RT Journal Article
SR Electronic
T1 Clinical and MRI phenotypes of sarcoidosis-associated myelopathy
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e722
DO 10.1212/NXI.0000000000000722
VO 7
IS 4
A1 Murphy, Olwen C.
A1 Salazar-Camelo, Andrea
A1 Jimenez, Jorge A.
A1 Barreras, Paula
A1 Reyes, Maria I.
A1 Garcia, Maria A.
A1 Moller, David R.
A1 Chen, Edward S.
A1 Pardo, Carlos A.
YR 2020
UL http://nn.neurology.org/content/7/4/e722.abstract
AB Objective To determine the characteristic clinical and spinal MRI phenotypes of sarcoidosis-associated myelopathy (SAM), we analyzed a large cohort of patients with this disorder.Methods Patients 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.Results Of 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).Conclusions Recognition 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.BSCB=blood-spinal cord barrier; IQR=interquartile range; mRS=modified Rankin Scale; NMOSD=neuromyelitis optica spectrum disorder; SAM=sarcoidosis-associated myelopathy