• Korean Journal of Clinical Laboratory Science
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• v.53 no.2
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• pp.151-157
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• 2021

Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary degenerative disease and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits. The purpose of this study was to analyze and compare the electrophysiological characteristics observed in sensory nerve conduction studies (SNCS) of both diseases. A retrospective study of 65 patients with a diagnosis of CIDP (N=35) and CMT type I (N=30) was performed. This study analyzed No potentials ratio, distal compound nerve action potential (dCNAP) of various nerve types, and a correlation coefficient analysis of the sensory nerve conduction velocity (SNCV). As a result, I found that CMT 1 was more severe systemic demyelinating and axonal polyneuropathy better than CIDP (P<0.05). In a quantitative analysis of dCNAP and SNCV, especially sural nerve was the most severe nerve injury observed in both diseases. In correlation and scatter plot analysis, CMT 1 showed relatively high correlations compared to CIDP based on the correlation coefficient analysis (Fisher's Z test) of SNCV. The results of this study suggested that CMT 1 showed the slowness in SNCV, one of the characteristics of demyelinating polyneuropathy, and this slowing had a uniform pattern. In conclusion, electrophysiological characteristic of SNCS may be useful in the diagnosis and research between patients with CMT 1 and CIDP.

• Annals of Clinical Neurophysiology
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• v.22 no.1
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• pp.8-12
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• 2020

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous features. Appropriate treatment will produce a favorable outcome, but a poor treatment response and severe disability have also been reported. The roles of the clinical phenotypes and electrophysiological features of CIDP as well as of autoantibodies against nodal and paranodal proteins have been highlighted previously due to their association with the treatment response and long-term prognosis. This review addresses the diverse factors associated with the prognosis of CIDP.

• Annals of Clinical Neurophysiology
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• v.13 no.2
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• pp.97-100
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• 2011

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyneuropathy. Corticosteroids, intravenous immunoglobulin (IVIG) and plasmapheresis have been reported to be effective treatment. Rarely, CIDP can occur in the patients with HIV infection. The clinical features and electrophysiological findings of CIDP are known to be similar in patients with and without HIV infection. We report a 30-year-old male with HIV infection associated CIDP who improved after the administration of intravenous immunoglobulin and long term oral prednisone.

• Korean Journal of Radiology
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• v.21 no.4
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• pp.483-493
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• 2020

Objective: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency. Materials and Methods: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained. Results: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy. Conclusion: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

• Clinical and Experimental Pediatrics
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• v.58 no.5
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• pp.194-198
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• 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

• Annals of Clinical Neurophysiology
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• v.10 no.1
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• pp.70-73
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• 2008

By definition, the time to reach nadir in Guillain-Barre syndrome (GBS) is within four weeks. This is in contrast to the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which progress for at least two months. However, CIDP can take a relapsing and remitting form and could mimic treatment related fluctuations of GBS (GBS-TRFs) especially during the early phase of disease. We report a patient with CIDP who initially presented with a rapidly progressive limb weakness mimicking GBS, but finally showed good recovery after long term corticosteroid therapy.

• Annals of Clinical Neurophysiology
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• v.24 no.2
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• pp.59-62
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• 2022

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.

• Clinical Endoscopy
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• v.56 no.2
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• pp.245-251
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• 2023

A plethora of paraneoplastic syndromes have been reported as remote effects of colorectal carcinoma (CRC). However, there is a dearth of data pertaining to the association of this cancer with demyelinating neuropathies. Herein, we describe the case of a young woman diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP). Treatment with intravenous immunoglobulins and prednisone did not improve her condition, and her neurological symptoms worsened. Subsequently, she was readmitted with exertional dyspnea, lightheadedness, malaise, and black stools. Colonoscopy revealed a necrotic mass in the ascending colon, which directly invaded the second part of the duodenum. Pathologic results confirmed the diagnosis of locally advanced CRC. Upon surgical resection of the cancer, her CIDP showed dramatic resolution without any additional therapy. Patients with CRC may develop CIDP as a type of paraneoplastic syndrome. Clinicians should remain cognizant of this potential association, as it is of paramount importance for the necessary holistic clinical management.

• Annals of Clinical Neurophysiology
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• v.7 no.1
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• pp.17-19
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• 2005

Polyneuropathy that is associated with monoclonal gammopathy of undetermined significance (MGUS) similar to chronic inflammatory demyelinating polyneuropathy (CIDP) has been reported before, whereas a connection to acute inflammatory demyelinating polyneuropathy (AIDP) has not been. A 52 year-old man was presented with ascending paralysis beginning 1 day ago. Neurological examinations showed facial diplegia and decreased motor power and deep tendon reflexes in all extremities. On electrophysiologic study, sensorimotor polyneuropathy was observed. Protein-and immunoelectrophoresis revealed IgA $\lambda$ monoclonal gammopathy. High dose steroid therapy was given and the symptoms improved slightly.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.50-53
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• 2017

An infection is less likely to elicit chronic inflammatory demyelinating polyneuropathy (CIDP) than Guillain-$Barr{\acute{e}}$ syndrome. We here report a case of acute-onset CIDP following hepatitis A virus infection and briefly comment on the potential mechanisms regarding the induction and chronicity of autoimmunity after a viral infection.