Neuralgic amyotrophy with phrenic nerve involvement

Abstract

Phrenic nerve involvement is a rare feature in patients with neuralgic amyotrophy (Parsonage–Turner syndrome). We report four patients who initially presented with severe dyspnea in the absence of lung disease. All patients had a history of infectious disease or surgery and of pain of sudden onset in the shoulder region. Weakness of the proximal arm was observed in only one. Radiographic and pulmonary function studies, phrenic nerve conduction studies, and needle electromyogram (EMG) of the diaphragm documented diaphragmatic paralysis which was unilateral in one patient, bilateral in two patients, and recurrent on alternating sides in another one. Follow‐up studies remained abnormal for up to 4 years. Neuralgic amyotrophy with phrenic nerve involvement should be considered in patients presenting with severe, unexplained dyspnea of sudden onset. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 437–442, 1999.

A 54-Year-Old Man with Acute Onset Orthopnea and Sleep-Related Hypoxia

“…1,2 The differential diagnoses for this patient's subacutely evolving bilateral diaphragmatic weakness included bilateral isolated phrenic neuropathy (BIPN), [3][4] neuralgic amyotrophy ([NA] also known as Parsonage-Turner Syndrome), [5][6][7] and amyotrophic lateral sclerosis. There was no evidence for other conditions which may rarely cause bilateral phrenic neuropathies, such as diabetes, 8 multifocal motor neuropathy with conduction block, 9 POEMS syndrome, 10 sarcoidosis, 11 arsenic poisoning, 12 prior irradiation, 13 or acid maltase deficiency.…”

“…[2][3][4] NA is a distinct and painful brachial plexus neuropathy causing patchy, multifocal paresis and sensory loss in one or both arms, with rare involvement of the phrenic nerves. [5][6][7] A NA variant known as familial brachial plexus neuritis (hereditary neuralgic amyotrophy [HNA]) may lead to recurrent episodes of pain, numbness, and weakness in one or both upper extremities, with episodes triggered by extreme upper extremity movements or postures, infections, immunizations, or stress. HNA has been genetically linked to chromosome 17q25, where mutations may occur in the septin-9 (SEPT9) gene.…”

“…15 Prognosis for recovery of normal function in affected limbs is generally favorable in NA, although in some cases persisting weakness may occur, and recurrent episodes may cause residual deficits. [5][6][7][15][16][17] Our patient was diagnosed with BIPN due to painless onset, subacutely evolving symmetrical diaphragmatic weakness, and absence of other upper limb symptoms or electromyographic denervation to suggest the alternative diagnosis of NA. His symptoms did not improve over time, making the diagnosis of idiopathic bilateral phrenic nerve paralysis much more likely than neuralgic amyotrophy.…”

Neuroradiologic and Neurophysiologic Findings of Neuralgic Amyotrophy

“…Paresis may occur within the first 2 weeks after the initial presentation 15) . Paresis involves the brachial plexus, including the long thoracic nerve and anterior interosseus nerve, and can sometimes affect the phrenic nerve or lower cranial nerve 2,9,13,22) . In most patients, improvement in motor power begins between the first and sixth months 22) .…”

Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea

“…Thermal trauma or direct laceration during dissection of the internal mammary artery in coronary artery revascularization [3,4] and surgery for tumors involving the phrenic nerve should be mentioned especially. Diaphragm paralysis can also be part of a neuropathological entity called neuralgic amyotrophy [5][6][7]. These patients have a history of a viral infection like a common cold or influenza and sudden pain in the shoulder or neck region before the onset of progressive dyspnea [5,6].…”