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July 2017; 4 (4) ArticleOpen Access

Elsberg syndrome

A rarely recognized cause of cauda equina syndrome and lower thoracic myelitis

Filippo Savoldi, Timothy J. Kaufmann, Eoin P. Flanagan, Michel Toledano, Brian G. Weinshenker
First published May 11, 2017, DOI: https://doi.org/10.1212/NXI.0000000000000355
Filippo Savoldi
From the Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
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Timothy J. Kaufmann
From the Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
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Eoin P. Flanagan
From the Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
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Michel Toledano
From the Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
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Brian G. Weinshenker
From the Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
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Citation
Elsberg syndrome
A rarely recognized cause of cauda equina syndrome and lower thoracic myelitis
Filippo Savoldi, Timothy J. Kaufmann, Eoin P. Flanagan, Michel Toledano, Brian G. Weinshenker
Neurol Neuroimmunol Neuroinflamm Jul 2017, 4 (4) e355; DOI: 10.1212/NXI.0000000000000355

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    Figure 1 Flow chart of patient selection

    Interrogation of the electronic database for specific key terms highlighted in the first box returned a list of 1,035 patients. We excluded patients with an ICD code identifying NMO and MS, yielding 837 patients. Further interrogation of the database for patients with the following key terms returned 337 patients: “urinary retention” OR “paresthesia” OR “neuralgic pain” OR “constipation” OR “impotence” OR “perineal” OR “retention” OR “anus” OR “anal” OR “saddle”. After the review of individual clinical records of the remaining patients, we excluded 213 subjects who had likely or established alternative diagnoses, as listed in the figure, and only patients who were confirmed to have urinary retention and other sacral sensory symptoms were retained. Of the remaining 49 patients, 19 did not satisfy any of the levels of diagnostic certainty proposed in table 1. The remaining 30 were assigned a specific diagnostic category according to the level of suspicion for ES. ES = Elsberg syndrome; ICD = International Classification of Disease; NMO = neuromyelitis optica.

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    Figure 2 MRI evidence of myelitis and radiculitis in 6 patients

    Patient A presents with concomitant presence of multiple and discontinuous T2 hyperintense lesions (A.a) that enhance on T1-weighted images after gadolinium injection (A.b) concomitantly with nerve roots of the cauda equina (A.b and A.c). Nerve root enhancement is prominent in 2 other patients (B.a, B.b, C.a, and C.b), with greater nerve root thickening in the latter (C.c). Two other patients had multifocal, discontinuous, T2-hyperintense lesions (D.a and E.a) as well as enhancement (D.b and E.b) in both the conus and lower thoracic cord. Finally, cord T2-hyperintense abnormality (F.a) may precede the onset of nerve root enhancement (F.b) by 26 days.

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