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RACP > Neurology > Flashcards

Neurology Flashcards

1
Q

Gerstmanns Syndrome

A

Stroke at the angular gyrus of the dominant parietal lobe
Alcalculia
Finger Agnosia - cannot name fingers
Agraphia - cannot write, but can copy (Alexia)
L)/R) dissassociation

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2
Q

Non-dominant parietal lobe infarct

A

Inattention
Neglect
Apraxia
Impaired spacial perception

PCA infarct

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3
Q

Non- Dominant Parietal infarct

A

Inattention
Neglect
Apraxia
Spatial unawareness

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4
Q

Foot Drop

A

Due to Peroneal Nerve injury (branch of Sciatic nerve)
- Common peroneal - Dorsiflexion and eversion
- Deep peroneal - Just dorsiflexion

(The other branch of the sciatic nerve is the Tibial Nerve which supplies plantarflexion)

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5
Q

Internuclear Opthalmoplegia

A

Interruption Medial longitudinal fasiculus which carries information about the direction that the eyes should move in midbrain

  • Leading eye nystagmus
  • Failure of adduction of other eye
  • Normal convergence
  • Ipsilateral lesion to adduction deficit
  • Bilateral is most suggestive of MS
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6
Q

Anton Syndrome

A

Lesion is in Bilateral occipital lobes supplied by Posterior cerebral artery – Presents with bilateral visual loss and unawareness or denial of blindness.

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7
Q

S1 radiculopathy

A
  • Inability to plantar flex (walk on toes) “S1/S2 I stand on my shoe” via Tibial Nerve (branch sciatic nerve)
  • Loss of sensation plantar aspect of lateral aspect foot
  • Loss of ankle jerk reflex
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8
Q

Diagnosis GBS

A

Nerve Conductive Study and EMG - but may not be diagnostic in first week
Elevated CSF protein level (Albumino-cytological dissociation)
Mild increase in CSF WCC
Antiganglioside GM1 antibodies
GQ1b antibodies in Miller Fisher Syndrome

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9
Q

MRI findings PSP

A

Midbrain atrophy on MRI presenting as “Mickey Mouse “ and “Hummingbird” Signs

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10
Q

Progressive Supranuclear Palsy

A

Vertical gaze ophthalmoplegia
Early Falls with Axial rigidity
Facial dystonia
Asymmetrical Parkinsons
Poor response to L-Dopa

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11
Q

Lewy body Dementia

A

Early Dementia
Parkinsons
Dream-like behaviour with visual hallucinations

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12
Q

Corticobasal degeneration

A

Parkinsons Plus syndrome
Asymmetrical Parkinsons
Fronto-temporal dementia
Alien Limb Phenomenan
Apraxia

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13
Q

Multi-systems atrophy

A

Parkinsons Plus
Severe Autonomic dysfunction - Urinary retention, Postural hypotension
Cerebellar dysfunction
Pathological laughter and crying
Less likely to have hallucinations and cognitive dysfunction
Hot Cross Bun sign on MRI - increased T2 pons

mulTi-sysTem aTrophy = T similar to Hot Cross Bun sign

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14
Q

Shy- Drager Syndrome

A

Variant of Multi-system Atrophy (Parkinson’s Plus syndrome) - with severe autonomic dysfunction

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14
Q

Hemiballismus

A

Rare movement disorder characterised by a large movement of an entire limb on one side of the body.
Acute development of hemiballismus caused by focal lesions in the contralateral basal ganglia and subthalamic nucleus

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15
Q

HINTS exam

A

Head Impulse test
- positive; correctional saccade in peripheral, negative in central
Nystagmus
- Bidirectional in Central
- Towards the pathological side in Peripheral
Test of vertical skew

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16
Q

Meineres Disease

A
  • Vertigo
  • Head Impulse - Saccade positive
  • Unidirectional Nystagmus
  • Aural Fullness
  • Tinnitus
  • Sensori-neural hearing loss
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17
Q

Acute stroke MRI

A

DWI - hyperintense region indicating poor perfusion
ADC - Hypodense region indicating poor free water flow

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18
Q

Ab in Myasthenia Gravis

A

Attack post-synaptic ACh receptors
Anti-AchR (90%, or only 50% in ocular MG), cause increase internalisation & degradation of receptors
Anti -MUSK (10%), cause clustering of AchR on endplate, more frequently respond to PLEX > IVIG

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19
Q

Management for MG

A

Pyrodistigmine - for symptom relief only - not for crisis!
Thymectomy - even if no pathology but refractory to treatment
IVIG
PLEX - better response with MUSK +
Steroids - cautiously as can cause crisis
Immunosuppression - AZA, Mycophenolate, Rituximab

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20
Q

Acute Disseminated encephalomyelitis (ADEM)

A
  • Post- Vaccination or viral illness
  • Anti-MOG myelin oligodendrocyte glycoprotein)
  • Rapidly progressive neurological decline in young patient who is apyrexial
  • Clinical evidence of demyelination with PNS and CNS signs
  • Mimics MS or transverse myelitis on imaging
  • Rx with steroids
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21
Q

Syndromes/Signs associated with MS

A
  • Optic Neuritis
  • Intranuclear Ophthalmoplegia
  • C-spine dermatome pruritus
  • MS hug - neuropathic pain at torso
  • Trigeminal Neuralgia
  • Dyschromatopsia - Impaired colour vision (red becomes dark and bleached)
  • Transverse myelitis - inflammation of spinal cord mimicking Cauda equina
  • Cerebellar ataxia - with cerebellar lesions
  • Lhermittes sign; Lhermittes sign; intense burst of pain like an electric shock that runs down back on flexion of neck “barber chair sign”; indicates dorsal column demyelination in c-spine
  • Worsening of neurological signs with increased body tempretures
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22
Q

Lhermittes sign

A

Intense burst of pain like an electric shock that runs down back on flexion of neck “barber chair sign”; indicates dorsal column demyelination in c-spine

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23
Q

McDonald Criteria

A

For MS - Dissemination in space AND time

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24
Q

MS lesions; Dissemination in Space

A

Spinal
Infra tentorial
Periventricular
Juxtacortical

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25
Q

MS - Dissemination in Time

A

Differing neurological attacks of > 24 hours duration or multiple plaques of differing intensities on MRI scan

OR oligoclonal bands

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26
Q

Expanded Disability Status Scale

A

Used for disability in MS;
0; normal
1; minimal signs, no disability
2; minimal disability
3; moderate disability
4; severe disability
5; disability affecting ADLs
6; Assistance required to walk
7; Wheelchair bound
8; Bed bound
9; Unable to communicate/eat
10; Death

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27
Q

MS drugs

A

“DONT ForGet Preload In ACS”

Maintenance;

Dimethyl Fumurate - mechanism not well understood
Ocrelizumab - Anti-CD20
Natalizumab - Anti- alpha 4 integrin
Teriflunomide - Pyrimidine synthesis inhibition
Fingolimide - S1P inhibition to inhibit lymphocyte migration from LN
Glatiramer - Shunts Th1 to Th2
Peg IFN
Interferon Beta

Immune reconstitution;

Alemtuzumab - Anti-CD52
Cladrabine - Inhibits purine synthesis
Stem Cell Transplant

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28
Q

Side effects/benefits of MS drugs

A

DONT ForGet Preload In ACS

Dimethyl Fumurate - Best in pregnancy
Ocrelizumab - works for progressive MS
Natalizumab - JC virus/PML
Teriflunomide - teratogenic, alopecia
Fingolomide - Heart block, Macular oedema, CI CVD
Glatiramer - panic attacks & flushing, safe in pregnancy
Peg IFN & IFN Beta - Hepatitis, Flu-like illness, Depression
Alemtuzumab - Autoimmune conditions, thyroiditis, haemolytic anaemia, Anti-GBN GN, ITP, Breast Ca, Melanoma
Cladribine - Increased Cancer risk, PML

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29
Q

Anti- MOG

A

Anti- myelin oligodendrocyte glycoprotein
- Acute disseminated encephalopathy in children
- Neuromyelitis optica in adults

(Mimics transverse myelitis and MS)

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30
Q

Type 1 Charcot Marie Tooth

A
  • Hereditary sensory and motor neuropathy
  • Hypertrophy of myelin producing “onion rings” on biopsy
  • Demyelinating
  • Slow Nerve conduction
  • Onset in Childhood
31
Q

Type 2 Charcot Marie Tooth

A
  • Hereditary Motor and Sensory neuropathy
  • Non-demylinating
  • Caused by direct damage to nerve axons
  • Does not affect nerve conduction velocity
  • Weakness of foot and leg predominate
  • Less prominent sensory changes/deformity
  • Onset adolescence
32
Q

Hereditary neuropathies with pressure palsies

A
  • Mutation in PMP22 gene causing insufficient maintenance of myelin sheath. Most commonly on chromosome 17.
  • Persistant symptoms when pressure applied to nerves i.e. perineal, ulnar nerve palsies
33
Q

Anti-Hu

A

Paraneoplastic encephalitis associated with small cell lung cancer

34
Q

Lambert Eaton Syndrome

A
  • Antibodies against pre-synaptic VGCC
  • Improves with exertion
  • Associated with SCLC, Males, 50-60 years
  • Weakness, Areflexia & Autonomic dysfunction
  • NCS with low CMAP which increases with exercise
  • Rx;
  • 3-4 diaminopyradine (blocks K+ depolarising channels to increase Calcium channel opening)
  • IVIG
  • PLEX
35
Q

Anti-NMDA Encephalitis

A
  • Young Female
  • Ovarian teratoma, Sex Cord stromal tumours, SCLC
  • Extrema delta brush delay on NCS
  • MRI showing mesial-temporal hyperintensities
  • CSF; Increased protein, lymphocytic pleocytosis, Anti-NMDA ab, Oligoclonal bands
36
Q

Huntingtons

A
  • Triplet repeat disorder - CAG repeats > 27 - full penetrance > 40 repeats - causes mutant Huntington gene
  • Affects Basal ganglia
  • Caudate atrophy on MRI
    Rx with Tetrabenzine
37
Q

Types of inclusions in Neurological conditions

A

Tau
- PSP
- Corticobasal degeneration
- Picks disease/frontotemporal dementia

Synuclein
- Parkinsons Disease
- Lewy body dementia
- Multisystem Atrophy

Amyloid
- Alzheimers
- Vascular Dementia

Prion
- CJD

38
Q

Creutzfeldt-Jakob disease (CJD)

A

CJD sporadic
CJD Variant - Acquired “Mad Cow Disease”
Progressive dementia, neuropsychiatric changes, mutism, ataxia with death within 1 year

39
Q

Motor Neurone Disease

A

Motor neurone denervation due to glutamate excitotoxicity spreads UMN -> LMN
- SOCD1
- C9ORF 72 -> associated with coexisting frontotemporal dementia

40
Q

Risk Factors for MND

A

Low BMI
Healthy, Fit
FHx (SOCD1, C9ORF 72)

41
Q

Split Hand Syndrome

A

Associated with MND
Wasting at First dorsal interosseous (flexor digitorium mini inervated by ulnar N) & Adductor policies brevis inervated by Median N

42
Q

Types of MND

A

Amyotrophic lateral Sclerosis (ALS) - UMN +LMN
ALS plus - UMN + LMN + executive dysfunction
ALS-FTD - UMN + LMN + Frontoremporal dementia
Primary lateral sclerosis - UMN only
Primary muscular atrophy - LMN (remember because LMN denervation causes muscle atrophy!)

PMA + mild sensory change = Kennedy syndrome

43
Q

Management MND

A
  • Riluzole - Na channel blocker to decrease Glutamate release and decrease glutamate excitotoxicity -> slows progression of disease
  • Endaravone - Free Radical Scavanger
  • NIV - increases survival and QOL if commenced before bulbar dysfunction - indications
  • SNIP < 30
  • Low MIP indicative of diaphragm
  • Low MEP indicative of abdominal muscles
44
Q

Genetic predisposition Alzheimers Disease

A

APO E4 - Late onset Alzheimer’s and cardiovascular disease

Presenilin 1 and 2, Amyloid Precurser Protein - Early onset Alzheimers disease

APO E2 - protective Gene!

45
Q

Treatment Alzheimers Disease

A

Donepezil - Ach Esterase inhibitor - Increases Ach in synaptic cleft

Memantine - Glutaminergic NMDA receptor antagonist

46
Q

Types Muscular Dystrophy

A
  • Duchenne - absence dystrophin protein, X linked, males affected, female carriers. Severe disease beginning in infancy
  • Beckers - Milder form, variable dystrophin protein phenotype, more cardiomyopathy
  • Fascioscapular - facial weakness, scapula winging
  • Oculopharyngeal - Ptosis and difficulty swallowing
  • Emery Drifiuss - weakness shoulder and upper limb
  • Myotonic Dystrophy - Autosomal dominant, Percussion myotonia, endocrine abnormalities, Cataracts, cardiomyopathy, cognitive impairment
47
Q

Type 1 Myotonic Dystrophy

A

Autosomal Dominant
3 x CTG repeats on DMPK gene
Onset < 10 years of age

Features
- Facial weakness (not prominent in Type 2)
- Distal muscle weakness (Type 2 is proximal)
- Cardiomyopathy common
- Grip Myotonia
- Dive Bomber on EMG

48
Q

Type 2 Myotonic Dystrophy

A

Autosomal dominant
CCTG repeat on CNBP gene (Previously ZnF9)
Onset over 10 years of age

Features
- Milder disease
- Diabetes
- Proximal muscle weakness

49
Q

Myotonic dystrophy complications

A

Diabetes
Gynacomastia
Testicular Atrophy
Cognitive impairment
Cardiomyopathy
Hatchet face
Frontal balding
Facial muscle atrophy
Hypothyroidism
Increased cancer risk
IBS

50
Q

Types of Epilepsy

A

Focal - most commonly due to mesial-temporal sclerosis at hippocampus
Generalised idiopathic epilepsy; Na valproate as first line treatent
Frontal Lobe epilepsy; Nocturnal motor symptoms

51
Q

Levetiracetam

A

“Keppra”
- Blocks SV2A-mediated pre-synaptic vesicle release of neurotransmitter
- Causes mood changes including aggression & Suicidal ideations

52
Q

Na channel blockers in treatment of epilepsy

A
  • Phenytoin
  • Topiramate
  • Lamotrigine
  • Carbamazepine

Others;
- Keppra; SV2A modulator
- Topiramate also works to potentiate GABA & as mild diuretic by inhibiting carbonic anhydrase
- Vigabatrin; Potentiates GABA
- Lamotrogine also inhibits NMDA glutamate receptor & blocks Ca channels

53
Q

Rx generalised epilepsy

A

Keppra
Na Valproate

54
Q

Rx focal seizures

A
  • Carbemazepine
  • Lamotrigine
55
Q

Rx epilepsy in pregnancy

A

Lamotrigine

56
Q

Na Valproate

A
  • Inhibits GABA transaminase
  • Used for focal seizures in non-childbearing age women
  • Causes weight gain
  • Causes hyperammoneic encephalopathy
  • Teratogenic
57
Q

Drugs metabolised by CYP3A4

A
  • Lamotrogine
  • Carbamazepine
  • Paracetamol (with 2E1)
  • Simvastatin and Atorvastatin (therefore cannot be used for Leukodystrophy associated with Protease inhibitors in HIV - must Rx w Pravastatin which is not a CYP 3A4 substrate)
58
Q

Inhibitors CYP3A4

A

Increased metabolism Carbamazepine

SICKFACES.COM

Sodium Valproate
Isonazid
Cimetidine (H2 receptor antagonist)
Ketaconazole
Fluconazole
Alcohol and Grapefruit juice
Chloramphenicol
Erythromycin
Sulphonamides
Ciprofloxacin
Omeprazole
Metronidazole

59
Q

Inducers of CYP3A4

A

Decreases concentration carbamezepine & Lamotrigine
Decreases efficiency of OCP

CRAPGPS

  • Carbemazepine (increases own metabolism!)
  • Rifampicin
  • Alcohol
  • Phenobarbitone
  • Griseofulvin (for ring worms)
  • Primidone, Phenytoin
  • St John’s wart
60
Q

Na Valproate and Lamotrigine

A

Na Valproate decreases clearance of Lamotrogine - therefore combination is good in refractory epilepsy

61
Q

Lamotrigine

A

Side effects
- SJS
- Ataxia
- Can aggravate myoclonic jerks of infancy
- Concentration increased by Na Valproate & decreased by OCP
- Really excreted

62
Q

Rx absence seizures

A

Ethosuxamide or Lamotrigine

63
Q

Rx Juvenile myoclonic Epilepsy

A

Na Valproate, or Lamotrigine if child bearing age

64
Q

Third Nerve Palsy

A

“Down and Out”, Ptosis, Miosis

Surgical - Pupil involved - commonly due to PCOM Aneurysm

Medical - Pupil spared - commonly due to diabetes of vasculitis such as GCA

Webers Syndrome - Ipsilateral 3rd Nerve palsy due to midbrain infarct at Occulomotor nucleus, along with contralateral hemiplegia due to disruption of corticospinal tracts (sometimes can have Parkinson’s due to Substantia nigra involvement) - often due to HTN

  • Uncal herniation
  • Cavernus sinus thrombosis
65
Q

Aphasia

A

Brocas - Expressive, non-fluent, comprehends
Wernickes (Brodman area 22) - Receptive, fluent, does not comprehend

Connected by arcuate fasciculus

66
Q

PICA/Lateral medullary/Wellenbergs syndrome

A
  • Ipsilateral facial pain and temp loss
  • Contralateral body pain and temp loss
  • Vertigo, Ataxia, ipsilateral nystagmus
  • Ipsilateral Horners
  • Dysphagia & ipsilateral loss of taste
67
Q

Brainstem anatomy

A

Midbrain - 2 3, 4
Pons - 5, 6, 7, 8
Medulla - 9, 10, 11, 12

68
Q

Contraindication to thrombolysis in acute stroke

A
  • Intracranial Haemorrhage
  • Extensive Hypodense region suggestive of subacute bleed
  • Active bleeding
  • BP > 180/100
  • Recent GU/GI Surgery
  • Recent trauma
  • Brain tumour
  • Aortic dissection
  • BSL < 2.7
  • Infective endocarditis
  • Pl < 100
  • INR > 1.2
  • DOAC use < 48 hours

Complicated by unilateral, contralateral, orolingual oedema -> bradykinin induced

69
Q

Thrombectomy indications

A

ICA or M1 infarct within 6 hours

70
Q

Acute haemorrhagic stroke management

A
  • BP target of 140mmHg
  • Reverse anticoagulation
  • Idarucizumab for Dabigatran
  • Factor Xa for Apixaban and Rivaroxaban
  • Vitamin K for warfarin
  • Prothrombin X (factors II, IX and X)
    AVOID platelet transfusion!
71
Q

Genetic predisposition MS

A

HLA DR B1 501

(3 x risk)

72
Q

Parkinsons Medications

A
  • Selegiline - MOA B inhibitors - decreases Dopamine degradation - serotonin syndrome
  • Amantadine - NMDA receptor antagonist to reduced glutamate excitotoxicity - Livedo reticularis, hallucinations
  • Benztropine - Ach R antagonist - cholinergic side effects

-Pramipexole, Cabergoline, Rotigotine Patch - Dopamine Agonists, moderate disease in young patients - Compulsive behaviours (most common with pramipexole)

  • L-Dopa with carbidopa - Dopamine replacement - for moderate disease in older patients - most severe motor fluctuations within 5 years - Punding, compulsive behaviours
  • Entacapone - COMT inhibitors to decrease degradation of dopamine - counteracts motor fluctuations and “off periods”
  • Apomorphine - Similar structure to Dopamine - decreases off-time - risk of subcutenous nodules, haemolytic anaemia, sleep attacks (cannot drive!)
  • Duodopa - continuous levodopa-carbidopa infusion - Increased risk melanoma, peritonitis, neuropathies. CI heart failure, acute angle glaucoma, arrhythmias, Hx stroke, Use of MOA, dementia
  • Deep brain stimulation @ sub thalamic nucleus and internal globes pallidus
73
Q

Anti-synthetase syndrome

A
  • Raynauds
  • ILD
  • Dermatomyositis
  • Non-erosive arthritis
  • Mechanics hands
  • Anti-Jo
  • Anti-PL-12

Associated with malignancy like all dermatomyositis and polymyositis

74
Q

Immune mediated necrotising myopathy

A
  • Isolated, severe proximal myopathy (i.e. no skin or other organ involvement)
  • Due to autoimmune attack on HMG-CoA reductase even in absence of statin use
  • Very high CK in ‘thousands’
  • Myonecrosis on biopsy
75
Q

Inclusion body myositis

A
  • Proximal and distal myopathy
  • Only mildly raised CK
  • Biopsy showing endomysial inclusion
  • Only inflammatory myopathy most common in Men - ALL others female predominant
  • EMG showing myopathic and neuropathic process
  • Treatment resistance - slow progression 5 - 15 years

RACP (17 decks)

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