This report concerns a 32-year old man with non-herpetic acute limbic encephalitis. He was admitted to our hospital because of high fever and consciousness disturbance. Cranial MRI revealed abnormal signal intensities in the bilateral mesial temporal lobes. On the second hospitalization day, he developed status epilepticus, which necessitated general anesthesia. Following administration of intravenous (IV) methylprednisolone pulse therapy (1 g/day, 3 days), his consciousness disturbance began to improve. Treatment with high-dose IV methylprednisolone was continued for about 2 weeks, but on the 7th day, he showed severe anterograde amnesia and retrograde amnesia regarding the preceding 5 or 6 years. Subsequently, however, his amnesic disorders improved markedly, and on the 46th day, memory dysfunction had disappeared. Autoantibodies against the glutamate receptor subunits epsilon2 and delta2 were detected in both the CSF and serum, but these antibodies in the CSF became normal during the clinical course. The voltage-gated potassium channel antibody was negative. This case report indicates that high-dose IV methylprednisolone therapy may be an affective treatment for non-herpetic acute limbic encephalitis, possibly associated with autoimmune mechanisms.
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Rinsho Shinkeigaku
June 2020
Department of Neurology, Japan Community Health care Organization, Hoshigaoka Medical Centre.
A 60-year-old, right-handed woman was admitted to our hospital for amnesia as the only neurological abnormal findings following the autonomic symptoms and transient episodes of loss of awareness. EEG during the amnesia showed rhythmic alpha activity arising from the left mid-temporal region. Although this ictal activity showed evolution in the frequency and amplitude, the location was limited in the bilateral temporal areas.
View Article and Find Full Text PDFJ Clin Neurosci
July 2018
Department of Neurosurgery, Sapporo Medical University, Sapporo, Japan.
We report a case of moyamoya disease (MMD), which developed after non-herpetic acute limbic encephalitis (NHALE) associated with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. The patient's mother had a history of MMD. No vascular lesions were identified at the time of the NHALE.
View Article and Find Full Text PDFCase Rep Neurol
November 2017
Department of Pediatrics, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.
This report describes a rare case presenting with dystonic seizures due to anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. The patient was an 18-year-old woman with repeated right-dominant dystonic seizures even under sedation. Single-photon emission computed tomography (SPECT) showed intense hyperperfusion of the caudate nuclei, putamen, globus pallidus, thalamus, and insula on the left side, suggesting encephalitis.
View Article and Find Full Text PDFTo evaluate the diagnostic value of SPECT (single photon emission computed tomography) brain blood flow imaging for patients with non-herpetic acute limbic encephalitis (NHALE). A retrospective review of three patients who had clinical symptoms compatible to NHALE and were positive for anti-N-methyl-d-aspartate-type glutamate receptor (GluRε2) antibody. The patients consisted of a 6-year-old female, a 10-year-old female and a 13-year-old male, all of whom had limbic symptoms and were anti-GluRε2 antibody-positive.
View Article and Find Full Text PDFRinsho Shinkeigaku
March 2016
Department of Internal Medicine, Sasebo City General Hospital.
A 62-year-old man experienced pain and swelling in both of his auricles. One and a half months later, he was referred to us because of a memory disturbance. A neurological examination revealed disorientation and recent memory impairment.
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