Chronic inflammatory demyelinating polyneuropathy

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The chronic inflammatory demyelinating polyneuropathy (also ... polyradiculoneuropathy ), CIDP is a very rarely occurring inflammatory disease of the peripheral nerves ( radiculoneuritis ), which manifests itself by a gradually increasing weakness in the legs and sometimes arms. These increasing weaknesses develop over a period of two months or longer, which is the main diagnostic criterion for differentiation from Guillain-Barré syndrome .

The disease is based on damage to the myelin layer that covers the nerve processes. Chronic inflammatory demyelinating polyneuropathy is treatable. Since the first description in 1890, clinical manifestations and diagnostic criteria have been determined on the basis of several studies, which vary only slightly from one another.

Epidemiology

Chronic inflammatory demyelinating polyneuropathy can affect both males and females of all ages. Men are affected about twice as often as women. It may be diagnosed too seldom, however, because the incidence of the disease ( prevalence ) is 1–2 / 100,000, with the peak of the disease in the fifth to sixth decade of life.

Origin (pathogenesis) of CIDP

Current theories suggest that the body's immune system, which normally protects it from pathogens, perceives components of the myelin layer as foreign matter and defends them. However, it is unclear what triggers this process.

In some patients, the blood contains abnormal proteins that promote damage. Current pathogenetic concepts suggest a different (aberrant) immune response at the cellular and humoral level, which is directed against antigens of the peripheral nerves and in which antibodies , complement , cytokines , autoreactive T cells and macrophages play a role.

CIDP is often associated with other diseases, for example HIV infection , systemic lupus erythematosus , diabetes mellitus , hepatitis C , paraproteinemia and malignancies such as lymphoma and osteosclerotic myeloma.

One of the first research groups to describe the spectrum of peripheral neuropathy in HIV infection was the research group headed by Robert Miller / San Francisco, who as early as 1988 described essentially four variants of clinically differentiated clinical pictures of HIV neuropathy:

  • distal sensorimotor polyneuropathy,
  • chronic inflammatory demyelating polyneuroradiculitis,
  • Mononeuropathia multiplex, and
  • progressive polyradiculitis.

The histological proof of circulating antibodies against myelin and therapeutic approaches using plasmapheresis, which also respond to Guillain-Barre syndrome, were successful.

Clinical manifestation

CIDP develops slowly and by definition reaches its maximum eight weeks and later after the onset of symptoms. Symmetrical paralysis ( paresis ) occurs, proximal (towards the center of the body) or distal (away from the center of the body) with weakening of the reflexes ( hyporeflexia ) or loss ( areflexia ) and varying levels of sensitivity. Patients often complain of tiredness, sensory disorders (paresthesia), which are noticeable by tingling or burning, or feelings of compression in the extremities. Paralysis of the upper extremities causes impaired fine motor skills.

Lower extremity paralysis leads to loss of knee and ankle reflexes, walking difficulties, difficulty climbing stairs and getting up from seating. Sensitive gait ataxia (legs apart, unsteady, unsteady gait) can be present, but it can also be the only symptom, especially in children. Urination discomfort can also occur. For reasons that are still unclear, there is occasional tremor (shaking).

The frequency distribution of the neurological deficits:

  • Motor deficits with 94%
  • Paresthesia with 64%
  • Cranial nerve involvement in 2–32%

Variants of the CIDP

  • Sensory CIDP : characterized by predominantly sensitive symptoms or an irregular (atactic) neuropathy. Electrophysiological evidence shows that the motor nerves are involved; after a longer course, motor failures can also be demonstrated.
  • Lewis Sumner Syndrome : is a multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). This shows an asymmetrical distribution, initially occurs on the upper extremity (mainly sensory or sensorimotor). The detection of multifocal conduction blocks in electrophysiology and a good response to intravenous immunoglobulins are characteristic. There is agreement in the literature with patients with focal demyelinating neuropathy of the upper extremity.
  • CIDP with additional monoclonal IgM gammopathy and detection of antibodies against myelin glycoprotein (MAG-AK)
  • CIDP with MGUS (monoclonal gammopathy of undetermined significance): approx. 10–20% of patients with typical CIDP have monoclonal IgG or IgA gammopathy of undetermined significance. The clinical manifestation and therapy responses are similar to those in CIDP patients without paraproteinemia.
  • Axonal variants : Cases of chronically relapsing and progressive axonal polyneuropathy (steroid-responsive) are known. To date, however, there is no evidence that these are immune-mediated neuropathies. It is also unclear whether there is an association with an acquired multifocal sensorimotor neuropathy, characterized by an asymmetric axonal neuropathy with evidence of ganglioside antibodies.

Diagnosis

The electroneurography provides evidence of slowed nerve conduction velocities (demyelination) to less than 70-80% of the lower limit of normal, extended distal latencies prolonged F-wave latency or F-wave loss. In CSF diagnostics, there is an increase in protein (barrier disorder), which, however, is completely unspecific. Using magnetic resonance imaging symmetrically distributed under circumstances inflammatory changes and / or thickening can be detected spinal roots. The detection of ganglioside antibodies in the serum is possible for laboratory diagnosis.

Differential diagnosis

The POEMS syndrome must be distinguished .

Nerve biopsy

Segmental loss of myelin sheath in a single fiber plucked specimen

Even if a nerve biopsy is not absolutely necessary if the other diagnostic criteria are met to confirm the clinical diagnosis, a biopsy of the sural nerve is usually used to confirm the diagnosis in a differential diagnosis. In the context of clinical studies, a nerve biopsy is mandatory ( Research Criteria. Of the American Academy of Neurology ).

In addition to the detection of demyelinating inflammatory neuropathy in the semi-thin section, the detection of segmental demyelination in the single fiber plucked preparation is of particular importance.

Therapy options

The treatment of CIDP requires a special approach because patients respond differently to it. Since CIDP shows a continuous (progressive), albeit sometimes intermittent, course, long-term drug therapy is usually necessary.

In contrast to GBS, corticosteroids have a positive effect on CIDP. They reduce the expression of pro-inflammatory cytokines and inhibit the formation of T cells ( proliferation ). The intravenous administration of immunoglobulin and the administration of immunosuppressive substances are also therapeutic options. These include azathioprine , cyclophosphamide , cyclosporine , methotrexate , mycophenolate mofetil , rituximab and interferon β 1-a. About two thirds of the patients show a positive therapeutic success. In CIDP patients that are refractory to standard medical therapy, including steroids, IVIG and immunosuppressants provide apheresis procedure such as plasma exchange or immunoadsorption more treatment options. Some patients may require long term outpatient therapy. Plasma exchange or immunoadsorption are then carried out at regular intervals to stabilize the patient.

The CIDP variant Lewis Sumner syndrome responds well to intravenous immunoglobulin administration, but less well to prednisolone .

The CIDP variant with additional IgM gammopathy and MAGAK hardly respond to prednisolone. The response to intravenous immunoglobulin is incomplete.

forecast

The age of onset seems to have an influence on the course. Patients less than 20 years of age often develop motor neuropathy with subacute progression, relapsing-remitting course and good regression. Patients over 45 years of age often show chronic, progressive sensorimotor neuropathy with remaining neurological deficits.

Web links

Individual evidence

  1. ↑ a b D. Kiprov, W. Pfaeffl, G. Parry, R. Lippert, W. Lang, R. Miller: Antibody-mediated peripheral neuropathies associated with ARC and AIDS: successful treatment with plasmapheresis . In: J Clin Apher. tape 1 , no. 4 , 1988, pp. 3-7 .
  2. ^ RG Miller, GJ Parry, W. Pfaeffl, W. Lang, R. Lippert, D. Kiprov: The spectrum of peripheral neuropathy associated with ARC and AIDS . In: Muscle Nerve . tape 11 , no. 8 , 1988, pp. 857-863 .
  3. ↑ L. Germaniskis, EJ Singer: HIV and peripheral neuropathy . In: J Int Assoc Physicians AIDS Care . tape 1 , no. 1 , 1995, p. 30-33 .
  4. ↑ H. Köller, BC Kieseier, S. Jander, H.-P Hartung: Acute and chronic inflammatory neuropathies - diagnostics. In: Dtsch Med Wochenschr. Volume 128, No. 24, Jun 13, 2003, pp. 1357-1360. PMID 12802746 .
  5. ↑ Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. In: Neurology. Volume 41, No. 5, 1991, pp. 617-618. PMID 2027473
  6. ^ R. Press, FL Hiew, YA Rajabally: Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice. In: Acta Neurol Scand . tape 133 , no. 4 , 2016, p. 228-238 , PMID 26437234 .
  7. ↑ N. Galldiks, L. Burghaus, C. Dohmen, S. Teschner, M. Pollok, J. Leebmann, N. Frischmuth, P. Hollinger, N. Nazli, C. Fassbender, R. Klingel, T. Benzing, GR Fink, WF Haupt: Immunoadsorption in patients with chronic inflammatory demyelinating polyradiculoneuropathy with unsatisfactory response to first-line treatment. In: Eur Neurol . tape 66 , no. 4 , 2011, p. 183-189 , PMID 21912134 .
  8. ↑ I. Lieker, T. Slowinski, L. Harms, K. Hahn, J. Klehmet: A prospective study comparing tryptophan immunoadsorption with therapeutic plasma exchange for the treatment of chronic inflammatory demyelinating polyneuropathy. In: J Clin Apher . tape 32 , no. 6 , 2017, p. 486-493 , PMID 28485075 .