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Clinical Pathologic Conference |

Rapidly Progressive Quadriplegia and Encephalopathy ONLINE FIRST

DonRaphael Wynn, MD1; Donald McCorquodale III, MD, PhD1; Angela Peters, MD1; Kelsey Juster-Switlyk, MD1; Gordon Smith, MD1; Safdar Ansari, MD1
[+] Author Affiliations
1Department of Neurology, University of Utah, Salt Lake City
JAMA Neurol. Published online September 06, 2016. doi:10.1001/jamaneurol.2016.1622
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A woman aged 77 years was transferred to our neurocritical care unit for evaluation and treatment of rapidly progressive motor weakness and encephalopathy. Examination revealed an ability to follow simple commands only and abnormal movements, including myoclonus, tongue and orofacial dyskinesias, and opsoclonus. Imaging study findings were initially unremarkable, but when repeated, they demonstrated enhancement of the cauda equina nerve roots, trigeminal nerve, and pachymeninges. Cerebrospinal fluid examination revealed mildly elevated white blood cell count and protein levels. Serial electrodiagnostic testing demonstrated a rapidly progressive diffuse sensory motor axonopathy, and electroencephalogram findings progressed from generalized slowing to bilateral periodic lateralized epileptiform discharges. Critical details of her recent history prompted a diagnostic biopsy. Over time, the patient became completely unresponsive with no further abnormal movements and ultimately died. The differential diagnosis, pathological findings, and diagnosis are discussed with a brief review of a well-known yet rare diagnosis.

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Figure 1.
MRI of Patient

A, Axial fluid-attenuated inversion recovery (FLAIR) magnetic resonance image (MRI) with contrast, showing the brainstem at the level of the pons with enhancement of the left trigeminal nerve (arrowhead). B, Coronal FLAIR MRI with contrast image, also showing trigeminal nerve enhancement on the left side (arrowhead). C, Axial FLAIR MRI with contrast at a higher level, demonstrating pachymeningeal enhancement (arrowhead). D, Sagittal short T1 inversion recovery (STIR) MRI of the lumbar spine with contrast, demonstrating enhancement of nerve roots at the cauda equine (arrowhead).

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Figure 2.
Electroencephalogram Recorded on Hospitalization Day 8

The electroencephalogram was calibrated as follows: the low-frequency filter was set to 1 Hz; the high-frequency filter was set to 70 Hz; the notch filter was off; and the sensitivity was at 10 uV/mm. The red line runs through synchronous periodic discharges resembling generalized periodic epileptiform discharges, which were beginning to attenuate in amplitude.

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