NMJ Disorders Flashcards by Deependra Singh

NMJ Disorder
Characteristic Features

⭐ FATIGUABILITY on repetitive movement
⭐ Diurnal variation
⭐ Opthalmoplegia
⭐ Ptosis
⭐ Pharyngeal muscle / Bulbar Involvement
⭐ May progress to PROXIMAL MUSCLE Weakness

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Myasthenia Gravis types
Based on Organ Involvement

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Occular Myasthenia is diagnosed if

Myasthenia remains localised to Occular muscles for 3 years or more

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Pathogenesis of Myasthenia Gravis

T-cell and B-cell mediated

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Incidence of Myasthenia is MORE IN

Severity of Myasthenia is MORE IN

Incidence of Myasthenia is MORE IN
⭐ Females

Severity of Myasthenia is MORE IN
⭐ Males

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Bimodal Distribution of Myasthenia Gravis

⭐ EARLY-ONSET Myasthenia
20-40yrs
F > M

⭐ LATE-ONSET Myasthenia
> 60 yrs
M > F

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EARLY-ONSET Myasthenia
HLA-Association

HLA DR3
HLA DR9
HLA B8

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LATE-ONSET Myasthenia
HLA-Association

HLA DR2

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THYMIC HYPERPLASIA is ASSOCIATED eith

EARLY-ONSET Myasthenia

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Anti-Musk Antibodies in Myasthenia Gravis
IMPLICATIONS

Seen in 50% of ACh R ⛔
Females without THYMUS involvement
Neck Extensor Weakness
Absent in Occular Myasthenia
Tongue Fasiculations ➕
Pyridostigmine REFRACTORY

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HEAD DROP in Neurology
(OR)
Dropped head syndrome
(OR)
Floppy Head Syndrome
(OR)
Broken Neck Sign

Weakness of Extensor muscles
1. GBS
2. ALS
3. INFLAMMATORY MUSCLE DISEASE
4. MYASTHENIA with Anti-Musk ➕
5. Polymyositis
6. Fascio-scapulo-humeral Dystrophy
7. Neurotoxic Snake Bite
8. Organophosphorus poisoning

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In Neurology

HLA DR2
HLA DR3

Dr2: Multiple Sclerosis

Dr3: Myasthenia Gravis

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EARLY-ONSET Myasthenia
Antibodies

Anti-ACh R

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LATE-ONSET Myasthenia
Antibodies

Anti-AChR
Anti-Titin
Anti-Ryanodine

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INITIAL PRESENTATION OF MYASTHENIA

⭐ 66% start with OCCULAR MYASTHENIA
⭐ 10% start with PROXIMAL LIMB WEAKNESS
⭐ Remaining: BULBOPHARYNGEAL Involvement (dysphagia, difficulty chewing and swallowing)

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⭐ B/L SYMMETRICAL PTOSIS Seen in

⭐ U/L or B/L ASYMMETRICAL PTOSIS seen in

⭐ B/L SYMMETRICAL PTOSIS Seen in
✨ CPEO

⭐ U/L or B/L ASYMMETRICAL PTOSIS seen in
✨ MYASTHENIA GRAVIS

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On SUSTAINED UPGAZE IN MYASTHENIA GRAVIS

Ptosis ⬆️ ⬆️

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CURTAIN sign

On trying to lift the PTOTIC EYE, the other eye goes for PTOSIS

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PEAK SIGN

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Can’t close the eyes tightly, EYELASHES Don’t get Buried

PUPIL SPARING 3rd NERVE PALSY Seen in

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Myasthenia Gravis

⚡⚡ MOST COMMON EXTRAOCULAR MUSCLE INVOLVED IN MYASTHENIA

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Medial Rectus

FACIES in MYASTHENIA GRAVIS

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SNARLING FACIES
Expressionless facies

COGAN’S LID TWITCH
Seen in

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⭐ MYASTHENIA GRAVIS

⭐ Overshoot of the lids when the patient suddenly looks up

DROOPED POSTURE
SEEN.IN
WHY

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Myasthenia Gravis
⭐ neck extensor affected

Dysarthria in MYASTHENIA GRAVIS

Myasthenic Dysarthria

ICE PACK TEST

Improves weakness Improves ptosis by ≥ 2mm

⚡⚡ MOST SPECIFIC TEST FOR MYASTHENIA GRAVIS

Acetylcholine Receptor Antibody test

⚡⚡ MOST SENSITIVE TEST FOR MYASTHENIA GRAVIS

Single Nerve Fiber Electromyography ⬇️ Show JITTERINESS

Edrophonium vs Pyridostigmine in MYASTHENIA GRAVIS

5D of Myasthenia GRAVIS

Drugs that exacerberate MYASTHENIA

Penicillamine Cyclosporine

Lambert Eaton Syndrome vs MYASTHENIA GRAVIS

DOIS Sign Seen in

Lambert Eaton Syndrome ⭐ on repeated nerve stimulation test ➡️ Incremental response ➕

💊💉 MANAGEMENT of Lambert Eaton Syndrome

3,4 Diaminopyridine

PRESENTATION OF LAMBERT EATON SYNDROME

Proximal LOWER LIMB WEAKNESS ➕ AUTONOMIC FEATURES

CONGENITAL MYASTHENIC SYNDROMES

⭐ Slow Channel (AD) ⭐ Low affinity Fast Channel (AR) ⭐ Severe AChR deficiency (AR) ⭐ AChE deficiency

INDICATIONS of THYMECTOMY

⭐ Age 18-65 yrs ➕ Anti-AChR ➕ Generalized myasthenia ⭐ THYMOMA

Good's Syndrome

Thymoma ➕ Hypogammaglobulinemia (Combined B and T cell immunodeficiency)

Cholinergic Crisis Features 🧠⚡SLUDGE B³ ⚡

Use of IVIG or PLEX in MYASTHENIA GRAVIS

1. Myasthenic Crisis: Respiratory insufficiency and Dysphagia 2. Refractory MYASTHENIA 3. Before Surgery in Bulbar Dysfunction 4. Musk ➕ cases refractory to cholinesterase inhibitors

Baseline CMAP in ⭐ LAMBERT EATON SYNDROME ⭐ MYASTHENIA GRAVIS

⭐ LAMBERT EATON SYNDROME ✨ REDUCED ⭐ MYASTHENIA GRAVIS ✨ NORMAL

Low Frequency 3Hz Stimulation in ⭐ LAMBERT EATON SYNDROME ⭐ MYASTHENIA GRAVIS

⭐ LAMBERT EATON SYNDROME ✨ VARIABLE ⭐ MYASTHENIA GRAVIS ✨ DECREMENTAL Response

HIGH FREQUENCY STIMULATION in ⭐ LAMBERT EATON SYNDROME ⭐ MYASTHENIA GRAVIS

⭐ LAMBERT EATON SYNDROME ✨ 100% RISE ⭐ MYASTHENIA GRAVIS ✨ Mild Incremental Response

LOWER LIMB INVOLVEMENT IN ⭐ LAMBERT EATON SYNDROME ⭐ MYASTHENIA GRAVIS

⭐ LAMBERT EATON SYNDROME Early and PRESENTING FEATURE ⭐ MYASTHENIA GRAVIS VERY LATE or Absent also ➡️ Hip muscles

Variants of MYASTHENIA GRAVIS

1. Ocular Myasthenia 2. Early Onset Myasthenia 3. Late Onset Myasthenia 4. Anti-Musk Myasthenia 5. Double Negative Myasthenia

Antibodies in Double Negative Myasthenia

Anti Lipoprotein Receptor-4 ➕

ASSOCIATED disease with MYASTHENIA

1. Type 1 DM 2. Vitiligo 3. Addison's disease 4. Hashimoto's thyroiditis 5. Pernicious anemia

NMJ Disorders Flashcards

(47 cards)