Abstract
We report the clinical case of a nine-year-old girl who presented with progressive motor neuropathy, revealed via the detection of a higher delay in F-wave recording using digitalized nerve conduction/electromyography. Since the lupus anticoagulant (LAC) positivity, detected using diluted Russell viper venom time (dRVVT), switched to persistent serological anticardiolipin immunoglobulin G (IgG) positivity, a possible non-thrombotic antiphospholipid antibody (aPL)-related clinical manifestation was suspected, and intravenous immunoglobulin treatment (IVIG) was started. The IVIG treatment was well tolerated and the complete resolution of motor impairment was obtained after the third IVIG infusion. Our findings suggest that it could be useful to check for antiphospholipid antibodies in children with a rapid onset of progressive neurological signs in order to provide the beneficial use of IVIG in the treatment of pediatric aPL neurological conditions.
Highlights
In the last few years, an increased number of children affected by underlying autoimmune diseases associated with neurological manifestations as the first clinical sign was evidenced [1]
intravenous immunoglobulin treatment (IVIG) treatment was administered every month for five months, and the girl passed to a monthly subcutaneous immunoglobulin treatment with a good outcome
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic inflammatory neuropathy in children, and it is usually characterized by slowly progressive, symmetric, proximal, and distal paresis and sensory dysfunction. This young girl developed a form of CIDP in the context of transient lupus anticoagulant (LAC) and persistent immunoglobulin G (IgG)
Summary
In the last few years, an increased number of children affected by underlying autoimmune diseases associated with neurological manifestations as the first clinical sign was evidenced [1]. The timing and the type of immunomodulatory therapy could be essential in allowing a successfully outcome. An early and detailed clinical evaluation, together with accurate patient medical history, represents a step in diagnosis, and they could help clinicians to address a more specific early type of treatment. Could support the clinical diagnosis, confirming a more immune than degenerative or hereditary pattern, even in childhood [2]. We report a pediatric clinical case characterized by a progressive inflammatory motor polyneuropathy successfully treated with intravenous immunoglobulin (IVIG), in the presence of transient lupus anticoagulant (LAC) positivity later switching to persistent anticardiolipin immunoglobulin G (IgG) positivity. Brain Sci. 2020, 10, 156; doi:10.3390/brainsci10030156 www.mdpi.com/journal/brainsci
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