Pulse steroid inflammatory polyneuropathy

A 17-year-old male diagnosed with systemic lupus erythematosus (SLE), showing poor compliance with medication, presented to our facility with a 20-day history of fever, polyarthritis, and cough. Additionally, he had experienced a seizure episode, followed by a one-day history of altered mentation. Subsequently, he developed pneumonia, respiratory distress, and shock, necessitating ventilator and inotropic support. Neuropsychiatric lupus (NP-lupus) was suspected, and hence high-dose steroids, hydroxychloroquine, and broad-spectrum antibiotics were initiated...

Systemic lupus erythematosus (SLE) is an autoimmune disorder that can manifest with a wide range of clinical features, including peripheral nervous system involvement. Among the neurological complications associated with SLE, chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare but significant entity. This case report explores the complex relationship between CIDP and SLE, emphasizing the challenges in diagnosis and the complexities of treatment strategies. We present the case of a patient diagnosed with CIDP as the initial manifestation of SLE, who exhibited a remarkable response to a unique treatment approach...

PURPOSE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) developing in the postvaccination period was distinctly unusual and its course was rarely well described. We aimed to clearly depict the clinical features of acute-onset multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) caused by mRNA-1273 COVID-19 vaccination. CASE REPORT: A 74-year-old man noticed weakness of hands 2-3 days after he accepted the second dose of mRNA-1273 COVID-19 vaccine...

PURPOSE OF REVIEW: Peripheral nervous system vasculitides (PNSV) are a heterogeneous group of disorders with a clinical subset that may differ in prognosis and therapy. We provide a comprehensive update on the clinical assessment, diagnosis, complications, treatment, and follow-up of PNSV. RECENT FINDINGS: Progress in neuroimaging, molecular testing, and peripheral nerve biopsy has improved clinical assessment and decision-making of PNSV, also providing novel insights on how to prevent misdiagnosis and increase diagnostic certainty...

INTRODUCTION/AIMS: A model for predicting responsiveness to immunotherapy in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) has not been well established. We aimed to establish a new classifier for CIDP patients based on clinical characteristics, laboratory findings, and electrophysiological features. METHODS: The clinical, laboratory, and electrophysiological features of 172 treatment-naïve patients with CIDP between 2003 and 2019 were analyzed using an unsupervised hierarchical clustering...

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare affection of the peripheral nervous system. Its diagnostic criteria have evolved since 1975. The aim of our work is to study the epidemiological, clinical, and paraclinical aspects of CIDP. METHODS: We conducted a retrospective study of 28 CIDP patients of the neurology department of the military hospital of Tunis between January 2000 and December 2017. All these patients met the European Federation of Neurological Societies/Peripheral Nerve Society(EFNS/PNS)2010 diagnostic criteria for definite CIDP...

We report the case of a 53-year-old woman with severe chronic inflammatory demyelinating polyneuropathy (CIDP) who developed progressive tetraplegia with respiratory failure despite receiving a standard dose of intravenous immunoglobulin therapy (IVIg), steroid pulse therapy, plasma exchange, and cyclosporine. We administered high-dose IVIg (3 g/kg; 0.6 g/kg/day for 5 consecutive days at monthly intervals). The patient's muscle weakness gradually improved after IVIg. She recovered completely 2 years after the onset of symptoms...

INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disorder with progressive weakness. Acute-onset CIDP resembles Guillain-Barre syndrome (GBS), a rapidly progressive disorder, and follows a chronic course. To our knowledge, no case of acute-onset CIDP in hantavirus and hepatitis B virus (HBV) coinfection has been reported previously. CLINICAL FINDINGS: We report a case of acute-onset CIDP that was initially diagnosed as GBS...

Anti-ganglioside antibodies have been reported in various peripheral neuropathies, including Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, Fisher syndrome, monoclonal gammopathy-associated neuropathy, and other idiopathic neuropathies. To our knowledge, there has been no report of anti-ganglioside-positive sarcoidosis. We report a 62-year-old man with acute weakness of the limbs and sensory disturbance of the right arm and trunk resembling GBS...

Polyneuropathy associated with antibodies directed against myelin-associated glycoprotein (MAG) is a chronic symmetric sensorimotor demyelinating neuropathy caused by monoclonal IgM against MAG (anti-MAG neuropathy). Intravenous immunoglobulin therapy (IVIg) has been partially successful in patients with anti-MAG neuropathy. A placebo-controlled trial of rituximab in patients with anti-MAG neuropathy has been reported. We report rapid improvement in a patient with anti-MAG neuropathy using rituximab. A 58-year-old man presented with abnormal sensation, weakness of the limbs, and unsteadiness...

We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies...

Early and effective treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) is important to minimize axonal degeneration that occurs secondary to demyelination. The disease course is invariably chronic, so long-term treatment is often required, and adverse effects and costs are important considerations in devising a treatment plan. CIDP responds to prednisone, but long-term treatment can result in significant adverse effects. Azathioprine, mycophenolate mofetil, and cyclosporine can be used as steroid-sparing agents and may facilitate more rapid and successful tapering of prednisone...

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy that responds to various immunosuppressive treatments. Oral daily prednisone therapy is effective and inexpensive, but the long-term treatment that is usually necessary leads to serious adverse effects. Consequently, intravenous immunoglobulin and plasma exchange have been widely used to treat CIDP, making treatment expensive and inconvenient. A steroid regimen that reduces adverse effects but preserves efficacy would simplify treatment...

We present a young Thai man who developed acute flaccid paralysis after receiving pulse methylprednisolone for chronic inflammatory demyelinating polyneuropathy. Hypokalemia from intracellular shift was confirmed by calculation of transtubular potassium gradient (TTKG). His muscle strength and serum potassium fully recovered with a small amount of potassium replacement. Graves' disease was subsequently diagnosed and treated with radioactive iodine. We suggest that acute paralysis after the use of steroids should raise a suspicion of thyrotoxic periodic paralysis (TPP)...

OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune syndrome where certain autoantibodies define clinicopathologic subgroups. In the present study, serum anti-cardiolipin antibodies (aCL) were evaluated. MATERIALS AND METHODS: We investigated aCL in sera from 21 patients diagnosed with CIDP in our hospital between 1991 and 2001. The four CIDP patients with aCL (aCL+) were compared with 17 patients without aCL (aCL-). RESULTS: All aCL+ patients displayed sensory-motor polyneuropathy, with severity and distribution of weakness resembling those in aCL- patients...

Eight patients with neuroimmunological disorders refractory to conventional immunotherapies were given totally 17 courses of pulse-dose cyclophosphamide. The regimen consisted of 600 mg/m2 of intravenous cyclophosphamide at days 1,2,4,6 and 8 and infusion of 2,500-3,000 ml of fluids in order to avoid hemorrhagic cystitis. Some cases were followed by a monthly intravenous dose of cyclophosphamide as a booster treatment. As a result, all but one case of neuro-Behçet disease have shown neurological improvements...

A 47-year-old female patient with chronic inflammatory demyelinating polyneuropathy started to recover from her numbness and weakness within a few hours following the commencement of intravenous methylprednisolone 1,000 mg. In parallel with the recovery of muscle strength, a prolonged latency time of the M-wave was normalized within a day by a revival of the new motor units with a normal latency. In many cases with CIDP, it has been recognized that the gradual decrease in latency time over weeks is a later phenomenon following early increase in amplitude of the M-wave during recovery of weakness, which is explained by remyelinating process...

Immunosuppressive treatments of neuro-immunologic diseases: myasthenia gravis, polymyositis, chronic inflammatory demyelinating polyneuropathy and multiple sclerosis were reviewed. The treatments need to be planned in terms of 2-5 years. Cautions must be taken for adverse effects of short and long terms. Corticosteroids were the most well used and were studied medication of the first choice among immunosuppressants in these diseases except for CIDP in which large amounts of IV-Ig or plasmapheresis are the first choice...

A case of early-onset and slowly progressive chronic inflammatory demyelinating polyneuropathy (CIDP) was reported. Her progressive gait disturbance began at six years of age and she developed pes cavus. At the age of 13 years, a diagnosis of CIDP was made, and oral corticosteroid therapy was started. This therapy was effective, but the disease relapsed soon. Muscular strength improved after supplementation of an immunosuppressant with oral corticosteroid, following steroid pulse therapy. On peripheral motor conduction study, M waves showed very low amplitudes and remarkably delayed onset-latencies...