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Key Words: Bilateral
Winging of scapula, Brachial Plexus neuritis, Parsonage-Turner
syndrome
Synonyms: Brachial
Neuritis, Brachial Plexus Neuritis, Idiopathic Brachial Plexus
Neuropathy, Neuralgic Amyotrophy, Feinberg’s syndrome, Tinel’s
syndrome, Kiloh-Nevin syndrome III
Introduction
Brachial Plexus neuritis, often referred to as Parsonage-Turner
syndrome, can be a vexing problem for both the patient and the
physician. It is a common condition characterized by
inflammation of a network of nerves that control and innervate
the muscles of the chest, shoulders, and arms (brachial plexus).
Individuals with the condition first experience a sudden onset
of severe pain across the shoulder and upper arm. Within a few
hours or days, the muscles of the involved shoulder may be
affected by, weakness, wasting (atrophy), and paralysis
(atrophic paralysis). Although individuals with the condition
may experience paralysis of the affected areas for months or, in
some cases, years, recovery is usually complete. The exact cause
of Parsonage-Turner Syndrome is not known.
Today
different mononeuropathies are described as syndromes and
cervical plexus neuropathies are indicated by their etiology.
This condition presents with severe pain in the shoulder and
arm, followed by atrophic paralysis of some muscles of shoulder
girdle. Often a generalized aching and fever precede it. If
serratus anterior is involved, and it usually is, the scapula
wings and the patient has difficulty in raising his arm upward
and outward. This type of condition was documented by J. D.
Spillane in 1943(1). The pain decreased spontaneously and
eventually resolved completely in all patients. Weakness in the
shoulder had developed at a mean of approximately four weeks
after the initial onset of pain. The weakness decreased
spontaneously but very gradually in one case, one patient had
persistent, mild weakness at the most recent follow-up
evaluation.
J.Orthopaedics 2005;2(5)e4
Case Report
Two cases of Brachial Plexus
neuritis (Parsonage-Turner syndrome) were reviewed here. The
patients had been followed for two years after the onset of the
symptoms. Both of them had an acute onset of intense pain in the
shoulder without antecedent trauma. No systemic disorder that
might affect the musculoskeletal system, and an onset of
weakness in the involved shoulder within a few weeks after the
onset of the pain. Both of them were male with age 35yrs and 42
yrs and they were right handed. The dominant right upper
extremity was affected in both cases. They were in good health,
with no history of current or pre-existing systemic disorders or
neurological abnormalities. None of them were involved in
strenuous overhead sports and none of them were head load
workers. One patient presented with weakness both shoulders with
bilateral winging of scapula (Angels wing sign), who had
paresthesia in the extremity, and he was found to have
widespread involvement of the brachial plexus on either side
(mainly C5 C6 involvement with mild involvement of C7, C8 and
had involvement of the long thoracic nerve).
History and symptoms that
were suggestive of Parsonage-Turner syndrome, in addition the
pain and weakness had decreased spontaneously and
electromyographic studies had demonstrated findings
(fibrillation potentials and positive waves) that were
consistent with brachial neuritis. Also, the patient had been
followed for a period of two years since the time of the
evaluation. Both of the patients do not have abnormalities of
the rotator cuff, cervical spondylosis, herniation of a disc,
syringomyelia, and peripheral neuromuscular disorders. Patients
were evaluated with serological testing (including a complete
blood-cell count, determination of the Westergren erythrocyte
sedimentation rate, and a blood-chemistry profile), radiographs
of the shoulder and neck, and electromyography. One patient who
had involvement of the suprascapular nerve was initially
evaluated with MRI to rule out a tear of the rotator cuff
Treatment for our patients
consisted of analgesics as needed, physical therapy, and
rehabilitation exercises. Because the pain resolved
spontaneously, modalities such as ultrasound and cryotherapy
were not needed. Physical therapy was focused on the maintenance
of the range of motion of the shoulder and on the restoration of
strength. Our patients participated in a home-exercise program
that was designed to strengthen the rotator cuff and the
scapular stabilizing muscles.
Regular
schedule for the timing of follow-up visits was used with
monthly for the initial six months followed by every two months.
Muscle strength was tested by means of a manual assessment of
maximum strength and was graded subjectively by the first
author. No devices were used to measure strength at any of the
follow-up examinations.
Results:
The intense pain continued
for twenty days before spontaneous improvement, in one patient
and sixteen days in the second case, was noted. As the intense
pain subsided, it was replaced by a dull ache. One patient noted
a slight increase in the ache during use of the upper extremity
for daily activities. None of them had persistent pain at night
after the resolution of the initial intense pain. Both patients
noted the onset of weakness after the resolution of the initial
intense pain. Both of these patients had involvement of the long
thoracic nerve Electromyographic and nerve-conduction testing
revealed involvement of only the peripheral nervous system in
both patients. One patient had widespread, marked involvement of
the entire upper trunk; electromyography revealed involvement of
the infraspinatus, deltoid, and biceps muscles. He had bilateral
Brachial Plexus neuritis with bilateral winging of scapula. His
right side involvement was predominant than left side lesion.
That patient also had evidence of abnormalities distally along
the course of the median nerve (right side) and who had pain and
paresthesias distally in the extremity. The second patient had
predominant involvement of one nerve (long thoracic nerve) with
the moderate involvement of suprascapular nerve.
Electromyographic testing that was performed three to four weeks
after the onset of the pain showed fibrillation potentials and
positive waves. Testing that was done three to four months after
the onset revealed polyphasic action potentials with increased
duration and amplitude but with a reduction in the number of
action potentials.
Physical examination at the
time of presentation revealed objective weakness in both
patients. Both patients had scapular winging because of the
involvement of the long thoracic nerve at the time of
presentation. The patient who had diffuse involvement of the
brachial plexus had atrophy and weakness of both the deltoid and
the infraspinatus muscle at the time of presentation. One
patient noted symptoms on the contralateral side four months
after the onset of the initial symptoms.
The patients were followed
for a mean of two years after the onset of the symptoms. Both of
the two patients had a spontaneous decrease in pain during the
follow-up period, and they reported the pain to be relatively
minor within three months after its onset. The patient with
bilateral involvement had near normal recovery on the less
involved side (left side) with complete disappearance of the
winging of left scapula over seven months follow up. The
predominantly involved right side is recovering gradually. Both
the patients had additional improvement in strength between the
one-year and latest follow-up visits.
Discussion:
The exact etiology of
Parsonage-Turner syndrome has not been delineated, but both
viral infection and immunological causes have been proposed. In
previously reported series, many patients were noted to have had
an antecedent illness or a recent immunization. Such an
association could not be determined for our patients. Trauma
generally is not considered to be a factor in the development of
Parsonage-Turner syndrome. Patients who had a history of trauma
were included in the study by Mulvey et al(2), but there had
been a prolonged asymptomatic period before the onset of pain.
In addition, the injuries in those patients were very minor. One
patient in our study recalled that he may have sustained a minor
injury of the affected shoulder before the onset of pain, but he
had been asymptomatic for more than two weeks before the onset
of symptoms.
Our two patients were male.
Magee and DeJong reported a male-to-female ratio of 11.5 to
one(3). Tsairis et al. and Turner and Parsonage found a much
lower male-to-female ratio of two to one.
A wide distribution of
nerve involvement has been reported in the literature. In the
original description by Parsonage and Turner, the long thoracic
nerve was involved most commonly(4); the suprascapular and
axillary nerves were involved only if multiple peripheral-nerve
lesions were found. Magee and DeJong as well as Tsairis et al.
reported a much higher frequency of isolated involvement of the
suprascapular nerve. Two of the patients in the present study
had primary involvement of the suprascapular nerve as confirmed
with both physical examination and electromyographic studies.
Mulvey et al(2) reported on sixteen patients who had acute
brachial neuritis with primary involvement of the phrenic nerve.
No patient in the present study had involvement of the phrenic
nerve.
Previous authors have noted
frequent bilateral involvement, and it has been suggested that
as many as one-third of patients have bilateral symptoms. Turner
described the case of a patient in whom symptoms developed on
the contralateral side approximately six months after the onset
of the initial symptoms. In the current series, one out of the
two patients had evidence of brachial neuritis on the
contralateral side.
Sensory changes frequently
are noted in patients who have Parsonage-Turner syndrome.
Tsairis et al. noted such changes in sixty seven (67 per cent)
of ninety-nine patients who had this disorder. Turner noted no
sensory changes in any patient in whom involvement was limited
to a motor nerve with no cutaneous innervation. Our two patients
had sensory involvement in the area of the root involved.
Two patients in the current
series had primary involvement of both the long thoracic and the
suprascapular nerve. The one patient who had more widespread
involvement of the brachial plexus had sensory changes in the
hand, in the area of the median nerve.
Both of our patients had an
increase in strength during the follow-up period. As reported by
Dillin et al(5). Both of our patients noted an improvement in
strength within the first two months after the onset of
weakness. However, Magee and DeJong(3) found that it might take
as long as eight years to achieve maximum recovery.
Patients with
Parsonage-Turner syndrome who are seen soon after the onset of
symptoms can pose a diagnostic challenge. This disorder can be
confused with a variety of other diagnoses, including acute
calcific tendinitis, adhesive capsulitis, cervical spondylosis,
cervical radiculopathy, tumor of the spinal cord,
peripheral-nerve compression, amyotrophic lateral sclerosis,
acute poliomyelitis, and abnormalities of the rotator cuff(6).
The sudden onset of intense pain, with no inciting trauma and
with normal findings on examination of the neck and shoulder,
distinguishes this disorder from most of those included in the
differential diagnosis. The diagnosis becomes more obvious when
the pain decreases spontaneously and weakness develops.
Electromyography at that time should confirm the neuropathy.
The most confusing
differential diagnosis is that of peripheral-nerve compression.
However, patients who have that condition do not have the
sudden, intense pain associated with Parsonage-Turner syndrome
but rather have a more insidious onset of pain. Also, the fairly
rapid, spontaneous resolution of pain that is typical in
patients who have Parsonage-Turner syndrome is not seen in
patients who have peripheral-nerve compression.
MRI has been reported to
reveal early an increased signal intensity on T2-weighted
spin-echo images in the affected shoulder muscles, related to
neurogenic oedema. In more chronic cases, signs of denervation
atrophy can be seen on T1-weighted spin-echo images,
demonstrating an increased signal intensity and reduction of
muscle volume. Imaging is also useful to exclude other causes of
shoulder pain and muscle atrophy (e.g. periarticular ganglion).
Although Parsonage-Turner
syndrome is not seen frequently in a typical orthopaedic
practice, it is still important for orthopaedists to be familiar
with this disorder and to include it in the differential
diagnosis when a patient describes pain or weakness about the
shoulder. The diagnosis is made primarily by exclusion. The
prognosis is reasonably good in that the pain typically resolves
spontaneously. However, complete restoration of strength is not
always achieved. Non-operative treatment is the accepted
protocol, although recovery from this disorder can be quite
protracted.
References:
1. J. D. Spillane:Localised
neuritis of the shoulder girdle. The Lancet, London, 1943,
ii: 532-535
2. Mulvey, D. A.; Aquilina, R. J.; Elliott, M. W.; Moxham, J.;
and Green,M.: Diaphragmatic dysfunction in neuralgic amyotrophy;
anelectrophysiologic evaluation of 16 patients presenting with
dyspnea. Am. Rev. Respir. Dis.1993; 147:66-71.
3. Magee, K. R.; and DeJong, R. N.: Paralytic brachial neuritis.
J. Am. Med. Assn. 1960; 174:1258-1262.
4. Parsonage, M. J.; and Turner, J. W. A.: Neuralgic amyotrophy.
The shoulder-girdle syndrome. Lancet 1948; 1:973-978.
5. Dillin, L.; Hoaglund, F. T.; and Scheck, M.: Brachial
neuritis. J. Bone and Joint Surg. July 1985; 67-A:878-883.
6. Misamore, Gary W., Lehman, Daniel E., Indianapolis,
Indiana:Parsonage Turner Syndrome (Acute Brachial Neuritis) J Bone Joint Surg [Am] 1996; 78-A; 1405-8
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