Parsonage-Turner syndrome: an unusual cause of hemidiaphragmatic paralysis Patrick J. Sweeney, M.D. Asa J. Wilbourn, M.D. Department of Neurology Muzaffar Ahmad, M.D. Department of Pulmonary Disease John P. Conomy, M.D. Department of Neurology Parsonage-Turner syndrome is a well-defined, idiopathic neurological disorder characterized by abrupt onset of unilateral shoulder girdle and arm pain followed by weakness, wasting and atrophy of the proximal or, occasionally, distal musculature of the involved extremity. Several unusual features of the syndrome have been described. 1 In this report, we present another of these variations, hemidia- phragmatic paralysis. Case report A 42-year-old right-handed woman presented for eval- uation of right shoulder and arm pain. Six weeks earlier she had suffered from a flulike illness characterized by fever, myalgia and nausea. Shortly thereafter, she expe- rienced neck pain, which radiated across the epaulet of the right shoulder into the shoulder itself and subse- quently down into the right arm and right anterior chest. The pain was initially severe, requiring narcotic analge- sics for relief. Two weeks after the onset of discomfort, she experienced weakness in the right upper extremity. She had been treated for arterial hypertension in the past. She was initially hospitalized at another institution for suspected cervical disc disease and cervical radicu- lopathy. General admission physical examination revealed an alert, cooperative, pleasant woman complaining of pain and weakness in the right arm. The blood pressure varied between 180/110 and 160/110 mm Hg. The general 145 require permission. on October 5, 2022. For personal use only. All other uses www.ccjm.org Downloaded from
Parsonage-Turner syndrome: an unusual cause of hemidiaphragmatic paralysis
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Parsonage-Turner syndrome: an unusual cause of hemidiaphragm paralysisDepartment of Neurology
Muzaffar Ahmad, M.D.
Department of Neurology
Parsonage-Turner syndrome is a well-defined, idiopathic neurological disorder characterized by ab rup t onset of unilateral shoulder girdle and arm pain followed by weakness, wasting and atrophy of the proximal or, occasionally, distal musculature of the involved extremity. Several unusual features of the syndrome have been described.1 In this report, we present another of these variations, hemidia- phragmat ic paralysis.
Case report
A 42-year-old right-handed woman presented for eval- uation of right shoulder and arm pain. Six weeks earlier she had suffered from a flulike illness characterized by fever, myalgia and nausea. Shortly thereafter, she expe- rienced neck pain, which radiated across the epaulet of the right shoulder into the shoulder itself and subse- quently down into the right arm and right anterior chest. The pain was initially severe, requiring narcotic analge- sics for relief. Two weeks after the onset of discomfort, she experienced weakness in the right upper extremity. She had been treated for arterial hypertension in the past. She was initially hospitalized at another institution for suspected cervical disc disease and cervical radicu- lopathy.
General admission physical examination revealed an alert, cooperative, pleasant woman complaining of pain and weakness in the right arm. The blood pressure varied between 180/110 and 160/110 mm Hg. The general
145
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146 Cleveland Clinic Quarter ly Vol. 49, No. 3
Fig. 1. Admiss ion PA view of the chest shows e levated right h e m i d i a p h r a g m .
physical examination was otherwise within normal limits. Neurologic abnormalities con- sisted of early hypertensive retinal change noted in both fundi. Weakness was noted in the right supraspinatus, deltoid, biceps and serratus anterior with winging of the right scapula. Right biceps reflex was absent.
Admission chest radiograph showed ele- vation and fixation of the right hemidia- phragm (Fig. I). (A plain film of the chest taken 18 months earlier had demonstrated normal position of the diaphragm.) Paradox- ical motion was observed at fluoroscopy. Plain films of the cervical spine, myelograms, and CSF examination were unremarkable.
Extensive electromyography (EMG) was performed. The motor amplitudes recording deltoid and biceps were borderline low; otherwise, all motor and sensory nerve con- duction study results were well within the normal range. On needle examination, fibrillations and severe motor unit potential loss were seen in the right serratus anterior, with lesser but similar changes in the right biceps, deltoid, infraspinatus and supra- spinatus muscles. Although the EMG find- ings were consistent with both a right C6
radiculopathy and acute brachial plexitis, the latter was considered the more likely possibility because of the following: (1) ab- sence of fibrillations in cervical paraspinal muscles; (2) abundant fibrillations and mo- tor unit potential loss in the serratus anterior; and (3) clinical winging of the scapula.
Discussion
The brachial plexus (Fig. 2) is formed by the ventral rami of C5-8 and Ti with variable contributions from C4 and T2. At the lateral border of the scalenus muscles, the roots unite to form the upper, middle and lower trunks of the plexus. These subsequently divide be- hind the clavicle to form cords that ultimately disperse into the upper axilla as the major nerve trunks to the shoul- ders, upper chest, back and arms. T h e phrenic nerve derives primarily from C^ with contributions from C3 and CR, as well; it provides the major motor inner- vation to the d iaphragm.
Parsonage-Turner syndrome has a va-
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
Fall 1982 Parsonage-Turner syndrome 147
Fig. 2. S impl i f ied scheme of the a n a t o m y of the b rach ia l plexus.
riety of rubrics, the most common being idiopathic brachial plexopathy. In 1943, Spillane was the first to delineate the disorder, followed by Parsonage and Turner 3 in 1948 who described the 136 cases that constituted their entire World War II experience with the syndrome. It has been described following nonspecific upper respiratory tract infections, vac- cination, and administration of foreign sera, the latter circumstance raising the possibility of allergic mechanisms. T h e disorder may be viral in etiology.4
The predominant symptom is abrup t onset of severe shoulder girdle pain fol- lowed by weakness and atrophy of the shoulder girdle musculature and loss of appropriate reflexes. T h e majority of
patients are between the third and sev- enth decades in age, with a male pre- dominance of 2.5 to 1. An afebrile ill- ness, it is not associated with systemic signs. T h e most common nerve-muscle involvement has been the serratus an- terior (long thoracic nerve), supra- spinatus and infraspinatus (suprascap- ular nerve), deltoid (circumflex-axil- lary nerve), biceps (musculocutaneous nerve), and triceps (radial nerve).
T h e differential diagnosis includes cervical spondylosis, cervical radicu- lopathy, extradural tumor, and primary rotator cuff injuries of the shoulder joint.
Several other conditions resulting in phrenic nerve injury must also be con- sidered: (1) lung carcinoma; (2) sur-
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
148 Cleveland Clinic Quarter ly Vol. 49, No. 3
gically induced atelectasis, or thoracot- omy for pulmonary tuberculosis; (3) neck surgery; and (4) idiopathic. [In view of the association of a rm pain and the clinical findings in our patient , how- ever, the best diagnosis was Parsonage- Turne r syndrome.]
Despite the severity and extent of the lesion, overall prognosis is excellent. Re- covery usually begins within a month or two but may not be complete for u p to two years.
T h e first reported association of h e m i d i a p h r a g m a t i c pa ra lys i s w i t h brachial plexitis appeared in the French literature in 1950.5 Since then, several case reports have been published. In all cases, paralysis has been ipsilateral to the plexus involvement. T h e mode of onset is difficult to ascertain as it may exist without significant impairment of respiration. In symptomatic patients, onset was abrupt , like the pain of brach- ial plexopathy. In some patients, dia- phragmat ic paralysis, unlike the revers- ible shoulder and extremity weakness, may be permanent . T h e incidence of
diaphragmatic dysfunction in Parson- age-Turner syndrome is not known.
In a small number of cases, awareness of this neurologic entity may afford an explanation to an otherwise mysterious paralysis of the hemidiaphragm.
References
1. Renne l s G D , O c h o a J . Neura lg ic a m y o t r o p h y m a n i f e s t i n g as an te r io r interosseous nerve palsy. Musc l e Nerve 1980; 3: 160-164.
2. Spi l lane J D . Localised neuri t is of t h e shoulder girdle. R e p o r t of 46 cases in M E F . Lance t 1943; 2: 532-535 .
3. Pa r sonage M J , T u r n e r J W . Neura lg ic a m y - o t rophy ; the shoulder -g i rd le syndrome. L a n - cet 1948; 1: 973-978 .
4. Tsa i r i s P, Dyck PJ , M u l d e r D W . N a t u r a l history of b rach ia l plexus n e u r o p a t h y . Repo r t on 99 pa t ien ts . Arch Neuro l 1972; 27: 109- 117.
5. A l a j o u a n i n e T , Bla t r ix C . Paralysie amy- o t r o p h i q u e des muscles per iscapulaires , bi lat- érale et a symét r ique , a d é b u t b r u s q u e et dou- loureux. Rev Neuro l 1950: 82: 280-281.
6. C a p e CA, F i n c h a m R W . Para ly t ic b rach ia l neur i t i s wi th d i a p h r a g m a t i c paralysis; con t ra - la tera l recurrence . Neuro logy 1965; 15: 191- 193.
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
Muzaffar Ahmad, M.D.
Department of Neurology
Parsonage-Turner syndrome is a well-defined, idiopathic neurological disorder characterized by ab rup t onset of unilateral shoulder girdle and arm pain followed by weakness, wasting and atrophy of the proximal or, occasionally, distal musculature of the involved extremity. Several unusual features of the syndrome have been described.1 In this report, we present another of these variations, hemidia- phragmat ic paralysis.
Case report
A 42-year-old right-handed woman presented for eval- uation of right shoulder and arm pain. Six weeks earlier she had suffered from a flulike illness characterized by fever, myalgia and nausea. Shortly thereafter, she expe- rienced neck pain, which radiated across the epaulet of the right shoulder into the shoulder itself and subse- quently down into the right arm and right anterior chest. The pain was initially severe, requiring narcotic analge- sics for relief. Two weeks after the onset of discomfort, she experienced weakness in the right upper extremity. She had been treated for arterial hypertension in the past. She was initially hospitalized at another institution for suspected cervical disc disease and cervical radicu- lopathy.
General admission physical examination revealed an alert, cooperative, pleasant woman complaining of pain and weakness in the right arm. The blood pressure varied between 180/110 and 160/110 mm Hg. The general
145
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
146 Cleveland Clinic Quarter ly Vol. 49, No. 3
Fig. 1. Admiss ion PA view of the chest shows e levated right h e m i d i a p h r a g m .
physical examination was otherwise within normal limits. Neurologic abnormalities con- sisted of early hypertensive retinal change noted in both fundi. Weakness was noted in the right supraspinatus, deltoid, biceps and serratus anterior with winging of the right scapula. Right biceps reflex was absent.
Admission chest radiograph showed ele- vation and fixation of the right hemidia- phragm (Fig. I). (A plain film of the chest taken 18 months earlier had demonstrated normal position of the diaphragm.) Paradox- ical motion was observed at fluoroscopy. Plain films of the cervical spine, myelograms, and CSF examination were unremarkable.
Extensive electromyography (EMG) was performed. The motor amplitudes recording deltoid and biceps were borderline low; otherwise, all motor and sensory nerve con- duction study results were well within the normal range. On needle examination, fibrillations and severe motor unit potential loss were seen in the right serratus anterior, with lesser but similar changes in the right biceps, deltoid, infraspinatus and supra- spinatus muscles. Although the EMG find- ings were consistent with both a right C6
radiculopathy and acute brachial plexitis, the latter was considered the more likely possibility because of the following: (1) ab- sence of fibrillations in cervical paraspinal muscles; (2) abundant fibrillations and mo- tor unit potential loss in the serratus anterior; and (3) clinical winging of the scapula.
Discussion
The brachial plexus (Fig. 2) is formed by the ventral rami of C5-8 and Ti with variable contributions from C4 and T2. At the lateral border of the scalenus muscles, the roots unite to form the upper, middle and lower trunks of the plexus. These subsequently divide be- hind the clavicle to form cords that ultimately disperse into the upper axilla as the major nerve trunks to the shoul- ders, upper chest, back and arms. T h e phrenic nerve derives primarily from C^ with contributions from C3 and CR, as well; it provides the major motor inner- vation to the d iaphragm.
Parsonage-Turner syndrome has a va-
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
Fall 1982 Parsonage-Turner syndrome 147
Fig. 2. S impl i f ied scheme of the a n a t o m y of the b rach ia l plexus.
riety of rubrics, the most common being idiopathic brachial plexopathy. In 1943, Spillane was the first to delineate the disorder, followed by Parsonage and Turner 3 in 1948 who described the 136 cases that constituted their entire World War II experience with the syndrome. It has been described following nonspecific upper respiratory tract infections, vac- cination, and administration of foreign sera, the latter circumstance raising the possibility of allergic mechanisms. T h e disorder may be viral in etiology.4
The predominant symptom is abrup t onset of severe shoulder girdle pain fol- lowed by weakness and atrophy of the shoulder girdle musculature and loss of appropriate reflexes. T h e majority of
patients are between the third and sev- enth decades in age, with a male pre- dominance of 2.5 to 1. An afebrile ill- ness, it is not associated with systemic signs. T h e most common nerve-muscle involvement has been the serratus an- terior (long thoracic nerve), supra- spinatus and infraspinatus (suprascap- ular nerve), deltoid (circumflex-axil- lary nerve), biceps (musculocutaneous nerve), and triceps (radial nerve).
T h e differential diagnosis includes cervical spondylosis, cervical radicu- lopathy, extradural tumor, and primary rotator cuff injuries of the shoulder joint.
Several other conditions resulting in phrenic nerve injury must also be con- sidered: (1) lung carcinoma; (2) sur-
require permission. on October 5, 2022. For personal use only. All other useswww.ccjm.orgDownloaded from
148 Cleveland Clinic Quarter ly Vol. 49, No. 3
gically induced atelectasis, or thoracot- omy for pulmonary tuberculosis; (3) neck surgery; and (4) idiopathic. [In view of the association of a rm pain and the clinical findings in our patient , how- ever, the best diagnosis was Parsonage- Turne r syndrome.]
Despite the severity and extent of the lesion, overall prognosis is excellent. Re- covery usually begins within a month or two but may not be complete for u p to two years.
T h e first reported association of h e m i d i a p h r a g m a t i c pa ra lys i s w i t h brachial plexitis appeared in the French literature in 1950.5 Since then, several case reports have been published. In all cases, paralysis has been ipsilateral to the plexus involvement. T h e mode of onset is difficult to ascertain as it may exist without significant impairment of respiration. In symptomatic patients, onset was abrupt , like the pain of brach- ial plexopathy. In some patients, dia- phragmat ic paralysis, unlike the revers- ible shoulder and extremity weakness, may be permanent . T h e incidence of
diaphragmatic dysfunction in Parson- age-Turner syndrome is not known.
In a small number of cases, awareness of this neurologic entity may afford an explanation to an otherwise mysterious paralysis of the hemidiaphragm.
References
1. Renne l s G D , O c h o a J . Neura lg ic a m y o t r o p h y m a n i f e s t i n g as an te r io r interosseous nerve palsy. Musc l e Nerve 1980; 3: 160-164.
2. Spi l lane J D . Localised neuri t is of t h e shoulder girdle. R e p o r t of 46 cases in M E F . Lance t 1943; 2: 532-535 .
3. Pa r sonage M J , T u r n e r J W . Neura lg ic a m y - o t rophy ; the shoulder -g i rd le syndrome. L a n - cet 1948; 1: 973-978 .
4. Tsa i r i s P, Dyck PJ , M u l d e r D W . N a t u r a l history of b rach ia l plexus n e u r o p a t h y . Repo r t on 99 pa t ien ts . Arch Neuro l 1972; 27: 109- 117.
5. A l a j o u a n i n e T , Bla t r ix C . Paralysie amy- o t r o p h i q u e des muscles per iscapulaires , bi lat- érale et a symét r ique , a d é b u t b r u s q u e et dou- loureux. Rev Neuro l 1950: 82: 280-281.
6. C a p e CA, F i n c h a m R W . Para ly t ic b rach ia l neur i t i s wi th d i a p h r a g m a t i c paralysis; con t ra - la tera l recurrence . Neuro logy 1965; 15: 191- 193.
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