Brachial plexus syndromes

Author: Mark B Bromberg, MD, PhD

Section Editor: Jeremy M Shefner, MD, PhD
Deputy Editor: Richard P Goddeau, Jr, DO, FAHA

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.

Literature review current through: Apr 2023. | This topic last updated: Sep 23, 2021.

INTRODUCTION

The brachial plexus is a network of nerve fusions and divisions that originate
from cervical and upper thoracic nerve roots and terminate as named nerves
that innervate muscles and skin of the shoulder and arm. Although detailed
knowledge of the elements of the network is important for distinguishing
between radiculopathy and mononeuropathy, a syndromic approach is more
useful for diagnosing lesions involving the plexus itself.

This topic will briefly review the underlying anatomy, pathogenesis, and
general clinical features of brachial plexopathies, and discuss specific brachial
plexopathies, classified by clinical setting into traumatic, nontraumatic,
iatrogenic, and neonatal types.

ANATOMY

Nerve roots from C5 through T1 contribute to the brachial plexus ( figure 1

and figure 2). The plexus can be divided into regions that include (from
proximal to distal) trunks, divisions, cords, branches, and nerves. Trunks and
divisions are further subdivided with a nomenclature based on overall
relationships with other upper extremity anatomic structures and include
upper, lower, and middle trunks, and posterior, lateral, and medial cords.
 ● C5 and C6 roots merge to form the upper trunk. The C7 root forms the
middle trunk. C8 and T1 roots merge to form the lower trunk.

● The upper trunk divides and gives branches to the lateral and posterior
cords. The middle trunk divides and gives branches to the lateral and
posterior cords. The lower trunk divides and gives branches to the
posterior and medial cord.

● The lateral cord branches and gives rise to the musculocutaneous nerve
and contributes to the median nerve. The posterior cord branches and
gives rise to the axillary nerve and then becomes the radial nerve. The
medial cord branches and contributes to the median nerve and then
becomes the ulnar nerve.

● Other nerves arise from various elements of the plexus. The dorsal
scapular nerve arises from the C5 root. The long thoracic nerve arises
from C5, C6, and C7 roots. The suprascapular nerve arises from the
upper trunk.

● Contributions to motor and sensory function vary within the brachial

plexus. The largest percentages of motor fibers are from C5 and C6
roots, and the least from C7 and T1 roots. The greatest number of
sensory fibers is from the C7 root, with lesser amounts from C5, C6, C8,
and T1 roots [1]. Postganglionic sympathetic nerve fibers from vertebral
ganglia course through the brachial plexus.

PATHOGENESIS

The pathologic basis and histologic changes seen with brachial plexus lesions
vary with the underlying causes, which include compression, transection,
ischemia, inflammation, metabolic abnormalities, neoplasia, and radiation
therapy. Because the brachial plexus is relatively inaccessible to direct
investigation, most pathologic processes are deduced.
● Nerve compression is relatively uncommon because the brachial plexus
is protected by bony structures. Contact sports are the most frequent
cause of nerve compression injury to the brachial plexus; focal forces to
the shoulder region result in brief compression of underlying plexus
elements.

● Overt nerve transection occurs with major trauma to the neck and
shoulder, causing downward traction on the shoulder and movement of
the neck to the contralateral side, or with trauma to the arm, causing
upward traction on the arm and shoulder. High-force traction to the
plexus may also be associated with avulsion of nerve roots.

● Ischemia due to occlusion of small intraneural (vasa nervorum) vessels is

more common than ischemia due to occlusion of large vessels. Small
vessel ischemia may be a common pathologic basis for inflammatory,
metabolic, and radiation-induced plexopathies [2-4]. Ischemia causes
localized axonal damage, resulting in denervation of muscle and skin
receptors.

● Inflammation, perhaps leading to small vessel occlusion, is hypothesized

to cause brachial plexopathies of acute onset (excluding those associated
with mechanical causes). However, precipitating events for such an
inflammatory response and its consequences are poorly understood.

● Metabolic abnormalities are the most likely initiating factors underlying

plexopathies related to diabetes mellitus. That said, the specific factors
are not known and the pathophysiology may include inflammation and
focal ischemia [3].

● Neoplastic plexopathies can result from direct pressure by a local cancer

mass, but invasion by cancer cells along nerves or tracking along
connective tissue is more common [2].

● Radiation treatment for cancer is a common cause of damage to brachial

plexus nerves [2]. The toxic effects of radiation may injure axons directly
 or injure the vasa nervorum causing ischemic changes to axons with
multifocal denervation.

EPIDEMIOLOGY

Brachial plexus syndromes are rare, as illustrated by the prevalence of

plexopathies associated with cancer (approximately 0.4 percent of patients
with cancer) and those associated with radiation treatment (approximately 2
to 5 percent of those treated) [2]. Idiopathic brachial plexopathy, or brachial
amyotrophy, has an estimated annual incidence of 2 to 3 per 100,000 [5,6]. In
one large series, the male-to-female ratio was 2:1 [7].

CLINICAL FEATURES AND DIAGNOSIS

Symptoms and signs — The onset of symptoms from brachial plexopathies

may vary from acute to insidious. Acute onset is usually characterized by pain
in the shoulder or upper arm, while insidious onset can manifest as
progressive pain, evolving numbness, weakness of selected muscles, or any
combination. Early on, distinguishing between symptoms arising from bones
and ligaments about the shoulder and those from nerves in the plexus may
be difficult. Acute onset, in the absence of trauma, favors a metabolic or
inflammatory process. Chronic progression of symptoms in patients with a
history of cancer and radiation treatment suggests either as a cause.

Clinical signs of brachial plexopathy include muscle weakness, atrophy, and

sensory loss. With a painful plexopathy of acute onset, it may be difficult to
distinguish true weakness from reduced effort due to pain. Muscle atrophy
may not be appreciated for several weeks. Tendon reflexes may be reduced in
weak muscles. Sensory loss commonly involves the axillary nerve distribution,
but may be diffuse or reflect the distributions of other involved nerves
( figure 3).

Nerve conduction studies and needle electromyography — The primary

pathologic lesion in most plexopathies is axonal loss, with demyelination and
conduction block as secondary processes. Nerve conduction studies primarily
assess terminal segments of nerves, and the distribution of abnormalities
can be helpful in localizing lesion sites lying more proximally in the plexus.
They can also determine if there are alternative diagnoses, such as nerve
entrapment syndromes. Sensory nerve conduction studies are more sensitive
to axonal loss than motor nerve studies, and application of a battery of
sensory nerve tests can be diagnostically helpful [1].

Needle electromyography (EMG) is the most sensitive test of axonal damage

in motor nerves and can further localize the lesion [8]. In addition, essentially
any muscle can be studied with needle EMG, and thus any element of the
plexus can be assessed. However, abnormal spontaneous activity in the form
of positive waves and fibrillation potentials may not be apparent for three
weeks after the acute onset of plexopathies.

Imaging — Routine chest and spine films may reveal osseous injuries or

altered posture due to muscle weakness. Plain computed tomography (CT) is
useful for detecting bony abnormalities, and CT myelography is useful for
detecting root avulsions. Magnetic resonance imaging (MRI) is more sensitive
for structural abnormalities than CT.

Magnetic resonance neurography is a specialized procedure that can

visualize individual roots, segments of the plexus, and peripheral nerves [9-
11]. It has low specificity but is more sensitive than conventional MRI for
plexus lesions, and can identify local factors relevant to possible
demyelination and/or compression, including nerve edema, thickening, and
T2 hyperintensities.

Ultrasound has been used to image the brachial plexus [12] and may be able
to distinguish preganglionic from postganglionic traumatic lesions
noninvasively [13].

TRAUMATIC PLEXOPATHIES
Traumatic injuries are the most common cause of brachial plexus lesions in
children and adults [14]. Motor vehicle accidents (particularly involving
motorcycle riders), industrial accidents, falls, objects falling on a shoulder,
sports injuries, and prolonged pressure on the plexus during deep sleep are
among the many causes of closed brachial plexus trauma [14-16]. Open
traumatic brachial plexus injuries result from gunshot wounds, lacerations,
and animal bites [14]. Open injuries are frequently associated with trauma to
nearby blood vessels, with the result that the plexus suffers secondary injury
from expanding hematomas, pseudoaneurysms, and arteriovenous fistulas.

In the setting of closed or open traumatic brachial plexus injuries caused by

acute trauma, it is important to evaluate the patient for concomitant head
injuries, bone fractures, dislocations, rotator cuff tears, and vascular
disruptions [17-19]. (See "Initial management of trauma in adults" and
"Trauma management: Unique pediatric considerations" and "Management
of acute moderate and severe traumatic brain injury" and "Acute mild
traumatic brain injury (concussion) in adults" and "Severe traumatic brain
injury (TBI) in children: Initial evaluation and management" and "Minor head
trauma in infants and children: Management" and "Traumatic causes of acute
shoulder pain and injury in children and adolescents" and "Presentation and
diagnosis of rotator cuff tears".)

Root avulsions, the burner syndrome, and backpack palsy are types of
traumatic plexopathies discussed in further detail below.

Root avulsions — Root avulsions are caused by high-energy traction (stretch)

and occur in parallel with brachial plexus injuries [8,14]. Thus, traumatic
injuries may include a combination of plexopathy and root avulsions. The
torn nerve roots and axons are incapable of regeneration and are not
amenable to surgical repair. Thus, the resulting motor and sensory deficits
are permanent. Multiple root avulsions are frequent with traumatic injury,
resulting in extensive deficits. In addition, most patients develop severe
avulsion pain, often involving the hand.
The lowest two brachial plexus roots (C8 and T1) are the most susceptible to
root avulsion, while the upper two (C5 and C6) are more susceptible to
extraforaminal rupture [8]. Unlike root avulsions, extraforaminal nerve
ruptures may be accessible to surgical repair.

Burner syndrome — The burner syndrome (also called the stinger

syndrome) is a term that describes the transient burning and stinging
sensations experienced during contact sports due to forceful contact by a
helmet to the neck or anterior shoulder region. This type of athletic injury
causes transient dysfunction of the brachial plexus and occasionally the
spinal cord. The upper trunk is most commonly involved, due to downward
traction of the shoulder. (See "Burners (stingers): Acute brachial plexus injury
in the athlete", section on 'Pathophysiology'.)

Symptoms are usually transient with no nerve damage. However, some cases
are associated with denervation, with or without weakness. Some clinicians
use the term "burner" to indicate more severe episodes of numbness in limbs
that implicate damage to the spinal cord. (See "Burners (stingers): Acute
brachial plexus injury in the athlete", section on 'Clinical features'.)

The diagnosis of burner syndrome is straightforward in the context of

American football or other contact sports. There may be a degree of
weakness that raises the question of a more substantial injury. Needle
electromyography (EMG) is usually normal, but can detect mild degrees of
denervation when there is no weakness, and more marked changes when
there is weakness. However, EMG is only indicated if symptoms persist for
more than three weeks, since the effects of acute injury are not evident prior
to this time. The physical examination should include assessment for a
myelopathy if symptoms suggest spinal cord damage, and when there is a
concern, an imaging study of the cervical spine is appropriate. (See "Burners
(stingers): Acute brachial plexus injury in the athlete", section on 'Diagnosis
and evaluation'.)

Since most burners and stingers are transient, lasting minutes to hours, no
work-up or treatment is usually required. Those associated with weakness
typically improve without treatment, but refraining from football and other
contact sports is appropriate until strength is regained. (See "Burners
(stingers): Acute brachial plexus injury in the athlete", section on
'Management'.)

Backpack palsy — Backpack palsy (also known as rucksack paralysis and

cadet palsy) is an upper plexus disorder. Patients with this condition present
with painless arm and/or shoulder weakness, usually unilateral, after wearing
a backpack or similar apparatus (eg, child carrying harness) for a prolonged
period of time, resulting most often in an upper trunk lesion [8]. Some
sensory loss is also present in the same distribution.

The pathogenesis of backpack palsy is thought to be direct compression from

the weight of the backpack. The lesions are predominantly demyelinating
conduction block, and full recovery usually ensues over a few months. In a
minority, axon loss predominates, and recovery may be incomplete or slow.

Prognosis and treatment — The natural history of traumatic brachial

plexopathies, whether from forceful trauma or iatrogenic trauma, is difficult
to document but is generally poor, with most natural improvements
occurring within six months [20]. However, there are few published data
regarding the outcome of unoperated traumatic plexopathies.

Most surgical interventions are performed after documenting no

improvement over three to four months, although there are situations when
emergent intervention is appropriate. The type of surgical intervention is
dependent upon the nature and degree of the lesion, and includes
neurolysis, nerve grafts, nerve transfers, and tendon and muscle transfers
[21-24]. There are data suggesting that delaying surgical treatment for longer
than two to six months diminishes the chance for an improved level of
function [25-27]. Injuries that have a degree of axonal continuity may have a
more favorable outcome, and nerve grafts are most successful for injuries of
C5, C6, and C7 roots; upper and middle trunks; and lateral and posterior
cords [28].
Outcome may be partially dependent upon surgical experience with brachial
plexus injuries, and data from experienced centers support improved
function in approximately 60 percent after surgical interventions. However,
many patients remain disabled, with one survey finding that one-half of those
previously employed did not return to work [29]. In a case report, three
patients with a traumatic global brachial plexus injury regained some useful
hand function 2 to 17 years after the injury with a novel bionic reconstruction
technique [30]. This technique involves a combination of selective nerve and
muscle transfers, elective amputation of the nonfunctional hand,
reconstruction with a prosthetic hand, and cognitive rehabilitation to activate
muscles attached to the prosthetic hand. More data are needed to determine
whether there is sufficient benefit to warrant this heroic procedure.

NONTRAUMATIC PLEXOPATHIES

Nontraumatic brachial plexopathies include neuralgic amyotrophy, hereditary

brachial plexopathy, neoplastic and radiation-induced plexopathy, thoracic
outlet syndrome (TOS), and brachial plexopathy related to diabetes.

Neuralgic amyotrophy — Neuralgic amyotrophy is considered to be an

inflammatory disorder of the brachial plexus. It is known by a number of
terms, including Parsonage-Turner syndrome, paralytic brachial neuritis,
idiopathic brachial plexopathy, brachial plexus neuropathy, and acute
brachial radiculitis. It has been described in children and adults [31].

The pathology for all forms of idiopathic brachial plexopathy is speculative, as

most cases are neither biopsied nor come to autopsy. However, careful
clinical and electrophysiologic assessment suggests that neuralgic
amyotrophy is a multifocal rather than a global process, with motor nerves
seeming more vulnerable than sensory [32,33]. Hypothesized underlying
causes focus on immune-mediated processes [34]. In this regard,
approximately 50 percent of patients describe some type of antecedent event
or possible predisposing condition, such as infection, exercise, surgery,
pregnancy and puerperium, or vaccination [4,7].
Classic form — In its classic form, neuralgic amyotrophy is characterized
clinically by the onset of severe pain followed by patchy weakness in the
distribution of the upper and/or middle brachial plexus, usually involving
winging of the scapula. There is rapid or subacute onset of unilateral
shoulder girdle and lateral arm pain, often awakening the individual in the
night. The pain can be excruciating and may last up to four weeks before
subsiding [7]. Bilateral onset of pain has been reported in up to 29 percent of
patients, almost always with asymmetric involvement [7]. Sensory symptoms
occur in a majority of patients, most commonly manifesting as hypesthesia
and/or paresthesia, and usually involving the lateral shoulder and arm and/or
the hand.

The time to onset of weakness is highly variable, as illustrated by the finding

that weakness occurs within the first 24 hours after onset of pain in one-third
of patients, and more than two weeks after onset of pain in approximately
one-quarter [7]. The distribution of muscle atrophy and weakness may be
limited to muscles supplied by one nerve, or in extreme cases may involve all
muscles of the shoulder or arm. There is a predilection for muscles
innervated by the suprascapular, long thoracic, musculocutaneous, radial,
anterior interosseous, and axillary nerves [7,33]. Variable weakness may also
be present in the contralateral limb. Recovery of strength begins within
several months, and the maximal degree of recovery may not be achieved for
several years [4].

Clinical variability — It is worth emphasizing that the pattern of nerve

involvement observed in neuralgic amyotrophy can be more diverse than the
classic syndrome [34]. Any part of the brachial plexus or muscles it innervates
may be involved [7]. In very severe cases, there may be bilateral arm
paralysis. Other patients may have involvement of single or multiple nerves
in the limb, more akin to a mononeuritis or mononeuritis multiplex.
Individual limb nerves that may be affected include long thoracic, anterior
interosseous, radial, median, and a variety of cutaneous nerves [32]. As an
example, isolated anterior interosseous nerve involvement may be the only
motor manifestation in some patients [7].
In a minority of patients, attacks may involve nerves outside of the brachial
plexus, including the lumbosacral plexus, phrenic nerve, and recurrent
laryngeal nerve [5,7]. Symptomatic diaphragm dysfunction due to unilateral
or bilateral phrenic neuropathy is often unrecognized but has been identified
in approximately 7 percent of cases of neuralgic amyotrophy [7,35]. The most
common symptoms are exertional dyspnea, sleep disturbance, and
orthopnea. Chest radiograph has suboptimal sensitivity for detecting
diaphragm dysfunction, particularly when involvement is bilateral [35].
Spirometry in the supine and sitting positions is more sensitive and typically
shows a decrease in forced vital capacity (FVC) that is more pronounced in the
supine position. (See "Diagnosis and management of nontraumatic unilateral
diaphragmatic paralysis (complete or partial) in adults", section on
'Diagnostic evaluation'.)

A similar clinical disorder to neuralgic amyotrophy occurs in association with

a variety of surgical procedures [36]. Onset is heralded by shoulder girdle
pain 1 to 14 days after surgery, so that direct nerve traction and compression
are not likely causative factors. Weakness ensues within several days and has
similar distributions to that seen in idiopathic brachial plexopathy.
Electrodiagnostic studies support axonal loss. Recovery is good.

Diagnosis — Neuralgic amyotrophy is primarily a clinical diagnosis

supported by electrodiagnostic testing. Clinical suspicion is based upon the
pattern of initial sudden and severe pain followed by atrophic weakness with
slow recovery. Nerve conduction studies are supportive and can exclude
more common mononeuropathies, while needle electromyography (EMG) is
important to document denervation. The pattern of denervation supports
either specific nerve involvement or a more patchy distribution.

The primary role of laboratories and imaging is to exclude alternative causes

of an acute plexopathy such as infection (eg, Lyme disease, human
immunodeficiency virus [HIV]), diabetes, or malignancy [37-40]. We typically
obtain a complete blood count, serum glucose and HbA1C, erythrocyte
sedimentation rate (ESR), and serologic testing for rarely implicated but
treatable infections including Borrelia burgdorferi, syphilis, and HIV. In most
patients with a suspected idiopathic brachial plexopathy, one-time imaging is
indicated to exclude the possibility of a mass lesion, especially in those with
atypical clinical symptoms or a history suggestive of malignancy. If EMG
localizes to the plexus, magnetic resonance imaging (MRI) of the brachial
plexus without and with contrast is the best study, provided there are no
contraindications to MRI; if EMG or clinical features suggest possible cervical
root involvement, MRI of the cervical spine should also be obtained in most
cases. Lumbar puncture is not indicated unless there is suspicion for an
alternative infectious, inflammatory, or neoplastic etiology involving the
cerebrospinal fluid.

Laboratory studies in patients with neuralgic amyotrophy are normal, and

imaging studies are often unrevealing. MRI and magnetic resonance
neurography of the brachial plexus and affected nerves may show
abnormalities including focal thickening, increased T2 signal, and gadolinium
enhancement, but sensitivity appears to be low, particularly in the acute
phase of neuralgic amyotrophy [7,41-43]. Neuromuscular ultrasound at
centers with appropriate technical expertise in this method may reveal focal
or diffuse nerve enlargement, focal constriction, torsion, or fascicular
entwinement, though data are limited [43,44]. Multifocal nerve involvement
often obviates the need for imaging. (See "Diagnostic ultrasound in
neuromuscular disease".)

Patients with symptoms of diaphragm dysfunction (eg, orthopnea, exertional

dyspnea) should undergo chest radiograph and spirometry. Ultrasound of the
diaphragm may be the most accurate test for neuromuscular diaphragmatic
dysfunction [45,46]. (See "Diagnosis and management of nontraumatic
unilateral diaphragmatic paralysis (complete or partial) in adults", section on
'Diagnostic evaluation' and "Diagnostic ultrasound in neuromuscular
disease", section on 'Physics of neuromuscular sonography'.)

Management and prognosis — There is no specific treatment for neuralgic

amyotrophy. Management is conservative. Physical therapy may be helpful to
preserve range of motion but will not hasten recovery. Glucocorticoids have
been advocated by some [34], but no clinical studies have demonstrated
efficacy. In the first weeks, analgesic drugs, including narcotics, may be
required to manage patients with severe pain.

Recovery occurs slowly over one to three years, and some patients have
persistent disability, as illustrated by the following observations:

● An early series of 99 patients with neuralgic amyotrophy suggested that

most had good recovery, essentially to normal strength [4].

● Another series included 199 patients with idiopathic neuralgic

amyotrophy [7]. Among 39 with three or more years of follow-up, mild,
moderate, or severe paralysis was still present in 69, 14, and 3 percent,
respectively. Overall, recurrence of idiopathic neuralgic amyotrophy was
observed in 26 percent of patients, with a median time to recurrence of
just over two years. This recurrence rate is higher than the estimated rate
of 5 percent previously reported [4].

● A third series surveyed 248 patients with either idiopathic or hereditary

neuralgic amyotrophy; approximately 90 percent had the idiopathic form
[47]. After 6 to 36 months of follow-up, residual pain or severe fatigue
was present in approximately 60 percent.

● Patients with phrenic neuropathy usually have spontaneous

improvement within two years, but persistent complaints are common
[35]. Treatment of diaphragm dysfunction consists primarily of
supportive measures; overnight polysomnography and noninvasive
positive pressure ventilation is typically indicated in more symptomatic
patients. (See "Treatment of bilateral diaphragmatic paralysis in adults".)

There is a pathologically distinct, yet clinically similar, hereditary recurrent

brachial plexopathy that should be considered when there are multiple
episodes in an individual or among related family members. (See 'Hereditary
brachial plexopathy' below.)
Hereditary brachial plexopathy — Hereditary brachial plexopathy (MIM
162100), also known as hereditary neuralgic amyotrophy, is a rare autosomal
dominant disorder characterized by recurrent, painful brachial plexopathies.
Genetic studies in families with this condition identified variants in the septin
9 gene (SEPT9) on chromosome 17q25 [48-50]. Hereditary brachial
plexopathy is distinct from hereditary neuropathy with predisposition to
pressure palsies (HNPP), a recurrent demyelinating neuropathy that can
involve the brachial plexus. (See "Charcot-Marie-Tooth disease: Genetics,
clinical features, and diagnosis", section on 'Hereditary neuropathy with
liability to pressure palsy'.)

Childhood onset of hereditary brachial plexopathy is not unusual [51]. In one

series of 47 patients, the age at onset ranged from 3 to 54 years, with a mean
of 28 years [7]. Many patients exhibit a relapsing-remitting course
characterized by attacks that resolve spontaneously, either completely or
incompletely, leaving additive residual weakness. Reported triggering events
include physical exertion, anesthesia, surgery, pregnancy, and childbirth [52].

The disorder can also follow a progressive pattern. In one study that analyzed
101 attacks in 24 patients from nine different families, both a classic
relapsing-remitting type and a chronic undulating type with exacerbations
were observed [53]. Only one type occurred per family.

Attacks are heralded by pain and paresthesias, followed by paresis of the

shoulder and arm. While any nerve in the brachial plexus can be involved,
injury to the upper part of the brachial plexus is most frequent. Winging of
the scapula caused by long thoracic nerve involvement is common, and facial
weakness and autonomic nervous system dysfunction may occur [51].
Sympathetic activity with flushing can be observed. Sensory involvement
(mainly involving hypesthesia and paresthesias of the shoulder, arm, or hand)
is present in most patients [7]. Nerves outside the brachial plexus can also be
involved, including the lumbosacral plexus, the phrenic nerve (causing
weakness of the diaphragm), and the recurrent laryngeal nerve [7].
Characteristic somatic features of hereditary brachial plexopathy include
short stature, hypotelorism, a small face, unusual skin folds, and creases on
the neck [54].

Although electrophysiologic studies are limited, sensory nerve conduction

velocity is typically normal, while motor conduction latencies are prolonged.
These findings suggest an axonal neuropathy, but are also compatible with
focal demyelination, conduction block, or a chronic process with
remyelination [55].

The histopathology of hereditary brachial plexopathy is nonspecific. Muscle

demonstrates pathologic changes consistent with neurogenic atrophy. Both
motor and sensory nerves show widely dispersed pathologic involvement
throughout the brachial plexus; the changes involve very focal damage to
individual nerves with lesions restricted to isolated fascicles within the nerve
[56]. The changes are predominantly demyelinating, but axonal changes are
also present.

Diagnosis — A pattern of recurrent brachial plexopathies and a similar

history in related family members should raise consideration for the
diagnosis of hereditary neuralgic amyotrophy. Finding of dysmorphic
features is strongly supportive.

Management and prognosis — Most patients with hereditary brachial

plexopathy have some degree of disability. In a series that followed 10
patients for three or more years, mild, moderate, or severe weakness was
present in four, three, and two patients, respectively [7]. Management is
supportive, although glucocorticoids have been given in uncontrolled studies
[52].

Neoplastic and radiation-induced brachial plexopathy — These conditions

are briefly reviewed here, and discussed elsewhere in greater detail. (See
"Overview of cancer pain syndromes", section on 'Tumor-related neuropathic
pain' and "Overview of cancer pain syndromes", section on 'Postradiation
pain syndromes'.)
Neoplastic — Breast and lung cancers are the most frequent neoplasms
leading to brachial plexopathy. Pain in the shoulder and axilla is the most
common presenting symptom of neoplastic brachial plexopathy. Invasion of
the lower plexus (inferior trunk and medial cord) occurs more frequently than
invasion of the upper trunk. (See "Overview of cancer pain syndromes",
section on 'Plexopathies'.)

The Pancoast syndrome is most commonly due to non-small cell lung cancer
in the superior sulcus, and is associated with a Horner syndrome in three-
quarters of patients. Weakness usually occurs in a lower plexus distribution,
but may also be more widespread and patchy in distribution. (See "Superior
pulmonary sulcus (Pancoast) tumors" and "Horner syndrome".)

In the setting of a known neoplasm and radiation therapy, distinguishing

between cancer recurrence and radiation-induced plexopathy can be
challenging. In general, compared with radiation-induced brachial
plexopathies, neoplastic plexopathies are more likely to produce pain at
symptom onset, more often involve the lower plexus, and are more
frequently associated with a Horner syndrome.

Primary brachial plexus tumors, such as schwannomas or neurofibromas, are

uncommon, and most occur as solitary tumors. These rarely cause
symptomatic plexopathies. However, multiple tumors occur in patients with
neurofibromatosis type 1, and these are more likely than solitary tumors to
present with pain or clinical deficits.

The diagnosis of neoplastic brachial plexopathy is suggested by the clinical

setting, including symptoms, past medical history (eg, smoking), and physical
examination, particularly the breast examination. Imaging of the chest by
computed tomography (CT) is the most sensitive way to detect a pulmonary
tumor. A Horner syndrome in the setting of shoulder pain and weakness is
strongly supportive of a pulmonary tumor. Electrodiagnostic studies and MRI
of the brachial plexus are useful to more precisely define the relationship
between the tumor and the brachial plexus ( image 1 and image 2).
There are limited data on the benefit of treating neoplastic brachial
plexopathy with radiation therapy to the plexus; the goal is mostly directed at
reducing pain. Medical management of pain is appropriate if radiation
therapy is not beneficial. These options are discussed in greater detail
separately. (See "Cancer pain management: General principles and risk
management for patients receiving opioids" and "Cancer pain management
with opioids: Optimizing analgesia" and "Cancer pain management: Role of
adjuvant analgesics (coanalgesics)".)

Radiation-induced — Radiation-induced brachial plexopathy is a

consideration in patients with cancer who develop plexopathy after radiation
therapy in the vicinity of the brachial plexus. As mentioned above, either
cancer recurrence or radiation therapy can cause a brachial plexopathy in this
setting.

Lung and breast cancers are the most common neoplasms that are treated
with radiation therapy in the vicinity of the brachial plexus, but any patient
with radiation therapy to the region is at risk for subsequent plexopathy.
Nevertheless, brachial plexopathy is most frequently seen in females with
early stage breast cancer, who have been treated with conservative surgery
and radiation therapy [57-59]. (See "Breast-conserving therapy" and
"Adjuvant radiation therapy for women with newly diagnosed, non-metastatic
breast cancer".)

The features of brachial plexopathy in this setting are illustrated by an

analysis of 1624 females, in which the following findings were noted [57]:

● The incidence of brachial plexopathy was 1.3 percent among females

who received less than 50 Gy to the axilla, and 1.8 percent in those
receiving radiation to the supraclavicular fossa.

● The incidence was higher in females who also received chemotherapy

(4.5 percent) and in those treated with axillary doses greater than 50 Gy
(5.6 percent).
 ● The median time to development of symptoms after therapy was
approximately 10 months.

The use of larger radiation fractions may increase the risk of developing
radiation-induced brachial plexus damage. In a series of 449 females, the
incidence of plexopathy was 6 percent in patients receiving 45 Gy in 15
fractions versus 1 percent for those receiving 54 Gy in 30 fractions [58].

There are few clinical features that distinguish radiation-induced damage

from that due to cancer, but radiation-induced plexopathy frequently has less
pain than neoplastic brachial plexopathy, and what pain there is typically
occurs later in the course of symptoms. In addition, the degrees of
paresthesia and weakness typically are more severe with radiation-induced
plexopathy than with neoplastic brachial plexopathy. Survival in the setting of
radiation-induced plexopathy is related to the underlying cancer and is good
with respect to the side effects of radiation.

The diagnosis of a radiation-induced rather than neoplastic plexopathy is

supported by the presence of fasciculations on electrodiagnostic testing.
Myokymic discharges add further support to the diagnosis, since these are
rarely found in other conditions. However, myokymic discharges are not a
universal feature of radiation-induced plexopathy, and their absence does not
exclude the diagnosis.

Symptomatic management of patients with radiation-induced plexopathy

includes medications used for other types of chronic or neuropathic pain
such as gabapentinoids, glucocorticoids, and tricyclic antidepressants [60,61].
Individual reports have described benefit for refractory cases with hyperbaric
oxygen therapy, neurolysis, or pulsed radiofrequency ablation [62-64].
Medications for symptomatic management are discussed in greater detail
separately. (See "Cancer pain management: Role of adjuvant analgesics
(coanalgesics)".)

Thoracic outlet syndrome — Thoracic outlet syndrome (TOS) is a term used

to denote a variety of upper extremity syndromes, with only a small number
having a neurologic basis [65]. TOS is generally divided into five subtypes,
which are [66,67]:

● Arterial TOS
● Venous TOS
● Traumatic neurovascular TOS
● True neurogenic TOS
● Disputed TOS

The vascular forms of TOS are reviewed in detail separately. (See "Overview of
thoracic outlet syndromes".)

True neurogenic TOS is rare, with an estimated incidence of 1:1,000,000, and

occurs most commonly in females, with a female-to-male ratio of 9:1 [68].
Symptoms of true TOS include slowly progressive unilateral atrophic
weakness of intrinsic hand muscles and numbness in the distribution of the
ulnar nerve, with occasional numbness of the ulnar aspect of the forearm.
The motor involvement is much greater than the sensory involvement [65].
Weakness and atrophy involve the thenar eminence (median-innervated
hand intrinsic muscles) more than the ulnar-innervated hand intrinsic
muscles, while medial forearm muscles are less involved. The syndrome may
be caused by a taut congenital fibrous band from the first rib to the tip of an
elongated C7 transverse process or to a rudimentary cervical rib. The lower
portion of the plexus is stretched over this band, and chronic traction injury
results ( figure 4). This explains why true TOS has the clinical features of a
lower trunk plexopathy [68]. Other potential causes of TOS include
hypertrophy of the scalene, subclavius, or pectoralis minor muscles, as might
be seen in weight lifters or other kinds of athletes. (See "Overview of thoracic
outlet syndromes", section on 'Pathogenesis'.)

The incidence of the nonneurogenic forms of TOS is less clear. Arterial and
venous forms of TOS are caused by compression of the subclavian artery or
vein. Patients may present with arm ischemia (claudication and/or acute
embolic events) or venous thrombosis. (See "Overview of thoracic outlet
syndromes", section on 'Arterial TOS' and "Overview of thoracic outlet
syndromes", section on 'Venous TOS'.)

Disputed TOS (also called nonspecific TOS) is the most controversial form
[66,67,69,70]. Proponents of this entity believe that pathologic processes in
the thoracic outlet, such as soft tissue anomalies that can only be appreciated
at time of surgery, are the cause of a myriad of symptoms affecting the neck,
shoulder, and arm, including involvement of upper trunk nerves [69]. Other
experts consider disputed TOS to be a cervicoscapular pain syndrome
because it lacks a clear anatomic abnormality, an established pathogenesis,
consistent clinical manifestations, and a reliable method of diagnostic testing
[66,67].

Diagnosis — The diagnosis of true neurogenic TOS is based on a highly

characteristic pattern of nerve conduction abnormalities involving the C8 and
T1 fibers, and disproportionally involving the T1 sensory and motor fibers
more than the C8 fibers [65,71,72]:

● Reduced or absent ulnar and medial antebrachial cutaneous sensory

responses and normal median sensory response

● Reduced or absent ulnar and median motor responses, with the median
response often more affected than the ulnar

Needle EMG shows denervation in ulnar and median innervated muscles [71].

Imaging studies in true neurogenic TOS are of limited diagnostic utility.

Cervical ribs can be visualized on plain films, but the fibrous band
compressing the brachial plexus is radiolucent and therefore cannot be
demonstrated reliably by plain films or CT [65,73]. There are sparse and
inconsistent data regarding the ability of MRI to identify a fibrous band or
distortion of the brachial plexus ( image 3) in patients with true neurogenic
TOS [65,74-77].

The diagnosis of true vascular TOS is supported by abnormal arteriography

or venography, while electrodiagnostic tests are normal. The diagnosis of
axillosubclavian vein thrombosis is discussed separately. (See "Primary
(spontaneous) upper extremity deep vein thrombosis", section on
'Diagnosis'.)

Most advocates of disputed TOS maintain that testing will be normal and the
diagnosis is purely clinical with confirmation at surgery.

Management — With true neurogenic TOS, surgical release of the fibrous

band or resection of the rudimentary cervical rib is appropriate. Symptoms of
pain typically respond, but strength is unlikely to return due to axonal
damage [65,71].

Decompressive surgery for vascular TOS may also be helpful. The treatment
of venous and arterial TOS is reviewed in detail elsewhere. (See "Overview of
thoracic outlet syndromes", section on 'Approach to management' and
"Overview of thoracic outlet syndromes", section on 'Thoracic outlet
decompression'.)

Surgery for disputed TOS is not to be undertaken lightly because of high

postoperative complication rates, which include postoperative iatrogenic
brachial plexopathy [68,78]. Conservative measures include passage of time,
weight reduction, and a strengthening program to improve sloping shoulders
and posture [79].

Diabetic-related brachial plexopathy — Plexopathies related to diabetes

mellitus usually involve the lumbosacral plexus, but upper extremity
involvement has also been observed as part of the syndrome. In
contemporary series, upper extremity involvement occurs in up to one-third
of patients and may be in the form of mononeuropathies of the ulnar and
median nerves, or at more proximal sites in the brachial plexus [80]. Most
upper extremity symptoms are in association with lumbosacral plexus
involvement, and thus, clinical features in the arm, such as presence of pain,
are not fully reported.

Since most cases of brachial plexus involvement occur in the setting of

lumbosacral plexus symptoms, the diagnosis of brachial symptoms is linked.
Needle EMG studies show denervation in the distribution of a nerve or more
diffusely in the plexus.

Lumbar polyradiculopathy and diabetic amyotrophy are discussed in greater

detail elsewhere. (See "Lumbosacral plexus syndromes" and "Diabetic
amyotrophy and idiopathic lumbosacral radiculoplexus neuropathy".)

IATROGENIC PLEXOPATHIES

Iatrogenic brachial plexopathies related to surgery and other medical

procedures account for approximately 7 to 10 percent of all brachial
plexopathies [8,81]. Classic postoperative paralysis, postmedian sternotomy
plexopathy, medial fascial compartment syndrome, and nerve injury
associated with regional anesthetic blocks are briefly reviewed in the
following sections. An iatrogenic plexopathy has also been reported following
surgery for the disputed form of thoracic outlet syndrome (TOS). (See
'Thoracic outlet syndrome' above.)

Classic postoperative paresis — Classic postoperative paresis is caused by

traction or pressure during surgery [8,14]. The presentation is painless
weakness in the distribution of the upper brachial plexus. Some cases are
accompanied by paresthesias, and occasional cases are bilateral. The lesions
are predominantly demyelinating conduction block, and most patients have
rapid and complete recovery unless there is significant axonal loss. Thus,
conservative management is employed.

Postmedian sternotomy plexopathy — As the name suggests, postmedian

sternotomy plexopathy occurs in the setting of open heart surgery and other
operations requiring median sternotomy [8,14]. The typical presentation is
that of hand weakness accompanied by paresthesias, and sometimes pain,
involving the fourth and fifth fingers. Weakness of muscles supplied by C8 via
the median nerve (eg, flexor pollicis longus) and radial nerve (eg, extensor
indicis proprius, extensor pollicis brevis) clinically distinguishes this from an
ulnar neuropathy. Localization is aided by electrodiagnostic studies.
Although the mechanism is not entirely clear, the leading hypothesis is that
this type of plexopathy results from traction injuries to the C8 anterior
primary ramus [14]. The lesions are mainly demyelinating conduction block,
and the prognosis for full recovery is generally good unless there is
significant axon loss.

Anesthetic block plexopathy — Plexopathy after regional infraclavicular

brachial plexus blockade has been described with a frequency of 1 to 4
percent [82]. Mechanisms include trauma from the infusion needle,
hematoma formation, and neurotoxicity from local anesthetic. Evaluation
includes imaging and electromyography (EMG).

Medial brachial fascial compartment syndrome — The medial brachial

fascial compartment extends from the clavicle to the elbow and is formed by
the medial intramuscular septum, which divides into two fascial extensions
that enclose the neurovascular bundle, including the five terminal nerves of
the brachial plexus [83]. Puncture of the axillary or brachial artery during
procedures such as arteriography or axillary regional anesthetic blocks can
produce an expanding hematoma that causes a compartment syndrome and
compresses one or more of the terminal nerves [8,14,83-85]. The median and
ulnar nerves are most frequently affected.

Initial symptoms of the medial brachial fascial compartment syndrome may

be delayed for two weeks or longer after the procedure [14]. Unilateral pain
and paresthesia are followed by progressive weakness. Distal pulses typically
remain normal. Urgent surgical decompression may improve recovery, which
is otherwise poor [8,86].

NEONATAL BRACHIAL PLEXUS PALSY

Neonatal brachial plexus palsy (NBPP) is an uncommon disorder identified by

arm weakness at birth. It may involve any of the nerve roots from C5 to T1.
NBPP is discussed in detail elsewhere. (See "Neonatal brachial plexus palsy".)
SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected

countries and regions around the world are provided separately. (See "Society
guideline links: Neuropathy".)

SUMMARY
● Anatomy – Nerve roots from C5 through T1 contribute to the brachial
plexus ( figure 1). The plexus can be divided into regions that include
(from proximal to distal) trunks, divisions, cords, branches, and nerves.
Trunks and divisions are further subdivided into upper, lower, and middle
trunks and posterior, lateral, and medial cords. (See 'Anatomy' above.)

● Clinical features – The onset of symptoms from brachial plexopathies

may be acute or insidious. Acute onset is usually characterized by pain in
the shoulder or upper arm, while insidious onset can manifest as
progressive pain, evolving numbness, and/or weakness of selected
muscles. Clinical signs include muscle weakness, atrophy, and sensory
loss. (See 'Symptoms and signs' above.)

● Traumatic plexopathies – Traumatic injuries are the most common

cause of brachial plexus lesions. Motor vehicle accidents, industrial
accidents, falls, objects falling on a shoulder, and sports injuries are
among the many causes of closed brachial plexus trauma. Open
traumatic brachial plexus injuries may result from gunshot wounds,
lacerations, and animal bites. (See 'Traumatic plexopathies' above.)

● Neuralgic amyotrophy – Neuralgic amyotrophy is characterized

clinically by the onset of severe pain followed by patchy weakness in the
distribution of the upper and/or middle brachial plexus, usually involving
winging of the scapula. Sensory symptoms typically involve the lateral
shoulder and arm and/or the hand. (See 'Neuralgic amyotrophy' above.)
● Hereditary brachial plexopathy – Hereditary brachial plexopathy is a
rare autosomal dominant disorder characterized by recurrent, painful
brachial plexopathies. In some families the cause has been identified as
variants in the SEPT9 gene. (See 'Hereditary brachial plexopathy' above.)

● Neoplastic and radiation-induced brachial plexopathy –Neoplastic

brachial plexopathy is most frequently related to breast and lung cancer.
Pain in the shoulder and axilla is the most common presenting symptom
of neoplastic brachial plexopathy. Invasion of the lower plexus (inferior
trunk and medial cord) occurs more frequently than invasion of the
upper trunk. (See 'Neoplastic and radiation-induced brachial plexopathy'
above and "Overview of cancer pain syndromes", section on
'Plexopathies'.)

Radiation-induced brachial plexopathy is a consideration in patients with

cancer who develop plexopathy after radiation therapy in the vicinity of
the brachial plexus. Either cancer recurrence or radiation therapy can
cause a brachial plexopathy in this setting. (See 'Neoplastic and radiation-
induced brachial plexopathy' above and "Overview of cancer pain
syndromes", section on 'Plexopathies'.)

● Thoracic outlet syndrome – Thoracic outlet syndrome (TOS) is a term

used to denote a variety of upper extremity syndromes, with a minority
having a neurologic cause. Symptoms of neurogenic TOS include slowly
progressive unilateral atrophic weakness of intrinsic hand muscles and
numbness in the distribution of the median and ulnar nerve. (See
'Thoracic outlet syndrome' above.)

● Diabetic-related brachial plexopathy – Plexopathies related to diabetes

mellitus usually involve the lumbosacral plexus, but additional upper
extremity involvement may also occur. Upper extremity diabetic
plexopathy may be at proximal sites in the brachial plexus. (See 'Diabetic-
related brachial plexopathy' above.)
 ● Iatrogenic brachial plexopathy – Iatrogenic brachial plexopathies
related to surgery and other medical procedures include classic
postoperative paralysis, postmedian sternotomy plexopathy, and medial
fascial compartment syndrome. (See 'Iatrogenic plexopathies' above.)
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