ATS 2021 will feature presentations of original research from accepted abstracts. Mini Symposia and Thematic Poster Sessions are abstract based sessions.
Please use the form below to browse scientific abstracts and case reports accepted for ATS 2021. Abstracts presented at the ATS 2021 will be published in the Online Abstract Issue of the American Journal of Respiratory and Critical Care Medicine, Volume 203, May 3, 2021.
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Acute Respiratory Failure Associated with Neuromyelitis Optica
Session Title
TP45 - TP045 DYSPNEA AND COUGH CASE REPORTS
Abstract
A2401 - Acute Respiratory Failure Associated with Neuromyelitis Optica
Author Block: S. Chauhan1, Q. Abdiani2, R. Dadhwal3, A. Shalonov4; 1Pulmonology, Jamaica Hospital Medical Center, Richmond Hills, NY, United States, 2Internal Medicine, Jamaica Hospital Medical Center, Richmond Hills, NY, United States, 3Pulmonology, Corpus Christi Medical Center, Corpus Christi, TX, United States, 4Pulmonology and Critical Care, Jamaica Hospital Medical Center, Richmond Hills, NY, United States.
Neuromyelitis Optica (NMO) is a rare spectrum of disorders caused by immune-mediated demyelination of the central nervous system. The prevalence is approximately 10 per 100000 [1] with an incidence higher in women affecting people in their thirties or forties. Hallmark features are signs of optic neuritis or transverse myelitis which is mostly longitudinally extensive over more than three spinal cord segments. NMO antibodies are specific and target aquaporin-4 channels which are expressed on astrocytes. In a case series, only 22 % of patients with NMO developed acute respiratory failure (ARF) [2]. Here we present a case of ARF from NMO later on developing acute respiratory distress (ARDS) from plasma exchange.
A 56-year-old female with a history of diabetes and hypertension presented with worsening lower extremity weakness associated with visual disturbances and urinary retention for one month. Physical exam was significant for decreased power and reflexes in lower extremities. MRI spine showed patchy areas of enhancement in the cervical spinal cord extending from medulla to upper thoracic level likely representing active demyelination. Cerebrospinal Fluid (CSF) showed a white blood cell count of 28/cumm, protein level of 143 mg/dl, and glucose level of 83.5 mg/dl. Serum NMO antibodies and CSF oligoclonal bands were not detected. Later on, she got intubated for acute hypercapnic respiratory failure. She was given intravenous glucocorticoids and plasma exchange therapy. After plasma exchange, she developed ARDS which was most likely from transfusion-related acute lung injury. Her hospital course was later on complicated by sepsis after which she expired.
It is Important to diagnose NMO and intervene sooner as half of the untreated patients become wheelchair-bound if left untreated. Negative NMO antibodies does not rule out the disease. ARF can occur from muscle weakness or involvement of the respiratory center in the medulla. Treatment of initial attack is with high dose intravenous glucocorticoid for at least five days. If no response plasma exchange is the next step. Maintenance usually requires immunosuppression therapy.
[1]Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012 Sep;69(9):1176-80. doi: 10.1001/archneurol.2012.314. PMID: 22733096.[2]Elsone L, Goh YY, Trafford R, et alHOW OFTEN DOES RESPIRATORY FAILURE OCCUR IN NEUROMYELITIS OPTICA?Journal of Neurology, Neurosurgery & Psychiatry 2013;84:e2.
Author Block: S. Chauhan1, Q. Abdiani2, R. Dadhwal3, A. Shalonov4; 1Pulmonology, Jamaica Hospital Medical Center, Richmond Hills, NY, United States, 2Internal Medicine, Jamaica Hospital Medical Center, Richmond Hills, NY, United States, 3Pulmonology, Corpus Christi Medical Center, Corpus Christi, TX, United States, 4Pulmonology and Critical Care, Jamaica Hospital Medical Center, Richmond Hills, NY, United States.
Neuromyelitis Optica (NMO) is a rare spectrum of disorders caused by immune-mediated demyelination of the central nervous system. The prevalence is approximately 10 per 100000 [1] with an incidence higher in women affecting people in their thirties or forties. Hallmark features are signs of optic neuritis or transverse myelitis which is mostly longitudinally extensive over more than three spinal cord segments. NMO antibodies are specific and target aquaporin-4 channels which are expressed on astrocytes. In a case series, only 22 % of patients with NMO developed acute respiratory failure (ARF) [2]. Here we present a case of ARF from NMO later on developing acute respiratory distress (ARDS) from plasma exchange.
A 56-year-old female with a history of diabetes and hypertension presented with worsening lower extremity weakness associated with visual disturbances and urinary retention for one month. Physical exam was significant for decreased power and reflexes in lower extremities. MRI spine showed patchy areas of enhancement in the cervical spinal cord extending from medulla to upper thoracic level likely representing active demyelination. Cerebrospinal Fluid (CSF) showed a white blood cell count of 28/cumm, protein level of 143 mg/dl, and glucose level of 83.5 mg/dl. Serum NMO antibodies and CSF oligoclonal bands were not detected. Later on, she got intubated for acute hypercapnic respiratory failure. She was given intravenous glucocorticoids and plasma exchange therapy. After plasma exchange, she developed ARDS which was most likely from transfusion-related acute lung injury. Her hospital course was later on complicated by sepsis after which she expired.
It is Important to diagnose NMO and intervene sooner as half of the untreated patients become wheelchair-bound if left untreated. Negative NMO antibodies does not rule out the disease. ARF can occur from muscle weakness or involvement of the respiratory center in the medulla. Treatment of initial attack is with high dose intravenous glucocorticoid for at least five days. If no response plasma exchange is the next step. Maintenance usually requires immunosuppression therapy.
[1]Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012 Sep;69(9):1176-80. doi: 10.1001/archneurol.2012.314. PMID: 22733096.[2]Elsone L, Goh YY, Trafford R, et alHOW OFTEN DOES RESPIRATORY FAILURE OCCUR IN NEUROMYELITIS OPTICA?Journal of Neurology, Neurosurgery & Psychiatry 2013;84:e2.
