[A case of Lissauer form of paretic neurosyphilis with drop attacks and dementia].

A 52-year-old man was admitted to our hospital due to drop attack accompanied with progressive right hand's clumsiness and recent memory impairment. Neurological examination revealed postural involuntary movement of the right hand, and impaired finger fine movements. He showed mild mental deterioration with lower MMSE score. Toreponema Pallidum Latex immuno Assay (TPLA) titers in serum and cerebrospinal fluid (CSF) were remarkably elevated. MRI revealed cerebral atrophy in the left hemisphere. The 99mTc-ECD SPECT analysis showed reduced cerebral blood flow in the left hemisphere. EEG showed slow background activity in the left hemisphere. Drop attacks and involuntary movement were diminished by the administration of the valproate. We diagnosed this case as Lissauer's general paresis and treated with intravenous benzylpenicillin potassium 24 million units per day for 2 weeks. After this treatment, the titer of TPLA in the serum and CSF decreased. The clinical symptoms and signs, the score of MMSE, findings of SPECT and EEG also improved. Although Lissauer's general paresis is a rare form of neurosyphilis, it is important to distinguish this disease in case of progressive dementia and focal neurological signs. Early diagnosis and treatment are the only way to prevent irreversible neurological damage in this disease.

[A case of myositis associated with clonal expansion of γδ T cells in peripheral blood and bone marrow].

We report a 45-year-old man with myositis associated with clonal expansion of γδ T cells. He was referred to our hospital because of slowly progressive (over 10 years) muscle weakness. On neurological examination, weakness and muscle atrophy were noted in the proximal upper and lower limbs. The level of creatinine kinase (CK) was 1,436 U/L. Neutropenia and monoclonal gammopathy were found in the peripheral blood. Flow cytometric analysis of peripheral blood and bone marrow revealed proliferation of CD3(+)CD4(-)CD8(+) and CD3(+)CD4(-)CD8(-) γδ T cells, and Southern blotting demonstrated a clonally rearranged T-cell receptor Jγ gene in peripheral blood and bone marrow. A biopsy of the right quadriceps muscle showed variations in muscle fiber size, and endomysial mononuclear cell infiltration. The expression of MHC Class I antigen was increased on the surfaces of most of muscle fibers, and TCRδ1 positive-lymphocytes invaded nonnecrotic muscle fiber. After starting treatment with cyclosporin A and steroids, his muscle weakness gradually ameliorated, the CK level decreased and neutrophils increased. Although reports of myositis associated with clonal expansion of γδ T cells are extremely rare, the present case suggests that γδ T cells might play a role in mediating myositis.