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NMJ Disorders Flashcards

1
Q

NMJ Disorder
Characteristic Features

A

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

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2
Q

Myasthenia Gravis types
Based on Organ Involvement

A
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3
Q

Occular Myasthenia is diagnosed if

A

Myasthenia remains localised to Occular muscles for 3 years or more

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4
Q

Pathogenesis of Myasthenia Gravis

A

T-cell and B-cell mediated

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5
Q

Incidence of Myasthenia is MORE IN

Severity of Myasthenia is MORE IN

A

Incidence of Myasthenia is MORE IN
⭐ Females

Severity of Myasthenia is MORE IN
⭐ Males

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6
Q

Bimodal Distribution of Myasthenia Gravis

A

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

⭐ LATE-ONSET Myasthenia
> 60 yrs
M > F

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7
Q

EARLY-ONSET Myasthenia
HLA-Association

A

HLA DR3
HLA DR9
HLA B8

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8
Q

LATE-ONSET Myasthenia
HLA-Association

A

HLA DR2

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9
Q

THYMIC HYPERPLASIA is ASSOCIATED eith

A

EARLY-ONSET Myasthenia

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10
Q

Anti-Musk Antibodies in Myasthenia Gravis
IMPLICATIONS

A
  1. Seen in 50% of ACh R ⛔
  2. Females without THYMUS involvement
  3. Neck Extensor Weakness
  4. Absent in Occular Myasthenia
  5. Tongue Fasiculations ➕
  6. Pyridostigmine REFRACTORY
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11
Q

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

A

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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12
Q

In Neurology

HLA DR2
HLA DR3

A

Dr2: Multiple Sclerosis

Dr3: Myasthenia Gravis

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13
Q

EARLY-ONSET Myasthenia
Antibodies

A

Anti-ACh R

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14
Q

LATE-ONSET Myasthenia
Antibodies

A

Anti-AChR
Anti-Titin
Anti-Ryanodine

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15
Q

INITIAL PRESENTATION OF MYASTHENIA

A

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

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16
Q

⭐ B/L SYMMETRICAL PTOSIS Seen in

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

A

⭐ B/L SYMMETRICAL PTOSIS Seen in
✨ CPEO

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

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17
Q

On SUSTAINED UPGAZE IN MYASTHENIA GRAVIS

A

Ptosis ⬆️ ⬆️

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18
Q

CURTAIN sign

A

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

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19
Q

PEAK SIGN

A

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

20
Q

PUPIL SPARING 3rd NERVE PALSY Seen in

A

Myasthenia Gravis

21
Q

⚡⚡ MOST COMMON EXTRAOCULAR MUSCLE INVOLVED IN MYASTHENIA

A

Medial Rectus

22
Q

FACIES in MYASTHENIA GRAVIS

A

SNARLING FACIES
Expressionless facies

23
Q

COGAN’S LID TWITCH
Seen in

A

⭐ MYASTHENIA GRAVIS

⭐ Overshoot of the lids when the patient suddenly looks up

24
Q

DROOPED POSTURE
SEEN.IN
WHY

A

Myasthenia Gravis
⭐ neck extensor affected

25
Q

Dysarthria in MYASTHENIA GRAVIS

A

Myasthenic Dysarthria

26
Q

ICE PACK TEST

A

Improves weakness
Improves ptosis by ≥ 2mm

27
Q

⚡⚡ MOST SPECIFIC TEST FOR MYASTHENIA GRAVIS

A

Acetylcholine Receptor Antibody test

28
Q

⚡⚡ MOST SENSITIVE TEST FOR MYASTHENIA GRAVIS

A

Single Nerve Fiber Electromyography
⬇️
Show JITTERINESS

29
Q

Edrophonium vs Pyridostigmine in MYASTHENIA GRAVIS

30
Q

5D of Myasthenia GRAVIS

31
Q

Drugs that exacerberate MYASTHENIA

A

Penicillamine
Cyclosporine

32
Q

Lambert Eaton Syndrome vs MYASTHENIA GRAVIS

33
Q

DOIS Sign
Seen in

A

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

34
Q

💊💉 MANAGEMENT of Lambert Eaton Syndrome

A

3,4 Diaminopyridine

35
Q

PRESENTATION OF LAMBERT EATON SYNDROME

A

Proximal LOWER LIMB WEAKNESS ➕ AUTONOMIC FEATURES

36
Q

CONGENITAL MYASTHENIC SYNDROMES

A

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

37
Q

INDICATIONS of THYMECTOMY

A

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

38
Q

Good’s Syndrome

A

Thymoma ➕ Hypogammaglobulinemia
(Combined B and T cell immunodeficiency)

39
Q

Cholinergic Crisis
Features
🧠⚡SLUDGE B³ ⚡

40
Q

Use of IVIG or PLEX in MYASTHENIA GRAVIS

A
  1. Myasthenic Crisis: Respiratory insufficiency and Dysphagia
  2. Refractory MYASTHENIA
  3. Before Surgery in Bulbar Dysfunction
  4. Musk ➕ cases refractory to cholinesterase inhibitors
41
Q

Baseline CMAP in
⭐ LAMBERT EATON SYNDROME
⭐ MYASTHENIA GRAVIS

A

⭐ LAMBERT EATON SYNDROME
✨ REDUCED

⭐ MYASTHENIA GRAVIS
✨ NORMAL

42
Q

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

A

⭐ LAMBERT EATON SYNDROME
✨ VARIABLE

⭐ MYASTHENIA GRAVIS
✨ DECREMENTAL Response

43
Q

HIGH FREQUENCY STIMULATION in
⭐ LAMBERT EATON SYNDROME
⭐ MYASTHENIA GRAVIS

A

⭐ LAMBERT EATON SYNDROME
✨ 100% RISE

⭐ MYASTHENIA GRAVIS
✨ Mild Incremental Response

44
Q

LOWER LIMB INVOLVEMENT IN
⭐ LAMBERT EATON SYNDROME
⭐ MYASTHENIA GRAVIS

A

⭐ LAMBERT EATON SYNDROME
Early and PRESENTING FEATURE

⭐ MYASTHENIA GRAVIS
VERY LATE or Absent also ➡️ Hip muscles

45
Q

Variants of MYASTHENIA GRAVIS

A
  1. Ocular Myasthenia
  2. Early Onset Myasthenia
  3. Late Onset Myasthenia
  4. Anti-Musk Myasthenia
  5. Double Negative Myasthenia
46
Q

Antibodies in Double Negative Myasthenia

A

Anti Lipoprotein Receptor-4 ➕

47
Q

ASSOCIATED disease with MYASTHENIA

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

3 NERVOUS SYSTEM (40 decks)

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