Respiratory failure and hypoventilation secondary to neurosarcoidosis. 1998

L R Robinson, and R Brownsberger, and G Raghu
Department of Rehabilitation Medicine, University of Washington School of Medicine, Seattle, USA.

We report a patient manifesting severe hypoventilation resulting from diaphragmatic paresis secondary to sarcoid. A 34yr-old female presented with chest pain. Chest radiograph revealed hilar adenopathy and left pleural effusion; computed tomography scan showed a 2-cm solid lesion in the aorto-pulmonary window. Bronchoscopy and thoracentesis failed to further identify the nature of the lesion. An episode of aspiration resulted in cardiopulmonary arrest, necessitating cardiopulmonary resuscitation. Electrodiagnostic findings were consistent with bilateral phrenic neuropathy with axon loss, suggesting a poor prognosis. Biopsy via mediastinoscopy revealed noncaseating confluent granulomas with many multinucleated epithelioid histiocytes consistent with the diagnosis of sarcoidosis. Eight months after initial diagnosis and immunosuppressive treatment, the patient was successfully extubated and became ambulatory. Upon tapering her prednisone, however, she became dyspneic and manifested hypoventilation secondary to muscle weakness. Her corticosteroids were increased, but she eventually died of an opportunistic lung infection 2 yr later. This case establishes phrenic neuropathy and peripheral polyneuropathy secondary to neurosarcoidosis as a cause of respiratory failure. It also illustrates the utility of phrenic and diaphragmatic electrodiagnosis in the evaluation as well as prognostication of such lesions.

UI MeSH Term Description Entries
D007040 Hypoventilation A reduction in the amount of air entering the pulmonary alveoli. Hypoventilations
D010523 Peripheral Nervous System Diseases Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. Peripheral Nerve Diseases,Peripheral Neuropathies,PNS (Peripheral Nervous System) Diseases,PNS Diseases,Peripheral Nervous System Disease,Peripheral Nervous System Disorders,Nerve Disease, Peripheral,Nerve Diseases, Peripheral,Neuropathy, Peripheral,PNS Disease,Peripheral Nerve Disease,Peripheral Neuropathy
D010791 Phrenic Nerve The motor nerve of the diaphragm. The phrenic nerve fibers originate in the cervical spinal column (mostly C4) and travel through the cervical plexus to the diaphragm. Nerve, Phrenic,Nerves, Phrenic,Phrenic Nerves
D012133 Respiratory Paralysis Complete or severe weakness of the muscles of respiration. This condition may be associated with MOTOR NEURON DISEASES; PERIPHERAL NERVE DISEASES; NEUROMUSCULAR JUNCTION DISEASES; SPINAL CORD DISEASES; injury to the PHRENIC NERVE; and other disorders. Diaphragmatic Paralysis,Paralysis, Respiratory Muscle,Muscle Paralyses, Respiratory,Muscle Paralysis, Respiratory,Paralysis, Diaphragmatic,Paralysis, Respiratory,Respiratory Muscle Paralysis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012507 Sarcoidosis An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands. Besnier-Boeck Disease,Boeck's Sarcoid,Besnier-Boeck-Schaumann Syndrome,Boeck Disease,Boeck's Disease,Schaumann Disease,Schaumann Syndrome,Schaumann's Syndrome,Besnier Boeck Disease,Besnier Boeck Schaumann Syndrome,Boeck Sarcoid,Boecks Disease,Boecks Sarcoid,Disease, Schaumann,Sarcoid, Boeck's,Sarcoidoses,Schaumann's Syndromes,Syndrome, Besnier-Boeck-Schaumann,Syndrome, Schaumann,Syndrome, Schaumann's

Related Publications

L R Robinson, and R Brownsberger, and G Raghu
May 2021, Anesthesia and analgesia,
L R Robinson, and R Brownsberger, and G Raghu
November 1998, Archives of physical medicine and rehabilitation,
L R Robinson, and R Brownsberger, and G Raghu
June 2000, Sleep,
L R Robinson, and R Brownsberger, and G Raghu
September 1961, The American journal of cardiology,
L R Robinson, and R Brownsberger, and G Raghu
August 1990, Kokyu to junkan. Respiration & circulation,
L R Robinson, and R Brownsberger, and G Raghu
April 2001, Journal of the American Geriatrics Society,
L R Robinson, and R Brownsberger, and G Raghu
March 2020, Clinical case reports,
L R Robinson, and R Brownsberger, and G Raghu
June 2000, Sleep,
L R Robinson, and R Brownsberger, and G Raghu
August 2021, BMJ case reports,
L R Robinson, and R Brownsberger, and G Raghu
October 2008, Archives of neurology,
Copied contents to your clipboard!