mrcp paces manual

MRCPPACES

MANUAL

Louise PealingMA Hons (Cantab) MBBSMScMRCPMRCGPGeneral Practitioner and Clinical Research Fellow, Nuffield Department of Primary

Care Health Sciences, University of Oxford

BenjaminMullish MBBChirMA (Cantab) MRCPAFHEASpecialty Registrar/Academic Clinical Fellow, Gastroenterology and Hepatology,

St Mary’s Hospital, London

PhilipJ Smith BMedSci (Hons) BMBS (Hons) MRCPMSc (Nutrition)Gastroenterology Specialist Registrar and MRC Clinical Research Training Fellow

Gastroenterology Department, University College London Hospital, London

Edited byDouglas CMacdonald BM (Hons) BSc (Hons) MRCPPhDConsultant Hepatologist, Royal Free London NHS Foundation Trust

Royal Free Hospital, London

Contents

Preface vi

Introduction vii

STATION1 ^ Respiratory and Abdominal Examinations 1The abdominal examination 3Abdominal scenarios 7The respiratory examination 47Respiratory scenarios 52

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

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

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

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

Abbreviations 599

Index 605

v

Station 3Neurological scenarios

1. Multiple sclerosis

2. Parkinson’s disease

3. Motor neuron disease

4. Hemiparesis

5. Spastic paraparesis

6. Cervical myelopathy

7. Syringomyelia/Syringobulbia

8. Myotonic dystrophy

9. Myasthenia gravis

10. Muscular dystrophy

11. Charcot–Marie–Toothdisease

12. Friedreich’s ataxia

13. Cerebellar syndrome

14. Peripheral neuropathy

15. Facial nerve palsy

16. Wasting of the smallmuscles of the hand

17. Median nerve palsy

18. Ulnar nerve palsy

19. Radial nerve palsy

20. Common peroneal nervepalsy and L4–5 root lesions

21. Nystagmus

22. Ophthalmoplegia

23. Visual field defect

24. Dysphasia

25. Bulbar and pseudobulbarpalsy

26. Brain-stem syndromes

27. Cerebellopontine anglelesion

28. Jugular foramen syndrome

29. Old polio

30. Involuntary movements andHuntington’s disease

SCENARIO 17. MEDIAN NERVE PALSY

Identifying clinical signs

The median nerve serves the intrinsic small mus-cles of the hand not served by the ulnar nerve.The median nerve also serves muscles of thevolar surface of the forearm, which includes themain wrist flexors.

The most common median nerve palsy appearingin the PACES examination is a carpal tunnelsyndrome. Indeed this is the most common per-ipheral mononeuropathy. However, there can bemore proximal lesions of the median nerve thatoffer more objective clinical signs.

Distal median nerve lesions (carpaltunnel syndrome)Inspection: the hand shows thenar wasting (alate sign) with hypothenar and first dorsal inter-osseous sparing. The thumb is externally rotatedand adducted into the plain of the palm (Figure3.18) due to weakness of opponens pollicis andabductor pollicis, and some refer to this as ‘ape-hand’ deformity (but it is best to describe theposition of the thumb). The index (first) andmiddle (second) fingers may also be held inextension due to weakness of flexor digitorum

profundus I and II and flexor digitorum super-ficialis, and unopposed action of the radialnerve-innervated finger extensors. Some call thisthe ‘papal sign’. Look for surgical or traumaticscars at the wrist, palmar crease, forearm orelbow.

Motor function: there is weak thumb abduction(abductor pollicis brevis), tested for by asking thepatient to place the hand palm up and point thethumb up to the sky perpendicular to the planeof the palm and test against resistance of yourthumb. There is weakness of thumb opposition,tested for by asking the patient to oppose thethumb with the little finger and stop you pullingthem apart. There may be normal flexion at thethumb MCP joint due to intact ulnar nerve in-nervation of flexor pollicis brevis. There is nor-mal flexion of the thumb at the IP joint due tointact innervation of flexor pollicis longus in theforearm. Arm pronation and wrist flexion arealso normal if the median nerve lesion is distal.

Sensory loss: there is sensory loss in the palmaraspect of the thumb and lateral two and a halffingers but normal sensation over the thenareminence (supplied by the palmar cutaneousbranch of the median nerve which does not passthrough the carpal tunnel).

Additional tests: you can ask to perform Tinel’stest – tapping over the median nerve at the wristmay cause paraesthesia in the distal median dis-tribution. Phalen’s test involves flexing the wristto 908 for at least 60 seconds, which may causeparaesthesia in the distal median distribution.The median nerve compression test involvespressing on the palmar aspect of the wrist for upto 60 seconds, which may cause paraesthesia inthe distal median distribution.

Additional signsExamine for signs of other diseases associatedwith carpal tunnel syndrome such as:

Figure 3.17 Median nerve cutaneous dis-tribution

306 MRCP PACES Manual

• Hypothyroidism: goitre, slowly relaxingreflexes, pretibial myxoedema

• Acromegaly: supraorbital ridge, prognathism,interdental separation, bitemporalhemianopia, large doughy hands,hypertension

• Rheumatoid arthritis: deforming arthropathy,elbow nodules, steroid skin changes,episcleritis/scleritis, interstitial lung disease

• Diabetes mellitus: finger-prick testing,peripheral neuropathy, retinopathy

• Gout: deforming arthropathy and gouty tophion hands, ears and feet.

Proximal median nerve lesionsPronator syndrome: compression of the mediannerve can occur as it passes between the twoheads of the pronator teres, high in the volaraspect of the forearm. It can present with purelysensory symptoms of pain over the volar surfaceof the forearm at rest or with forearm pronation.There will be sensory loss within the mediandistribution including the thenar eminence (un-like carpal tunnel syndrome).

Anterior interosseous nerve palsyThis nerve has branches to flexor digitorum pro-fundus I and II, flexor pollicis longus and prona-tor quadratus. It is typically affected by amidshaft fracture of the radius, excessive exerciseor penetrating injuries of the forearm. There isweakness of the thumb and index finger flexion,best shown with the ‘okay’ sign, ie flattened dueto failure of distal flexion (see Figure 3.17). Thethenar eminence muscles are spared. There is nosensory loss.

Di¡erential diagnosis

• Elbow lesions:• Fracture: supracondylar fractures are most

common• Dislocation• Compression: ligament of Struthers

• Forearm lesions:• Fracture: midshaft radial fracture causing

anterior osseous nerve palsy• Injury: penetrating injuries of the forearm

Pronator teres

Flexor carpi radialis

Palmaris longus

Flexor digitorumsuperficialis

Median nerve

Anterior interosseous nerveFlexor digitorum profundus I & II

Flexor pollicis longus

Pronator quadratus

Second lumbrical

Abductor pollicis brevis

Flexor pollicis brevis

Opponens pollicisFirst lumbrical

Figure 3.18 Route and muscles innervated by the median nerve

The Cardiovascular and Neurological Examinations 307

• Compression: pronator teres syndrome• Wrist lesions:

• Fracture/trauma• Carpal tunnel syndrome (CTS).

Clinical judgement andmaintaining patient welfare

CTS is caused by compression of the mediannerve within the carpal tunnel, which is boundby the carpal bones below, flexor retinaculumabove, radially by the scaphoid and trapezium,and medially by the pisiform and hamate.

It is most commonly idiopathic, but associatedconditions include: pregnancy, menopause, hy-

pothyroidism, diabetes, acromegaly, rheumatoidarthritis, gout, renal failure, multiple myelomaand amyloidosis.

InvestigationsElectrophysiology: nerve conduction studies andEMG.

Imaging: seldom needed, but MRI might be con-sidered.

Management• Treat any underlying associated conditions• Physiotherapy and splint the wrists with a

degree of dorsiflexion• Steroid injections into the carpal tunnel area• Surgical decompression of the carpal tunnel.


SCENARIO 18. ULNAR NERVE PALSY


The ulnar nerve serves most of the intrinsic smallmuscles of the hand. However, the ulnar nervealso supplies two important extrinsic muscles ofthe hand: flexor carpi ulnaris and flexor digitor-um profundus 4 and 5; it is important to remem-ber this when considering a claw hand deformity.Clues pointing towards an ulnar nerve lesioninclude unilateral signs, clawing of the hand andwasting of the hypothenar eminence with sparingof the thenar eminence.

Distal ulnar nerve lesion (distal to theelbow)Inspection: with the hands held prone there isdorsal guttering and marked wasting of the firstinterosseous between the thumb and index fin-ger. The hand shows a claw deformity (you mightwant to say a clawed appearance) with hyperex-tension at the fourth and fifth MCP joints andflexion at the fourth and fifth PIP and DIP joints,due to paralysis of the medial lumbricals. Thereis slight ulnar deviation of the fifth finger (knownas Wartenburg’s sign) from unopposed action ofextensor digiti minimi (which inserts into theulnar side of the little finger and is innervated by

the radial nerve). With the hands held supine,marked hypothenar wasting with sparing of thethenar eminence can be seen, and no wasting ofthe forearm muscles. Look for scars or deformityfrom trauma, surgery or arthritis around the fore-arm and wrist.

Motor function: there is weak abduction (dorsalinterossei) and weak adduction (palmar inter-ossei) of the fingers. There is weak thumb adduc-tion (adductor pollicis) as demonstrated byattempting to grip a piece of paper held betweenthe borders of the index finger and extendedthumb. Grip can be maintained only using thumbflexion (intact flexor pollicis longus served by themedian nerve), known as Froment’s sign. There isintact flexion of the fourth and fifth DIP joints,as shown by the marked ulnar claw appearance(it is difficult to test actively when the fingers areheld in fixed flexion). There is intact medial/ulnar flexion of the wrist.

Sensory loss: in the ulnar distribution over thefifth finger (see Figure 3.19), adjacent ulnar sideof the fourth finger, ulnar side of the palm anddorsal aspect of the hand. (In distal ulnar nervelesions this sensory loss may be variable orpresent only in the fifth finger.) Test along theradial side of the fourth finger and up the ulnarborder of the wrist and forearm to check that thisdoes not represent a C8–T1 lesion instead (mus-cle wasting would also be different, see Scenario16).

Additional tests: you can ask to perform Tinel’stest tapping along the course of the ulnar nervefrom the wrist along the ulnar border of theforearm and up to the elbow, which may elicittingling from irritation of the nerve.

Proximal ulnar nerve lesion (at theelbow)Inspection: with the hands held prone there isdorsal guttering and marked wasting of the first

Figure 3.19 Ulnar nerve cutaneous distri-bution


interosseous between the thumb and index fin-ger. The hand shows a mild/no clawed appear-ance with hyperextension at the fourth and fifthMCP joints but only mild flexion at the fourthand fifth PIP joints and no flexion at the DIPjoints. This is known as the ulnar paradox, withproximal lesions resulting in less clawing. Thereis slight ulnar deviation of the fifth finger knownas Wartenburg’s sign (see above). With the palmsfacing up, there is marked hypothenar wasting,with sparing of the thenar eminence. In addition,there is wasting of the ulnar border of the fore-arm. Look for scars or deformity from trauma,surgery or arthritis around the elbow.

Motor function: there is weak abduction (dorsalinterossei) and weak adduction (palmar interos-sei) of the fingers. There is weak thumb adduc-tion (adductor pollicis) with Froment’s sign (seeabove). There is loss of flexion of the fourth andfifth DIP joints (loss of flexor digitorum profun-dus with high lesions) as shown by holding thefourth and fifth MCP and PIP joints extended andasking the patient to try to flex the fourth and fifthDIP joints (this takes practice to demonstrateeffectively!). There is weakness of medial/ulnarflexion of the wrist (flexor carpi ulnaris) notfound in distal lesions (see above).

Sensory loss: this occurs in the ulnar distributionover the fifth finger, ulnar side of the fourth finger,ulnar side of the palm and dorsal aspect of thehand (see Figure 3.19).

Additional tests: you can ask to perform Tinel’stest, tapping along the course of the ulnar nervefrom the wrist up the ulnar border of the forearmtowards the elbow, which may elicit tingling fromnerve irritation.

The elbow flexion test can be used to test forulnar nerve compression at the elbow, particu-larly in cubital tunnel syndrome. The elbow isflexed fully with the forearm supinated, and with-in 60 seconds the patient starts to feel pain ortingling in the fourth and fifth fingers.


Elbow lesions• Fractures:

• Supracondylar fractures are most commonand late complications of fracture/surgerycan include cubitus valgus deformity ofthe elbow joint.

• Dislocation:• Arthritis: bony spurs and narrowing of the

ulnar groove• Compression: cubital tunnel syndrome

describes constriction under the fibrousarch of the two points of insertion offlexor carpi ulnaris. Certain occupationsare more at wrist such as secretaries (fromleaning on elbows) and decorators (fromrepeated elbow flexion and extension).

Wrist lesions• Fractures• Ganglion• Tumour• Mononeuritis multiplex.


InvestigationsImaging: plain radiograph of elbow joint, ultra-sonography of the cubital tunnel or MRI.

Nerve conduction studies localise the site of thelesion

Blood tests: FBC, CRP, ESR, anti-nuclear antibodies(ANAs), anti-DNA, pANCA (perinuclear anti-neutrophil cytoplasmic antibody or ANCA), cAN-CA (cytoplasmic ANCA), rheumatoid factor, hepa-titis B and C serology (mononeuritis multiplex).

ManagementConservative: avoidance of aggravating factors,physiotherapy, splinting, NSAIDs.

Surgical: transposition of the ulnar nerve and/ordecompression of the cubital tunnel.


SCENARIO 19. RADIAL NERVE PALSY


The radial nerve serves the extensors of theelbow, wrist and fingers. With knowledge of theanatomy of radial nerve innervation it is possibleto describe the level of the lesion giving rise toclinical signs (see Figure 3.20).

Radial nerve lesion at the axillaInspection: there is wrist drop and slight fingerflexion but with no wasting of the hand muscles.Look for surgical or traumatic scars anywherealong the route of the radial nerve from the axillato the wrist.

Posterior interosseousnerve

Deltoid

Triceps, long head

Triceps, lateral head

Brachioradialis

Extensor carpi radialis longus

Extensor carpi radialis brevis

Supinator

Extensor carpi ulnaris

Extensor digitorum

Abductor pollicis longusExtensor pollicis longusExtensor pollicis brevis

Extensor indicis

Teres minor

Triceps, medial head

Radial nerve

Figure 3.20 Route and muscles supplied by the radial nerve


Motor function: there is weakness of all radiallyinnervated muscles:

• Weakness of elbow extension and flexion(midway between supination and pronation)

• Forearm supination (tested with arm by theside and attempted supination of forearmagainst resistance applied to the patient’shand)

• Wrist extension and finger extension at theMCP joints.

Both triceps and brachioradialis (biceps) deeptendon reflexes are absent.

Sensory loss: this occurs over the triceps, poster-ior forearm and first dorsal interosseous.

Radial nerve lesion in the spiral grooveof the humerusInspection: there is wrist drop and slight fingerflexion but with no wasting of the hand muscles.Look for surgical or traumatic scars.

Motor function: there is weakness of all radiallyinnervated muscles below the triceps. There isweakness of elbow flexion (midway betweensupination and pronation) but elbow extension isintact (triceps innervation above the spiral grove).There is weakness of forearm supination, wristextension and finger extension at the MCP joints.The triceps reflex is preserved but the brachio-radialis (biceps) deep tendon reflex is absent.

Sensory loss: there is sensory loss over the poster-ior forearm and first dorsal interosseous. Theremay be variable loss of sensation over the tri-ceps.

Radial nerve lesion con¢ned to theposterior interosseous nerveInspection: there is wrist drop and slight fingerflexion but with no wasting of the hand muscles.Look for surgical or traumatic scars.

Motor function: there is weakness of radiallyinnervated muscles below the supinator (the su-pinator nerve comes off before the posterior inter-

osseous nerve dips beneath the fibrous arcade ofFrohse (‘supinator arch’) which is the commonentrapment area). There is intact elbow flexion(midway between supination and pronation) andintact elbow extension and forearm supination.There is weakness of wrist extension and fingerextension at the MCP joints. Triceps and bra-chioradialis deep tendon reflexes are intact.

Sensory loss: there is no sensory loss because theposterior interosseous branch is a purely motornerve.

Radial nerve lesion at thewristInspection: the arm and hand appear normal.Look for surgical or traumatic scars around thewrist.

Motor function: there is no motor weakness(because the radial nerve serves only extrinsicextensor muscles of the hand from above thewrist).

Sensory loss: there is sensory loss in the firstdorsal interosseous only (as the sensory branchbecomes superficial at the wrist).


• Axillary lesions:• Fracture/dislocation of humeral head• Compression: use of shoulder crutch or

‘Saturday night palsy’ from prolongedhanging of the arm over the back of achair (when intoxicated)

• Spiral groove lesions:• Fracture: mid-shaft fracture of the humerus• Compression: wheelchair users resting

back of their arm against the chair• Posterior lesions:

• Compression: from the arcade of Frohse/supinator arch

• Interosseous lesions:• Tumours/lipomas or ganglia near the

elbow


• Wrist lesions:• Fracture: at the distal radius• Compression: tight bracelets/handcuffs/

plaster casts.



Imaging: ultrasonography/MRI may rarely be con-sidered.

Management• Conservative management of ‘Saturday night

palsy’ with spontaneous improvement• Physiotherapy and splinting for mild

compressive lesions• Surgical correction of fracture/dislocations.


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


Common peroneal nerve palsy is the most com-mon cause of foot drop. However, a lesion inany of the areas, including the motor cortex,spinal cord, lumbar nerve roots L4–5, lumbo-sacral plexus and sciatic nerve, and peripheralneuropathies or myopathies, can cause foot dropwith different associated clinical signs. Know-ledge of the anatomy of the common peronealnerve will help determine the level of the lesion.

The sciatic nerve (L4, L5, S1–3) divides into itsterminal branches, the tibial nerve and the com-mon peroneal nerve, two-thirds down the poster-ior thigh. The tibial nerve serves the posteriorcompartment of the lower leg, producing plantar-

flexion and inversion. The common peronealnerve (Figure 3.21) serves the anterior part of thelower leg, winding around the neck of the fibulaand dividing into the superficial peroneal nerve(foot eversion and sensation to lateral lower legand dorsum of foot) and the deep peroneal nerve(foot and toe dorsiflexion and sensation to thedorsal web space between the hallux and secondtoe).

Common peroneal nerve palsyInspection: there is foot drop with a high-stepping gait. There is wasting of the antero-lateral compartment of the lower leg. Look forsurgical or traumatic scars near the knee andneck of the fibula. Look for any ankle supports/adapted footwear.

Common peroneal n.

Deep peroneal n. (cut)

Superficialperoneal n.

Peroneus longus

Peroneus brevis

Medial cutaneousbranch

Lateral cutaneousbranch

Lateral cutaneous n. of calf

Lateral Anterior

Cutaneous distribution

Common peroneal nerve

Superficial peroneal nerve

Deep peroneal nerveserving web space

Figure 3.21 Route and muscles supplied by the common peroneal nerve and cutaneousdistribution


Motor function: there is weakness of ankle dorsi-flexion, and hallux extension (deep peronealnerve) and eversion (superficial peroneal nerve).Test for eversion in a passively dorsiflexed footbecause the everters cannot exert their action ifthe foot is in plantarflexion. The ankle jerk isintact and plantar reflex is downwards. There isnormal plantarflexion and inversion. In mildcases, weakness may be seen only when askingthe patient to walk on the heels.

Sensory loss: over the lateral calf and dorsum ofthe foot, but sparing the little toe. The little toehas sensation from the sural nerve, a branch ofthe tibial nerve.

Super¢cial peroneal nerve palsyInspection: there is wasting of the lateral com-partment of the lower leg but no obvious high-stepping gait. Look for surgical or traumaticscars near the knee and neck of the fibula. Lookfor any ankle supports/adapted footwear.

Motor function: there is slight weakness of ankledorsiflexion which might be seen only when thepatient is asked to walk on the heels. There is noweakness in hallux extension, but there is weak-ness of eversion. There is normal plantarflexionand inversion.

Sensory loss: over the lower lateral calf anddorsum of the foot, but sparing the little toe.

Deep peroneal nerve palsyInspection: there is foot drop with a high-stepping gait. There is wasting of the anteriorcompartment of the lower leg. Look for surgicalor traumatic scars near the knee and neck of thefibula, anterior lower leg. Look for any anklesupports/adapted footwear.

Motor function: there is weakness of ankle dorsi-flexion (deep peroneal nerve) and hallux exten-sion but intact eversion (superficial peronealnerve).

Sensory loss: only in the dorsal web space be-tween the hallux and second toe.

Di¡erential diagnosisMyopathy: tends to give rise to proximal weak-ness but there are distal variants. Signs will bebilateral. All foot movements will be weak in-cluding plantarflexion and hallux flexion. Theankle jerk may be reduced. There will be nosensory loss. There will be myopathic signs in theupper limbs and possibly the face.

Sensorimotor peripheral neuropathy: there isalso weak plantarflexion and a stocking patternof sensory loss including the little toe. Signs willbe bilateral. There may be dorsal column signsand a positive Romberg’s test and sensorimotorsigns in the upper limbs.

Neuromuscular junction disorder: signs tend tobe bilateral with upper limb and facial involve-ment. Weakness will affect plantarflexion and befatigable. Reflexes and sensation will be normal.

MND: signs are bilateral, although in early dis-ease there can be asymmetry. There will be amixture of UMN and LMN signs with increasedtone, brisk ankle jerks, possibly upgoing plantarreflexes, marked wasting and fasciculations.There tend to be signs in the upper limbs andface. There are no sensory findings.

Sciatic nerve lesion: a peripheral nerve lesionaffecting the sciatic nerve can cause foot drop,but also weak plantarflexion and inversion, weakknee flexion (with preserved knee extension) andhip extension. The ankle jerk is absent with apreserved knee jerk. Sensory loss will be alongthe posterior thigh, lower leg and foot, sparingthe medial side of the lower leg. Injury to thesciatic nerve can occur through hip surgery, mis-placed gluteal injections and pelvic pathology,such as trauma, haematoma, abscess or tumours.

Lumbosacral plexus lesion: presents similarly to asciatic nerve lesion but with femoral nerve invol-vement. This will cause additional weakness in


hip flexion, abduction and adduction, and kneeextension. The knee and ankle jerks are lost.There will be more extensive sensory loss includ-ing the anterior thigh and medial lower leg.

L4–5 radiculopathy: leads to similar findings as acommon peroneal nerve lesion. In addition therewill be weak hip abduction and adduction andvariable effects on knee flexion and extension.The knee jerk (L3–4) may be lost but ankle jerks(S1–2) preserved. Sensory loss will include themedial part of the lower leg (L5). The patientoften has back pain because nerve root compres-sion usually arises from lumbar disc herniation(less commonly a tumour). Straight leg raise,stimulating nerve root irritation from stretching,will reproduce symptoms.

Spinal cord lesion: this can cause weakness infoot dorsiflexion but will also cause weakness inother muscle groups of the leg depending on thelevel of the lesion. Foot drop is not so obviousdue to the UMN spasticity. Lesions can be uni-lateral or bilateral. Sensory signs will depend onthe location of the lesion in the cord and theremay be dissociated sensory loss, giving rise tothe Brown–Sequard syndrome if the lesion isunilateral.

Cortical lesion: a tumour or stroke affecting themotor cortex, such as a lacunar infarct or para-sagittal meningioma, can rarely cause localisedlower limb weakness and foot drop. There willbe UMN signs.

Causes of common peroneal nerve palsy• Trauma• Fibular fracture• Knee surgery• Compression: plaster cast, leg crossing,

weight loss.



Imaging: MRI might be considered.

Management• Avoidance of aggravating factors such as leg

crossing or squatting• Physiotherapy and splinting of the ankle/foot• Surgical repair or release if there has been

transection or tethering.


SCENARIO 21. NYSTAGMUS

Nystagmus will usually be a sign as part ofanother disorder such as cerebellar syndrome orMS, but rarely it can form the full case in thePACES neurology station.

Nystagmus is an involuntary rhythmic oscillationof the eye(s) that may be physiological, congeni-tal or acquired. It represents a problem with theneural mechanisms/centres involved in maintain-ing image fixation on the fovea for optimal visualacuity.


Nystagmus is usually described with reference to:

• Monocular or binocular/conjugate• Position: primary (looking forward) or only

gaze related• Type: pendular (equal velocity in either

direction) or jerk: a slow drift then fastcorrective phase – the direction of nystagmusrefers to the fast phase

• Plane: horizontal, vertical or rotatory/torsional(sometimes it is easier to tell by looking at thepull on the conjunctival vessels).

Cerebellar nystagmus

This is a binocular/conjugate, primary and gaze-related jerk nystagmus which is in the horizontalplane, and the direction of the nystagmus (fastphase) is towards the same side as the cerebellarlesion and maximal on looking towards this side.The nystagmus does not fatigue with continuedgaze to the affected side. There is also loss ofsmooth saccades.

Further cerebellar testing will show homolateralpoor finger–nose pointing with dysmetria andintention tremor, dysdiadochokinesis, poor heel–shin coordination and an ataxic gait falling to-

wards the side of the lesion. There may bedysarthric speech.

AdditionalFinish your exam by asking to test the cranialnerves, particularly looking for any abnormalityof cranial nerve V (corneal and facial sensation)or cranial nerve VIII which may suggest a lesionat the cerebellopontine angle (see Scenario 27).Ask to examine the fundus for optic atrophywhich may be present in MS.

Ask to examine the upper and lower limbs,which may show pyramidal weakness consistentwith MS, or dorsal column signs in alcohol mis-use or vitamin B12 deficiency.

Romberg’s test is not a test of cerebellar function,but rather of dorsal column/proprioceptive func-tion. A patient with a cerebellar lesion cannotusually stand steady, feet together with arms bythe sides even with the eyes open, so Romberg’stest cannot be performed.

Di¡erential diagnosisUnilateral cerebellar pathology and nystagmuswill tend to be caused by structural lesions:

• Cerebrovascular events, eg lateral medullarysyndrome

• Demyelination, eg MS• Cerebellar/posterior fossa tumours, eg

astrocytomas, haemangioblastomas,medulloblastomas and metastatic disease(breast, lung, skin, kidney).

Bilateral cerebellar nystagmus will be caused bysystemic pathology:

• Toxins (alcohol, chemotherapy andanticonvulsants)

• Autoimmune and paraneoplastic processes• Inherited disorders involving cerebellar

degeneration, eg olivopontocerebellar


degeneration and Friedreich’s ataxia (seeScenario 12).

Vestibular nystagmus

Peripheral vestibular nystagmus: this is a binocu-lar/conjugate horizontal or rotatory/torsional nys-tagmus which is a primary and gaze-relatedunidirectional jerk nystagmus with the fast com-ponent maximal to the opposite side of thevestibular lesion. With upward or downwardgaze the nystagmus remains horizontal and inthe same direction. With continued gaze awayfrom the side of the lesion the nystagmus fa-tigues. There are no cerebellar signs. The gaitmay be unsteady, falling towards the side of thelesion, and the patient may describe vertigosymptoms. These are often worse when testinggait. Tinnitus and deafness may be found on theside of the lesion.

Di¡erential diagnosisThe peripheral vestibular system includes thesemicircular canals, otoliths and the vestibularportion of cranial nerve VIII.

The causes of peripheral vestibular nystagmus in-clude labyrinthitis, acoustic neuroma, Meniere’sdisease, benign paroxysmal positional vertigo(BPPV), autoimmune inner ear disease (AIED) anddegenerative middle-ear disease such as oto-sclerosis.

Central vestibular nystagmus

This is a binocular/conjugate horizontal/verti-cal/rotatory or mixed (may appear chaotic) nys-tagmus, which is a primary and gaze-relatednystagmus. It is multidirectional so that on look-ing to the left it is leftward (fast component to theleft), looking to the right it is rightward andlooking up it is upward. There is no fatigue of thenystagmus on sustained gaze in any direction.The patient may have a tendency to fall in anydirection on testing gait; vertigo is unusual. There

are no peripheral symptoms such as tinnitus ordeafness and no cerebellar signs.

Di¡erential diagnosisThe central vestibular system includes the vestibu-lar nerve nuclei and their projections to the cere-bellum, extraocular nuclei via the mediallongitudinal fasciculus, the spinal cord via thevestibulospinal tract and projections to the cortex.

Causes of central vestibular nystagmus includebrain-stem stroke and vertebrobasilar insuffi-ciency, brain-stem tumours, demyelination suchas MS, syringobulbia (see Scenario 7) and basilar-type migraine (some classify this as a cause ofperipheral vestibular nystagmus when it is asso-ciated with benign paroxysmal positional vertigo).

Pendular nystagmus

This is a conjugate or monocular multidirectionalnystagmus that can appear chaotic. The oscilla-tion has equal velocity in all directions. It ispresent in all positions including the primaryposition.

Additional: look around the bed for clues ofvisual aids used by the blind patient and ageneral appearance that might suggest albinism.Ask to perform fundoscopy to look for opticatrophy, signs of retinitis pigmentosa and cata-racts with disruption of the red reflex.

Di¡erential diagnosisMonocular or binocular visual deprivation is themost common cause of pendular nystagmus, butother causes include demyelinating disease suchas MS and brain-stem dysfunction.

Internuclear ophthalmoplegia (INOor ataxic nystagmus)

There is failure of conjugate eye movements onlateral gaze. In the primary position there may bea divergent strabismus. In a left-sided INO (lesion


in the ipsilateral MLF) there is partial or totalfailure of adduction of the left eye on lookingright, but normal abduction of the right eye, withjerk horizontal nystagmus in the abducting eyewith fast corrective phase towards the right side(opposite side to the lesion). The nystagmus ofthe abducting eye is not necessary for the diag-nosis of INO. The patient may describe diplopiawhen looking to the right (contralateral) side. Itcan be shown that this is not a left medial rectuspalsy by the fact that, by covering the rightabducting eye, the left eye adducts normally withconvergence. Sometimes convergence can beaffected if the lesion extends into the midbrain.In a bilateral INO there will be failure of adduc-tion of either eye with lateral gaze in the oppositedirection.

AdditionalState that you would like to look for additionaleye signs supportive of MS such as optic atrophy,visual field defects, optic disc pallor on fundo-scopy and/or a relative afferent pupillary defect(RAPD). Look for cerebellar signs in support ofMS or brain-stem infarction.

Di¡erential diagnosisINO is due to a lesion in the MLF within thepons and midbrain, which connects the contra-lateral nerve VI nucleus to the ipsilateral oculo-motor (nerve III) nucleus.

MS is the most common cause in a youngerperson, and brain-stem infarction causing unilat-eral INO is the most common cause in an olderperson. Other causes include brain-stem tu-mours, viral infection, syphilis infection, Lymedisease, trauma, Arnold–Chiari malformation, syr-ingobulbia, and drug (phenothiazines, phenytoin,tricyclic antidepressants) and alcohol intoxica-tion.

Downbeat nystagmus

There is a bilateral downbeat nystagmus in theprimary gaze, which remains downbeat in all

directions of gaze. Lateral gaze may accentuatethe nystagmus.

AdditionalLook for signs of syringobulbia and syringomyeliawhich may occur together with an Arnold–Chiarimalformation, eg bulbar palsy, INO, balaclava-helmet loss of facial sensation, a dissociatedsensory loss usually in a cape distribution, LMNsigns in the upper limbs and UMN signs in thelower limbs. Look for cerebellar signs that mayalso suggest syringobulbia or a posterior fossatumour.

Di¡erential diagnosisDownbeat nystagmus usually signifies pathologyat the craniocervical junction. Arnold–Chiarimalformation is the most common cause. Down-beat nystagmus may also be seen with brain-stemstroke, syringobulbia, spinocerebellar degenera-tion, MS and drug (phenytoin, lithium) and alco-hol toxicity.

Upbeat nystagmus

There is a bilateral vertical nystagmus in theprimary position with the fast phase beating inthe upward position.

If the nystagmus increases on upward gaze, thissuggests pathology in the anterior vermis of thecerebellum. There may be other cerebellar signssuch as loss of smooth saccades, slurred speechand truncal ataxia.

If the nystagmus increases on downward gaze,this suggests pathology in the medulla. Theremay be associated brain-stem signs such as pala-tal weakness with nasal speech.

Physiological nystagmus

This is a gaze-evoked jerk nystagmus occurringat the extremes of gaze and absent in the primaryposition. The elastic pull of the extraocular mus-


cles and tendons exerts a force that tends to bringthe eye back to the midline, but the neuralintegrator tries to overcome this with correctivequick movement (jerk nystagmus) to the desiredextreme of gaze.


InvestigationsThese will be determined by the type of nystag-mus and the associated differential diagnosis.

Management• Cessation of causative medications• Correction of refractive errors with contact

lenses• Downbeat nystagmus may be treated with

base-out prisms, which induce convergence• Botulinum toxin injection into rectus muscles

can ameliorate acquired nystagmus.However, this diminishes all types of eyemovement and can cause diplopia, ptosis andincreased nystagmus in the uninjected eye.


SCENARIO 22. OPHTHALMOPLEGIA

The most common gaze palsies seen in thePACES examination are cranial nerve III and VIpalsies. Remember to look for associated cranialnerve abnormalities or peripheral examinationsigns that will point towards a specific disease orhelp further localise the lesion.

It is worth revisiting the anatomy and function ofthe extraocular nerves III, IV and VI together withtheir muscles so that their examination becomesstraightforward (see Figures 3.22 and 3.23).

Nerve III (oculomotor) palsy

See Figure 3.24. Nerve III supplies most of theextraocular muscles including superior rectus(elevation), medial rectus (adduction) and inferior

Superior oblique muscle(IV cranial nerve)

Superior rectus muscle(III cranial nerve)

Medial rectus muscle(III cranial nerve)

Lateral rectus muscle(VI cranial nerve)

Inferior rectus muscle(III cranial nerve)

Inferior oblique muscle(III cranial nerve)

Figure 3.22 Right eye and its muscles

Superior rectus andinferior oblique

(III cranial nerve)

Up

Up/In

In

Down/In

Down

Down/Out

Out

Up/Out

Lateral rectus(VI cranial nerve)

Superior rectus(III cranial nerve)

Medial rectus(III cranial nerve)

Inferior rectus(III cranial nerve)

Inferior rectusand superior oblique

(III cranial nerve)

Superior oblique(IV cranial nerve)

Inferior oblique(III cranial nerve)

Figure 3.23 Cardinal directions of gaze and extorsion/intorsion


oblique (extorsion, elevation and abduction). Italso elevates the eyelid through its innervation oflevator palpebrae superioris and carries pregan-glionic parasympathetic innervation whichcauses pupil constriction via the sphincter (con-strictor) pupillae.

The nerve III nucleus in the midbrain is divided intosubnuclei to innervate the separate muscles, and itis partnered with the nearby Edinger–Westphalnucleus which gives rise to the preganglionicparasympathetic fibres; these lie superficially with-in the nerve and are therefore easily compromisedby compressive lesions.

The subnuclei serve the IPSILATERAL extraocularmuscles (medial rectus, inferior rectus and infer-ior oblique) with two important exceptions:

1. The nucleus sends fibres across to the oppositeoculomotor nucleus, which then innervates theCONTRALATERAL superior rectus.

2. The nucleus supplies BOTH levator palpebraewith crossed and uncrossed fibres.

The nerve III fascicles/trunks leave the midbrainpassing ventrally. Nerve III then passes in thesubarachnoid space and runs close to the poster-ior communicating artery. It enters the lateral sideof the cavernous sinus, crosses over the trochlearnerve, and exits medially as superior and inferior

branches through the superior orbital fissure. Thesuperior branch serves levator palpebrae super-ioris and superior rectus, which is also joined bysympathetic supply from the internal carotid ar-tery. The inferior branch serves the other oculo-motor muscles and carries the parasympatheticaxons to the constrictor pupillae.

Identifying clinical signsThere is a left-sided ptosis (see Figure 3.24) andwhen the eyelid is raised the eye is in a downand out position (due to the unopposed com-bined action of lateral rectus and superior obli-que), giving a divergent strabismus/squint. Thepatient cannot move the affected eye across themidline. Diplopia is maximal on trying to lookaway from the affected side and up. There is adilated and non-reactive pupil (to light or at-tempted accommodation) from parasympatheticfibre involvement.

With a nuclear lesion there will be contralateraleye signs in addition to the ipsilateral signs. Thereis a contralateral partial ptosis (bilateral innervationof levator palpebrae superioris) but ptosis will bemore pronounced on the ipsilateral side. There willalso be a contralateral elevation palsy (the nucleusinnervates the contralateral superior rectus). Re-member, there is an ipsilateral elevation palsybecause fibres from the contralateral subnucleus

Figure 3.24 Left-sided III nerve palsy. Reproduced with the kind permission of Professor ChuaChung Nen


pass through the ipsilateral subnucleus before in-nervating the superior rectus.

Di¡erential diagnosisNuclear lesion: infarction, haemorrhage, tumour,abscess, demyelination.

Midbrain lesion: herniation, infarction, haemor-rhage, tumour, abscess, demyelination (MS).

Subarachnoid lesion: aneurysm, haemorrhage,meningitis, inflammation including vasculitides(giving rise to mononeuritis multiplex), tumour,migraine.

Cavernous sinus lesion: tumour (pituitary, cranio-pharyngioma), thrombosis, aneurysm, fistula, in-fection, inflammatory.

Orbital lesion: trauma, tumour.

Small vessel disease: diabetes, hypertension,atherosclerosis.

Infection: Lyme disease, syphilis, basilar menin-gitis (bacterial, mycobacterial, fungal, parasitic).

Nerve IV (trochlear) palsy

Nerve IV supplies the superior oblique musclewhich intorts, depresses and abducts the globe.This combined action allows the eye to lookdown and in. The nerve IV nuclei lie in themidbrain where the nerves decussate and thenexit dorsally. It is the only cranial nerve to exitthe brain dorsally and it has a long course,making it susceptible to trauma.

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 ChuaChung Nen


Identifying clinical signsThe right eye appears slightly elevated/normaland the patient has a head tilt away from the sideof the lesion, tucking the chin in slightly to bringthe visual axis of the affected eye central again(see Figure 3.25). The affected eye cannot lookdown in adduction (towards the nose). It is in thisposition that the patient experiences most verti-cal diplopia (giving rise to the classic history ofdifficulty reading books or climbing stairs whenthis direction of gaze is needed). Remember thatdiplopia is always worse in the direction of gazeof the paretic muscle. The false outer/upper im-age disappears when the affected eye is covered.

Di¡erential diagnosis• Congenital• Trauma (most common cause)• Small vessel disease: diabetes, hypertension,

atherosclerosis• Inflammatory: mononeuritis multiplex,

peripheral neuropathy

• Infection: Lyme disease, syphilis, basilarmeningitis (bacterial, mycobacterial, fungal,parasitic)

• Midbrain/nuclear lesion: infarct,haemorrhage, tumour, abscess, demyelination(MS)

• Cavernous sinus lesion: tumour (pituitary,craniopharyngioma), thrombosis, aneurysm,fistula, haemorrhage, infection, inflammatory.

Nerve VI (abducens) palsy

Nerve VI innervates the ipsilateral lateral rectus,which abducts the eye. The nerve VI nucleus isin the caudal part of the pons. Approximately40% of its neurons pass to the nearby MLF, tothen cross over to the contralateral nerve IIInucleus to innervate the contralateral medialrectus and produce conjugate lateral gaze.

Figure 3.26 Left sided VI nerve palsy. Reproduced with the kind permission of Professor ChuaChung Nen


Identifying clinical signsThere is a convergent strabismus in the primaryposition due to the unopposed action of theintact medial rectus (see Figure 3.26). There ishorizontal diplopia maximal on attempted gazein the direction of the paretic muscle. The outerimage disappears on covering the affected eye.

On testing the remaining cranial nerves, payparticular attention to nerves VII and VIII, andcheck for nystagmus and other cerebellar signsthat would indicate a cerebellopontine anglelesion. There may be signs of bilateral papilloe-dema on fundoscopy from a space-occupyinglesion or idiopathic intracranial hypertension.Here, the nerve VI palsy acts as a ‘false localisingsign’ due to downward displacement of the brainstem causing stretching of the abducens nerve.

Di¡erential diagnosis• Congenital: congenital absence of nerve VI –

Duane’s syndrome• Trauma• Raised ICP: space-occupying lesion or

idiopathic intracranial hypertension• Small vessel disease: diabetes, hypertension,

atherosclerosis• Inflammatory: mononeuritis multiplex,

postviral, peripheral neuropathy• Infection: Lyme disease, syphilis, basilar

meningitis (bacterial, mycobacterial, fungal,parasitic)

• Pontine/nuclear lesion: infarct, haemorrhage,tumour, abscess, demyelination (MS)

• Petrous bone pathology: in severe ongoingotitis media there can be infiltrativeosteomyelitis involving the petrous temporalbone

• Cavernous sinus lesion: tumour (pituitary,craniopharyngioma), thrombosis, aneurysm,fistula, haemorrhage, infection, inflammatory.

Complex ophthalmoplegia

Identifying clinical signsThyroid ophthalmopathy will usually present asa complex ophthalmoplegia not attributable toany single nerve lesion. This does not represent atrue ophthalmoplegia. It is due to soft-tissueinflammation and swelling within the orbit caus-ing restriction of eye movements. There is usuallyproptosis, chemosis, lid lag and other thyroidsigns.

Myasthenia gravis may present as a complexophthalmoplegia not attributable to any singlenerve lesion. Eye movements are fatigable andthere are no pupillary signs.

The Miller–Fisher variant of Guillain–Barre syn-drome may present initially with an ophthalmo-plegia, with the descending paralysis fromperipheral demyelination giving the classic triadof ophthalmoplegia, ataxia and areflexia.

Chronic progressive external ophthalmoplegia(CPEO) is the most common manifestation ofmitochondrial myopathy, in itself very rare, andusually presents as a bilateral progressive ptosis,which proceeds to a bilateral ophthalmoplegiawithout pupillary changes. Kearns–Sayre syn-drome is a mitochondrial myopathy that presentswith the triad of age ,20 years, CPEO andretinitis pigmentosa. Other associated featuresinclude cerebellar syndrome, cognitive impair-ment, Babinski’s sign, hearing loss, seizures, shortstature and delayed puberty, with other endo-crine abnormalities and cardiac conduction de-fects.

Oculopharyngeal dystrophy (see Scenario 10) isan autosomal dominant trinucleotide repeat dis-ease occurring in 60–70 year olds, with progres-sive ptosis and ophthalmoplegia withoutpupillary changes, leading to dysphagia and


facial weakness, and in the latter stages of dis-ease to proximal muscle weakness.

Cavernous sinus syndrome

Structures contained within the cavernous sinus:

• Internal carotid artery• Sympathetic carotid plexus• Cranial nerves: III, IV, VI and V1 and V2

branches (V3 lies outside the sinus).

Cavernous sinus syndrome signs:

• Painful ophthalmoplegia (unilateral single orusually combined nerve III, IV and VI palsies)

• Horner’s syndrome (with no associatedanhidrosis because the lesion occurs after thesuperior cervical ganglion and the pupil maybe mid-position and fixed with bothparasympathetic and sympathetic disruption)

• Anaesthesia of forehead, maxilla andconjunctiva (V1 and V2 branches)

• Proptosis (if pulsating suggests carotid–cavernous fistula)

• Conjunctival injection with chemosis• Papilloedema � visual loss• Orbital bruit.

Di¡erential diagnosis of cavernous sinussyndrome• Tumours: meningiomas, extension of pituitary

or craniopharyngiomas, metastatic disease• Vascular: cavernous sinus aneurysms or

fistulae• Thrombosis: usually complicating infection of

the ethmoid, frontal and sphenoid sinuses orextension of dental or orbital infection

• Inflammatory: herpes zoster, sarcoidosis andWegener’s granulomatosis

• Idiopathic: Tolosa–Hunt syndrome is a raregranulomatous inflammation of the cavernoussinus and superior orbital fissure. It causes apainful ophthalmoplegia � pupillary effects.


Investigations• These are urgent if an aneurysm,

subarachnoid haemorrhage (SAH), uncalherniation, meningitis, stroke or trauma issuspected.

• Imaging: CT or MRI of the brain is indicated ifsuspecting aneurysmal, SAH, stroke, space-occupying lesion � herniation, or traumaticcause. Cerebral angiography may be neededto investigate aneurysmal disease andarteriovenous (AV) malformations includingfistulae.

• Blood tests: investigations for small-vesseldisease will include fasting blood glucose/HbA1c, autoimmune profile, pANCA andcANCA, ESR and CRP if suspecting giant cellarteritis (GCA).

• Lumbar puncture: indicated if suspectingmeningitis, and a space-occupying lesion hasbeen ruled out.

ManagementThis is directed by the underlying cause.

Nerve III (oculomotor) palsies may resolve spon-taneously over months if the underlying cause isischaemia (typically in hypertensive or diabeticpatients) of the vasa nervosa. This typically givesrelative sparing of the pupil and is often painfulfor unknown reasons. NSAIDs may amelioratethis. Patching of the deviated eye can be a usefulshort-term measure. In the long term, surgicalcorrection may be indicated for a non-resolvingstable angle.

Nerve IV (trochlear) palsies have been treatedsuccessfully with botox injection (of other mus-cles), prisms and surgical correction.


Nerve VI (abducens) palsies, when isolated inchildren and young patients, are often benignand resolve spontaneously within 6 months. Thecause is unclear. Alternate patching may be use-ful to prevent amblyopia. In older patients GCAshould be considered and treated with steroids ifappropriate.


STATION5

IntegratedClinical Assessment

393

STRUCTURE OF THE ICA

The older Station 5 (until 2009) involved a seriesof three short cases based around examination ofthe eyes, an endocrine disorder or rheumatologi-cal condition. Like the other examination sta-tions, this involved a ritualised examinationfollowed by presentation of findings to the exam-iners. There are still ‘echoes’ of this old format inmany textbooks and courses; skin, locomotorand endocrine scenarios have been shoe-hornedinto an ‘integrated clinical assessment’ (ICA) for-mat. This slightly misrepresents the content of theexam, which is highly varied and demands farmore than an ability to elicit clinical signs. In-deed, this is the only station of the PACES examthat assesses candidates in all categories of themarking scheme.

Station 5 is organised as follows:

• Two 10-minute cases known as ‘brief clinicalconsultations’.

• The candidate has 8 minutes with the patientto take a focused history, carry out a relevantexamination, reach a diagnosis or identify aclinical problem and then communicate thisto the patient.

• The remaining 2-minute discussion with theexaminers will not begin until these 8 minuteshave elapsed.

• The examiner will ask the candidate to statethe positive physical findings, theirconcluding diagnosis and differentialdiagnoses (if appropriate) based on theirassessment.

CONTENT AND MARKING OF

THE ICA

The cases found in Station 5 typically involve apresenting complaint that guides you to the rele-vant system for a targeted examination. Alterna-tively, there may be obvious clinical signs (egthyroid eye disease) and a rather vague history.

The seven areas in which candidates are scoredare summarised below, with candidates beingscored on a three-point scale: satisfactory, border-line or unsatisfactory.

Clinical communication skills

• Eliciting a history relevant to the complaint• Explaining information to the patient in a

focused, fluent and professional manner

Physical examination

Performing an examination in a correct, appro-priate, practised and professional manner

Clinical judgement

Selecting a sensible and appropriate investigationand treatment plan

Managing patient’s concerns

• Detecting, acknowledging and attempting toaddress patient’s concerns

• Listening• Demonstrating empathy

Identifying physical signs

• Identifying the correct physical signs• Not finding signs that are not present

Differential diagnosis

Constructing a sensible differential diagnosis, in-cluding the correct diagnosis

Maintaining patient welfare

Treating the patient respectfully and sensitively,ensuring comfort, safety and dignity

A formal marksheet is shown on page can befound on the MRCPUK website.

Integrated Clinical Assessment 395

INFORMATION GIVEN TOCANDIDATES, PATIENTS ANDEXAMINERS

Before each Station 5 examination, each candi-date, patient and examiner will be given someinformation.

Below is a worked example of the informationgiven to each person based on guidance on theMRCP website (www.mrcpuk.org).

Information to candidate

You will be asked to see two patients at thisstation. The clinical information about one ofthese patients is given in the box below. Youshould have a second sheet giving you informa-tion about the other patient.

• You have 10 minutes with each patient. Theexaminers will alert you when 6 minuteshave elapsed and will stop you after8 minutes.

• In the remaining 2 minutes, one examinerwill ask you to report abnormal physicalsigns (if any), your diagnosis or differentialdiagnosis, and your plan for management (ifnot already clear from your discussion withthe patient).

Your role: You are the medical doctor on call

Patient name: Mrs Beverley Gordon – age 39years

This lady was admitted to the orthopaedic wardfor a carpal tunnel release operation.

She mentioned to the orthopaedic doctors thatshe has swelling in her neck and they haveasked for the opinion of a physician. You wereasked by your consultant to see the patient andto assess the suspected swelling in her neck.

Your task is to assess the patient’s problems andaddress any questions or concerns raised by thepatient.

• You should assess the problem by means ofa relevant clinical history and a relevantphysical examination. You do not need tocomplete the history before carrying outappropriate examination.

• You should respond to any questions thepatient may have, advise the patient of yourprobable diagnosis (or differentialdiagnoses) and your plan for investigationand treatment where appropriate.

• You have 8 minutes to complete the task.

Accompanying notes are given to each patientand may take the following format.


Information for patients

The doctors sitting the examination have beenasked to assess your problem. They will have8 minutes to ask you about the problem andany other relevant issues. They will also exam-ine you. They should explain to you what theythink is wrong and what action should be takenand answer any questions you have, for exam-ple about the diagnosis, tests that may beneeded, or treatment. One of the examinerswill ask them to describe any abnormal exam-ination findings and give their diagnosis.

Your history is described below.

You are: Mrs Beverley Gordon – age 39 years

Your problem: A swelling in your neck

You are in the orthopaedic ward and you men-tioned to the admitting doctor that your neckseemed swollen. One of the medical doctorshas been asked to see you about this.

You have been suffering from pain in your rightforearm and a numb or tingly feeling in yourright hand – affecting your third, fourth and fifthfingers. The aching in your arm is worse atnight and you have found it more and moredifficult to get comfortable. The problem hasbeen present for several years and was worsewhen you were pregnant with your daughter, 3years ago, but improved for a time after that.

A trapped nerve in the wrist has been diag-nosed. You are having a short admission tohave the nerve released.

You mentioned that you thought your neck wasswollen when the admitting doctor was exam-ining you. You have not mentioned this to adoctor before, but your sister has been com-menting on it for a few years. Your sister is 36and has an underactive thyroid.

You do not have any of the symptoms yoursister had when her thyroid was underactive.

Indeed you feel well. Your weight is stable. Yourskin and hair are normal. You do not seem tofeel the heat or the cold any more than anyoneelse. Your bowel works normally. You do nothave any problem with swallowing.

You should ask why your neck seems swollenand whether there is something wrong withyour thyroid gland. You should ask if any testsare required and, if so, what these tests will be.

Finally, for the same worked example, the ex-aminers have the following information, whichwould typically list the findings the candidatewould be expected to observe or elicit.


Information for examiners

Patient: Mrs Beverley Gordon – age 39 years

Examiners should discuss and agree the criteriafor pass and for fail in the competencies beingassessed.

As a general guide, candidates would be ex-pected to:

• Note the history of neck swelling and thefamily history of thyroid disease

• Enquire about symptoms of disturbedthyroid function

• Examine the neck and identify the smoothand symmetrical thyroid enlargement. Noteabsence of bruit

• Examine for signs of overactive andunderactive thyroid gland and confirmclinical euthyroid state

• Confirm to the patient that her thyroidgland does seem enlarged but reassure herthat the gland seems to be workingnormally judging from clinical examination.Advise on appropriate further investigations.

The lead examiner should:

(a) Advise the candidate after 6 minutes haveelapsed that ‘You have two minutesremaining with your patient’

(b) Ask the candidate to describe any abnormalphysical findings that have been identified

(c) Ask the candidate to give the preferreddiagnosis and any differential diagnosis thatis being considered

(d) Ensure any remaining areas of uncertainty,eg regarding the plan for investigation ormanagement of the problem, are addressedin any time that remains.

THE APPROACH TO STATION 5

It is very easy to fall into the familiar modes ofthe history-taking station or the examination sta-tions, then find, with a minute remaining, that

you have either almost wordlessly examined yourpatient without garnering any information abouttheir symptoms or that you have taken a thor-ough history but examined nothing. You maychoose to attempt to divide each consultationbetween history and examination, but westrongly recommend taking a history and exam-ination in parallel, just as you would during abusy on-call or clinic.

It is also crucial to remember that the examinersare not looking for the ritualised, detailed andcomplete examination of systems as in otherstations, or for a full history. They are testing yourability to perform a targeted examination andelicit the most salient symptoms. This is notsolely for the purpose of forming a differentialdiagnosis – don’t forget that you have beenspecifically asked to address the patient’s princi-pal concerns and must therefore explain to themwhat you think the underlying problem is andhow you are going to investigate and manage it.

Each of the Station 5 cases in this chapter isbased around a flow chart that gives a workedexample of how you might perform an ‘inte-grated clinical assessment’ (ICA) for a given pre-senting condition.

All cases can be divided into a new condition oran exacerbation of an existing chronic illness.These require slightly different emphasis in thehistory taking and examination, and this is re-flected in each flow chart.

An important feature of this approach is the ‘call-back’, wherein towards the end of the consulta-tion candidates re-state the presenting complaintof the patient and any other specific concernsthey raised during the consultation. This is aneasy way of demonstrating to the examiners thatyou have registered and understood the patient’smain concern (which is not necessarily the mainclinical priority). It also gives the patient anopportunity to mention other salient points whichthe candidate missed. If you have built a goodrapport with the patients/actor, they may help


you out at this stage by volunteering importantinformation.

The consulation should end with you describing:

• How you will address the patient’s mainconcern (eg analgesia)

• What you think the diagnosis may be• What investigations and initial treatments are

necessary.

This is an oppurtunity to demonstrate to theexaminer that you have come to a reasonabledifferential diagnosis and formed a safe and effi-cient investigation and management plan.

EXAMINATION TECHNIQUES FORSTATION 5

Station 5 often requires examination routines notencountered elsewhere in PACES, for exampleexamination of a goitre. These are describedbelow.

Examination of the hands

It is important to ensure the patient is comforta-ble and to rest the hands on a pillow. Expose thehands and forearms up to and including theelbows. A large proportion of the patients mayhave painful and tender joints and it is imperativetherefore to ask about this before palpating thejoints, which should be done after careful inspec-tion.

A rheumatological examination typically involvesinspection, palpation, neurological assessment,functional assessement and examination for ex-tra-articular signs. However, you may not havetime to do all of these completely.

1. InspectionThe bulk of the available information will begathered by inspection rather than palpation oractive movement.

• Peripheral accessories, eg walking stick• Peripheral arthropathies, eg knees, ankles• Systemic sclerosis – tight, shiny, stretched skin

with beaked nose +/ÿ telangectasia• Cushingoid appearance – steroid treatment• Horner’s syndrome – T1 lesion (see page. . ...)• Ears for evidence of psoriasis, or gouty tophi

in helix of ear.

Then make an assessment of:

• Nails – pitting, onycholysis, clubbing, nailfold infarcts, Beau’s lines

• Skin – tight shiny skin over dorsum of hand orfingers (scleroderma); tissue paper thin +/ÿpurpura (steroid therapy); surgical scars (jointreplacement); tar staining

• Muscles• bilateral wasting of the small muscles with

dorsal guttering (rheumatoid arthritis,syringomyelia, motor neurone disease)

• unilateral wasting of the small muscles ofthe hand (C8/T1 root lesion, eg cervicalrib, Pancoast tumour)

• unilateral wasting involving thenareminence (median nerve, eg carpal tunnelsyndrome)

• unilateral wasting sparing thenareminence (ulnar nerve, eg elbow trauma)

• Joints (in order to describe the location of theabnormality accurately, candidates shouldknow the names of the bones and joints)

• Distribution of any abnormalities –symmetrical (eg rheumatoid arthritis) orasymmetrical (eg seronegative arthritides);proximal or distal joints

• Specific deformities eg ‘swan neck’,‘Boutonniere’, Z-shaped thumb, subluxation,ulnar deviation, Heberden’s nodes, goutytophi

• Inflammation – calor, rubor, dolor, tumor andloss of function• NB rubor is replaced with shininess of the

skin in those with dark skin

Once the hands have been inspected, an assess-ment of the elbows is essential, as this can reveal


psoriatic plaques or rheumatoid nodules (indi-cating sero-positive rheumatoid arthritis).

The whole examination can be achieved by threemovements – assessment of the dorsum of thehand, then plantar aspect, followed by crossingthe arms over and exposing the extensors surfaceof the elbows.

2. Palpation (with caution)This must only be assessed with extreme cautionon the examination and only where there is anabsolute requirement to do so in the time given,such as if there is a specific complaint by thepatient (ie painful joints) or inspection hasdemonstrated a specific abnormality.

• Palm – Dupuytren’s contracture• Elbow nodules• Joints – palpate any swelling to determine

whether it is soft and boggy (rheumatoidarthritis) or hard and bony (Heberden’s nodesor gouty tophi)

• Skin – tightness or calcinosis in finger pulps(scleroderma/CREST).

This does not mean that you need to examine everyjoint in the hands, but merely the joints involved, egMCP joints.

3. Functional assessment of the handsYou will be expected to perform this assessmentif the patient reports reduced function.

• First, you can ask the patient to make a fist,followed by the prayer sign, followed by thereverse prayer sign.

• Finally, you could ask the patient to do up abutton on a shirt or hold a pen.

These manoeuvres have been validated againstmore complex assessments of function and providea quick means of assessing the likelihood of im-paired function in daily activities.

Neurological assessment – if relevant to the pre-senting complaint (eg symptoms of carpal tunnelsyndrome), one should proceed to perform a

neurological assessment focusing particularly onthe median and ulnar nerve. Single nerve lesionsare occasionally encountered in this station.

4. Extra-articular signsHaving screened for symptoms suggestive of sys-temic manifestions of joint disease, examinationshould then focus on the affected system.

Examination of the axial spine

A patient may present with stiffness of the axialspine and restricted movements (eg as in ankylos-ing spondylitis and other spondylarthropathies).

Inspection is crucial again. For example, the‘question mark posture’ (loss of lumbar lordosis,fixed kyphoscoliosis of the thoracic spine withextensive of the cervical spine) of ankylosingspondylitis can be a ‘spot diagnosis’. The charac-teristic posture is usually immediately evidentunless the patient is lying down with the headsupported by pillows.

The following examination routine should stagedisease and detect associated signs:

1. Establish restricted spinal movement

Two quick tests can be perfomed:

Lumbar spine: modified Shober’s index:With the patient standing upright, place twomarks 10 cm apart on the lumbar spine in themidline. The lower mark is at the level of theposterior superior iliac spines. The patient thenflexes forward (ask them to touch their toes) andat maximal flexion the distance is re-measured.In normal subjects there is an expansion of atleast 5 cm between the two marks. Lower valuesindicate decreased mobility of the lumbar spine.

Thoracic spine: Occiput-to-wall distance:The subject stands with their back against a wall(both heels and buttocks must be touching thewall) with a horizontal gaze. In normal subjects


the occiput will touch the wall. Any wall-to-occiput gap is a measure of restriction of thethoracic and cervical spines.

2. Examine for sacroiliitis/enthesitis

Examine (carefully) for tenderness over the sa-croiliac joints. Palpate for evidence of other en-thesitides over the heels, costochondral jointsand iliac crest.

Tell the examiner you would like to perform theFABERE (flexion, abduction, external rotation andextension) test. The patient places one ankle onthe opposite knee and allows the ipsilateral kneeto fall outwards (external rotation at the hip) toform a figure ‘4’. If this causes pain over thesacroiliac joint, sacroiliitis should be suspected.

3. Exclude extra-articular manifestations fromhead to toe:

If a spondylarthropathy is suspected (eg ankylos-ing spondylitis), a focused history should includequestions related to the extra-articular manifesta-tions associated with these conditions.

• Eyes: acute uveitis• Mouth: mucosal inflammation manifesting as

oral ulceration is common• Chest: apical fibrobullous disease (1%)• Cardiac: aortic root dilatation and associated

aortic valve incompetence• Abdomen: 15–20% will develop symptomatic

Crohn’s disease (stoma present?). Look forevidence of amyloidosis (hepatomegaly,evidence of renal failure or replacementtherapy)

• Nervous system: paraesthesia, signs of cordcompression

• Feet: Achilles tendonitis and plantar fasciitis

Examination of thyroid status

The assessment of the thyroid status of a patientis a fundamental clinical skill that should notpresent difficulties provided that the followingscheme is followed. For obvious reasons, patients

with severe hyper- or hypothyroidism are unlikelyto appear, but over/under-replacement of thyrox-ine is quite a common scenario.

General observation• Hypothyroidism

• Pale dry skin• ‘Peaches and cream’ complexion• Dry hair

• Note: Loss of the outer one-third of theeyebrows is unreliable and non-specific

• Hyperthyroidism• Anxious, fidgety patient• Staring eyes (lid retraction)• Sweating

HandsShake their hands

• Warm and sweaty or cool and dry?• Fine tremor – hands outstretched with a piece

of paper resting on fingers• Pulse

• Rate• Rhythm (AF often occurs in thyrotoxicosis)• Volume (typically large volume and

collapsing in hyperthyroidism)• Thyroid acropachy – a rare feature of Graves’

disease• Tar staining – Graves’ ophthalmopathy is

worse in smokers

Neuromuscularmanifestations• Reflexes: Slow relaxing in hypothyroidism;

brisk in thyrotoxicosis• Proximal myopathy: Thyrotoxicosis – ask the

patient to stand from a chair unaided

Dermatological manifestations ofthyroid disease• Graves’ dermopathy

Sheet-like myxoedema – coarse diffuse skin withnon-pitting oedemaNodular localised – violaceous infiltrative waxyarea on the shin, resembling erythema nodosumHorny – papilliform irregular firm red dermopathyon shin/upper foot


Examination of the thyroid gland

Follow the sequence of inspection, palpation andpercussion. If you find a goitre, it would beprudent to comment on the thyroid status, com-bining reported symptoms and a formal examina-tion of thyroid status as above. You must alsocomment on the most likely aetiology.

InspectionAsk the patient to swallow a sip of water andlook for upward movement of the thyroid gland.NB a thyroglossal cyst will move upwards bothon swallowing and protrusion of the tongue, andcan be trans-illuminated.

Is there any evidence from the history or exam-ination that the thyroid is compressing any of thefollowing:

• Trachea• monophonic syncope (rare), but the

history may suggestmsome dyspnoea,particularly on lying flat

• Recurrent laryngeal nerve• hoarseness

• Oesophagus• dysphagia, very rarely odynophagia

• Venous return from the head• superior vena cava obstruction (very rare)

Look carefully for a scar – previous hemi/totalthyroidectomy

PalpationStand behind the patient and gently palpate thegland, located two finger widths below the thyr-oid cartilage, with one hand on each side andthe neck gently flexed.

• If a goitre is present, comment on its:• Size• Consistency

• soft• firm• hard

Note: Soft – ‘like lips’, firm – ‘like the tip of thenose’ and hard ‘like the forehead’ is a good aidememoir to remember (but not repeat in the exam)when thinking about how to measure the consis-tency of the goitre.

• Diffuse or nodular• If nodular – multinodular or a single

nodule• Tender – suggests thyroiditis• Lymphadenopathy• Tethered – this suggests cancer.

PercussionPercuss gently for retro-sternal extension.

AuscultateBruit – classically occurs in Graves’ thyrotoxi-cosis.

Examination of thyroid eye disease

This may not be necessary in the exam unless thepatient is complaining of eye symptoms or if oninspection the patient has obvious thyroid eyedisease.

Note that lid lag and lid retraction are signs ofhyperthyroidism rather than Graves’ disease,although the two may clearly co-exist.

Lid retractionIndicated by visible sclera above the superiorlimbus of the cornea

This results from sympathetic stimulation of leva-tor palpabrae superioris of any aetiology, egthyrotoxicosis, anxiety, â-agonists.

Lid lagAsk the patient to follow the slow downwardmovement of your finger at a distance of about50 cm. The upper lid lags behind the descendingeyeball.


Look for clinical features of Graves’sa¡ecting the eyes

ExophthalmosSclera visible below the inferior limbus of thecornea with the patient sitting at the same levelas you and looking straight ahead.

This sign only occurs in Graves’ disease (cv), theterm is synonymous with proptosis; it can beunilateral, although a retro-orbital tumour shouldalways be excluded.

Other features of Graves’• Periorbital oedema• Chemosis• Conjunctival injection• Opthalmoplegia

If Graves’ ophthalmopathy is present on inspectionit is necessary to perform a more detailed examina-tion and take further history.• Are the eyes painful in any way? Are they

gritty or dry? Also ask if any part of thesubsequent exam causes pain or discomfort.

• Is eyelid closure adequate?• With exophthalmos there is a greater

volume of eye to be covered with eachblink, the frequency of blinking is reducedand the time of each blink is increased.

• Ask patient to follow your finger (and to say ifthey experience diplopia) as you test alldirections of gaze.• Limitation of upward gaze is the most

common abnormality in Graves’ophthalmopathy.

• However, the combination of enlargedocular muscles +/ÿ subsequent fibrosismay lead to complex ophthalmoplegiathat is not explained by either single nerveor muscle disease.

• Ptosis – a very rare occurrence in eitherGraves’ disease or hyperthyroidism. Itspresence should raise the possibility of co-existent myasthenia gravis.

• Acuity (see page 405) – full assessment (withophthalmoscopy) rather than gross assessment

of acuity may be unnecessary, unless there isa high suspicion this has been compromised.• Since the most important concern of

Graves’ ophthalmopathy is a threat to thesight, it is critical to assess vision in thefollowing fashion.• Acuity using a Jaeger chart or Snellen

chart (+ pinhole)• Colour vision – either using Ishihara

plates or a red pin to look fordesaturation

• Fields – compression of the nerve headat the orbital apex can cause constrictionof the fields

• Ophthalmoscopy• Is there papilloedema or consecutive

optic atrophy?• If closure is poor or you have concerns

about the cornea you may want anophthalmologist to perform slit lampexamination to look for corneal scars orulcers – you should state this to theexaminer. One can get a reasonableview with a direct ophthalmoscope, butnot good enough to preclude a properexamination.

Examination of a skin lesion/rash

The dermatology cases in the Station 5 ICArequire a special mention. Classically, the historyassociated with the condition may not be long ordetailed, and most of the differentiating informa-tion is gleaned from examination. Furthermore,the presentation of the examination is essentialtoo. Remember that many dermatological condi-tions reflect underlying medical conditions thatshould not be overlooked.

It is important to ensure you are fluent with thecommon terminology:

Macule Circumscribed area of erythematouschange without elevation

Papule Solid raised lesion , 1 cm in sizeNodule Solid raised lesion > 1 cm in size


Plaque Circumscribed elevated confluence ofpapules > 1 cm in size

Pustule Circumscribed area containing pusVesicle Circumscribed fluid-filled area

, 1 cm in sizeBulla Circumscribed fluid-filled area

> 1 cm in size

When examining the patient, you need to exposethe patient as much as the situation will allow(which the examiner should guide) and inspectthe patient as a whole, considering:

• Is there a rash and what is its distribution?• Is there any hair growth or loss?• Are there any features of systemic disease?

And more specifically:• If there is a rash – is it red or not?• Is the rash macular, papular, in patches or

plaques?• Are there scales or evidence of excoriation?• Are there fluid-filled lesions?• Are these vesicles, pustules or blisters?• Are there signs of systemic disease giving

clues as to the aetiology of the skin lesion, ega colostomy bag suggesting ulcerative colitis?

On presenting your findings, use the correctterminology and be succinct. If there is a sug-gestion from the history that the dermatologicalcondition is associated with an underlying condi-tion (eg erythema nodosum and inflammatorybowel disease), then an examination of the rele-vant system may be necessary.

For example:

‘There is a vesicular rash bilaterally on the ex-tensor aspects of the elbow, with associatedevidence of excoriation, on a background of apatient with symptoms consistent with coeliacdisease, suggesting that this is dermatitis herpeti-formis.’

This gives a clear and precise description of therash, its location, associated excoriation and linksit to the most likely underlying aetiologicalcause.

Examination of the eyes

As with the dermatology ICA cases, the historyassociated with an ophthalmological conditionmay be brief, but the examination is crucial.Taking a history and examining simultaneouslycan be easily achieved. Be cautious, however,since red flag symptoms must not be missed(such as in papilloedema or diabetic retinopa-thy), as some conditions reflect very seriousunderlying medical conditions or even malig-nancy.

This does not mean, however, that all aspects ofthe eye examination must be completed in theexamination. For example:

In a patient with a painful red eye you mayconsider examining:

• Visual acuity – may be reduced• Fundoscopy – may show anterior uveitis or

corneal scarring• Pupil – may be irregular, small, or unreactive

However, you might expect visual fields and eyemovements to be normal and tell the examineryou would include the examination of these aspart of your further investigations.

In a patient with a history of progressive dete-rioration in vision you may consider examining:

• Visual acuity• Fundoscopy – retinitis pigmentosa, diabetic

retinopathy• Eye movements• Visual fields – peripheral loss first

It is important to tailor your examination accordingto the presenting complaint.

However, it is still important to know how toexamine the eye in a systematic way. Fundo-scopy is a core skill for a practising clinician andMRCP candidate, but one with which many haveconsiderable difficulty.


General appearance of the eyeLook carefully for symmetry of the eyelids, pupilsand general eye movements. In particular, checkfor signs such as ptosis, lid retraction or irregularpupils.

Look for clues suggesting decreased visual acuity,eg white stick, adjacent Braille books, or forevidence of other systemic diseases such as dia-betes, eg glucose testing sticks, diabetic drinks,foot ulcers or evidence of co-existent dialysis.

Visual acuityAssess acuity with the patient wearing their nor-mal glasses.

Ask the patient if they can see from each eye,and then assess individually by use of a Snellenchart. Visual acuity is defined as V ¼ d/D whered ¼ distance at which numbers are read andD ¼ distance at which they should be read.

Pupillary re£exesEach eye should be tested in turn for direct andconsensual pupilary reflexes with a pen torchwhile the patient faces forward with eyes focuseddirectly ahead.

Visual ¢eldsVisual fields should be assessed by confrontationwith you sitting approximately 1 metre from thepatient and with your eyes at the same level.When assessing the patient’s right eye, ask themto cover up their left eye, and after covering yourright eye, slowly bring an object in from theperiphery in a plane equal between you and thepatient.

What should the object be? A moving finger iscommonly used and may be fine for a screeningtest if examining the cranial nerves. The top of apen, placed in the quadrants in turn, is better, butdo not bring it inwards too quickly, as the patientmust have the time to say when they first see it.

A red pin can be used for assessing centralcolour vision that is located around the macula.

Where it is first visible gives a good indication ofperipheral vision.

Eyemovements (see p 225)

FundoscopyThis should be performed in a dark room – if it isnot dark ask to turn the lights off.

A mydriatic (dilating the pupil) such as tropica-mide may have been used. It is essential that youexamine the patient’s right eye with your righteye and their left eye with your left eye. The latteris a routine many candidates have difficulty with,but failure to do this, for whatever reason, isfrowned upon.

Find the red reflex, keep it in view and get inclose!

Although the temptation and tendency is to gostraight to the retina, you should get into theroutine of gradually racking down through thelens strengths, examining first the front of the eyeand gradually, by reducing the strength of thelens, moving to the retina, which is usually ob-served with a zero or a slightly negative lens,depending on whether or not the patient is myo-pic.

The disc and each quadrant of the retina shouldbe identified and studied in turn.

Disc – note its shape, colour (pale in opticatrophy), margins and if there is papilloedema.

Retinal blood vessels – examine the vessels,noting their diameter (arteries are narrower thanveins) and the point at which they cross. Is thereA–V nipping?

Look for arterial emboli (arteries become muchthinner or thread like) or venous thrombosis(veins become engorged with surrounding hae-morrhages).


Each retinal quadrant should be examined for thepresence of:

• Haemorrhages• dot, blot or flame shaped

• Microaneurysms• Pigmentation

• Exudates, distinguish between:• hard – white/yellow and shiny with well-

defined edges• soft (‘cotton wool spots’)

• The presence of new vessels should beidentified and any previous photocoagulationscars.

The macula – It should be specifically examinedas the surrounding area is, as the name suggests,the site of diabetic maculopathy.

Examine the peripheries of the retina, lookingparticularly for evidence of retinitis pigmentosa.

Considering the cases you might be asked to see;look around the bedside for clues.

FINAL THOUGHTS

Remember that Station 5 is very different fromthe other stations. You are somewhat liberatedfrom the regimented examination routines ofthe other stations. However, you need to gathera great deal of information and communicate aplan to the patient in a very short space oftime. It is perhaps the most accurate and globalreflection of how you will perform as a registrarin clinic or on-call. This should not be intimi-dating – if in doubt just resort to what youwould normally do in exactly that scenario atwork!


IntegratedClinical Assessment

1. Acromegaly

2. Addison’s disease

3. Ankylosing spondylitis

4. Anterior uveitis

5. Asthma

6. Atrial fibrillation

7. Coeliac disease

8. Congestive cardiac failure

9. Cranial nerve III palsy

10. Crohn’s disease/bloodydiarrhoea

11. Cushing’s disease

12. Dermatomyositis

13. Diabetic retinopathy

14. Eczema

15. Erythema nodosum

16. Gout

17. Graves’ disease

18. Henoch–Schonlein purpura

19. Hereditary haemorrhagictelangiectasia

20. Herpes zoster

21. Horner syndrome

22. Hypothyroidism

23. Impetigo

24. Marfan syndrome

25. Osteogenesis imperfecta

26. Paget’s disease

27. Papilloedema

28. Pemphigus

29. Pleural effusion

30. Psoriasis

31. Psoriatic arthropathy

32. Pyoderma gangrenosum

33. Retinitis pigmentosa

34. Rheumatoid arthritis

35. Scleroderma

36. Systemic lupuserythematosus

37. Tremor

38. Turner syndrome

39. Vitiligo

1–2

minutes

5minutes

1–2

minutes

SCENARIO 1. ACROMEGALY

Introduce yourself

Confirm the information you’ve been given and patient details: this 36-year-old man has presented with headaches.Please assess him.

‘What is the main problem from your point of view?’ ‘For the last 3 months I have been having headaches, and I amworried about my vision as I have recently crashed my car into a parked car when driving along the road. A friend has

commented that my appearance has changed.’

New condition

Focused history of the presenting complaint and relevantsystems enquiry. This should include screening forendocrine disturbance (any aspect of the hypothalamic–pituitary axis can be disrupted) and for localisingneurology (eg diplopia, visual field defect, carpal tunnelsyndrome)

Identify alarm symptoms: sudden headache, diplopia,ptosis (may imply pituitary apoplexy). Polyuria/polydipsiamay imply either diabetes insipidus or diabetes mellitus

Pre-existing conditions: hypertension, diabetes, colonicpolyps, sleep apnoea

Social/occupational/family circumstances: especially ifdriving professionally (eg taxi driver, group 1 driver)

Drug history – antihypertensives, oral hypoglycaemicsDRUG ALLERGIES

Other medical conditions: any co-morbidity that mightpreclude hypophysectomy (eg bleeding diathesis)?

Begin a targeted examination of relevant area/system andcontinue while taking the history

General appearance (see Figure 5.1)

• Prominent supraorbital ridges, nose and lips

• Pronounced jawline (prognathism)

• Macroglossia and increased interdental separation

• Enlarged sweaty hands with thickened skin

In addition

• Examine visual fields for bitemporal hemianopia.There may be optic atrophy if the adenoma extendsinto the parasellar region

• Assess for cranial nerves palsies, especially nerves III,IV and VI (if raised intracranial pressure)

• Examine for carpal tunnel syndrome/scars fromprevious decompression

• Myopathy (especially proximal) and arthropathy iscommon, but no synovitis will be found

• Examine for associated endocrine features, includinggoitre, gynaecomastia and/or hirsutism

• Perform a cardiac and abdominal examination if hassymptoms, looking for associated cardiomegaly andhepatomegaly

• Measure blood pressure, assess for glycosuria (mayreflect associated impaired glucose tolerance/diabetesmellitus)

Any old photographs of the patient available?

CALLBACK: ‘You said at the beginning that the main problem was headaches, change in vision and appearance. Is thereanything else troubling you that we haven’t covered?

RESPOND TO PATIENT’S CONCERNS (1–2 min)

1. I think that your headaches, change in vision and appearance are due to a condition resulting from acromegaly,which is caused by a benign growth in the pituitary gland that produces too much growth hormone

2. For your headaches, I can give you some painkillers as a temporary measure. However, it is very important toestablish the diagnosis by doing some urgent investigations. As your vision is impaired, I am afraid that you will haveto stop driving immediately because of the risk to yourself and other road users

3. I am going to organise some urgent blood tests (glucose tolerance tests, IGF-1, other pituitary hormones). We willneed to arrange an urgent MRI of your pituitary gland and ask the eye specialists to see you to assess to what extentthis growth has affected your vision

Thank the patient


Acromegalic facies Large tongue

Increased interdental spacing

Figure 5.1 Acromegaly


Differential

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Acromegaly is a clinical syndrome resulting fromthe hypersecretion of growth hormone (GH). Mostcases are caused by GH-secreting adenomas situ-ated in the anterior pituitary. Approximatelythree-quarters of these are macroadenomas (ie.10 mm diameter) at the time of diagnosis. Theseadenomas account for approximately a third of allhormonally secreting pituitary adenomas. Rarecauses of acromegaly include growth hormone-releasing hormone (GHRH) secretion fromhypothalamic tumours, or ectopic GHRH or GHsecretion (eg from neuroendocrine tumours).

Adenomas tend to grow very slowly; it is esti-mated that there is an average delay of more than10 years between initial symptoms and diagnosis.The most consistent early symptoms (and thosemost likely to form the stem of a Station 5question on the condition) include headachesand visual disturbance. Other relatively earlyfeatures are those of endocrine disturbance, parti-cular gonadotrophin deficiency, ie oligo-/amenor-rhoea in women, or erectile dysfunction, loss oflibido and testicular atrophy in men. As thecondition progresses, a large number of organsystems may be affected, and a number of physi-cal signs may develop, as described in the differ-ential diagnosis table. There may also be signs oftreatment of the condition (eg scars around thenose reflecting previous transsphenoidal surgery).

Acromegaly incurs a significantly increased mor-tality rate (estimated as at least twice as high) asthe healthy population, with those with the high-est GH levels at the greatest risk of prematuredeath. This is mostly attributable to increasedcardiovascular disease.

Investigations1. Establish the presence of GH excess• Random GH plasma levels in themselves are

not diagnostic, because these fluctuate greatlybetween individuals and at different times ofthe day. However, serum insulin-like growthfactor 1 (IGF-1) levels are elevated in almostall patients with acromegaly.

• The most effective means of diagnosing GHexcess is with an oral glucose tolerance test;although GH levels suppress to very lowlevels after a glucose load in healthyindividuals, GH levels will fail to suppress orparadoxically rise in patients withacromegaly.

2. Establish the aetiology (Table 5.1)• Pituitary imaging should be performed; MRI is

the most sensitive modality.• Establish whether a pituitary adenoma is

functional.

3. Look for complications• Visual perimetry: to establish visual fields.• ECG, echocardiogram and chest radiograph:

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, performa dynamic test, ie insulin or glucose tolerance testThyrotroph: T4, T3 and TSHGonadotroph: LH, FSH, testosterone and SHBG orestradiolLactotroph: prolactin

ADH secretion – plasma and urinary sodium andosmolality. A water deprivation test may also berequired


• Oral glucose tolerance test: used to make thediagnosis, but also to establish the presenceof associated impaired glucose tolerance/diabetes mellitus.

• Nerve conduction studies: if an associatedcarpal tunnel syndrome is suspected.

• Sleep studies: if obstructive sleep apnoea issuspected.

• Colonoscopy: surveillance for polyps andcolorectal malignancies, a recognisedcomplication of acromegaly.

• Bone profile and parathyroid hormone: thecombination of acromegaly andhyperparathyroidism raises the possibility ofmultiple endocrine neoplasia type 1 (MEN-1)syndrome; there should be a low threshold toinvestigate for a possible pancreatic tumour.

TreatmentSurgical• Transsphenoidal hypophysectomy is

successful in up to 90% of patients withmicroadenomas and 50% of those withmacroadenomas in the hands of the mostexperienced neurosurgeons.

• Long-term deficiency in one or more of thepituitary hormones will develop inapproximately 70% of patients.

Radiotherapy• Pituitary external irradiation is indicated for

patients unfit for or not cured by surgery. Itresults in a 50% decline in GH levels by2 years, but with a continuing exponentialdecline thereafter.

• There is a significant risk of latehypopituitarism, with other complications

including cranial neuropathies and visualfield impairment.

Medical• Somatostatin analogues (eg octreotide or

lancreotide) are administered subcutaneously.They reduce serum GH in 90% of patients,and to a level of GH that will preventadenoma growth in approximately 50% ofpatients. Side effects are nausea andgallstones (approximately 50% by 5 years).

• Dopamine agonists (eg bromocriptine,cabergoline) are effective in only 10% ofpatients.

• Pegvisomant is a GH receptor antagonist,administered subcutaneously daily, whichnormalises serum IGF-1 in .90% of patients,helping to reduce many of the clinicalfeatures. However, it does not reduce GHsecretion or constrain tumour growth, so it iscurrently reserved for patients with ongoingdisease despite surgery, radiotherapy andother medical therapies.

Maintaining patient welfareIt is important in managing acromegalic patientsto think not only about the immediate treatmentfor the condition, but also about the means ofminimising long-term complications. Candidatescan show that they are aware of these by sug-gesting colonoscopic screening for colonic ma-lignancy (this is currently recommended every 3years), and minimising the patient’s risk ofcardiovascular disease by optimising their bloodpressure, lipid profile and smoking cessationadvice.


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