Neuronal intranuclear inclusion disease misdiagnosed as CIDP： a case report / 中风与神经疾病杂志

@#A middle-aged male patient，had the chief complaint of recurrent lower limb weakness，and he was misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy. Later the patient attended the hospital again due to lower limb weakness，recurrent headache，and nausea and vomiting. The cerebral magnetic resonance imaging showed diffuse restricted high-signal lesions at the bilateral cerebral corticomedullary junctions； immunohistochemical staining showed that inclusion bodies strongly stained with P62 and ubiquitin antibodies were observed in the nuclei of some sweat gland cells，adipocytes，and fibroblasts； genetic testing showed 142 times of the abnormal amplification of GGC in the NOTCH2NLC gene. Therefore，a confirmed diagnosis of neuronal intranuclear inclusion disease （NIID） was made. This case suggests that we should pay attention to whether the central nervous system is involved when peripheral neuropathy is observed.

Recent advance in pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经医学杂志

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a motor-sensory demyelinating neuropathy mediated by immunity. The pathogenesis is still unclear, and the clinical manifestations are complex. Its diagnoses are difficult, and the treatment effect is not good. At present, most studies consider it to be an autoimmune disease, but the pathogenic antigen and its target to the immune damage mechanism of the disease are still unclear. In this paper, we review the literature on the pathogenesis of CIDP in recent years, and introduce the specific mechanisms which cause CIDP from cellular and humoral immunity.

Anesthesitic management for insulinoma resection in a patient having chronic inflammatory demyelinating neuropathy / Manejo anestésico para resección de insulinoma en un paciente portador de neuropatía desmielinizante crónica

Chronic Inflammatory Demyelinating Polyneuropathy associated with hypoglycemia 2 to insulinoma is unusual, and to our knowledge, very few patients have been reported in literature. Despite varying presentations in these patients, the clinical characteristics are usually the same. The syndrome usually occurs after several episodes of protracted hypoglycemia. The neuropathy is nearly always symmetrical. We report anesthetic management for a young female patient presenting with CIDP & repeated hypoglycemic episodes during a 2-year period scheduled for insulinoma enucleation.

La polineuropatía desmielinizante inflamatoria crónica asociada con hipoglicemia secundaria a insulinoma es inusual y, hasta donde sabemos, muy pocos pacientes han sido reportados en la literatura. A pesar de las diferentes presentaciones en estos pacientes, las características clínicas suelen ser las mismas. El síndrome generalmente ocurre después de varios episodios de hipoglicemia prolongada. La neuropatía es casi siempre simétrica. Presentamos el manejo anestésico para una paciente joven que se presenta con polineuropatía desmielinizante inflamatoria crónica y episodios repetidos de hipoglicemia durante un período de 2 años programado para la enucleación de insulinoma.

Clinical and electrophysiological profiles in early recognition of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome / 中华医学杂志(英文版)

Background@#The detection of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome at early stage is challenging for neurologists. Since polyneuropathy could be the first manifestation, it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.@*Methods@#The data of a group of patients with POEMS (n = 17) and patients with CIDP (n = 17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study. The clinical features, neurological symptoms, and electrophysiological findings were compared between the two groups.@*Results@#Clinically, patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8% vs. 11.8%, P = 0.01). Multisystem features like edema, skin change, organomegaly, and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome. Electrophysiologically, terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve: 0.39 [0.17–0.52] vs. 0.30 (0.07–0.69), Z = –2.413, P = 0.016; ulnar nerve: 0.55 [0.23–0.78] vs. 0.42 [0.12–0.70], Z = –2.034, P = 0.042). Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs. 17.6%, P = 0.031), less conduction block (ulnar nerve: 0 vs. 35.3%, P = 0.018), and less temporal dispersion (median nerve: 17.6% vs. 58.8%, P = 0.032) than CIDP group. The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%, respectively.@*Conclusions@#POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice. The combination of serum monoclonal protein and high TLI might raise the sensitivity of detecting POEMS syndrome.

Diagnostic value of sensory sparing patterns in childhood immune-mediated acute or chronic inflammatory polyneuropathy / 中华神经医学杂志

Objective To investigate the characteristics and significance of sural sparing pattern and radial sparing pattern in children immune-mediated acute or chronic inflammatory peripheral neuropathy.Methods Forty children with Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) aged<14 years, admitted to our hospital from September 2014 to August 2019, were chosen as immune-inflammatory group; 15 children (<14 years old) with multiple peripheral neuropathy of other etiologies from the same source were classified as other etiologies group. The characteristics of sensory nerve damage measured by nerve conduction velocity in the two groups were compared. The existences of sural sparing (SS) pattern and radial sparing (RS) pattern, and sensory ratio (SR)>1 were conformed. SR=(sural nerve sensory nerve action potentials+radial nerve sensory nerve action potentials)/(median nerve sensory nerve action potentials+ulnar nerve sensory nerve action potentials).Results (1) In 40 patients from immune-inflammatory group, 38 were GBS and two were with CIDP; Among the 38 with GBS, 34 were with acute inflammatory demyelinatingpolyneuropathies (AIDP), 3 were with acute motor axonal neuropathy (AMAN), and one was with acute motor sensory axonal neuropathy. Among the 15 patients from the other etiologies group, 9 were with charcot-marie-tooth (CMT) type 1, one was with CMT type 2, one was with CMT intermediate type, and two were with hereditary neuropathy with liability to pressure palsies (HNPP), and two were with mitochondrial disease involves polyneuropathy. (2) As compared with patients from other etiologies group, patients from immune-inflammatory group had significantly higher positive rates of SS (72.5％vs. 6.7％) , RS(42.5％vs.6.7％) and SR>1 (75.0％vs.13.3％). In the diagnoses of childhood acute or chronic inflammatory polyneuropathy, the sensitivity of SS pattern was 0.73, specificity was 0.93, and positive likelihood ratio was 10.88. SR>1 had moderate specificity (0.87) and low sensitivity (0.41).Conclusion The sensory sparing patterns, especially SS pattern, have valuable clinical significance in the diagnoses of acute or chronic inflammatory peripheral neuropathy in children.

Intravenous Immunoglobulin in the Treatment of Neurological Diseases / 대한소아신경학회지

Intravenous immunoglobulin (IVIG) is used in treating many cases of autoimmune and inflammatory conditions thanks to its multiple anti-inflammatory and immunomodulatory properties. The clinical use of IVIG has been for the patients with primary immunodeficiencies, but lately it is expanding its usage to the realms of treating patients with neurological conditions. Both the efficacy and safety of IVIG treatment in chronic inflammatory demyelinating polyradiculoneuropathy and Guillain–Barré syndrome have been studied successfully. However, the use of IVIG treatment in other neurological conditions still remains investigational despite several successful reports. Considerable numbers of mechanisms have been suggested in order to explain the effects of IVIG, but the exact mechanisms are not understood yet. This review covers the new developments in clinical fields and the possible ways in which IVIG could help in the future.

The clinical analysis of diabetic patients with chronic inflammatory demyelinating polyradiculoneuropathy / 中华内科杂志

To explore clinical characteristics,electrophysiological findings and treatment response in diabetic patients with chronic inflammatory demyelinating polyradiculoneuropathy (DM-CIDP). Thirty-six CIDP patients were identified, 10 were DM-CIDP and 26 were non-DM-CIDP. Clinical medical records, electrophysiological data and treatment were retrospectively reviewed.DM-CIDP patients were significantly older than non-DM-CIDP patients [(56.7 ± 10.0) years old vs. (40.4 ± 16.9) years old, P=0.001]. However, clinical characteristics and abnormalities of electrophysiological tests in both groups were comparable. DM-CIDP subjects responded to corticosteroids or intravenous immunoglobulin, thus with better prognosis.

Clinical characteristics of chronic inflammatory demyelinatingpolyradiculoneuropathy in diabetes patients

Background & Objective: Co-exiting of CIDP and diabetes mellitus had been reported. Idiopathic CIDP(I-CIDP) is a treatable disease and had favorable response to immunosuppressive therapies but there is no established data for CIDP in diabetic patients (DM-CIDP). This study aims to determine clinical characteristics, phenotypes, electrophysiological tests and treatment response of CIDP in diabetic patients; and to determine the response to immunosuppressive therapy in DM-CIDP and I-CIDP. Methods: The study was a retrospective chart review of Prasat Neurological Institute patients with diagnosis of CIDP between January 1st, 2008 and December 31th, 2015. Results: Sixty four CIDP patients were identified, 12 were DM-CIDP and 52 were I-CIDP. Clinical characteristics, phenotypes, disease duration and disease severity in DM-CIPD were not different from I-CIDP. Demyelinating changes in nerve conduction studies were not different in the two entities but axonal features were more predominant in DM-CIDP. DM-CIDP also responded to immunosuppressive treatment, with modified Ranking Scale decreased after treatment as in I-CIDP. There was no difference in treatment response in DM-CIDP and I-CIDP. Conclusion: Clinical characteristics, phenotypes, disease severity and treatment response to immunosuppressive treatment in DM-CIPD were not different from I-CIDP. Demyelinating features in nerve conduction studies were not different in the two entities but axonal features were more predominant in DM-CIDP.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy Mimicking Miller-Fisher Syndrome

A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy Mimicking Miller-Fisher Syndrome

A 77-year-old man developed diplopia, gait ataxia, and paresthesia. A clinical examination also revealed ophthalmoplegia, facial palsy, ataxia of the limbs and trunk, and reduced deep tender reflexes. Laboratory and electrophysiological studies revealed albuminocytological dissociation and demyelination. He was diagnosed as Miller-Fisher syndrome and received intravenous immunoglobulin therapy. His clinical symptoms deteriorated at 12 weeks after onset. We diagnosed acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, and which the patient recovered from following corticosteroid therapy.

An Experience of Chronic Inflammatory Demyelinating Polyradiculoneuropathy with Pregnancy.

Pregnancy with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a very rare association. Although pregnancy influences the clinical outcome of CIDP, whether the opposite also occurs is yet to be proved. We have described here successful outcome of a pregnancy experienced with CIDP and tried to emphasize on important management options during gestation.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints / Polineuropatia inflamatoria desmielinizante cronica: qualidade de vida, perfil sociodemografico e queixas fisicas

Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. Method 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). Results The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. Conclusion CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations. .

A avaliação da qualidade de vida (QV) e dos possíveis impactos dos déficits funcionais sobre o estado mental de pacientes com polirradiculoneuropatia inflamatória desmielinizante crônica (PIDC) pode contribuir para a melhor compreensão de aspectos evolutivos da doença. A presente investigação teve como objetivo estudar as atividades da vida diária depacientes com PIDC e avaliar a sua QV. Método Foram avaliados 41 pacientes através do Mini Exame do Estado Mental (MEEM) e do inventário de saúde SF-36®. Resultados A média de idade dos participantes foi 50,6 anos, 63,4% homens. Problemas adicionais de saúde foram referidos por 65,9%: 39% relataram necessitar de ajuda para atividades de vida diária, 49% dormiam menos de 8 horas por noite e 34,1% referiam alguma dificuldade de memória. A média do MEEM foi 26. Através do SF-36 foi verificado maior prejuízo na capacidade funcional; a referência a dor foi proeminente. Conclusão A PIDC pode ter importante impacto social e econômico em decorrência dos prejuízos funcionais primários e secundários que podem levar ao afastamento do trabalho. .

Demyelinative diseases after liver transplantation: The etiology / 第二军医大学学报

Liver transplantation is the only definitive treatment modality of end stage liver diseases. Demyelinative disease is rarely seen in patients after liver transplantation, but a higher incidence has been noticed recently. The disease is liable to be misdiagnosed as immunosuppressant-induced psychiatric disorders at early stage. Central pontine myelinolysis is more disastrous and it will greatly influence the short-term survival and long-term life quality of the patients after liver transplantation. When it is manifested as peripheral nervous system disorder, the disease usually has a subtle onset, making it difficult for diagnosis and treatment. The causes for various demyelinative diseases should be understood to prevent it in patients after liver transplantation. The specific mechanism for demyelinative disease after liver transplantation remains unclear. We introduce the possible causes of common demyelinative diseases, hoping to provide reference for prevention and treatment of such conditions after liver transplantation.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Patient with Systemic Lupus Erythematosus: A case report

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been reported rarely in systemic lupus erythematosus (SLE). We report a case of a 33 year-old woman who was diagnosed as SLE 2 years ago and presented with both feet and hands tingling sensation over 2 months. She also showed clinical features of areflexia, elevated CSF protein, and demyelination in the nerve conduction study. Her lower limb weakness and ataxic gait gradually progressed. Her symptoms which had shown minimal improvement to immunoglobulin therapy responded to immunosuppressant treatment.

A Case of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Perianal Abscess by Klebsiella pneumoniae / 대한소아신경학회지

Chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) is a rare acquired demyelinating disease of peripheral nervous system(PNS), characterized by relapsing or progressive proximal and distal muscle weakness with possible sensory loss. It is one of several chronic neuropathic syndromes that are believed to have an autoimmune etiology. We experienced a case of CIDP in a 3-month-old boy associated with perianal abscess by Klebsiella pneumoniae who had a precipitous onset of symptoms after anesthesia. He was treated with intravenous immunoglobuline with little improvement but showed a remarkable clinical and electrophysiologic improvement after methylprednisolone pulse therapy. We report this case with a brief review of related literature.

Clinical analysis on 32 cases of chronic inflammatory demyelinating polyradiculoneuropathy / 第三军医大学学报

Objective To summarize the clinical, electrophysiological, pathological characteristics of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods From Jan 1990 to Dec 2003, 32 patients of CIDP were admitted by our hospital, 14 male and 18 female. The age range of onset of illness was 13 to 74 years old (averaging 42.56) with the prime period of 40 to 50. Before hospitalization, the course was from 2 months to 5 years. Among all patients, there were 8 cases of a relapsing course and 24 cases of a chronic progressive course. Twenty-four patients were treated with corticosteroids, while 7 received immuglobin (IG) and corticosteroids. The clinical data before and after the treatment was studied retrospectively. Results Most of the patients had subacute or chronic onsets. The common initial symptoms are numbness, paresthesia, and extremity weakness. Diplopia, decreased visual acuity, dysarthria, and dysphagia could also be found initially. It was usually a symmetric sensorimotor neuropathy with either a relapsing course or a chronic progressive course. It could be accompanied with autonomic dysfunction and cranial nerve involvement. Electromyogram demonstrated that the motive and sensory nerve conduction velocities were slow. The sural nerve biopsy showed demyelination and remyelination. `IG and corticosteroids were both effective. Conclusion CIDP might result in widespread peripheral nerve damages, in which autonomic dysfunction and cranial nerve involvement were common. The dominant electrophysiological changes showed peripheral nerve demyelination accompanied by axon degeneration. The sural nerve biopsy played an important role of diagnosis. The treatment with IG and corticosteroids was a most effective way for CIDP nowadays.