[A case of Guillain-Barré syndrome with severe pain successfully controlled with acetaminophen, gabapentin, and parenterally infused fentanyl].

We presented a case of an 8-year-old boy with Guillain-Barré syndrome characterized by severe intractable pain in the soma and lower extremities, which appeared 2 weeks after a febrile cold. At his first visit to our hospital, he could not stand or walk because of the severe pain, and muscle weakness and absence of deep tendon reflexes were observed. Guillain-Barré syndrome was diagnosed on the basis of cerebrospinal fluid study results, nerve conduction velocity, and spinal cord magnetic resonance imaging. His pain was scored as a five on a six-point visual analog scale, and it persisted despite routine supportive therapy. The pain was successfully controlled with parenterally infused fentanyl. It is suspected that opioid analgesics are useful for severe pain control in patients with Guillain-Barré syndrome.

Acute motor axonal neuropathy during intensive immunosuppressive therapy for macrophage activation syndrome.

We describe a 2-year-old girl with refractory macrophage activation syndrome (MAS), which is a serious complication of inflammatory disorders associated with rheumatic disease in children. Although she was treated with intensive immunosuppressive therapies such as immunoglobulin, plasma exchange, dexamethasone, methotrexate, cyclosporine, and etoposide, she subsequently developed motor deficit with the abolition of deep tendon reflexes. Since nerve conduction study revealed low-amplitude compound muscle action potentials and motor conduction slowing, she was diagnosed as having acute motor axonal neuropathy (AMAN) associated with refractory MAS. This is the first report of AMAN occurring during immunosuppressive therapy for extremely refractory MAS, suggesting that hypercytokinemia or activated macrophages may have played a critical role in the pathogenesis of AMAN in this patient.

Electrophysiological subtypes and prognosis of childhood Guillain-Barré syndrome in Japan.

Guillain-Barré syndrome (GBS) is classified into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), but little is known about the incidence of the subtypes and the prognosis of childhood GBS. To elucidate the features and long-term prognosis, clinical and electrophysiological data for 31 Japanese GBS children were reviewed. By electrodiagnostic criteria, children were classified as having AIDP (35%) or AMAN (48%), or were unclassified (16%). The AMAN children invariably had normal sensory nerve potentials. Between the two groups, age, sex, and clinical disability did not differ significantly, but the AIDP children more frequently had cranial and sensory nerve involvement, and the AMAN children more frequently had preceding gastroenteritis. By 6 months after onset, all the AIDP and 80% of the AMAN children had regained the ability to walk; by 2 years, all but one of the AMAN children could walk. In Japanese childhood GBS, the proportion of AIDP and AMAN appears to be similar. Recovery is generally favorable in both subtypes, but some of the AMAN children experienced delayed recovery.