Author Of 2 Presentations
P0681 - A severe case of tetraparesis with conus involvement in neuromyelitis optica spectrum disorder (ID 1038)
Abstract
Background
Neuromyelitis optica spectrum disorder (NMOSD) is a severe neuroinflammatory disorder primarily affecting the optic nerves and spinal cord. It is associated with a variety of clinical phenotypes and radiological features that can make diagnosis challenging.
Objectives
We present an atypical and diagnostically challenging case of NMOSD with severe tetraparesis and conus involvement.
Methods
This is a case report detailing the complex clinical presentation, workup, and treatment of a patient who was ultimately diagnosed with NMOSD.
Results
A previously healthy 46-year-old woman presented to the emergency room with 2 weeks of unidentifiable nausea/vomiting after a recent ear infection. Shortly after discharge, she returned to the hospital with complaints of worsened generalized weakness and no longer being able to walk. Over the next few days, she became flaccid with 0/5 strength in all 4 extremities. Magnetic resonance imaging (MRI) of the brain showed several nonenhancing lesions in the hypothalamic, anterior medial thalamic, right posterior pontine, and right middle cerebellar peduncular regions. MRI spine showed extensive enhancing lesions in the upper cervical cord and conus medullaris with leptomeningeal enhancement. Cerebrospinal fluid (CSF) studies were notable for lymphocytic-predominant pleocytosis with negative oligoclonal bands and negative cytology. Serum and CSF autoimmune panels were negative. Infectious and malignancy workup was negative. Cell-based assay testing for myelin oligodendrocyte glycoprotein (MOG-Abs) and aquaporin-4 (AQP4-Abs) antibodies were negative. Workup was negative for systemic signs of sarcoidosis or malignancy. She was treated with 5 days of intravenous steroids followed by intravenous immunoglobulin (IVIG) without improvement. She then underwent plasma exchange for 5 sessions. Within a week after completion, her strength gradually improved such that she was antigravity with 4/5 strength in all extremities by discharge to rehab. Because it was unclear if this was a monophasic event, a tentative diagnosis of atypical acute disseminated encephalomyelitis (ADEM) was made. Three months later, she developed muscle spasms in her right arm and was found to have a new enhancing lesion in the right spinal cord at C2. Repeat testing was sent and her AQP4-Ab test returned positive, leading to a final diagnosis of NMO.
Conclusions
This is a complex case with multiple differential diagnoses, including NMO, atypical ADEM, neurosarcoidosis, or lymphoma. Conus involvement is unusual for NMO and tend to be associated more with MOG-Abs compared to AQP4-Abs. Though cell-based assays have high sensitivities (90-94%), testing should be repeated on negative values if clinical suspicion is high.
P0887 - Multiple sclerosis evaluation and diagnosis may be delayed in male and African American patients (ID 336)
Abstract
Background
Sex and race seem to influence multiple sclerosis (MS) prognosis. Men with MS overall appear to have a worse prognosis than women with MS. Black (African American) MS patients are believed to have a worse prognosis than white MS patients. These observations suggest that men and black patients with MS may have a more aggressive disease. The possibility that delays in MS evaluation or MS diagnosis in these populations could contribute to worsened outcomes remains underexplored.
Objectives
To evaluate whether sex or race may be associated with delays in being evaluated for possible MS or in being diagnosed with MS.
Methods
We surveyed patients with a confirmed diagnosis of MS at our center in the Washington, DC region from November 2019 to March 2020, asking them to recall three events: their initial symptom onset, their first neurology visit, and their eventual MS diagnosis.
Results
Out of 101 total surveys, 94 (93.1%) were included in this analysis. 72.3% of respondents were women. Most respondents self-identified as black (N=49, 52.1%) or white (N=45, 47.9%). While there was no difference in age at symptom onset, men tended to be older than women at first neurology visit and at MS diagnosis (mean 35.9 vs 33.8 years and 39.8 vs 35.0 years, respectively). There was a trend towards longer median time from symptom onset to first neurology visit, and from first neurology visit to MS diagnosis for men (7.5 months and 2.5 months, respectively) vs. women (1 month and 1 month, respectively). The overall time from symptom onset to MS diagnosis was significantly increased for men compared to women with MS (21 months vs. 5.5 months, p=0.03). Black patients were more likely to have impaired gait at time of diagnosis than white patients (48.9% vs 28.6%, p=0.04), but there was also a trend towards longer median time from symptom onset to first neurology visit, from first neurology visit to MS diagnosis, and from symptom onset to MS diagnosis in black patients (2 months, 2 months, and 12 months, respectively) vs. white patients (1 month, 1 month, and 7 months, respectively). Black men in particular had longer median times from symptom onset to first neurology visit (12 months) and from first neurology visit to MS diagnosis (6.5 months) than other patient groups: black women (2 months and 1 month, respectively), white men (4.5 months and 1 month, respectively), and white women (1 month and 1 month, respectively).
Conclusions
There may be delays before men and black patients are evaluated for MS and before they are diagnosed with MS, compared to women and white patients. These delays in assessment and diagnosis could theoretically contribute to the observed poorer prognosis for patients of those at-risk populations.
Presenter Of 2 Presentations
P0681 - A severe case of tetraparesis with conus involvement in neuromyelitis optica spectrum disorder (ID 1038)
Abstract
Background
Neuromyelitis optica spectrum disorder (NMOSD) is a severe neuroinflammatory disorder primarily affecting the optic nerves and spinal cord. It is associated with a variety of clinical phenotypes and radiological features that can make diagnosis challenging.
Objectives
We present an atypical and diagnostically challenging case of NMOSD with severe tetraparesis and conus involvement.
Methods
This is a case report detailing the complex clinical presentation, workup, and treatment of a patient who was ultimately diagnosed with NMOSD.
Results
A previously healthy 46-year-old woman presented to the emergency room with 2 weeks of unidentifiable nausea/vomiting after a recent ear infection. Shortly after discharge, she returned to the hospital with complaints of worsened generalized weakness and no longer being able to walk. Over the next few days, she became flaccid with 0/5 strength in all 4 extremities. Magnetic resonance imaging (MRI) of the brain showed several nonenhancing lesions in the hypothalamic, anterior medial thalamic, right posterior pontine, and right middle cerebellar peduncular regions. MRI spine showed extensive enhancing lesions in the upper cervical cord and conus medullaris with leptomeningeal enhancement. Cerebrospinal fluid (CSF) studies were notable for lymphocytic-predominant pleocytosis with negative oligoclonal bands and negative cytology. Serum and CSF autoimmune panels were negative. Infectious and malignancy workup was negative. Cell-based assay testing for myelin oligodendrocyte glycoprotein (MOG-Abs) and aquaporin-4 (AQP4-Abs) antibodies were negative. Workup was negative for systemic signs of sarcoidosis or malignancy. She was treated with 5 days of intravenous steroids followed by intravenous immunoglobulin (IVIG) without improvement. She then underwent plasma exchange for 5 sessions. Within a week after completion, her strength gradually improved such that she was antigravity with 4/5 strength in all extremities by discharge to rehab. Because it was unclear if this was a monophasic event, a tentative diagnosis of atypical acute disseminated encephalomyelitis (ADEM) was made. Three months later, she developed muscle spasms in her right arm and was found to have a new enhancing lesion in the right spinal cord at C2. Repeat testing was sent and her AQP4-Ab test returned positive, leading to a final diagnosis of NMO.
Conclusions
This is a complex case with multiple differential diagnoses, including NMO, atypical ADEM, neurosarcoidosis, or lymphoma. Conus involvement is unusual for NMO and tend to be associated more with MOG-Abs compared to AQP4-Abs. Though cell-based assays have high sensitivities (90-94%), testing should be repeated on negative values if clinical suspicion is high.
P0887 - Multiple sclerosis evaluation and diagnosis may be delayed in male and African American patients (ID 336)
Abstract
Background
Sex and race seem to influence multiple sclerosis (MS) prognosis. Men with MS overall appear to have a worse prognosis than women with MS. Black (African American) MS patients are believed to have a worse prognosis than white MS patients. These observations suggest that men and black patients with MS may have a more aggressive disease. The possibility that delays in MS evaluation or MS diagnosis in these populations could contribute to worsened outcomes remains underexplored.
Objectives
To evaluate whether sex or race may be associated with delays in being evaluated for possible MS or in being diagnosed with MS.
Methods
We surveyed patients with a confirmed diagnosis of MS at our center in the Washington, DC region from November 2019 to March 2020, asking them to recall three events: their initial symptom onset, their first neurology visit, and their eventual MS diagnosis.
Results
Out of 101 total surveys, 94 (93.1%) were included in this analysis. 72.3% of respondents were women. Most respondents self-identified as black (N=49, 52.1%) or white (N=45, 47.9%). While there was no difference in age at symptom onset, men tended to be older than women at first neurology visit and at MS diagnosis (mean 35.9 vs 33.8 years and 39.8 vs 35.0 years, respectively). There was a trend towards longer median time from symptom onset to first neurology visit, and from first neurology visit to MS diagnosis for men (7.5 months and 2.5 months, respectively) vs. women (1 month and 1 month, respectively). The overall time from symptom onset to MS diagnosis was significantly increased for men compared to women with MS (21 months vs. 5.5 months, p=0.03). Black patients were more likely to have impaired gait at time of diagnosis than white patients (48.9% vs 28.6%, p=0.04), but there was also a trend towards longer median time from symptom onset to first neurology visit, from first neurology visit to MS diagnosis, and from symptom onset to MS diagnosis in black patients (2 months, 2 months, and 12 months, respectively) vs. white patients (1 month, 1 month, and 7 months, respectively). Black men in particular had longer median times from symptom onset to first neurology visit (12 months) and from first neurology visit to MS diagnosis (6.5 months) than other patient groups: black women (2 months and 1 month, respectively), white men (4.5 months and 1 month, respectively), and white women (1 month and 1 month, respectively).
Conclusions
There may be delays before men and black patients are evaluated for MS and before they are diagnosed with MS, compared to women and white patients. These delays in assessment and diagnosis could theoretically contribute to the observed poorer prognosis for patients of those at-risk populations.