The coefficient of variation of R-R (CV-RR) interval on electrocardiography was revealed to be 1
The coefficient of variation of R-R (CV-RR) interval on electrocardiography was revealed to be 1.55%, which showed an autonomic disturbance. window of magnetic resonance imaging (MRI) revealed that the right hamstring and gluteus maximus muscles were more atrophic than the left (Fig. 2a). MRI with short tau inversion recovery revealed areas of high intensity in the lumbar paraspinal muscles, gluteus maximus muscle and internal obturator muscle (Fig. 2b). A cerebrospinal fluid examination revealed the following: total protein, 88.9 mg/dL; albumin, 364.2 mg/L; glucose, 55 mg/dL; nucleated cells, 13 cells (12 mononucleated cells, one multinucleated Fexinidazole cell). The coefficient of variation of R-R (CV-RR) interval on electrocardiography was revealed to be 1.55%, which showed an autonomic disturbance. An electromyogram revealed that the lumbar paraspinal muscle contraction was impaired. A nerve conduction velocity study revealed that the motor and sensory nerves of the leg had a low amplitude and a low conduction velocity. These results indicated an axonal disorder and demyelination of the peripheral nerves. Moreover, serological examinations were positive for anti-3 subunit gAChR antibodies, and anti-4 subunit gAChR antibodies (8). These findings were consistent with a diagnosis of AAG with peripheral neuropathy. Open in a separate window Figure 2. a) The right hamstring and gluteus maximus muscles were more atrophic than the muscles on the left in the T1 window of MRI. The upper image shows the gluteus maximus muscle; the lower image shows the internal obturator muscle. b) The upper image shows the lumbar paraspinal muscles, the middle image shows the internal obturator muscle, and lower image shows the gluteus maximus muscle. A high intensity area is observed in the right lumbar paraspinal muscles, both internal obturator muscles and the right gluteus maximus muscle in the STIR window of MRI. On the 25th day of hospitalization, the Fexinidazole patient was treated for symptomatic MM with BD [a 5-week cycle of bortezomib (1.3 mg/m2, via subcutaneous injection on days 1, 8, 15 and 22) plus dexamethasone (20 mg/body, orally on days 1, 2, 8, 9, 15, 16, 22 and 23)] because AAG is reported to be one of the paraneoplastic neurological syndromes (3-5). After two courses of BD, 18 rounds of plasma exchange, and intravenous immunoglobulin, the patient’s free kappa chain level decreased from 9,650.0 mg/L to 138.0 mg/L. This indicated a PR (of the patient’s MM), as defined by the International Myeloma Working Group (9). Accordingly, the patient could sit down on her own, her CV-RR interval was improved, and her anti-3 subunit gAChR antibody and anti-4 subunit gAChR antibody titers became negative on the 72nd day of hospitalization (Fig. 3). However, bortezomib worsened her peripheral sensory neuropathy (CTCAE version 4.0: grade Fexinidazole III), and was therefore discontinued. Unfortunately, LD [a 4-week cycle of lenalidomide (5 mg/body, orally on days 1-21) plus dexamethasone (20 mg/body, orally on days 1-4 and 15-18)] Rabbit Polyclonal to OR4A16 also worsened her sensory peripheral neuropathy (CTCAE version 4.0: grade III). Thus, she was treated with high-dose dexamethasone [This consisted of a 4-week cycle of dexamethasone (20 mg/body, orally on days 1-4, 9-12 and 17-20)] and rehabilitation. As a result, she was discharged on foot on the 138th day of hospitalization. Open in a separate window Figure 3. The clinical course of the patient. The left vertical axis represents the percentage of CV-RR, the right vertical axis represents the kappa chain and lambda chain. The horizontal axis represents the days after admission. K chain: kappa chain, L chain: lambda chain, BD: bortezomib+dexamethasone, IVIG: intravenous immunoglobulin, Rd: lenalidomide+dexamethasone, HDD: high-dose dexamethasone, PE: plasma exchange, #1: anti-3 subunit gAChR antibody and anti-4 subunit gAChR antibody were positive, #2: anti-3 subunit gAChR antibody and anti-4 subunit gAChR antibody were negative. Discussion The present case was significant for two findings. First, MM and AAG were found to coexist. Second, treatment for MM, plasma exchange, and intravenous immunoglobulin induced a good remission of AAG with a reduction of the anti-gAChR antibody titer, despite the fact that only PR of MM was achieved. This is the first report on the coexistence of MM and AAG in a Fexinidazole patient. MM has been reported to induce peripheral neuropathy; this symptom occurs in 7.2%.