Tues 10-20 Peripheral Nerve Disorders - A Practical Overview

Peripheral nerve disorders:
A practical overview
Praveen Dayalu, MD
Clinical Associate Professor
Department of Neurology
University of Michigan
What is the Peripheral Nervous System?
• CNS is confined to brain and spinal cord

• PNS includes (in anatomical order)
– Anterior horn cell (located within spinal cord)
– Spinal nerve roots (radicles)
– Plexi (brachial and lumbosacral)
– Named peripheral nerves (e.g. median, peroneal)
– Tiny nerve endings (sensory fibers and tiny branches
of lower motor axons at the neuromuscular junction)
– Neuromuscular junction and muscle
2
The PNS
(Emphasizing Motor Structures)
Brachial
Anterior horn
plexus
cells (LMN)
C8 spinal nerve root
Ulnar nerve
Neuromuscular junction and muscle

Many peripheral axons are myelinated
Myelin (Schwann cells)
4
Symptoms of PNS Disease
• Single focal lesions: weakness/numbness/ pain in one

limb, often defined to one part of the limb
• Multiple or diffuse lesions: weakness/ numbness/pain

in more than one limb, usually bilateral and distal
5
Signs of PNS Disease
• Lower motor neuron signs (atrophy, fasciculations)
• Hyporeflexia or areflexia
• Patch of sensory loss, or stocking-glove sensory loss
• Not UMN signs (spasticity, hyperreflexia, upgoing

toe) or “brain” signs (impaired consciousness,
cognition, or language)
6
Reflexes… repeated
• Hyperreflexia and spasticity occur with upper

motor neuron lesions (CNS)
• Hyporeflexia, fasciculations, atrophy with

lower motor neuron lesions (PNS)
7
Workup
• Serologies, especially for treatable causes
• EMG helps localize and characterize lesions of
PNS
• Imaging for some focal lesions, or to exclude CNS
mimics (such as cord lesion or stroke)
• CSF analysis in demyelinating neuropathies, or
polyradiculopathy
• Nerve biopsy
8
Anterior Horn Cell
9
Amyotrophic lateral sclerosis
• Anterior horn cells (lower motor neurons) and
upper motor neurons degenerate
• Mix of UMN and LMN signs/symptoms
• Weakness, spasticity, multifocal muscle atrophy
• No sensory loss from ALS!
• Loss of speech, swallow, respiration Death in
2-5 years
10
Radiculopathy
Brachial
plexus
Ulnar nerve

Spinal Nerve Root Disorders
• Most common: monoradiculopathy (cervical or
lumbosacral)
• Radiating pain, +/- weakness, +/- sensory loss.
Reduced reflex for that root level
• Commonest causes: disk herniation, minor
trauma, degenerative change
• Usually self-limited
• Image if severe, worsening, or concern for cancer,
infection
12
13
Plexopathy
Brachial
plexus
Ulnar nerve

Plexopathy
• PNS syndrome in one limb not explained by a
single spinal root, or by a single “named”
peripheral nerve
• Causes: trauma or stretch, compression by
tumor or hematoma, radiation, diabetic
• EMG confirms plexopathy
• Image if compressive lesion suspected
15
Brachial Plexus
behind clavicle, in upper thorax
16
Lumbosacral
Plexus: Pelvic,
Retroperitoneal
17
Mononeuropathy
Brachial
plexus
Ulnar nerve

Mononeuropathy
• Weakness, numbness, pain, paresthesias

confined to the distribution of
– UE: median nerve, radial n., ulnar n.
– LE: femoral n., sciatic n., peroneal n.
• Most common causes: entrapment, trauma,

prolonged limb immobility (e.g., surgery)
19
Important Mononeuropathies
• Median mononeuropathy at the wrist (carpal tunnel

syndrome)
• Ulnar mononeuropathy at the elbow (cubital tunnel
syndrome)
• Radial mononeuropathy (“Saturday night palsy”)
wrist and finger drop
• Peroneal mononeuropathy (e.g., from leg
crossing) one cause of footdrop
20
Named peripheral nerves have well-defined sensory
territories (and muscle targets)
Ulnar n.
Median n.
Peroneal n.
21
Peripheral Polyneuropathy
23
“Peripheral Neuropathy”
• Distal symmetric polyneuropathy
• Affects longest sensory/ motor/ autonomic
nerves
• Nerves are “dying back”
• Length dependent (“stocking glove”)
• Symmetric loss of pin/ temp / vibration/
proprioception; distal reflex loss
• Usually chronic. Many possible causes!
24
Peripheral polyneuropathy symptoms
• Initially, feet numb with paresthesia/ pain
• Symptoms ascend:  legs fingertips
• Distal weakness (feet, or fingers/grip), atrophy,
• Severe sensory loss can cause “steppage gait”,
“sensory ataxia”, imbalance, falls
• Feet prone to injuries, ulcers, deformation
(e.g., “Charcot foot”)
• Autonomic: orthostasis, bladder and erectile
dysfunction
25
Causes of peripheral polyneuropathy
• Usually toxic or metabolic
• #1 cause: diabetes & impaired glucose tolerance
• B12
• Hematologic (e.g., multiple myeloma) or other
immunoglobulin disorders (check SPEP)
• Drugs: Li, chemotherapy
• Alcoholic neuropathy
• Liver or kidney disease
• HIV and neurosyphilis
• Inflammatory causes: connective tissue disease
26
Workup for peripheral neuropathy?
• For typical distal symmetric sensory > motor

neuropathy: glucose / a1c, B12, SPEP with ifix
• Need EMG and more if rapid or severe,

prominent weakness, asymmetry, young
patient
AIDP = Acute Inflammatory Demyelinating
Polyneuropathy
• Develops over ~2 weeks

• Follows viral infection or vaccination
• Weakness/ numbness/ paresthesia start in legs and
ascend; reflexes lost early
• Intubate if severe weakness
• Autonomic instability, arrhythmia
• Rx: plasma exchange & IVIG
• Most have good long-term recovery
28
Myopathy = muscle
disease
• “Can’t raise arms up”

• “Tough to comb my hair”
• “Hard to get out of a chair”
• “Can’t climb stairs”
29
Clues to a Myopathy
• Usually cause symmetric proximal limb

weakness (upper/lower)
• Other possible sx: muscle pain, tenderness,

atrophy; fatigue; change in urine color
• Extensive list of causes: TSH, CK, and med

review are a good place to start
30
Time for review questions?
Over 3 days, a 42 y.o. RN rapidly weakens in all four
limbs. She says “I think I have Guillain-Barré!”
Which is the most urgent first step?
A. EMG
B. Spinal cord imaging
C. Lumbar puncture
D. Brain imaging
32
A 61 y/o woman wakes up with a weak right arm. Zero strength in finger extensors, wrist extensors, and trace elbow extension.
Flexion muscles are 5/5.
Which is the most likely diagnosis?
A. Left frontal stroke affecting motor cortex

B. Carpal tunnel syndrome
C. Right radial mononeuropathy
D. Peripheral polyneuropathy
33
Decreased reflexes are seen in all of the following except:
A. AIDP
B. Peripheral polyneuropathy
C. Carcinomatous polyradiculopathy
D. Alcoholic neuropathy
E. Steroid myopathy
34
Weakness and sensory loss in the left hand can be caused by all of
the following except:
A. Right hemisphere stroke

B. Lung cancer invading brachial plexus
C. Median mononeuropathy
D. Amyotrophic lateral sclerosis
E. Cervical herniated disk
Which is the commonest symptom of radiculopathy
in the general public?
A. Weakness
B. Radiating pain down a limb
C. A patch of distinct sensory loss
D. Bladder incontinence
36

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Peripheral nerve disorders:

• CNS is confined to brain and spinal cord

C8 spinal nerve root

Neuromuscular junction and muscle

Myelin (Schwann cells)

• Single focal lesions: weakness/numbness/ pain in one

• Multiple or diffuse lesions: weakness/ numbness/pain

• Not UMN signs (spasticity, hyperreflexia, upgoing

• Hyperreflexia and spasticity occur with upper

• Hyporeflexia, fasciculations, atrophy with

C8 spinal nerve root

Neuromuscular junction and muscle

C8 spinal nerve root

Neuromuscular junction and muscle

C8 spinal nerve root

Neuromuscular junction and muscle

• Weakness, numbness, pain, paresthesias

• Most common causes: entrapment, trauma,

• Median mononeuropathy at the wrist (carpal tunnel

• For typical distal symmetric sensory > motor

• Need EMG and more if rapid or severe,

• Develops over ~2 weeks

• “Can’t raise arms up”

• Usually cause symmetric proximal limb

• Other possible sx: muscle pain, tenderness,

• Extensive list of causes: TSH, CK, and med

Which is the most urgent first step?

Which is the most likely diagnosis?

A. Left frontal stroke affecting motor cortex

A. Right hemisphere stroke

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