Chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired neuropathy, presumably of immunological origin. Its clinical presentation and course are extremely variable. CIDP is one of the few peripheral neuropathies amenable to treatment. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In other instances the neuropathy is predominantly or exclusively motor or sensory, CSF normal and electrophysiological studies fail to show evidence of demyelination. In such cases conventional diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In this paper we review the diagnostic pitfalls and clinical variants of CIDP to illustrate the problems that may arise. The different therapeutic options are reviewed. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment.     

Chronic inflammatory demyelinating polyneuropathy in childhood

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is relatively rare. However, it has been recognized for many years. In patients presenting with this disease, subacute onset of weakness usually develops over at least 2 months and often progresses to a loss of ambulation. Some children's initial presentations may mimic Guillain-Barré syndrome. Dysasthesias are common. Males are affected more than females, and antecedent illnesses or vaccinations occur in approximately half of patients. Physical examination reveals diffuse, proximal greater than distal weakness, with an absence or depression of muscle stretch reflexes. Electrophysiology confirms demyelination, and spinal fluid examination demonstrates albuminocytologic dissociation. The clinical presentation, diagnosis, and prognosis of childhood CIDP are reviewed. Treatment and immunologic features are also discussed in this article.     

Chronic inflammatory demyelinating polyneuropathy and other immune-mediated demyelinating neuropathies

The identification of specific disorders caused by immune-mediated attack on Schwann cells and myelin have both improved and confused our understanding and classification of chronic inflammatory and immune-mediated neuropathies. Some investigators consider all neuropathies that respond to immunosuppressive treatment as chronic inflammatory demyelinating polyneuropathy (CIDP), regardless of whether there is evidence for demyelination. Other investigators rely on a very strict definition of CIDP and do not include variations. The goal of this article is to clarify the spectrum of these disorders and provide a logical classification scheme that has clinical utility and yet allows for new information that might distinguish new variations. Therapeutic strategies are also discussed.     

Chronic inflammatory demyelinating polyneuropathy and respiratory failure

Neuromuscular respiratory failure is not considered to be a clinical feature of chronic inflammatory demyelinating polyneuropathy (CIDP). We present 4 patients with CIDP who required respiratory assistance and mechanical ventilation. Two patients needed emergent intubation and one patient lapsed in a stupor from hypercapnia. Respiratory failure in CIDP should be considered exceptional, but more formal studies in CIDP may be needed to assess its prevalence.     

Chronic inflammatory demyelinating polyneuropathy following malignant melanoma

Polyneuropathy has rarely been encountered in association with melanoma. Although this association may be a biologically plausible phenomenon considering the common embryonic origin of the melanocytes and Schwann cells with proven antibody cross reactivity, only few such cases have been reported so far. We report 2 new cases of chronic inflammatory demyelinating polyneuropathy in association with malignant melanoma, which we believe will add more evidence of this association. We also include a short review of the literature for similar cases.     

Chronic inflammatory demyelinating polyneuropathy complicating liver transplantation

We report on a patient who had chronic inflammatory demyelinating polyradiculoneuropathy following liver transplantation for hepatitis B-induced fulminate hepatic failure. Possible symptomatic recurrence of hepatitis B in the graft prompted a reduction in the cyclosporine dosage, which may have triggered the immune response leading to demyelination. Initial plasma exchange and then intravenous immune globulin after relapse led to Marchked clinical improvement.     

Chronic inflammatory demyelinating polyneuropathy associated with carcinoma.

The association of chronic inflammatory demyelinating polyneuropathy (CIDP) and carcinoma has rarely been reported and its relevance is debated. Thirty three consecutive patients with probable or definite CIDP (idiopathic or associated with M protein) were investigated. Three patients with definite CIDP had a concomitant carcinoma. One had an IgM paraprotein. Steroids and intravenous immunoglobulins were effective.     

Chronic inflammatory demyelinating polyneuropathy in two siblings.

A familial occurrence of chronic inflammatory demyelinating polyneuropathy is reported. The diagnostic problems in distinguishing the progressive form of this disease in childhood from hereditary motor and sensory neuropathy types I and III are discussed. Criteria for a definite diagnosis of chronic inflammatory demyelinating polyneuropathy are proposed.     

Chronic inflammatory demyelinating polyneuropathy after treatment with interferon-alpha

Treatment with interferon-alpha (IFN-alpha) has been associated with the occurrence of a number of autoimmune disorders. We report a case of chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a patient with a chronic viral hepatitis C infection who received a novel, long-acting form of IFN-alpha. After withdrawal of the interferon treatment, this patient responded to a single extended course of plasma exchange that resulted in a complete clinical remission of symptoms without relapse.     

Chronic inflammatory demyelinating polyneuropathy in dogs and cats

Over the past several years, we have accumulated data on a spontaneous demyelinating peripheral neuropathy that is not well identified in domestic animals. This disorder occurs in dogs and cats of either sex and does not appear breed-related. Onset of signs is usually insidious and the course is typically chronic, sometimes relapsing, and often slowly progressive. Mature animals of any age may be affected. Clinical signs include tetraparesis, sometimes progressing to tetraplegia, stumbling gait, and hyporeflexia. Motor nerve conduction velocities are decreased. Pathologically, changes in teased single fibers from peripheral nerves are dominated by multifocal paranodal demyelination. Scattered, thinly myelinated fibers are seen on semithin sections. Ultrastructural studies reveal macrophages within myelinated fibers stripping the myelin sheaths, naked and remyelinating axons, and focal/multifocal endoneurial mononuclear cells. Indirect immunofluorescence revealed positive IgG staining in peripheral nerve myelin sheaths from two dogs. The course of the disease, clinical signs, electrophysiology, and pathology have similarities to chronic inflammatory demyelinating polyneuropathy in people.     

Chronic inflammatory demyelinating polyneuropathy in common variable immunodeficiency

Common variable immunodeficiency comprises a heterogeneous group of primary antibody deficiencies with complex clinical and immunologic phenotypes. Immune dysregulation leads to the generation of multiple autoantibodies against various antigenic targets in patients with common variable immunodeficiency. Chronic inflammatory demyelinating polyneuropathy is a heterogeneous disorder that indicates an autoimmune response against peripheral nerve myelin. We describe a 7-year-old girl with common variable immunodeficiency who developed chronic inflammatory polyneuropathy. A 5-day course of intravenous immunoglobulin (500 mg/kg/day) improved her neurologic disorder. Chronic inflammatory demyelinating polyneuropathy should be added to the broadening spectrum of neurologic complications in common variable immunodeficiency. Early detection and consequent treatment may reverse the neurologic sequelae.     

Chronic inflammatory demyelinating polyneuropathy in a patient with hyperIgEaemia

We herein report the case of a 46 year old man with chronic inflammatory demyelinating polyneuropathy (CIDP) with hyperIgEaemia. The patient presented with bilateral weakness, generalized hyporeflexia, and mild paresthesia of the fingers of both hands. Nerve conduction studies revealed multiple sites of motor conduction block in the absence of sensory abnormalities. Muscle strength increased, as did compound muscle action potential (CMAP) amplitude immediately after the intravenous infusion of immunoglobulin (IVIg). Serum IgE levels also fluctuated in parallel with his relapsing-remitting clinical course. We propose that pure motor CIDP may be immune mediated and suggest that IgE-mediated allergy may be one potential cause of this condition.     

Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis

We report a patient with chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis (PBC). Except for minimal biochemical abnormalities, clinical symptoms of PBC were not observed, and we diagnosed our patient with asymptomatic PBC from the results of a liver biopsy. Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves. The disturbed sensory pattern was asymmetrical, and sensory nerve action potentials were not evoked. From these observations, we diagnosed this patient with chronic inflammatory demyelinating polyneuropathy. Neuropathy associated with PBC is very rare. We must differentiate demyelinating neuropathy with PBC in patients with asymmetrical sensory dominant neuropathy with high immunoglobulin M titers, and investigate for the presence of anti-mitochondrial antibodies to rule out a complication of asymptomatic PBC.     

Chronic inflammatory demyelinating polyneuropathy presenting with features of GBS

The authors report five patients with inflammatory demyelinating polyneuropathy with a Guillain-Barré syndrome (GBS)-like onset and initial clinical features, but with persistent symptoms similar to chronic inflammatory demyelinating polyneuropathy (CIDP). Patients in the chronic phase improved with corticosteroid or IV immunoglobulin therapy. Patients with apparent GBS who show persistent symptoms may benefit from corticosteroids or other treatment that is beneficial in the management of CIDP.