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Neurology Flashcards

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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
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MS - Dissemination in Time
Differing neurological attacks of > 24 hours duration or multiple plaques of differing intensities on MRI scan OR oligoclonal bands
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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
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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
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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
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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
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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
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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
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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
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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
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Risk Factors for MND
Low BMI Healthy, Fit FHx (SOCD1, C9ORF 72)
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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
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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
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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
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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!
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Treatment Alzheimers Disease
Donepezil - Ach Esterase inhibitor - Increases Ach in synaptic cleft Memantine - Glutaminergic NMDA receptor antagonist
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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
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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
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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
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Myotonic dystrophy complications
Diabetes Gynacomastia Testicular Atrophy Cognitive impairment Cardiomyopathy Hatchet face Frontal balding Facial muscle atrophy Hypothyroidism Increased cancer risk IBS
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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
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Levetiracetam
"Keppra" - Blocks SV2A-mediated pre-synaptic vesicle release of neurotransmitter - Causes mood changes including aggression & Suicidal ideations
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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
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Rx generalised epilepsy
Keppra Na Valproate
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Rx focal seizures
- Carbemazepine - Lamotrigine
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Rx epilepsy in pregnancy
Lamotrigine
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Na Valproate
- Inhibits GABA transaminase - Used for focal seizures in non-childbearing age women - Causes weight gain - Causes hyperammoneic encephalopathy - Teratogenic
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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)
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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
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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
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Na Valproate and Lamotrigine
Na Valproate decreases clearance of Lamotrogine - therefore combination is good in refractory epilepsy
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Lamotrigine
Side effects - SJS - Ataxia - Can aggravate myoclonic jerks of infancy - Concentration increased by Na Valproate & decreased by OCP - Really excreted
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Rx absence seizures
Ethosuxamide or Lamotrigine
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Rx Juvenile myoclonic Epilepsy
Na Valproate, or Lamotrigine if child bearing age
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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
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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
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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
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Thrombectomy indications
ICA or M1 infarct within 6 hours
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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)
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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
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