Clinical and MRI phenotypes of sarcoidosis-associated myelopathy

Abstract

ObjectiveTo determine the characteristic clinical and spinal MRI phenotypes of sarcoidosis-associated myelopathy (SAM), we analyzed a large cohort of patients with this disorder.MethodsPatients diagnosed with SAM at a single center between 2000 and 2018 who met the established criteria for definite and probable neurosarcoidosis were included in a retrospective analysis to identify clinical profiles, CSF characteristics, and MRI lesion morphology.ResultsOf 62 included patients, 33 (53%) were male, and 30 (48%) were African American. SAM was the first clinical presentation of sarcoidosis in 49 patients (79%). Temporal profile of symptom evolution was chronic in 81%, with sensory symptoms most frequently reported (87%). CSF studies showed pleocytosis in 79% and CSF-restricted oligoclonal bands in 23% of samples tested. Four discrete patterns of lesion morphology were identified on spine MRI: longitudinally extensive myelitis (n = 28, 45%), short tumefactive myelitis (n = 14, 23%), spinal meningitis/meningoradiculitis (n = 14, 23%), and anterior myelitis associated with areas of disc degeneration (n = 6, 10%). Postgadolinium enhancement was seen in all but 1 patient during the acute phase. The most frequent enhancement pattern was dorsal subpial enhancement (n = 40), followed by meningeal/radicular enhancement (n = 23) and ventral subpial enhancement (n = 12). In 26 cases (42%), enhancement occurred at locations with coexisting structural changes (e.g., spondylosis).ConclusionsRecognition of the clinical features (chronically evolving myelopathy) and distinct MRI phenotypes (with enhancement in a subpial and/or meningeal pattern) seen in SAM can aid diagnosis of this disorder. Enhancement patterns suggest that SAM may have a predilection for areas of the spinal cord susceptible to mechanical stress.

A Comprehensive Characterization of Patients with Spinal Cord Neurosarcoidosis: A Single Center Cross-Sectional Study of Clinical Outcomes

“…Our patient distribution may be more related to the demographic distribution of our patient population. We also observed that paresthesia/neuropathic pain was the most common symptom at presentation, similar to other studies [13,14]. However, other studies have reported higher levels of back pain [3] and motor deficit [9] than we observed.…”

A Comprehensive Characterization of Patients with Spinal Cord Neurosarcoidosis: A Single Center Cross-Sectional Study of Clinical Outcomes

“…Our patient distribution may be more related to the demographic distribution of our patient population. We also observed that paresthesia/neuropathic pain was the most common symptom at presentation, similar to other studies [13,14]. However, other studies have reported higher levels of back pain [3] and motor deficit [9] than we observed.…”

A Multi‐Center Retrospective Cohort Study of Neurosarcoidosis Myelitis: Current Observations and Future Directions

“…While most with this condition display inflammatory CSF via pleocytosis and/or elevated CSF protein, it is important to test for other markers of inflammation as well, specifically for CSF OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports [ 18 ], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

“…Many descriptive characteristics of this cohort align with those published. We found a slight male predominance in this cohort (male: female 1.3:1) and a median age of onset of neurosarcoidosis myelitis of 47 years, similar to other published cohorts [ 16 , 17 , 18 ]. Interestingly, this ~1:1 or slight male‐predominant sex ratio interestingly conflicts with the observed female predominant (2:1) sex ratio of systemic sarcoidosis in the United States [ 19 ].…”

“…(Continued) OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports[18], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

A Unique Case of Sarcoid-associated Myelopathy Accompanied by Lung Cancer

“…Myelopathy was observed in about 20% of neuro-sarcoidosis cases ( 2 ). Meningeal gadolinium enhancement without parenchymal hyperintensity on MRI, as was the case with the present patient, was observed in 23% of cases of sarcoidosis-associated myelopathy without malignancy ( 19 ). However, sarcoidosis-associated myelopathy in a patient with malignancy is rarely reported, and this is the first reported case of sarcoid-associated myelopathy accompanied by lung cancer.…”

A Comprehensive Characterization of Patients with Spinal Cord Neurosarcoidosis: A Single Center Cross-Sectional Study of Clinical Outcomes

“…Our patient distribution may be more related to the demographic distribution of our patient population. We also observed that paresthesia/neuropathic pain was the most common symptom at presentation, similar to other studies [13,14]. However, other studies have reported higher levels of back pain [3] and motor deficit [9] than we observed.…”

A Multi‐Center Retrospective Cohort Study of Neurosarcoidosis Myelitis: Current Observations and Future Directions

“…While most with this condition display inflammatory CSF via pleocytosis and/or elevated CSF protein, it is important to test for other markers of inflammation as well, specifically for CSF OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports [ 18 ], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

“…Many descriptive characteristics of this cohort align with those published. We found a slight male predominance in this cohort (male: female 1.3:1) and a median age of onset of neurosarcoidosis myelitis of 47 years, similar to other published cohorts [ 16 , 17 , 18 ]. Interestingly, this ~1:1 or slight male‐predominant sex ratio interestingly conflicts with the observed female predominant (2:1) sex ratio of systemic sarcoidosis in the United States [ 19 ].…”

“…(Continued) OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports[18], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

A Unique Case of Sarcoid-associated Myelopathy Accompanied by Lung Cancer

“…Myelopathy was observed in about 20% of neuro-sarcoidosis cases ( 2 ). Meningeal gadolinium enhancement without parenchymal hyperintensity on MRI, as was the case with the present patient, was observed in 23% of cases of sarcoidosis-associated myelopathy without malignancy ( 19 ). However, sarcoidosis-associated myelopathy in a patient with malignancy is rarely reported, and this is the first reported case of sarcoid-associated myelopathy accompanied by lung cancer.…”

A Comprehensive Characterization of Patients with Spinal Cord Neurosarcoidosis: A Single Center Cross-Sectional Study of Clinical Outcomes

“…Our patient distribution may be more related to the demographic distribution of our patient population. We also observed that paresthesia/neuropathic pain was the most common symptom at presentation, similar to other studies [13,14]. However, other studies have reported higher levels of back pain [3] and motor deficit [9] than we observed.…”

A Multi‐Center Retrospective Cohort Study of Neurosarcoidosis Myelitis: Current Observations and Future Directions

“…While most with this condition display inflammatory CSF via pleocytosis and/or elevated CSF protein, it is important to test for other markers of inflammation as well, specifically for CSF OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports [ 18 ], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

“…Many descriptive characteristics of this cohort align with those published. We found a slight male predominance in this cohort (male: female 1.3:1) and a median age of onset of neurosarcoidosis myelitis of 47 years, similar to other published cohorts [ 16 , 17 , 18 ]. Interestingly, this ~1:1 or slight male‐predominant sex ratio interestingly conflicts with the observed female predominant (2:1) sex ratio of systemic sarcoidosis in the United States [ 19 ].…”

“…(Continued) OCBs. In our cohort, CSF OCBs were elevated in ~24%, similar to other reports[18], with three of these cases having elevated OCBs with normal CSF cell count and protein. It is important to note this possibility of elevated CSF OCBs in neurosarcoidosis myelitis to avoid misdiagnosis of multiple sclerosis.…”

A Unique Case of Sarcoid-associated Myelopathy Accompanied by Lung Cancer

“…Myelopathy was observed in about 20% of neuro-sarcoidosis cases ( 2 ). Meningeal gadolinium enhancement without parenchymal hyperintensity on MRI, as was the case with the present patient, was observed in 23% of cases of sarcoidosis-associated myelopathy without malignancy ( 19 ). However, sarcoidosis-associated myelopathy in a patient with malignancy is rarely reported, and this is the first reported case of sarcoid-associated myelopathy accompanied by lung cancer.…”