Blood tests for systematic autoimmune diseases and cancer screening were also negative. without enhancement [Figure ?[Figure1AC1C].1AC1C]. Cerebral spinal fluid (CSF) analysis showed elevated pressure (250 mmH2O) and presence of leukocytes (142 cells/mm3) and protein (66.9?mg/dL). Oligonucleotide band was negative. CSF was positive for immunoglobulin (Ig)G but not IgM to rubella virus, herpes simplex types I and II, cytomegalovirus and Epstein-Barr virus. Electroencephalograms showed slow and sharp wave activity in the right hemisphere. The patient was treated DG051 with intravenous acyclovir DG051 and dexamethasone. Pressure, leukocytes, and protein in the CSF had improved at the time of discharge, but his headache and fever recurred 3 weeks later. Cell-based assays for all serum/CSF Abs associated with autoimmune encephalitis (AIE), demyelinating disease, and paraneoplastic neurologic syndrome were negative. Blood tests for systematic autoimmune diseases and cancer screening were also negative. Methylprednisolone (MP) (80?mg/d for 5 days) therapy ameliorated elevated CSF protein and leukocytes and his MRI returned to normal. Open in a separate window Figure 1 Brain magnetic resonance imaging (MRI) of patient 1 (ACJ) and patient 2 (KCV). (ACC) Brain MRI showed hyperintensity on FLAIR imaging in the right temporal, parietal, and occipital gyrus. Images obtained with the left medulla oblongata (D) and the right medial temporal lobe (E). Follow-up brain MRI showed lesion enlargement in the medulla oblongata (F) and a new lesion in the right basal ganglia (G). New lesions of the left margin of brain stem (H) with patchy enhancement (I). (J) Orbital MRI showed the left optic nerve flexion and enhancement DG051 on gadolinium-enhanced T1WI. (KCN) Imaging features of the cortical lesions of the left insula and parietal cortex. Brain MRI showed cortical lesions of the left temporal lobe (O, P), hippocampus (Q), and right basal ganglia (R) with mild enhancement (SCV). Six months later, the patient returned to the hospital because of right hemianesthesia and left upper limb numbness. MRI showed new lesions in the left medulla oblongata and right temporal lobe [Figure ?[Figure1D1D and 1E]. Follow-up MRI revealed enlargement of the lesions and new enhanced lesion [Figure ?[Figure1F1F and 1G]. The serum/CSF Abs listed above were again negative. The patient was treated with intravenous immunoglobulin G (IVIG) (0.4?g/kg daily for 5 days) for 3 consecutive months combined with azathioprine. A stable phase was reached for 6 months until the patient developed orbital pain and decreased visual Rabbit Polyclonal to B-RAF acuity in the left eye. New enhanced lesions in the brainstem and the left optic nerve were detected [Figure ?[Figure1HC1J].1HC1J]. He was re-tested and found positive for serum/CSF MOG-Ab (1:320/1:32) and NMDAR Ab (negative/1:1). No lesion was identified on spinal cord MRI. Tumor markers were still undetectable. Despite treatment with IVIG and MP via retrobulbar DG051 injection, his visual acuity recovered incompletely over 1-year follow-up (from counting fingers at 30?cm to 0.6). He experienced no seizures or adverse events. A 50-year-old Chinese man had a history of headache, fever, and seizures. He had been diagnosed with viral encephalitis 15 years previously by a different hospital, but the clinical data had been lost. His seizures were well-controlled until February 2011, when he was sent to our hospital for recurrence of seizures with normal CSF. He had no previous medical history or family history of seizure. Fluid attenuation inversion recovery imaging revealed high-intensity lesions in the left insula and parietal cortex [Figure ?[Figure1KC1N].1KC1N]. Electroencephalograms showed multiple sharp and slow waves in the left central and parietal regions. A analysis of gray matter heterotopia was regarded as during neurosurgical discussion and he underwent a parietal lobotomy. During hospitalization, the patient experienced paroxysmal visual and auditory hallucinations. Following administration of carbamazepine and levetiracetam, his symptoms disappeared. In October 2017, the patient was admitted to the CNS demyelinating disease registry because he had been suffering from progressive cognitive decrease, somnolence, visual hallucinations, and irregular behavior. MRI exposed multiple lesions with slight enhancement [Number ?[Number1OC1V].1OC1V]. He had no abnormal findings on spinal MRI and CSF exam except for positive serum/CSF NMDAR-Ab (1:100/1:32) and MOG-Ab (1:32/1:10). His CSF was positive for IgG to cytomegalovirus and Epstein-Barr computer virus. Cancer testing and autoimmune disease-related indices were negative. After initial administration of pulse intravenous MP (1000?mg/d for 5 days) and IVIG (0.4?g/kg daily for 5 days) therapies, his symptoms improved gradually. He received maintenance therapy with oral prednisone, azathioprine, olanzapine, oxcarbazepine, and levetiracetam without adverse events. NMDARE can primarily present with psychosis, memory space deficits, dyskinesia, involuntary motions, decreased level of consciousness, and central hypoventilation. The medical manifestation of NMDARE may be heterogeneous, ranging from total to slight/partial forms. Some instances may be DG051 asymptomatic. Despite the initial typical.