Asli Buyukkurt1, David Acero-Garces1, Paula Barreras2, Olwen Murphy1, Scott Newsome1, Carlos Pardo-Villamizar1
1Myelitis and Myelopathy Center, Department of Neurology, Johns Hopkins University, 2Department of Neurology, Cedars-Sinai Medical Center
Objective:
To describe clinical and magnetic resonance imaging (MRI) features of idiopathic myelitis.
Background:
Despite progress in identifying the etiological diagnoses of myelitis, the cause of inflammatory myelopathies remains unknown in some persons.
Design/Methods:
Retrospective chart review was performed for individuals referred to a specialized myelopathy center with “idiopathic myelitis” between 2006-2023. We propose a definition of idiopathic myelitis as: 1) sensory, motor, or autonomic dysfunction attributable to the spinal cord, 2) MRI lesion gadolinium enhancement or cerebrospinal fluid white cell count >5/uL, 3) absence of MRI evidence of compressive or vascular myelopathies, 4) exclusion of multiple sclerosis, 5) negative serum aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies, and 6) absence of rheumatological disorder and infection/vaccination within 4 weeks of onset. Diagnosis, time to symptom nadir, and brain and spinal cord MRI features were evaluated.
Results:
Of 140 individuals diagnosed with idiopathic myelitis, 24 met our proposed definition. Thirteen (54%) were male and 15 (62%) were white with a median age of 52.5 years (range, 23-74 years). Temporal profile was subacute (48 hours to 21 days) in 14 (58%) individuals and chronic (>21 days) in 6 (25%). MRI of 22 individuals showed spinal cord signal abnormalities. Of these, 15 (68%) were short segment lesions (<3 vertebral bodies in length), 15 were monofocal, and all showed gadolinium enhancement. In 20 individuals with available MRIs, 19 (95%) lesions involved the central spinal cord with 5 exclusive to the central cord and others also affecting a combination of anterior, posterior, and lateral white matter tracts. No lesions were isolated to anterior white matter tracts.
Conclusions:
In this cohort of individuals with idiopathic myelitis, subacute to chronic symptom evolution was frequent. Common MRI features included short segment, monofocal, gadolinium enhancing lesions involving the central cord. Updated diagnostic criteria are needed for further clinical and radiographic phenotyping of idiopathic myelitis.
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