Children with rapid-onset paralysis: is it GBS?
by Patricia Blomkwist
An interesting article* was published in the “European Journal of Neurology” about a study in children. Prof. Bart Jacobs (Erasmus Medical Center, Rotterdam, The Netherlands) was one of the authors. He wondered why, contrary to expectations, some children diagnosed with GBS did not recover well. That is why he and his team initiated this research.
Diagnosis in children, especially young children, who suffer from rapid muscle weakness (paralysis) can sometimes be difficult. There are several diseases other than GBS that can cause such a paralysis in children.
One of those other diseases is Acute Flaccid Myelitis (AFM). Acute Flaccid Myelitis means ‘acute flaccid paralysis due to spinal cord inflammation’. Paralysis of the muscles occurs within a few days. In addition to the muscles in the arms, legs, neck and trunk, the respiratory muscles, swallowing muscles and muscles of the face can also be affected. See https://www.cdc.gov/acute-flaccid-myelitis/about-afm.html
AFM is usually caused by an infection with specific viruses, the enteroviruses, including type D68. AFM caused by that type of virus more often has a poorer outcome.
The researchers compared the data of children diagnosed with AFM (after D68) with those of children diagnosed with GBS. This involved collaborating with several doctors from the University of Groningen who are experts in the field of AFM. The researchers found that in addition to the similarities in the symptoms of GBS and AFM, there were also clear differences that could be helpful in establishing the correct diagnosis. AFM showed a much faster decline than GBS. In children with AFM, the paralysis was much more asymmetrical than in GBS. Children with GBS suffered from sensory disorders much more often than those with AFM. An MRI of the spinal cord showed abnormalities in AFM not seen in GBS. There were also clear differences when examining the cerebrospinal fluid (from a spinal tap).
Sometimes AFM is so similar to GBS that only diligent testing and examination can reveal that it is AFM and not GBS.
In general, children with AFM are treated in the same way as children with GBS. Unfortunately, the prognosis of AFM is often less favorable. In order to give a prognosis, it is therefore important to establish at an early stage which of the two diagnoses is accurate. After all, the patient and family will base their expectations on the prognosis provided by the doctors. At a later stage (more than a few weeks later) it is no longer possible to demonstrate whether it was a case of AFM.
The researchers therefore recommend that children with rapid muscle weakness while a diagnosis of GBS is considered should also be tested at an early stage to confirm or rule out AFM. An MRI of the spinal cord is the most important test for this.
*Helfferich J, Roodbol J, de Wit M-C, Brouwer OF, Jacobs BC; the 2016 Enterovirus D68 Acute Flaccid Myelitis Working Group and the Dutch Pediatric GBS StudyGroup. Acute flaccid myelitis and Guillain–Barré syndrome in children: A comparative study with evaluation of diagnostic criteria. Eur J Neurol. 2021;00:1–12. doi:10.1111/one.15170