The key points in diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy relies on clinical features, demyelinating changes on motor and sensory nerve conduction studies, elevated cerebrospinal fluid protein, peripheral nerve image, nerve pathology, and response to immune therapy. Each diagnostic proof should be interpreted in light of clinical background and other findings, to avoid misdiagnosis or overdiagnosis, treatment delay or unnecessary treatment with immunotherapy.

Research advances in chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune disease affecting the peripheral nervous system mediated by cellular and humoral immunity, characterized by limb weakness and sensory impairment. The main feature of CIDP by electrophysiological and pathological examinations is the demyelination of peripheral nerves. First-line treatment for CIDP includes glucocorticoids, intravenous immunoglobulins, and plasmapheresis. Some patients respond to current treatment not well and have a poor prognosis. Progress in the pathogenesis, diagnosis, and treatment of CIDP worldwide was reviewed in this article, aiming to provide references for the clinical diagnosis and treatment of CIDP.

Chronic inflammatory demyelinating polyneurophaty: assessment of the cognitive function and quality of life / Polineuropatia desmielinizante inflamatória crônica: avaliação da função cognitiva e qualidade de vida

Abstract Background Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed. Objectives To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters. Methods Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36). Results The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, p = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, p < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps. Conclusion There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways.

Resumo Antecedentes Estudos foram realizados com o objetivo de avaliar a qualidade de vida (QV) de pacientes acometidos pela polirradiculopatia desmielinizante inflamatória crônica (PDIC). No entanto, a questão cognitiva ainda é pouco abordada. Objetivos Avaliar a QV e o comprometimento cognitivo em pacientes com PDIC bem como se existe correlação entre esses parâmetros. Métodos Sete pacientes com PDIC e sete controles pareados foram submetidos a: miniexame do estado mental (MEEM); avaliação cognitiva de Montreal (MoCA); teste de substituição de símbolo de dígito/cópia de símbolo (DSST); escala de gravidade da fadiga (FSS);Beck depressive inventory-I (BDI-I) e um short-form of health survey (SF-36). Resultados A média de idade dos pacientes foi de 50 anos (71,4% do sexo masculino). O MMSE e o MoCA não apresentaram diferença estatística entre os grupos. Os pacientes apresentaram resultados superiores no domínio memória do MoCA (5 vs. 2, p = 0,013). No DSST, observamos uma tendência de os pacientes serem mais lentos. Houve forte correlação negativa entre os níveis de fadiga e o domínio vitalidade (SF-36). Não houve correlação significativa entre níveis de depressão e QV. Não houve correlação entre depressão e os resultados obtidos nos testes cognitivos. Níveis elevados de depressão foram observados nos pacientes (15,28 vs. 3,42, p < 0,001). Um total de 57% apresentou fadiga intensa, 28,8% dor autorreferida, e 57,1% queixam-se de câimbras. Conclusão Não há comprometimento cognitivo nos pacientes estudados. Observamos somente uma tendência de lentificação na velocidade de processamento. Para melhor avaliar as alterações encontradas, será necessário estudo com um número maior de indivíduos. A PDIC afeta de diferentes formas o nível de QV de seus portadores.

Controversias en neuroinmunología: esclerosis múltiple, vacunación, SARS-CoV-2 y otros dilemas / Controversies in neuroimmunology: multiple sclerosis, vaccination, SARS-CoV-2 and other dilemas

La neuroinmunología es una disciplina que cada vez amplía más sus horizontes en la comprensión de las enfermedades neurológicas. Contemporáneamente, y a la luz de los nexos fisiopatológicos de las enfermedades neurológicas y la inmunología, se han planteado enfoques diagnósticos y terapéuticos específicos. A pesar de los importantes avances de esta disciplina, existen múltiples dilemas que le conciernen y se filtran en la práctica clínica. En esta revisión, se presentan y discuten 15 controversias, las cuales se construyen con la información clínica disponible más actualizada. Los temas incluidos son: disminución de esteroides en recaídas de esclerosis múltiple; recomendaciones terapéuticas en esclerosis múltiple a la luz de la pandemia por el SARS-CoV-2; evidencia de vacunación en esclerosis múltiple y en otras enfermedades desmielinizantes; panorama actual del síndrome clínico y radiológico aislado; y fallas terapéuticas en esclerosis múltiple; además, criterios para suspender las terapias modificadoras de la enfermedad; evidencia del manejo en recaídas leves; recomendaciones para la profilaxis contra Strongyloides stercolaris; utilidad de un segundo ciclo de inmunoglobulina en el síndrome de Guillain-Barré; criterios para diferenciar una polineuropatía crónica desmielinizante inflamatoria de inicio agudo de un síndrome de Guillain-Barré y, utilidad de la enzima convertidora de angiotensina en neurosarcoidosis. En cada una de las controversias, se presenta la problemática general y se ofrecen recomendaciones específicas que pueden adoptarse en la práctica clínica diaria.

Neuroimmunology is a discipline that increasingly broadens its horizons in the understanding of neurological diseases. At the same time, and in front of the pathophysiological links of neurological diseases and immunology, specific diagnostic and therapeutic approaches have been proposed. Despite the important advances in this discipline, there are multiple dilemmas that concern and filter into clinical practice. This article presents 15 controversies and a discussion about them, which are built with the most up-to-date evidence available. The topics included in this review are: steroid decline in relapses of multiple sclerosis; therapeutic recommendations in MS in light of the SARS-CoV-2 pandemic; evidence of vaccination in multiple sclerosis and other demyelinating diseases; overview current situation of isolated clinical and radiological syndrome; therapeutic failure in multiple sclerosis, as well as criteria for suspension of disease-modifying therapies; evidence of the management of mild relapses in multiple sclerosis; recommendations for prophylaxis against Strongyloides stercolaris; usefulness of a second course of immunoglobulin in the Guillain-Barré syndrome; criteria to differentiate an acute-onset inflammatory demyelinating chronic polyneuropathy versus Guillain-Barré syndrome; and, the utility of angiotensin-converting enzyme in neurosarcoidosis. In each of the controversies, the general problem is presented, and specific recommendations are offered that can be adopted in daily clinical practice.

Processing speed impairment in chronic inflammatory demyelinating polyneuropathy patients: a cross-sectional study / Prejuízo na velocidade de processamento em pacientes com polineuropatia inflamatória desmielinizante crônica: um estudo transversal

Abstract Background There is a lack of evidence of cognitive involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) and, the reports about the involvement of the brain and central nervous system (CNS) are few and controversial. The Five Digit Test (FDT) evaluates processing speed (PS) and executive functions orally. Objective To evaluate the performance on the FDT of CIDP patients with and without CNS (brain/cerebellum) alterations observed on brain Magnetic Resonance Imaging (MRI) scans. Methods The Hospital Anxiety and Depression Scale (HADS, to assess neuropsychiatry symptoms), the Rasch-built Overall Disability Scale (R-ODS; to assess disability), and the FDT (to assess cognition) were applied to 14 CIDP patients and 24 age-matched healthy control subjects. The patients were submitted to routine brain MRI and, according to the results, they were divided into two groups: those with abnormalities on the MRI (CIDPabnl) and those with normal parameters on the MRI (CIDPnl). The FDT data of five CIDPnl patients and nine CIDPabnl subjects were analyzed. Comparisons between the groups were performed for each task of the FDT. Results We found statistical differences for both groups of CIDP patients in terms of PS, for the patients spent more time performing the PS tasks than the controls. The PS measures were negatively associated with disability scores (reading: r = −0.47; p = 0.003; counting: r = −0.53; p = 0.001). Conclusions Our data suggested the presence of PS impairment in CIDP patients. Disability was associated with slow PS.

Resumo Antecedentes Faltam evidências de envolvimento cognitivo na polineuropatia inflamatória desmielinizante crônica (PIDC), e há poucos e controversos estudos que tratam do envolvimento cerebral e do sistema nervoso central (SNC). O Teste dos Cinco Dígitos (Five Digit Test, FDT, em inglês) avalia a velocidade de processamento (VP) e as funções executivas oralmente. Objetivo Avaliar o desempenho no FDT de pacientes com PIDC com e sem alterações no SNC (cérebro/cerebelo) de acordo com o exame de imagem cerebral por ressonância magnética (RM). Métodos Ao todo, 14 pacientes e 24 controles saudáveis pareados por idade responderam a Escala Hospitalar de Ansiedade e Depressão (que avalia sintomas neuropsiquiátricos), a Escala de Incapacidade Geral elaborada pelo método Rasch (que avalia a incapacidade) e o FDT (que avalia a cognição). Os pacientes foram submetidos a RM cerebral e, de acordo com os resultados, divididos em dois grupos: aqueles com anormalidades (PIDCabnl) e aqueles sem alterações (PIDCnl) na RM. Cinco pacientes PIDCnl e nove PIDCabnl tiveram os dados analisados. Comparações entre os grupos foram realizadas para cada parte do FDT. Resultados Os dois grupos de pacientes foram estatisticamente mais lentos nas tarefas de VP comparados ao grupo controle. As medidas de VP foram negativamente associadas às pontuações de incapacidade (leitura: r = −0,47; p = 0,003; contagem: r = −0,53; p = 0,001). Conclusões Os dados indicaram a presença de prejuízo na VP em pacientes com PIDC. A incapacidade foi associada à lentidão na VP.

Antibody detection and clinical characteristics of chronic inflammatory demyelinating polyradiculopathy with anti-nodal/paranodal antibodies / 中华检验医学杂志

Objective:To investigate the detecting method and clinical characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculopathy.Methods:Serum samples were collected from 212 patients with chronic inflammatory demyelinating polyradiculopathy who were admitted to Huashan Hospital of Fudan University or from other clinical centers from January 2018 to July 2021. Autoantibodies (anti-NF155, anti-NF186, anti-CNTN1) and IgG subtypes were detected with cell-based assay. According to the test results, patients were divided into anti-NF155 positive group, anti-NF186 positive group and anti-CNTN1 positive group, clinical characteristics of patients in each group, including limb weakness, superficial sensation and proprioception, tremor, cerebrospinal fluid protein level, brachial plexus magnetic resonance (MRI) were retrospectively analyzed and compared.Results:A total of 23 patients (10.8%,23/212) were positive for anti-NF155 antibody, 12 (5.7%,12/212) for anti-NF186 antibody, and 4 (1.9%,4/212) for anti-CNTN1 antibody. IgG 4 was the predominant subtype in anti-NF155 and anti-CNTN1 groups. In the anti-NF186 group, all cases were IgG positive and antibody subtypes could be detected in 4 cases (4/12). In anti-NF155 group, 23 patients (100%,23/23) had limb weakness and deep sensory disturbance, 19 patients (82.6%,19/23) had superficial sensory disturbance, 22 patients (95.7%,22/23) were symmetrically involved, 18 patients (78.3%,18/23) showed tremor, 19 patients (19/19) showed abnormal in brachial plexus MRI. In anti-NF186 group, 12 patients had limb weakness (12/12), 9 patients (9/12) and 6 patients (6/12) had superficial sensory disturbance and deep sensory disturbance respectively, 8 patients (8/12) were asymmetrically involved, and only 1 patient (1/12) showed tremor, 1 (1/7) showed abnormal brachial plexus MRI. In anti-CNTN1 group, 4 cases showed symmetrical limb weakness and sensory disturbance, 3 patients had tremor, and four patients showed brachial plexus MRI abnormality. There were statistically significant differences in onset age, proprioception, tremor and MRI abnormalities of brachial plexus among the 3 groups ( P<0.01). Conclusions:The clinical characteristics of CIDP patients with anti-NF155, anti-NF186 and anti-CNTN1 antibodies are different. Screening anti-nodal/paranodal antibodies is of great significance for accurate diagnosis and treatment of patients with peripheral neuropathy.

The changes in European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

European Academy of Neurology/Peripheral Nerve Society revised the guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Typical CIDP and CIDP variants were distinguished. The levels of diagnostic certainty of CIDP and possible CIDP can be defined according to the changes in motor and sensory nerve conduction studies. The utility of cerebrospinal fluid, peripheral nerve image, and nerve biopsy in diagnosis of CIDP was suggested. Serum auto-antibodies, including anti-nodal and paranodal antibodies, anti-myelin-associated glycoprotein antibodies were discussed. Monoclonal protein should be tested routinely in CIDP. Principles for treatment of CIDP were recommended. This guideline updated the development in CIDP and is more suitable for clinical practice.

Diagnostic challenges in systemic amyloidosis: a case report with clinical and laboratorial pitfalls

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.

Paranodal disease with anti-contactin-associated protein 1 antibodies: a case report and literature review / 中华神经科杂志

Objective:To summarize the clinical, pathological and molecular biological characteristics of one patient of paranodal disease with anti-contactin-associated protein 1 (Caspr 1) antibodies.Methods:The patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) admitted to Qilu Hospital of Shandong University from August 2018 to December 2020 were retrospectively studied. The clinical data of one acute onset CIDP patient with anti-Caspr 1 antibodies were collected and retrospectively analyzed with literature review. Anti-nodal/paranodal IgG and their subclasses in serum and cerebrospinal fluid (CSF) were investigated by immuno?uorescence assays. Pathological characteristics were explored by sural nerve biopsy further.Results:The patient presented with tremor, ataxia and neuropathological pain besides symmetrical limb muscle weakness and hypaesthesia. The CSF protein was elevated significantly. The brachial plexus and lumbosacral plexus magnetic resonance imaging showed enlarged nerve roots. The patient was responsive well to intravenous immunoglobulin and steroids in acute phase, while the symptoms improved significantly with rituximab in chronic phase. Autoantibodies against Caspr 1 were detectable in serum and CSF, with IgG4 predominant. Sural nerve biopsy revealed segmental demyelination and myelin digestion chamber. Dispersed lamellae of myelin sheath and axonal degeneration were confirmed by electron microscopy.Conclusions:Tremor, ataxia, neuropathic pain, significantly elevated CSF protein and enlarged nerve roots are suggestive of paranodal diseases with anti-Caspr 1 antibodies. For patients with suspected Guillain-Barre syndrome/CIDP and above phenotypes, nodal/paranodal antibodies and antibody subtypes should be detected to optimize the treatment.

Factores asociados a respuesta favorable de tratamiento corticoidal en pacientes con polirradiculoneuropatía desmielinizante inflamatoria crónica / Variables associated with a good response to steroids in chronic inflammatory demyelinating polyradiculoneuropathy

Background: The treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on corticosteroids, immunoglobulin and plasmapheresis. In our Health System, corticosteroids are commonly used as first line therapy for economic reasons and accessibility. However, the factors associated with a good response are not well known. Aim: To assess the association of demographic, clinical and laboratory variables with a favorable response to corticosteroid therapy in patients with CIDP. Material and Methods: Observational, descriptive, longitudinal and retrospective study of 33 patients with a diagnosis of typical, definitive or probable CIDP, treated with corticosteroids for at least six months. Results: Twenty-three patients had a good clinical response to corticosteroid treatment and 10 were non-responders. The variables significantly associated with a good response to steroids were a disease lasting less than 1 year prior to the start of treatment, the absence of axonal damage in electromyography a relapsing-recurrent course and a favorable response within two months of treatment. Conclusions: Most of these patients with CIDP had good response to corticosteroid therapy.

Association between electromyography and magnetic resonance neurography in patients with typical chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

Objective@#To explore the association among clinical features, electromyography (EMG) and magnetic resonance neurography (MRN) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). @*Methods@#A cross-sectional survey was conducted to enroll consecutively typical CIDP patients in Renmin Hospital of Wuhan University from May 2017 to May 2019. The Hughes Disability Scale (HDS) was used to evaluate the illness severity of the patients. The electrodiagnostic parameters including motor conduction velocity (MCV), compound muscle action potential (CMAP), F-wave latency, sensory nerve conduction velocity (SCV) and sensory nerve action potential (SNAP) of upper and lower limbs were analyzed. The patients whose response waveform can be elicited in all nerves were defined as group A, and those without response in one or more nerves as group B. MRN quantitative technique was used to calculate the cross-sectional area of nerves roots (nr-CSA) of brachial plexus and lumbosacral plexus. The linear regression method was used to analyze the correlation among clinical features, EMG and nr-CSA. @*Results@#A total of 32 patients with typical CIDP met the criteria, 75% (24/32) of whom were males. There were 16 patients in the mild group (group A) and 16 in the severe one (group B). The abnormal rate of F-wave latency was the highest. Cerebrospinal fluid (CSF) protein, HDS score were correlated significantly with the nr-CSA of brachial plexus and lumbosacral plexus in the two groups (group A: CSF protein and brachial plexus nr-CSA: r=0.498, P=0.004; CSF protein and lumbosacral plexus nr-CSA: r=0.479, P=0.007; HDS score and brachial plexus nr-CSA: r=0.650, P=0.000; HDS score and lumbosacral plexus nr-CSA: r=0.625, P=0.000. group B: CSF protein and brachial plexus nr-CSA: r=0.497, P=0.049; CSF protein and lumbosacral plexus nr-CSA: r=0.503, P=0.047; HDS score and brachial plexus nr-CSA: r=0.605, P=0.001; HDS score and lumbosacral plexus nr-CSA: r=0.648, P=0.000). MCV of median nerve and ulnar nerve was negatively correlated with nr-CSA of brachial plexus in the two groups (group A: MCV of median nerve and nr-CSA of brachial plexus: r=-0.309, P=0.019; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.286, P=0.027. group B: MCV of median nerve and nr-CSA of brachial plexus: r=-0.660, P=0.000; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.581, P=0.001). The F-wave latencies of median and ulnar nerves were positively correlated with nr-CSA of brachial plexus, and the CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were positively correlated with nr-CSA of lumbosacral plexus in group B. @*Conclusions@#Male patients with CIDP are predominant. The higher the nr-CSA in brachial plexus and lumbosacral plexus, the higher the CSF protein and disability score, and the larger the nr-CSA in brachial plexus, the slower the MCV in the median and ulnar nerve. For group B patients with more severe nerve injury, the larger nr-CSA of brachial plexus was, the longer F-wave latency of median and ulnar nerve was, and the larger nr-CSA of lumbosacral plexus was, the lower CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were. As a non-invasive test, MRN can be used to assist in the diagnosis of CIDP and to assess the severity of the disease.

Chinese guidelines for diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy 2019 / 中华神经科杂志

There are progresses in diagnose, novel anti-bodies and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in recent years. The treatment of paranodal anti-bodies mediated CIDP is specific. Based on the guideline of CIDP in China in 2012, this guideline revised the definitions of typical, untypical and IgG4 antibodies mediated CIDP. Moreover, the clinical and electrodiagnostic criteria as well as treatment of CIDP are published. This guideline aims to provide the diagnosis criteria and treatment of CIDP to clinical neurologists.

Interpretation of Electrodiagnostic Tests in Chronic Inflammatory Demyelinating Polyneuropathy: Classification Using Nerve Conduction Study / 대한신경근육질환학회지

Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.

Clinicopathological analysis of chronic inflammatory demyelinating polyneuropathy in the elderly / 中华老年医学杂志

Objective To study the clinical and pathological features in the elderly patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Methods The features of the clinical manifestation, cerebrospinal fluid, electromyogram(EMG) and the biopsy results of sural nerve were presented and analyzed in 11 elderly patients with CIDP. Results Two cases had history of upper respiratory tract infection before the onset. As the initial symptoms , there were three cases with distal limb numbness, five cases with both distal lower extremities numbness, two cases with both distal upper extremities numbness and one case with difficulties to raise his head. Motor disorder was common to all the patients. There were eight patients with sensory dysfunction, three with limb muscle atrophy, one with muscle tenderness, eight with tendon reflexes weakened or disappeared, five with cranial nerve damaged, three with the autonomic nerve lesion, one with respiratory muscle involved, three with relapse. The score of the peak incidence as Modified Rankin was 3.02 points on average. Five cases had obvious albuminocytolgoic dissociation by the examination of cerebrospinal fluid, ten cases had neurogenic damage and one case had a combined myogenic and neurogenic damage by the EMG. The biopsy showed that six cases were with amyelination,six cases with inflammatory cell infiltrated, two cases with obvious remyelination, two cases with auxiliary fibers degeneration.And the methylprednisolone therapy was effective for eight cases. Conclusions The numbness of the distal limb is the initial symptom of the elderly patients with CIDP,most of whom are with sensory dysfunction ,and some with cranial nerves and autonomic nerve damage. The sural nerve biopsy has an important value for the diagnosis of the elderly with CIDP. The methylprednisolone therapy is proved to be effective for most patients.

A Charcot-Marie-Tooth disease resembling to chronic inflammatory demyelinating polyradiculoneuropathy:a report of two cases / 中华神经科杂志

Objective To observe the clinical, neuropathogical, neurophysiological characteristics of Charcot-Marie-Tooth disease, type 1A (CMT1A) which was similar to chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).Methods The clinical data, neuroelectrophysiological changes and pathological features of sural nerve biopsy were taken from 2 CMT1A patients who were proven to be 17p12 duplication with CIDP features, and analyzed comprehensively. Results Two CMT1A cases with classical chronic course showed both subacute course and clinical manifestations similar to CIDP, however, the changes of neuroelectrophysiology and pathological characteristics of the nerve biopsy in 2 cases were different from CIDP in some way. We confirmed the chronic inflammatory demyelinating type of the CMT1A patients whom the immunotherapy was effective on.Conclusion The classical CMT1A 17p12 duplication patients who obtained immunotherapy effects might resemble CIDP both in clinical course and manifestation.

Analyzing the clinical characteristic of CIDP and spine degeneration diseases / 中华骨科杂志

Objective To prevent wrong diagnosis and treatment of chronic inflammatory demyelinating polyneuritis(CIDP) by analyzing the clinical characteristics of CIDP and spine degeneration disorders. Methods In order to distinguish CIDP from spine degeneration diseases, we retrospectively reviewed 16 CIDP patients from December 2000 to December 2003 (male 10, female 6; range 38-58 years old, mean 49.1 years old) who were misdiagnosed as spine degeneration diseases. The duration of the disease was 2 months to 2 years and 8 months, with an everage of 106 months. All clinical data of the 16 patients were analyzed. The data included clinical manifestation, physical examination, protein content of cerebrospinal fluid (CSF) and upper-lower extremity electrophysiologic study(EPS). Comparing the clinical charateristics of CIDP with those of spine degeneration disease. Results All 16 patients of CIDP sufferred limbs sensory disturbance or abnormality, walking difficulty. Upper or lower extremity were involved at equal pace and symmetry; limb tendon reflexes weakened or dissappeared, but limbs muscular atrophy were not significant. X-ray showed degeneration of cervical or thoracic and lumbar spine; MRI showed that there were disc herniation in different intervertebral. The content of protein of CSF remarkably increased(mean 479.9 mg/L). There were significant deviation compare to normal. Electrophysiology study found that sensory nerve action potential(SNAP) wave amplitude were descending; motor nerve conduction velocity(MNCV) were slower. Conclusion We could rightly diagnose and distingnish CIDP from spine degeneration diseases by analyzing clinical manifestation, physical examination, cerebrospinal fluid and electrophysiological study. [