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A patient with motor neuron disease and tonic pupil who had an antinuclear antibody (Ab) in the serum and oligoclonal pattern in IgG in the CSF is described. Sera and CSF from this patient and controls (37 sera and 30 CSF) were screened for an antineuronal Ab using immunoblotting. Only the serum and CSF from this patient contained an Ab to a 70-kD protein in the human spinal cord but not in the human muscle or cerebellar cortex. This patient’s serum immunohistochemically stained human and Japanese monkey anterior horn cells but not Japanese monkey dorsal root ganglion.

Background Many patients with primary CNS lymphoma (PCNSL) relapse despite complete response (CR) after initial therapy. Relapsed PCNSL (rPCNSL) has a poor prognosis, and no adequate standard of treatment has been established. Autologous stem cell transplant (ASCT)-assisted high-dose chemotherapy (HDC) for rPCNSL is a promising treatment option, as is thiotepa (TT), which has been used in malignant lymphoma as a treatment option for PCNSL. In March 2020, TT was approved in Japan as a pre-treatment for ASCT in malignant lymphoma. TBC, TT with busulfan and cyclophosphamide, and BCNU+TT are used in Europe and the US, but treatment-related mortality (TRM) has been reported in about 10% of patients. The regimen approved in Japan is Buslufan-TT(BuTT) therapy in combination with busulfan, which differs from TBC in dosage, and there is little information on efficacy and safety for rPCNSL. In our hospital, we have introduced BuTT therapy for rPCNSL in patients under 65 years of age. In this report, we describe our experience with this treatment. Subjects: Five patients with rPCNSL since May 2021, age <66 years, PS 0-2, are included in this study. Methods After obtaining CR with (R)MPV therapy after relapse, peripheral blood stem cells were harvested after administration of G-CSF and plerixafor, pretreated with BuTT therapy (Bu: 3.2 mg/kg, Day-8, -7, -6, -5: TT: 5 mg/kg, Day-4, -3) and transplanted on Day 0. Results Adverse events of Grade 3 or higher were abdominal pain in 2 cases, diarrhea in 1 case, pharyngitis in 2 cases, sepsis in 1 case, and pleuritis in 1 case, but all recovered, and no TRM was observed. All five patients are currently alive without recurrence. Conclusion HDC/ASCT is expected to be a curative treatment for rPCNSL. Future issues include the establishment of an upper age limit for the indication.

A 69-year-old woman was treated with immunosuppressive agents(methotrexate, tacrolimus, cyclosporine, etanercept, adalimumab, azathioprine, and mycophenolate mofetil)for a 30-year history of systemic lupus erythematosus, rheumatoid arthritis, and interstitial pneumonia. She presented to our hospital for evaluation of right-sided hemiparesis. Magnetic resonance imaging revealed a ring-enhanced mass lesion(maximum diameter 36 mm)in the left parietal lobe. She was hospitalized for further evaluation and treatment of suspected high-grade glioma, metastatic brain tumor, and malignant lymphoma. We performed subtotal tumor resection, and histopathological evaluation of the resected specimen confirmed diagnosis of diffuse large-cell B-cell lymphoma, an iatrogenic lymphoproliferative disease caused by immunosuppressive drugs. This disorder was attributed to mycophenolate mofetil therapy initiated 1 year before symptom onset. Mycophenolate mofetil therapy was discontinued, and the patient received six cycles of rituximab and local radiation. Iatrogenic lymphoproliferative disease is known to occur during mycophenolate mofetil treatment；however, primary lymphoproliferative disease of the central nervous system associated with mycophenolate mofetil therapy is rare.

Introduction: Primary pituitary lymphoma (PPL) is a rare disease characterized by lymphoma confined to the sella or parasellar region without systemic involvement. The clinical symptoms of PPL may include headache, hypopituitarism, visual field disturbance and visual impairment. To date, there is no established standard treatment for this condition. Here, we present a case of successful treatment with a Bruton’s tyrosine kinase (BTK) inhibitor. Case report: A 78-years-old man with a history of severe left renal insufficiency caused by retroperitoneal fibrosis, and sequential right nephrostomy, underwent brain magnetic resonance imaging (MRI) due to the altered hormonal status. An enlarged pituitary stalk was noted and led to a diagnosis of lymphocytic hypopituitarism. Six months later, visual field disturbance and visual acuity deterioration developed, and an MRI revealed a neoplastic lesion and further enlargement of the stalk and the pituitary itself, with an obvious optic nerve compression. Expedited transsphenoidal partial resection was performed to relieve the compression. Pathohystology led to the diagnosis of the large B-cell lymphoma of the germinal center origin. Because of the patient’s poor renal function, high-dose methotrexate therapy was not an option; rather, the patient was treated with a BTK inhibitor - tirabrutinib. Symptoms improved within a week, and a follow-up MRI confirmed a marked reduction of the pituitary lesion. Conclusion: BTK inhibitors may be considered as a first-line treatment option for PPL, especially in patients with contraindications for other treatment protocols.