Conclusions. The long term prognosis of CIDP patients was generally favourable, but 39% of patients still required immune treatments and 13% had severe disabilities.
Continuing Education Activity Chronic inflammatory demyelinating polyradiculoneuropathy can be caused by multiples diseases, from infectious as well as immunology diseases. It is one of the most challenging diagnoses, and sometimes underdiagnosis for variable presentation.
To avoid high mortality as well as morbidity, it must be promptly diagnosed and treated. This activity reviews the evaluation, diagnosis, and treatment of neuropathy, demyelinating polyradiculoneuropathy, chronic, inflammatory, Is CIDP serious? highlights the role of the interprofessional team in evaluating and treating patients with this condition. Although it has diverse clinical presentations, the classical presentation includes symmetric proximal and distal sensory and motor involvement.
The first case was described by Eichhorst Burns in 1890. However, it has variants that can be seen in association with a neoplastic process e. Epidemiology A recent meta-analysis showed a crude incidence rate of 0.
Overall prevalence has been reported around 0. Due to diverse clinical presentations and diagnostic criteria used around the world, the incidence and prevalence rates also vary. However, the response to plasmapheresis suggests a possible role of B cell-mediated immune processes.
Both T cell, as well as B cell-mediated inflammation, lead to neuronal damage and dysfunction. The activated T cells, along with macrophages, act as antigen-presenting cells and bind directly to targeted structures to promote demyelination. Additionally, gliomedin Is CIDP serious? contactin one have been shown to be targeted as well. The demyelination and remyelination can be visualized on teased fibers analysis in 48% to 68% of the patients, while in 21% of patients, mixed demyelination an axonal changes are seen.
The immunopathological study of the nerve biopsy has shown inflammatory T cells and macrophages that surround the muscle units through the perivascular space in the subunits within the sarcomeres. Ultrastructural studies have demonstrated macrophages extending their processes between myelin leading to the degradation of its components.
Other findings that can be seen are nerve edema, nerve fibrosis, Is CIDP serious? inflammatory infiltrates. The relapsing-remitting Is CIDP serious? can be seen in up to a third of patients. The development of motor weakness is symmetric, affecting proximal or distal muscles with a predominance of large fiber neuropathy paresthesias compared to small-fiber neuropathies. Small fiber includes subjective complaints of burning, jabbing, or shooting pain with decreased sensation to pain and temperature.
Typical motor findings include difficulties lifting themselves up from a chair, climbing stairs, lifting objects over their heads, difficulty with ambulation, and frequent Is CIDP serious? of falls. They may have difficulties with fine motor activities such as dressing, opening jars, and dropping objects at home.
Diffuse areflexia or hyporeflexia, and a positive Romberg are common. However, it has to fall within the eight weeks or more time course with relapsing or progressive course. Severe or atypical variants may have additional symptoms. Bulbar and autonomic symptoms Is CIDP serious? include dysarthria, dysphagia, dyspnea, dysrhythmias, hypotension or hypertension, anhidrosis or hyperhidrosis, urinary retention, impotence, and constipation. Lumbar radiculopathy and cauda equina symptoms may be present in Is CIDP serious?
setting of root nerve hypertrophy. Bilateral phrenic nerve palsy has also been reported, but Is CIDP serious? rare. Tremors, sensory ataxia, neuropathic pain, cramps, and fatigue can be seen in the asymmetric multifocal, purely motor, or purely sensory predominant forms. Atypical symptoms should prompt the clinician to rule out alternate diagnoses. Ancillary testing, which will be discussed in the following section, is essential to distinguish typical from atypical variants. Due to a lack of consensus, the diagnosis is essentially based on clinical features followed by electrophysiological criteria.
The laboratory, cerebrospinal fluid analysis, Is CIDP serious? nerve biopsy are ancillary testings. Often there are conduction deficits on motor potentials. Advanced imaging can help distinguish this variant from other by gross hypertrophy of the plexus affected.
Nerve Biopsy It is ancillary with high specificity. It's particularly useful in challenging cases. Nerve biopsy can exclude other causes of neuropathy such as amyloidosis, vasculitis, toxic or hereditary neuropathies.
Imaging With more understanding of this entity and advancement, nerve and root imaging is incorporated in aiding the diagnosis. The breach in the blood-brain barrier due to inflammatory cells can be seen as gadolinium enhancement on magnetic resonance imaging.
Hypertrophy Is CIDP serious? the nerves can provide a clue of active inflammation. The high-resolution ultrasound helps to visualize the gross anatomy of the nerves and perhaps has the potential to monitor disease activity. Both of them have similar efficacy. However, the long-term adverse effects, as well as the cost-effectiveness, needs to be weighed against the benefits of continuing any maintenance therapy. Steroid-sparing immunosuppressive agents may be used as maintenance therapy e.
The main caveat is the requirement of central venous catheter placement and the associated risk of infections. Some other adverse effects are hypotension, hypocalcemia, allergic reaction to albumin infusion, citrate toxicity. Based on the clinical response and relapses, maintenance therapy is usually given for about six months.
There are no major randomized trials for most of these therapies; however, they have shown benefits in smaller case studies or anecdotal reports. Methotrexate 15 mg weekly has no benefit. Rather can cause serious adverse effects compared to placebo. Similar to Azathioprine, it can cause bone marrow suppression Is CIDP serious?
is contraindicated in pregnant females. In such cases, targeted therapies such Is CIDP serious? rituximab and alemtuzumab can be used. Usually, these patients are followed up for years, the longer they are followed up the more are the chances of relapses.
Based on clinical examination and nerve conduction study, if there is no worsening seen then the immunosuppressive therapy can be eventually be tapered off. A study with 40 patients by Dyck et al demonstrated 72% of patients required immunosuppressive therapy while 27% achieved remission off therapy.
Such patients are committed to long-term immunosuppressive therapy. Furthermore, the patients suffer from several treatment-related side effects such as hypertension, thromboembolic events, risks of infections, bone marrow suppression, nephrotoxicity, and malignancies such as lymphoma.
Patients need to know that early diagnosis and treatment can prevent the progression as well as disabling deficits. The patients need to be aware of the diverse symptoms and the need for timely evaluation by a neurologist or a neuromuscular specialist. The long course of the disease, the side effects of medications, and medical expenses that come along can sometimes be discouraging to the patients for continuing therapy.
However, there are several therapeutic options available that can not only prevent relapses but some patients also achieve complete remission.
Aggressive immunomodulatory therapy combined with physical neurorehabilitation is essential for a long-term favorable outcome. Some of the barriers that may hinder Is CIDP serious? improvement include social determinants of health, pain control, aggressive clinical variants, among others.
An interprofessional team approach will result in the best outcomes.