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Introduction To The Peripheral Nervous System: - Mount Sinai Medical Center
Introduction To The Peripheral Nervous System: - Mount Sinai Medical Center
Brain
Spinal cord
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)
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!