Case Report. Severe Asthma Complicated by Bilateral Diaphragmatic Paralysis Attributed to Parsonage-Turner Syndrome

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1 Case Report Severe Asthma Complicated by Bilateral Diaphragmatic Paralysis Attributed to Parsonage-Turner Syndrome DAVID L. PATTERSON, M.D., RICHARD A. DEREMEE, M.D., AND LOREN W. HUNT, M.D. Progressive dyspnea that developed in a 52-year-old woman with a lifelong history of asthma did not respond to high-dose orally administered glucocorticoids. Initially, a diagnosis of allergic bronchopulmonary aspergillosis or hypersensitivity pneumonia was suggested as the cause of the worsening dyspnea. Pulmonary function tests demonstrated severe airway obstruction; substantial improvement was noted after bronchodilator therapy. Maximal inspiratory pressure was decreased, and the diffusing capacity of the lungs was abnormal. Computed tomography of the chest showed no parenchyma! or mediastinal abnormalities. During a sniff test, fluoroscopy of her diaphragm disclosed paradoxical motion of both hemidiaphragms during inspiration, consistent with bilateral hemidiaphragmatic paralysis. Parsonage-Turner syndrome was diagnosed. The dose of glucocorticoids was tapered. Follow-up of the patient by telephone contact in March 1994 (9 months after her initial examination at our clinic) revealed that the dyspnea was still severe. (Mayo Clin Proc 1994; 69: ) PTS = Parsonage-Turner syndrome Parsonage-Turner syndrome (PTS), also known as paralytic brachial neuritis, neuralgic amyotrophy, or brachial plexus neuropathy, was named after two neurologists who thoroughly described 136 cases of this disorder in 1948.' The syndrome consists of sudden onset of severe pain in the shoulder, chest, or upper arm (or in a combination of these areas). The pain persists for hours to days and is followed by paralysis or paresis of the shoulder girdle or arm muscles (or both) in the distribution of a nerve, nerve root, or plexus. Sensory changes often accompany the motor deficits. Involvement of the phrenic nerve (or nerves) that leads to hemidiaphragmatic or bilateral diaphragmatic paralysis is rare. Herein we describe a patient with asthma who had worsening dyspnea attributed to PTS and bilateral diaphragmatic paralysis. REPORT OF CASE A 52-year-old woman was examined because of progressive dyspnea that was thought to be attributable to asthma but was unresponsive to high-dose orally administered glucocorticoids. She had had asthma since childhood; exacerbations From the Division of Allergic Diseases and Internal Medicine (D.L.P., L.W.H.) and Division of Pulmonary and Critical Care Medicine (R.A.D.), Mayo Clinic Rochester, Rochester, Minnesota. Address reprint requests to Dr. L. W. Hunt, Division of Allergic Diseases, Mayo Clinic Rochester, 200 First Street SW, Rochester, MN were frequent during the spring and fall of each year. During the 2 years before she came to the Mayo Clinic (1991 and 1992), frequent bursts of orally administered glucocorticoids were given to control her asthma. In March 1993, the patient had sudden onset of excruciating pain in the left shoulder and dyspnea but no wheezing, cough, malaise, fever, or other systemic symptoms. No recent history of trauma was recalled. Three weeks later, the pain in her left shoulder had diminished, but her right shoulder began to hurt. The dyspnea progressed until she had dyspnea at rest, especially when supine. The dose of prednisone was increased to 40 mg/day, but her symptoms did not abate. The dyspnea remained severe; she had to sleep upright in a chair, and use of an inhaled ß-agonist every 2 to 4 hours was necessary. An assessment at our clinic in June 1993 disclosed an afebrile woman with cushingoid appearance and tachypnea but no audible wheezing; decreased bibasilar breath sounds and diaphragmatic excursion of less than 2 cm (normal, 3 to 5) were noted. When supine, she experienced dyspnea with resultant pursed-lip breathing and tachypnea. A neurologic examination disclosed only mild weakness of the left biceps. Electrocardiographic findings and results of a complete blood cell count and serum protein electrophoresis were normal, as were the rheumatoid factor, erythrocyte sedimentation rate (Westergren method), antinuclear antibody, and α,-antitrypsin levels. Initially, a diagnosis of allergic Mayo Clin Proc 1994; 69: Mayo Foundation for Medical Education and Research

2 Mayo Clin Proc, August 1994, Vol 69 DIAPHRAGMATIC PARALYSIS AND PARSONAGE-TURNER SYNDROME 775 bronchopulmonary aspergillosis or hypersensitivity pneumonitis was suggested as an explanation for the worsening dyspnea. Results of a radioallergosorbent test for IgE antibody specific to aspergillosis and sérologie studies for farmer's lung antigens were negative. A chest roentgenogram demonstrated elevation of both hemidiaphragms (Fig. 1 ). Results of the pulmonary function tests performed at our institution and elsewhere are shown in Table 1. The pulmonary function tests performed at our institution demonstrated severe airway obstruction; substantial improvement was noted after bronchodilator therapy. Maximal inspiratory pressure was decreased, an indication of a neuromuscular abnormality. The diffusing capacity of the lungs was abnormal, and the patient had mild oxygen desaturation during exercise. Computed tomography of the chest was performed to exclude parenchymal lung disease or a mediastinal process that could have been impinging on her phrenic nerves; no parenchymal or mediastinal abnormalities were noted. This scan was done with the patient in the lateral decubitus position because the dyspnea was too severe for her to be supine. During a sniff test, fluoroscopy of her diaphragm revealed paradoxical motion of both hemidiaphragms during inspiration, consistent with bilateral hemidiaphragmatic paralysis. PTS was diagnosed. The dose of glucocorticoids was tapered, and long-term observation is under way to determine whether she regains function of one or both hemidiaphragms. Follow-up of the patient by telephone in March 1994 revealed that her condition had improved only slightly. She was still unable to lie flat because of dyspnea, and daily activities reportedly cause severe dyspnea. The results of the pulmonary function tests done at her local hospital 6 months after she was examined at our clinic are shown in Table 1. DISCUSSION Before 1940, the medical literature had only isolated reports of brachial neuritis. In 1943, Spillane2 published an analysis of 46 cases of "localized neuritis of the shoulder girdle," and in 1948 and 1957, Parsonage and Turner' 3 described two large series of patients with the syndrome that now bears their names. On the basis of a study by Magee and DeJong,4 PTS has been found to occur regularly in the United States. In our experience, however, this syndrome is uncommon. Diagnoses that have been confused with PTS (not involving the diaphragm) include poliomyelitis, spinal cord tumors, ruptured intervertébral cervical disks, cervical spondylosis, and amyotrophic lateral sclerosis. We believed that these conditions could be excluded in our patient on the basis of her history and physical examination findings, including a complete neurologic assessment. Fig. 1. Chest roentgenogram of 52-year-old woman with severe dyspnea, demonstrating elevation of both hemidiaphragms. The diagnosis of PTS is based on the clinical history and physical findings. A typical clinical history is sudden, predominantly nocturnal onset of severe constant pain localized to the shoulder and upper arm, but occasionally it involves the elbow and forearm. After a variable period (usually hours to weeks), the patient experiences atrophie paralysis or paresis of various muscles in the region affected by the pain. The paralysis or paresis commonly begins as the pain subsides. Cutaneous sensory changes, particularly in the distribution of the circumflex nerve, occur in approximately 25% of patients. Patients with PTS are afebrile and have no constitutional symptoms. Analysis of the cerebrospinal fluid during an acute attack has failed to demonstrate abnormalities. The pathologic process may involve one or more peripheral nerves or may be localized to nerve roots or the spinal cord.5 The disease may involve the upper, lower, or entire brachial plexus and the plexus bilaterally in up to 25% of cases,6 and it may recur in approximately 5% of patients.7 Familial types of brachial plexus neuropathy have been reported,8 and lumbosacral plexus neuropathy, a possible counterpart to brachial plexus neuropathy, has also been described.9 Multiple causes for this disease, including previous administration of foreign sera or prior vaccination, have been proposed, but to date, none has been proved; thus, the etiologic factor remains obscure. Treatment of PTS consists of analgesics to control the pain and physical therapy to prevent stiffness.10 Apparently, glucocorticoids have no role in the treatment of this syndrome. The prognosis for patients with

3 776 DIAPHRAGMATIC PARALYSIS AND PARSONAGE-TURNER SYNDROME Mayo Clin Proc, August 1994, Vol 69 Table 1. Results of Pulmonary Function Tests of 52-Year-Old Woman With Dyspnea, Stratified by Date* Actual October 1991f FEÏWO^) TLC(L) Deo (ml/min/mm Hg) Actual May 1993f FEF 2, 7W (L/s) TLC (L) Deo (ml/min/mm Hg) Normal Range Actual Actual % change June TLC (L) RV/TLC (%) FEV,(L) FEF * (L/s) Deo (ml/min/mm Hg ) Pimax PEmax >3.88 >2.54 <2.22 <44.9 >2.54 >2.13 >70.9 >1.1 >68 >16 <-53 > / December 1993t FEP^i» (^) For footnotes to table, see page

4 Mayo Clin Proc, August 1994, Vol 69 DIAPHRAGMATIC PARALYSIS AND PARSONAGE-TURNER SYNDROME 777 uncomplicated PTS is good; most patients recover completely within 3 years after the onset." Rarely, PTS involves one or both phrenic nerves, an outcome that leads to hemidiaphragmatic or bilateral diaphragmatic paralysis. This situation can occur because the phrenic nerve is derived from the brachial plexus (primarily from C4 with variable contributions from C3 and C5). Forty-six cases of hemidiaphragmatic or bilateral diaphragmatic involvement have been reported in the literature 1214 since Alajouanine and associates 15 first described diaphragmatic involvement in PTS in Our case illustrates several features of involvement of the diaphragm with PTS. Dyspnea and inability to tolerate being supine are typical. Although some patients with PTS have bilateral symptoms and multiple muscles involved on clinical examination, one or both of these findings are not always present. Depending on the duration of symptoms and extent of brachial plexus involvement, patients may initially have only unilateral involvement of one muscle (as with our patient), and they may have no sensory abnormalities. Many such patients (including our patient) are unable to sleep lying flat because of breathlessness. Despite this problem with sleeping, no pronounced nocturnal alveolar hypoventilation is evident in these patients, especially in the absence of lung disease.' 4 Chest roentgenograms invariably show one or both hemidiaphragms to be elevated, but this finding can be confused with poor inspiratory effort. Typically, pulmonary function studies demonstrate a severely decreased peak maximal inspiratory pressure and a global reduction in lung volumes in conjunction with preservation of the diffusing capacity. We speculate that the diffusing capacity was low in our patient because of bibasilar atelectasis and intrapulmonary shunting.' Treatment for such patients involves supportive care and a period of observation to determine whether they recover diaphragmatic function. Some patients need to sleep in a sitting or semireclining position when paralysis is bilateral. A recent study by Mulvey and colleagues 14 was the first attempt to analyze the prognosis for recovery of diaphragmatic function in patients with PTS. Those who have phrenic nerve involvement have a poorer prognosis than do those without such involvement. In their series, most patients required many months to recover diaphragmatic function, and in some, no recovery was noted. Those patients who had bilateral phrenic nerve involvement seemed to have the worst prognosis. CONCLUSION Physicians should suspect PTS in patients who have sudden onset of severe pain in the shoulder or upper arm (with no constitutional symptoms or history of recent trauma) followed hours to days later by paralysis or paresis of a muscle or multiple muscles of the shoulder or arm (usually the upper arm). Such patients may also have slight sensory impairment, especially on the outer side of the upper arm. Diagnosis is based primarily on history and clinical examination findings. Electromyography and nerve conduction studies may help delineate which nerves and muscles are involved, but electromyography is not necessary for the diagnosis. If involvement of the diaphragm is suspected, this can be diagnosed by a combination of physical examination findings, decreased peak inspiratory pressure on pulmonary function testing, and paradoxical motion of one or both hemidiaphragms during fluoroscopy. Even though in our experience PTS is uncommon and only rarely causes diaphragmatic paralysis, we believe that physicians should be familiar with this syndrome. REFERENCES 1. Parsonage MJ, Turner JWA. Neuralgic amyotrophy: the shoulder-girdle syndrome. Lancet 1948;1: Spillane JD. Localised neuritis of the shoulder girdle: a report of 46 cases in the MEF. Lancet 1943;2: Turner JWA, Parsonage MJ. Neuralgic amyotrophy (paralytic brachial neuritis) with special reference to prognosis. Lancet 1957;2: Magee KR, DeJong RN. Paralytic brachial neuritis: discussion of clinical features with review of 23 cases. JAMA I960; 174: Vanermen B, Aertgeerts M, Hoogmartens M, Fabry G. The syndrome of Parsonage and Turner: discussion of clinical features with a review of 8 cases. Acta Orthop Belg 1991; 57: Michotte A, Dierckx R, Deleu D, Herregodts P, Schmedding E, Bruyland M, et al. Recurrent forms of sporadic brachial plexus neuropathy: a report of two cases. Clin Neural Neurosurg 1988;90:71-74 *Dco = diffusing capacity of the lungs for carbon monoxide; FEF = forced expiratory flow; FEV l = forced expiratory volume in 1 second; FRC = functional residual capacity; FVC = forced vital capacity; MVV = maximal voluntary ventilation; PEmax = maximal expiratory pressure; Pimax = maximal inspiratory pressure; RV = residual volume; Rx = treatment; TLC = total lung capacity; VC = vital capacity. ftests done at patient's local hospital. ttests done at the Mayo Clinic. Hemoglobin adjusted. //% of normal predicted.

5 778 DIAPHRAGMATIC PARALYSIS AND PARSONAGE-TURNER SYNDROME Mayo Clin Proc, August 1994, Vol Gregory RP, Loh L, Newsom-Davis J. Recurrent isolated 12. alternating phrenic nerve palsies: a variant of brachial neuritis? Thorax 1990;45: Smith BH, Ramakrishna T, Schlagenhauff RE. Familial bra- 13. chial neuropathy: two case reports with discussion. Neurology 1971;21: Evans BA, Stevens JC, Dyck PJ. Lumbosacral plexus neuropathy. Neurology 1981;31: Ferrini L, Delia Torre P, Perticoni G, Cantisani TA. Neuralgic amyotrophy of the shoulder girdle: the Parsonage- Turner syndrome. Ital J Orthop Traumatol 1986; 12: Tsairis P, Dyck PJ, Mulder DW. Natural history of brachial plexus neuropathy: report on 99 patients. ArchNeurol 1972; 27: Walsh NE, Dumitru D, Kalantri A, Roman AM Jr. Brachial neuritis involving the bilateral phrenic nerves. Arch Phys MedRehabil 1987;68:46-48 Pieters T, Lambert M, Huaux JP, Nagant De Deuxchaisnes C. Hemidiaphragmatic paralysis, an unusual presentation of Parsonage-Turner syndrome. Clin Rheumatol 1988; 7: Mulvey DA, Aquilina RJ, Elliott MW, Moxham J, Green M. Diaphragmatic dysfunction in neuralgic amyotrophy: an electrophysiologic evaluation of 16 patients presenting with dyspnea. Am Rev Respir Dis 1993;147:66-71 Alajouanine T, Thurel R, Blatrix C. Paralysie amyotrophique des muscles périscapulaires, bilatérale et asymétrique, à début brusque et douloureux. Rev Neurol 1950; 82: