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UPPER LIMB

NEUROPATHIES
1. SPINAL ACCESSORY NEUROPATHY

Definition
• Dysfunction of the spinal accessory nerve (cranial nerve XI)
supplying the sternocleidomastoid and trapezius muscles.

Etiology
• Common: surgical procedures in the posterior triangle (lymph
node biopsy or dissection).

Examination

Shoulder shrug (trapezius), tilting head towards ipsilateral


shoulder and rotating head towards contralateral shoulder
(sternocleidomastoid).
Clinical features
• Drooping of the ipsilateral shoulder and lateral winging of the
scapula.
• Winging accentuated by shoulder abduction to 90°.
• Most lesions are distal to sternocleidomastoid innervation,
affecting only the trapezius.
Investigations
• NCS of the spinal accessory nerve recording off of the
trapezius and comparing the CMAP (compound muscle action
potential) from both sides.
• EMG of the trapezius and sternocleidomastoid
Prognosis
Recovery depends on degree of nerve injury.
2. LONG THORACIC NEUROPATHY

Definition and epidemiology


• Dysfunction of the long thoracic nerve to the serratus
anterior.
• Uncommon.
Etiology
• Trauma.
• Surgery to the chest wall: radical mastectomy, axillary node
dissection, thoracostomy.
• IBPN (inherited brachial plexus injury) [rare]: often
involves other nerves in addition to the long thoracic nerve.
Examination
Inspect for scapular winging (748), which may be present in the
resting position.
• Ask the patient to push against a wall with both arms slightly
flexed at the elbow. Look for winging of the scapula on the
affected side, which indicates weakness of the serratus anterior.
• If weakness is so severe, the patient may not be able to flex the
extended arm at the shoulder and will require assistance from the
examiner. Ask the patient to push their fist forward and watch for
winging.
• Elevation of the arm may not be possible. This can be achieved if
the examiner presses the patient’s scapula against the chest wall.
Clinical features
• Difficulty with elevating the upper arm during activities such as
shaving, combing hair, eating, and drinking.
• Dull shoulder ache, mainly because of strain on the shoulder
muscles and ligaments in the absence of the serratus anterior
muscle tightening the scapula against the rib cage.

Investigations
• NCS: record from the serratus anterior and compare bilateral
CMAPs.
• EMG: abnormalities noted in the serratus anterior.
Differential diagnosis
• C6 or C7 radiculopathy, but usually there is additional weakness of the
extensors of the arms, wrists, or fingers.
• Myopathy: weakness is usually bilateral and involves additional muscles of
the shoulder and upper arm.
• Distinguish scapular winging from that of spinal accessory neuropathy and
dorsal scapular neuropathy.

Treatment and prognosis


• Physical therapy.
• If shoulder function does not improve, surgery to stabilize the scapula is an
option.
• Most recover spontaneously.
3. SUPRASCAPULAR NEUROPATHY
Definition and epidemiology
• Dysfunction of the suprascapular nerve.
• Uncommon.
Etiology
• Trauma to shoulder region:
• Stab wounds above the scapula.
• Improper use of crutches.
• Stretching of the nerve that may occur with serving a
volleyball or pitching a baseball.
Examination
• Test the supraspinatus muscle with the patient abducting the
upper arm from resting position against resistance.
• To test the infraspinatus muscle, ask the patient to flex the
elbow to 90°. The examiner should stabilize the patient’s elbow
against the trunk and ask the patient to rotate the upper arm
externally against resistance on the dorsum of the patient’s hand.
Clinical features
• May have pain at the superior margin of the scapula radiating
towards the shoulder.
• Atrophy of the supraspinatus and infraspinatus muscles
Investigations
• Motor NCS are technically limiting
• EMG is more helpful:
• Denervation in both supraspinatus and infraspinatus muscles if the
lesion is proximal to the suprascapular notch.
• Denervation is limited to the infraspinatus muscle if the lesion
occurs at the spinoglenoid notch.
Treatment
• Conservative therapy with pain control is recommended.
• Corticosteroid injections to the sites of compression.
• Surgical decompression of the entrapment sites is controversial
4. AXILLARY NEUROPATHY
Definition and epidemiology
• Dysfunction of the axillary nerve.
• Uncommon.

Etiology
• Trauma:
• Fracture or dislocation of the head of the humerus.
• Hyperextension of the shoulder (e.g. during sleep or surgery).
• Intramuscular injections into the deltoid.
Examination
• Ask the patient to keep the arm abducted in the horizontal
plane against resistance. The supraspinatus muscle initiates the
first 30° of arm abduction.
• The teres minor muscle cannot be examined in isolation
because it acts together with the infraspinatus muscle in external
rotation of the upper arm.
• Loss of sensation in a small area of skin overlying the deltoid
muscle (750).
Clinical features
• Atrophy of the deltoid muscle.
• Prominence of the acromion and head of the humerus (due to
deltoid wasting).
Investigations
NCS:
• Axillary CMAPs recorded from the deltoid muscle with
supraclavicular stimulation of the brachial plexus.
• Bilateral comparison to identify asymmetric loss of
amplitude on the affected side.
EMG: denervation in deltoid and teres minor muscles (although
teres minor is difficult to localize for testing).
Treatment and prognosis
• Conservative treatment with physical therapy and
occupational therapy, primarily to prevent a frozen shoulder
(the elderly are particularly vulnerable).
• If there is no improvement within 6 months, surgical
treatment and nerve grafting should be considered.
• Axillary neuropathy due to penetrating injury should be
surgically explored.
• Partial lesions tend to recover ­spontaneously. Otherwise,
recovery occurs very slowly over many m
5. MUSCULOCUTANEOUS NEUROPATHY
Definition and epidemiology
• Dysfunction of the musculocutaneous nerve. • Rare in isolation
Etiology
Trauma:
• Fractures or dislocations of the shoulder.
• Clavicle fracture.
• Axillary node dissection.
• Strenuous exercise of the arm (e.g. heavy weight training, repetitive
push-ups) resulting in hypertrophy of the biceps muscle compressing
the nerve.
Examination
• With the forearm in full supination, ask the patient to flex the
elbow against resistance to test the biceps and brachialis muscles.
• Coracobrachialis weakness results in difficulty with arm
elevation.

Clinical features
• Numbness or paresthesias of the lateral forearm.
• May have pain in the elbow or forearm.
• Absent biceps stretch reflex.
• Weakness of elbow flexion with the forearm supinated.
Differential diagnosis
• Nonneurogenic: ruptured biceps tendon, but no sensory loss
and on contraction of the biceps muscle, a hardening mass
evolves under the insertion of the pectoralis major muscle.
• Neurogenic: C6 radiculopathy, although this is usually
accompanied by sensory loss in the hand and weakness of
other C6-innervated muscles
Investigations
NCS
• Musculocutaneous CMAP can be obtained with recording from
the biceps muscle and stimulating the brachial plexus in the
supraclavicular fossa. Comparison of both sides is necessary.
EMG
• Denervation in the biceps, brachialis, and coracobrachialis
muscles.
Treatment
• Most cases are treated conservatively.
• Nerve injury from severe trauma may require surgical treatment
RADIAL NEUROPATHY

Definition and epidemiology


• Dysfunction of the radial nerve.
• Reasonably common

Etiology
Axillary lesions
• Rare in isolation.
• Compression from crutches, but usually involves the median and ulnar nerves as
wellUpper arm lesions
• Fracture of the humerus.
• External compression against the spiral groove:
• Falling asleep after intoxication, with the arm folded over the back of a chair (Saturday
night palsy).
• Improper positioning during general anesthesia.
• Stretch injury due to hyperabduction of the arm.
• HNPP.
• Traumatic aneurysm of the radial artery.
• Soft-tissue or peripheral nerve tumor.
• IBPN.
• Ischemia (i.e. vasculitis).
• Multifocal motor neuropathy.
Forearm lesions (posterior interosseous neuropathy)
• IBPN.
• Compression by tumors, ganglion cysts, lipoma.
• Compression by the arcade of Frohse (753).
• Dislocation of the elbow.
• Fracture of the ulna with dislocation of the radial head.
• Rheumatoid arthritis of the elbow joint.
• Arteriovenous fistula for dialysis.
• Congenital hemihypertrophy of the supinator muscle.
• Accessory brachioradialis muscle.
• Soft-tissue or peripheral nerve tumor.
• Ischemia (e.g. vasculitis).
• Multifocal motor neuropathy.
Wrist lesions (superficial radial neuropathy)
• External compression (e.g. handcuffs, tight watch bands).
• De Quervain tenosynovitis.
• Soft-tissue or peripheral nerve tumor.
• Transposition of a flexor tendon towards the thumb.
Examination
Axillary lesions
• Weakness of the triceps and all muscles extending the wrist,
fingers, and thumb.
• Decreased sensation on the back of the upper arm and
forearm, in the web between the index finger and the thumb,
and lateral dorsum of the hand.
Upper arm lesions
• Hand and finger drop due to weakness of wrist extensors
and metacarpophalangeal joints.
• Sparing of the triceps muscle and sensation in the upper
arm.
• May have decreased sensation in the posterior aspect of the
forearm and lateral dorsum of the hand.
• Weakness of brachioradialis and supinator.
Forearm lesions (posterior interosseous
neuropathy)
• Dropped fingers without dropped hand.
• Despite severe weakness of the extensor carpi
ulnaris, wrist extension is possible because the
extensor carpi radialis functions normally (the
branch to the extensor carpi radialis leaves the
radial nerve above the elbow and proximal to
innervation of the supinator muscle).
• If extensor carpi ulnaris is weak, a distinct
lateral deviation of the extended hand occurs
when the patient tries to make a fist.
• The examiner should be careful to check
interossei (ulnar nerve) muscle strength
appropriately. The interossei can be assessed
with the fingers supported on a flat surface.
Wrist lesions (superficial radial neuropathy)
Reduced sensation over the lateral dorsum of
the hand, the dorsum of the thumb (except the
nail area), the index finger (proximal to the mid
Clinical features
Axillary lesions
• Pain is not prominent.
Upper arm lesions
• Sudden onset of inability to extend wrist, fingers, and thumb and
numbness/paresthesia of the lateral forearm.
• May have pain in the elbow or forearm.
Forearm lesions
• Slowly progressive onset of symptoms.
• Initially, the little finger gets curled up during tasks such as retrieving
something from a trouser pocket.
• Later, inability to extend the metacarpophalangeal joint of the little finger and
then similar weakness begins in other fingers, one after the other.
• May have difficulty playing the piano, but writing and grip strength remain
normal.
• Pain is uncommon.
Wrist lesions
• Shooting pain in the lateral side of the wrist.
• Painful paresthesias in the thumb and index finger evoked by palpating the
lateral side of the wrist.
• Reduced sensation on the lateral side of the hand
Investigations
NCS • Decreased or absent superficial radial SNAP. • Radial nerve
CMAP: • Recorded from the extensor indicis with stimulation at
various locations along the nerve. • Important to stimulate below and
above the spiral groove to assess for conduction block or conduction
velocity slowing across this site, indicating compression of the nerve
at the spiral groove. EMG • Localize the site and severity of a radial
nerve lesion. • For example, denervation of the extensor carpi ulnaris
and not the extensor carpi radialis is consistent with posterior
interosseous neuropathy.
Treatment • Surgical exploration is recommended for mass lesions (i.e.
tumor, lipoma, aneurysm of the radial artery) and penetrating trauma
with severe axonal injury. • Closed trauma injury, including humerus
fracture, usually recovers spontaneously. Thus, conservative therapy is
tried prior to surgery. • Conservative therapy: finger and wrist splints,
pain control, physical therapy, occupational therapy. • Posterior
interosseous neuropathy: • Surgery is recommended if a posterior
interosseous neuropathy is related to open trauma. If not, itshould be
managed conservatively. • Decompressive surgery is controversial,
with rare cases improving with surgery.
MEDIAN NEUROPATHY
Definition and epidemiology
• Dysfunction of the median nerve.
• Common
Etiology
Proximal lesions • Compression in the axilla (improper use of crutches). •
Trauma: shoulder dislocation, humerus fracture, tourniquet paralysis. •
Compression by ligament of Struthers. • Pronator teres syndrome:
controversial syndrome as there is usually no objective evidence of
weakness in median-innervated muscles. This is caused by a thickened
lacertus fibrosum, fibrous arch of the flexor digitorum superficialis, or
tendonous band or hypertrophied pronator teres muscle. • Ischemia (e.g.
vasculitis). • IBPN. • Soft-tissue or peripheral nerve tumor. • Multifocal
motor neuropathy
Anterior interosseous syndrome
• IBPN.
• Compression by a fibrous band between the deep head of the
pronator teres muscle and the flexor digitorum superficialis. •
Compartment syndrome. • Ischemia (e.g. vasculitis). • Soft-tissue or
peripheral nerve tumor. • Multifocal motor neuropathy.
Wrist lesions (i.e. carpal tunnel syndrome)
• Idiopathic (45% of cases). • Occurring in the setting of a
polyneuropathy (e.g. diabetic). • Obesity. • Pregnancy. • Anatomic
predisposition: limited longitudinal sliding of the median nerve
under the transverse carpal ligament, a smaller cross-sectional area
of the tunnel. • Degenerative joint disease or rheumatoid arthritis. •
Sarcoidosis or amyloidosis. • Endocrinopathies (i.e. hypothyroidism,
acromegaly, diabetes). • Structural lesions (i.e. ganglion cysts,
lipomas, hemangiomas, osteomas). • Trauma: fracture of carpal
bones, repetitive movement in the workplace
Carpal tunnel syndrome •
Paresthesias/numbness involving the palmar
surface of the hand (particularly thumb, index
finger, middle finger, ring finger) and may
extend into the forearm and arm. • Pain
frequently wakes the patient from sleep. • Pain
is relieved by rapid shaking of the hands (the
‘flick’ sign). This may help distinguish from
the pain of arthritis and soft-tissue injuries,
which may be exacerbated by this movement. •
Median nerve provocative tests (which are
often negative): • Tinel’s sign: percussing over
the flexor retinaculum of the carpal tunnel
causes paresthesias in the median nerve
territory. • Phalen’s sign: forced flexion of the
wrist for 60 seconds produces paresthesias in
the median nerve territory. • If severe axonal
damage, atrophy of the abductor pollicis brevis
may create a ‘scalloped’ appearance to the
thenar eminence (759).
Investigations NCS Proximal lesions • Median
SNAP and CMAP (usually recorded from the
abductor pollicis brevis) have reduced
amplitude depending on the amount of axon
loss. • Distal latency or conduction velocity of
the median SNAP is normal or slightly
prolonged compared with the loss of amplitude.
• Important to evaluate for conduction velocity
slowing, temporal dispersion, or focal
conduction block within the upper arm or
forearm (i.e. MMN)
Carpal tunnel syndrome
• 10% of cases with histories highly suggestive of carpal
tunnel syndrome will have normal NCS.
• Perform studies on median SNAP and CMAP. Include
other upper extremity nerves to exclude a more diffuse
process such as polyneuropathy.
• Median SNAP is more sensitive than CMAP in detecting
carpal tunnel syndrome abnormalities. CMAP amplitude is
usually affected much later in the course (as axon loss
progresses).
• Earliest abnormality: prolonged distal latencies or slowing
of the median SNAP.
• Compare median SNAP distal latency/conduction velocity
following wrist stimulation, with recordings following
palmar stimulation. This assesses for more focal slowing or
conduction block across the wrist and is valuable in those
who have polyneuropathy to check for a superimposed
carpal tunnel syndrome
EMG • Denervation noted in median-innervated muscles. Performed
to assist in further lesion localization. Treatment and prognosis •
Nonsurgical therapy: • 20–70% improve to some degree. • Wrist
splints (particularly while sleeping). • Corticosteroid injections into
the carpal tunnel. • Surgical decompression: division of the
transverse carpal ligament: • Rationale: to create an environment
under which the nerve can recover and the symptoms resolve; it does
not aim to improve nerve function itself. The capacity of the nerve to
recover also depends on patient age, coexisting disease, and severity
of the deficit. • Usually performed after a trial of conservative
therapy. • 75% success rate with about 8% worsening. • 50%
success rate in those with marked thenar atrophy, absent responses
on NCS, or denervation on EMG. In these cases, surgery can be
considered for pain relief rather than improved strength or sensation.
• Poor prognosis if there is significant axonal degeneration,
particularly with proximal lesions, due to the long distance the nerve
must grow to completely reinnervate. • Carpal tunnel syndrome has
the best prognosis if there are minimal electrodiagnostic
abnormalities and no active denervation on EMG, and conservative
therapy is initiated within 3 months
ULNAR NEUROPATHY
Definition and epidemiology
• Dysfunction of the ulnar nerve. • Reasonably
common

Etiology Proximal lesions (axilla to upper elbow) • Trauma:


improper crutches, tourniquet paralysis. • Compression during sleep.
• Soft-tissue or peripheral nerve tumor. • Ischemia (e.g. vasculitis). •
Multifocal motor neuropathy. Elbow lesions • External pressure –
compression at the ulnar groove: • Resting the elbow against a hard
surface. • Prolonged bed rest. • Malpositioning during general
anesthesia. • HNPP. • Polyneuropathy (e.g. diabetic): possibly more
susceptible to neuropathy at compression site. • Deformities of the
elbow joint: • Tardy ulnar palsy: deformities of the elbow due to
previous fractures of the humerus or other trauma to the joint. •
Compression by the arcade of Struthers. • Arthritis. • Ganglion cyst.
• Rheumatoid synovial cyst. Wrist and hand lesions • External
compression (e.g. bicyclist, walking cane). • Structural lesion (i.e.
ganglion cyst, lipoma, nerve sheath tumor). • Osteoarthritis and
rheumatoid arthritis
Examination Sensation • Decreased sensation of the little
finger and medial side of the ring finger. • Extent of sensory
changes depends on level of the lesion (760). • Sensory
abnormalities should be distal to the wrist and not extend
into the forearm.
Weakness • Proximal lesions have the same pattern of
weakness as compressive lesions at the elbow. • Early stages
of proximal lesions show weakness and wasting of the
hypothenar eminence and first dorsal interosseous. Flexor
carpi ulnaris and flexor digitorum profundus are rarely weak
or wasted initially. • To test flexor digitorum profundus III
and IV, fix the middle phalanx of the ring finger and little
finger and ask the patient to flex the distal interphalangeal
joint against resistance. • To test adductor pollicis, ask the
patient to squeeze a piece of paper between the base of the
thumb and the index finger. If the adductor pollicis is weak,
the interphalangeal joint of the thumb flexes due to the use
of the median-innervated flexor pollicis longus to hold onto
the paper (Froment’s sign).
Clinical features • Flexor carpi ulnaris often escapes
compression at the elbow, but if not, there may be a lateral
deviation of the hand on wrist flexion. Wrist flexion is
generally not affected due to an intact flexor carpi radialis
(median-innervated). • Severe ulnar neuropathy gives rise to
the ‘ulnar claw hand’ with guttering of the dorsum of the hand
from atrophy of the interosseous muscles and the third and
fourth lumbricals, hyperextension of the fourth and fifth
metacarpophalangeal joints, mild flexion of the
interphalangeal joints, and abduction of the little finger.
Examination Sensation • Decreased sensation of the little
finger and medial side of the ring finger. • Extent of sensory
changes depends on level of the lesion (760). • Sensory
abnormalities should be distal to the wrist and not extend into
the forearm. cactus 01375 401 387 (Katy) Title: Gorelick:
Hankey Clinical Neurology ISBN: Proof Stage: 1 Fig No:
21B.1
Investigations
NCS
EMG

Treatment • Prevention: adequate support of the


arms of bedridden patients and during
surgeries. • Nonsurgical therapy: • Elbow pads,
particularly while sleeping. • Splinting the
elbow in extension at night. • Avoidance of
leaning on the elbows. • Surgical approach: •
Options include simple decompression, medial
epicondylectomy, and nerve transposition. •
Appropriate candidates have failed
conservative therapy and have motor signs and
symptoms. However, 30% or more patients
have persisting symptoms.

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