Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination

Abstract

Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination.

Restless Legs Syndrome Presenting as an Acute Exacerbation of Multiple Sclerosis

“…However, CSF studies have demonstrated normal CSF ferritin levels in MS [19]. Furthermore, iron appears to be an important cofactor in CNS myelination, and van Toorn et al have reported two cases of iron deficiency associated with tumefactive demyelination in children [20–22]. …”

Iron and Neurodegeneration in Multiple Sclerosis

“…Conversely, analyses of iron status in two children with recurrent episodes of tumefactive cerebral demyelination revealed decreased serum iron and ferritin and constant iron supplementation was needed to prevent an iron deficiency state in both children [70]. …”

“…However, a clinical trial testing the iron chelating drug desferrioxamine in chronic progressive MS patients failed to demonstrate any effects on disease progression [76]. A recent observation revealed that supplementing nonanaemic iron deficiency in two children with recurrent episodes of tumefactive demyelination leads to sustained remission [70]. …”

Assessing Abnormal Iron Content in the Deep Gray Matter of Patients with Multiple Sclerosis versus Healthy Controls

“…23 They found no difference between the MS population and the control group. Another study 24 noted that iron deficiency was reported in the MS group studied, and iron supplementation was prescribed to these patients. Evidently, this treatment resulted in a partial recovery of their symptoms.…”