Shorts Masterset Flashcards

Question

**Scleroderma**

Answer 1

**CREST** - calcinosis, raynaud's, esophageal dysmotility, sclerodactylyl, telangectasia **Features** * Malabsorption * Bird-like facies **Hands** * Sclerodactylyl * Digital ulcerations/pits * Raynauds (temperatures) * Tendon friction rubs at palms/wrist * Calcinosis * Joint tenderness/synovitis * Function - key test **Arms** * Skin thickening (if distal to knee/elbow = limited) * "Salt/pepper" rash, hair loss * Proximal muscle weakness * Fistula/permacath * BP **Face** * Anaemia * Alopecia * Skin tightening * Telangiectasia * Mouth opening (3 fingers) **Systemic** * Skin tightening on chest * Cardiac - PAH, pericarditis, cardiac failure * Resp - ILD * Abdo - pulsatle liver **Investigations** * Antibodies: * ANA (95%) * ENA * anti-SCL-70 (dxSSC - ILD) * anti-centromere Ab (LcSSc - protects against ILD/renal) * anti-RNA polymerase III (dcSSC - renal) * Anti-Th/To (rare - both; PAH) * ESR/CR * ESR/CRP - disease activity * RF/CCF (ddx) * Urine PCR/renal function * RFTs/TTE/ECG - PAH/ILD * Hand X-Ray - acro-osteolysis (loss of distal phalanx)

Answer 2

**Demyelinating** * DDx: MS, CMT, CIDP, Diabetes, paraprotein (MM) * Normal amplitude * Reduced velocity * Increased distal latency **Axonal** * DDx: diabetes, toxins, metabolic, paraneoplastic * Reduced amplitude * Normal to mildly reduced velocity

Answer 3

**Hereditary** * Duchenne's Muscular Dystrophy (X-linked) * Affects only males * Calves and deltoids are hypertrophied early and weak later * Early proximal weakness * Tendon reflexes preserved in proportion to muscle strength * Severe progressive kyphoscoliosis * Dilated cardiomyopathy * CK high * Becker (less severe) muscular dystrophy * Less severe than Duchenne's * Later onset and less rapidly progressive * Limb girdle muscular dystrophy (autosomal recessive) * Facial and pectoral girdle weakness * Hypertrophy of the deltoids * FSHD (autosomal dominant) * Often asymmetric * Polyhill sign (deltoid typically spared) * Remember to test scapular winging: flexion + wall press * Limited abduction + flexion * Subtle facial weakness (ask pt to whistle, close eyes tightly, puff out cheeks) * Foot drop * Distal dystrophies * Autosomal dominant disease (rare - causes distal muscle atrophy and weakness) * Myotonic dystrophy * Mitochondrial myopathy **Acquired** * Inflammatory myositis - polymyositis, dermatomyositis * Drugs/Toxins - ETOH, steroids * Endrocinopathy - thyroid, Cushing's, acromegaly, hypopituitarism * Autoimmune - sarcoidosis * Malignancy * Osteomalacia **Investigations** * CK (highest in Duchenne's) * EMG - small motor units (NCS - normal) * ECG - especially Duchenne's and myotonic dystrophy) * Muscle biopsy * TTE - cardiac Ix **Differentials** * Myasthesia Gravis * Motor neurone disease * Polyradiculopathy

Answer 4

**"DAM IT BICH"** * Diabetes * Drugs + toxins - chemo (cisplatin), isoniazid, phenytoin, nitrofurantoin, amiodarone, heavy metals * Alcohol * Metabolic - uraemia, hypothyroidism, porphyria * Immune - GBS, CIDP * Tumour - lung (paraneoplastic) * B12/B6 deficiency * Idiopathic * Connective tissue - SLE/PAN * Hereditary * Charcot-Marie Tooth **Predominantly Motor (3C3D3M3P)** * CIDP/GBS * Cauda equina * CMT * Dapsone * Diptheria * Diabetes * MG * MND * Multifocal motor neuropathy * Polio * Porphyria * Poisoning (lead) **Predominantly Sensory** * Diabetes * Paraneoplastic/paraproteinaemia * Sjogrens * Syphilis * B12 deficiency/B6 intoxication * Idiopathic **Painful Peripheral Neuropathy** * Diabetes * ETOH * Vitamin B12/B1 deficiency * Carcinoma * Porphyria * Arsenic/thallium poisoning * Hereditary **Investigations** * FBC/eLFTs * ESR/CRP * HBA1c * TFTs * B12/Folate * Serum EPP, urine Bence Jones * ENA, ANCA * NCS + EMG * Nerve Biopsy * Genetic testing

Answer 5

**Compressive** * Syringomyelia * LMN UL, UMN LL * Dissociated sensory loss (loss of STT, preserved DCML) * Spinal level - go down not up * Caused by Arnold-Chiari, tumour, spinal cord injury, infection * Look for INO (C5), CN 9-12 (medulla involvement) * Spondylotic myelopathy (disc prolapse) * Neoplasm * Abscess **Inflammatory** * Transverse myelitis * Anterior spinal artery occlusion * Infection - bacterial, viral) * NMO * Vasculitis * Demyelination (ie MS) * Radiation * Connective tissue disease * MS * Sarcoidosis * Paraneoplastic syndrome **Infection** * Epidural abscess * Acute viral myelitis (enterovirus, flavivirus) * AIDS myelopathy * Syphilis (dorsal column) **Vascular** * Spinal cord infarction * Vascular malformation * Epidural haematoma **Toxic** * Subacute combined degeneration * Radiation myelopathy * Intrathecal chemotherapy

Answer 6

Hemisection of the Cord * Ipsilateral LMN at level of lesion, UMN below the lesion * Contralateral STT below the lesion * Ipsilaretal DCML below the lesion

Answer 7

* UMN in legs * Loss of DCML * Loss of ankle jerk (but hyper-reflexic in knees)

Answer 8

**Features** * Hypomimia * Masked facies * Slow blink rate (glabella tap) * Hypophonia * Gait - stooped, reduced arm swing (often asymmetric), short stride length/shuffling + note tremor --\> festination, freezing, turning en bloc + multiple steps to turn * Tremor - rest, asymmetric pill rolling - comfortable posture + distract (eyes closed, count backwards) * Rigidity - cogwheeling, lead pipe (increase with coactivation; head turning, painting a wall) * Bradykinesia with decrement * May have duodopa running through PEG-J or subcut apomorphine pump); look for DBS battery * Other exam: * EOM (vertical first, PSP) * Cerebellar signs (MSA), lying/standing BP, postural instability * Other: writing (micrographia), Archimedes spiral (tremor), cognitive Ax (DLB) **Differential Diagnoses** * Drugs * Typical and atypical antipsychotics - chlorpromazine, haloperidol, olanzapine * Metoclopramide * MSA * DLB * CBD * Vascular parkinsonism * Normal pressure hydrocephalus **Further Investigations** * Drug history * MRI (if atypical features) * Trial of levodopa

Answer 9

* Ptosis +/- weak EOM and orbicularis oculi * Fatiguability * Look at the ceiling for two minutes * Abduct the arms and push down every second, and see fatiguability **Investigations** * Antibodies * Acetylcholine receptor antibodies * Anti-MuSK antibodies * Repeat nerve stimulation * Single fibre EMG * RFTs * CXR - r/o thymoma

Answer 10

* Frontal balding * Dull triangular facies * Temporalis, masseter and sternomastroid atrophy * Partial bilateral ptosis * Neck - SCM atrophy; flexion weak, extension normal * UL * Grip myotonia (shake hands) * Percussion myotonia (tap over thenar eminence --\> contraction then slow relaxation of abductor pollicis brevis) * Wasting and weakness; especially of forearm muscles * Sensory changes with peripheral neuropathy * Gynaecomastia * LL if time **Investigations** * Family history + genetic testing (DMPK) * Diabetes - urine sugar, HbA1c * ECG and TTE - CMP, MVP, SVT/AF * RFTs (restrictive) * Sleep study - disordered breathing * MMSE - cognitive deficit * EMG: dive bomber effect with needle movement in muscle at rest

Answer 11

* Bilateral cerebellar signs including nystagmus and truncal ataxia * Dorsal column loss * UMN signs (but loss of ankle jerk due to weakness) * Distal weakness * Pyramidal - UL extensors, LL flexors d * Peripheral neuropathy * Optic atrophy * Pes cavus **Other Signs:** * Cardiomyopathy * Diabetes * Hearing loss * Dysphagia * Kyphoscoliosis * Sleep disordered breathing **Investigations** *

Answer 12

**Hereditary** * Benign familial chorea * Wilson's disease * Huntingtons * Spinocerebellar ataxia **Acquired** * Deep brain lesion * Drugs - phenytoin, carbamazepine, lithium, levodopa * Malignancy/paraneoplastic * Autoimmune - SLE, Sydenhams, rheumatic fever * Infections - syphilis, HIV, TB * Life - pregnancy, senility

Answer 13

* **Normal Pupils** * Senile ptosis * Myotonic dystrophy * FSHD * Ocular myopathy - mitochondrial * Thyrotoxic myopathy * MG * Botulism/snake bite * Congenital * **Constricted Pupil** * Horner's syndrome * Tabes dorsalis * **Dilated Pupil** * 3rd nerve palsy

Answer 14

* Carcinoma - lung, thyroid * Carotid artery dissection * Lateral medullary syndrome * Brainstem lesion * Retro-orbital lesion **Investigations** * MRI Brain and spine * USS Neck * CT Chest * CTA - arch to circle of willis

Answer 15

Lesion at the MLF * Demyelination (eg MS) * Tumour * Vascular - thrombus * Trauma * Infective (bacterial, viral) * Toxin - ETOH, TCA, lithium * Hepatic encephalopathy

Answer 16

**Superior Orbital Fissure** * CN 3, 4, 6 **Cavernous Sinus** * CN 3, 4, 5, 6 **CPA Tumour** * CN 5, 7, 8 **Lateral Medullary Syndrome** (usually PICA infarct) * CN 5 - trismus due to masseter, ipsilateral pain and temp * CN 9 - dysphagia, dysarthria, dysphonia. Hoarse voice, absent gag * CN 10 - cough * Ipsilateral Horner's * Ipsilateral cerebellar/vestibular * Contralateral pain and temperature of trunk and limbs

Answer 17

From lesion in dorsal pons (stroke, MS, tumour) Horizontal gaze palsy to affected side, impaired adduction to other side

Answer 18

* MS * Toxic - ethambutol, ETOH, nicotine * Metabolic (B12) * Ischaemic - DM, temporal arteritis, atheroma * Infective - EBV

Answer 19

**Central** * Vascular (ie brainstem) * Demyelination (rare) * Tumour * Trauma * Idiopathic **Peripheral** * Compressive * PCOM aneurysm * Raised ICP * Orbit lesions - superior orbital fissure * Basal meningitis * Nasopharyngeal carcinoma * Infarction - DM, arteritis * Trauma * Cavernous sinus lesion

Answer 20

**Bilateral** * Trauma (had injury) * Wernicke's encephalopathy * Raised ICP * Mononeuritis multiplex **Unilateral** * Central * Tumour * Stroke * Wernicke's encephalopathy * MS (rare) * Peripheral * Diabetes, other vascular lesions * Trauma * Idiopathic * Raised ICP

Answer 21

* Progressive supranuclear palsy * Parinaud's syndrome (lesion in dorsal midbrain)

Answer 22

**Central** (pons, medulla and upper cervical cord) * Vascular * Tumour * Syringobulbia * MS **Peripheral** (posterior fossa) * Aneurysm * Tumour (skull base eg acoustic neuroma) * Chronic meningitis **Trigeminal Ganglion** (petrous temporal bone) * Meningoma * Fracture of the middle fossa **Cavernous Sinus** (Ax 3rd/4th/6th) * Aneurysm * Thrombosis * Tumour **Other** * Sjogren's syndrome * SLE * Toxins * Idiopathic

Answer 23

**UMN** * Vascular * Tumour **LMN** * Pontine (Ax V/VI) * Vascular * Tumour * Syringobulbia * MS * Posterior fossa * Acoustic neuroma * Meningoma * Petrous temporal bone * Bell's palsy * Ramsay Hunt syndrome * Otitis Media * Fracture * Parotid * Tumour * Sarcoid * Bilateral Disease * GBS * Bilateral parotid disease (eg sarcoidosis) * Mononeuritis multiplex (rare) * Myopathy and NMJ can mimic

Answer 24

**Webers Test:** * Conduction - localises to bad ear * Sensorineural - localises to good ear **Rinne's Test** * Sensorineural: AC \> BC (positive) * Conduction: BC \> AC (negative) **Sensorineural Deafness** * Degeneration * Trauma (eg high noise exposure, fracture of petrous temporal bone) * Toxic (eg aspirin, ETOH, streptomycin) * Infection (eg congenital rubella/syphilis) * Tumour (eg acoustic neuroma) * Brain stem lesions * Vascular disease of internal acoustic artery **Conductive Deafness** * Wax * Otitis Media * Otosclerosis * Paget's Disease of bone

Answer 25

**Myotomes:** * Shoulder abduction: C5 (axillary) * Elbow flexion: C6 (musculocutaneous) * Wrist flexion: C7 (median) * Finger flexion: C8 (median) * Elbow extension: C7 (radial) * Wrist extension: C6 (radial) * Finger extension: C7 (radial) * Finger abduction/adduction: T1 (ulnar) **Reflex****es** * Supinator C5/6 (radial) * Biceps C5/6 (musculocutaneous) * Triceps C7/8 (radial) **Localising Radial Nerve Injury** * If only elbow ext weak - C7 * If elbow ext and all below weak - at spiral groove * If elbow and finger weak but normal brachioradialise - posterior interosseous

Answer 26

* Common peroneal nerve palsy * Sciatic nerve palsy * Lumbosacral plexus lesion * L4/L5 root lesion * Peripheral motor neuropathy * Distal myopathy * Motor neurone disease * Precentral gyrus lesion Test ankle jerk --\> if absence, S1/sciatic nerve lesion suspected; if normal common peroneal/L5; if increased UMN/MND Inversion is normal in common peroneal but absent in L5 radiculopathy (eversion is absent in both)

Answer 27

* Ischaemia * Drugs - ETOH, phenytoin, lithium, carbamazepine * Hereditary - Fredreich's ataxia, spinocerebellar ataxia * Paraneoplastic (ovary, uterus, breast, HL, SCLC) * MS * Posterior fossa tumour **UMN (Spastic) + Cerebellar (Ataxic)** * Fredreich's ataxia * Spinocerebellar ataxia * MS * Infarction (upper pons, bilateral internal capsule) * Arnold Chiari * Syringomyelia

Answer 28

* Pes cavus (short high arched feet with hammer toes) * Distal muscle atrophy (from peripheral generation) --\> inverted champagne bottle leg * Does not extend above the elbows or above the middle one third of the thighs * Areflexia/hyporeflexia (always show reinforcing manoeuvres) * Slight or not sensory deficits in the limbs * Thickened nerves * Foot drop --\> high stepping gait, look for AFOs/Dictus bands * Bedside: opthalmoscope (optic atrophy) * Ix: NCS, genetics (PMP22), OT for functional assessment

Shorts Masterset Flashcards

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