Abstract | BACKGROUND: METHODS: The case is a male infant with MOCOD who died at nine months of age from pneumonia. At autopsy, a complete neuropathological examination was performed including conventional immunohistochemical staining. In addition, brain sections were stained cytochemically with shikata and orcein which stain for disulphide bonds. The elemental composition of cortical cells was then analyzed in the scanning electron microscope using backscatter electron imaging and energy dispersive X-ray spectrometry. RESULTS: Neurons demonstrated cytoplasmic staining with shikata and orcein cytochemically when compared to control sections. Energy dispersive X-ray spectrometry analysis of these neurons confirmed the presence of excess sulphur and unexpectedly revealed excess magnesium accumulation. None of these findings was found in an age-matched control. CONCLUSIONS: In MOCOD we found abnormal accumulation of sulphur and magnesium in neurons. It is postulated that sulphur-containing compound(s) that are formed as a result of MOCOD cause excitotoxic neuronal injury in the presence of excess magnesium.
|
Authors | Michael S Salman, Cameron Ackerley, Christof Senger, Laurence Becker |
Journal | The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
(Can J Neurol Sci)
Vol. 29
Issue 1
Pg. 91-6
(Feb 2002)
ISSN: 0317-1671 [Print] England |
PMID | 11858544
(Publication Type: Case Reports, Journal Article)
|
Chemical References |
- Coenzymes
- Metalloproteins
- Molybdenum Cofactors
- Pteridines
- Sulfur
- molybdenum cofactor
- Oxidoreductases Acting on Sulfur Group Donors
- Magnesium
|
Topics |
- Autopsy
- Coenzymes
- Humans
- Immunohistochemistry
- Infant
- Magnesium
(analysis)
- Male
- Metalloproteins
(deficiency)
- Molybdenum Cofactors
- Neurodegenerative Diseases
(metabolism, pathology)
- Oxidoreductases Acting on Sulfur Group Donors
(deficiency)
- Pteridines
- Spectrometry, X-Ray Emission
- Sulfur
(analysis)
|