Cochlear implantation in chronic demyelinating inflammatory polyneuropathy.

OBJECTIVE: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. METHODS: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. RESULTS: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss. CONCLUSIONS: Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.

[Anesthetic Management of Three Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy].

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronically progressing or relapsing disease caused by immune-mediated peripheral neuropathy. We report the anesthetic management of three CIDP patients who underwent elective orthopedic surgeries. Owing to the risk of neuraxial anesthetics triggering demyelination, general anesthesia was selected to avoid epidural or spinal anesthesia or other neuraxial blockade. It was also judged prudent to avoid prolonged perioperative immobilization, which might compress vulnerable peripheral nerves. For Patient 1, general anesthesia was induced with propofol, remifentanil, and sevoflurane, and was maintained with sevoflurane and remifentanil. For Patients 2 and 3, general anesthesia was induced and maintained with propofol and remifentanil. For tracheal intubation, under careful monitoring with peripheral nerve stimulators, minimal doses of rocuronium (0.6-0.7 mg x kg(-1)) were administered. When sugammadex was administered to reverse the effect of rocuronium, all patients rapidly regained muscular strength. Postoperative courses were satisfactory without sequelae.

Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP.

OBJECTIVE: Only 70-80% of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) respond satisfactorily to the established first-line immunomodulatory treatments. Autologous haematopoietic stem cell transplantation (AHSCT) has been performed as a last treatment resort in a few therapy-refractory cases with CIDP. We describe the results of AHSCT in 11 consecutive Swedish patients with therapy-refractory CIDP with a median follow-up time of 28 months. METHOD: Case data were gathered retrospectively for AHSCT treatments in 11 patients with CIDP refractory to the first-line immunomodulatory treatments, intravenous high-dose immunoglobulin, corticosteroids and plasma exchange and to one or more second-line treatments used in 10 of the 11 patients. RESULTS: The median Inflammatory Neuropathy Cause and Treatment (INCAT) score within 1 month prior to AHSCT was 6 and the Rankin score 4. Total INCAT and Rankin scores improved significantly within 2-6 months after AHSCT and continued to do so at last follow-up. The motor action potential amplitudes (CMAP) improved already within 4 months (median) after AHSCT. Three of the 11 patients relapsed during the follow-up period, requiring retransplantation with AHSCT in one. Eight of the 11 patients maintained drug-free remission upon last follow-up. AHSCT was safe but on the short term associated with a risk of cytomegalovirus (CMV) and Epstein-Barr virus reactivation, CMV disease, haemorrhagic cystitis and pancreatitis. CONCLUSIONS: Our results though hampered by the limited number of patients and the lack of a control group suggest AHSCT to be efficacious in therapy-refractory CIDP, with a manageable complication profile. Confirmation of these results is necessary through randomised controlled trials.

[Anesthetic case report of a patient with chronic inflammatory demyelinating polyneuropathy].

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease characterized by progressive neurological distress and motor weakness in the legs and arms. We report a patient with CIDP who underwent thoracoscopic surgery under general anesthesia. A 43-year-old man was hospitalized for examination of unidentified fever, and PET detected accumulations in the inguinal and mediastinal lymph nodes. The inguinal lymph node biopsy could not reveal the cause, and he was scheduled for thoracoscopic mediastinal lymph node biopsy. He had been diagnosed CIDP by lower motor weakness and sensory disorder for five months, and underwent peritoneal dialysis for chronic renal failure over the past nine months. Anesthesia was induced with propofol, remifentanil, and high-dose sevoflurane. He could be intubated easily with a left-sided Broncho-Cath double-lumen tube with no muscle relaxants. Anesthesia was maintained by sevoflurane (1.5-1.7%) and remifentanil (0.10-0.15 microg x kg(-1) x min(-1)). After the operation, spontaneous respiration appeared immediately after discontinuing anesthetics. Endotracheal tube was removed because of the following data; Sp(O2), 99%; tidal volume, about 600 ml; respiratory rate, 12-15 min(-1); level of consciousness was good. Arterial blood gas determination at this time revealed pH, 7.418: Pa(CO2), 36.0 mmHg : Pa(O2), 329.3 mmHg under 8 l x min(-1) oxygen. The patient showed an uncomplicated postoperative course.

Optic nerve sheath fenestration for intracranial hypertension associated with chronic inflammatory demyelinating polyneuropathy.

The authors report a case of papilledema in a 61-year-old woman with chronic demyelinating polyneuropathy. The cerebrospinal fluid protein level was elevated (2.68 to 4.51 g/L), as was the cerebro-spinal fluid opening pressure (27 to 40 cm H2O). A unilateral optic nerve sheath fenestration proved to be effective in preserving vision, and the papilledema resolved completely.