RESUMO
We herein report a rare case of distal chronic inflammatory demyelinating polyneuropathy (CIDP) following coronavirus disease 2019 (COVID-19) vaccination. A 39-year-old woman with a solitary plasmacytoma developed general weakness 7 days after receiving the second dose of the Pfizer-BioNTech COVID-19 vaccine, which had progressed for 3 months. A neurological examination revealed limb weakness with areflexia. Serological tests identified the presence of IgG antibodies against anti-GM1 and anti-GM2 gangliosides. Comprehensive evaluations met the criteria of distal CIDP. Intravenous immunoglobulin, intravenous methylprednisolone, oral prednisolone, and plasma exchange were administered, and she gradually improved. Physicians should be aware of CIDP as a rare complication of COVID-19 vaccination.
Assuntos
Vacina BNT162 , Neoplasias Ósseas , COVID-19 , Plasmocitoma , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Adulto , Feminino , Humanos , Vacina BNT162/efeitos adversos , COVID-19/prevenção & controle , Plasmocitoma/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologiaAssuntos
Transplante de Células-Tronco Hematopoéticas , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Polirradiculoneuropatia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Imunoglobulinas Intravenosas , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Esteroides/uso terapêuticoAssuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Síndrome da Veia Cava Superior/etiologia , Timoma/complicações , Neoplasias do Timo/complicações , Adulto , Anticoagulantes/uso terapêutico , Eletrodiagnóstico , Eletromiografia , Gastrite/induzido quimicamente , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Condução Nervosa , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Síndrome da Veia Cava Superior/diagnóstico , Síndrome da Veia Cava Superior/tratamento farmacológico , Timoma/diagnóstico por imagem , Neoplasias do Timo/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Tuberculose Miliar/complicações , Tuberculose Miliar/diagnóstico por imagem , Tuberculose Pulmonar/complicações , Tuberculose Pulmonar/diagnóstico por imagemRESUMO
Monoclonal gammopathy is encountered quite frequently in the general population. This type of hematologic abnormality may be mild, referred to as monoclonal gammopathy of undetermined significance or related to different types of hematologic malignancies. The association of a peripheral neuropathy with monoclonal gammopathy is also fairly common, and hemopathy may be discovered in an investigation of peripheral neuropathy. In such a situation, it is essential to determine the exact nature of the hematologic process in order not to miss a malignant disease and thus initiate the appropriate treatment (in conjunction with hematologists and oncologists). In this respect, nerve biopsy (discussed on a case-by-case basis) is of great value in the management of such patients. We therefore propose to present the objectives and main interests of nerve biopsy in this situation.
Assuntos
Paraproteinemias/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/etiologia , Anemia Hemolítica Autoimune/patologia , Anemia Hemolítica Autoimune/fisiopatologia , Ataxia/diagnóstico , Ataxia/etiologia , Ataxia/patologia , Ataxia/fisiopatologia , Autoanticorpos/imunologia , Biópsia , Árvores de Decisões , Eletrodiagnóstico , Humanos , Imunoglobulina A , Imunoglobulina G , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Imunoglobulina M , Gamopatia Monoclonal de Significância Indeterminada , Glicoproteína Associada a Mielina/imunologia , Condução Nervosa/fisiologia , Oftalmoplegia/diagnóstico , Oftalmoplegia/etiologia , Oftalmoplegia/patologia , Oftalmoplegia/fisiopatologia , Síndrome POEMS/diagnóstico , Síndrome POEMS/etiologia , Síndrome POEMS/patologia , Síndrome POEMS/fisiopatologia , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Nervos Periféricos/patologia , Nervos Periféricos/ultraestrutura , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Disautonomias Primárias/diagnóstico , Disautonomias Primárias/etiologia , Disautonomias Primárias/patologia , Disautonomias Primárias/fisiopatologia , Neuropatia de Pequenas Fibras/diagnóstico , Neuropatia de Pequenas Fibras/etiologia , Neuropatia de Pequenas Fibras/patologia , Neuropatia de Pequenas Fibras/fisiopatologia , Macroglobulinemia de WaldenstromRESUMO
We here report a single case of chronic paraneoplastic inflammatory demyelinating polyradiculoneuritis secondary to a nasal natural killer (NK) non-Hodgkin T lymphoma.
Assuntos
Linfoma Extranodal de Células T-NK/complicações , Neoplasias Nasais/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Humanos , Linfoma Extranodal de Células T-NK/diagnóstico , Linfoma Extranodal de Células T-NK/patologia , Masculino , Pessoa de Meia-Idade , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnósticoRESUMO
We report a 77-year-old man who presented with numbness and weakness of the feet bilaterally, that had progressed over 13 years. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) on the basis of nerve conduction studies and a sural nerve biopsy; however, he was inadequately treated and his weakness had progressed. At 76 years of age, he developed spasticity in the legs as well as bladder and rectal incontinences. Gd-enhanced MRI revealed severe compression of the cervical cord by massively enlarged nerve roots. A cervical laminectomy was performed to decompress the cervical cord. A fascicular biopsy of the C5 dorsal root showed a prominent lymphocyte infiltration and edema. Repeated methylprednisolone pulse therapy and IVIg ameliorated the weakness. We concluded that the main cause of nerve root hypertrophy in this patient was active inflammation.
Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Compressão da Medula Espinal/etiologia , Doenças da Medula Espinal/etiologia , Doenças da Medula Espinal/terapia , Raízes Nervosas Espinhais/patologia , Idoso , Vértebras Cervicais , Edema , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Laminectomia , Linfócitos/patologia , Masculino , Metilprednisolona/administração & dosagem , Pulsoterapia , Compressão da Medula Espinal/terapia , Resultado do TratamentoRESUMO
Many patients with plasma cell disorders suffer from peripheral neuropathy, but differential diagnosis with chronic inflammatory demyelinating polyneuropathy (CIDP) is difficult. We aimed to (1) identify factors useful for differential diagnosis between peripheral neuropathy associated with plasma cell disorders versus CIDP and (2) determine whether neuropathy presentations and severity varied across the spectrum of different plasma cell disorders. A retrospective chart review of 18 monoclonal gammopathy of unknown significance (MGUS) patients, 15 POEMS syndrome patients and 34 CIDP patients between January 2005 and December 2016 was conducted. The peripheral neuropathy associated with plasma cell disorders seemed to be more sensory oriented compared to CIDP. MGUS patients were significantly older than CIDP patients (median age 70 vs. 59, respectively, p = 0.027). POEMS syndrome patients showed significantly higher platelet count at the time of neuropathy presentation compared to CIDP (p = 0.028). Lambda type MGUS patients were associated with less severe symptoms compared to POEMS syndrome patients despite harboring lambda monoclonal gammopathy as a common denominator. Kappa type MGUS patients showed predominantly axonal type neuropathy compared to its counterpart and POEMS syndrome. Careful inspection of clinical profiles and symptoms of patients presenting with neuropathy can help to discriminate those with underlying plasma cell disorders. The phenotype of neuropathy, platelet count and age at presentation seem to be the most useful indicators.
Assuntos
Gamopatia Monoclonal de Significância Indeterminada/complicações , Síndrome POEMS/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome POEMS/etiologia , Síndrome POEMS/patologia , Contagem de Plaquetas , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
Immune-mediated neuropathies (IMNs) following hematopoietic stem cell transplantation have been described recently, which, excluding Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, may present with atypical patterns. This retrospective, nested, case-control study reviewed data from 3858 patients who received haploidentical hematopoietic stem cell transplantation (haplo-HSCT) during the past 10 years at a single center, and 40 patients (1.04%) with IMN following haplo-HSCT were identified. Chronic graft-versus-host disease (cGVHD) (Pâ¯=â¯.043) and cytomegalovirus (CMV) viremia (Pâ¯=â¯.035) were recognized as independent risk factors for the development of IMN after haplo-HSCT. There were no significant differences in overall survival (Pâ¯=â¯.619), disease-free survival (Pâ¯=â¯.609), nonrelapse mortality (Pâ¯=â¯.87), or the incidence of relapse (Pâ¯=â¯.583) between patients with and without IMN after haplo-HSCT. However, patients with post-transplant IMN were at higher risk of developing cGVHD (Pâ¯=â¯.012) than patients who did not develop IMN. Twenty-four of the 40 patients with IMN (60%) attained neurologic improvement after treatments including vitamins B1 and B12 and/or immunomodulatory agents. However, 19 (47.5%) patients still had persistent motor/sensory deficits despite receiving timely treatment. More studies are needed to help develop standardized diagnostic and therapeutic strategies for patients with post-transplant IMN.
Assuntos
Doença Enxerto-Hospedeiro , Síndrome de Guillain-Barré , Transplante de Células-Tronco Hematopoéticas , Fatores Imunológicos/administração & dosagem , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Tiamina/administração & dosagem , Vitamina B 12/administração & dosagem , Adolescente , Adulto , Aloenxertos , Doença Crônica , Intervalo Livre de Doença , Feminino , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/mortalidade , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/mortalidade , Humanos , Incidência , Masculino , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/mortalidade , Fatores de Risco , Taxa de SobrevidaAssuntos
Antineoplásicos Imunológicos/efeitos adversos , Doença de Hodgkin/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Adulto , Antineoplásicos Imunológicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/patologia , HumanosAssuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia-Linfoma de Células T do Adulto/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Condicionamento Pré-Transplante/métodos , Transplante Homólogo/métodos , Adulto , Idoso , Doença Crônica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologiaRESUMO
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is probably the best recognized progressive immune-mediated peripheral neuropathy. It is characterized by a symmetrical, motor-predominant peripheral neuropathy that produces both distal and proximal weakness. Large-fiber abnormalities (weakness and ataxia) predominate, whereas small-fiber abnormalities (autonomic and pain) are less common. The pathophysiology of CIDP is inflammatory demyelination that manifests as slowed conduction velocities, temporal dispersion, and conduction block on nerve conduction studies and as segmental demyelination, onion-bulb formation, and endoneurial inflammatory infiltrates on nerve biopsies. Although spinal fluid protein levels are generally elevated, this finding is not specific for the diagnosis of ClDP. Other neuropathies can resemble CIDP, and it is important to identify these to ensure correct treatment of these various conditions. Consequently, metastatic bone surveys (for osteosclerotic myeloma), serum electrophoresis with immunofixation (for monoclonal gammopathies), and human immunodeficiency virus testing should be considered for testing in patients with suspected CIDP. Chronic inflammatory demyelinating polyradiculoneuropathy can present as various subtypes, the most common being the classical symmetrical polyradiculoneuropathy and the next most common being a localized asymmetrical form, multifocal CIDP. There are 3 well-established, first-line treatments of CIDP-corticosteroids, plasma exchange, and intravenous immunoglobulin-with most experts using intravenous immunoglobulin as first-line therapy. Newer immune-modulating drugs can be used in refractory cases. Treatment response in CIDP should be judged by objective measures (improvement in the neurological or electrophysiological examination), and treatment needs to be individualized to each patient.
Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Humanos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologiaAssuntos
Transplante de Medula Óssea/efeitos adversos , Polineuropatias/etiologia , Anti-Inflamatórios/uso terapêutico , Ciclosporina/uso terapêutico , Doenças Desmielinizantes/etiologia , Doenças Desmielinizantes/terapia , Feminino , Doença Enxerto-Hospedeiro , Humanos , Imunização Passiva , Imunossupressores/uso terapêutico , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/terapia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/complicações , Mieloma Múltiplo/terapia , Polineuropatias/tratamento farmacológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Adulto JovemAssuntos
Febre de Causa Desconhecida/etiologia , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico , Paraproteinemias/diagnóstico , Paraproteinemias/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Neoplasias Esplênicas/complicações , Neoplasias Esplênicas/diagnóstico , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Febre de Causa Desconhecida/patologia , Humanos , Linfoma Difuso de Grandes Células B/patologia , Paraproteinemias/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Baço/patologia , Neoplasias Esplênicas/patologiaRESUMO
BACKGROUND: Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. CASE PRESENTATION: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP. The patient initially had some resolution of the lipodystrophy phenotype, but subsequently the abnormal fat distribution recurred and the patient developed additional systemic abnormalities, including mild pancytopenia and hepatic fibrosis. CONCLUSIONS: Our patient represents a novel association of acquired lipodystrophy, craniopharyngioma, and CIDP, possibly due to an as yet unidentified paraneoplastic autoantibody.
Assuntos
Craniofaringioma/complicações , Lipodistrofia/etiologia , Síndromes Paraneoplásicas/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Adolescente , Humanos , Lipodistrofia/patologia , Masculino , Síndromes Paraneoplásicas/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , PrognósticoRESUMO
BACKGROUND: Zoledronic acid is a nitrogen-containing bisphosphonate that is frequently used in the treatment of osteoporosis. Many patients experience a so-called acute-phase reaction during initial treatment; this is characterized by flu-like symptoms and fever. CASE DESCRIPTION: We describe a 61-year-old woman who suffered from chronic inflammatory demyelinating polyneuropathy (CIDP), and who was started on intravenous zoledronic acid treatment as adjuvant therapy for breast cancer. Within 24 hours of the initial treatment she developed cold shivers, extreme fatigue, muscle pain and headache. These symptoms resolved spontaneously in the course of the following days. At the same time, she also experienced a CIDP relapse, with a tingling sensation in her arms and legs and deterioration in her ability to walk. Considering the course of the symptoms and the absence of any other cause, CIDP relapse was very probably the result of the acute-phase reaction induced by zoledronic acid. CONCLUSION: Caution is warranted when using nitrogen-containing bisphosphonates such as zoledronic acid, particularly in patients with known neuromuscular disorders.
Assuntos
Difosfonatos/efeitos adversos , Imidazóis/efeitos adversos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Conservadores da Densidade Óssea/efeitos adversos , Neoplasias da Mama/tratamento farmacológico , Feminino , Humanos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Ácido ZoledrônicoAssuntos
Linfoma Difuso de Grandes Células B/diagnóstico , Debilidade Muscular/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Animais , Dor nas Costas/etiologia , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/patologia , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Ovário/diagnóstico por imagem , Ovário/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Tomografia por Emissão de Pósitrons , Raízes Nervosas Espinhais/patologia , Coluna Vertebral/diagnóstico por imagem , Ultrassonografia , Útero/diagnóstico por imagem , Útero/patologia , Redução de PesoRESUMO
Nivolumab, an anti-programmed death-1-specific monoclonal antibody, has demonstrated a durable response and effect on overall survival and has become one of the standard treatments for patients with advanced melanoma. Reported herein is a case of nivolumab-induced chronic inflammatory demyelinating polyradiculoneuropathy, in which an 85-year-old woman with stage IV melanoma developed grade 1 paresthesia 2 weeks after the initial dose of nivolumab was administered. With continued treatment, the neurological deficiency deteriorated rapidly, mimicking Guillain-Barré syndrome, causing such a dramatic decrease in her activities of daily living that she could no longer function in daily life. Thus, nivolumab treatment was discontinued. A course of intravenous immunoglobulin infusion yielded a dramatic clinical improvement; in particular, improved motor control was observed within a few days. Her initial presentation was suggestive of acute inflammatory demyelinating polyradiculoneuropathy, a subtype of Guillain-Barré syndrome; however, the good response to steroids and exacerbation 8 weeks after the onset were suggestive of chronic inflammatory demyelinating polyradiculoneuropathy induced by nivolumab. This is the first case of Guillain-Barré syndrome-like autoimmune polyradiculoneuropathy induced by programmed death-1/programmed death-ligand 1 inhibitors. Although neurological adverse events related to nivolumab are rare, they can become severe, requiring early diagnosis and intervention. Intravenous immunoglobulin may be considered as an effective initial treatment for patients who develop acute autoimmune nervous system disorders due to nivolumab.
Assuntos
Anticorpos Monoclonais/efeitos adversos , Antineoplásicos/efeitos adversos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Atividades Cotidianas , Idoso de 80 Anos ou mais , Anticorpos Monoclonais/uso terapêutico , Antineoplásicos/uso terapêutico , Medula Cervical/diagnóstico por imagem , Feminino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Infusões Intravenosas , Imageamento por Ressonância Magnética , Melanoma/tratamento farmacológico , Nivolumabe , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
Chronic inflammatory demyelinating polyradiculoneuropathy occurred in an 11-year-old Labrador Retriever dog. Spinal cord compression resulted from massive radiculitis with prominent cholesterol granulomas. Cholesterol deposition and associated granuloma formation is unique in chronic inflammatory demyelinating polyradiculoneuropathy, in both its human and canine expressions.
Assuntos
Colesterol/metabolismo , Doenças do Cão/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/veterinária , Raízes Nervosas Espinhais/patologia , Animais , Doenças do Cão/etiologia , Cães , Feminino , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologiaRESUMO
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare complication of Crohn's disease (CD), and it is uncertain whether it is associated with CD itself or with its treatment. We describe a case of CIDP-like neuropathy as an initial symptom of CD. The neurologic symptoms of the patient which responded partially to intravenous immunoglobulin (IVIG) recovered after resection of the appendiceal CD. CASE PRESENTATION: A 17-year-old male had experienced three separate attacks of motor weakness and paresthesia of all four extremities over a period of 7 months. The electrophysiologic findings revealed a demyelinating sensory-motor polyneuropathy which was compatible with CIDP. However, repeated intravenous IVIG (2 g/kg) treatment gave only a partial response. Four days after the last discharge, he was diagnosed as appendiceal CD after surgical resection of a periappendiceal abscess. His neurologic symptoms and electrophysiologic findings recovered without any maintenance therapy. CONCLUSIONS: CIDP-like neuropathy can be an initial presentation of CD, and recovery of the CIDP symptoms may result from resection of the CD. Clinicians should be aware of the possibility of CD in patients with intractable CIDP symptoms.