Neurology

13.

Neuromuscular Junction Disorders

13.1.

Normal Neuromuscular Transmission

13.1.1.

Anatomy of the Neuromuscular Junction

13.1.1.1.

Presynaptic Terminal

13.1.1.2.

13.1.1.3.

Postsynaptic Membrane

13.1.2.

Physiology of Neuromuscular Transmission

13.1.2.1.

Acetylcholine Synthesis

13.1.2.2.

Vesicle Release

13.1.2.3.

Receptor Binding

13.1.2.4.

Muscle Contraction

13.1.2.5.

Acetylcholine Degradation

13.1.3.

13.2.

Myasthenia Gravis

13.2.1.

13.2.1.1.

Incidence and Prevalence

13.2.1.2.

Age and Gender Distribution

13.2.1.3.

Geographic Variations

13.2.2.

Pathophysiology

13.2.2.1.

Autoimmune Mechanism

13.2.2.2.

Acetylcholine Receptor Antibodies

13.2.2.3.

MuSK Antibodies

13.2.2.4.

LRP4 Antibodies

13.2.2.5.

Seronegative Myasthenia Gravis

13.2.2.6.

Thymic Abnormalities

13.2.3.

Clinical Features

13.2.3.1.

Fluctuating Weakness

13.2.3.2.

13.2.3.3.

Ocular Symptoms

13.2.3.3.1.

13.2.3.3.2.

13.2.3.4.

Bulbar Symptoms

13.2.3.4.1.

13.2.3.4.2.

13.2.3.4.3.

Chewing Difficulty

13.2.3.5.

13.2.3.6.

Respiratory Symptoms

13.2.4.

13.2.4.1.

Ocular Myasthenia Gravis

13.2.4.2.

Generalized Myasthenia Gravis

13.2.4.3.

MGFA Classification

13.2.5.

13.2.5.1.

Clinical Assessment

13.2.5.2.

Pharmacological Tests

13.2.5.2.1.

Edrophonium Test

13.2.5.2.2.

13.2.5.3.

Serological Tests

13.2.5.3.1.

AChR Antibodies

13.2.5.3.2.

MuSK Antibodies

13.2.5.3.3.

LRP4 Antibodies

13.2.5.4.

Electrophysiological Tests

13.2.5.4.1.

Repetitive Nerve Stimulation

13.2.5.4.2.

Single-Fiber EMG

13.2.5.5.

13.2.5.5.1.

13.2.6.

13.2.6.1.

Symptomatic Treatment

13.2.6.1.1.

Cholinesterase Inhibitors

13.2.6.1.1.1.

13.2.6.1.1.2.

13.2.6.2.

Immunosuppressive Therapy

13.2.6.2.1.

Corticosteroids

13.2.6.2.2.

13.2.6.2.3.

Mycophenolate Mofetil

13.2.6.2.4.

13.2.6.2.5.

13.2.6.2.6.

13.2.6.3.

Acute Treatment

13.2.6.3.1.

Plasma Exchange

13.2.6.3.2.

Intravenous Immunoglobulin

13.2.6.4.

13.2.6.4.1.

13.2.6.4.2.

Surgical Approaches

13.2.6.4.3.

13.2.6.5.

Emerging Therapies

13.2.6.5.1.

13.2.6.5.2.

13.2.6.5.3.

13.2.7.

Myasthenic Crisis

13.2.7.1.

Precipitating Factors

13.2.7.2.

Clinical Features

13.2.7.3.

13.2.7.3.1.

Respiratory Support

13.2.7.3.2.

13.2.7.3.3.

Supportive Care

13.2.8.

Drug-Induced Myasthenia

13.2.8.1.

13.2.8.2.

13.2.8.3.

Checkpoint Inhibitors

13.3.

Lambert-Eaton Myasthenic Syndrome

13.3.1.

13.3.2.

Pathophysiology

13.3.2.1.

Voltage-Gated Calcium Channel Antibodies

13.3.2.2.

Presynaptic Dysfunction

13.3.2.3.

Associated Malignancies

13.3.3.

Clinical Features

13.3.3.1.

Proximal Weakness

13.3.3.2.

Autonomic Symptoms

13.3.3.3.

13.3.3.4.

13.3.4.

13.3.4.1.

Clinical Criteria

13.3.4.2.

Electrophysiological Tests

13.3.4.2.1.

Repetitive Nerve Stimulation

13.3.4.2.2.

Post-Exercise Facilitation

13.3.4.3.

Serological Tests

13.3.4.3.1.

VGCC Antibodies

13.3.4.4.

Malignancy Screening

13.3.5.

13.3.5.1.

Symptomatic Treatment

13.3.5.1.1.

3,4-Diaminopyridine

13.3.5.1.2.

13.3.5.2.

Immunosuppressive Therapy

13.3.5.3.

Cancer Treatment

13.4.

Congenital Myasthenic Syndromes

13.4.1.

13.4.1.1.

Presynaptic CMS

13.4.1.2.

13.4.1.3.

Postsynaptic CMS

13.4.2.

13.4.2.1.

13.4.2.2.

13.4.2.3.

CHRNE Mutations

13.4.2.4.

13.4.2.5.

Other Genetic Causes

13.4.3.

Clinical Features

13.4.3.1.

13.4.3.2.

Fluctuating Weakness

13.4.3.3.

Respiratory Symptoms

13.4.4.

13.4.4.1.

Genetic Testing

13.4.4.2.

Electrophysiological Studies

13.4.4.3.

13.4.5.

13.4.5.1.

Cholinesterase Inhibitors

13.4.5.2.

3,4-Diaminopyridine

13.4.5.3.

13.4.5.4.

13.5.

13.5.1.

13.5.1.1.

Clostridium botulinum

13.5.1.2.

Botulinum Toxin Types

13.5.2.

Types of Botulism

13.5.2.1.

Foodborne Botulism

13.5.2.2.

13.5.2.3.

Infant Botulism

13.5.2.4.

Iatrogenic Botulism

13.5.2.5.

Inhalational Botulism

13.5.3.

Pathophysiology

13.5.3.1.

Toxin Mechanism

13.5.3.2.

SNARE Protein Cleavage

13.5.3.3.

Acetylcholine Release Blockade

13.5.4.

Clinical Features

13.5.4.1.

Descending Paralysis

13.5.4.2.

Cranial Nerve Involvement

13.5.4.3.

Autonomic Symptoms

13.5.4.4.

Respiratory Failure

13.5.5.

13.5.5.1.

Clinical Criteria

13.5.5.2.

Laboratory Tests

13.5.5.2.1.

Toxin Detection

13.5.5.2.2.

13.5.5.3.

Electrophysiological Studies

13.5.6.

13.5.6.1.

Supportive Care

13.5.6.2.

13.5.6.3.

Antibiotics (Wound Botulism)

13.5.6.4.

Respiratory Support

13.5.7.

13.6.

Other Neuromuscular Junction Disorders

13.6.1.

Organophosphate Poisoning

13.6.1.1.

13.6.1.2.

Clinical Features

13.6.1.3.

13.6.1.4.

13.6.2.

Snake Bite Envenomation

13.6.2.1.

Neurotoxic Snakes

13.6.2.2.

Clinical Features

13.6.2.3.

13.6.3.

13.6.3.1.

13.6.3.2.

Clinical Features

13.6.3.3.

13.6.4.

Hypermagnesemia

13.6.4.1.

13.6.4.2.

Clinical Features

13.6.4.3.

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12. Disorders of the Peripheral Nervous System

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14. Muscle Diseases