Neurology Flashcards

Question

Positive Prognostic Factors for MS

Answer 1

"Typical MS patient" - Young age at onset (20-30s) - Female - Relasping-remitting type - Sensory symptoms only - Long intervals between relapses/Complete recovery between relapses

Answer 2

MDT approach - PT/OT/SLT SYMPTOMATIC RX - Antispasmodics e.g. baclofen - Anticholinergics/ISC or LTC if bladder issues ACUTE RX - High dose steroids for 5-7 days (e.g. PO pred or IV methylpred DISEASE-MODIFYING THERAPIES (immunomodulatory) - Beta-Interferon - Glatiramer - Natalizumab

Answer 3

Autoimmune demyelinating disorder 90% relapsing-remitting Aquaporin 4 +ve antibodies = Bilateral optic neuritis + myelitis with 2 of the following: 1) Spinal cord lesion > 3 levels 2) Normal MRI brain 3) AQ4 positive serum antibody

Answer 4

1) Rigidity - Unilateral increased tone - Cogwheeling - Synkinesia 2) Bradykinesia - Shuffling gait - Hypomimia - Quiet, slow speech - "Finger tapping" or "Heel tapping" 3) Tremor - Resting, rotational element - Usually asymmetrical esp at first - Synkinesia

Answer 5

*Idiopathic Parkinson's Disease *Vascular Parkinsonism (e.g. Basal Ganglia strokes) *Drug-Induced Parkinsonism e.g. metoclopramide, haloperidol, and risperidone *Wilson's Disease PARKINSON'S PLUS *Lewy Body Disease - Hallucinations - Faster progression, less responsive to medication *Supranuclear Palsy - Falls, reduced vertical gaze, poor response to L-dopa *MSA Type P - Autonomic and cerebellar dysfunction *Corticobasal degeneration

Answer 6

MMSE/MOCA L+S BP Routine bloods - vascular RFs Neuroimaging - CT head - MRI head (vasculature of basal ganglia) - DAT scan (looks at levels of presynaptic dopamine) Assess response to treatment

Answer 7

DISEASE MODIFYING MEDICATIONS - Dopamine replacement = L-dopa + decarboxylase inhibitors = Carbidopa - Dopamine Receptor Agonists e.g. Ropirinole, Pramipexole, Rotigotine (PATCH!), Apomorphine (LATE) - IMPULSE CONTROL DISORDERS!!!!!! - COMT inhibitors e.g. Entacapone - MAO-B inhibitors e.g. selegiline SYMPTOM CONTROL - Tremor = Antimuscarinics e.g procyclidine - Dementia = Rivastigmine - L-dopa dyskinesias - Amantidine

Answer 8

MDT Approach - neurologists, PT, OT, SLT, Nutrition team, psychology MEDICAL - Dopamine replacement therapies/agents to help reduce breakdown of dopamine - Symptom control e.g. tremor SURGICAL - Deep Brain Stimulation (indicated if on medication >5x day, nil cognitive imp and suitable surgical candidate)

Answer 9

ESSENTIAL - Postural/on action (kinetic) - FHx - Alcohol/beta blockers help PARKINSONIAN - Present at rest - Low frequency, rolling - Asymmetrical (at first) - Increased with distraction DRUG INDUCED - Fine tremor, usually bilateral - E.g. salbutamol, tacrolimus, ciclosporin CEREBELLAR - Absent at rest, present with movement, getting worse closer towards target = INTENTION - Low frequency

Answer 10

Chorea = d2 damage to Basal Ganglia (esp caudate nucleus) ST VITUS DANCE + Wilson's S - Sydenham's chorea (Rheum fever) T - Trauma V - Vascular (stroke) I - Increased RBC (Polycythaemia) T - Thyrotoxicosis U - Uraemia S - SLE D - Drugs e.g. L-dopa, antipsychotics. COCP A - APS N - Neurodegenerative e.g Huntington's, Prion disease C - Chorea Gravidarum E - Exposure to toxins

Answer 11

Wasting/muscle fasciculations Reduced tone Reduced/absent reflexes Normal or downgoing plantar reflexes

Answer 12

Anterior horn cell --> nerve root --> plexus --> peripheral nerve --> NMJ --> muscle

Answer 13

ANTERIOR HORN CELL - MND (Nil sensory!!) - Poliomyelitis CAUDA EQUINA - Disc prolapse/abscess/malignancy LUMBOSACRAL PLEXOPATHY - Trauma/tumour/abscess (Usually unilateral) PERIPHERAL NEUROPATHY (See separate flashcards) - Metabolic vs Inflamm vs Infective vs Congenital vs Toxic NEUROMUSCULAR JUNCTION - Myasthenia Gravis, LEMS MYOPATHIES (See separate flashcards)

Answer 14

ANTERIOR HORN CELL - MND (No sensory features!!!!!!!!!!!!) - Poliomyelitis CAUDA EQUINA - Disc prolapse - Abscess - Malignancy (Predominantly MOTOR) PERIPHERAL NEUROPATHY - Inflam = GBS, CIDP, sarcoid - Infective = HIV - Toxic = Lead - Diabetic amyotrophy - HSMN NMJ - Myasthenia, LEMS MYOPATHIES - Polymyositis, dermatomyositis - Muscular dystrophies - Myotonia

Answer 15

If acute onset - MRI to exclude spinal cord compression 1) Take a full history - ?back pain/bladder or bowel disturbance/visual problems 2) Perform a PR (anal tone, saddle anaesthesia) 3) Examine upper limbs and cranial nerves in full as well as speech

Answer 16

BP, L+S BP, ECG ?Spirometry (FVC), ABG Bloods - Routine - Peripheral neuropathy screen (see other flashcards) - Anti-AChR antibodies - CK Neuro imaging *Special Extra Tests* (guided by history) * LP - ?GBS (raised protein) * EMG/NCS * Nerve biopsy or Muscle biopsy * Genetic testing

Answer 17

Progressive degenerative disease of UMN and LMN Affects Anterior Horn Cells and Motor Cranial Nuclei Progressive disease (50% die within 2 years of diagnosis) Mixture of UMN and LMN signs - Brisk reflexes, upgoing plantars - Wasting and fasciculations NO SENSORY DEFICIT Average age at diagnosis 65 Most common type Amyotrophic Lateral Sclerosis (ALS)

Answer 18

Clinical diagnosis of exclusion - exclude peripheral neuropathy, myopathies EMG may show active denervation MDT approach - PT/OT/SLT/nutrition Riluzole only disease modifying therapy - slows progression by 3 months NIV - can prolong life by 7 months Advanced care planning and palliative team involvement

Answer 19

= rare, X-linked slowly progressive neuromuscular disorder Trinucleotide CAG repeat expansions in androgen receptor (AR) gene Typically an adult-onset disease (20 - 50s) Limb and facial weakness Fasciculations of the tongue / face (perioral) Decreased or absent deep tendon reflexes Dysphagia, Dysarthria Androgen insensitivity --> gynaecomastia, erectile dysfunction, reduced fertility, testicular atrophy MANAGEMENT Symptomatic and supportive Life expectancy is normal - ~10% die from swallowing complications d2 bulbar weakness.

Answer 20

Caused by the poliovirus Mainly affects children under 5 years of age. 1 in 200 infections leads to irreversible paralysis. Flaccid paralysis - usually of one limb, with wasting Typically no sensory features

Answer 21

Motor Nerves = Myelinated Sensory Nerves = Mixture of myelinated and unmyelinated Autonomic nerves = Unmyelinated

Answer 22

DEMYELINATING LOSS - Mainly motor fibres first - NCS = reduced velocity AXONAL LOSS - Sensory loss first - Affects longest nerve fibres first (feet before hands) - NCS = reduced Amplitude

Answer 23

A - Alcohol B - B12 or B1 deficiency C - CKD, CTD, Cancer (e.g. myeloma), Chemotherapy agents (e.g. vincristine, cisplatin) D - Diabetes (NUMBER 1 CAUSE) - Drugs (amiodarone, isoniazid etc) E - Endocrine causes (Hypothyroid) F - Folate deficiency G - Granulomatous disease e.g. sarcoidosis H - Hereditary e.g. HSMN I - Infection (HIV, leprosy)

Answer 24

- Acute inflammatory demyelinating polyradiculopathy (GBS) - Chronic IDP - Porphyria - Heavy Metal toxins e.g Lead - HSMN - Charcot-Marie-Tooth disease - Diabetes (diabetic amyotrophy)

Answer 25

Urine dip - glucose, protein BM Bloods - Routine FBC, U&Es, LFTs - B12 and folate - HbA1C, fasting glucose - TFTs Nerve Conduction Studies and EMG - reduced Amplitude = Axonal EXTRA TESTS (if initial N) - Electrophoresis & Immunoglobulins - Vasculitis screen - HIV/syphilis serology - LP - Genetic testing ?HSMN

Answer 26

Encompasses Charcot-Marie-Tooth disease HSMN Type 1 - Autosomal Dominant - Includes CMT 1 = defect in PMP-22 gene (myelin) - Symptoms start in puberty - MOTOR features predominate e.g. distal muscle wasting (inverted champagne bottle legs, clawed hands), pes cavus, hammer toes - Areflexia common - Sensory loss also common (can have sensory ataxia with positive Rhombergs) - Bilateral foot drop with high-stepping gait. Demyelinating = reduced velocity on NCS Rx = Supportive; MDT approach with PT/OT/orthotics/orthopaedics; Analgesia

Answer 27

Charcot Marie Tooth disease Spina bifida Polio Friedreich’s ataxia Syringomyelia Cerebral palsy Muscular dystrophy

Answer 28

Trivial trauma to peripheral nerves = mononeuropathy Auto Dominant (PMP 22 gene on chromo 17) 20-30 year olds Reduced velocity on NCS (demyelinating) Rx = supportive (splints, padding, orthoses)

Answer 29

= acute inflammatory demyelinating polyneuropathy (AIDP) Onset over days- weeks. Often preceeded by resp infection or diarrhoeal illness (e.g. campylobacter) FEATURES Ascending flaccid limb weakness Distal paraesthesiae - sensory deficits usually mild/patchy Areflexia. Can also affect autonomic system = tachycardiac, labile BP, arrhythmias, bladder/bowel issues Can also affect cranial nerves: ptosis, opthalmoplegia, facial nerve palsy, bulbar weakness Becomes chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if doesn’t cease by 6 weeks.

Answer 30

= form of acute inflammatory demyelinating polyneuropathy (AIDP) Miller-fischer syndrome is a proximal variant of GBS causing ataxia, opthalmoplegia, areflexia Anti-GQ1b antibodies!

Answer 31

Spirometry Bedside ECG (risk of arrhythmias) Bloods - Routine (FBC, renal function, liver function, inflam markers) - Antibodies to gangliosides (anti-GM1) Lumbar Puncture - Raised CSF protein, normal WCC Management = mainly supportive, MDT approach - Refer to ITU if vital capacity <1.3L/bulbar dysfunction. May need tracheostomy - Give IVIG +/- methylprednisolone, or plasma exchange (liaise with neurology) - SLT & nutrition team esp if bulbar involvement

Answer 32

Ulnar nerve root = C8-T1 Elbow key site for injury/compression Wasting of dorsal interossei and hypothenar eminance Clawing of 4th and 5th fingers MOTOR = - Small muscles of hand (excluding LOAF) e.g. palmar and dorsal interossei (PAD/DAB) = palmar ADduct, dorsal ABduct - Flexor carpi ulnaris - Adductor pollicis SENSORY = - Dorsal and palmar surface of hand over 5th and half of 4th finger FROMENT'S sign = weak adductor pollicis brevis

Answer 33

Median nerve root = C6-T1 Wrist (carpal tunnel) key site for injury/compression Wasting of thenar eminence MOTOR = - LOAF muscles of hands = mainly affects thumb movement - Weak thumb abduction, flexion and opposition - Wrist and finger flexion SENSORY = -Palmar surface of hand from thumb to half of 4th digit - Dorsal surface of digits 1-4 (not on back of hand) PHALEN'S test = numbness on upside down prayer sign TINEL's test = numbness whilst tapping over flexor retinaculum

Answer 34

= median nerve compression between flexor retinaculum and carpal bones Idiopathic Work-related repetitive strain MSK - RA - Gouty tophi - OA - Wrist fractures Endocrine - Pregnancy - Acromegaly - DM - Hypothyroidism CKD Myeloma Amyloidosis Vasculitides

Answer 35

Radial nerve root = C5-T1 Site for injury/compression can occur in axillary, humeral and elbow regions Wrist drop! Finger drop! MOTOR = - Extensors of forearm, wrist and fingers & triceps brachii - Weak wrist and elbow extension - Unable to straighten fingers - Weakness of finger adduction and abduction (can be overcome if hand placed on flat surface) SENSORY = - Palmar base of thumb, dorsal surface of radial side of hand (does not innervate any finger sensation)

Answer 36

Charcot-Marie-Tooth Disease Common Peroneal Nerve palsy MND L4/L5 root lesion

Answer 37

*Think anterior horn cell—nerve root—plexus—peripheral nerve—NMJ—muscle* Muscle (weak anterior tibialis): any cause of myopathy Peripheral Nerve: ** Common peroneal nerve palsy (mono/polyneuropathy) eg. trauma to fibular head, surgery on leg, compression of fibula neck, mononeuritis multiplex of any cause, Charcot Marie Tooth disease ** Sciatic nerve palsy eg. trauma Plexus: Lumbosacral plexopathy Nerve Root: L5 root lesion eg. prolapsed disc Anterior horn cell eg. MND (NB: no sensory deficit)

Answer 38

FEATURES Weak ankle dorsiflexion INversion is INtact, EVersion "EVicted" Ankle reflex intact If Deep branch only: preserved eversion and sensory loss only in webspace between 1st and 2nd toes and not lateral lower leg or foot dorsum

Answer 39

L4 - S3 FEATURES Weak knee flexion Weak plantarflexion and dorsiflexion Weak eversion and inversion Widespread lower limb sensory loss

Answer 40

L5 lesion: Cannot straight leg raise Weak inversion and eversion Weak dorsiflexion Reduced sensation on sole of foot as well as anterolateral shin and foot dorsum Ankle jerk preserved

Answer 41

Diabetes! (Top cause) (CALVS) C - Cancer A - Amyloidosis L - Lyme disease/Leprosy V - vasculitides e.g. EPGA, GW, RhA, SLE S - Sarcoidosis

Answer 42

1) "Glove and Stocking" Polyneuropathy - Distal, symmetrical - Progressive (distal to proximal) - Axonal loss 2) Autonomic Neuropathy - Postural hypotension - Gastroparesis - Gustatory sweating 3) Diabetic Amyotrophy - Microvasculitis - Asymmetrical, proximal weakness - Hair loss, poor vascular supply

Answer 43

Autoimmune disease of Acetylcholine receptors at NMJ (IgG antibodies to AChR in 85%) Associated with thymomas (15%) and other AI conditions e.g. pernicious anaemia Muscle fatiguability! Proximal muscle weakness Extraocular muscle weakness --> diplopia, ptosis Reflexes normal but reduce with repeated testing 3 Forms - Ocular (85%) - Oropharyngeal - Generalised

Answer 44

Spirometry (FVC) Bloods - Routine, including TFTs - CK (exclude myopathy) - Anti AChR antibodies Single fibre EMG (jitter) CT thorax (exclude thymoma) {Sussman Guidelines}

Answer 45

MDT Approach - neuro, PT, OT, SLT, respiratory team Assess respiratory function Avoid precipitating medications Pyridostigmine (inhibits anticholinesterase) Immunosuppression e.g. steroids (risk of paradoxical reaction in first 2 weeks = inpatient), AZA/MTX ?Thymectomy

Answer 46

Exacerbation requiring mechanical ventilation (FVC < 1.5L) Rx = mainly supportive, consider early referral to ICU for ventilatory support - Remove trigger - IVIg or Plasma exchange

Answer 47

Gentamicin Macrolides Tetracyclines Quinolones Procainamide Beta blockers Lithium Phenytoin

Answer 48

MND Subacute combined degeneration of the cord (vit B12) Friedrich's Ataxia Syphilis

Answer 49

Most likely = dual pathology e.g. cervical myelopathy (UMN) and peripheral neuropathy (LMN) MND (nil sensory signs) Subacute Combined degeneration of the Cord (B12) Syringomyelia = cyst in central canal of spinal cord = UMN signs initially - As expands, starts to damage Anterior Horn Cells = LMN signs

Answer 50

Vitamin B12 deficiency results in damage to: 1) Doral columns (UMN) - Symmetrical distal sensory neuropathy (legs > arms) - Loss of light touch, proprio and vibration 2) Lateral corticospinal tract (UMN) - Muscle weakness and spasycity - Hyper-reflexia - Upgoing plantars 3) Spinocerebellar tract (UMN) - Sensory ataxia 4) Peripheral nerves (LMN) - Glove and stocking distribution - Absent ankle reflexes Rx = B12 supplementation (neuropathy recovers better than myelopathy) Investigate cause - ?malnutrition ?malabsorption ?pernicious anaemia ?Nitrous oxide abuse

Answer 51

Syringomyelia = fluid filled cyst in central canal of spinal cord CAUSES/ASSOCIATIONS - Chiari malformations - Spinal cord tumour - Trauma - Idiopathic

Answer 52

Reduced pain and temp sensation in "cape-like distribution" Light touch/vibration/proprio preserved LMN signs of upper limbs = wasting and fasciculations UMN signs of lower limbs = brisk reflexes, upgoing plantars Bladder and bowel dysfunction

Answer 53

Autoimmune - Dermatomyositis, polymyositis, inclusion body myositis Connective Tissue Disease - SLE, Vasculitides, RA, systemic sclerosis Metabolic - Mitochondrial disease, glycogen storage disorders (e.g. McArdle's) Endocrine - Thyroid disorders, Cushing's, Addison's, Acromegaly, Diabetes Dystrophies - Myotonic Dystrophy, Duchenne's, Becker's , FSHD Drugs - Alcohol, heroin, statins, steroids, amiodarone

Answer 54

Urine dip & PCR (dip for blood could be myoglobin) Bloods - Routine - CK, AST, ALT, LDH - TFTs, HbA1c, cortisol - Rheum screen including ACE - Immunoglobulins - Myositis antibodies NCS and EMG ?MRI muscle Muscle biopsy Systemic screen = ECG, PFTs, CXR, echocardiogram Malignancy screen (if dermato/polymyositis) = FOB, mammogram, tumour markers, CT, endoscopy

Answer 55

Inherited degenerative condition of muscle X-linked, mutations on dystrophin gene on X chromosome Typically occurs in childhood (can have later onset in Becker's) Progressive proximal muscle weakness Calf pseudohypertrophy Risk of cardiomyopathy (less in Becker's) Raised CK, deranged transaminases Muscle biopsy Genetic testing

Answer 56

Inherited degenerative myopathy Autosomal dominant Slowly progressive disease of facial muscles and shoulder girdle FEATURES - Facial weakness --> difficulty closing eyes, asymmetric smile, can't whistle, dysarthria - Difficulty raising arms above head - Winging scapulae - Triceps more wasted than biceps (deltoid spared) - Waddling gait (pelvic girdle involvement) - Preserved IQ - Lung/cardiac involvement rare

Answer 57

Autosomal dominant (trinucl rep disorder) - >50 repeats = full penetrance Most common mutation = DMPK gene on chromosome 19 Genetic anticipation

Answer 58

Most common adult muscular dystrophy FEATURES - Myotonia = failure of relaxation of voluntary contraction - Frontal balding - Partial ptosis - Facial muscle wasting "Hatchet facies" - Distal muscle weakness - Dysarthria - Dysphagia Usually wheelchair dependent within 15-20 years of onset NORMAL CK (in comparison to Duchenne's & Beckers)

Answer 59

Diabetes mellitus Testicular atrophy Cardiomyopathy Conduction disorders Cholecystitis and biliary spasm Constipation Slow gastric emptying Hypersomnolence (?cause)

Answer 60

Neurofibromatosis & Tuberous Sclerosis Both Autosomal dominant!

Answer 61

Autosomal dominant (chromosome 17) Cafe au Lait spots (>6) Peripheral neurofibromas (>2) Iris hamartomas (>2) Axillary/groin freckles Scoliosis Phaeochromocytomas

Answer 62

Autosomal dominant (Chromosome 22) Bilateral acoustic neuromas/vestibular schwannomas Multiple intracranial tumours e.g. schwannomas, meningiomas

Answer 63

Majority autosomal dominant (TsC2 gene on chromosome 16 = most severe form) CUTANEOUS FEATURES Ash-leaf spots (fluoresce under UV) Shagreen patches (rough patches over spine) Angiofibromas (butterfly over nose) Subungual fibromata NEURO FEATURES Developmental delay Lower IQ Epilepsy ASSOCIATED FEATURES Retinal hamartomas Cardiac rhabdomyomas Polycystic kidney disease Lung cysts

Answer 64

V - Stroke, ICH I - Multisystem Atrophy C T - Traumatic brain injury, Alcohol, Carbamazepine, phenytoin A - MS, Miller-Fisher Syndrome M - B12 deficiency, Copper deficiency I - Spinocerebellar Ataxia, Friedrich's Ataxia, Ataxia Telangiectasia, VHL N - Cerebellar space occupying lesion, paraneoplastic (small cell lung cancer, breast, gynae, testicular)

Answer 65

D - Dysdiadochokinesis A - Ataxic (gait, limb or truncal) N - Nystagmus (gaze-evoked) I - Intention tremor S - Slurred or staccato speech (dysarthria) H - Hypotonia Also - Rebound phenomenon!

Answer 66

Spinocerebellar Ataxias - group of conditions, autosomal dominant trinucleotide repeats Ataxic telangiectasia - autosomal recessive Friedrich's Ataxia - autosomal recessive

Answer 67

Autosomal recessive disorder (trinucleotide repeat, FRATAXIN gene on chromosome 9) Most common hereditary ataxia in UK Combination of spinocerebellar signs, corticospinal tract signs and dorsal column loss FEATURES Onset in adolescence Gait ataxia Kyphoscoliosis Mix of UMN and LMN - absent ankle reflexes, upgoing plantars Spasticity Loss of proprioceptive and vibration sensation ASSOCIATED FEATURES HOCM (90%, common cause of death) Diabetes High arched palate High arched feet Preserved IQ {Vitamin E deficiency = rare mimic!}

Answer 68

Autosomal recessive disorder (ATM gene) Inherited Combined Immunodeficiency disorder - 10% risk of development malignancy (esp Leukaemias, lymphoma) FEATURES Typically presents in childhood as abnormal movements Cerebellar ataxia Skin & eye telangiectasia Recurrent LRTIs (due to IgA deficiency) Dystonia Chorea

Answer 69

Group of autosomal dominant disorders - majority trinucleotide repeats FEATURES Usually develops in 30-40s Progressive cerebellar signs UMN and extrapyramidal signs Peripheral neuropathy Ophthalmoplegia

Neurology Flashcards

(93 cards)