Dataset Information

Clinical, neuroimaging, and nerve conduction characteristics of spontaneous Conus Medullaris infarction.

ABSTRACT:

Background

Spontaneous conus medullaris infarction is a rare disease. We describe two patients with spontaneous conus medullaris infarction presenting as acute cauda equina syndrome and their unique electromyography (EMG) findings.

Case presentation

Two patients developed acute low back pain with mild asymmetric paraparesis, loss of perianal sensation and sphincter dysfunction. Ankle deep tendon reflexes were reduced in bilaterally. Neither patient had cardiovascular risk factors. Magnetic Resonance imaging showed infarction in the conus medullaris. Functional recovery was good in both patients, but progressive asymmetric calf wasting and sphincter dysfunction remained. EMG studies at follow-up of at least 3?years demonstrate active denervation at the muscles innervated by the first sacrum anterior horn cells.

Conclusion

Spontaneous conus medullaris infarction can occur in healthy individuals and presents as cauda equina syndrome. Findings of needle EMG studies indicate a progressive course of sacrum anterior horn cell disorder during long-term follow-up.

SUBMITTER: Weng YC

PROVIDER: S-EPMC6916224 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Publications

Clinical, neuroimaging, and nerve conduction characteristics of spontaneous Conus Medullaris infarction.

Weng Yi-Ching YC Chin Shy-Chyi SC Wu Yah-Yuan YY Kuo Hung-Chou HC

BMC neurology 20191217 1

<h4>Background</h4>Spontaneous conus medullaris infarction is a rare disease. We describe two patients with spontaneous conus medullaris infarction presenting as acute cauda equina syndrome and their unique electromyography (EMG) findings.<h4>Case presentation</h4>Two patients developed acute low back pain with mild asymmetric paraparesis, loss of perianal sensation and sphincter dysfunction. Ankle deep tendon reflexes were reduced in bilaterally. Neither patient had cardiovascular risk factors. ...[more]

PMID: 31847829

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Project description:Objective Conus medullaris teratomas are very rare tumors. Traditional preoperative diagnosis depended on the findings from magnetic resonance imaging (MRI). Tractography is a novel technique that has recently been utilized to diagnose spinal cord lesions. This case report shows that fiber tractography has great potential in preoperative diagnosis and postoperative follow-up of teratomas of the conus medullaris. Methods A 50-year-old man with a conus medullaris teratoma underwent tractography with the aim of visualizing the tumor in relation to the white matter tracts. The patient underwent a T12-L2 laminectomy, and the lesion was resected. The histopathology diagnosis was of a mature teratoma. Study Design Case report. Results Diffusion tensor imaging (DTI) and tractography provide more details about the white matter tracts in relation to space-occupying lesions that may be more sensitive than conventional MRI and have recently been utilized in spinal cord lesions. Fiber tracking has the ability to visualize the integrity of the white matter tracts at the level of the conus medullaris in relation to the lesion. The tracts appeared to be displaced by the lesion at the conus medullaris. Tractography also showed no white matter tracts within the lesion. Such findings are consistent with the characteristics of a benign lesion. Exploiting tractography in this case was helpful in predicting the nature of the lesion preoperatively and in planning the surgical intervention. Conclusions Conus medullaris teratomas mostly affect adults. Patients generally present with a long history of clinical symptoms prior to diagnosis. Surgery is required for diagnosis, and the goal should be complete tumor excision without sacrificing any neurologic functions. The use of DTI and tractography, in addition to conventional MRI, has the potential to be very valuable for the diagnosis, surgical planning, and follow-up of patients with conus medullaris teratomas.

Project description:ImportanceSpinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research.ObjectiveTo describe the characteristics of spontaneous SCI and propose diagnostic criteria.Design, setting, and participantsAn institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280).Main outcomes and measuresA descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated.ResultsOf 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI.Conclusions and relevanceThis large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.

Dataset Information

Clinical, neuroimaging, and nerve conduction characteristics of spontaneous Conus Medullaris infarction.

Background

Case presentation

Conclusion

Publications

Clinical, neuroimaging, and nerve conduction characteristics of spontaneous Conus Medullaris infarction.

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