Chronic inflammatory demyelinating polyneuropathy in children: modern criteria for establishing diagnosis and pathogenetic treatment

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is a rare disease, the genesis of which is autoimmune disorders. In CIDP, autoimmune inflammatory processes are mediated by disorders of both the cellular and humoral links of the immune system. Manifestations of demyelination can be observed on any segment of the peripheral nerve from the spinal roots to its distal parts. Currently, there are typical CIDP and CIDP variants. The diagnosis of CIDP is based on the results of the analysis of the patient’s anamnesis, neurological examination and electromyography (EMG) data, which indicate typical signs of demyelinating peripheral nerve damage. Recognition of the clinical phenotype of CIDP variants is crucial, since the diagnostic process and differential diagnosis may differ when compared to typical CIDP. In accordance with the recommendations of the European Academy of Neurology and Peripheral Nerve Society in 2021, fulfillment or incomplete fulfillment with diagnostic clinical and EMG criteria allows making the diagnosis in two categories - “CIDP” and “possible CIDP”. Supportive criteria are used to verify the diagnosis of CIDP only in patients with a “possible” diagnosis. If two supportive criteria are met, then the diagnosis is changed, for example, to “Typical CIDP” or one of the CIDP variants. In pediatric practice, of the supportive criteria, an objective response to treatment with immunomodulatory agents with an objective assessment of clinical improvement and cerebrospinal fluid analysis to detect protein-cell dissociation are most often used. As an induction of pathogenetic treatment of the first line in CIDP children, it is preferable to use 10% intravenous immunoglobulins (IVIg) with an IgG content of more than 95%. The earliest possible use of first-line pathogenetic treatment makes it possible to achieve remission and stop the progression of the disease. The use of adequate supportive treatment using IVIg or subcutaneous immunoglobulins help to stabilize the child’s condition and prevent the formation of neurological deficits.