mycophenolic-acid and Polyradiculoneuropathy--Chronic-Inflammatory-Demyelinating

The authors retrospectively reviewed the efficacy of mycophenolate mofetil (MMF; mean dose, 2 g/day given for an average of 14 months) in 21 patients with immune demyelinating polyneuropathy. There were no significant differences in the median strength, sensory, or Rankin scores before and after treatment. In patients with immunoglobulin (Ig) M neuropathy, the M-protein decreased by 22%. Three patients with chronic inflammatory demyelinating polyneuropathy (30%) and one patient with IgM neuropathy (13%) improved. The authors found that MMF induced a modest benefit in ~20% of their patients and stabilized patient condition, allowing reduction of steroid or IVIg therapy.

Topics: Adrenal Cortex Hormones; Adult; Aged; Combined Modality Therapy; Drug Evaluation; Female; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Immunosuppressive Agents; Lymphocyte Activation; Male; Middle Aged; Mycophenolic Acid; Nausea; Neurologic Examination; Paraproteinemias; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retrospective Studies; Treatment Outcome

Topics: Adult; Aged; Disability Evaluation; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Treatment Outcome

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder in which early effective treatment is important to minimize disability from axonal degeneration. It has been suggested that some patients with CIDP may benefit from rituximab therapy, but there is no definitive evidence for this.. Baseline and post-rituximab-therapy neuromuscular Medical Research Council (MRC) sum scores, Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, and functional status were assessed in 11 patients with refactory CIDP.. The MRC sum score, INCAT disability score, and functional status improved in all patients after rituximab therapy.. Our study provides evidence of the efficacy of rituximab therapy in at least some patients with CIDP. A placebo-controlled study to assess the effectiveness of rituximab therapy in CIDP with and without nodal antibodies is required to identify disease markers that predict responsiveness.

Topics: Adult; Aged; Azathioprine; Canes; Cyclophosphamide; Female; Foot Orthoses; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Male; Middle Aged; Mobility Limitation; Mycophenolic Acid; Plasma Exchange; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Quadriplegia; Rituximab; Treatment Failure; Treatment Outcome; Walkers

Chronic inflammatory axonal polyneuropathy (CIAP) is defined on the basis of the clinical, electrophysiological and nerve biopsy findings and therapeutic responses of 'immunotherapy responding chronic axonal polyneuropathy (IR-CAP)'.. The diagnosis of IR-CAP was made when all of three of the following mandatory criterion were met: (1) acquired, chronic progressive or relapsing symmetrical or asymmetrical polyneuropathy with duration of progression >2 months; (2) electrophysiological evidence of axonal neuropathy in at least two nerves without any evidence of 'strict criteria of demyelination'; and (3) definite responsiveness to immunotherapy.. Thirty-three patients with IR-CAP showed similar clinical features of chronic inflammatory demyelinating polyneuropathy (CIDP) except 'motor neuropathy subtype'. High spinal fluid protein was found in 27/32 (78%) cases. 'Inflammatory axonal neuropathy' was proven in 14 (45%) of 31 sural nerve biopsies.. IR-CAP could well be 'axonal CIDP' in view of clinical similarity, but not proven as yet. Thus, IR-CAP is best described as CIAP, a distinct entity that deserves its recognition in view of responsiveness to immunotherapy.. Diagnosis of CIAP can be made by additional documentation of 'inflammation' by high spinal fluid protein or nerve biopsy in addition to the first two diagnostic criteria of IR-CAP.

Topics: Adolescent; Adult; Aged; Autoimmune Diseases of the Nervous System; Axons; Azathioprine; Biopsy; Child; Child, Preschool; Chronic Disease; Cyclophosphamide; Cyclosporine; Electromyography; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Inflammation; Male; Middle Aged; Mycophenolic Acid; Neural Conduction; Polyneuropathies; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Sural Nerve; Young Adult

Multifocal motor neuropathy (MMN) is a motor only, asymmetric onset neuropathy that is relatively treatment-refractory compared with classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy.. We reviewed 35 patients seropositive for GM1 (monosialo-asialo [immunoglobulin M, IgM; immunoglobulin G, IgG]) and/or GD1b (disialo [IgG, IgM]) autoantibodies having MMN, classic CIDP, or MADSAM. Immune-treatment responsiveness and clinical course was compared with antibody negative disease controls.. Seventy-nine percent of seropositives with an initial diagnosis of MMN were immunotherapy responsive compared with 46% of seronegatives (P = 0.045). Eight ganglioside antibody positive MMN patients of 19 (42%) developed sensory findings consistent with MADSAM compared with 3 of 41 (7%) seronegative MMN patients (P = 0.003). MMN and MADSAM patients with ganglioside antibody positivity had more sustained treatment responses (P = 0.03).. Patients initially diagnosed with MMN seropositive for diverse GM1 autoantibodies appear more likely to have sustained treatment response and evolution to MADSAM. Muscle Nerve 57: 1000-1005, 2018.

Topics: Autoantibodies; Gangliosides; Humans; Immunoglobulin G; Immunoglobulin M; Immunoglobulins, Intravenous; Immunosuppressive Agents; Mycophenolic Acid; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Rituximab; Treatment Outcome

There are other options open to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are non-responders to conventional treatment, including immunosuppressive and immunomodulatory agents (IA). The aim of this study was to assess whether the use of IA is able to increase the number of responders.. Clinical and electrophysiological data of patients with refractory CIDP, followed at 10 Italian centres, were collected, and the clinical outcome (Rankin Scale) and drug side effects (SE) for the different therapies were analysed.. A total of 110 patients were included. These patients underwent 158 different therapeutic procedures with IA. Seventy-seven patients were treated with azathioprine, 18 rituximab, 13 cyclophosphamide, 12 mycophenolate mofetil, 12 cyclosporine, 12 methotrexate, 11 interferon-alpha and three interferon beta-1a. The percentage of patients who responded to azathioprine (27%) was comparable to the percentage of responders to other therapies, after the exclusion of interferon beta-1a that was not effective in any of the three patients treated. The percentage of SE ranges from 8% (methotrexate) to 50% (cyclosporine).. One-fourth of patients, refractory to conventional treatment, showed an improvement in their disability with IA. Methotrexate had the lowest SE; cyclosporine was associated with severe SE and often led to drug discontinuation.

Topics: Adolescent; Adult; Aged; Antibodies, Monoclonal, Murine-Derived; Axons; Azathioprine; Child; Cyclophosphamide; Cyclosporine; Drug Resistance; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Interferon beta-1a; Interferon-alpha; Interferon-beta; Italy; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Plasmapheresis; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Remission Induction; Retrospective Studies; Rituximab; Severity of Illness Index; Treatment Outcome; Young Adult

Based on case history and clinical and electrophysiological examinations, the authors report on a case of an 8-year-old girl who was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy. The disease was complicated by deafness and kidney fibrosis. During treatment with methylprednisolone and intravenous immunoglobulin, followed by mycophenolate mofetil, prompt improvement of neurological findings occurred. The improvement of hearing was poor. Because the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy has still not been clear, and on the grounds of several cases of chronic inflammatory demyelinating polyradiculoneuropathy conjoined with the kidney disease described in literature (glomerulopathy, interstitial nephritis), every patient with chronic inflammatory demyelinating polyradiculoneuropathy needs to undergo the urinalyses.

Topics: Anti-Inflammatory Agents, Non-Steroidal; Child; Deafness; Female; Fibrosis; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Kidney Diseases; Methylprednisolone; Mycophenolic Acid; Neural Conduction; Neuroprotective Agents; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Treatment Outcome

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may occur in association with diabetes mellitus (DM). We report a case of a poorly controlled diabetic patient who presented with rapid onset of bilateral lower extremity weakness and sensory loss associated with sacral and posterior thigh paresthesias and urinary and bowel incontinence, indicative of cauda equina syndrome (CES). Subsequent evaluation was consistent with CIDP. Monthly infusions with intravenous immunoglobulins (IVIg) with strict glycemic control using insulin resulted in remarkable clinical and electrophysiological recovery. This case report describes a rare presentation of CIDP and emphasizes the importance of early utility of electrodiagnostic (EDX) studies in the clinical evaluation of diabetic patients presenting with rapidly progressive lower extremity weakness and sensory loss associated with diminished reflexes.

Topics: Aged; Central Nervous System; Diabetic Neuropathies; Diagnosis, Differential; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Muscle Weakness; Mycophenolic Acid; Paresis; Peripheral Nerves; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyradiculopathy; Somatosensory Disorders; Treatment Outcome; Urination Disorders

Topics: Humans; Mycophenolic Acid; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

There has been an increasing interest in the use of mycophenolate as an immunomodulatory drug in neuromuscular diseases. We report five consecutive patients with treatment-resistant chronic inflammatory demyelinating polyradiculoneuropathy or multifocal motor neuropathy who were treated with mycophenolate. None showed clinically significant benefit. The use of mycophenolate did not result in the reduction in the dose of corticosteroid or other immunosuppressive agents in any patient. Side-effects, although not serious, were troubling enough for two patients to stop mycophenolate.

Topics: Adjuvants, Immunologic; Adolescent; Adult; Drug Resistance; Humans; Middle Aged; Motor Neuron Disease; Mycophenolic Acid; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retreatment; Treatment Failure