Conductive hearing loss in chronic inflammatory demyelinating polyneuropathy(CIDP):A case report

Chronic inflammatory demyelinating polyneuropathy(CIDP) is a progressive autoimmune disorder that targets peripheral nerves. It commonly presents with motor-predominant dysfunction and enlargement of cranial nerves. With regards to hearing loss, a few cases of sensorineural loss have been described. We present a novel case of conductive hearing loss caused by a mass on the tympanic segment of the facial nerve in the setting of CIDP.

Tacrolimus treatment for relapsing-remitting chronic inflammatory demyelinating polyradiculoneuropathy:Two case reports

BACKGROUND This study describes the efficacy of a tacrolimus treatment regimen used to treat two patients with relapsing-remitting chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).CASE SUMMARY Two patients(17-year-old female and 27-year-old male)were enrolled in the current study and were followed up for 12 mo.The first patient was administered tacrolimus(2 mg/d)for 12 mo and prednisolone(40 mg/d)for six months.The second patient was administered tacrolimus(3 mg/d)for six months.Both patients were followed up for 12 mo and the degree of recurrent weakness or normalized motor function was monitored.In addition,nerve conduction studies and tacrolimus levels were recorded.Following tacrolimus treatment,both patients showed marked improvement in clinical outcomes.In the first patient,prednisolone treatment was successfully withdrawn after six months.Sensory as well as motor nerve conduction velocities showed evident recovery following treatment.However,conduction velocities did not completely return to normal,suggesting that electrophysiological recovery can be slower than clinical recovery.CONCLUSION Neither patient exhibited any adverse effects due to the tacrolimus therapy.Therefore,tacrolimus can be effective for the treatment of patients with steroidresistant CIDP.

Spinal canal decompression for hypertrophic neuropathy of the cauda equina with chronic inflammatory demyelinating polyradiculoneuropathy:A case report

BACKGROUND Hypertrophic neuropathy of the cauda equina(HNCE)is a rare disease,especially in children.It can be caused by different etiological agents such as inflammation,tumor or hereditary factors.Currently,there is no uniform standard for clinical treatment of HNCE.Furthermore,it is unclear whether spinal canal decompression is beneficial for patients with HNCE.CASE SUMMARY We report the case of a 13-year-old boy with enlargement of the cauda equina.The onset of the disease began at the age of 6 years and was initially marked by radiating pain in the buttocks and thighs after leaning over and weakness in the lower limbs when climbing a ladder.The child did not receive any medical treatment.As the disease slowly progressed,the child needed the help of others to walk,and he had a trendelenburg gait.He underwent spinal canal decompression and a nerve biopsy during his hospital stay.A diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was made based on electrophysiological findings and pathological examination results.Immunoglobulin or hormone therapy was recommended during hospitalization,but his mother refused.After discharge,the boy’s mother helped him carry out postoperative rehabilitation training at home.His lower-limb muscle strength gradually increased,and he could stand upright and take steps.Six mo after surgery,the child was readmitted and began immunoglobulin therapy.Long-term oral steroid treatment was initiated after discharge.The movement and sensation of the lower limbs were further improved,and the boy could walk normally 1 year after surgery.CONCLUSION Spinal canal decompression can improve the clinical symptoms of HNCE caused by inflammation,even in children.When combined with specific etiological interventions,spinal cord decompression can lead to optimal outcomes.

Tailoring of therapy for chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy （CIDP） is a treatable immune-mediated disorder, which causes in its typical form, symmetric proximal and distal weakness with large fibre sensory impairment involving the four limbs. There are currently three main first-line therapeutic options for CIDP. These consist of corticosteroids, immunoglobulins and plasma exchanges （PE） which have all been found effective in a number of trials conducted over the past several years （Van den Bergh and Rajabally, 2013）. No immunosuppressant therapy has shown benefit in CIDP, although they are utilized by many clinicians in various circumstances despite absence of an evidence base.

Diagnostics of Sensory Ataxia in Patients with Sensory Predominant Chronic Inflammatory Demyelinating Polyneuropathy from Republic of Sakha (Yakutia) and Krasnoyarsk Region

Materials and Methods: A group of healthy volunteers (24 people) and patients with SP-CIDP from Republic of Sakha (Yakutia) (42 people) and Krasnoyarsk region (87 people). Diagnostics Methods: Clinical neurologic, neurophysiological. Results: The results of stabilometry research of patients with SP-CIDP have revealed area expansion of pressure centre in phase EO and EC with deflection PC forward by anteropulsion type among patients with SP-CIDP from Republic of Sakha (Yakutia). Also in the Yakut group has been noted to have severer clinical course in comparison with inhabitants of Krasnoyarsk region. Conclusion: The method of computer stabilometry allows estimating objectively presence and degree of manifestation of sensitive ataxia in patients with SP-CIDP.

Idiopathic inflammatory demyelinating diseases of the central nervous system in patients following allogeneic hematopoietic stem cell transplantation： a retrospective analysis of incidence, risk factors and survival

Background AIIogeneic hematopoietic stem cell transplantation （allo-HSCT） is a curative therapy for many hematological diseases, but there are many complications following alIo-HSCT, among which neurological complications （NC） are one of the most commonly described ones. However, little is known about idiopathic inflammatory demyelinating diseases （IIDDs） of the central nervous system （CNS） in patients following alIo-HSCT. Methods A nested case-control study was conducted in a large cohort of 1365 patients, who underwent alIo-HSCT at the Institute of Hematology and Peking University People＇s Hospital, between January 2004 and December 2009, 36 patients of whom developed CNS IIDDs. Kaplan-Meier method, univariate and multivariate Cox regression were applied in our statistical analysis using SPSS 16.0. Results The cumulative incidence of all cases of IIDDs at 6 years posttransplantation was 3.6%. Thirty-five patients （97.2%） suffered IIDDs after transplantation, 16 patients （44.4%） between day 0 to day 100 post-transplantation, 10 patients （27.8%） between day 100 to 1 year post-transplantation, and 9 patients （25.0%） 1 year post-transplantation. Multivariate regression analysis identified donor type （P=0.031）, infection （P=0.009）, and acute lymphatic leukemia （P=0.017） as independent risk factors for posttransplantation IIDDs. The median survival time of patients with IIDDs was 514 days after transplantation （95% CI： 223-805）. Survival at 6 years was significantly lower in patients who developed the diseases compared to those who did not （26.6% vs. 73.5%, P 〈0.001）. Of the 36 patients experiencing IIDDs, 58.3% （n=21） died. The causes of death were graft-versus-host disease （GVHD） （n=4）, underlying disease relapse （n=3）, infections （n=12）, and other causes （n=2）. Conclusions IIDDs is an uncommon but serious complication of alIo-HSCT, especially in patients with a primary diagnosis of acute lymphatic leukemia, mismatched transplants, and infections. Our study results indicate that patients with IIDDs tend toward a poor prognosis following alIo-HSCT.

Demyelinating polyneuropathy and lymphoplasmacytic lymphoma coexisting in 36-year-old man:A case report

BACKGROUND Lymphoplasmacytic lymphoma is a rare non-Hodgkin’s lymphoma,occurring mostly in the elderly.It develops slowly and leads to malignant proliferation of lymphoid line cells in the bone marrow,lymph nodes and spleen.It may also affect nerve roots and meninges;some patients develop sensorimotor polyneuropathy which may precede general symptoms of lymphoma.CASE SUMMARY We present a case of a 36-year-old man diagnosed in 2012 with chronic inflammatory demyelinating polyneuropathy(CIDP),then he was hospitalized in 2019 due to progressive symptoms of heart failure and significant weight loss over the previous four months.Based on clinical and laboratory findings a diagnosis of lymphoplasmacytic lymphoma was suspected and confirmed by bone marrow flow cytometry.There was no improvement in the results of laboratory tests and the patient's condition after immediate implementation of chemotherapy.Patient died on the fifth day of treatment.CONCLUSION While CIDP and malignant disease co-occurrence is rare,it should be suspected and investigated in patients with atypical neuropathy symptoms.

Nerve biopsy findings contribute to diagnosis of multiple mononeuropathy: 78% of findings support clinical diagnosis

Multiple mononeuropathy is an unusual form of peripheral neuropathy involving two or more nerve trunks. It is a syndrome with many different causes. We reviewed the clinical, electrophysi- ological and nerve biopsy findings of 14 patients who suffered from multiple mononeuropathy in our clinic between January 2009 and June 2013. Patients were diagnosed with vasculitic neurop- athy （n = 6）, perineuritis （n = 2）, chronic inflammatory demyelinating polyradiculoneuropathy （n = 2） or Lewis-Sumner syndrome （n = 1） on the basis of clinical features, laboratory data, elec- trophysiological investigations and nerve biopsies. Two patients who were clinically diagnosed with vasculitic neuropathy and one patient who was clinically diagnosed with chronic inflamma- tory demyelinating polyradiculoneuropathy were not confirmed by nerve biopsy. Nerve biopsies confirmed clinical diagnosis in 78.6% of the patients （11/14）. Nerve biopsy pathological diagno- sis is crucial to the etiological diagnosis of multiple mononeuropathy.

A rare presentation of Guillain-Barre syndrome with GQ1b positivity:A case report

Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presented with headache,vomiting,and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking,13 days after ChAdOx1 nCoV-19 vaccination.Diagnosis:Guillain-Barre syndrome with GQ1b positivity.Intervention:Five cycles of plasmapheresis were given.Outcome:The patient’s clinical condition improved.Palatal weakness improved and she could walk without support.There were mild sensory symptoms involving upper limbs which gradually improved.Lessons:AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination.Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.

Insights into Initial Demyelinating Episodes of Central Nervous System during Puerperium

Background：Inflammatory demyelinating disease of central nervous system （CNS） is an inflammatory disease characterized by a high childbearing female predominance.Labor-related alterations for postpartum demyelinating attacks are not entirely clear.This study aimed to summarize clinical features of female patients of reproductive age with initial CNS inflammatory demyelinating attacks during puerperium.Methods：Fourteen female patients with initial demyelinating events during puerperium between January 2013 and December 2016 were retrospectively studied.Records of clinical features,neuroimaging,serum antibodies,cerebrospinal fluid （CSF） findings,annualized relapse rate （ARR）,and treatment were analyzed.Results：Among 14 patients,5 patients were diagnosed with multiple sclerosis （MS）,four as neuromyelitis optica （NMO）,two as longitudinal extensive transverse myelitis,two as clinical isolated syndrome （CIS）,and one as acute brainstem syndrome.All the 14 puerperal female patients presented with more than one manifestation of hemiplegia,paraplegia,uroschesis,visual loss or dysarthria,and with mild to moderate abnormalities of CSF.Attacks occurred during the first trimester postpartum and cesarean section was the main delivery way （n =10）.Median Expanded Disability Status Scale （EDSS） scores were 5.0 （range：2.0-9.0） at the onset and 2.5 （range：0-7.0） at the end of follow-ups.Patients with MS and CIS had a significantly lower EDSS scores than patients with NMO spectrum disorders （P 〈 0.05）.Median ARR was 0.46 （range：0-1.16）;all patients had a low ARR （0.49 ± 0.34,95% confidence interval：0.29-0.69） with standardized treatments.Conclusion：Labor-related alterations in the mother＇s immune system might result in newly-onset demyelinating diseases of central nervous system.

Different distributions of nerve demyelination in chronic acquired multifocal polyneuropathies

Background:Multifocal motor neuropathy(MMN),Lewis-Sumner syndrome(LSS),and many chronic inflammatory demyelinating polyradiculoneuropathies(CIDPs)are representative of acquired multifocal polyneuropathy and are characterized by conduction block(CB).This retrospective study aimed to investigate the demyelinating distribution and the selective vulnerability of MMN,LSS,and CIDP with CB(CIDP-CB)in nerves.Methods:Fifteen LSS subjects(107 nerves),24 MMN subjects(176 nerves),and 17 CIDP-CB subjects(110 nerves)were included.Their clinical information was recorded,blood and cerebrospinal fluid tests were conducted,and nerve conductions of the median,ulnar,radial,peroneal,and tibial nerves were evaluated.CB,temporal dispersion,distal motor latency(DML),and F-wave latency were recorded,and nerve conduction velocity,terminal latency index,and modified F-wave ratio were calculated.Results:CB was more likely to occur around the elbow in CIDP-CB than in MMN(78.6%vs.6.8%,P<0.01)but less likely to occur between the wrist and the elbow than in LSS(10.7%vs.39.3%,P<0.05).Tibial nerve CB was most frequently observed in MMN(47.4%,P<0.05).CIDP-CB was characterized by a prolonged DML in all nerves,and slow motor nerve velocity of the upper limb was significant when CB nerves were excluded(P<0.05).Conclusions:We report the different distributions of segmental and diffuse demyelination of the ulnar and tibial nerves in LSS,MMN,and CIDP-CB.These distinct distributions could help in differentiating among these conditions.

Mind the gap:acute bilateral vocal cord palsy in CIDP after extending the IVIG treatment interval?

Cranial nerve involvement is rarely seen in chronic inflammatory demyelinating polyneuropathy(CIDP).We present a patient diagnosed with CIDP who was in a stable medical condition under long-term treatment with intravenous immunoglobulin(IVIG)every five weeks for more than seven years.Following a 12-day delay in the patient's regular IVIG therapy,he developed acute bilateral vocal cord palsy.The patient had to be intubated and tracheostomized because of acute respiratory distress.Weaning from mechanical ventilation was complicated due to pneumonia.After antibiotic treatment and restarting IVIG therapy vocal cord palsy rapidly improved allowing for subsequent decannulation.Although coincidence between treatment delay and symptom development does not prove definitive causality this case report may serve as a reminder how time critical IVIG therapy can be for sufficient symptom control.Moreover,it provides evidence that IVIG therapy may be effective for the treatment of cranial nerve symptoms in CIDP.