P Parsonage-Turner syndrome (PTS) also known
as acute brachial neuritis, idiopathic brachial plexus neuropathy, brachial
plexus neuritis or neuralgic amyotrophy following an influenza vaccination is
an uncommon but clinically important presentation [1]. The incidence of this
syndrome is about two to three individuals per every 100,000 people, most
commonly between the 3rd and 7th decades of life. This syndrome has also shown
a greater predilection for males over females [2].
Its pathophysiology remains obscure, and the
majority of the cases have been ascribed to surgical procedures, trauma, recent
viral infections (varicella virus, herpes simplex, HIV, Coxsackie B virus,
Hepatitis B virus, Hepatitis C virus, Epstein-Barr virus, cytomegalovirus,
SARS-CoV2), autoimmune disorders such as systemic lupus erythematosus,
polyarteritis nodosa, temporal arteritis, and vaccinations [3-6]. Most occurred
after polio, chickenpox, hepatitis B, influenza, and HPV immunizations.
However, post-vaccination PTS is an infrequent entity, with only 4.3-15% of all
cases being attributed to vaccines [7]. Possible immune-mediated mechanisms
include molecular mimicry and bystander activation, both of which may ensue
following either infection (e.g., hepatitis E and SARS-CoV-2 [6]), or
vaccination.
PTS commonly presents with acute, diffuse
shoulder girdle and upper arm pain followed by proximal upper extremity
weakness, commonly in the muscles innervated by the upper plexus
(supraspinatus, infraspinatus, serratus anterior, deltoid, and biceps) [8].
This syndrome most commonly affects the upper trunk of the brachial plexus,
suprascapular nerve, long thoracic nerve, axillary nerve, and anterior
interosseous nerve [4]. Progressive neurological deficits, including weakness,
atrophy, and occasionally sensory abnormalities usually appear between 2 and 6
weeks [9]. Discomfort can last for several weeks, with one study reporting more
than 10% of patients having initial pain lasting more than 60 days and more
than 75% of patients who experienced two additional phases of
position-dependent neuropathic pain lasting several months. This same study
documented pain primarily at night in 60% of patients [10].
Differential diagnosis of PTS includes focal
extremity pathologies including subacromial bursitis, facioscapulohumeral
dystrophy, adhesive capsulitis, or other nervous pathologies including
radiculopathy, entrapment neuropathies, multifocal motor neuropathy, hereditary
neuropathy, and mononeuritis multiplex [11,12]. Intrinsic hourglass-like
constrictions of affected nerves or nerve fascicles have been recognized in the
acute (?4 weeks) phase of PTS [13] to date, they have not been observed in
other spontaneous neuropathies [13,14].
Diagnosis is further complicated by the
heterogeneity of symptoms among patients, which vary according to the nerves
injured and the speed at which the disease progresses [12]. Therefore, a
comprehensive approach involving accurate history taking, physical examination,
and specific tests (e.g., electromyography and brachial plexus magnetic
resonance imaging [MRI]) is required to ensure a proper diagnosis, while a
delay of diagnosis and treatment may result in lasting functional damage [15].
There is no consensus on the treatment of
PTS, but treatment generally involves conservative measures such as analgesia,
corticosteroids, and physical therapy [4]. A proposed protocol for
corticosteroid treatment is oral prednisone at 1 mg/kg/day for 1–2 weeks
followed by a taper-off over an additional 1–2 weeks [4,16]. Recently, there
has been some degree of evidence suggesting benefits from the administration of
immunoglobulins, with some patients responding positively, but there is little
data, and more rigorous research is required to determine their ultimate
efficacy [17]. PTS is typically a self-resolving process, with 80-90% recovery
of muscle strength within 2-3 years, but residual paresis and exercise
intolerance can happen in up to 70% of patients [18,12].
Presently, a causal relationship between PTS
and the influenza vaccine has been suggested by only a few case reports, hence
our case is significant as it adds weight to the existing literature. Our
patient presented with PTS after receiving the influenza vaccine, presenting
symptoms being severe upper extremity pain and weakness. Other etiologies of
the shoulder pain were ruled out by doing a CT scan that showed calcific
tendinitis and no frank rotator cuff tear. EMG/NCS was also indicative of
brachial plexopathy.