NEUROLOGY FOR MRCP The Essential Guide to Neurology for MRCP Part 1, Part and PACES NE The Esser P arti, Pa Jonathan D Rohrer Jonathan Kennedy UCL Institute of Neurology, Queen Square, UK Imperial College Press Im perial C o lle g e Press S h elto n Street C o v en t G arden L o n d on W C H H E D istributed by W o rld S c ie n tific P u blish in g C o P te Ltd T o h T u ck L in k , Sin gap ore 2 USA office: W arren S treet, Su ite -4 , H ack en sack , N J UK office: S h elto n S treet, C o ven t G ard en , L on d on W C H H E British L ib ry Cataloguing-in-Publication D ata A catalo g u e reco rd fo r this b o o k is av ailab le fro m the B ritis h L ib rary C o v e r im ag e: © D r M ich a el M ille r & M T P In c T o k y o N EURO LO G Y FO R M R C P T he Essential Guide to Neurology for M R C P P a r t 1, P a r t and P A C E S C o p yrig ht © 1 by Im p erial C o lle g e Press A ll rights reserved This book, or p a n s thereof, m ay not be reproduced in any fo rm or by any means, electronic o r mechanical, including photocopying, recording or any information storage and retrieval system now known or to be invented, without written perm ission fro m the Publisher F o r p h o to co p y in g o f m aterial in th is v o lu m e, p le a se p ay a co p y in g fe e th rough th e C o p y rig h t C le a n ce C e n ter, In c , 2 R o se w o o d D riv e , D a n v ers, M A , U S A In th is c a s e p e rm issio n to p h o to co p y is n ot re q u ired fro m the p u b lish er I S B N - - - 8 - - (p b k ) I S B N - 1 - 8 - - (p b k ) T y p e s e t by S ta llio n P ress E m a il: e n q u irie s@ s ta llio n p r e s s c o m Printed in Sin gap ore Contents List o f Figures vii Acknowledgements ix Introduction xi Part N eurological Disorders Chapter Chapter Basic Anatomy Dementia and Delirium 10 Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter Chapter 15 20 22 28 30 33 40 43 45 55 60 62 Chapter 16 Epilepsy Raised Intracranial Pressure Cerebrovascular Disease Headache N euroinfectious Disease M ovem ent Disorders M ultiple Sclerosis Cerebellar Disease Cranial Nerve Disorders Disorders of the Spinal Cord Anterior Horn Cell Disorders Nerve R oot and Plexus Disease M ononeuropathies and M ononeuritis M ultiplex Peripheral Neuropathies Chapter 17 Chapter 18 Chapter 19 Neurom uscular Junction Disorders M uscle Disorders Investigations 73 77 81 10 11 12 13 14 15 V 65 68 vi Contents Part Neurological Exam ination for PACES Chapter 20 Chapter 21 Part Cranial Nerve Territory Exam ination Limb Exam ination Questions for Parts and 87 89 102 119 Questions for Part Questions for Part Answers to Questions 121 136 154 Index 157 List of Figures Cerebral hemisphere showing the frontal, tem poral, parietal and occipital lobes A diagram of the brainstem showing the position of the cranial nerve nuclei Anterior and posterior dermatomal map C T scans showing a left middle cerebral artery infarction (on the left) and right hemisphere intracerebral haemorrhage (on the right) M R I scans showing demyelination in the brain and spine A schematic diagram explaining spinal cord syndromes Examples o f M R I scans showing coronal T l , axial T and axial FLA IR images A diagram showing visual field defects Vll 23 41 56 86 93 Acknowledgements W e would like to thank Rebecca M iller and Fiona Kennedy for their support during the writing of the book W e would also like to thank Dr M ichael M iller for providing the cover design and Dr Edward W ild for kind permission to use the dermatomal map Lastly, we would also like to thank Dr Camilla Clark, Dr Lucy Reynolds and in particular Dr Phillip Kennedy for reviewing the manuscript IX N eurology for MRCP 149 D) Poliomyelitis E) Spinal muscular atrophy Question 30 A 37-year-old factory worker presents to you with a one-week his tory of severe right shoulder and neck pain He is taking maxim al doses of paracetam ol and ibuprofen with little benefit You prescribe opioid-based analgesics He returns four weeks later with little pain but he complains of shoulder weakness affecting abduction On exam ination, you note mild winging of the scapula W hat is the most likely diagnosis? A) radiculopathy B) C5 radiculopathy C) Brachial neuritis D) Cervical rib E) Neoplastic infiltration of brachial plexus Question 31 A 27-year-old woman presents with a ten-day history of lower back and buttock pain In the last five days she has developed a dimin ished desire to micturate and defecate and when she does she feels the need to strain She also complains that toilet paper feels strange over the vulval and perianal area On exam ination, she has slight dif ficulty with standing on her tiptoes but power on the bed is full throughout Tone is normal Reflexes are present but the right ankle jerk requires reinforcement Plantars are flexor An urgent M R I scan o f the spine is requested and reveals the cause of her symptoms W hich of the following is most likely? A) B) C) D) E) Demyelination of the cervical cord Central disc prolapse at S I Infiltrative mass from the cervix Conus medullaris lesion Thoracic syrinx 150 Questions Question 32 A 36-year-old lady presents com plaining of nocturnal hand pain W hich of the following conditions are not associated with carpal tunnel syndrome? A) B) C) D) Acromegaly Hypothyroidism Pregnancy Rheumatoid arthritis E) Addison’s disease Question 33 A 43-year-old man presents with wasting o f the left hand and a burning pain in the right hand He was diagnosed with asthma five years ago and uses salmeterol and fluticasone inhalers twice a day He was admitted for an acute asthma attack earlier in the year requiring nebulisers and oral steroids On exam ination, he has mild expiratory wheeze There is wasting of the first dorsal interosseous in the left hand with weakness of finger abduction whilst in the right hand there is mild weakness of the abductor pollicis brevis and decreased sensation in the thumb and index finger Routine blood tests reveal a moderate eosinophilia, an ESR o f and a positive ANCA W hat is the most likely diagnosis? A) B) C) D) E) Sarcoidosis Polyarteritis nodosa Churg-Strauss vasculitis Diabetes H IV Question 34 A 27-year-old man is admitted to hospital with a one-week history of difficulty climbing stairs at work He was treated by his GP with antibiotics for a fever and cough two weeks ago which resolved with treatment On exam ination, he has bilateral foot drop but also more N eurology for MRCP 151 proxim al weakness in the legs He is areflexic but there is no sensory loss His upper limb and cranial nerve territory exam inations are normal W hat is the most likely diagnosis? A) Botulism B) G uillain-Barre syndrome C) Porphyria D) M yasthenia gravis E) Polymyositis Question 35 A 23-year-old woman developed an acute onset of weakness in her legs shortly after returning from a holiday in Spain Over the next few days she developed worsening weakness in the legs and also weakness in both arms She attends A+E W hat is the m ost im por tant initial investigation? A) FBC B) Peak flow C) Vital capacity D) Serum potassium E) ABG Question 36 A 32-year-old man presents with an acute onset o f weakness in the legs His partner has also noted him to be confused and agitated today He has been diagnosed with irritable bowel syndrome after several episodes of unexplained abdominal pain, one o f which required hospital admission Exam ination reveals distal limb w eak ness with areflexia The sensory exam ination is difficult to interpret given his confusion but appears grossly intact W hat is the likely diagnosis? A) G uillain-Barre syndrome B) Lead poisoning C) Botulism 152 Questions D) Porphyria E) M ultifocal motor neuropathy Question A 55-year-old woman has been com plaining of weakness in her hands and feet with some loss of sensation distally Nerve conduc tion studies showed reduced conduction velocity but norm al amplitude W hich disorder is the most likely cause? A) B) C) D) E) Diabetic neuropathy Paraproteinaemic neuropathy Vasculitic neuropathy Paraneoplastic neuropathy Drug-induced neuropathy Question 38 A 66-year-old man presents with a two-m onth history o f feeling weak and tired with difficulty getting out of his chair He had been constipated for the last month and in the week before seeing you he had had difficulty swallowing He used to smoke cigarettes per day for many years and drank around 30 units o f alcohol per week Limb exam ination revealed mild proxim al weakness in all four limbs as well as decreased reflexes throughout However, the reflexes returned to normal after exercising that muscle group W hat test would confirm the diagnosis? A) B) C) D) E) Acetlycholine receptor antibodies Nerve conduction studies CK Nerve biopsy M R I spine Question 39 A 41-year-old man presents with progressive difficulty in climbing stairs as well as difficulty at work as a carpenter with manipulation Neurology fo r MRCP 153 o f tools He reports no other neurological symptoms but that his work colleagues refer to him as ‘sleepy’ on account o f his facial appearance He is married but has no children as he and his wife have not been able to conceive His immediate family are well but a paternal uncle and his grandfather were both said to have had a ‘nerve disorder’ On exam ination, he has bilateral ptosis, weakness in the hands and feet but the rest o f the exam ination is norm al W hat is the most likely diagnosis? A) Graves’ disease B) C) D) E) M yasthenia gravis Leber’s optic atrophy M yotonic dystrophy Freidreich’s ataxia Question 40 A 22-year-old man presents to A+E with rapidly progressive w eak ness, developing over two hours following a large family meal to celebrate the Chinese New Year He is globally weak, unable to move at all with just a flicker of power in the limbs, with absent reflexes, but speaking and swallowing are normal as is sensation Cranial nerves are normal By the next morning he is completely better W hat is the diagnosis? A) B) C) D) E) Porphyria Hypokalaemic periodic paralysis Psychogenic weakness G uillain-Barre syndrome M yasthenia gravis Answers to Questions All questions are based on disorders discussed in the main text Refer to the particular section to learn more about the condition P art 1 D E A C 10 11 12 13 14 15 16 17 18 19 21 2 C E B D D A D B C and D E E D B B A E D D 154 N eurology fo r MRCP B 24 D 25 26 27 28 29 30 31 32 33 34 B D C D C D C D E B 35 36 37 38 39 40 E D B B D A P art B B D E C 10 11 12 13 14 15 A C B A B D C and G D C B 155 156 Questions 16 B and G 17 D 18 19 20 21 2 C E D B D A E 29 30 A E E B D C 31 32 33 34 B E C B 35 C 36 D 37 B 38 B 39 D 40 B Index Acoustic neuroma Adie’s pupil , 52, 82, Bitemporal hemianopia 47 Adrenoleukodystrophy (ALD) Blepharospasm Botulism , 83 Adrenomyeloneuropathy Akinetic-rigid syndrome Brachial plexus 33, 63 63 Brain tumours Alzheimer’s disease (AD) 20 B ro ca’s aphasia 10, 11, 101 Brown-Sequard syndrome 13, 101 Bulbar palsy 94 Antiphospholipid syndrome , 98 22, CADASIL 37 Argyll-Robertson pupil 2 ,2 Carbamazepine 47, 48, Carotid dissection 94 Arnold-Chiari malformation 43, 9, 96 Ataxia telangiectasia - , 4 , 51 , 48 Carpal tunnel syndrome 5, 14 Cauda equina syndrome 62 Cavernous sinus syndrome 43 Cerebellopontine angle Cerebellum (CVST) 1, 97 , 2 , , 28 C h a r c o t-M a rie -T o o th (CM T) Benign paroxysmal positional disease 2, 53 Beta-interferon 5, 14, 110 Cerebral venous sinus thrombosis 7 - , 118 vertigo 46, 50, 51, , 5, 12, Becker muscular dystrophy Bell’s palsy 46, , 92, 95, 96 33 Basal ganglia 56, 1 ,112 10, , 5, 67 , - Anisocoria Athetosis 38 , 75 Brachial neuritis 83, 116 - , 110 Amyloid , 2, 93 42 Chorea 157 71 33, 36, , , 110 158 Neurological Disorders Diphtheria Chronic inflammatory , 69 demyelinating polyneuropathy Dorsal columns (CIDP) Duchenne muscular dystrophy - , 84 6, 9, 56, 108 79, 118 Chronic progressive external ophthalmoplegia (CPEO) 78 , Dysarthria , , , , 5, , - 0 , 06 , 17 79 Cluster headache Dyskinesias , , 48 Cogan’s syndrome 3 , , , , 100, 110 52 Commone peroneal neuropathy 66 Dysphasia Dystonia Complex ophthalmoplegia 9 , 100 33, , , 38, 82 49, Electromyography (EM G ) , , 95 Computed tomography (CT) 16, , , , , 74, 85 Conduction aphasia 101 Congenital myasthenic syndromes Encephalitis Epilepsy 13, , , 32, 5 - , , 80 Episodic memory E rb ’s palsy 73, 76 Corticobasal degeneration (CBD) Essential tremor 38 Fabry’s disease 5, , 3, , 98 12, 36 83 Facioscapulohumeral (FSH) dystrophy Creutzfeldt-Jakob disease (CJD) 118 Femoral neuropathy Cryptococcus Cysticercosis Foot drop , 31 Dementia 67 , 13, 11 , 118, 119 32 Friedreich’s ataxia Delirium 6, 4 , , 57, 104 Cranial nerves 64 Executive function 11, 3 , 35, 36 Corticospinal tract 61, 69, - , 7, , 84, 85 13, 10, 13 , 4 , 81, 11 , 115 - , - , 60, 101 Frontotemporal dementia (FTD) Dementia with Lewy Bodies (DLB) 10, 11, , , 101 10, 11, 13, 33, Dentatorubro-pallidoluysian atrophy (DRPLA) Dermatomyositis , , 81 77, 78, 116, 117 Diabetic amyotrophy 64 Gait 12, , 9, - , 11 , 119 Gaucher’s disease 82 Giant cell arteritis 46 Glatiramer acetate 42 159 Index Glomus jugulare 54 Juvenile myoclonic epilepsy (JME) Guillain-Barre syndrome (GBS) 18 - , 68, 69 , 72, 84, 97, 98 Kearns-Sayre syndrome Klumpke’s palsy Headache 15, , - , , 84, 78, 80 64 K orsakoff syndrome 14 85 Hemiballism 37 L am b ert-Eato n myasthenic Hereditary spastic paraparesis (HSP) Lamotrigine Herpes simplex virus (HSV) H IV syndrome ,5 ,1 31 12, , 32, , 70 , 94 Homonymous hemianopia 25, , 92, 94 , 92, 94 67 Lateral medullary syndrome neuropathy (LH O N ) Levodopa , 26, , , , 92 , , 98 , , , 110 41 Limb girdle dystrophy 118 Lumbosacral plexus 12, , 81 12, , Lyme disease 63 , , 51, , , 67, 70, 33 Magnetic resonance imaging (MRI) Idiopathic intracranial hypertension (IIH) 46 16 Lhermitte’s sign Huntington’s disease (HD) , 84 Inclusion body myositis (IBM) , , , , , , 27, 32, , , 51, , 82, 5, 86 M alaria 78 Internuclear ophthalmoplegia 14, , , , 95, 110,112 Intracerebral haemorrhage 23, 32 Marcus Gunn pupil 49 M edian neuropathy 65 M E LA S 85 2 , , 80 M eniere’s disease Meningitis 52, 53 13, , - , , 83 Jugular foramen syndrome , 97, 98 25, , , , , 98 Levetiracetam H o rn er’s syndrome (INO) cutaneous Leber’s hereditary optic Homonymous quadrantanopia Hyperkinesias Lateral femoral neuropathy Holmes-Adie syndrome Hydrocephalus , 74, 85 16, 17 46, Meralgia paraesthetica M ERRF 78, 80 67 160 N eurological Disorders Metachromatic leukodystrophy 46, 57 83 Migraine N iem ann-Pick disease , , , 53 Miller Fisher syndrome (MFS) , , 51, , , 5, Miosis Neuromyelitis optica (N M O ) , , , 2, 94 Mitochondrial disease , 50, 82 Normal pressure hydrocephalus (NPH) 12 Nystagmus 14, , , , 90, 92, 95, 96, 52 M itoxantrone Oculogyric crisis 42 Mononeuritis multiplex , 65, dystrophy (O PM D) 67 , 113, 114 M o to r neurone disease (MND) 11, , 53, 54, , , 98, Ophthalmoplegia , 3, 75 , 79, 80, - , Optic neuritis 12, 0, 41 , , , , , , 53, 54, Paget’s disease , 84, 98 , 1 , 112 Papilloedema Multiple system atrophy (MSA) 3 , 35, , 107 Myasthenia gravis 50, 51, 53, , , 2, 94 12, 18, 33, , 36, 82 Paraproteinaemic , 77, 79, 81, 85, 2, 97, 116, 1 48, 49 Parkinson’s disease (PD) 11, 33, , , 10 , 10 , 110 Periodic paralyses 78 Peripheral neuropathy Pes cavus 17, 83, 4 , 71, 11 , 15, 44 Phenytoin 42 65, 66, 71, 72 , 84, 85 Neuroacanthocytosis Neurofibromatosis -1 ,4 ,7 Plexopathy Nerve conduction studies (NCS) 37 71, 81 Neuroleptic malignant syndrome 38 0, 72 1 ,1 ,1 M yotonic dystrophy Natalizumab , 52 , , 93 Parinaud’s syndrome 73, 85, 92, 5, 97, 98, 116 Mydriasis 40, 46, 47, 49, 57 -7 ,1 Multiple sclerosis (MS) Myoclonus ,1 14, , , , ,1 -1 1 ,1 Multifocal motor neuropathy (M M N ) 38 Oculopharyngeal muscular Polio , 115 60, 61, 113 Polymyositis 7 , 78, 16 Polyneuropathy Porphyria 69, 70, 72 68 Pramipexole Prion disease 34 12, 43 161 Index Progressive multifocal Spinal muscular atrophy (SMA) leucoencephalopathy (PML) 32 60 Spinobulbar muscular atrophy (SBMA) Progressive myoclonic ataxia 36 Spinocerebellar ataxia (SCA) Progressive supranuclear palsy (PSP) 43, 81 1 ,3 ,3 ,4 ,9 , Spinothalamic tract 107 6, 9, , 57, 10 , 111 Pseudobulbar palsy Ptosis , 81 , 98 Status epilepticus , , , , 75, , 80, Stroke 89, 92, 4, , 10 , 1 - 1 16, 17 13, 2 - , , , 38, , , , , , , 80, 83, 85, 86, , , 00, 101, Radial neuropathy Radiculopathy 110,112 66 , 4, 113, Subacute combined degeneration 115 55 , , 111 Raised intracranial pressure 20, Subarachnoid haemorrhage (SAH) , , , , 85 Ramsay Hunt syndrome Refsum’s disease , , 84 51, 52 70 Relative afferent pupillary defect (RAPD) 49 Sydenham’s chorea ,4 Syringomyelia - , 111 Systemic lupus erythematosus Retinitis pigmentosa (RP) (SLE) 47, 12, , , , 57, 67, 70, 84 80, 93 R inne’s test Ropinirole 91 Tau 34 - , 35 T D P -43 Sarcoidosis , , , , 67, 11, 60 Temporal lobe epilepsy (TLE) , 84, 97 Seizures 18 -2 , 27, 31, 32, 41, Tension headache Thiamine 80, 82 Semantic memory Tics Sodium valproate 16, 17, , Topiramate 40, 44, 5 - , 83, 1 - 1 , 116, 28 14, 33 Torticollis 33 Spastic paraparesis 119 37 Syringobulbia Toxoplasm a ,2 38 31 Transient ischaemic attack (TIA) 22, 24 162 N eurological Disorders Transverse myelitis Trem or Ulnar neuropathy 57 66 16, 3 , , - , , 10 , 106, 10 , 10 , 110, 17, Vascular dementia 119 Vasculitis Trigeminal autonomic cephalgia Trigeminal neuralgia 7, 4 , 79, 81 Tuberculosis (TB) Vitamin D 77 Vitamin E 44 32 - , , 2, W eber’s test 82 W ernicke’s encephalopathy 90, 91 W ernicke’s aphasia 57 , 83 Tuberous sclerosis 12, 13, , 70, 115 , 51 Trinucleotide repeat disorder Trypanosomiasis 0, 1, 84 Vitamin B 28 10, 11 12, 2 , , , 67 , 68, West Nile virus Uhthoff’s phenomenon 41 W ilson’s disease 101 14 , 60 12, 33, , 43 ... Neurological Exam ination for PACES Chapter 20 Chapter 21 Part Cranial Nerve Territory Exam ination Limb Exam ination Questions for Parts and 87 89 102 119 Questions for Part Questions for Part Answers... iller and Fiona Kennedy for their support during the writing of the book W e would also like to thank Dr M ichael M iller for providing the cover design and Dr Edward W ild for kind permission to... hope fully prove that Neurology is not as difficult as it may at first seem The book is not designed, however, to be a definitive Neurology textbook but rather a revision guide for the M R C P exams