Modeling of Lung Function Recovery in Neuralgic Amyotrophy With Diaphragm Impairment.

BACKGROUND: Neuralgic amyotrophy is an inflammatory peripheral nerve disorder in which phrenic nerve involvement can lead to diaphragm paralysis. The prevalence, magnitude, and time course of diaphragm recovery are uncertain. METHODS: This study modeled the course of recovery of lung function in 16 subjects with diaphragm impairment from neuralgic amyotrophy. The first and last available vital capacity, sitting-to-supine decline in vital capacity, and maximal inspiratory pressures were compared. RESULTS: An asymptotic regression model analysis in 11 subjects with at least partial recovery provided estimates of the vital capacity at onset (47%, 95% CI 25-68%), the final vital capacity (81%, 95% CI 62-101%), and the half-time to recovery (22 months, 95% CI 15-43 months). In those subjects, there was a significant improvement between the first and last measured FVC (median 44-66%, P = .004) and maximal inspiratory pressure (mean 34-51%, P = .004). Five subjects (31%) with complete recovery had a final sitting-to-supine drop of vital capacity of 16% and a maximal predicted inspiratory pressure of 63%. CONCLUSIONS: Sixty-nine percent of subjects with diaphragm impairment from neuralgic amyotrophy experience recovery of lung function and diaphragm strength, but recovery is slow and may be incomplete.

Obstructive fibrinous tracheal pseudomembrane.

We present 2 patients who had upper airway obstruction develop secondary to a fibrinous tracheal pseudomembrane after short-term endotracheal intubations. Early recognition and removal of the pseudomembrane resulted in complete resolution of symptoms. We discuss the characteristic presentation of symptoms, diagnosis, and management.

Concomitant Castleman's disease and sarcoidosis.

Castleman's disease (CD) is an atypical lymphoproliferative disorder characterized by hyperplasia of lymphoid tissue that may develop at a single site or throughout the body. This disorder has frequently been associated with several systemic syndromes, including human immunodeficiency virus infection, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome and various connective tissue diseases. However, there have been no previously reported cases of concomitant sarcoidosis and CD. In this report, the authors describe a young woman with an enlarging neck mass, biopsy of which showed histopathological features consistent with the hyaline vascular type of CD along with the presence of non-necrotizing granulomas and was deemed unresectable due to encasement of vital neural and vascular structures. Further studies revealed hypermetabolic generalized lymphadenopathy with pulmonary perilymphatic nodules. Bronchoscopic investigations demonstrated the presence of non-necrotizing granulomas within the lung parenchyma and mediastinal lymph nodes, a CD4(+) T-lymphocyte predominant bronchoalveolar lavage and an elevated CD4/CD8 ratio consistent with a concomitant diagnosis of sarcoidosis. Institution of immunosuppression with prednisone and methotrexate led to reduction in size of the neck mass that allowed radical curative resection of the CD.

Transudative chylothorax associated with sclerosing mesenteritis.

Transudative chylothorax is an uncommon type of chylous pleural effusion, typically secondary to chyle leak and a coexisting disorder such as heart failure or liver cirrhosis. Sclerosing mesenteritis is a rare inflammatory disease of the small bowel mesentery, and has once previously been reported as a cause of chylothorax. We present the case of an 81-year-old man with a right-side transudative chylothorax associated with congestive heart failure and sclerosing mesenteritis. We discuss potential mechanisms.