Neuropathy, Myopathy, and NMD Flashcards

1
Q

What is a Mononeuropathy?

A

A single, major nerve is involved with sensory or motor deficits related to its anatomical distribution

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2
Q

Wha are the 2 most common causes of mononeuropathies?

A

Trauma or Compression

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3
Q

Common cause of Ulnar Mononeuropathy?

A

Leaning or falling on the elbow

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4
Q

Common cause of Peroneal Mononeuropathy?

A

Injury to the lateral knee

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5
Q

Carpal Tunnel Syndrome

Describe the pathophysiology

A

If there is inflammation or swelling of the flexor tendons in the carpal tunnel, they can compress the median nerve and cause pain

May also be caused by fluid retention in pregnancy

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6
Q

Carpal Tunnel Syndrome

Clinical Symptoms

A

Tingling numbness of hand (thumb, index, middle, 1/2 ring finger)

Thenar weakness and atrophy in very severe cases

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7
Q

Carpal Tunnel Syndrome

Treatment

A

Local rest

Anti-inflammatories

Splinting

Surgical decompression of the nerve

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8
Q

Describe Wallerian degeneration of nerves

A

Occurs at sites of severe nerve injury

Injury involves myelin and axons distal to the point of injury

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9
Q

Do nerves regenerate?

A

CNS neurons are not good at regenerating, but PNS neurons may regenerate. Likelihood of regrowth is based on distance needed to travel and the degree of injury sustained

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10
Q

What is a polyneuropathy?

A

Disorder of multiple, major and small nerves

Characterized by an early sensory loss in distal limbs

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11
Q

Polyneuropathy

Clinical Symptoms

A

Spontaneous tingling

Pins and needles = Paresthesia

Unpleasant stimulation from non-painful stimuli = Dysesthesia

Distal limb weakness and atrophy

Early loss or decrease of muscle stretch reflexes

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12
Q

What are two general big causes of Polyneuropathy?

A

Axonal degeneration (more common)

Demyelination

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13
Q

How are axonal degeneration and demyelination discriminated on nerve conduction studies?

A

Axonal degeneration – amplitude of waves are reduced

Demyelination – slowing of the nerve conduction

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14
Q

What is EMG testing?

A

Needle electromyography

Electrical activity of muscles within individual motor units is assessed for denervation, reinnervation, or muscle disease

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15
Q

When are nerve biopsies performed?

A

Rarely. Only for significant neuropathy and typically only done for sensory nerves.

The patient is left with numbness after the biopsy

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16
Q

Polyneuropathy

Treatment

A

Address underlying causes (diabetes, alcohol)

Orthotic devices – improve ambulation

Physical therapy

Meds to improve paresthesia or dysesthesia

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17
Q

Guillan Barre Syndrome

Clinical Symptoms

A

Ascending, areflexic paralysis

Acute polyneuropathy and weakness progressing quickly and at its worst by 4 wks

18
Q

Guillan Barre Syndrome

What often precedes this syndrome? Describe the pathology.

A

Acute viral respiratory infection precedes the syndrome 50% of the time.

Misdirection of immune system to attack normal peripheral nerve myelin

Get inflammation and demyelination of the peripheral nerves or roots

19
Q

Guillan Barre Syndrome

Diagnosis

A

Evidence of demyelination seen on EMG (slowing of conduction)

Elevated protein in CSF, few if any WBCs

20
Q

Guillan Barre Syndrome

Prognosis and Treatment

A

Good prognosis with medical treatment

Plasmapheresis or IVIG may shorten illness and hasten recovery

21
Q

List some potential causes of Chronic Acquired Polyneuropathies

A
Diabetes
Hypothyroid
Uremia
Cancer
Myeloma
Rheumatoid arthritis
Lupus
Nutritional defects (Vit B)
Infection (leprosy)
Toxins (alcohol, lead)
22
Q

Hereditary Neuropathies

Clinical Symptoms

A

Onset usually in childhood

Distal sensorimotor deficits

Little/no paresthesia or dysthesia

May affect skeletal development (scoliosis, hammertoes, pes cavus)

23
Q

What is a myopathy?

A

Primary disease of muscle

24
Q

What is the first sign of myopathy?

A

PROXIMAL limb weakness and atrophy (shoulder and hip)

25
Symptoms of Myopathy
Weakness Fatigue, maybe with cramping Proximal limb weakness (shoulders and hips) Late loss of reflexes (after significant atrophy) Intact sensation
26
Myopathy | Diagnostic Methods
Review family history Serum enzymes (CK) often elevated EMG demonstrates muscle disorder Muscle biopsy
27
What is Polymyositis?
Inflammation and weakness of multiple muscles
28
What are the causes of Polymyositis?
Viral infection Drug reaction Autoimmune
29
Polymyositis | Diagnosis
EMG testing Presence of inflammation and muscle fiber necrosis on muscle biopsy
30
Polymyositis | Treatment
Immunosuppression (corticosteroids)
31
Polymyositis | Clinical Symptoms
Proximal weakness (like a myopathy) Rash around eyes or fingers
32
What is Muscular Dystrophy?
Hereditary myopathy of variable progression and severity
33
Duchenne's Muscular Dystrophy | What is the inheritance? What is the pathological defect?
X linked inheritance Absence of dystrophin (a crucial structural protein in muscle)
34
Duchenne's Muscular Dystrophy | Clinical Symptoms
More common in boys (X linked) Proximal weakness Calf pseudohypertrophy from muscle replaced by fat Cardiorespiratory death by 3rd decade
35
What is Gower's Maneuver?
Have pt lay on floor belly down Tell them to get up --> weak hip girdle causes them to crawl up on hands and legs before being able to rise
36
What is Motor Neuron Disease (MND)?
Disorders, sometimes hereditary, where degeneration of UMNs and LMNs occur with varying severity and progression rate
37
Spinal Muscular Atrophy | What cells are involved?
MND with Anterior horn involvement
38
Spinal Muscular Atrophy | Clinical Symptoms
LMN signs of weakness, atrophy, areflexia, fasciculations
39
Spinal Muscular Atrophy | Infantile form is...
Werdnig Hoffman | Terminal condition due to rapid development of diffuse weakness
40
Amyotrophic Lateral Sclerosis | Describe the pathology
Progressive degeneration of ALL motor neuron subgroups (UMNs and LMNs)
41
Amyotrophic Lateral Sclerosis | Prognosis and Treatment
Worst prognosis (50% dead within 3 years) No curative treatment, but Riluzole (glutamate antagonist) may prolong survival by months
42
Amyotrophic Lateral Sclerosis | Clinical Symptoms
Start --> Regional weakness and atrophy of limb, which spreads and becomes bilateral Diffuse fasciculations Hyperreflexia Spasticity Babinski sign positive Weakness affecting speech, swallowing, chewing, breathing