Metals in spinal cord tissue of patients dying of motor neuron disease

Abstract

To evaluate the role of toxic metals in causing motor neuron disease (MND), we used a photon-excited, energy-dispersive x-ray analytical system to measure the metal content of spinal ventral horn tissue. Specimens were taken from the cervical and lumbar enlargements of 7 patients who died of MND and the results compared with those found in 12 control patients. Anterior horn lead levels were elevated in MND patients compared to controls (mean, 40.7 micrograms/gm versus 14.6 micrograms/gm; p less than 0.05) and lead levels correlated with the duration of illness (r = +0.84, p less than 0.05). Only 2 MND patients had detectable manganese levels (72.3 and 132.2 micrograms/gm) whereas 1 control had detectable manganese (14.3 micrograms/gm). One MND patient had 244 micrograms/gm selenium, but 3 controls had levels of 180, 58, and 62. Patients with the histories of greatest environmental exposure to metals during life exhibited the highest tissue levels of metals after death; despite chelation therapy for about a year, high lead levels remained in their tissue.

Implementation of X-ray Fluorescence Microscopy for Investigation of Elemental Abnormalities in Amyotrophic Lateral Sclerosis

“…However, these results were in contradiction with the ones obtained by Khare et al [55]. Kurlander and Patten [54] found that in the anterior horn tissue the iron content was higher (P < 0.05) in seven ALS patients than in 11 controls with neurological and non-neurological diseases. Nagata et al [61] showed that the Mn concentrations in blood cells from ALS patients were significantly lower than from controls (non-ALS subjects).…”

“…Dysmetabolism of Zn was also reported for ALS patients in spinal cord and blood. Zn was found to be either unchanged, decreased, or increased [52,[54][55][56]. Kapaki et al [30] has found decreased levels of Cu in CSF, whilst Zn, Mn and Mg levels were unchanged.…”

“…Increased spinal cord calcium concentrations were reported in 6 out of 16 cases of Guamanian ALS [67]. Additionally, Ca has been found in hippocampal neurofibrillary tangles in Guamanian ALS patients [68] but not in classical ALS [54]. Moreover, it is known that chronic dietary deficiency (since birth) in Ca, Mg and Zn induces excessive absorption of divalent cations which accelerate oxidant-mediated neuronal degeneration in a genetically susceptible population.…”

ALSUntangled 43: copper

“…One study suggests that people with sporadic ALS also have elevated levels of copper (and other metals) in the motor area of their spinal cords (20). However, a small human trial of a copper chelator showed no benefit (21).…”

127th ENMC International Workshop: Implementation of a European Registry of ALS Naarden, The Netherlands, 8–10 October 2004

“…Lead can cause peripheral neuropathy and encephalopathy in humans, with a dose-effect relationship existing between blood levels and clinical findings [19]. Lead concentrations are elevated in the anterior horn cells of ALS patients previously exposed to high levels of lead [20] and may cause upper motor neuron degeneration in predisposed individuals.…”