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Case Reports
. 2020 Jan 15;59(2):267-270.
doi: 10.2169/internalmedicine.3510-19. Epub 2019 Sep 11.

New-onset Refractory Status Epilepticus Involving the Limbic System, Spinal Cord, and Peripheral Nerves

Kensuke Daida  1 , Kenya Nishioka  1 , Masashi Takanashi  1 , Manami Kobayashi  1 , Keisuke Yoshikawa  2 , Susumu Kusunoki  2 , Kazumasa Yokoyama  1 , Nobutaka Hattori  1
Affiliations
Case Reports

New-onset Refractory Status Epilepticus Involving the Limbic System, Spinal Cord, and Peripheral Nerves

Kensuke Daida et al. Intern Med. .

Abstract

A healthy 28-year-old woman presented suddenly with intractable status epilepticus: a focal seizure evolved into a generalized seizure preceded by a high fever. Brain magnetic resonance imaging indicated bilateral hyperintensities in the hippocampus on T2-weighted imaging. Electroencephalograms continuously demonstrated diffuse sharp waves and poly-spikes. Comprehensive immunomodulation therapies and anti-epileptic drugs did not lead to any improvements. We therefore diagnosed her with cryptogenic limbic encephalitis and new-onset refractory status epilepticus (NORSE). We detected positive anti-ganglioside antibodies, IgG-GQ1b, GD1a, and GT1b, which were negative at six months after the onset. We emphasize the heterogeneous pathogenesis and intractable conditions of NORSE.

Keywords: anti-ganglioside antibodies; limbic encephalitis; myelitis; new-onset refractory status epilepticus; pathology; polyneuropathy.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Findings of brain MRI, electroencephalogram and positron emission tomography. Axial T2-weighted brain MRI (A and B). (A) Brain MRI on admission revealed hyperintensities and swelling bilaterally in the hippocampi (white arrows) and cortices. (B) Brain MRI three months after admission showed bilateral progressive severe atrophic changes in the hippocampi (white triangles) and cortices, especially in the temporal lobes. (C) An electroencephalogram indicated diffuse high sharp waves and poly-spikes under thiopental sedation five months after admission. (D) Brain positron emission tomography with [18F] fluorodeoxyglucose depicted hyper-metabolism in the bilateral hippocampi (above) and a widespread lesion in the temporal lobe, predominantly on the left side (below).
Figure 2.
Figure 2.
Neuropathological findings of the autopsied brain, spinal cord, and nerve root. (A) Macroscopic coronal section of the left cerebral hemisphere, along with atrophic and necrotic changes in the cingulate cortex and hippocampus (black triangles). (B) Severe spongiform necrosis and gliosis in the hippocampus on Hematoxylin and Eosin staining (scale bar, 500 μm). (C) Coronal section of the lumbar spinal cord immunostained with anti-neurofilament phosphorylated antibody (SMI31). Discolored parts (black arrowheads) represent selective axonal loss in the bilateral lateral funiculi. (D) Numerous macrophages have infiltrated the lateral funiculus, showing staining for anti-Iba1, which is a marker of microglia and macrophages (scale bar, 500 μm). Dorsal root ganglion immunostained with anti-Iba1 antibody (scale bar 500 μm) (E) and anti-IgG antibody (scale bar 200 μm) (F). Macrophages have infiltrated the interstitial space of the ganglion. The neurons of the ganglion are positively stained with anti-IgG antibody. r: right, l: left
See this image and copyright information in PMC

References

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