[Successful cyclosporine treatment in 2 patients with refractory CIDP, involving monitoring of both AUC(0-4h) and trough levels].

Cyclosporine A (CYA) treatment has been reported to be probably useful for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) that is resistant to conventional treatment. Although several studies have shown that appropriate area under the concentration-time curve (AUC) monitoring of CYA levels results in improved outcomes for refractory nephrotic syndrome patients, the importance of using AUC analysis for CIDP remains unclear. In this study, we measured both trough and AUC from 0 to 4 h (AUC(0-4 h)) levels of CYA in 2 patients with CIDP and compared the findings for the clinical parameters. On the basis of the CYA dosing recommendations for patients with nephrotic syndrome, we used a CYA concentration of 150 ng/ml for the trough level and an AUC(0-4 h) value of 2,500 ng/(ml·h). Patient 1 showed a significant increase in grip strength and a prolonged remission period. Patient 2 showed improvement in the modified Rankin scale and manual muscle test (MMT) scores. Monitoring both AUC(0-4 h) and trough levels of CYA seems to be a better option than monitoring the trough level alone because it leads to improved estimation of the efficacy and safety of CYA treatment in the case of CIDP patients.

Malignant lymphoma originating in the cauda equina mimicking the inflammatory polyradiculoneuropathy.

A 67-year-old woman was diagnosed with inflammatory polyradiculoneuropathy. The intravenously administered immunoglobulin (IVIG) treatment that she received several times over a 3-year period relieved her clinical symptoms of muscle weakness and sensory disturbances, but these symptoms had worsened thereafter despite further IVIG treatment. MRI detected a solid tumor involving the cauda equina and pathological examinations confirmed this to be malignant lymphoma. The clinical and radiological findings for the malignant lymphoma of the cauda equina in this patient were quite similar to those for the inflammatory polyradiculoneuropathy.

[Intracranial hypertrophic pachymeningitis with high perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) occurred in a patient on hemodialysis].

We present a 66-year-old woman undergoing hemodialysis who developed intracranial hypertrophic pachymeningitis. Neurological examinations revealed a loss of bilateral visual acuity with optic atrophy, headache, and markedly restricted bilateral extraocular movement. MRI examinations demonstrated homogenous hypertrophic dural enhancement compatible with hypertrophic cranial pachymeningitis, and biopsied dural specimen revealed chronic inflammatory changes with proliferation of dense collagen fibers. There was no direct evidence of vasculitis and specific infections including tuberculosis and troponema pallidum. Most of the inflammatory infiltrates were demonstrated to be T lymphocytes. Intriguingly, p-ANCA was found to be highly elevated at x 220 and decreased to x 110 after steroid treatment. Neurological manifestations and radiological findings also improved in accordance with the lowering of p-ANCA. Although a few reports have described similar conditions such as chronic renal failure accompanying hemodialysis and pachymeningitis, and though vasculitis was not depicted histologically in this patient, we considered that immunological mechanisms probably provoked the patient's glomerulonephritis and pachymeningitis. Additionally, positive reaction against hepatitis c virus might have influenced the immunological system leading to the occurrence of the pachymeningitis.