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Background and ObjectivesSarcoidosis is a multisystem granulomatous disease affecting the nervous system in 3%–5% of cases. It can affect almost any component of the nervous system. Involvement of the cauda equina is an understudied phenotype, and questions remain regarding its natural history and optimal approach to management. This study aims to study the long-term clinical evolution of neurosarcoidosis affecting the cauda equina, response to treatment, and clinical and radiographic outcomes.MethodsPatients with neurosarcoidosis treated at Emory University between January 1, 2011, and December 8, 2021, were retrospectively evaluated for manifestations of cauda equina disease and included if disease of the cauda equina could be substantiated by MRI or EMG.ResultsOf 216 cases, 14 (6.5%) involved the cauda equina. The median age was 49.5 years, and most were female (85.7%) and African American (64.3%). Chronic (>28 days) presentations were most common (78.6%), but acute (<7 days, 14.3%) and subacute (7–28 days, 7.1%) were also seen. The median modified Rankin Scale (mRS) score at nadir was 3 (range 2–4). Symptoms were asymmetric in 78.6% and included leg numbness (85.7%), leg weakness (64.3%), perineal numbness (35.7%), pain (42.3%), and incontinence (21.4%). On MRI, the cauda equina enhanced in 100%, appeared nodular in 78.6%, and was diffusely involved in 71.4%. Coexisting myelitis was common (cervical 28.6%, thoracic 35.7%, and conus medullaris 28.6%). Intracranial inflammation included leptomeningitis (71.4%) and cranial neuropathies (57.1%). Electrodiagnostic studies were conducted in 3 with only one showing features consistent with a radicular process. Serum and CSF angiotensin-converting enzyme levels were elevated in 38.5% and 0.0%, respectively. CSF white blood cell and protein were elevated in 92.9%. Corticosteroids were tried in all patients with durable stabilization or improvement in only 3 (21.4%). Second-line agents associated with improvement included methotrexate/infliximab (3/4, 75%), methotrexate (3/4, 75.0%), and azathioprine (1/1, 100%). During a median follow-up of 22.5 months, the final median mRS score was 3. Relapses occurred at a median of 6 months in 21.4%. In 9 patients with MRI follow-up, 6 improved (66.7%), 1 stabilized (11.1%), and 2 worsened (22.2%).DiscussionCharacteristic features of cauda equina involvement by neurosarcoidosis include chronically delayed presentations, nodular enhancement on MRI, poor response to corticosteroids, and substantial resultant neurologic disability.

Background and Purpose Spontaneous intracranial hypotension (SIH) is a known cause of headaches and neurologic symptoms, but the frequency of cranial nerve symptoms and abnormalities on magnetic resonance imaging (MRI) has not been well described. The purpose of this study was to document cranial nerve findings in patients with SIH and determine the relationship between imaging findings and clinical symptoms. Methods Patients diagnosed with SIH with pre‐treatment brain MRI at a single institution from September 2014 to July 2017 were retrospectively reviewed to determine the frequency of clinically significant visual changes/diplopia (cranial nerves 3 and 6) and hearing changes/vertigo (cranial nerve 8). A blinded review of brain MRIs before and after treatment was conducted to assess for abnormal contrast enhancement of cranial nerves 3, 6, and 8. Imaging results were correlated with clinical symptoms. Results Thirty SIH patients with pre‐treatment brain MRI were identified. Sixty‐six percent of patients had vision changes, diplopia, hearing changes, and/or vertigo. Cranial nerve 3 and/or 6 enhancement was present in nine patients on MRI, with 7/9 patients experiencing visual changes and/or diplopia (odds ratio [OR] 14.9, 95% confidence interval [CI] 2.2‐100.8, p = .006). Cranial nerve 8 enhancement was present in 20 patients on MRI, with 13/20 patients experiencing hearing changes and/or vertigo (OR 16.7, 95% CI 1.7‐160.6, p = .015). Conclusions SIH patients with cranial nerve findings on MRI were more likely to have associated neurologic symptoms than those without imaging findings. Cranial nerve abnormalities on brain MRI should be reported in suspected SIH patients as they may support the diagnosis and explain patient symptoms.