[Ictal electroencephalography (EEG) activity and cerebral blood flow dynamics as potential pathological indicators: a case of anti-leucine-rich glioma-inactivated 1 protein (LGI1) encephalitis].

A 29 year-old, right-handed woman was admitted to our hospital due to her headache with fever elevation lasting for two months followed by a prolonged loss of awareness with an involuntary movement in her left hand and mouth. This movement appeared very frequently, and the duration was very short, so called "faciobrachial dystonic seizures (FBDS)". Some of FBDS were followed by prolonged loss of awareness. Brain MRI fluid attenuated inversion recovery (FLAIR) image revealed high intensity lesion in the left mesial temporal lobe. Arterial spin labeling (ASL) image indicated hyper perfusion in this lesion and also the lateral temporal region. No ictal electroencephalography (EEG) change was observed before the onset of FBDS. FBDS was often followed by focal impaired awareness seizure (FIAS) in which ictal EEG showed rhythmic alpha activity arising from left mid-temporal region. This EEG seizure pattern was clearly visible in the time-frequency analysis. Given these clinical findings, along with an evidence of serum anti-leucine-rich glioma-inactivated 1 (LGI1) antibody positive, she was diagnosed with anti-LGI1 encephalitis. Immunotherapy (methylpredonisolone and intravenous immunoglobulin) with a multiple anti-epileptic drugs therapy (lacosamide, perampanel, and lamotrigine) was highly responsible to her symptoms. Although the high intensity lesion in FLAIR image still remained after the treatment, findings of ASL and EEG showed clear correlation to her cognitive function and seizures, respectively. Temporal change in ASL imaging suggested that the hyper perfusion in ASL during the acute stage could be provided by inflammation of the encephalitis its self and also the seizures activities (FBDS and FIAS). The pathophysiological indication of anti-LGI1 encephalitis was limited in terms of the therapeutic strategy, however, our findings collectively suggested that the combination analysis of EEG activity and cerebral blood flow dynamics (ASL) could be the potential candidate.

Pomalidomide-associated progressive multifocal leukoencephalopathy in multiple myeloma: cortical susceptibility-weighted imaging hypointense findings prior to clinical deterioration.

Although there are several case reports of progressive multifocal leukoencephalopathy (PML) in multiple myeloma (MM), there are few reports of cases associated with pomalidomide. Here, we report the case of a 69-year-old female who had received 41 cycles of pomalidomide and dexamethasone treatment for relapsed/refractory IgG-κ MM presented with right-hand weakness; she was diagnosed as pomalidomide-associated PML. Fluid-attenuated inversion recovery (FLAIR) on admission showed high signals in the bilateral front-parietal lobe white matter, with multiple punctate lesions in the vicinity of the main lesions. These punctate pattern findings on FLAIR were similar to that of natalizumab-associated PML. Susceptibility weighted imaging (SWI) showed hypointense rims within the cortex at unaffected sites, in the initial stages. Subsequently, the clinical manifestations deteriorated, and the FLAIR images showed new hyperintense white matter lesions at the sites where cortical SWI hypointense rims were detected on the initial MRI examination. Our patient's serial MRI findings suggest that cortical SWI hypointense rims appear prior to the visible demyelinating white matter lesions in patients with PML.

A case of anti-MOG antibody-positive unilaterally dominant meningoencephalitis followed by longitudinally extensive transverse myelitis.

BACKGROUND: There are few reports of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-positive, unilaterally dominant cerebral cortical encephalitis onset with epilepsy. We present such a case in a young female patient with myelitis. CASE PRESENTATION: A 19-year-old female developed generalized tonic seizures lasting several minutes. She had a low-grade fever and headache without other clinical neurological abnormalities while at our hospital. Cerebrospinal fluid (CSF) showed mononuclear pleocytosis. Other laboratory tests indicated no apparent abnormalities. Unilateral meningeal hyperintensity was seen on T2 fluid-attenuated inversion recovery MRI with associated cortical swelling and gadolinium enhancement of the cortical layer. One thousand mg/day of levetiracetam and a 3-day course of intravenous methylprednisolone at 1000 mg/day were administered. Dysuria appeared on the twentieth day of illness, and spinal MRI revealed a longitudinally extensive cord lesion from C5 to L1 consistent with myelitis. Two cycles of a 3-day course of intravenous methylprednisolone at 1000 mg/day were administered, and all symptoms disappeared. We found the patient to be anti-MOG antibody-positive using serum and CSF (titer: serum 1:256; CSF 1:128). CONCLUSION: Our report illustrates a unique case of anti-MOG presenting as new onset epilepsy secondary to unilaterally dominant meningoencephalitis preceding the onset of longitudinally extensive transverse myelitis.