Daniel J. Loes
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View article: Neurofilament light chain as a prognostic marker in cerebral adrenoleukodystrophy
Neurofilament light chain as a prognostic marker in cerebral adrenoleukodystrophy Open
Cerebral adrenoleukodystrophy (C-ALD) is a rapidly progressing inflammatory neurodegenerative disease with unpredictable onset in males with ALD. It must be treated at an early stage of demyelination, determined by MRI, to preserve neuroco…
View article: Neurofilament light chain as a biomarker to indicate early activation of cerebral disease in boys with adrenoleukodystrophy
Neurofilament light chain as a biomarker to indicate early activation of cerebral disease in boys with adrenoleukodystrophy Open
As far as we are aware, this is the first report highlighting that a bloodborne biomarker, plasma NfL, could potentially be used as a concomitant indicator of cerebral disease initiation, thus simplifying surveillance for cerebral ALD.
View article: Diffusion Tensor Imaging in Boys With Adrenoleukodystrophy
Diffusion Tensor Imaging in Boys With Adrenoleukodystrophy Open
DTI was sensitive to lesion presence and severity as well as clinical neurocognitive effects of C-ALD. DTI metrics quantify C-ALD even at an early stage.
View article: Hematopoietic stem-cell gene therapy is associated with restored white matter microvascular function in cerebral adrenoleukodystrophy
Hematopoietic stem-cell gene therapy is associated with restored white matter microvascular function in cerebral adrenoleukodystrophy Open
Blood-brain barrier disruption marks the onset of cerebral adrenoleukodystrophy (CALD), a devastating cerebral demyelinating disease caused by loss of ABCD1 gene function. The underlying mechanism are not well understood, but evidence sugg…
View article: Variables affecting outcomes after allogeneic hematopoietic stem cell transplant for cerebral adrenoleukodystrophy
Variables affecting outcomes after allogeneic hematopoietic stem cell transplant for cerebral adrenoleukodystrophy Open
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) in early cerebral adrenoleukodystrophy can stabilize neurologic function and improve survival but has associated risks including transplant-related mortality (TRM), graft failu…
View article: Volume of Gadolinium Enhancement and Successful Repair of the Blood-Brain Barrier in Cerebral Adrenoleukodystrophy
Volume of Gadolinium Enhancement and Successful Repair of the Blood-Brain Barrier in Cerebral Adrenoleukodystrophy Open
Up to 40% of boys with adrenoleukodystrophy develop a severe central nervous system demyelinating form (cALD) characterized by white matter changes and gadolinium enhancement on magnetic resonance imaging (MRI). Hematopoietic cell transpla…
View article: Failure of intrathecal allogeneic mesenchymal stem cells to halt progressive demyelination in two boys with cerebral adrenoleukodystrophy
Failure of intrathecal allogeneic mesenchymal stem cells to halt progressive demyelination in two boys with cerebral adrenoleukodystrophy Open
Cerebral adrenoleukodystrophy is an inflammatory demyelinating condition that is the result of a mutation in the X-linked ABCD1 gene, a peroxisomal very long chain fatty acid transporter. Although mutations in this gene result in adrenal i…
View article: Childhood Cerebral Adrenoleukodystrophy: MR Perfusion Measurements and Their Use in Predicting Clinical Outcome after Hematopoietic Stem Cell Transplantation
Childhood Cerebral Adrenoleukodystrophy: MR Perfusion Measurements and Their Use in Predicting Clinical Outcome after Hematopoietic Stem Cell Transplantation Open
Leading enhancing edge measurements likely remain normal post-hematopoietic stem cell transplantation in cerebral adrenoleukodystrophy, suggesting local disease stabilization. Meanwhile, parieto-occipital WM and splenium of the corpus call…