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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
MS lesions; Dissemination in Space
Spinal Infra tentorial Periventricular Juxtacortical
25
MS - Dissemination in Time
Differing neurological attacks of > 24 hours duration or multiple plaques of differing intensities on MRI scan OR oligoclonal bands
26
Expanded Disability Status Scale
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
27
MS drugs
"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
28
Side effects/benefits of MS drugs
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
29
Anti- MOG
Anti- myelin oligodendrocyte glycoprotein - Acute disseminated encephalopathy in children - Neuromyelitis optica in adults (Mimics transverse myelitis and MS)
30
Type 1 Charcot Marie Tooth
- Hereditary sensory and motor neuropathy - Hypertrophy of myelin producing "onion rings" on biopsy - Demyelinating - Slow Nerve conduction - Onset in Childhood
31
Type 2 Charcot Marie Tooth
- 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
Hereditary neuropathies with pressure palsies
- 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
Anti-Hu
Paraneoplastic encephalitis associated with small cell lung cancer
34
Lambert Eaton Syndrome
- 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
Anti-NMDA Encephalitis
- 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
Huntingtons
- 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
Types of inclusions in Neurological conditions
Tau - PSP - Corticobasal degeneration - Picks disease/frontotemporal dementia Synuclein - Parkinsons Disease - Lewy body dementia - Multisystem Atrophy Amyloid - Alzheimers - Vascular Dementia Prion - CJD
38
Creutzfeldt-Jakob disease (CJD)
CJD sporadic CJD Variant - Acquired "Mad Cow Disease" Progressive dementia, neuropsychiatric changes, mutism, ataxia with death within 1 year
39
Motor Neurone Disease
Motor neurone denervation due to glutamate excitotoxicity spreads UMN -> LMN - SOCD1 - C9ORF 72 -> associated with coexisting frontotemporal dementia
40
Risk Factors for MND
Low BMI Healthy, Fit FHx (SOCD1, C9ORF 72)
41
Split Hand Syndrome
Associated with MND Wasting at First dorsal interosseous (flexor digitorium mini inervated by ulnar N) & Adductor policies brevis inervated by Median N
42
Types of MND
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
Management MND
- 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
Genetic predisposition Alzheimers Disease
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
Treatment Alzheimers Disease
Donepezil - Ach Esterase inhibitor - Increases Ach in synaptic cleft Memantine - Glutaminergic NMDA receptor antagonist
46
Types Muscular Dystrophy
- 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
Type 1 Myotonic Dystrophy
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
Type 2 Myotonic Dystrophy
Autosomal dominant CCTG repeat on CNBP gene (Previously ZnF9) Onset over 10 years of age Features - Milder disease - Diabetes - Proximal muscle weakness
49
Myotonic dystrophy complications
Diabetes Gynacomastia Testicular Atrophy Cognitive impairment Cardiomyopathy Hatchet face Frontal balding Facial muscle atrophy Hypothyroidism Increased cancer risk IBS
50
Types of Epilepsy
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
Levetiracetam
"Keppra" - Blocks SV2A-mediated pre-synaptic vesicle release of neurotransmitter - Causes mood changes including aggression & Suicidal ideations
52
Na channel blockers in treatment of epilepsy
- 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
Rx generalised epilepsy
Keppra Na Valproate
54
Rx focal seizures
- Carbemazepine - Lamotrigine
55
Rx epilepsy in pregnancy
Lamotrigine
56
Na Valproate
- Inhibits GABA transaminase - Used for focal seizures in non-childbearing age women - Causes weight gain - Causes hyperammoneic encephalopathy - Teratogenic
57
Drugs metabolised by CYP3A4
- 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
Inhibitors CYP3A4
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
Inducers of CYP3A4
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
Na Valproate and Lamotrigine
Na Valproate decreases clearance of Lamotrogine - therefore combination is good in refractory epilepsy
61
Lamotrigine
Side effects - SJS - Ataxia - Can aggravate myoclonic jerks of infancy - Concentration increased by Na Valproate & decreased by OCP - Really excreted
62
Rx absence seizures
Ethosuxamide or Lamotrigine
63
Rx Juvenile myoclonic Epilepsy
Na Valproate, or Lamotrigine if child bearing age
64
Third Nerve Palsy
"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
Aphasia
Brocas - Expressive, non-fluent, comprehends Wernickes (Brodman area 22) - Receptive, fluent, does not comprehend Connected by arcuate fasciculus
66
PICA/Lateral medullary/Wellenbergs syndrome
- Ipsilateral facial pain and temp loss - Contralateral body pain and temp loss - Vertigo, Ataxia, ipsilateral nystagmus - Ipsilateral Horners - Dysphagia & ipsilateral loss of taste
67
Brainstem anatomy
Midbrain - 2 3, 4 Pons - 5, 6, 7, 8 Medulla - 9, 10, 11, 12
68
Contraindication to thrombolysis in acute stroke
- 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
Thrombectomy indications
ICA or M1 infarct within 6 hours
70
Acute haemorrhagic stroke management
- 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
Genetic predisposition MS
HLA DR B1 501 (3 x risk)
72
Parkinsons Medications
- 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
Anti-synthetase syndrome
- Raynauds - ILD - Dermatomyositis - Non-erosive arthritis - Mechanics hands - Anti-Jo - Anti-PL-12 Associated with malignancy like all dermatomyositis and polymyositis
74
Immune mediated necrotising myopathy
- 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
Inclusion body myositis
- 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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