Peripheral Nerve Disorders Flashcards

1
Q

Peripheral Nerve Disorders

Subtypes

A

Peripheral nerve ⇒ neuropathy

Nerve root ⇒ radiculopathy

Lumbar or brachial plexus ⇒ plexopathy

Anterior horn ⇒ motor neuron disease

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2
Q

Peripheral Nervous System

Basic Anatomy

A
  • Consists of nerves and ganglia outside of the brain and spinal cord
  • Peripheral nerves connected to spinal cord by two roots:
    • Ventral root ⇒ motor
    • Dorsal root ⇒ sensory
  • Interconnects all other tissues with the CNS
  • Innervates all voluntary muscles
  • Transmits the sensory impulses to the whole body
  • Individual nerve fibers ⇒ vary widely in diameter and may be heavily myelinated, lightly myelinated or unmyelinated
    • Amount of myelin ∝ conduction speed
  • Largely under conscious (cerebral) control
  • Injury results in both sensory and motor loss
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3
Q

Sensory Innervation

A
  • A sensory nerve (afferent nerve) carries sensory information toward the CNS
  • It is a bundle of nerve fibers coming from sensory receptors in the PNS
  • Afferent nerve fibers leave the sensory neuron from the dorsal root ganglia of the spinal cord
  • Large myelinated sensory fibers ⇒ vibration and proprioception
  • Small myelinated and unmyelinated sensory fibers ⇒ pain and temperature
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4
Q

Motor Innervation

A
  • Upper Motor Neuron
    • Neuron in the motor cortex
    • Travels down and decussates at the level of the medulla
    • Travels down the spinal cord
  • Lower Motor Neuron
    • Neuron in the spinal cord or cranial nerve of brainstem
    • Travel to the skeletal muscle
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5
Q

Motor Unit

A

A motor neuron and all the skeletal fibers it innervates.

  • Groups of motor units work together to coordinate contraction of a single muscle
  • It is a functional unit made up of:
    • Lower motor neuron
    • Motor axon
    • NMJs
    • Muscle fibers innervated by the motor neuron
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6
Q

Upper Motor Neuron

Signs

A
  • Weakness
  • Hyperreflexia
  • Spasticity
  • Stiffness
  • Babinski sign
  • Brisk jaw jerk
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7
Q

Lower Motor Neuron

Signs

A
  • Fasciculations
  • Atrophy/muscle wasting
  • Weakness
  • Muscle cramps
  • Loss of reflexes
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8
Q

Disease Localization

A

Differentiating PNS from CNS disease

  • Weakness or sensory loss confined to one limbperipheral nerve problem
    • Associated pain in the extremity ⇒ ↑ likelihood of a peripheral process
  • Localization less obvious when weakness/sensory loss involves > 1 extremity or when there is no associated pain or discomfort
  • The patient approach will include taking a detailed hx including family hx of similar sx, time course of sx, and comorbidities (ex. DM)
  • Complete neurologic exam will be extremely helpful
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9
Q

Peripheral Nerve

Composition

A

Peripheral nerve is composed of:

  • Motor axon/neuron
  • Sensory axon/neuron
  • Autonomic fibers
  • Myelin
  • Other supporting structures
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10
Q

Peripheral Nerve

Characteristics

A
  • Individual nerve fibers ⇒ vary widely in diameter and may be heavily myelinated, lightly myelinated or unmyelinated
    • Amount of myelin ∝ conduction speed
    • Myelin made by Schwann cells
    • Distance b/t nodes of Ranvier determines conduction rate
  • Largely under conscious (cerebral) control
  • Injury results in both sensory and motor loss
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11
Q

Peripheral Nerve Damage

A
  • Segmental demyelination
    • Diseases primarily affect Schwann cells ⇒ loss of myelin
  • Nerve injury/transection
    • Primary destruction of the axon ⇒ secondary disintegration of its myelin sheath
    • Results in axonal degeneration
      • Distal portion ⇒ Wallerian degeneration
      • Proximal portion ⇒ retrograde degeneration
      • Begins in the periphery and proceeds back toward the cell body ⇒ “dying back“
    • Cell body may become chromatolytic
    • If process reversed early phases, some regeneration may be possible
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12
Q

Axonal Injury

A

Traumatic transection of a nerve

  • Distal portion of the nerve fiber degenerates ⇒ Wallerian degeneration
    • Axons begin to break down
    • Schwann cells begin to catabolize myelin and later engulf axon fragments, forming myelin ovoids
    • MΦ recruited and phagocytose axonal and myelin-derived debris
  • Proximal portion of severed nerve degenerates ⇒ retrograde degeneration
    • Degenerative changes involving only the most distal two or three internodes
    • Proximal stump then undergoes regenerative activity
    • Regenerated fiber has shorter internodes
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13
Q

Segmental Demyelination

A
  • Selective loss of individual myelin internodes with preservation of the axon
  • Process affects some Schwann cells and their corresponding internodes while sparing others
  • Disintegrating myelin is engulfed initially by Schwann cells and later by MΦ
  • After segmental demyelination, Schwann cells can proliferate and remyelinate
  • Example: Guillain-Barré Syndrome
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14
Q

Regenerative Processes

A
  • Axonal Sprouting
    • After axonal damage, the axon may sprout from the distal end
    • Regenerating axons re-grow at ~ 1 mm/day
  • Remyelination stimulated by denuded axon
    • Segmentally demyelinated axons may remyelinate ⇒ shorter internodes = slower conduction
    • Axonal sprouts will be re-myelinated by surviving Schwann cells
    • Remyelination may restore some function
    • Repeated episodes of demyelination and remyelination → accumulation of layers of Schwann cell processes
      • Onion Bulb: a single thinly myelinated axon surrounded by concentrically arranged Schwann Cells and redundant BM
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15
Q

Neurodiagnostic Testing

A

Electromyography (EMG) and Nerve conduction studies (NCS)

  • Most common diagnostic tests for dx of nerve and muscle disease after hx and exam
  • Both are complementary and are always performed together during the same setting
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16
Q

Nerve Conduction Studies (NCS)

A

Measure the ability of sensory and motor fibers to conduct an action potential

Used mainly for eval of paresthesias and/or weakness of arms and legs

  • Axonal injury/neuropathy
    • ↓ amplitude of AP
    • Mild slowing
    • Can see mix of both
  • Demyelination neuropathy
    • Conduction block (failure of AP to propogate)
    • Slow conduction
    • Amplitude of AP may be normal or reduced
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17
Q

Electromyography (EMG)

A

Measures muscle response or electrical activity in response to nerve stimulation of the muscle

Used to determine NMJ abnormalities, myopathy, and denervation

  • Increased insertion activity
    • Denervation ⇒ will fire spontaneously as a “Fibrillation potential”
    • Polymyositis
  • Decreased insertional activity
    • Myopathy ⇒ loss of response of motor units
      • Small motor units
      • Early recruitment of other motor units
      • Muscle is replaced by fat
  • Muscle fibers s/p denervation and reinnervated ⇒ very large due to reorganization and formation of large motor units
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18
Q

Peripheral Nerve Biopsy

A
  • Used when the pathology is in question and when therapy is contemplated
  • Superficial sensory branches of the sural or radial nerves are often sampled and examined pathologically
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19
Q

Peripheral Neuropathy Patterns

A

Patterns of peripheral neuropathy aids in clinical dx and finding underlying pathologic processes:

  • Mononeuropathy
  • Mononeuropathy Multiplex
  • Polyneuropathy
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20
Q

Focal Neuropathies

(Mononeuropathies)

A
  • Typically have damage to a single nerve
  • Etiologic agents are focal ⇒ process tends to be asymmetric
  • Classic cause is vasculitis ⇒ produces discrete infarctions in nerve trunks
  • NO distal-proximal gradient b/c an individual nerve trunk is affected
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21
Q

Median Nerve

A

Responsible for:

  • Flexion of the thumb, index, and middle fingers
  • Wrist flexion
  • Thumb opposition
  • Forearm protonation
  • Hand abduction
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22
Q

Median Nerve Entrapment

(Carpal Tunnel Syndrome)

A

Secondary to compression of the median nerve as it passes through the carpal tunnel

Most common nerve entrapment

  • Due to any condition that causes decreased space within the carpal tunnel
    • Tissue edema, pregnancy, inflammatory arthritis, hypothyroidism, amyloidosis acromegaly, DM, and excessive repetitive motions of the wrist
  • Pain primarily at the wrist, may radiate to forearm, arm, or shoulder
  • Sensory findings: paresthesia and sensory loss in the thumb, index and lateral half of ring fingers of affected hand
    • Often noted more at night
  • Motor findings: Weakness of thumb abduction and atrophy of the thenar eminence
  • Diagnostic signs:
    • Phalen’s Sign: elicited when wrist flexion produces paresthesias in the median nerve distribution
    • Tinel’s Sign: present when tapping over the median nerve at the wrist elicits paresthesias in the median nerve distribution
  • NCS: focal slowing or conduction block of median nerve fibers
  • Treatment: wrist splints in mild cases and surgical decompression in severe cases
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23
Q

Ulnar Nerve

A

Responsible for:

  • Wrist flexion
  • Flexion of 4th and 5th digits
  • Thumb adduction
  • Finger abduction and adduction
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24
Q

Ulnar Neuropathy

(Cubital Tunnel Syndrome)

A

Nerve entrapment due to compression of the ulnar nerve in the cubital tunnel at the elbow.

  • Sensory signs: numbness of the little finger and half of the ring finger
  • Motor signs: decreased grip and problems with finger dexterity
  • NCS: Slow conduction velocity at the elbow, or conduction block
  • Treatment: avoidance of behaviors involving leaning on elbows, elbow pads or surgical decompression in severe cases
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25
Radial Nerve
Responsible for: * Supination of forearm * Brachioradialis reflex * Extension of fingers, thumb, wrist, and elbow
26
Radial neuropathy “Saturday Night Palsy”
Results from **compression of the radial nerve** at the _radial/spiral groove._ * Classic case seen with sleeping with arm draped over a chair * Can also be seen with **humerus fracture** or other insult * _Sensory sx:_ **sensory loss over lateral dorsal hand** * _Motor sx:_ **wrist drop, finger drop, weakness of supination, mild elbow flexion weakness** * Treatment is **supportive** with generally spontaneous recovery
27
Peroneal (Fibular) Nerve
* **Deep peroneal nerve** * Ankle and toe dorsiflexion * Foot eversion * **Superficial peroneal nerve** * Sensory over anterior and lateral lower leg
28
Peroneal Neuropathy
Results from **compression of the peroneal nerve** as it _wraps around the fibular head._ * Often results from **leg crossing, prolonged bedrest, knees casts, fibular fractures** * Motor sx: **weakness of toe and foot dorsiflexion and foot eversion** * Sensory sx: **sensory loss over the dorsum of the foot and lateral distal lower extremity** * NCS: Slow conduction velocity at fibular head * Management is usually supportive with removing cause of compression
29
Lateral Femoral Cutaneous Nerve
Provides _sensation_ to the: * Anterior thigh * Knee * Upper part of lateral thigh
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Lateral Femoral Cutaneous Neuropathy | (Meralgia Paresthetica)
Results from **entrapment of the lateral femoral cutaneous nerve** as it _passes under the inguinal ligament._ * Causes include **tight clothing, obesity, and pregnancy** * Presents with **paresthesias in the lateral thigh** * NCS/EMG not helpful * Tx: supportive, meds
31
Mononeuritis Multiplex
* Less common focal neuropathy * **Painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy** * Involves **isolated damage to at least two separate nerve areas** * Multiple nerves can be affected * Can be confused with polyneuropathies * As condition worsens, becomes less multifocal and more symmetrical * Has variable etiologies including **vasculitis, DM, HIV, CMV**
32
Polyneuropathy Overview
Generalized process **affecting many peripheral nerves.** * Most common pattern of peripheral nerve disease * Usu. occurs as a side effect of medication or manifestation of systemic disease * Can be caused by both axonal degeneration and demyelinating disease * Affects longest nerves most severely * Presents first with **distal signs** in the arms and legs * Muscle weakness of the hands and feet * Loss of sensation in what is refer to as the "**glove and stocking" distribution** * Tends to be **symmetric**
33
Polyneuropathy Etiologies
* Usu. occurs as a side effect of medication or manifestation of systemic disease * **DM, ETOH, HIV, CMT** * Peripheral nerves susceptible to various factors ⇒ ∆ health & function ⇒ polyneuropathy * **Toxins** (acrylamide and hexacarbons) * **Chemotherapeutic agents** such as Vincristine and Isoniazid * **Inflammation** (Amyloidosis) * **Dietary** * **Metabolic** (B12, hypothyroid) * **Hereditary** * **Infection** (VZV, Leprosy) * **Parainfectious**
34
Polyneuropathy Clinical Manifestations
* **Length dependent sensory loss and weakness** * **Reflexes are decreased** * **Autonomic dysfunction** can occur in severe cases * Sx may be variable
35
Polyneuropathy Neurodiagnostic Testing
Abnormal studies provide a higher level of specificity Unclear etiology ⇒ NCS/EMG testing * **EMG w/ axonal neuropathy** * Systemic disorder, toxins, drugs * **EMG w/ demyelinating neuropathy** * Autoimmune or hereditary d/o * **EMG w/ mixed neuropathy** * Work-up same as demyelinating physiology * If not diagnostic, then evaluate for axonal causes
36
Diabetic Polyneuropathy
* **Most common cause of neuropathy** * Most common complication of DM * Involves **peripheral and autonomic nervous system** * Has a distal-proximal gradient * **Duration and severity of hyperglycemia** ⇒ crucial risk factor for development of diabetic neuropathy * **Microvascular injury to blood vessels that supply the nerve** * Prominent axonal degeneration w/ myelin debris, distal \> proximal * _Sx vary depending on areas affected:_ * Sensitive to touch * Pain * Anesthesia * Burning sensation * Muscle weakness or wasting * Ataxia * Treatment includes **better glycemic control, gabapentin, pregabalin, SSNRI**
37
Vitamin B12 Deficiency Neuropathy
* Vit B12 is an **essential nutrient** * **Deficiency is common in the US** * 10-25% of people \> 80 y/o may have Vit B12 deficiency * _Multiple etiologies:_ * Strict vegetarian diet * Inability of stomach acid to aid in absorption * Pernicious anemia * Ass. w/ i**mpaired erythropoiesis** and **nervous system demyelination** * Early dx is important * Treatment: ↑ Vit B12 in diet, oral or subcutaneous supplements if needed
38
Alcoholic Neuropathy
* ETOH abuse ⇒ high incidence of polyneuropathy * **Primarily an axonal neuropathy** * Complicated by **demyelination** when associated w/ **nutritional deficiency** * Predominant ↓in density of **small myelinated and unmyelinated fibers** * **Gradually progressive** * Clinically sx usually **symmetric and predominantly distal** * Sx include **numbness, paresthesias, burning pain, weakness, cramps and ataxia** * Specific treatment is not available * Pts should receive **thiamine** * Improved nutrition and cessation of drinking have been associated with sx improvement
39
Guillain-Barre Syndrome (GBS) ("Acute Inflammatory Demyelinating Polyneuropathy (AIDP)"
**Severe** **ascending, symmetric weakness** with **areflexia.** * Rapidly progressive **acute segmental demyelination** * **± P****receding illness** * Distal to proximal gradient * Begins with **distal paresthesias**, **pain in back/neck**, and **leg weakness** * ± Cranial nerve involvement * ± Secondary respiratory failure * ± Autonomic instability ⇒ arrythmia and BP changes * Progresses for up to **several weeks** * ↑ CSF protein but normal WBC count (albumnio-cytological dissociation) * NCS/EMG: slow conduction velocities or conduction block * Management: **Plasmapheresis** or **IVIG** ⇒ slows progression
40
Chronic Inflammatory Demyelinating Polyneuropathy
* _Immune mediated_ attack of **peripheral myelin** * **Slowly progressive**, **usually symmetric weakness and sensory loss** _over months to years_ * **Sensory loss to all modalities**, usually worse in BLE * **Generalized hyporeflexia** * ± Nerves enlargement * ↑ CSF protein * NCS/EMG: prolonged distal latencies, significantly slowed conduction velocities, conduction block * Treatment includes steroids, plasmapheresis, IVIG, and immunosuppressants
41
Demyelinating Neuropathies Other Etiologies
* Infectious mononucleosis (occasionally) * Toxic polyneuropathies * tri-(ortho) cresyl phosphate * [Ginger Jake (leg) Paralysis] * Reactions to immunization: Vaccination for the swine flu scare [1976]
42
Varicella-Zoster Virus
* **Infectious Polyneuropathy** * One of the most common viral infections of the PNS * **Latent infection of neurons in the sensory ganglia** of the spinal cord and brain stem follows **chickenpox** * **Reactivation** ⇒ painful, vesicular skin eruptions in the _distribution of sensory dermatomes_ (***shingles***) * Most frequently thoracic or trigeminal * Virus may be transported along the sensory nerves to the skin ⇒ **active infection of epidermal cells** * _Affected ganglia_ ⇒ **neuronal destruction**, **mononuclear infiltrates** * _Peripheral nerve_ ⇒ **axonal degeneration** after the **death of the sensory neurons**
43
Leprosy
* **Infectious polyneuropathy caused by** *Mycobacterium leprae* * _Lepromatous leprosy:_ * **Schwann cells** **invaded** by *M. leprae* → proliferates and infects other cells * **Segmental demyelination and remyelination** * _Tuberculoid leprosy:_ * CMI response to *M. leprae* → **granulomatous inflammation** in the dermis * Injures nearby cutaneous nerves → **localized nerve involvement**
44
Hereditary Neuropathies
Heterogeneous, usu. progressive, and often disabling syndromes that affect peripheral nerves.
45
Hereditary motor and sensory neuropathies (HMSN)
* Most common form of hereditary neuropathies * **Charcot-Marie-Tooth** disease and **Dejerine-Sottas** disease * Caused by mutations in genes involved in the **formation and maintenance of myelin**
46
Charcot Marie Tooth (CMT)
**Hereditary motor and sensory neuropathy** * _Clinical manifestations:_ * **Foot drop** * **Claw toe / hammer toes** * **Pes cavus** (high arch of foot) * Wasting of hands, feet, and distal leg muscles * Mild sensory loss * Enlarged nerves * Pathology: **demyelination and onion bulbs** * Multiple variants with different transmission patterns: **autosomal dominant, autosomal recessive, or X-linked**
47
Hereditary Sensory and Autonomic Neuropathies (HSAN)
Numbness, pain, and **autonomic dysfunction** → orthostatic hypotension
48
Familial Amyloid Polyneuropathies | (FAP)
* **Deposition of amyloid** within the PNS * Most types exhibit mutations of the ***transthyretin* gene** * Clinical presentation is similar to that of HSAN * Numbness, pain, and autonomic dysfunction
49
Traumatic Neuropathies
* Peripheral nerves commonly injured in the course of trauma * Lesions include **lacerations and avulsions** * **Re-growth** may be complicated by **discontinuity b/t proximal and distal portions of the nerve sheath** as well as by the **misalignment of individual fascicles** * Dysregulated/abnormal axonal growth ⇒ mass of tangled axonal processes called a **traumatic neuronal** (pseudoneuroma or amputation neuroma)
50
Other Etiologies of Polyneuropathy
* Long standing HIV * Critical Illness * Uremic polyneuropathy * Amyloidosis * Hypothyroidism * Vitamin Deficiencies * Lyme disease * Paraneoplastic * Heavy metal * Connective Tissue Disease (Sjogrens) *
51
Pressure Palsy
Nerve dysfunction seen after pressure is applied to nerve
52
Radiculopathy Overview
* **Due to damage to a nerve root** * **Disc herniation** is the most common cause * Characterized by **sensory complaints in a dermatomal distribution** and **weakness in the muscles innervated by the root** * If nerve root involved in a reflex, **reflex may be decreased** * Pain * NCS/EMG: sensory AP’s are normal, EMG w/ fibrillations in paraspinal muscles (acute) * Treatment: * Supportive - PT, pain management * Surgery
53
Cervical Radiculopathy
Presents with neck pain, arm pain, or both Sensory and/or motor deficits
54
C6 Radiculopathy
* C5-C6 disc herniation * Weakness of **shoulder abduction, elbow flexion, wrist flexion/extension** * Sensory loss of the **lateral forearm, thumb, and index finger** * **Loss of bicep and brachioradialis reflex**
55
C7 Radiculopathy
* C6-C7 disc herniation * **Most common cervical radiculopathy** * Weakness of **elbow and wrist extension** * Sensory disturbance of **middle finger** * Pain in neck and dorsum of forearm * **Loss of triceps reflex**
56
Lumbosacral Radiculopathy
* Pain syndrome caused by **compression or irritation of nerve roots in the lower back** * Caused by **lumbar or sacral disc herniation, DDD, and narrowing of foramen** where nerve exits * Results in **low back pain with radiation to the leg**
57
L2,3,4 Radiculopathy
* Marked overlap of L2, L3, L4 innervation * Radiculopathies considered as a group * Most commonly involved in _older pts_ with sx of **spinal stenosis** * **Acute back pain** **→** **anterior thigh** **→** **knee** **→** **medial lower leg** **→** **arch of the foot** * Weakness: **hip flexion, knee extension, hip adduction** * ↓ Sensation: **anterior thigh to medial lower leg**
58
L5 Radiculopathy
* **Most common radiculopathy affecting the lumbosacral spine** * **Back pain** **→** **lateral aspect of the leg** **→** **dorsum foot** * ↓ Strength in **foot dorsiflexion, toe extension, and foot eversion** * ± Weakness of **leg abduction** in severe cases * **Atrophy of the extensor digitorum brevis** of muscle of the foot and **tibialis anterior** of the lower leg * Reflexes are normal
59
S1 Radiculopathy
* **Pain** **→** **posterior aspect of the leg** **→** **foot from the back** * Weakness of **plantar flexion** * ± Weakness of **leg extension and knee flexion** * Sensation is reduced on the **posterior aspect of the leg** and **lateral edge of the foot** * **Loss of ankle reflex**
60
Plexopathy
* Disorder affecting a **network of nerves, blood vessels, or lymph vessels** * **Brachial or lumbosacral plexus** * Sx include pain, loss of motor control, atrophy and sensory deficits * The entire plexus, or just portions may be involved
61
Brachial Plexopathy
* Can be d/t compression, transection, ischemia, inflammation, metabolic abnormalities, neoplasia, radiation therapy, trauma, burns, postoperatively, autoimmune or post-infectious, or direct infiltration * **Muscle weakness, atrophy, sensory loss, and pain** * _Acute onset_ ⇒ pain in the shoulder and upper arm * _Insidious onset_ ⇒ progressive pain, evolving numbness, weakness of selected muscle groups
62
Lumbosacral Plexopathy Clinical Manifestations
Pattern of weakness/numbness helps localize lesion: * _Lumbar plexus:_ * Weakness of **hip flexion and adduction** and/or **knee extension** * Sensory disturbance in **anterior thigh and medial leg** * _Lumbosacral trunk and upper sacral plexus:_ * **Foot drop, flail foot, weakness of knee flexion or hip abduction** * Sensory disturbance in **leg, dorsum of foot, posterior thigh, perineum**
63
Lumbosacral Plexopathy
* Secondary to various causes including: DM, neoplasms, ischemia, post-op, trauma, surgery (OB), infection, CT disorder * Often results from **mass lesions** * Compression from pelvic tumors * Psoas abscess * Retroperitoneal hemorrhage * **Asymmetric, focal weakness, numbness, dysesthesia and/or paresthesia** in _multiple lumbosacral nerve root distributions_ * Pattern of weakness/numbness helps localize lesion * _Lumbar plexus:_ * Weakness of **hip flexion and adduction** and/or **knee extension** * Sensory disturbance in **anterior thigh and medial leg** * _Lumbosacral trunk and upper sacral plexus:_ * **Foot drop, flail foot, weakness of knee flexion or hip abduction** * Sensory disturbance in **leg, dorsum of foot, posterior thigh, perineum**
64
Motor Neuron Disease
_Neurodegenerative disorders_ that selectively affect **motor neurons of the spinal cord** * ALS is most common * Can have UMN + LMN signs or just LMN * Progressive
65
Amyotrophic Lateral Sclerosis | (ALS)
Neurodegenerative disease of the **upper and lower motor neurons.** * **Onset 55-75 years** * _UMN dysfunction_ ⇒ **increased tone, spasticity, hyperreflexia and positive Babinski** * _LMN dysfunction_ ⇒ **atrophy, weakness, fasciculation and loss of reflexes** * Extraocular muscles and sensation usually spared * Late in course, **spasticity** may occur * _Bulbar weakness_ ⇒ **dysarthria, dysphagia** * Pts present with **asymmetric distal weakness** * Usually weakness starts in one region then spreads to adjacent regions then spreads to contralateral limb * Pts often die with **respiratory insufficiency or aspiration pneumonia** * Management: **Supportive** (multidisciplinary clinics), **Riluzole** (may prolong survival)