Neurology Flashcards

Question

NMJ disorder

Answer 1

Repetitive stimualtion: decremental CMAPs: MG incremental CMAPs: LES or botulism

Answer 2

**Fibrillations:** represents a** single muscle fibre** that's lost it's innervation by the nerve, thus it's dying - could be relation damage in the anterior horn cells, radiculopathy, plexopathy - regular firing **- always bad ** - can be due to denervation or myositis - AXONAL neuropathies i.e. alcohol Fasciculations: due to entire motor unit. can visibly see it. A spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. - can be benign or anything that affects the peripheral nerve fibre - classically seen in damage to anterior horn neurons Motor unit potential (MUP): - neurogenic: less number of MUPs, but bigger due sprouting - myopathic: smaller MUPs, normal number i.e. DMD

Answer 3

Anti NT5c1a

Answer 4

- bulbar involvment - resp subtype - severity at baseline - speed of progression

Answer 5

flail arm or leg syndrome predominace in UMN or LMN

Answer 6

Age >40 Male Rapid onset Resp involvement Preceding diarrhoeal illness High Anti GM1 titre

Answer 7

occlusion of the **paramedian branches** of the PCA or basilar artery --> infarction of midbrain ipsilateral occulomotor nerve palsy contralateral hemiparesis or hemiplegia ## Footnote Eyes: down and out

Answer 8

AD inherited myopathy 20-30y/o It affects skeletal, cardiac and smooth muscle CTG repeat disorder DM1 - **distal **weakness DM2: prox. weakness Associated with: - cataracts - dysarthria - frontal balding - bilateral ptosis - myotonic facies - - DM - testricular atrophy - cardiac: HB, CM - dysphagia ## Footnote DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19 DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3

Answer 9

Pregnancy is protective against developing MS - more the better MS activity increases in the 3 months post partum Exclusive breastfeeding provides some reduction (30-50%) in MS disease activity postpartum

Answer 10

dominant occipital lobe

Answer 11

Ipsilateral: up and out eye due to inability to abduct, depress and internally rotate ## Footnote The trochlear nerve is purely a motor nerve and is the only cranial nerve to exit the brain dorsally. The trochlear nerve supplies one muscle: the superior oblique. The cell bodies that originate the fourth cranial nerve are located in ventral part of the brainstem in the trochlear nucleus.

Answer 12

antidromic second CMAP slight reduction in axonal neuropathy highly reduced in demyelination

Answer 13

Sodium valproate

Answer 14

Homonomous hemianopia is an example of retrochiasmal visual field defect

Answer 15

Reversible cerebral vasoconstriction syndrome: a weeks-long course of thunderclap headaches, sometimes focal neurologic signs, and occasionally seizures. A 'sausage on string' appearance is typical on CT angiogram.

Answer 16

Is associated with age of onset and duration of disease *(rather than duration of treatment)* Annual incidence is 10% Improved with increasing the frequency

Answer 17

damage to dorsal mid brain clinical manifestation: upward gaze palsy, convergence retraction nystagmus, bilateral lid retraction, Light-near dissociation ## Footnote Occurs due to damage to dorsal pons common cause: pineal gland tumour, haemorrhage or infarct

Answer 18

1. Asymmetric limb weakness 2. Bulbar sx

Answer 19

Single fibre electromyography

Answer 20

Anti ACh - R antibodies

Answer 21

Intra: C/T Extra: L/S Majjority of the spinal supply is by anterior spinal artery Central cord syndrome presents with dissociative sensory loss

Answer 22

Chiari I malformation (CM-I) is characterized by abnormally shaped cerebellar tonsils that are displaced below the level of the foramen magnum *Chiari II malformation (CM-II), also known as Arnold-Chiari malformation, is characterized by downward displacement of the cerebellar vermis and tonsils, a brainstem malformation with **beaked midbrain on neuroimaging**, and a **spinal myelomeningocele**.

Answer 23

C7 radiculopathy - wrist flexors are affected

Answer 24

Lamotrigine level is decreased in states of high estrogen i.e pregnancy, OCP

Answer 25

3 years this is irrespective of age ## Footnote Interestingly patients who develop Parkinsons at an older age are at a higher risk of mortality and developing dementia compared to younger patients Posterior visuospatial symptoms correlate with dementia and mortality

Answer 26

3 years this is irrespective of age ## Footnote Interestingly patients who develop Parkinsons at an older age are at a higher risk of mortality and developing dementia compared to younger patients Posterior visuospatial symptoms correlate with dementia and mortality

Answer 27

highly specific for IBM cf other myositis only found in about 35 % of IBM Other facts: Vacuoles are not present in 20% of IBM Anti NT5c1a can be seen in patients with SLE and sjogrens but WITHOUT muscle weakness. Anti NTC5c1a also has a prognostic role.

Answer 28

L1-2 involvement Sudden BILATERAL *less severe radicular pain* **More severe back pain** Mixture of both UMN and LMN findings Early faecal and urinary incontinence

Answer 29

Split hand syndrome is a neurological syndrome in which the hand muscles on the side of the thumb (lateral, thenar eminence) appear wasted, whereas the muscles on the side of the little finger (medial, hypothenar eminence) are spared. Anatomically, the **abductor pollicis brevis and first dorsal interosseous muscle are more wasted than the abductor digiti minimi.** It is a specific sign for anterior horn cell disease such as **ALS**

Answer 30

female caucasion affected sibling (worse in monozygotic twins) high altitude low vit D smoker obese ** HLA-DRB1*15:01 confers the greatest risk.**

Answer 31

Autoreactive T cells (esp Th17) migrate across BBB reacte to antigen via APC Secretion of proinflammatory cytokines Stimulation of astrocytes and microglia destruction of myelin --> exposed nerve results in axonal death

Answer 32

Grey matter lesions are common in progressive disease and correlates with cognitive impairment

Answer 33

VEP: visual evoke potential normal VEP - excludes optic nerve involvement

Answer 34

1. optic neuritis 2. acute myelitis 3. Area postrema syndrome: intractable hiccups, nasuea, or vomiting 4. Acute brainstem syndrome 5. Symptomatic cerebral syndrome with NMOSD typical brain lesions

Answer 35

1. optic neuritis 2. acute myelitis 3. Area postrema syndrome: intractable hiccups, nasuea, or vomiting 4. Acute brainstem syndrome 5. Symptomatic cerebral syndrome with NMOSD typical brain lesions

Answer 36

NMOSD: restricted to optic nerve and spinal cord Severe attack Longitudinal extensive central necrotic lesions Pleocytosis during attacks absent OCB More female cf MS Frequently have coexisting autoimmune conditions

Answer 37

IVMP then Oral CS +/- PLEX MMF, AZA Rituximab (off label) To note: MS treatment can be harmful: fingolimod, natalizumab, IF

Answer 38

* Most common presentation is with bilateral (consecutive) optic neuritis. * Also LETM and brainstem syndromes

Answer 39

* Most common presentation is with bilateral (consecutive) optic neuritis. * Also LETM and brainstem syndromes Typically very severe Steroid resposive --> good vision recovery Treat like NMOSD if relapses off steroids ## Footnote More recently identified in ~50% of seronegative NMOSD patients. * Also seen in CRION and MS patients.

Answer 40

frequent relapse in the first 2 yrs of dx short interval between the first 2 relapses rapid early disability progression high lesion load (**esp spinal or infratentorial)** Cerebral atrophy **MALE** **Later age of onset**

Answer 41

Tx for RRMS: **alpha4 integrin** (prevents bindinf to VAM1) * 68% reduction in relapse rates * 92% reduction in MRI activity * 42% reduction in disease progression. SE: <5% hypersensitivity * Increased risk of herpes virus reactivation (especially zoster) * Risk of progressive multifocal leukoencephalopathy (PML) caused by John Cunningham virus (JCV). *** If JCV serology positive, risk of PML related to: * Any prior immunosuppression (highest risk). * > 2 years on Natalizumab * JCV index (level of positivity)**.

Answer 42

Anti CD52 (found on all differentiated lymphocytes and monocytes) Highly effective in reducing relapses (~50% compared to IFNB-1a) and MRI activity SE: Infusion reactions common ***Increased infection risk** * Risk of **secondary autoimmunity** * Autoimmune **thyroid disease** (~30%) * Risk of **ITP, glomerular basement membrane disease,** autoimmune colitis etc * Requires monitoring with monthly blood tests for 5 years (Bloodwatch program). | Given IV for 5 days (yr 1) then IV for 3 days (yr 2).

Answer 43

Anti CD 20 for RRMS and** PPMS** Significant relative reduction in relapses (~50% cw IFNB-1a) * MRI activity reduction (94% cw IFNB1a) * Reduced disability progression. IN PPMS: 24% reduction in confirmed disability progression cw placebo SE: *Infusion reaction common. *Increased infection risk (beware **Hep B reactivation)**. * Estimated risk of PML (based on other anti-CD20+ therapies) ~1:30,000. *Non-significant increased risk of malignancies in one RCT (esp breast). | IV 6 mothly

Answer 44

Anti CD 20 for RRMS Fully humanised Reduced annualised relapse rate (0.11 vs 0.22) * Reduced disability progression (8.1% vs 12%) * Reduced new T2 lesions on MRI. * Reduced NFL levels. * NO change in brain volume. SE: 2.5% serious infection injection site reaction

Answer 45

For RRMS * Deoxyadenosine analogue prodrug that selectively targets lymphocytes due to preferential intracellular activation. ~50% relative relapse rate reduction versus placebo * Significant reduction in MRI activity and disability progression. * 48% of patients NEDA at 4 years after treatment initiation. Generally well tolerated: SE: Lymphopenia short term risk of infection esp. zoster | Weight based oral dosing for 5-days, week 1 and week 5. Repeated year 2.

Answer 46

ORAL, Binds to S-1-P receptors on lymphocytes blocking their egress from lymph node. * Additional putative effect directly on astrocytes. Relative relapse reduction of 54% cw placebo. * Reduced MRI activity * Slows disability progression * Reduced brain atrophy SE: *** First dose braydcardia.** * LFT derangements * Lymphopenia *Increased infection risk especially **herpes viruses.** *can casue disseminated HSV or VZV* ***Increased risk of non-melanomatous skin cancers.** * Annual skin checks * PML risk ~1:10,000 | Daily tab ## Footnote Ozanimod: different R specificity, and lower risk of cardiac complications

Answer 47

MS activation of Nrf2 in he antioxidant pathways SE: Flushin GI upset Lymphopenia rate LFT derangements Risk of PML 1:18,000 Least tolerated

Answer 48

Inhibits dihydroorotate dehydrogenase and interferes with pyrimidine synthesis. * Relative relapse risk reduction of 32% cw placebo * Reduced disability progression. * Reduced brain atrophy rates SE- hepatotoxicity, hair thinning, HTN, GT upset. Teratogenic! ## Footnote ORal tab

Answer 49

Synthetic polypeptide antigenically similar to myelin basic protein. * ~30% relative reduction in relapse rates cw placebo. SE: injection site reaction | used in **pregnancy and breast feeding**

Answer 50

Diverts immune system away from proinflammatory Th1 and Th17 pathways to a more anti-inflammatory Th2 pathway. * Reduces annualised relapse rate by ~30%. * Can delay conversion from CIS to RRMS. * SE- flu-like symptoms, depression, LFT derangements

Answer 51

for **SPMS** Like Fingolimod, selective sphingosine 1-phosphate rec modulator. Key differences include: * Narrower spectrum of action on **SIP receptors.** * Baseline pharmacogenomics **(CYP2C9 genotyping)** dictate starting dose. * **No cardiac monitoring** needed unless pre-existing cardiac condition SE: lymphopenia, increased LFT’s, (bradyarthymia), macular oedema, HTN, VZV reactivation, convulsions.

Answer 52

Safest: GA, IFN Likely safe- Natalizumab

Answer 53

Older MS pt male higher EDSS presence of comorbidities MS pt on b-cell depleting therapy: Ocre, ofan Recent methylpred ## Footnote * ATAGI guidelines stipulate all MS patients are eligible for at least 4 covid-19 vaccines. * MS patients who are on B-cell depleting therapies or SIP inhibitors are eligible for additional Covid-19 vaccinations, anti-viral treatments and Evusheld (tixagevimab and cilgavimab) due to blunted humoral responses.

Answer 54

Both genetic and environmental factors contribute to the risk of MS * 30 fold increase if Caucasian *** 3 fold increase if female** * 20 fold increase if affected sibling * Up to 7 fold increase for Both genetic and environmental factors contribute to the risk of MS * 30 fold increase if Caucasian *** 3 fold increase if female** * 20 fold increase if affected sibling * Up to 7 fold increase for **high latitude of residence** * 2 fold increase if low vitamin D levels * 1.5 fold increase if smoker * 1.3 fold increase if obese (females) * 2 fold increase if low vitamin D levels * 1.5 fold increase if smoker * 1.3 fold increase if obese (females) *** HLA-DRB1*15:01 confers the greatest risk. **

Answer 55

Both genetic and environmental factors contribute to the risk of MS * 30 fold increase if Caucasian *** 3 fold increase if female** * 20 fold increase if affected sibling * Up to 7 fold increase for Both genetic and environmental factors contribute to the risk of MS * 30 fold increase if Caucasian *** 3 fold increase if female** * 20 fold increase if affected sibling * Up to 7 fold increase for **high latitude of residence** * 2 fold increase if low vitamin D levels * 1.5 fold increase if smoker * 1.3 fold increase if obese (females) * 2 fold increase if low vitamin D levels * 1.5 fold increase if smoker * 1.3 fold increase if obese (females) *** HLA-DRB1*15:01 confers the greatest risk. **

Answer 56

sensory symptoms in limbs

Answer 57

in 21% of pt with MS, ON is the first presenting complaint f>M 30s Sx: **Monocular, central vision loss** **Pain with movement or touch** altered colour vision (red/green in late stage) reduced contrast sensitivity Phosphenes Uhthoff phenomenon Poor prognosis: - severe vision loss at presentation - Longer lesions, NMO IgG - African origin - Children ## Footnote - vision recovery over 6/12 - 12/12 -

Answer 58

- most common of the early-onset hereditary ataxias. - AR - GAA trinucleotide repeat on X25 gene on Chr 9 - DOES NOT SHOW ANTICIPATION The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features. Neurological features absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration Other features hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate

Answer 59

Ethosuximide

Answer 60

Lamotrigine; it's better than carbamazepine, topiramate and gabapentin for focal seizrues

Answer 61

lamotrigine level decreases

Answer 62

SVp2A inhibition --> reduce calcium current Doesn't interact with other AED 100% oral availability 95% renal excretion ADR: Fatigue, thrombocytopenia, blurred vision **memory, irritability, mood swings, increased suicidality ** ## Footnote Brivaracetam - 2nd gen - only diff is less dizziness

Answer 63

SVp2A inhibition --> reduce calcium current Doesn't interact with other AED 100% oral availability 95% renal excretion ADR: Fatigue, thrombocytopenia, blurred vision **memory, irritability, mood swings, increased suicidality ** ## Footnote Brivaracetam - 2nd gen - only diff is less dizziness

Answer 64

Tuberous sclerosis epilepsy

Answer 65

drowsiness, fatigue, lethargy, insomnia, dizziness, hyponatraemia, irritability, paraethesia (esp. phenytoin)

Answer 66

issue with attention, language and causes weight loss

Answer 67

CBZ and PHY --> SJS

Answer 68

CBZ induced rash/DRESS

Answer 69

inhibits sodium channel Lacosamide binds to collapsin response mediator protein-2 (CRMP-2)

Answer 70

glutamnergic AMPA antagonist ADR: weight gain, aggression, dizziness and ataxia

Answer 71

Lamotrigine - major congenital malformation is similar to baseline 2.9% Dose dependent defect with valproate and other AED Folate is not protective for AED induced teratogenicity ## Footnote Lamotrigine and Keppra are favourable

Answer 72

concentration reduced for. lamotrigine, keppra, lacosamide Topiramate reduced in 3rd tri and rebounds PP

Answer 73

PHY --> SJS or DRESS

Answer 74

Anti Potassium channel antibody - anti LGI1 (older male) Anti NMDA antibody (young woman, teratoma)

Answer 75

Clinical clues Limbic encephalitis Faciobrachial seizures (esp LGI1) Encephalopathy evolving rapidly, psychosis Onset with status

Answer 76

Spikes, sharp waves = epileptiform Low sensitivitty ~40% Odd #: left Even # : right Hepatic encephalopathy: triphasic waves

Answer 77

Spikes, sharp waves = epileptiform Low sensitivitty ~40% Odd #: left Even # : right Hepatic encephalopathy: triphasic waves

Answer 78

SUDEP: - Most common cause of death is central hypoventilation - RF: ○ Gender (male) ○ Uncontrolled generalised seizures (>2) Nocturnal seizures

Answer 79

typical onset is in the teenage years, more common in girls infrequent generalized seizures, often in **morning**//following sleep deprivation daytime absences **sudden, shock-like myoclonic seizure **(these may develop before seizures) often triggered by lack of sleep and flashing lights. Tx. Valproate or lamotrigine ## Footnote Seizures in most people with JME tend to improve after the fourth decade of life. Seizures are generally well controlled with medications in up to 90% of people

Answer 80

Sodium valproate

Answer 81

Vigabatrin ## Footnote inhibiting the GABA-degrading enzyme, GABA transaminase, resulting in a widespread increase in GABA concentrations in the brain.

Answer 82

Anti Mi Anti TIF-1gamma - associated with malignancy Clinical: Gottron's papules Heliotroph rash (upper eye lid) photosensitive rash - V sign and Shawl sign mechanics hands Histo: perifascicular muscle atrophy MRI-STIR: odema in patchy distribution

Answer 83

Antibodies again aminocyl transfer RNA synthatase Antibodies: **Anti-Jo1,** Anti PL12 Histo: perifascicular necrosis, perimysium fragmentation and increased ALP activity ## Footnote Clinical: Inflammatory myositis, ILD, arthritis, raynauds, mechanic hands, fever

Answer 84

Elevated CK in the 100s, median 4700 Histo: myofibre necrosis and regeneration without lymphocytic infiltrate Severe proximal muscle weakness Ab: Anti HMGCoA reductase Ab negative immune mediated is associated with malignancy Anti SRP: severe muscle involvement | Not all associated with statin

Answer 85

dx of exclusion subacute proximal muscle weakness, elevated CK, myopathic EMG biopsy: endomysial inflammation with CD8+ infiltrate

Answer 86

common myositis associated ab - non specific

Answer 87

Myositis/SSc overlap: associated with lung and oesophageal involvement

Answer 88

another Myositis/SSc overlap: associated with joint involvment, raynauds, ILD

Answer 89

Myosisis, Scleroderma, SLE and GN

Answer 90

1. Steroids 2. AZA/MTX/MMF/CYC/TAC/IVIG/Ritux/Cyclophosphamide | Ritux: refractory DM/PM, Anti synthetase sx, ref, anti SRP antiby nec

Answer 91

Most common muscle disease in >50 NT5C1A: predicts severe disease - and higher mortality it's not specific or sensitive sx: Quadriceps and distal fingers affected first (MRI - ant. thigh muscle selectively involved) reduced/absent knee reflex early in disease slow progression EMG/NCS: mixed muscle/nerve disease Biopsy: endomysial inflammation (required) rimmed vacuoles and proteinecous inclusions (not required)

Answer 92

**X-linked recessive** prox. LL --> prox UL Symmetrical weakness disphragm and swallowing weakness occurs later in disease Cardiac involvement, muscle cramps, myalgia, cognitive impairment Duchenne: More severe phenotype, onset with **1st eyar of life**. Loss of ambulation by 12-13 Becker MD: milder and variable phenotype, but **more cardiac involvement** Dx: biopsy/genetic - absence of dystrophin Tx: 1. Steroids - prolongs ambulation, preserved UL strength, reduce scoliosis, Cardio-pulm benefit * female carriers may develop CM

Answer 93

Type 1 (95%) **AD** hypomethylation of the subteleomeric region of Chr4q --> aberrant expression of **DUX4** *is a transcription factor* **Asymmetric** weakness of the orbicularis oculi, oris, rhomboids, serratus anterior, tricep, paraspinal, rectus abdominis, tibialis anterior - weakness of the facial muscles, the upper arms and the hip girdle Symptoms normal develop from the **age of 15 to 30** Associated with **retinal vasculopathy + hearing loss** with large deletions CK normal or mildly elevated EMG/biopsy is not specific Tx: Supportive

Answer 94

Creutzfeldt-Jakob disease (CJD) is rapidly progressive neurological condition caused by **prion proteins.** These proteins induce the formation of *amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases*. Features - dementia (rapid onset) - myoclonus Investigation * CSF is usually normal * EEG: **biphasic, high amplitude sharp waves (only in sporadic CJD)** * MRI: hyperintense signals in the **basal ganglia and thalamus**

Answer 95

- typically parietal-occipital stroke/stroke-like events - not in defined vascular territories - cognitive impairment/dementia - epilepsy and subclinical epilepsy - myopathy - elevated lactate in blood and CSF **Mutation in mitochondrial DNA tRNA LEu gene**

Answer 96

**AD** typically presents early childhood **complete paralysis of all four limbs and absent reflexes** Abortive episodes episodes may be triggered bt specific factors or situation Continues episodes --> proximal muscle weakness

Answer 97

mutation in calcium channel CACNA1S (+/- SCN4A) Triggered by carb or prolonged rest

Answer 98

mutation in SCN4A (sodium channel) Triggered by fasting or exercise Myotonia in eyes and hands PAINFUL

Answer 99

K+ channel KCNJ2 severe cardiac arrhythmias facial and skeletal features usually HYPOkalaemic

Answer 100

Glycogen storage disease myopathies (type2) **AR** Mutation in GAA (acid alpha glucosidase) gene --> enzymatic acid maltase deficiency Spectrum of severity in myopathy Elevated CK Tx: High protein with complex carbs Avoid strenuous exercise Enzyme replacement: aglucosidase Monitor for cardiac

Answer 101

**80% have AchR antibodies** (50% of occular MG) *very specific* *to note some sero convert over a 6 month period* Genetic RF in HLA genes and CHRNA1 W:M 7:3 <40yo M>W >50y/o Antibody mediated **T-cell dependent attack** on the postsynaptic mb: - bind to the alpha subunit - block the binding - increased internalisation of the receptor/degradation - MAC formation and damage Fatiguable **Thymus**: thymoma in 15%, thymic hyperplasia ~65% **Anti-MuSK** *muscle specific TK* Block MuSK-LEP4 binding --> reduced AChR clustering at the end plate found in a third of AChR neg MG, **mostly women 85%** - atypical presentation (neck extensor, shoulder and resp muscles) - severe bulbar, facial and ocular weakness - poor response to tx - **PLEX **>IVIG **Anti LRP4** - 20% of double neg MG **Anti striational** -80% thymoma without MG 30% adult onset MG ## Footnote antibodies are not used for monitoring

Answer 102

Sx: ptosis and binocular diplopia is the most common px symptoms Bulbar weakness, facial weakness, weak neck flexion Limb weakness (proximal) and symmetric Fnger/wrist extension, ankle dorsiflexion also affected

Answer 103

Abx: - amino glycosides, fluoroquinolones, tetracyclines, sulfonamides, penicillins, nitrofurantoin CVS: - betablocker, CCB, quinine, quinidine Checkpoint inhibitors IF alpha Mag D-penicillamine

Answer 104

Stroids, IVIG or PLEX - PLEX: fast onset in MG crisis can't use in sepsis or hypotension - IVIG: moderate - severe exacerbation improves after few days to weeks, effects last weeks to months SE: flu like sx, aseptic meningitis, increased thrombosis risk **Pyridostgmine:** - used for symptomatic tx alone - effetive 30-60mins later, last 3-4hrs - Anti MuSK don't respond **Corticosteroids** - effective within 2-3 weeks - short term exac can occur esp bulbar weakness **Steroid sparing** - Azathioprine (effective after 4-8 months) SE: myelosuppresion, toxic hepatitis, pancreatitis Chest TPMT genotype - MMF: inhibits monophosphaste dehydrogenase - effective after **1-2 months** - SE: nausea, diarrhoea, abdominal pain, leukopenia, anaemia Rituximab: - significant improvement in severe or refractory cases - esp in anti MuSK positive cases Thymectomy Eculizumab for anti AChR gen. MG

Answer 105

Antibodies Electrodiagnostic repetitive stimulation **single fibre electromyography** - most sensitive ice pack test - highly specific and sensitive for GM ptosis Edrophonium test: rapid acting esterase inhibitor CT chest TFT: **autoimmune thyroid disease** often associated wiht MG

Answer 106

- if purely ocular and symmetric ptosis think *chronic progressive external ophthalmoplegia*

Answer 107

P/Q VGCC antibodies less ACh release from presynaptic terminal 50/60s **male predominance** associated with SCLC incrementation NCS - decrement with low rates 3Hz, increment with fast rate 20-50Hz Tx: 3,4-diaminopyridine IVIG/PLEX acutely

Answer 108

PNS Classic: Acute onset of weakness and sensory sx in the LL that ascends to involve the UL/CN ACUTE FLACCID PARALYSIS M>F Subtypes: AIDP, AMAN, AMSAN >95% monophasic course **antecedent rsep or diarrhoel illness 4-6 weeks prior** Neuro nadir 2-4 weeks recovery after 2-3 weeks from nadir **Pathology**: - complement activation - upregulation of **Fc** receptor for **IgG** - macrophage induced contact-dependent injury - AIDP: schwann cell/myelin - AMAN/AMSAN: axon **Antigangioside antibodies:** relevant to AMAN and Miller Fisher sx

Answer 109

Campy CMB, EBV, mycoplasma, H. influenzae, hep E, Zika, Covid Vaccine, pregnancy, immunosuppression, surgical **if someone develops GBS within 6-8 weeks of a vaccine, that vaccine should not be given**

Answer 110

most common >90% form of GBS Initial presentation is sensory sx or pain (back pain is the most common sx - diffuse) Predom. motor polyradiculopathy with **acute** progressive symmetric weakness involving proximal and distal muscles **Reduced or absent reflexes** you can get resp muscle involvement 30% CN nerve >50% dysautonomia sphincter dysfunction can occur ## Footnote Anti glycan ab: LM1 or GM1 only 25-30%

Answer 111

**Ophthalmoplegia (diplopia), ataxia + areflexia** GQ1b antibodies in 80%

Answer 112

ophthalmoplegia, ataxia, altered consciousness/hyperreflexia

Answer 113

Normal cell count (some have WCC, but <50cell/mm3) Elevated protein ## Footnote IVIG can increase CSF WCC and protein

Answer 114

Early can be normal prolonged F wave latencies or absent response **AIDP: sural sparing pattern**, but UL sensory response absent or reduced Prolonged distal latencies, reduced motor nerve conduction velocities, prolonged F wave, increased temporal dispersion, and conduction block at non compressible sites AMAN: reduced cMAP AMSAN:reduced cMAP amplitude and reduced sNAP amplitudes ## Footnote NCS finding peak at 2 weeks of sx

Answer 115

IVIG + PLEX - equally efficacious - 40-50% don't have clinical response - combined treatment is not superior - don't given second course of IVIG (increases AE incl VTE) | if worsens, consensus is to retreat with the original tx ## Footnote IVIG is considered first line -need to give 2 weeks of onset PLEX: beneficial upto 4 weeks from onset

Answer 116

>40 preceding diarrhoeal illness short interval from onset to nadir mechanical ventilation high greade neuro deficit persistly low cMAP amp

Answer 117

- bilateral facial weakness (diplegia) with parasthesias

Answer 118

Tibial neuropathy would cause loss of ankle jerk, but weakness of plantarflexion (not dorsiflexion).

Answer 119

- unilateral lesion of the ventrocaudal pons may involve the basis pontis and te fascicles of CN VI and VII Symptoms: 1. Contralateral hemiplegia (sparing the face) 2. Ipsilateral lacteral rectus palsy wtih diplopia - accentuated when pt looks to the side of the lesion 3. Ipsilateral peripheral facial paresis (due to CN VII involvement)

Answer 120

KNEE EXTENSION KNEE JERK SENSORY TO INNER ASPECT OF THIGH AND LEG

Answer 121

CIM: - proximal muscle - failure to wean ventilator - Normal sensation - steroid use - Sensory nerv amp: >80% - Motor amp <80% - Needle EMG: normal MUPs recruitment - Short duration and low amp of MUPs +/- fibrillation potentials - CK high - Myopahty with myosin loss CIP: - Sensory <80% - Motor <80% - EPS: axonal motor and sensory poly neuropathy - Reduced recruitment of normal MUPs - Fibrillation and reduced recruitment of long duration, high amplitudes MUPs (late) -CK normal - neurogenic atrophy