The Proof's in the Pudding: Case of Cauda Equina Syndrome Due to Known Sarcoid

Byun, John J.; Glantz, Michelle; Khandelwal, Sonali

doi:10.1016/j.amjmed.2020.06.036

The American Journal of Medicine

2021

DOI: 10.1016/j.amjmed.2020.06.036

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The Proof's in the Pudding: Case of Cauda Equina Syndrome Due to Known Sarcoid

John J. Byun

Michelle Glantz

Sonali Khandelwal

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“…10,11 Neurosarcoidosis has only rarely been described as a potential cause of cauda equina syndrome, most often in single case reports or small series. [12][13][14][15] In this retrospective cohort analysis, we review the clinical features, diagnostic testing results, treatment strategies, and long-term outcomes of 14 patients with neurosarcoidosis involving the cauda equina. We attempt to clarify the following points: (1) the frequency with which neurosarcoidosis affects the cauda equina; (2) the correlation between radiographic abnormalities of the cauda equina and symptoms suggestive of the syndrome; (3) its clinical course and responsiveness to treatment; and (4) final neurologic sequelae.…”

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confidence: 99%

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Neurosarcoidosis of the Cauda Equina

Bou

García-Santibáñez

Castilho

et al. 2022

Neurol Neuroimmunol Neuroinflamm

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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.

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confidence: 99%

“…10,11 Neurosarcoidosis has only rarely been described as a potential cause of cauda equina syndrome, most often in single case reports or small series. 12-15…”

mentioning

confidence: 99%

Neurosarcoidosis of the Cauda Equina

Bou

García-Santibáñez

Castilho

et al. 2022

Neurol Neuroimmunol Neuroinflamm

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show abstract

Infliximab/methotrexate

2021

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The Proof's in the Pudding: Case of Cauda Equina Syndrome Due to Known Sarcoid

Cited by 2 publications

References 4 publications

Neurosarcoidosis of the Cauda Equina

Neurosarcoidosis of the Cauda Equina

Infliximab/methotrexate

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