Inflammatory neuropathies in childhood – update

Aim: Description of nosology and treatment of inflammatory neuropathies (NP) in children.

Diagnosis: Inflammatory NPs occur much less frequent in children than in adults. According to the clinical signs focal NP, plexus-NP, mononeuritis multiplex (MNM), symmetrical polyneuropathy (PNP), sensory and/or motor NP, cranial and spinal NP can be discerned. Infectious NP is associated with specific organisms, while post- and parainfectious NP is caused by rather unspecific autoimmunological mechanisms. Elektrophysiological investigations allow distinction between axonal and demyelinated NP. Concerning pathophysiology, direct invasion by the pathogen, vasculitic changes, or cell- or antibody mediated anatomic and functional disturbance of axon and Schwann cell can be found. As a differential diagnosis hereditary, toxic and ischemic NPs have to be considered. Epidemiologically, in children the following NPs occur with decreasing frequency: focal NP of cranial nerves, postinfectious demyelinated PNP with acute or chronic course, toxic PNP and MNM in the context of autoimmunological or malignant diseases.

Treatment: Pathogen associated infective NPs can be treated causally (Borreliosis, Zoster, Leprosy). In postinfectious PNP (GBS, CIDP) remission can frequently be accelerated by plasmaexchange or immunoglobulins; chronic variants also respond to corticoids and immunosuppressants. Plexus-NP and shoulder amyotrophy are reported to bear a good prognosis; in severe cases an immunomodulatory or -suppressive treatment has also to be discussed.

Keywords: Guillain-Barré Syndrom, CIDP, mononeuritis, mononeuritis multiplex