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Department of Neurology

 

Milestone 5: Neuromuscular Disorders — Patient Care

 

Specifics about each Level and each Item:  What you do you need to know:
 

Level 2

• Identifies patterns of neuromuscular disease (e.g., anterior horn cell disease, nerve root, plexus, peripheral nerve, neuromuscular junction, muscle)

• Identifies neuromuscular disorder emergencies

Guillain Barré Syndrome
Myasthenic Crisis
Critical Illness Myopathy and Neuropathy
Botulism
Mononeuropathy Multiplex
Peripheral nerve injury

• Orders NCS (nerve conductive study)/EMG (electromyography) testing appropriately


Level 3

• Diagnoses and manages common neuromuscular disorders

Common entrapment/compressive neuropathies (carpal tunnel syndrome, ulnar neuropathy at the elbow, radial neuropathy across the spiral groove, peroneal neuropathy at the fibular neck)
Peripheral polyneuropathy
Diabetic polyneuropathy
Guillain-Barré syndrome
Myasthenia Gravis
Amyotrophic lateral sclerosis
Cervical and lumbosacral radiculopathy
Myopathy
Syncope
Orthostatic hypotension

• Manages neuromuscular disorder emergencies

Guillain Barré Syndrome
Myasthenic Crisis
Critical Illness Myopathy and Neuropathy
Botulism
Mononeuropathy Multiplex
Peripheral nerve injury

• Interprets results of NCS/EMG testing in context of clinical presentation


Level 4 

• Diagnoses uncommon neuromuscular disorders

Amyotrophic lateral sclerosis variants (primary lateral sclerosis, progressive muscular atrophy, bulbar palsy)
Spinal muscular atrophy (including Kennedy’s disease)
Poliomyelitis and post-poliomyelitis syndrome
Charcot-Marie-Tooth Diseases
Guillain-Barré syndrome variants
Chronic inflammatory demyelinating polyneuropathy and its variants
Diabetic neuropathies variants (amyotrophy, mononeuropathies, autonomic)
Dysproteinemic neuropathies
Nutritional and alcoholic neuropathies
Toxic neuropathies
Ischemic/vasculitic neuropathies
Neuropathies of connective tissue disorders
Infectious and granulomatous neuropathies
Lambert-Eaton myasthenic syndrome
Botulism
Congenital myasthenic syndromes
Muscular dystrophies (including Duchenne and Becker, limb girdle, facioscapulohumeral, myotonic dystrophies)
Metabolic and mitochondrial myopathies
Inflammatory myopathies (Polymyositis/dermatomyositis/necrotizing immune myopathy/
Inclusion body myositis)
Paraspinous myopathy (Dropped head and bent spine syndromes)
Endocrine myopathies
Toxic myopathies
Infectious and granulomatous myopathies
Critical illness myopathy and neuropathy
Myotonia Congenita
Periodic Paralysis
Myokymia and neuromyotonia syndromes
Paraneoplastic neuromuscular syndromes

• Recognizes when tissue biopsy is warranted

Muscle biopsy (muscular dystrophies, inflammatory myopathies, mitochondrial myopathies)
Nerve biopsy (mononeuritis multiplex, amyloidosis, and sarcoidosis)
Skin biopsy (small fiber neuropathy, autonomic neuropathy)


Resources for each Level:

Level 2 Resources:

Inpatient rotations
Introduction and Emergency Neurology lecture series
Neuromuscular grand rounds


Level 3 Resources:

EMG and NCS and Neuromuscular Lecture Series

NCS Basics
NCS Patterns
Late Responses
Bedside NM Exam
Artifacts
Anomalous Innervations
Repetitive Nerve Stimulation
Nerve Conduction Testing
Needle EMG: Spontaneous Activity
Needle EMG: Spontaneous Activity
Needle EMG: Voluntary Activity
Autonomic Testing
Basic Anatomy and Physiology of Nerve and Muscle
Laboratory Testing NM Diseases
Autonomic Disorders
Spinal Anatomy and Imaging
Basic Anatomy and NCS
UE Neuropathies
LE Neuropathies
Radiculopathy
Carpal tunnel Injection and Tensilon Test
Diabetic Neuropathies
Approach to Peripheral Neuropathy
NMJ Disorders
Muscle & Nerve Biopsy (normal and major abnormal patterns)
Neuronopathies (Motor and Sensory)
Myopathy (non-inherited)
GBS and CIDP
Gait and Voice disorders in NM Disease
Myotonic Disorders
Small fiber neuropathies
Inherited Muscle Disease

Neuromuscular Grand Rounds

Outpatient clinics

Self-reading


Level 4 Resources

Neuromuscular Grand Rounds

Inpatient Genral service

Outpatient clinics

Self-reading

References

Chapters

Preston DC, Shapiro B, Proximal, Distal and Generalized Weakness, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.

Wang LH, Pestronk A, Muscle pain and Cramps, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.

Katirji B, Koontz D. Peripheral Neuropathy, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.

Meriggolli MN, Sanders DB. Disorders of Neuromuscular Junction, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.

Amato AA, Brooks MH. Disorders of Skeletal Muscle, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.

Chelimsky T, Robertson D, Chelimsky G. Disorders of The Autonomic Nervous System, in Daroff RB, Fenichel GM, Jankovic J, Maziotta J (eds). Neurology in Clinical Practice. Sixth edition. Philadelphia, Elsevier/Saunders, 2012.
 

Books

Katirji B, Kaminski HJ, Ruff RL (eds). Neuromuscular disorders in Clinical Practice. Second edition. Springer, New York, 2014.

Preston DC, Shapiro B. Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic Correlations. Third edition. Elsevier/Saunders, New York, 2013.