Internal medicine
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We present a 76-year-old man with cryptogenic new-onset refractory status epilepticus (C-NORSE) with an initial abnormal signal in the nucleus accumbens and a remarkable hyperintense signal on T1-weighted magnetic resonance imaging in the bilateral basal ganglia (BG). His status epilepticus did not respond to most anti-epileptic therapies or immunotherapies, and he died of sepsis. An autopsy revealed severe neuronal loss and hypertrophic astrocytes in the BG and limbic system, with no signs of inflammation or malignancy. This case suggests that lesions in the BG may reflect secondary degeneration and predict poor outcomes in C-NORSE.
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A 21-year-old man was diagnosed with myeloid/natural killer precursor leukemia (MNKPL) with bone marrow infiltration of blasts of cyCD3+, CD7+, CD33+, CD34dim, CD56+/-, HLA-DR+, cyMPO+, and TdT- immunophenotypes. Although hyper-CVAD therapy was unsuccessful, induction treatment with idarubicin and cytarabine resulted in complete remission (CR). ⋯ He had been in good health without relapse for over nine months since transplantation. Timely allogeneic hematopoietic stem cell transplantation using an available donor source may be a promising treatment strategy for MNKPL.
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Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a treatable form of limbic encephalitis, marked by frequent focal seizures and cognitive decline (particularly memory disturbance); however, it can be difficult to diagnose in patients with subtle cognitive decline. Ictal pouting, a rare seizure feature, has not yet been reported in anti-LGI1 encephalitis. ⋯ Steroid treatment alone resolved seizures and improved subtle visual memory. Middle-aged and older patients experiencing subacute-onset frequent focal seizures should be thoroughly evaluated for memory disturbances to determine the need for anti-LGI1 antibody measurement.