Chronic Inflammatory Demyelinating Polyneuropathy In Patient Research Articles

Difficult patient with polyneuropathy: CIDP associated with type 1 diabetes mellitus

Polyneuropathies are a heterogeneous group of diseases that can be caused both by a primary lesion of peripheral nerves, and secondarily, against the background of various somatic diseases. The most common cause of chronic polyneuropathy is distal symmetrical diabetic polyneuropathy. In clinical practice, it is important to be aware of dysimmune polyneuropathy, such as Guillain Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and paraproteinemic polyneuropathy, which lead to severe motor impairment and disability in patients. Identification of the cause of polyneuropathy requires real art, which includes knowledge of the clinical, electrophysiological picture and variants of the course of the disease, as well as a wide range of conditions leading to their development. Timely diagnosis of polyneuropathies and early assignment of etiological and pathogenetic therapy reduce the risk of developing irreversible changes in peripheral nerves caused by axonal degeneration. In the treatment of polyneuropathy of various origins, a special place is occupied by B vitamins, which have a neurotropic effect. Cyanocobalamin is a pathogenetic therapy in patients with diabetes who take metformin for a long time and who developed polyneuropathy due to vitamin B12 deficiency. In one patient, a combination of several variants of polyneuropathies is possible. The article presents a clinical case of a patient with type 1 diabetes mellitus (DM) who developed dysimmune chronic inflammatory demyelinating polyneuropathy (CIDP) associated with DM on the background of distal symmetrical painless diabetic polyneuropathy after a COVID- 19 infection. A feature of the development of CIDP was the acute onset of the disease. Variants of the clinical picture, ENMG criteria, as well as features of treatment, the effectiveness of therapy and the prognosis of CIDP in patients with DM are discussed.

Existing and Emerging Approaches to Treating Chronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare disabling disease with an incompletely understood autoimmune etiology. Differentiating the condition from other neurological diseases can be challenging and appropriate treatment is often delayed. Intravenous immunoglobulin (IVIg), plasmapheresis, corticosteroids and subcutaneous immunoglobulin (SCIg) have all been demonstrated to be beneficial in placebo-controlled, randomized clinical trials. Corticosteroids, including methylprednisolone and dexamethasone are effective and frequently used in CIDP but their long-term use is limited by side effects. One of the most commonly prescribed treatments for CIDP is IVIg which diminishes inflammatory processes and prevents disease progression. Treatment with IVIg has proven effective in randomized, double blind, placebo controlled, clinical trials and the results support its use in CIDP. For some patients, the benefit of IVIg, is limited by the frequency of infusions and systemic side effects such as flu-like symptoms, headache, and nausea. Other effective treatments for CIDP include corticosteroids that are associated with serious side effects in long-term use and plasmapheresis which requires specialized facilities. More recently, SCIg has been demonstrated in double blind, placebo-controlled studies to be effective for maintenance use in CIDP in patients whose disease has been controlled by IVIg. In a large clinical trial, 0.2 g/kg and 0.4 g/kg body weight doses of 20% SCIg equivalent to 1 mL/kg or 2 mL/kg, respectively, administered weekly, demonstrated efficacy in CIDP and were well tolerated. Immunomodulating treatments such as cyclophosphamide, mycophenolate mofetil and rituximab have also shown efficacy in select populations with CIDP.

Diagnostic criteria of chronic inflammatory demyelinating polyneuropathy in diabetes mellitus.

The possibility of co-association between diabetes mellitus (DM) and chronic inflammatory demyelinating polyneuropathy (CIDP) has long been a focus of interest as well as of clinical significance. As CIDP is a potentially treatable condition, it is diagnosis in the context of DM is of great importance. However, diagnostic criteria to identify CIDP in patients with diabetes are not available. We propose a diagnostic tool that should help clinicians to decide what is the probability that a patient with diabetes might have CIDP. We list several clinical, electrophysiological, and laboratory parameters that, when combined, have the power of discriminating an immune-mediated neuropathy in patients with DM. By summing the points assigned to each of these parameters, we define four levels of probability for a patient with diabetes to have CIDP. To analyze the validity of the diagnostic toll, we applied it in three different patient populations: (i) Patients with diabetes with peripheral neuropathy, (ii) Patients with CIDP without DM, and (iii) Patients with diabetes with CIDP. The scores of patients with diabetes without CIDP ranged from -7 to 2, while those of patients with DM-CIDP ranged from 2 to 20. The scores of non-diabetic patients with CIDP were similar to those of patients with DM-CIDP and ranged from 6 to 16. The mean score of patients with DM-CIDP was 9.083, while the score of patients with CIDP was 11.16 and that of patients with diabetic polyneuropathy was -3.59. These results show that this diagnostic tool is able to identify patients with diabetes with overlapping CIDP.

A European multicentre reappraisal of distal compound muscle action potential duration in chronic inflammatory demyelinating polyneuropathy

The electrodiagnostic value of distal compound muscle action potential duration (DCMAPD) has been studied rarely in chronic inflammatory demyelinating polyneuropathy (CIDP). Cut-offs proposed have not been widely evaluated. The influence of low-cut EMG filter settings ≤ 10 Hz as used in Europe is uncertain. We retrospectively reviewed records of 110 patients with typical, treatment-responsive CIDP, from Leicester, U.K., Paris and Angers, France. All fulfilled revised European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) clinical and electrodiagnostic criteria for typical CIDP (2010), before consideration of DCMAPD prolongation. Results were compared with those of 110 controls with chronic sensory/sensory-motor axonal neuropathy. We constructed receiver operating characteristic (ROC) curves for each nerve and derived cut-offs for DCMAPD prolongation, offering specificity of ≥ 98% vs. controls. DCMAPD was significantly greater in all nerves for CIDP patients, compared with controls (P < 0.001). ROC curves allowed derivation of cut-offs of sensitivities ranging from 27.1% (ulnar nerve) to 60% (tibial nerve). Using these cut-offs to define DCMAPD prolongation in any studied motor nerve offered a sensitivity of 69.1% for CIDP and specificity of 97.3% vs. controls. Cut-offs for DCMAPD are dependent on EMG filter settings. DCMAPD prolongation in any motor nerve, using our derived cut-offs, represents a sensitive and specific marker of CIDP in patients studied with EMG equipment with low-cut filter settings ≤ 10 Hz. Appropriate use of this parameter appears an essential criterion to consider in assessing suspected CIDP, which may be helpful in limiting extensiveness and duration of electrophysiological testing, thereby reducing patient discomfort.

Biomarkers of CIDP in patients with diabetes or CMT1

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease that targets the myelin sheaths in peripheral nerves. Primary demyelination can be detected by electrodiagnostic studies or nerve biopsy, but these do not distinguish between demyelination resulting from CIDP or from non-inflammatory causes such as diabetes or Charcot-Marie-Tooth type I. Consequently, the diagnosis of CIDP in such patients is often missed. Studies are needed to establish electrodiagnostic criteria for CIDP in patients with diabetes, and to identify biomarkers that distinguish between inflammatory and non-inflammatory causes of demyelinating neuropathy.

A Case of Chronic Inflammatory Demyelinating Polyneuropathy in a Patient with Systemic Lupus Erythematosus

Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by symmetrical weakness, impaired sensation, absent or diminished tendon reflexes, an elevated cerebrospinal fluid protein level and nerve-conduction studies that show demyelination. The occurrence of CIDP in patients with systemic lupus erythematosus (SLE) has been rarely reported. We experienced a case of a 33 year-old woman with SLE and she presented with fever, abdominal pain, a tingling sensation of both of her hands and feet, and symmetrical weakness in both the proximal and distal extremities. Her symptoms had persisted for over 1 months before she visited our department. The CSF examination showed an elevated protein level and the nerve conduction studies revealed demyelination. Her symptoms showed minimal improvement with high dose steroid and immunoglobulin therapy, but she responded to cyclophosphamide therapy.

Demyelinating neuropathy in diabetes mellitus.

Recent studies have reported that patients with diabetes mellitus (DM) have a predisposition to develop chronic inflammatory demyelinating polyneuropathy (CIDP). To determine whether patients with DM have a polyneuropathy fulfilling electrophysiologic criteria for CIDP, and whether CIDP is more frequent in patients with type 1 than in patients with type 2 DM. We prospectively studied the frequency of electrophysiologic changes meeting the criteria for CIDP in patients with DM seen in our electrophysiology laboratory during a 51-month period (period 1). To evaluate the relationship between DM and CIDP, we prospectively determined during a 14-month period (period 2) the frequency of DM in patients seen in our electrophysiology laboratory with other neuromuscular diseases, and the frequency of idiopathic CIDP. During period 1, 120 patients with DM met the electrophysiologic criteria for CIDP (DM-CIDP). The most frequent clinical features of DM-CIDP were those of a predominantly large-fiber sensorimotor neuropathy, with recent motor deterioration and a moderately increased cerebrospinal fluid protein concentration. Twenty-six of the 120 patients were given intravenous immunoglobulin (400 mg/kg per day for 5 days), and 21 patients (80.8%) had significant improvement in the neurologic deficit at the end of 4 weeks of therapy. The DM-CIDP occurred equally in type 1 and type 2 DM. During period 2, 1127 patients were seen. Of these, 189 (16.8%) had DM with various neurologic disorders, including 32 patients (16.9%) with DM-CIDP. Among the remaining 938 patients without DM, 17 (1.8%) had idiopathic CIDP. The odds of occurrence of DM-CIDP was 11 times higher among diabetic than nondiabetic patients (P<.001). Demyelinating neuropathy meeting the electrophysiologic criteria for CIDP occurred in both types of DM, and its occurrence was significantly higher in diabetic than in nondiabetic patients.