, leg crossing, (S1–2) preserved. Sensory loss will include the weight loss. medial part of the lower leg (L5). The patient often has back pain because nerve root compres- sion usually arises from lumbar disc herniation Clinical judgement and (less commonly a tumour). Straight leg raise, maintaining patient welfare stimulating nerve root irritation from stretching, will reproduce symptoms. Investigations Electrophysiology: nerve conduction studies and Spinal cord lesion: this can cause weakness in EMG. foot dorsiflexion but will also cause weakness in other muscle groups of the leg depending on the Imaging: MRI might be considered. level of the lesion. Foot drop is not so obvious due to the UMN spasticity. Lesions can be uni- Management lateral or bilateral. Sensory signs will depend on • Avoidance of aggravating factors such as leg the location of the lesion in the cord and there crossing or squatting may be dissociated sensory loss, giving rise to • Physiotherapy and splinting of the ankle/foot the Brown–Se´quard syndrome if the lesion is • Surgical repair or release if there has been unilateral. transection or tethering. The Cardiovascular and Neurological Examinations 317

SCENARIO 21. NYSTAGMUS

Nystagmus will usually be a sign as part of wards the side of the lesion. There may be another disorder such as cerebellar syndrome or dysarthric speech. MS, but rarely it can form the full case in the PACES station. Additional Finish your exam by asking to test the cranial Nystagmus is an involuntary rhythmic oscillation , particularly looking for any abnormality of the eye(s) that may be physiological, congeni- of cranial nerve V (corneal and facial sensation) tal or acquired. It represents a problem with the or cranial nerve VIII which may suggest a lesion neural mechanisms/centres involved in maintain- at the cerebellopontine angle (see Scenario 27). ing image fixation on the fovea for optimal visual Ask to examine the fundus for optic atrophy acuity. which may be present in MS.

Ask to examine the upper and lower limbs, Identifying clinical signs which may show pyramidal weakness consistent with MS, or dorsal column signs in alcohol mis- Nystagmus is usually described with reference to: use or vitamin B 12 deficiency. • Monocular or binocular/conjugate • Romberg’s test is not a test of cerebellar function, Position: primary (looking forward) or only but rather of dorsal column/proprioceptive func- gaze related • tion. A patient with a cerebellar lesion cannot Type: pendular (equal velocity in either usually stand steady, feet together with arms by direction) or jerk: a slow drift then fast the sides even with the eyes open, so Romberg’s corrective phase – the direction of nystagmus test cannot be performed. refers to the fast phase • Plane: horizontal, vertical or rotatory/torsional (sometimes it is easier to tell by looking at the pull on the conjunctival vessels). Di¡erential diagnosis Unilateral cerebellar pathology and nystagmus will tend to be caused by structural lesions: Cerebellar nystagmus • Cerebrovascular events, eg lateral medullary syndrome This is a binocular/conjugate, primary and gaze- • Demyelination, eg MS related jerk nystagmus which is in the horizontal • Cerebellar/posterior fossa tumours, eg plane, and the direction of the nystagmus ( fast astrocytomas, haemangioblastomas, phase) is towards the same side as the cerebellar medulloblastomas and metastatic disease lesion and maximal on looking towards this side . (breast, lung, skin, kidney). The nystagmus does not fatigue with continued Bilateral cerebellar nystagmus will be caused by gaze to the affected side. There is also loss of systemic pathology: smooth saccades. • Toxins (alcohol, chemotherapy and Further cerebellar testing will show homolateral anticonvulsants) poor finger–nose pointing with dysmetria and • Autoimmune and paraneoplastic processes intention tremor, dysdiadochokinesis, poor heel– • Inherited disorders involving cerebellar shin coordination and an ataxic gait falling to- degeneration, eg olivopontocerebellar 318 MRCP PACES Manual

degeneration and Friedreich’s ataxia (see are no peripheral symptoms such as tinnitus or Scenario 12). deafness and no cerebellar signs.

Di¡erential diagnosis Vestibular nystagmus The central vestibular system includes the vestibu- lar nerve nuclei and their projections to the cere- Peripheral vestibular nystagmus : this is a binocu- bellum, extraocular nuclei via the medial lar/conjugate horizontal or rotatory/torsional nys- longitudinal fasciculus, the spinal cord via the tagmus which is a primary and gaze-related vestibulospinal tract and projections to the cortex. unidirectional jerk nystagmus with the fast com- ponent maximal to the opposite side of the Causes of central vestibular nystagmus include vestibular lesion. With upward or downward brain-stem stroke and vertebrobasilar insuffi- gaze the nystagmus remains horizontal and in ciency, brain-stem tumours, demyelination such the same direction. With continued gaze away as MS, syringobulbia (see Scenario 7) and basilar- from the side of the lesion the nystagmus fa- type migraine (some classify this as a cause of tigues. There are no cerebellar signs. The gait peripheral vestibular nystagmus when it is asso- may be unsteady, falling towards the side of the ciated with benign paroxysmal positional vertigo ). lesion, and the patient may describe vertigo symptoms. These are often worse when testing gait. Tinnitus and deafness may be found on the Pendular nystagmus side of the lesion. This is a conjugate or monocular multidirectional Di¡erential diagnosis nystagmus that can appear chaotic. The oscilla- The peripheral vestibular system includes the tion has equal velocity in all directions . It is semicircular canals, otoliths and the vestibular present in all positions including the primary portion of cranial nerve VIII. position.

The causes of peripheral vestibular nystagmus in- Additional: look around the bed for clues of clude labyrinthitis, acoustic neuroma, Me´nie`re’s visual aids used by the blind patient and a disease, benign paroxysmal positional vertigo general appearance that might suggest albinism. (BPPV), autoimmune inner ear disease (AIED) and Ask to perform fundoscopy to look for optic degenerative middle-ear disease such as oto- atrophy, signs of retinitis pigmentosa and cata- sclerosis. racts with disruption of the red reflex.

Di¡erential diagnosis Central vestibular nystagmus Monocular or binocular visual deprivation is the most common cause of pendular nystagmus, but This is a binocular/conjugate horizontal/verti- other causes include such cal/rotatory or mixed (may appear chaotic) nys- as MS and brain-stem dysfunction. tagmus, which is a primary and gaze-related nystagmus. It is multidirectional so that on look- ing to the left it is leftward (fast component to the Internuclear ophthalmoplegia (INO left), looking to the right it is rightward and or ataxic nystagmus) looking up it is upward. There is no fatigue of the nystagmus on sustained gaze in any direction. There is failure of conjugate eye movements on The patient may have a tendency to fall in any lateral gaze . In the primary position there may be direction on testing gait; vertigo is unusual. There a divergent strabismus. In a left-sided INO (lesion The Cardiovascular and Neurological Examinations 319 in the ipsilateral MLF) there is partial or total directions of gaze. Lateral gaze may accentuate failure of adduction of the left eye on looking the nystagmus. right, but normal abduction of the right eye, with jerk horizontal nystagmus in the abducting eye Additional with fast corrective phase towards the right side Look for signs of syringobulbia and syringomyelia (opposite side to the lesion). The nystagmus of which may occur together with an Arnold–Chiari the abducting eye is not necessary for the diag- malformation, eg bulbar palsy, INO, balaclava- nosis of INO. The patient may describe diplopia helmet loss of facial sensation, a dissociated when looking to the right (contralateral) side. It sensory loss usually in a cape distribution, LMN can be shown that this is not a left medial rectus signs in the upper limbs and UMN signs in the palsy by the fact that, by covering the right lower limbs. Look for cerebellar signs that may abducting eye, the left eye adducts normally with also suggest syringobulbia or a posterior fossa convergence. Sometimes convergence can be tumour. affected if the lesion extends into the midbrain. In a bilateral INO there will be failure of adduc- Di¡erential diagnosis tion of either eye with lateral gaze in the opposite Downbeat nystagmus usually signifies pathology direction. at the craniocervical junction. Arnold– is the most common cause. Down- Additional beat nystagmus may also be seen with brain-stem State that you would like to look for additional stroke, syringobulbia, spinocerebellar degenera- eye signs supportive of MS such as optic atrophy, tion, MS and drug (phenytoin, lithium) and alco- visual field defects, optic disc pallor on fundo- hol toxicity. scopy and/or a relative afferent pupillary defect (RAPD). Look for cerebellar signs in support of MS or brain-stem infarction. Upbeat nystagmus

Di¡erential diagnosis There is a bilateral vertical nystagmus in the INO is due to a lesion in the MLF within the primary position with the fast phase beating in pons and midbrain, which connects the contra- the upward position. lateral nerve VI nucleus to the ipsilateral oculo- motor (nerve III) nucleus. If the nystagmus increases on upward gaze, this suggests pathology in the anterior vermis of the MS is the most common cause in a younger cerebellum. There may be other cerebellar signs person, and brain-stem infarction causing unilat- such as loss of smooth saccades, slurred speech eral INO is the most common cause in an older and truncal ataxia. person. Other causes include brain-stem tu- mours, viral infection, syphilis infection, Lyme If the nystagmus increases on downward gaze, disease, trauma, Arnold–Chiari malformation, syr- this suggests pathology in the medulla. There ingobulbia, and drug (phenothiazines, phenytoin, may be associated brain-stem signs such as pala- tricyclic antidepressants) and alcohol intoxica- tal weakness with nasal speech. tion.

Physiological nystagmus Downbeat nystagmus This is a gaze-evoked jerk nystagmus occurring There is a bilateral downbeat nystagmus in the at the extremes of gaze and absent in the primary primary gaze , which remains downbeat in all position. The elastic pull of the extraocular mus- 320 MRCP PACES Manual cles and tendons exerts a force that tends to bring Management the eye back to the midline, but the neural • Cessation of causative medications integrator tries to overcome this with corrective • Correction of refractive errors with contact quick movement (jerk nystagmus) to the desired lenses extreme of gaze. • Downbeat nystagmus may be treated with base-out prisms, which induce convergence • Botulinum toxin injection into rectus muscles Clinical judgement and can ameliorate acquired nystagmus. maintaining patient welfare However, this diminishes all types of eye movement and can cause diplopia, ptosis and Investigations increased nystagmus in the uninjected eye. These will be determined by the type of nystag- mus and the associated differential diagnosis. The Cardiovascular and Neurological Examinations 321

SCENARIO 22. OPHTHALMOPLEGIA

The most common gaze palsies seen in the PACES examination are cranial nerve III and VI Superior oblique muscle palsies. Remember to look for associated cranial (IV cranial nerve) nerve abnormalities or peripheral examination Superior rectus muscle (III cranial nerve) signs that will point towards a specific disease or help further localise the lesion. Medial rectus muscle (III cranial nerve) It is worth revisiting the anatomy and function of the extraocular nerves III, IV and VI together with their muscles so that their examination becomes Lateral rectus muscle straightforward (see Figures 3.22 and 3.23). (VI cranial nerve) Inferior rectus muscle (III cranial nerve)

Nerve III (oculomotor) palsy Inferior oblique muscle (III cranial nerve) See Figure 3.24. Nerve III supplies most of the extraocular muscles including superior rectus Figure 3.22 Right eye and its muscles (elevation), medial rectus (adduction) and inferior

Superior rectus and inferior oblique (III cranial nerve)

Inferior oblique Up Superior rectus (III cranial nerve) (III cranial nerve) Up/Out Up/In

Lateral rectus Medial rectus (VI cranial nerve) Out In (III cranial nerve)

Down/Out Down/In Superior oblique Inferior rectus (IV cranial nerve) Down (III cranial nerve)

Inferior rectus and superior oblique (III cranial nerve) Figure 3.23 Cardinal directions of gaze and extorsion/intorsion 322 MRCP PACES Manual oblique (extorsion, elevation and abduction). It branches through the superior orbital fissure. The also elevates the eyelid through its innervation of superior branch serves levator palpebrae super- levator palpebrae superioris and carries pregan- ioris and superior rectus, which is also joined by glionic parasympathetic innervation which sympathetic supply from the internal carotid ar- causes pupil constriction via the sphincter (con- tery. The inferior branch serves the other oculo- strictor) pupillae. motor muscles and carries the parasympathetic axons to the constrictor pupillae. The nerve III nucleus in the midbrain is divided into subnuclei to innervate the separate muscles, and it Identifying clinical signs is partnered with the nearby Edinger–Westphal There is a left-sided ptosis (see Figure 3.24) and nucleus which gives rise to the preganglionic when the eyelid is raised the eye is in a down parasympathetic fibres; these lie superficially with- and out position (due to the unopposed com- in the nerve and are therefore easily compromised bined action of lateral rectus and superior obli- by compressive lesions. que), giving a divergent strabismus/squint. The patient cannot move the affected eye across the The subnuclei serve the IPSILATERAL extraocular midline. Diplopia is maximal on trying to look muscles (medial rectus, inferior rectus and infer- away from the affected side and up. There is a ior oblique) with two important exceptions: dilated and non-reactive pupil (to light or at- tempted accommodation) from parasympathetic 1. The nucleus sends fibres across to the opposite fibre involvement. oculomotor nucleus, which then innervates the CONTRALATERAL superior rectus. With a nuclear lesion there will be contralateral 2. The nucleus supplies BOTH levator palpebrae eye signs in addition to the ipsilateral signs. There with crossed and uncrossed fibres. is a contralateral partial ptosis (bilateral innervation The nerve III fascicles/trunks leave the midbrain of levator palpebrae superioris) but ptosis will be passing ventrally. Nerve III then passes in the more pronounced on the ipsilateral side. There will subarachnoid space and runs close to the poster- also be a contralateral elevation palsy (the nucleus ior communicating artery. It enters the lateral side innervates the contralateral superior rectus). Re- of the cavernous sinus, crosses over the trochlear member, there is an ipsilateral elevation palsy nerve, and exits medially as superior and inferior because fibres from the contralateral subnucleus

Figure 3.24 Left-sided III nerve palsy. Reproduced with the kind permission of Professor Chua Chung Nen The Cardiovascular and Neurological Examinations 323 pass through the ipsilateral subnucleus before in- Small vessel disease : diabetes, hypertension, nervating the superior rectus. atherosclerosis.

Di¡erential diagnosis Infection : , syphilis, basilar menin- Nuclear lesion: infarction, haemorrhage, tumour, gitis (bacterial, mycobacterial, fungal, parasitic). abscess, demyelination.

Midbrain lesion : herniation, infarction, haemor- rhage, tumour, abscess, demyelination (MS). Nerve IV (trochlear) palsy Subarachnoid lesion : aneurysm, haemorrhage, meningitis, inflammation including vasculitides Nerve IV supplies the superior oblique muscle (giving rise to mononeuritis multiplex), tumour, which intorts, depresses and abducts the globe. migraine. This combined action allows the eye to look down and in. The nerve IV nuclei lie in the Cavernous sinus lesion: tumour (pituitary, cranio- midbrain where the nerves decussate and then pharyngioma), , aneurysm, fistula, in- exit dorsally. It is the only cranial nerve to exit fection, inflammatory. the brain dorsally and it has a long course, making it susceptible to trauma. Orbital lesion: trauma, tumour. A nerve IV palsy can be very subtle so a candi- date must actively look for it.

Figure 3.25 Right-sided IV nerve palsy. Reproduced with the kind permission of Professor Chua Chung Nen 324 MRCP PACES Manual

Identifying clinical signs • Infection: Lyme disease, syphilis, basilar The right eye appears slightly elevated/normal meningitis (bacterial, mycobacterial, fungal, and the patient has a head tilt away from the side parasitic) of the lesion, tucking the chin in slightly to bring • Midbrain/nuclear lesion: infarct, the visual axis of the affected eye central again haemorrhage, tumour, abscess, demyelination (see Figure 3.25). The affected eye cannot look (MS) down in adduction (towards the nose). It is in this • Cavernous sinus lesion: tumour (pituitary, position that the patient experiences most verti- craniopharyngioma), thrombosis, aneurysm, cal diplopia (giving rise to the classic history of fistula, haemorrhage, infection, inflammatory. difficulty reading books or climbing stairs when this direction of gaze is needed). Remember that diplopia is always worse in the direction of gaze of the paretic muscle. The false outer/upper im- age disappears when the affected eye is covered. Nerve VI (abducens) palsy

Di¡erential diagnosis Nerve VI innervates the ipsilateral lateral rectus, • Congenital which abducts the eye. The nerve VI nucleus is • Trauma (most common cause) in the caudal part of the pons. Approximately • Small vessel disease: diabetes, hypertension, 40% of its neurons pass to the nearby MLF, to atherosclerosis then cross over to the contralateral nerve III • Inflammatory: mononeuritis multiplex, nucleus to innervate the contralateral medial peripheral neuropathy rectus and produce conjugate lateral gaze.

Figure 3.26 Left sided VI nerve palsy. Reproduced with the kind permission of Professor Chua Chung Nen The Cardiovascular and Neurological Examinations 325

Identifying clinical signs Complex ophthalmoplegia There is a convergent strabismus in the primary position due to the unopposed action of the Identifying clinical signs intact medial rectus (see Figure 3.26). There is Thyroid ophthalmopathy will usually present as horizontal diplopia maximal on attempted gaze a complex ophthalmoplegia not attributable to in the direction of the paretic muscle. The outer any single nerve lesion. This does not represent a image disappears on covering the affected eye. true ophthalmoplegia. It is due to soft-tissue inflammation and swelling within the orbit caus- On testing the remaining , pay ing restriction of eye movements. There is usually particular attention to nerves VII and VIII, and proptosis, chemosis, lid lag and other thyroid check for nystagmus and other cerebellar signs signs. that would indicate a cerebellopontine angle lesion. There may be signs of bilateral papilloe- Myasthenia gravis may present as a complex dema on fundoscopy from a space-occupying ophthalmoplegia not attributable to any single lesion or idiopathic intracranial hypertension. nerve lesion. Eye movements are fatigable and Here, the nerve VI palsy acts as a ‘false localising there are no pupillary signs. sign’ due to downward displacement of the brain stem causing stretching of the . The Miller–Fisher variant of Guillain–Barre´ syn- drome may present initially with an ophthalmo- plegia, with the descending paralysis from peripheral demyelination giving the classic triad Di¡erential diagnosis of ophthalmoplegia, ataxia and areflexia. • Congenital: congenital absence of nerve VI – Duane’s syndrome Chronic progressive external ophthalmoplegia • Trauma (CPEO) is the most common manifestation of • Raised ICP: space-occupying lesion or mitochondrial myopathy, in itself very rare, and idiopathic intracranial hypertension usually presents as a bilateral progressive ptosis, • Small vessel disease: diabetes, hypertension, which proceeds to a bilateral ophthalmoplegia atherosclerosis without pupillary changes. Kearns–Sayre syn- • Inflammatory: mononeuritis multiplex, drome is a mitochondrial myopathy that presents postviral, peripheral neuropathy with the triad of age ,20 years, CPEO and • Infection: Lyme disease, syphilis, basilar retinitis pigmentosa. Other associated features meningitis (bacterial, mycobacterial, fungal, include cerebellar syndrome, cognitive impair- parasitic) ment, Babinski’s sign, hearing loss, seizures, short • Pontine/nuclear lesion: infarct, haemorrhage, stature and delayed puberty, with other endo- tumour, abscess, demyelination (MS) crine abnormalities and cardiac conduction de- • Petrous bone pathology: in severe ongoing fects. otitis media there can be infiltrative osteomyelitis involving the petrous temporal Oculopharyngeal dystrophy (see Scenario 10) is bone an autosomal dominant trinucleotide repeat dis- • Cavernous sinus lesion: tumour (pituitary, ease occurring in 60–70 year olds, with progres- craniopharyngioma), thrombosis, aneurysm, sive ptosis and ophthalmoplegia without fistula, haemorrhage, infection, inflammatory. pupillary changes, leading to and 326 MRCP PACES Manual facial weakness, and in the latter stages of dis- Clinical judgement and ease to proximal muscle weakness. maintaining patient welfare

Investigations • Cavernous sinus syndrome These are urgent if an aneurysm, subarachnoid haemorrhage (SAH), uncal herniation, meningitis, stroke or trauma is Structures contained within the cavernous sinus: suspected. • Internal carotid artery • Imaging : CT or MRI of the brain is indicated if • Sympathetic carotid plexus suspecting aneurysmal, SAH, stroke, space- • Cranial nerves: III, IV, VI and V1 and V2 occupying lesion Æ herniation, or traumatic branches (V3 lies outside the sinus). cause. Cerebral angiography may be needed to investigate aneurysmal disease and Cavernous sinus syndrome signs: arteriovenous (AV) malformations including • Painful ophthalmoplegia (unilateral single or fistulae. usually combined nerve III, IV and VI palsies) • Blood tests : investigations for small-vessel • Horner’s syndrome (with no associated disease will include fasting blood glucose/ anhidrosis because the lesion occurs after the HbA1c, autoimmune profile, pANCA and superior cervical ganglion and the pupil may cANCA, ESR and CRP if suspecting giant cell be mid-position and fixed with both arteritis (GCA). parasympathetic and sympathetic disruption) • Lumbar puncture : indicated if suspecting • Anaesthesia of forehead, maxilla and meningitis, and a space-occupying lesion has conjunctiva (V1 and V2 branches) been ruled out. • Proptosis (if pulsating suggests carotid– cavernous fistula) • Conjunctival injection with chemosis Management • Papilloedema Æ visual loss This is directed by the underlying cause. • Orbital bruit.

Di¡erential diagnosis of cavernous sinus Nerve III (oculomotor) palsies may resolve spon- syndrome taneously over months if the underlying cause is • Tumours : meningiomas, extension of pituitary ischaemia (typically in hypertensive or diabetic or craniopharyngiomas, metastatic disease patients) of the vasa nervosa. This typically gives • Vascular : cavernous sinus aneurysms or relative sparing of the pupil and is often painful fistulae for unknown reasons. NSAIDs may ameliorate • Thrombosis : usually complicating infection of this. Patching of the deviated eye can be a useful the ethmoid, frontal and sphenoid sinuses or short-term measure. In the long term, surgical extension of dental or orbital infection correction may be indicated for a non-resolving • Inflammatory: herpes zoster, sarcoidosis and stable angle. Wegener’s granulomatosis • Idiopathic: Tolosa–Hunt syndrome is a rare granulomatous inflammation of the cavernous Nerve IV (trochlear) palsies have been treated sinus and superior orbital fissure. It causes a successfully with botox injection (of other mus- painful ophthalmoplegia Æ pupillary effects. cles), prisms and surgical correction. The Cardiovascular and Neurological Examinations 327

Nerve VI (abducens) palsies, when isolated in children and young patients, are often benign and resolve spontaneously within 6 months. The cause is unclear. Alternate patching may be use- ful to prevent amblyopia. In older patients GCA should be considered and treated with steroids if appropriate. STATION 5

Integrated Clinical Assessment

393 Integrated Clinical Assessment 395

STRUCTURE OF THE ICA

The older Station 5 (until 2009) involved a series The seven areas in which candidates are scored of three short cases based around examination of are summarised below, with candidates being the eyes, an endocrine disorder or rheumatologi- scored on a three-point scale: satisfactory, border- cal condition. Like the other examination sta- line or unsatisfactory. tions, this involved a ritualised examination followed by presentation of findings to the exam- Clinical communication skills iners. There are still ‘echoes’ of this old format in • Eliciting a history relevant to the complaint many textbooks and courses; skin, locomotor • Explaining information to the patient in a and endocrine scenarios have been shoe-horned focused, fluent and professional manner into an ‘integrated clinical assessment’ (ICA) for- mat. This slightly misrepresents the content of the Physical examination exam, which is highly varied and demands far more than an ability to elicit clinical signs. In- Performing an examination in a correct, appro- deed, this is the only station of the PACES exam priate, practised and professional manner that assesses candidates in all categories of the marking scheme. Clinical judgement

Station 5 is organised as follows: Selecting a sensible and appropriate investigation and treatment plan • Two 10-minute cases known as ‘brief clinical consultations’. Managing patient’s concerns • The candidate has 8 minutes with the patient to take a focused history, carry out a relevant • Detecting, acknowledging and attempting to examination, reach a diagnosis or identify a address patient’s concerns clinical problem and then communicate this • Listening to the patient. • Demonstrating empathy • The remaining 2-minute discussion with the Identifying physical signs examiners will not begin until these 8 minutes have elapsed. • Identifying the correct physical signs • The examiner will ask the candidate to state • Not finding signs that are not present the positive physical findings, their Differential diagnosis concluding diagnosis and differential diagnoses (if appropriate) based on their Constructing a sensible differential diagnosis, in- assessment. cluding the correct diagnosis

CONTENT AND MARKING OF Maintaining patient welfare THE ICA Treating the patient respectfully and sensitively, ensuring comfort, safety and dignity The cases found in Station 5 typically involve a presenting complaint that guides you to the rele- A formal marksheet is shown on page can be vant system for a targeted examination. Alterna- found on the MRCPUK website. tively, there may be obvious clinical signs (eg thyroid eye disease) and a rather vague history. 396 MRCP PACES Manual

INFORMATION GIVEN TO Your role : You are the medical doctor on call CANDIDATES, PATIENTS AND EXAMINERS Patient name: Mrs Beverley Gordon – age 39 years Before each Station 5 examination, each candi- This lady was admitted to the orthopaedic ward date, patient and examiner will be given some for a carpal tunnel release operation. information. She mentioned to the orthopaedic doctors that Below is a worked example of the information she has swelling in her neck and they have given to each person based on guidance on the asked for the opinion of a . You were MRCP website (www.mrcpuk.org). asked by your consultant to see the patient and to assess the suspected swelling in her neck. Information to candidate Your task is to assess the patient’s problems and address any questions or concerns raised by the You will be asked to see two patients at this patient. station. The clinical information about one of • these patients is given in the box below. You You should assess the problem by means of should have a second sheet giving you informa- a relevant clinical history and a relevant tion about the other patient. physical examination. You do not need to • complete the history before carrying out You have 10 minutes with each patient. The appropriate examination. examiners will alert you when 6 minutes • You should respond to any questions the have elapsed and will stop you after patient may have, advise the patient of your 8 minutes. • probable diagnosis (or differential In the remaining 2 minutes, one examiner diagnoses) and your plan for investigation will ask you to report abnormal physical and treatment where appropriate. signs (if any), your diagnosis or differential • You have 8 minutes to complete the task. diagnosis, and your plan for management (if not already clear from your discussion with Accompanying notes are given to each patient the patient). and may take the following format. Integrated Clinical Assessment 397

Information for patients A trapped nerve in the wrist has been diag- nosed. You are having a short admission to The doctors sitting the examination have been have the nerve released. asked to assess your problem. They will have You mentioned that you thought your neck was 8 minutes to ask you about the problem and swollen when the admitting doctor was exam- any other relevant issues. They will also exam- ining you. You have not mentioned this to a ine you. They should explain to you what they doctor before, but your sister has been com- think is wrong and what action should be taken menting on it for a few years. Your sister is 36 and answer any questions you have, for exam- and has an underactive thyroid. ple about the diagnosis, tests that may be needed, or treatment. One of the examiners You do not have any of the symptoms your will ask them to describe any abnormal exam- sister had when her thyroid was underactive. ination findings and give their diagnosis. Indeed you feel well. Your weight is stable. Your Your history is described below. skin and hair are normal. You do not seem to feel the heat or the cold any more than anyone You are: Mrs Beverley Gordon – age 39 years else. Your bowel works normally. You do not Your problem: A swelling in your neck have any problem with swallowing. You are in the orthopaedic ward and you men- You should ask why your neck seems swollen tioned to the admitting doctor that your neck and whether there is something wrong with seemed swollen. One of the medical doctors your thyroid gland. You should ask if any tests has been asked to see you about this. are required and, if so, what these tests will be. You have been suffering from pain in your right Finally, for the same worked example, the ex- forearm and a numb or tingly feeling in your aminers have the following information, which right hand – affecting your third, fourth and fifth would typically list the findings the candidate fingers. The aching in your arm is worse at would be expected to observe or elicit. night and you have found it more and more difficult to get comfortable. The problem has been present for several years and was worse when you were pregnant with your daughter, 3 years ago, but improved for a time after that. 398 MRCP PACES Manual

Information for examiners you have either almost wordlessly examined your patient without garnering any information about Patient: Mrs Beverley Gordon – age 39 years their symptoms or that you have taken a thor- ough history but examined nothing. You may Examiners should discuss and agree the criteria choose to attempt to divide each consultation for pass and for fail in the competencies being between history and examination, but we assessed. strongly recommend taking a history and exam- As a general guide, candidates would be ex- ination in parallel, just as you would during a pected to: busy on-call or clinic. • Note the history of neck swelling and the It is also crucial to remember that the examiners family history of thyroid disease • are not looking for the ritualised, detailed and Enquire about symptoms of disturbed complete examination of systems as in other thyroid function • stations, or for a full history. They are testing your Examine the neck and identify the smooth ability to perform a targeted examination and and symmetrical thyroid enlargement. Note elicit the most salient symptoms. This is not absence of bruit • solely for the purpose of forming a differential Examine for signs of overactive and diagnosis – don’t forget that you have been underactive thyroid gland and confirm specifically asked to address the patient’s princi- clinical euthyroid state • pal concerns and must therefore explain to them Confirm to the patient that her thyroid what you think the underlying problem is and gland does seem enlarged but reassure her how you are going to investigate and manage it. that the gland seems to be working normally judging from clinical examination. Each of the Station 5 cases in this chapter is Advise on appropriate further investigations. based around a flow chart that gives a worked The lead examiner should: example of how you might perform an ‘inte- grated clinical assessment’ (ICA) for a given pre- (a) Advise the candidate after 6 minutes have senting condition. elapsed that ‘You have two minutes remaining with your patient’ All cases can be divided into a new condition or (b) Ask the candidate to describe any abnormal an exacerbation of an existing chronic illness. physical findings that have been identified These require slightly different emphasis in the (c) Ask the candidate to give the preferred history taking and examination, and this is re- diagnosis and any differential diagnosis that flected in each flow chart. is being considered (d) Ensure any remaining areas of uncertainty, An important feature of this approach is the ‘call- eg regarding the plan for investigation or back’, wherein towards the end of the consulta- management of the problem, are addressed tion candidates re-state the presenting complaint in any time that remains. of the patient and any other specific concerns they raised during the consultation. This is an easy way of demonstrating to the examiners that you have registered and understood the patient’s THE APPROACH TO STATION 5 main concern (which is not necessarily the main clinical priority). It also gives the patient an It is very easy to fall into the familiar modes of opportunity to mention other salient points which the history-taking station or the examination sta- the candidate missed. If you have built a good tions, then find, with a minute remaining, that rapport with the patients/actor, they may help Integrated Clinical Assessment 399 you out at this stage by volunteering important • Peripheral accessories, eg walking stick information. • Peripheral arthropathies, eg knees, ankles • Systemic sclerosis – tight, shiny, stretched skin The consulation should end with you describing: with beaked nose +/ À telangectasia • Cushingoid appearance – steroid treatment • How you will address the patient’s main • Horner’s syndrome – T1 lesion (see page . . . ..) concern (eg analgesia) • Ears for evidence of psoriasis, or gouty tophi • What you think the diagnosis may be in helix of ear. • What investigations and initial treatments are necessary. Then make an assessment of: This is an oppurtunity to demonstrate to the • Nails – pitting, onycholysis, clubbing, nail examiner that you have come to a reasonable fold infarcts, Beau’s lines differential diagnosis and formed a safe and effi- • Skin – tight shiny skin over dorsum of hand or cient investigation and management plan. fingers (scleroderma); tissue paper thin +/ À purpura (steroid ); surgical scars (joint replacement); tar staining EXAMINATION TECHNIQUES FOR • Muscles STATION 5 • bilateral wasting of the small muscles with dorsal guttering (rheumatoid arthritis, Station 5 often requires examination routines not syringomyelia, motor neurone disease) encountered elsewhere in PACES, for example • unilateral wasting of the small muscles of examination of a goitre. These are described the hand (C8/T1 root lesion, eg cervical below. rib, Pancoast tumour) • unilateral wasting involving thenar eminence (median nerve, eg carpal tunnel Examination of the hands syndrome) • unilateral wasting sparing thenar It is important to ensure the patient is comforta- eminence (ulnar nerve, eg elbow trauma) ble and to rest the hands on a pillow. Expose the • Joints (in order to describe the location of the hands and up to and including the abnormality accurately, candidates should elbows. A large proportion of the patients may know the names of the bones and joints) have painful and tender joints and it is imperative • Distribution of any abnormalities – therefore to ask about this before palpating the symmetrical (eg rheumatoid arthritis) or joints, which should be done after careful inspec- asymmetrical (eg seronegative arthritides); tion. proximal or distal joints • Specific deformities eg ‘swan neck’, A rheumatological examination typically involves ‘Boutonniere’, Z-shaped thumb, subluxation, inspection, palpation, neurological assessment, ulnar deviation, Heberden’s nodes, gouty functional assessement and examination for ex- tophi tra-articular signs. However, you may not have • Inflammation – calor, rubor, dolor, tumor and time to do all of these completely. loss of function • NB rubor is replaced with shininess of the 1. Inspection skin in those with dark skin The bulk of the available information will be Once the hands have been inspected, an assess- gathered by inspection rather than palpation or ment of the elbows is essential, as this can reveal active movement. 400 MRCP PACES Manual psoriatic plaques or rheumatoid nodules (indi- neurological assessment focusing particularly on cating sero-positive rheumatoid arthritis). the median and ulnar nerve. Single nerve lesions are occasionally encountered in this station. The whole examination can be achieved by three movements – assessment of the dorsum of the 4. Extra-articular signs hand, then plantar aspect, followed by crossing Having screened for symptoms suggestive of sys- the arms over and exposing the extensors surface temic manifestions of joint disease, examination of the elbows. should then focus on the affected system.

2. Palpation (with caution) This must only be assessed with extreme caution Examination of the axial spine on the examination and only where there is an absolute requirement to do so in the time given, A patient may present with stiffness of the axial such as if there is a specific complaint by the spine and restricted movements (eg as in ankylos- patient (ie painful joints) or inspection has ing spondylitis and other spondylarthropathies). demonstrated a specific abnormality. Inspection is crucial again. For example, the • Palm – Dupuytren’s contracture ‘question mark posture’ (loss of lumbar lordosis, • Elbow nodules fixed kyphoscoliosis of the thoracic spine with • Joints – palpate any swelling to determine extensive of the cervical spine) of ankylosing whether it is soft and boggy (rheumatoid spondylitis can be a ‘spot diagnosis’. The charac- arthritis) or hard and bony (Heberden’s nodes teristic posture is usually immediately evident or gouty tophi) unless the patient is lying down with the head • Skin – tightness or calcinosis in finger pulps supported by pillows. (scleroderma/CREST). This does not mean that you need to examine every The following examination routine should stage joint in the hands, but merely the joints involved, eg disease and detect associated signs: MCP joints. 1. Establish restricted spinal movement 3. Functional assessment of the hands Two quick tests can be perfomed: You will be expected to perform this assessment if the patient reports reduced function. Lumbar spine: modified Shober’s index: With the patient standing upright, place two • First, you can ask the patient to make a fist, marks 10 cm apart on the lumbar spine in the followed by the prayer sign, followed by the midline. The lower mark is at the level of the reverse prayer sign. posterior superior iliac spines. The patient then • Finally, you could ask the patient to do up a flexes forward (ask them to touch their toes) and button on a shirt or hold a pen. at maximal flexion the distance is re-measured. These manoeuvres have been validated against In normal subjects there is an expansion of at more complex assessments of function and provide least 5 cm between the two marks. Lower values a quick means of assessing the likelihood of im- indicate decreased mobility of the lumbar spine. paired function in daily activities. Thoracic spine: Occiput-to-wall distance: Neurological assessment – if relevant to the pre- The subject stands with their back against a wall senting complaint (eg symptoms of carpal tunnel (both heels and buttocks must be touching the syndrome), one should proceed to perform a wall) with a horizontal gaze. In normal subjects Integrated Clinical Assessment 401 the occiput will touch the wall. Any wall-to- with severe hyper- or hypothyroidism are unlikely occiput gap is a measure of restriction of the to appear, but over/under-replacement of thyrox- thoracic and cervical spines. ine is quite a common scenario.

2. Examine for sacroiliitis/enthesitis General observation Examine (carefully) for tenderness over the sa- • Hypothyroidism croiliac joints. Palpate for evidence of other en- • Pale dry skin thesitides over the heels, costochondral joints • ‘Peaches and cream’ complexion and iliac crest. • Dry hair • Note: Loss of the outer one-third of the Tell the examiner you would like to perform the eyebrows is unreliable and non-specific FABERE ( flexion, abduction, external rotation and • Hyperthyroidism extension) test. The patient places one ankle on • Anxious, fidgety patient the opposite knee and allows the ipsilateral knee • Staring eyes (lid retraction) to fall outwards (external rotation at the hip) to • Sweating form a figure ‘4’. If this causes pain over the sacroiliac joint, sacroiliitis should be suspected. Hands Shake their hands 3. Exclude extra-articular manifestations from head to toe: • Warm and sweaty or cool and dry? • Fine tremor – hands outstretched with a piece If a spondylarthropathy is suspected (eg ankylos- of paper resting on fingers ing spondylitis), a focused history should include • Pulse questions related to the extra-articular manifesta- • Rate tions associated with these conditions. • Rhythm (AF often occurs in thyrotoxicosis) • Eyes : acute uveitis • Volume (typically large volume and • Mouth: mucosal inflammation manifesting as collapsing in hyperthyroidism) oral ulceration is common • Thyroid acropachy – a rare feature of Graves’ • Chest: apical fibrobullous disease (1%) disease • Cardiac: aortic root dilatation and associated • Tar staining – Graves’ ophthalmopathy is aortic valve incompetence worse in smokers • Abdomen: 15–20% will develop symptomatic Crohn’s disease (stoma present?). Look for Neuromuscular manifestations evidence of amyloidosis (hepatomegaly, • Reflexes: Slow relaxing in hypothyroidism; evidence of renal failure or replacement brisk in thyrotoxicosis therapy) • Proximal myopathy: Thyrotoxicosis – ask the • Nervous system: paraesthesia, signs of cord patient to stand from a chair unaided compression • Feet : Achilles tendonitis and plantar fasciitis Dermatological manifestations of thyroid disease • Graves’ dermopathy Examination of thyroid status Sheet-like myxoedema – coarse diffuse skin with non-pitting oedema The assessment of the thyroid status of a patient Nodular localised – violaceous infiltrative waxy is a fundamental clinical skill that should not area on the shin, resembling erythema nodosum present difficulties provided that the following Horny – papilliform irregular firm red dermopathy scheme is followed. For obvious reasons, patients on shin/upper foot 402 MRCP PACES Manual

Examination of the thyroid gland Note: Soft – ‘like lips’, firm – ‘like the tip of the nose’ and hard ‘like the forehead’ is a good aide Follow the sequence of inspection, palpation and memoir to remember (but not repeat in the exam) percussion. If you find a goitre, it would be when thinking about how to measure the consis- prudent to comment on the thyroid status, com- tency of the goitre. bining reported symptoms and a formal examina- • Diffuse or nodular tion of thyroid status as above. You must also • If nodular – multinodular or a single comment on the most likely aetiology. nodule • Tender – suggests thyroiditis Inspection • Lymphadenopathy Ask the patient to swallow a sip of water and • Tethered – this suggests cancer. look for upward movement of the thyroid gland. NB a thyroglossal cyst will move upwards both Percussion on swallowing and protrusion of the tongue, and Percuss gently for retro-sternal extension. can be trans-illuminated. Auscultate Is there any evidence from the history or exam- Bruit – classically occurs in Graves’ thyrotoxi- ination that the thyroid is compressing any of the cosis. following: • Trachea • monophonic syncope (rare), but the Examination of thyroid eye disease history may suggestmsome dyspnoea, particularly on lying flat This may not be necessary in the exam unless the • Recurrent laryngeal nerve patient is complaining of eye symptoms or if on • hoarseness inspection the patient has obvious thyroid eye • Oesophagus disease. • dysphagia, very rarely odynophagia • Venous return from the head Note that lid lag and lid retraction are signs of • superior vena cava obstruction (very rare) hyperthyroidism rather than Graves’ disease, although the two may clearly co-exist. Look carefully for a scar – previous hemi/total thyroidectomy Lid retraction Palpation Indicated by visible sclera above the superior limbus of the cornea Stand behind the patient and gently palpate the gland, located two finger widths below the thyr- This results from sympathetic stimulation of leva- oid cartilage, with one hand on each side and tor palpabrae superioris of any aetiology, eg the neck gently flexed. thyrotoxicosis, anxiety, â-agonists. • If a goitre is present, comment on its: • Size Lid lag • Consistency Ask the patient to follow the slow downward • soft movement of your finger at a distance of about • firm 50 cm. The upper lid lags behind the descending • hard eyeball. Integrated Clinical Assessment 403

Look for clinical features of Graves’s of acuity may be unnecessary, unless there is a¡ecting the eyes a high suspicion this has been compromised. • Since the most important concern of Exophthalmos Graves’ ophthalmopathy is a threat to the Sclera visible below the inferior limbus of the sight, it is critical to assess vision in the cornea with the patient sitting at the same level following fashion. as you and looking straight ahead. • Acuity using a Jaeger chart or Snellen chart (+ pinhole) This sign only occurs in Graves’ disease (cv), the • Colour vision – either using Ishihara term is synonymous with proptosis; it can be plates or a red pin to look for unilateral, although a retro-orbital tumour should desaturation always be excluded. • Fields – compression of the nerve head at the orbital apex can cause constriction Other features of Graves’ of the fields • Periorbital oedema • Ophthalmoscopy • Chemosis • Is there papilloedema or consecutive • Conjunctival injection optic atrophy? • Opthalmoplegia • If closure is poor or you have concerns about the cornea you may want an If Graves’ ophthalmopathy is present on inspection ophthalmologist to perform slit lamp it is necessary to perform a more detailed examina- examination to look for corneal scars or tion and take further history. ulcers – you should state this to the • Are the eyes painful in any way? Are they examiner. One can get a reasonable gritty or dry? Also ask if any part of the view with a direct ophthalmoscope, but subsequent exam causes pain or discomfort. not good enough to preclude a proper • Is eyelid closure adequate? examination. • With exophthalmos there is a greater volume of eye to be covered with each blink, the frequency of blinking is reduced Examination of a skin lesion/rash and the time of each blink is increased. • Ask patient to follow your finger (and to say if The cases in the Station 5 ICA they experience diplopia) as you test all require a special mention. Classically, the history directions of gaze. associated with the condition may not be long or • Limitation of upward gaze is the most detailed, and most of the differentiating informa- common abnormality in Graves’ tion is gleaned from examination. Furthermore, ophthalmopathy. the presentation of the examination is essential • However, the combination of enlarged too. Remember that many dermatological condi- ocular muscles +/ À subsequent fibrosis tions reflect underlying medical conditions that may lead to complex ophthalmoplegia should not be overlooked. that is not explained by either single nerve or muscle disease. It is important to ensure you are fluent with the • Ptosis – a very rare occurrence in either common terminology: Graves’ disease or hyperthyroidism. Its presence should raise the possibility of co- Macule Circumscribed area of erythematous existent myasthenia gravis. change without elevation • Acuity (see page 405) – full assessment (with Papule Solid raised lesion , 1 cm in size ophthalmoscopy) rather than gross assessment Nodule Solid raised lesion > 1 cm in size 404 MRCP PACES Manual

Plaque Circumscribed elevated confluence of Examination of the eyes papules > 1 cm in size Pustule Circumscribed area containing pus As with the dermatology ICA cases, the history Vesicle Circumscribed fluid-filled area associated with an ophthalmological condition , 1 cm in size may be brief, but the examination is crucial. Bulla Circumscribed fluid-filled area Taking a history and examining simultaneously > 1 cm in size can be easily achieved. Be cautious, however, since red flag symptoms must not be missed When examining the patient, you need to expose (such as in papilloedema or diabetic retinopa- the patient as much as the situation will allow thy), as some conditions reflect very serious (which the examiner should guide) and inspect underlying medical conditions or even malig- the patient as a whole, considering: nancy. • Is there a rash and what is its distribution? • Is there any hair growth or loss? This does not mean, however, that all aspects of • Are there any features of systemic disease? the eye examination must be completed in the examination. For example: And more specifically: In a patient with a painful red eye you may • If there is a rash – is it red or not? consider examining: • Is the rash macular, papular, in patches or plaques? • Visual acuity – may be reduced • Are there scales or evidence of excoriation? • Fundoscopy – may show anterior uveitis or • Are there fluid-filled lesions? corneal scarring • Are these vesicles, pustules or blisters? • Pupil – may be irregular, small, or unreactive • Are there signs of systemic disease giving However, you might expect visual fields and eye clues as to the aetiology of the skin lesion, eg movements to be normal and tell the examiner a colostomy bag suggesting ulcerative colitis? you would include the examination of these as On presenting your findings, use the correct part of your further investigations. terminology and be succinct. If there is a sug- gestion from the history that the dermatological In a patient with a history of progressive dete- condition is associated with an underlying condi- rioration in vision you may consider examining: tion (eg erythema nodosum and inflammatory • Visual acuity bowel disease), then an examination of the rele- • Fundoscopy – retinitis pigmentosa, diabetic vant system may be necessary. retinopathy • Eye movements For example: • Visual fields – peripheral loss first ‘There is a vesicular rash bilaterally on the ex- tensor aspects of the elbow, with associated evidence of excoriation, on a background of a It is important to tailor your examination according patient with symptoms consistent with coeliac to the presenting complaint. disease, suggesting that this is dermatitis herpeti- formis.’ However, it is still important to know how to This gives a clear and precise description of the examine the eye in a systematic way. Fundo- rash, its location, associated excoriation and links scopy is a core skill for a practising clinician and it to the most likely underlying aetiological MRCP candidate, but one with which many have cause. considerable difficulty. Integrated Clinical Assessment 405

General appearance of the eye Where it is first visible gives a good indication of Look carefully for symmetry of the eyelids, pupils peripheral vision. and general eye movements. In particular, check for signs such as ptosis, lid retraction or irregular Eye movements (see p 225) pupils.

Look for clues suggesting decreased visual acuity, Fundoscopy eg white stick, adjacent Braille books, or for This should be performed in a dark room – if it is evidence of other systemic diseases such as dia- not dark ask to turn the lights off. betes, eg glucose testing sticks, diabetic drinks, foot ulcers or evidence of co-existent dialysis. A mydriatic (dilating the pupil) such as tropica- mide may have been used. It is essential that you Visual acuity examine the patient’s right eye with your right Assess acuity with the patient wearing their nor- eye and their left eye with your left eye. The latter mal glasses. is a routine many candidates have difficulty with, but failure to do this, for whatever reason, is Ask the patient if they can see from each eye, frowned upon. and then assess individually by use of a Snellen chart. Visual acuity is defined as V ¼ d/D where Find the red reflex, keep it in view and get in d ¼ distance at which numbers are read and close! D ¼ distance at which they should be read. Although the temptation and tendency is to go Pupillary re£exes straight to the retina, you should get into the Each eye should be tested in turn for direct and routine of gradually racking down through the consensual pupilary reflexes with a pen torch lens strengths, examining first the front of the eye while the patient faces forward with eyes focused and gradually, by reducing the strength of the directly ahead. lens, moving to the retina, which is usually ob- served with a zero or a slightly negative lens, Visual ¢elds depending on whether or not the patient is myo- Visual fields should be assessed by confrontation pic. with you sitting approximately 1 metre from the patient and with your eyes at the same level. The disc and each quadrant of the retina should When assessing the patient’s right eye, ask them be identified and studied in turn. to cover up their left eye, and after covering your right eye, slowly bring an object in from the Disc – note its shape, colour (pale in optic periphery in a plane equal between you and the atrophy), margins and if there is papilloedema. patient.

What should the object be? A moving finger is Retinal blood vessels – examine the vessels, commonly used and may be fine for a screening noting their diameter (arteries are narrower than test if examining the cranial nerves. The top of a veins) and the point at which they cross. Is there pen, placed in the quadrants in turn, is better, but A–V nipping? do not bring it inwards too quickly, as the patient must have the time to say when they first see it. Look for arterial emboli (arteries become much thinner or thread like) or venous thrombosis A red pin can be used for assessing central (veins become engorged with surrounding hae- colour vision that is located around the macula. morrhages). 406 MRCP PACES Manual

Each retinal quadrant should be examined for the Considering the cases you might be asked to see; presence of: look around the bedside for clues. • Haemorrhages • dot, blot or flame shaped • Microaneurysms • Pigmentation FINAL THOUGHTS • Exudates, distinguish between: • hard – white/yellow and shiny with well- Remember that Station 5 is very different from defined edges the other stations. You are somewhat liberated • soft (‘cotton wool spots’) from the regimented examination routines of • The presence of new vessels should be the other stations. However, you need to gather identified and any previous photocoagulation a great deal of information and communicate a scars. plan to the patient in a very short space of The macula – It should be specifically examined time. It is perhaps the most accurate and global as the surrounding area is, as the name suggests, reflection of how you will perform as a registrar the site of diabetic maculopathy. in clinic or on-call. This should not be intimi- dating – if in doubt just resort to what you Examine the peripheries of the retina, looking would normally do in exactly that scenario at particularly for evidence of retinitis pigmentosa. work! Integrated Clinical Assessment

1. Acromegaly 20. Herpes zoster 2. Addison’s disease 21. Horner syndrome 3. Ankylosing spondylitis 22. Hypothyroidism 4. Anterior uveitis 23. Impetigo 5. Asthma 24. Marfan syndrome 6. Atrial fibrillation 25. Osteogenesis imperfecta 7. Coeliac disease 26. Paget’s disease 8. Congestive cardiac failure 27. Papilloedema 9. Cranial nerve III palsy 28. Pemphigus 10. Crohn’s disease/bloody 29. Pleural effusion diarrhoea 30. Psoriasis 11. Cushing’s disease 31. Psoriatic arthropathy 12. Dermatomyositis 32. Pyoderma gangrenosum 13. Diabetic retinopathy 33. Retinitis pigmentosa 14. Eczema 34. Rheumatoid arthritis 15. Erythema nodosum 35. Scleroderma 16. Gout 36. Systemic lupus 17. Graves’ disease erythematosus 18. Henoch–Scho¨nlein purpura 37. Tremor 19. Hereditary haemorrhagic 38. Turner syndrome telangiectasia 39. Vitiligo examination of relevant area/system and Integrated Clinical Assessment 409 targeted Assess for cranial nerves palsies,IV especially and nerves VI III, (if raisedExamine intracranial for pressure) carpal tunnel syndrome/scarsprevious from decompression Myopathy (especially proximal) and arthropathycommon, is but no synovitis willExamine be for found associated endocrine features,goitre, including gynaecomastia and/or hirsutism Perform a cardiac and abdominalsymptoms, examination looking if for has associated cardiomegalyhepatomegaly and Measure blood pressure, assess forreflect glycosuria associated (may impaired glucose tolerance/diabetes mellitus) Prominent supraorbital ridges, nose andPronounced lips jawline (prognathism) Macroglossia and increased interdental separation Enlarged sweaty hands with thickened skin Examine visual fields for bitemporalThere hemianopia. may be optic atrophyinto if the the parasellar adenoma region extends : this 36-year-old man has presented with headaches. • • • • • • • • • • • Begin a continue while taking the history General appearance (see Figure 5.1) Any old photographs of the patient available? In addition ‘For the last 3 months I have been having headaches, and I am Thank the patient Please assess him. Introduce yourself commented that my appearance has changed.’ RESPOND TO PATIENT’S CONCERNS (1–2 min) anything else troubling you that we haven’t covered? SCENARIO 1. ACROMEGALY circumstances: especially if : any co-morbidity that might : sudden headache, diplopia, : hypertension, diabetes, colonic of the presenting complaint and relevant – antihypertensives, oral hypoglycaemics : ‘You said at the beginning that the main problem was headaches, change in vision and appearance. Is there which is caused by a benign growth in theestablish pituitary the gland diagnosis that by produces doingto too some stop much urgent driving growth investigations. immediately hormone As because your of vision the is risk impaired, toneed I yourself to am and arrange afraid other an that road urgent youthis users MRI will growth of have has your affected pituitary your gland vision and ask the eye specialists to see you to assess to what extent Confirm the information you’ve been given and patient details ‘What is the main problem from your point of view?’ worried about my vision as I have recently crashed my car into a parked car when driving along the road. A friend has CALLBACK 1. I think that your headaches,2. change in vision and For appearance your are headaches, due I to can a give condition you resulting some from painkillers acromegaly, as3. a temporary measure. However, I it am is going very to important organise to some urgent blood tests (glucose tolerance tests, IGF-1, other pituitary hormones). We will systems enquiry. This should includeendocrine screening disturbance for (any aspect ofpituitary the axis hypothalamic– can be disrupted)neurology and (eg for diplopia, localising visual fieldsyndrome) defect, carpal tunnel Identify alarm symptoms New condition Focused history ptosis (may imply pituitary apoplexy).may Polyuria/polydipsia imply either diabetes insipidus or diabetesPre-existing conditions mellitus polyps, sleep apnoea Social/occupational/family driving professionally (eg taxi driver, group 1 driver) Drug history DRUG Other medical conditions preclude hypophysectomy (eg bleeding diathesis)?

1–2 minutes 5 minutes 1–2 minutes 410 MRCP PACES Manual

Acromegalic facies Large tongue

Increased interdental spacing Figure 5.1 Acromegaly Differential diagnosis Clinical communication skills and Physical examination and identifying Clinical judgement and maintaining managing patient concerns physical signs patient welfare

Craniopharyngioma Symptoms of raised intracranial pressure Visual defects In contrast to other pituitary Diagnosed by characteristic appearances A relatively rare tumour, (headache, nausea and vomiting), visual adenomas; this starts by causing a on pituitary imaging consisting of both solid field defects and endocrine deficiencies (eg bitemporal inferior quadrantanopia, Transcranial frontal surgery is the major and liquid components, pubertal delay from gonadotrophin because the suprasellar lesion initially modality of treatment, either with or which arises in Rathke’s deficiency) compresses the optic chiasma from above. without radiotherapy pouch Over time, this may progress to bitemporal A large proportion of patients will require hemianopia hormone replacement

Pituitary adenoma Symptoms of mass effects, visual field Mass effects Tumours are often diagnosed on pituitary These may be either defects and endocrine disorders May first present with headache, nausea imaging, with MRI being the preferred hormone secreting or and vomiting modality non-secreting Visual defects: initially bitemporal superior Assessment of pituitary function to assess quadrantanopia, as outlined above for hormone hypersecretion and/or any Endocrine disorders associated hormone deficiencies In hormone-secreting adenomas, clinical Visual field testing should be performed at features reflect the particular hormone the start of treatment and thereafter to being secreted excessively (eg Cushing’s assess efficacy of treatment disease from a corticotroph adenoma) Transsphenoidal surgery, either with or As tumours expand, they may disrupt other without radiotherapy. Careful ongoing hormone axes and cause symptoms and pituitary function assessment will be signs of hormone deficiency needed to assess if long-term hormone replacement is indicated

Pseudoacromegaly Symptoms very similar to true acromegaly Clinical phenotype similar to acromegaly Investigation focuses on exclusion of nertdCiia seset411 Assessment Clinical Integrated Excessive insulin as described below. No visual field defects without visual field defects growth hormone-secreting adenoma secretion and Disturbance of other hormone axes rare associated insulin resistance 412 MRCP PACES Manual

Clinical judgement and Acromegaly incurs a significantly increased mor- maintaining patient welfare tality rate (estimated as at least twice as high) as the healthy population, with those with the high- Acromegaly is a clinical syndrome resulting from est GH levels at the greatest risk of premature the hypersecretion of growth hormone (GH). Most death. This is mostly attributable to increased cases are caused by GH-secreting adenomas situ- cardiovascular disease. ated in the anterior pituitary. Approximately three-quarters of these are macroadenomas (ie Investigations .10 mm diameter) at the time of diagnosis. These 1. Establish the presence of GH excess adenomas account for approximately a third of all • Random GH plasma levels in themselves are hormonally secreting pituitary adenomas. Rare not diagnostic, because these fluctuate greatly causes of acromegaly include growth hormone- between individuals and at different times of releasing hormone (GHRH) secretion from the day. However, serum insulin-like growth hypothalamic tumours, or ectopic GHRH or GH factor 1 (IGF-1) levels are elevated in almost secretion (eg from neuroendocrine tumours). all patients with acromegaly. • The most effective means of diagnosing GH Adenomas tend to grow very slowly; it is esti- excess is with an oral glucose tolerance test; mated that there is an average delay of more than although GH levels suppress to very low 10 years between initial symptoms and diagnosis. levels after a glucose load in healthy The most consistent early symptoms (and those individuals, GH levels will fail to suppress or most likely to form the stem of a Station 5 paradoxically rise in patients with question on the condition) include headaches acromegaly. and visual disturbance. Other relatively early features are those of endocrine disturbance, parti- 2. Establish the aetiology (Table 5.1) cular gonadotrophin deficiency, ie oligo-/amenor- • Pituitary imaging should be performed; MRI is rhoea in women, or erectile dysfunction, loss of the most sensitive modality. libido and testicular atrophy in men. As the • Establish whether a pituitary adenoma is condition progresses, a large number of organ functional. systems may be affected, and a number of physi- cal signs may develop, as described in the differ- 3. Look for complications ential diagnosis table. There may also be signs of • Visual perimetry: to establish visual fields. treatment of the condition (eg scars around the • ECG, echocardiogram and chest radiograph: nose reflecting previous transsphenoidal surgery). if cardiomyopathy is suspected.

Table 5.1 Differentiation between anterior and posterior pituitary causes

Anterior pituitary Posterior pituitary

Corticotroph: 9 am cortisol. If ,500 nmol/L, perform ADH secretion – plasma and urinary sodium and a dynamic test, ie insulin or glucose tolerance test osmolality. A water deprivation test may also be Thyrotroph: T 4, T 3 and TSH required Gonadotroph: LH, FSH, testosterone and SHBG or estradiol Lactotroph: prolactin Integrated Clinical Assessment 413

• Oral glucose tolerance test: used to make the including cranial neuropathies and visual diagnosis, but also to establish the presence field impairment. of associated impaired glucose tolerance/ diabetes mellitus. • Medical Nerve conduction studies: if an associated • Somatostatin analogues (eg octreotide or carpal tunnel syndrome is suspected. • lancreotide) are administered subcutaneously. Sleep studies: if obstructive sleep apnoea is They reduce serum GH in 90% of patients, suspected. • and to a level of GH that will prevent Colonoscopy: surveillance for polyps and adenoma growth in approximately 50% of colorectal malignancies, a recognised patients. Side effects are nausea and complication of acromegaly. • gallstones (approximately 50% by 5 years). Bone profile and parathyroid hormone: the • Dopamine agonists (eg bromocriptine, combination of acromegaly and cabergoline) are effective in only 10% of hyperparathyroidism raises the possibility of patients. multiple endocrine neoplasia type 1 (MEN-1) • Pegvisomant is a GH receptor antagonist, syndrome; there should be a low threshold to administered subcutaneously daily, which investigate for a possible pancreatic tumour. normalises serum IGF-1 in .90% of patients, helping to reduce many of the clinical Treatment features. However, it does not reduce GH Surgical • secretion or constrain tumour growth, so it is Transsphenoidal hypophysectomy is currently reserved for patients with ongoing successful in up to 90% of patients with disease despite surgery, radiotherapy and microadenomas and 50% of those with other medical . macroadenomas in the hands of the most experienced neurosurgeons. • Long-term deficiency in one or more of the Maintaining patient welfare pituitary hormones will develop in It is important in managing acromegalic patients approximately 70% of patients. to think not only about the immediate treatment for the condition, but also about the means of Radiotherapy minimising long-term complications. Candidates • Pituitary external irradiation is indicated for can show that they are aware of these by sug- patients unfit for or not cured by surgery. It gesting colonoscopic screening for colonic ma- results in a 50% decline in GH levels by lignancy (this is currently recommended every 3 2 years, but with a continuing exponential years), and minimising the patient’s risk of decline thereafter. cardiovascular disease by optimising their blood • There is a significant risk of late pressure, lipid profile and smoking cessation hypopituitarism, with other complications advice.