Hematopoietic Stem Cell Transplantation in the Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Richard K. Burt, Alberto M. Marmont in Stem Cell Therapy for Autoimmune Disease, 2019
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune mediated neuropathy. The characteristic clinical picture is one of slowly progressive weakness and sensory loss. Muscle stretch reflexes are depressed or absent. The diagnosis is supported by findings of elevated CSF protein, demyelinating electrodiagnostic features and an abnormal nerve biopsy. There are several lines of evidence that suggest CIDP is an autoimmune disorder involving autoreactive antibodies and T cells. Beneficial treatments include corticosteroids, intravenous immunoglobulin and plasma exchange. Patients with a progressive course who are refractory to such traditional therapy may be candidates for a more aggressive treatment approach involving high dose immune suppression followed by hematopoietic stem cell transplantation (HSCT).
Peripheral Neuropathies
John W. Scadding, Nicholas A. Losseff in Clinical Neurology, 2011
CIDP is predominantly a symmetric polyradiculoneuropathy with symptoms of weakness, sensory loss and paraesthesiae. Both proximal and distal muscles may be affected early on in the illness, and although the weakness can be severe, muscle wasting is not a major feature, because the pathology is demyelination with areas of conduction block, rather than marked loss of axons. Neck weakness is common, but facial weakness is rare (<15 per cent) and is usually mild. Respiratory muscle weakness does not occur. There is hyporeflexia or areflexia and sensory loss in a stocking-and-glove distribution with an emphasis on loss of large fibres, leading to a sensory ataxia. Autonomic features can occur, but are uncommon.
Therapeutic apheresis
Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond in Transfusion Medicine in Practice, 2020
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) describes a somewhat heterogenous group of neurologic disorders characterized by a progressive or relapsing multifocal sensorimotor peripheral nerve demyelination (in 6–20% of cases, only sensory or motor nerves are involved) developing over at least 2 months. Patients are typically hypo- or areflexic in all four extremities. Like AIDP, the demyelination is thought to involve a humorally mediated autoimmunity directed against peripheral nerve myelin. Unlike AIDP, patients with CIDP frequently show some response to corticosteroid therapy. Small randomized studies of plasma exchange have shown clinical improvement in 33–80% of patients.37,38 Improvement is usually noted within 3–6 days of initiating therapy; however, relapse within 1–2 weeks following therapy has been reported in two-thirds of patients. The use of IVIG has also been shown to be effective in CIDP, and in the one small (20 patients) prospective trial of plasma exchange versus IVIG, there was no significant difference in effect.39 Similarly, a large retrospective study of plasma exchange and IVIG for CIDP (33 patients in the plasma exchange group, and 21 in the IVIG group) found similar responses to therapy.40 As with AIDP, given the equivalent therapeutic benefit and comparable cost of IVIG versus plasma exchange, since IVIG has a greater convenience of administration and less morbidity, it may be the preferred therapy. If plasma exchange is used, a course of four to six exchanges over 2 weeks followed by a taper as needed to achieve a stable response has been recommended.
Outcome measures and biomarkers in chronic inflammatory demyelinating polyradiculoneuropathy: from research to clinical practice
Published in Expert Review of Neurotherapeutics, 2021
Jeffrey A. Allen, Filip Eftimov, Luis Querol
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral nerve syndrome that affects 1.0 to 8.9 persons per 100,000 and has an incidence of up to 1.6 per 100,000 per year [1,2]. Although CIDP is clinically heterogeneous, the core clinical characteristic features include motor and/or sensory dysfunction that evolves over 8 weeks or more in a progressive or relapsing pattern [3–5]. While between 60% and 80% of patients develop relatively symmetric proximal and distal numbness and weakness, phenotypes with variable degrees of regional (proximal, distal, or asymmetric) or modality (motor or sensory) involvement are also recognized under the CIDP umbrella [6]. Named variants include multifocal CIDP (Lewis–Sumner syndrome, multifocal acquired demyelinating sensory and motor [MADSAM] neuropathy), distal CIDP (distal acquired demyelinating symmetric [DADS] neuropathy), sensory CIDP, and motor CIDP [4,5]. At disease onset, motor CIDP and sensory CIDP variants are estimated to each make up about 10% of all CIDP, while distal CIDP may be slightly more than 10% and asymmetric CIDP slightly less than 10% [6]. Over time, about half of all patients presenting initially with restricted phenotypes develop motor and sensory symptoms and signs that are proximal, distal, and symmetric [6].
Contemporary challenges in the diagnosis and management of chronic inflammatory demyelinating polyneuropathy
Published in Expert Review of Neurotherapeutics, 2022
The diagnosis of CIDP is clinical. Clinical assessment represents the starting point of the journey of any patient for whom CIDP becomes the suspicion. From then on, either adequate confirmatory electrophysiology leads to effective treatment, or, instead, may commence an unfortunate, long, risky, and costly process of further investigations of low reliability and eventual attempt at treatment. Decisions should solely depend on the clinical evaluation, which needs to be regularly re-visited and guide all subsequent steps. In simple terms, neither demyelinating physiology, nor raised CSF protein levels, nor nerve enlargement on imaging, should indicate CIDP in a subject with exclusively distal weakness, severe early atrophy, neuropathic pain, or autonomic dysfunction. The importance of the history and clinical examination features of the disorder cannot therefore be overemphasized.
Value of nerve biopsy in the management of peripheral neuropathies
Published in Expert Review of Neurotherapeutics, 2018
Stéphane Mathis, Laurent Magy, Gwendal Le Masson, Laurence Richard, Antoine Soulages, Guilhem Solé, Fanny Duval, Karima Ghorab, Jean-Michel Vallat, Mathilde Duchesne
An examination by electron microscopy is often required; in a well-organized and equipped laboratory, these ultrastructural technologies are fairly well automated and enable these examinations to be carried out relatively quickly and inexpensively by an experienced neuropathologist. Nevertheless, as in other areas of pathology, we note a certain disaffection for this technique, which can nevertheless provide useful information for the care of the patient as well as for research on peripheral nerve diseases. Undoubtedly, there are cases where NB is useful and sometimes indispensable. It may be useful in confirming the diagnosis of CIDP as we have reported, as there is no test or specific marker; the clinical and electrophysiological signs, without showing any specific abnormalities, can evoke it, but sometimes a microscopic examination is required to confirm this entity that has specific and effective treatments. NB is the only technique that can identify certain specific lesions of the nerves during hematological malignancies such as infiltration of malignant cells and immunological deposits; specific treatments are available for such cases. Clearly, the indication for NB has to be discussed on a case-by-case basis, and it needs to be integrated and associated with the clinical, electrophysiological, and immunological findings as well as molecular biology, which entails a comprehensive facility.
Related Knowledge Centers
- Central Nervous System
- Peripheral Nervous System
- Autoimmune Disease
- Peripheral Neuropathy
- Guillain–Barré Syndrome
- Chronic Condition
- Acute
- Progressive Inflammatory Neuropathy
- Anti-Neurofascin Demyelinating Diseases
- Homogeneity & Heterogeneity