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Introduction to the

Peripheral Nervous System


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i ng V
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Stephen Krieger, M.D. and


Jessica Robinson-Papp, M.D.
Mount Sinai Medical Center
Goals
 Review the anatomy of the peripheral
nervous system (PNS)
 Outline an approach to neuromuscular
symptoms
 Describe disorders affecting the PNS
 Evaluate real-life cases
Central Nervous System

 Brain
 Spinal cord

…and that’s enough about that


PNS Anatomy
Throughout the nervous system:
Anterior is Motor/Action,
Posterior is Sensory/Perception

Same holds true for the generation of the PNS:


Sensory
 Dorsal root
 Dorsal root
ganglion
Spinal
nerve
Motor
 Anterior horn
cell
 Ventral root
PNS Anatomy
Spinal
nerve

Nerve Plexus
Peripheral
Nerve
PNS Anatomy
Peripheral Nerve
Sensory Motor

NMJ

Muscle
What can go wrong in the PNS?
 Anterior horn cell  Motor neuron disease
 Nerve root  Radiculopathy
 Nerve plexus  Plexopathy
 Nerve  Neuropathy
– Mononeuropathy
– Mononeuropathy multiplex
– Polyneuropathy
 NMJ  Myasthenia gravis, Lambert-Eaton
 Muscle  Myopathy
Localizing principles
 Clues from the history
– Localization of pain: the Big Red Arrow
– Radiation
 Pure motor or sensorimotor?
– Pure motor: MND, NMJ, myopathy
 Distribution – Before asking ‘Where’s the lesion’?
– Focal
– Multifocal
– Diffuse
Localizing principles
 Focal
– Mononeuropathy
– Plexopathy
– Radiculopathy
– MND
 Multi-focal
– Mononeuropathy multiplex
– MND
 Diffuse
– Distal  polyneuropathy
– Proximal  myopathy, NMJ (…or is this actually a focal problem?)
Motor neuron disease
 Degenerative disorder of upper and lower
motor neurons which spares sensory
neurons
 Typical age @ onset 55-60
 LMN: atrophy, weakness, fasciculations,
and cramps
 UMN: stiffness, spasticity, hyperreflexia,
pathologic reflexes (e.g. Babinski signs)
Motor neuron disease

Both UMN/LMN signs….Think about where the “Anterior Horn Cell” is.
Mononeuropathy
 Sensory and motor disturbances confined to the
distribution of a single nerve
 Usually the result of entrapment or compression
@ an anatomically vulnerable site:
– There’s a very short list.
 Pain or tenderness @ compression site
 Most common mononeuropathies:
– Median @ wrist (carpal tunnel syndrome)
– Ulnar @ elbow (cubital tunnel syndrome)
– Radial @ spiral groove (Saturday night palsy)
– Peroneal @ fibular head
Carpal tunnel syndrome
 Early: symptoms and
signs predominantly
sensory
– Pain and parasthesias
of wrist and lateral
hand
– May radiate up arm
– Numbness lateral
fingers
– Wakes up @ night
– Shakes out hands Median
 Late: weakness and
wasting of thenar
muscles
Ulnar neuropathy @ the elbow
 Insidious onset of hand
weakness.
 Sensory symptoms in the
medial hand and pinky – like
a chronic “funny bone”
injury.
 Pain in elbow region, may
radiate down forearm to
wrist.

Ulnar
Radial neuropathy @ spiral groove
 “Saturday night palsy”
 Nerve lies close to
humerus and is
vulnerable to
compression.
 Typical history of
falling asleep (+/-
intoxication) with arm
draped over
something.
 Wrist and finger drop
with sparing of triceps
 Sensory disturbance on
dorsum of hand
Radial: not a lot of sensory loss
Peroneal neuropathy @ the fibular
head
 H/o leg crossing
 Presents with
foot drop and
numbness over
lateral calf and
dorsum of foot

 Basically, the
“Ulnar of the
leg”
Brachial plexopathy
 Relatively
uncommon
– Trauma
 Weakness and
numbness in
arm involving
multiple nerve
distributions
 May be painful
following viral
illness
– “Parsonnage-
Turner”
Radiculopathy
 Common
 Sensory and motor disturbances confined to the
distribution of a nerve root, or a few adjacent nerve
roots
 Usually the result of discs (younger patients) or
degenerative changes (older patients)

 Cervical radiculopathy: neck pain radiating to arm:


– Cervical region has mobility in all planes
 LS radiculopathy: low back pain radiating to leg
– Lumbosacral region carries the weight of everything above
Radiculopathy: the dermatomes
C5-6 Radiculopathies
 Motor
– Shoulder abduction
– Elbow flexion

Note: Dermatomes go
down, around, and back
 Sensory up, causing a midline split
between upper and lower
roots.
C7 Radiculopathy
 Motor
– Elbow extension
– Wrist extension
– Finger extension

Note: Distinguish
 Sensory Between a C7 radic
and a Radial nerve
palsy in part by the
area of sensory loss
involved.
C8-T1 Radiculopathy
 Motor
– Finger flexion
– Finger separation

 Sensory
Common lumbar root syndromes
 L3-4
– Pain: radiating from groin
to anterior thigh
– Sensory: Anterior medial
thigh, medial calf, medial
foot
– Weakness: Illiopsoas,
adductors, quads
– DTR: Knee
Common lumbar root syndromes
 L5-S1
– Pain: Radiating from
buttock down
posterolateral thigh and
calf
– Sensory: Foot and lateral
calf
– Weakness: Foot
movements, DTR: None
– DTR: Ankle
Polyneuropathy
Axonal

Demyelinating
Axonal polyneuropathy
 Most common type by far
 Symmetric sensory loss and weakness, first in
feet then spreading proximally
– Imagine dipping a person into a vat of glucose…

 Usually chronic
 Extensive differential diagnosis
Axonal polyneuropathy: etiologies
 Systemic diseases
– Endocrine: diabetes, thyroid
– Renal failure
– Liver disease
– Autoimmune: SLE, Sjogren’s, Sarcoid, RA
– Neoplastic
– Nutritional
 Toxins
– EtOH
– Drug-induced
– Industrial toxins
– Heavy metals
 Infectious
– HIV, lyme
 Hereditary
 Idiopathic
Demyelinating polyneuropathy
 Rarer
 Limited differential
 Acute inflammatory demyelinating
polyneuropathy (AIDP) aka: Guillan-Barre
syndrome  fulminant presentation of
rapidly progressive weakness
 Chronic:
– CIDP
– Hereditary
Myasthenia Gravis
 Typically presents in young women and
old men
 Caused by autoimmune attack on Ach
receptor in the neuromuscular junction
 Weakness of extraocular, bulbar, and
proximal limb muscles
 Fatigability  weakness worse with
progressive exercise
Myasthenic Syndrome
 Lambert-Eaton
 Unlike Myasthenia Gravis, this is a
paraneoplastic syndrome
 Like MG, Weakness of extraocular, bulbar,
and proximal limb muscles
 Unlike MG, there is the opposite of
fatigue: there is Increment. This is
important for recognizing there may be
underlying Cancer.
Myopathy
 Usually symmetric, proximal weakness
 May have associated cramps, pain, exercise intolerance
 Ddx:
– Inflammatory
– Endocrine associated
– Drug-induced/toxic
– Metabolic
– Congenital
– Muscular dystrophies
– Myopathy associated with periodic paralysis
Diagnostic studies
 Neuroimaging
– MRI
 Radiculopathies
 Plexopathies
– Blood work
 R/o systemic disease in polyneuropathy
 CK in myopathy
– LP
 Increased protein in inflammatory neuropathy
– NCS/EMG
EMG/NCS: Elements of the Study
 Nerve Condution Studies
– Motor
– Sensory
– Late Responses
 Needle EMG
 Special Studies
– Repetitive Nerve Stimulation
– Single Fiber EMG
Motor NCS: Median motor study,
distal stimulation
Sensory NCS: Median study
Summary
 Elements of the PNS include: anterior horn
cell, nerve root, plexus, nerve,
neuromuscular junction, and muscle
 When neuromuscular disease is
suspected, localization is essential to
developing a differential diagnosis
Summary
 Ask yourself:
– Is there pain? What is the distribution of the
pain?
– Is it pure motor or sensorimotor?
– Is it focal, multifocal, or diffuse?
– Is it symmetric or asymmetric?
– Is it proximal or distal?
Thanks!

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