Proton pump inhibitors and risk of bloodstream infection without an identifiable source: a hospital-based case-control study.

The association between proton-pump inhibitor (PPI) use and systemic infections caused by bacterial translocation is unclear. This study aims to investigate whether patients receiving PPI therapy have a higher risk for bloodstream infections (BSI) without an identifiable source of infection, as an alternative indicator of BSI secondary to bacterial translocation. We conducted a hospital-based case-control study which enrolled all patients aged 20 years and older who developed BSI confirmed by two sets of positive blood culture and had inpatient care in Ichinomiya-Nishi Hospital in 2019. Patients' data were collected from medical records, and bacterial translocation type (BT-type) BSI group were defined as those who had BSI without an identifiable source of infection, whereas the others were classified control group based on the diagnostic criteria for each infectious disease. We analyzed data from 309 patients, including 66 cases and 243 controls. PPI users had a 2.4-fold higher risk of developing BT-type BSI compared to non-PPI-users after controlling for potential confounders (OR: 2.41, 95% CI: 1.29-4.51, p=0.006). In conclusion, PPI use is associated with higher risk of BSI without an identifiable source and therefore, PPI use may increase the risk of septic morbidity secondary to bacterial translocation.

Lidocaine Tape Application for 3 Hours Prevents Vasovagal Syncope During Venipuncture: A Case Series.

Vasovagal syncope (VVS) or reflex is usually caused by physical and mental stress-related factors, like pain, anxiety, and fear, and it is one of the most frequent complications during dental treatment. Two patients, both with histories of dental phobia and of VVS during vaccination, venipuncture, and dental treatment with local anesthetics, were scheduled for dental treatment under intravenous (IV) sedation. However, both experienced episodes of VVS that occurred during venipuncture using a 24-gauge indwelling needle. We determined that pain was the main trigger of VVS for these patients and attempted to reduce venipuncture-associated pain using 60% lidocaine tape applied 3 hours before venipuncture at their next dental visits, respectively. Use of the lidocaine tape was successful and permitted comfortable placement of the IV catheter without any onset of VVS.

Cross-Sectional Analysis of Fall-Related Factors with a Focus on Fall Prevention Self-Efficacy and Self-Cognition of Physical Performance among Community-Dwelling Older Adults.

This study aimed to determine how fall prevention self-efficacy and degree of deviation in self-cognition of physical performance, which have recently received attention for their potential to explain falls in combination with a wide variety of fall-related factors, as well as affect falls. Older adults using day-care services (n = 27 with six men, mean age: 81.41 ± 7.43 years) were included in this study. Fall history in the past year, the modified fall efficacy scale (MFES), and physical performance and cognition errors were examined by evaluating the functional reach test (FRT), the stepping over test, and the timed up and go test (TUG), along with a questionnaire. In the fall (n = 14) and non-fall (n = 13) groups, logistic regression analysis using Bayesian statistical methods was used to identify factors associated with falls. The odds ratios for the MFES ranged from 0.97 to 1.0, while those of cognition-error items ranged from 3.1 to 170.72. These findings suggested that deviation in self-cognition of physical performance, particularly overestimation of timed cognitive ability, was a factor with more explanatory power for fall history. Future studies should analyze differences by disease and age group, which were not clarified in this study, to identify more detailed fall risk factors.

Spinocerebellar ataxia type 17-digenic TBP/STUB1 disease: neuropathologic features of an autopsied patient.

Spinocerebellar ataxia (SCA) type 17-digenic TBP/STUB1 disease (SCA17-DI) has been recently segregated from SCA17, caused by digenic inheritance of two gene mutations - intermediate polyglutamine-encoding CAG/CAA repeat expansions (polyQ) in TBP (TBP41 - 49) and STUB1 heterozygosity - the former being associated with SCA17, and the latter with SCA48 and SCAR16 (autosomal recessive). In SCA17, most patients carry intermediate TBP41 - 49 alleles but show incomplete penetrance, and the missing heritability can be explained by a new entity whereby TBP41 - 49 requires the STUB1 variant to be symptomatic. The STUB1 gene encodes the chaperone-associated E3 ubiquitin ligase (CHIP) involved in ubiquitin-mediated proteasomal control of protein homeostasis. However, reports of the neuropathology are limited and role of STUB1 mutations in SCA17-DI remain unknown. Here we report the clinicopathologic features of identical twin siblings, one of whom was autopsied and was found to carry an intermediate allele (41 and 38 CAG/CAA repeats) in TBP and a heterozygous missense mutation in STUB1 (p.P243L). These patients developed autosomal recessive Huntington's disease-like symptoms. Brain MRI showed diffuse atrophy of the cerebellum and T2WI revealed hyperintense lesions in the basal ganglia and periventricular deep white matter. The brain histopathology of the patient shared features characteristic of SCA17, such as degeneration of the cerebellar cortex and caudate nucleus, and presence of 1C2-positive neurons. Here we show that mutant CHIP fails to generate the polyubiquitin chain due to disrupted folding of the entire U box domain, thereby affecting the E3 activity of CHIP. When encountering patients with cerebellar ataxia, especially those with Huntington's disease-like symptoms, genetic testing for STUB1 as well as TBP should be conducted for diagnosis of SCA17-DI, even in cases of sporadic or autosomal recessive inheritance.

Factors Affecting the Life Satisfaction of Older People with Care Needs Who Live at Home.

The number of older people is increasing rapidly, and the number of older people with care needs who live at home is also increasing in Japan. Maintaining their life satisfaction has been a primary challenge. This study aimed to identify factors affecting the life satisfaction of older people with care needs. The study was conducted among older people using homecare services; 126 participants (mean age, 79.33 ± 7.51 years, 54 male) were included in the analysis. Logistic regression analysis with adjustment for age, sex, and economic status was conducted with life satisfaction as the objective variable and the Japanese version of occupational gaps questionnaire (OGQ-J), sense of coherence, functional independence measure, and environmental factors as explanatory variables. The variables that significantly affected life satisfaction were the OGQ-J (p = 0.0352, OR 0.90, 95% CI 0.81-0.99) and environmental factors (p = 0.0083, OR 4.41, 95% CI 1.52-14.11). This study's results indicate the importance of focusing on environmental factors and facilitating the participation of older people with care needs in activities they want to do to maintain and improve their life satisfaction.

A novel auricular prosthesis which incorporates a cartilage conduction hearing aid based on 3D data processing technique: a preclinical evaluation.

PURPOSE: To achieve the challenging goal of simultaneously improving aesthetics and auditory function in the treatment of microtia, we developed an auricular prosthesis incorporating a cartilage conduction hearing aid (APiCHA), which covers a cartilage conduction hearing aid (CC-HA). We evaluated the acoustic characteristics generated by the method of combining these two components using an artificial mastoid and investigated the possibility of clinical translation. METHODS: The first step in creating an APiCHA is to take molds of both the affected and normal auricles and invert the 3D data of the normal auricle to create data for the auricular prostheses. Grooves were then made inside the prosthetic data to fit the affected ear and a CC-HA, and the APiCHA was made of silicone. The acoustic characteristics were measured using an artificial mastoid, and the results were compared between CC-HA alone and with APiCHA. RESULTS: Compared with CC-HA alone, the gain was approximately 2 dB lower at high frequencies from 1 k Hz and higher, and approximately 2 dB higher at approximately 900 Hz when CC-HA was used with APiCHA. For the other frequencies, the acoustic characteristics were almost the same. CONCLUSION: The changes in acoustic characteristics caused by the combined use of APiCHA and CC-HA were minimal and did not pose a clinical problem. The combined use of APiCHA and CC-HA can be considered as a non-invasive and clinically applicable treatment option to achieve both aesthetic and auditory improvements for microtia.

A Case of Anaphylaxis in Which a Basophil Activation Test Was Used to Identify the Suspected Agent.

This is a case report of anaphylaxis in which the basophil activation test (BAT) was used to identify the etiological agent. Although skin tests are considered the most effective methods for identifying anaphylactic triggers, the test itself presents a risk of inducing anaphylaxis. The BAT is advantageous because of its inherent lack of risk, high sensitivity and specificity to identify the suspected anaphylactic agents, and diagnostic accuracy comparable to conventional skin testing. Therefore, in the future, the BAT is likely to become the preferred test for the detection of allergens over conventional skin tests.

Immunotherapy-refractory vacuolar myopathy with mucin deposition in scleromyxedema: A possible role of fibroblast growth factor 2.

Scleromyxedema (SME) is characterized by widespread waxy papules on the skin, with mucin deposits in the upper dermis. Twenty-one SME cases of myopathy have been reported; of the cases, six showed vacuolar formation, and two showed mucin deposition. We report the first case of SME with mucin-associated vacuolated fibers. A 45-year-old woman with SME developed progressive proximal muscle weakness. Muscle biopsy revealed myopathic changes with numerous vacuoles linked to mucin in the affected muscle fibers, which were heavily immunostained for fibroblast growth factor 2 (FGF2). Despite repeated high dose oral prednisolone and intravenous immunoglobulin administrations, muscle weakness recurred continuingly, culminating in death due to congestive heart failure. Immunotherapy was partly effective in our case, although it was refractory. Treatment responsiveness in patients with SME myopathy varied; however, due to its rarity, the mechanism remains to be elucidated. To address this issue, we investigated muscle specimens immunohistochemically and detected marked upregulation of FGF2 in the affected muscle fibers of our patient. FGF2, a strong myogenesis inhibitor, may exert a suppressive effect on muscle fiber regeneration, which may have conferred refractoriness to our patient's SME myopathy.

Use of the Microcuff® During General Anesthesia for Patients With Scoliosis.

Scoliosis may often be associated with a variety of cardiovascular and respiratory conditions or diseases, and depending on the severity of the spinal deformity, it may also complicate anesthetic management because of the difficulty of neck extension and tracheal deformity. Therefore, patients with scoliosis may require careful perioperative anesthetic considerations. A 14-year-old girl was scheduled to undergo extractions and restorative treatment for dental caries under general anesthesia. Her medical history was significant for intellectual disability and autism as well as previously undiagnosed scoliosis. After fixation of a 6.0 Portex® endotracheal tube (ETT), percutaneous oxygen saturation (SpO2) decreased to 93%, peak airway pressures increased, and unilateral lung ventilation was noted. Inadvertent mainstem bronchial intubation was immediately suspected, prompting removal of the Portex ETT and reintubation with a shorter 6.0 Microcuff® ETT. The dental treatment was completed successfully without further incident. Assessment of the ETTs used intraoperatively led to the determination that the distance from the glottis to the carina was considerably shorter than normal for this patient. It was speculated that the Microcuff ETT may be optimal for anesthetic management of scoliosis patients because of its shorter lengths compared with other style ETTs, which may reduce the risk of bronchial intubation in such cases.

Discriminative clinical and neuroimaging features of motor-predominant hereditary diffuse leukoencephalopathy with axonal spheroids and primary progressive multiple sclerosis: A preliminary cross-sectional study.

BACKGROUND: Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is a rare autosomal-dominant white matter disease, typically characterized by juvenile cognitive decline and frontoparietal white matter lesions. A portion of HDLS patients exhibit preferential motor dysfunctions as their initial symptoms, mimicking multiple sclerosis (MS). However, there is no study comparing this phenotype of HDLS and primary progressive multiple sclerosis (PPMS), which greatly resemble each other. This is the first preliminary study to clarify the clinical and neuroimaging features of motor-predominant HDLS, and compare it with PPMS, using cases whose colony stimulating factor 1 receptor (CSF1R) were sequenced. METHODS: Clinical and radiological data from Japanese patients at the Department of Neurology, Kyushu University Hospital, Fukuoka, Japan, were evaluated retrospectively and cross-sectionally. Twenty-nine brain and 18 spinal cord magnetic resonance imaging (MRI) scans from four motor-predominant HDLS patients with CSF1R mutations and 15 PPMS patients without CSF1R mutations, were evaluated using an HDLS MRI scoring system. RESULTS: Two patients with HDLS were initially diagnosed with MS and received immunotherapy. Clinically, motor-predominant HDLS and PPMS patients resembled each other in onset age and disability. However, motor-predominant HDLS patients had a significantly higher frequency of frontal release signs, lower positivity rates of oligoclonal IgG bands (OCB), and lower IgG index values. Total HDLS MRI scores, total white matter lesions (WMLs), and brain atrophy were similar between the diseases. However, motor-predominant HDLS patients had more marked atrophy of the corpus callosum (CC) body, more WMLs in the deep and subcortical regions of the frontoparietal lobes, fewer WMLs in the occipitotemporal periventricular regions, and more restricted diffusivity lesions on MRI than PPMS patients. There was a stronger association between disease duration and CC index in HDLS, suggesting more rapid progression compared with PPMS. CONCLUSIONS: Motor-predominant HDLS has characteristic frequent frontal release signs, normal findings for OCB and the IgG index, severe CC body atrophy, abundant deep and subcortical WMLs in the frontoparietal lobes, subtle occipitotemporal lobe periventricular WMLs, and more restricted diffusivity lesions on MRI. Although the present study was limited by the small number of HDLS cases, we propose that immunotherapy should be avoided in such cases.

Downregulation of Neuronal and Dendritic Connexin36-Made Electrical Synapses Without Glutamatergic Axon Terminals in Spinal Anterior Horn Cells From the Early Stage of Amyotrophic Lateral Sclerosis.

Connexin36 (Cx36) forms gap junctions between neurons, which are called electrical synapses, enabling adjacent neurons to communicate directly. The participation of chemical synapses in neurodegeneration in amyotrophic lateral sclerosis (ALS) has long been indicated, but it remains unclear whether electrical synapses are involved in the pathogenesis of ALS. We performed extensive immunopathological analyses using mutant superoxide dismutase 1 (SOD1G93A) transgenic mice and their littermates to investigate whether Cx36-made electrical synapses are affected in motor neuron diseases. We found that in the lamina IX of the lumbar spinal cord from wild type mice, about half of the Cx36 puncta existed independently of chemical synapse markers, while the rest coexisted with chemical synapse markers, such as vesicular glutamate transporter 1 (VGLUT1), which is a glutamatergic axon terminal marker, and/or glutamate decarboxylase 65 (GAD65), which is a GABAergic axon terminal marker. Cx36 single or Cx36/GAD65 double positive puncta, but not VGLUT1-containing puncta, were preferentially decreased on neuronal and dendritic surfaces of the anterior horn cells in the early stage of SOD1G93A ALS mice. Moreover, in five human autopsied sporadic ALS cases with bulbar or upper limb onset, Cx36 immunoreactivity was diminished in the proximal dendrites and neuropils of well-preserved large motor neurons in the lumbar anterior horns. These findings suggest that downregulation of neuronal and dendritic Cx36 in the spinal anterior horns commonly occurs from the early stage of hereditary and sporadic ALS. Cx36-made electrical synapses without glutamatergic signaling appear to be more vulnerable than other chemical synapses and electrical synapses with glutamatergic signaling in the early stage of motor neuron degeneration, suggesting involvement of Cx36-made electrical synapses in the pathogenesis of human ALS.

Risk of active tuberculosis among people with diabetes mellitus: systematic review and meta-analysis.

OBJECTIVE: To assess the risk of active TB in people with DM and the factors associated with this risk. METHODS: Systematic review and meta-analysis. We searched the literature for studies that reported the effect of DM on TB controlled for the effect of age. Studies that had not established the diagnosis of DM prior to detecting active TB were excluded. Study quality was assessed by Newcastle-Ottawa scale and we conducted a meta-analysis using random-effects models. RESULTS: Of 14 studies (eight cohort and six case-control studies) that involved 22 616 623 participants met the selection criteria and were included in the analysis. There was substantial variation between studies in the estimates of the effect of DM on TB. However, the pooled estimates from seven high-quality studies showed that diabetic people have a 1.5-fold increased risk of developing active TB vs. those without DM (95%CI 1.28-1.76), with relatively small heterogeneity (I2  = 44%). The increased risk of TB was observed predominantly among DM populations with poor glycaemic control. CONCLUSION: There is evidence suggesting an increased risk of developing TB among people with DM, and that improving glycaemic control in DM patients would reduce the risk of developing TB. An integrated approach is needed to control the dual burden of DM and TB.

Hopkins syndrome following the first episode of bronchial asthma associated with enterovirus D68: a case report.

BACKGROUND: Hopkins syndrome (HS) is a rare disorder presenting with acute flaccid paralysis of the limbs following an asthma attack. Neurologists encounter a diagnostic challenge if patients without a history of bronchial asthma develop neurologic features mimicking HS following acute respiratory distress. We report a case of HS occurring after a first episode of bronchial asthma associated with enterovirus D68 infection. CASE PRESENTATION: A 5-year-old girl developed acute respiratory distress. On the fourth hospital day, both her legs became paralyzed except for slight muscle contraction in the right lower limb. Tendon reflexes in the lower limbs were diminished and there was a positive Babinski sign on the right. Sensation was normal in all modalities, and there was no uro-rectal disturbance. Spinal magnetic resonance imaging identified T2-hyperintense lesions with spinal cord edema, mainly involving the bilateral T11 to L1 anterior horns, with left side dominance extending to the left posterior horn. The neurological and neuro-radiological findings of our case were suggestive of HS; however, she had no history of bronchial asthma. An acetylcholine inhalation challenge eventually proved the presence of reversible airway hyper-responsiveness, allowing us to diagnose HS. We identified enterovirus D68 in the patient's intratracheal aspirates using a sensitive polymerase chain reaction assay. Intravenous immunoglobulin administrations at 2 g/kg2 for 5 consecutive days were repeated every month up to four times. After these treatments, the muscle strength of her right lower limb slightly improved while her left lower leg remained completely paralyzed. CONCLUSION: This case emphasizes the importance of provocation tests to reveal the presence of airway hyper-responsiveness when a child shows neurological signs mimicking HS following acute respiratory distress. Furthermore, the present case suggests a possible link between HS and acute flaccid paralysis following lower respiratory tract infection by enterovirus D68.

Development of an external-to-internal convertible endoscopic biliary drainage device - a preliminary prospective feasibility study.

BACKGROUND AND STUDY AIMS: Endoscopic nasobiliary drainage (ENBD) for a malignant stricture in the bile duct has some advantages over endoscopic biliary stenting (EBS). However, ENBD may cause nasopharyngeal discomfort. We developed an external-to-internal convertible endoscopic biliary drainage (ETI-EBD) device that enables both internal and external drainage to occur during a single endoscopy. PATIENTS AND METHODS: This device consists of three parts, comprising a 5-Fr ENBD tube (250 cm) (ENBD-t), an 8.5-Fr EBS tube (7 cm) (EBS-t), and an 8-Fr pusher tube for EBS (230 cm) (P-t). The EBS-t is mounted over the ENBD-t at the distal end of the ENBD-t. The P-t is also placed over the ENBD-t. After an endoscopic sphincterotomy, the EBS-t of the device is inserted into the papilla, then the duodenal endoscope is withdrawn, leaving the device in place. After ENBD, only the ENBD-t was withdrawn from the P-t. At this point, the EBS-t was isolated and left without endoscopy or radiography. RESULTS: ETI-EBD was successfully placed in all consecutive 21 patients (100 %). The release of EBS-t from ENBD-t wit was successfully completed in 19 patients (90.5 %). There were 4 patients with kink of P-t when exchanging this device from the mouth to the nose. It was difficult for 2 patients to withdraw the ENBD-t because of poor lubrication performance. There were no significant complications associated with the use of the device. CONCLUSION: This device allows for both external and internal biliary drainage with a single endoscopy.

Meningeal plasma cell granuloma in the early stage of relapsing polychondritis.

A 77-year-old man showed an asymptomatic meningeal lesion beneath the dura matter in the left fronto-parietal region on MRI during an examination for recurrent hoarsness. The lesion showed no gadolinium enhancement, and extended to neither the sulci nor skull. Neurological examinations revealed hoarseness, cochlear and vestibular dysfunction of the right ear, and mildly decreased Achilles tendon reflexes bilaterally. Laboratory findings showed marked inflammatory responses, but no abnormalities for LDH, IgG4, angiotensin-converting enzyme, or soluble IL-2 receptor. There was no serum monoclonal protein. Autoantibody panels in the serum were unremarkable except for an elevation of anti-type II collagen antibodies to a borderline value. Cerebrospinal fluid analysis disclosed an elevated protein concentration (152 mg/dl) and IgG index (1.41) with normal cell counts, negative results for bacterial/tubercular infection, and a normal cytology. 18F-fluorodeoxyglucose positron emission tomography showed increased uptake in the left frontal region (Max SUV: 7.54). Swelling of the vocal cord, arytenoid cartilage, false vocal cord, and vocal cord palsy on the right side were seen on laryngoscopy, all of which were ameliorated by dexamethasone administration. A meningeal biopsy contained the dura matter and arachnoid, in which a granulation composed of massive mature plasma cells with many Russel bodies, accompanied by occasional lymphocytes and histiocytes were observed. Three months after the biopsy, he developed bilateral auricular chondritis and conjunctivitis. Based on these findings, we diagnosed him with relapsing polychondritis (RP). Prednisolone administration (40 mg/day) improved the chondritis and meningeal lesion. Central nervous system involvement is rare in patients with RP, and meningeal complications, such as aseptic meningitis or pachymeningitis, are mostly observed after the diagnosis of RP. However, due to its rarity, it still remains to be clarified whether a similar pathogenesis of meningeal complications underlies RP. The present case is indicative in that predominant meningeal granuloma arose during chondritis of several portions that had gradually developed, which suggests that meningeal complications could be derived from RP.

A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait. Tendon reflexes were brisk throughout, and Babinski and Chaddock reflexes were both positive bilaterally. Laboratory tests revealed elevated lactate and pyruvate concentrations in both serum and cerebrospinal fluid. Fluid attenuated inversion recovery magnetic resonance imaging showed high intensity lesions in the bilateral cerebellar hemispheres, pyramidal tracts in the brainstem, and internal capsules symmetrically. Brain magnetic resonance spectroscopy measurements revealed an elevated lactate/creatine plus phosphocreatine ratio and a decreased N-acetyl-aspartate/creatine plus phosphocreatine ratio in the cerebellum. At this point, mitochondrial diseases, particularly myoclonic epilepsy with ragged-red fibers (MERRF), to be the most likely cause. We performed a biopsy of his left biceps brachii muscle, showing variations in fiber size with occasional central nuclei and very few ragged-red fibers. Blood mitochondrial respiratory enzyme assays showed normal values with elevated citrate synthase activity, and mitochondrial DNA analyses for MERRF revealed no pathogenic mutations. We then explored other possibilities and detected an elevated serum cholestanol concentration of 20.4 µg/ml (reference value <4.0) and genetic analysis by direct sequencing method disclosed a novel frame-shift mutation (c. 43_44delGG) in CYP27A1 gene exon1, leading to a diagnosis of cerebrotendinous xanthomatosis (CTX). This case emphasizes importance of awareness of CTX as a possibility when patients present with clinical phenotypes mimicking mitochondrial diseases, but with negative results for muscle pathology or genetic analyses. The measurements of serum cholestanol concentrations might be useful in diagnosing such atypical cases.

A case of severe chronic inflammatory demyelinating polyradiculoneuropathy with monoclonal gammopathy of undetermined significance with alternating immunoglobulin class to IgM from IgA.

A 71-year-old woman with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with IgA-λ monoclonal gammopathy of undetermined significance (MGUS) showed the acute development of tetraplegia, respiratory failure, and a marked fluctuation of the blood pressure. Intravenous (IV) high-dose steroid therapy (methylprednisolone: 1 g/day × 3 days), followed by oral prednisolone (PSL) (40 mg/day), and IV immunoglobulin (IVIg, 0.4 g/kg/day × 5 days) administrations resulted in the amelioration of these symptoms. However, they soon relapsed, which eventually led to complete tetraplegia and the need for mechanical ventilation. At this time, serum components of IgA-λ and IgM-λ were biclonally positive. Seven courses of plasma exchange and the alternative administration of dexamethasone (12 mg/day) and methtorexate (15 mg/week) were conducted, but with no significant improvement. Nine months after admission, she showed totally-locked in syndrome. Cryo-preserved serum obtained at this time showed high titers of IgM class antibodies against ganglioside (GD3 +++, GT1a ++++, GT1b ++, GQ1b +++, and GD1b +++), which had been negative on admission. Biopsy of the left sural nerve showed moderate reductions of large and small myelinated fibers with no inflammation, no depositions of amyloid, IgG, IgA, or IgM, and teased fiber findings revealed neither myelin ovoids nor segmental demyelination. Alternatively, melphalan at 5 mg and PSL at 32 mg were administered, with no amelioration, while serum IgA-λ monoclonal protein diminished, and IgM-λ M protein positivity was continuously observed. She frequently developed sepsis; therefore, we could no longer continue any immunosupressive therapies, but monthly IVIg administrations were given. Twelve months after admission, her neurological symptoms gradually improved and she was weaned off of mechanical ventilation. Eighteen months after admission, her muscle strength corresponded to 2 on manual muscle testing, and wheelchair transfer became possible. To the best of our knowledge, the present case is the first report of CIDP with MGUS showing an alternating immunoglobulin class.