Pallido-nigral spheroids associated with iron deposition have been observed in some aged clinically normal nonhuman primates. In humans, similar findings are observed in neurodegeneration with brain iron accumulation diseases, which, in some cases, show associated mutations in pantothenate kinase 2 gene (PANK2). Here we present an aged gorilla, 40 years old, suffering during the last 2 years of...

OBJECTIVE Iron may contribute to the pathogenesis and progression of multiple sclerosis (MS) due to its accumulation in the human brain with age. Our study focused on nonheme iron distribution and the expression of the iron-related proteins ferritin, hephaestin, and ceruloplasmin in relation to oxidative damage in the brain tissue of 33 MS and 30 control cases. METHODS We performed (1) whole-...

The accumulation of iron in the brain is a common physiological process. However, alterations in the deposition of iron or other paramagnetic substances are associated with various diseases. In the present study, the deposition of paramagnetic substances in patients with brain tumours was evaluated using T2 relaxometry. A total of 23 patients with untreated tumours or with recurrent tumours fol...

BACKGROUND Increased iron deposition has been implicated in the pathophysiology of multiple sclerosis (MS), based on visual analysis of signal reduction on T(2)-weighted images. R(2)* relaxometry allows to assess brain iron accumulation quantitatively. OBJECTIVE To investigate regional brain iron deposition in patients with a clinically isolated syndrome (CIS) or relapsing-remitting MS (RRMS)...

The brain iron overload disorders, collectively known as neurodegeneration with brain iron accumulation (NBIA), would seem to be tantalizing targets for chelation therapy. Efforts to lower brain iron in these disorders date back to the 1960s with deferoxamine mesylate, but systemic iron depletion limited its use. With the advent of chelators better able to specifically relieve central nervous s...

Mutations in WDR45 gene, coding for a beta-propeller protein, have been found in patients affected by Neurodegeneration with Brain Iron Accumulation, NBIA5 (also known as BPAN). BPAN is a movement disorder with Non Transferrin Bound Iron (NTBI) accumulation in the basal ganglia as common hallmark between NBIA classes (Hayflick et al., 2013). WDR45 has been predicted to have a role in autophagy,...

The aim of the present study was to analyze brain and body iron levels among patients with mild cognitive impairment (MCI), Alzheimer's disease (AD), and normal controls (NCs). A total of 90 participants (30 MCI, 30 AD, 30 NC) were enrolled. Brain iron content was quantified using susceptibility weighted imaging (SWI). Phase values were used to calculate bilateral iron content in the hippocampu...

Background: Clinical studies have shown that disturbance of iron homeostasis in ischemic stroke influenced recovery and functional outcome following blood reperfusion of obstructed cerebral vessels, but the mechanism of iron-mediated toxicity in ischemic strokes has not been fully elucidated. Methods: A middle cerebral artery occlusion (MCAO) mouse model was established by introducing an embolu...

BACKGROUND AND PURPOSE Previous studies have suggested that delayed release of hemoglobin degradation products, particularly iron, is involved in intracerebral hemorrhage (ICH)-induced brain injury. However, a recent study found evidence of iron-induced brain injury soon after ICH. This study, therefore, examined whether another iron-containing component of blood, holo-transferrin (holo-Tf), mi...

hallervorden-spatz syndrome is a disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. the disease is caused by mutations in gene encoding pantothenate kinase 2 (pank2) and patients have pantothenate kinase-associated neurodegeneration. we present an 8-year-old boy with progressive muscle dystonia, neuroregression, frequent fall and multiple injury marks of diffe...