TY - JOUR
T1 - Morphologic and elemental analysis of primary melanosis of the dentate nucleus
T2 - Review and correlation with neuromelanin
AU - Hopley, Richard T.
AU - Haller, Edward
AU - Rojiani, Mumtaz V.
AU - Rojiani, Amyn M.
N1 - Publisher Copyright:
© 2017 American Association of Neuropathologists, Inc. All rights reserved.
PY - 2017/11/1
Y1 - 2017/11/1
N2 - Primary melanosis of the dentate nucleus is a rarely described entity with neither known cause nor definitive clinicopathologic correlation. We revisit this previously reported phenomenon by presenting one such case with a review of the pathology as well as additional investigations including elemental analysis by energydispersive X-ray, immunohistochemistry and electron microscopy. The lesion presented macroscopically as a sharply defined, black pigmentation that was restricted to the dentate nucleus of the cerebellum. Other deep nuclei were uninvolved. Similarly, other areas of the cerebellum, brainstem, and supratentorial regions were macroscopically free of pigment. Microscopically, however, the pigment was noted to be present, albeit in microscopic deposits, within layers of the cerebellar cortex. Additionally, immunohistochemistry and electron microscopy defined an intracellular component within astrocytes. X-ray analysis of the pigment showed it to consist almost entirely of sulfur, an element known to be prominent in neuromelanin. This report also describes an association of the pigment with astrocytes by ultrastructural examination. We discuss the results of our findings in the context of etiopathogenetic considerations, seeking to gain a better understanding of this abnormal pigmentation and its relationship to neuromelanin.
AB - Primary melanosis of the dentate nucleus is a rarely described entity with neither known cause nor definitive clinicopathologic correlation. We revisit this previously reported phenomenon by presenting one such case with a review of the pathology as well as additional investigations including elemental analysis by energydispersive X-ray, immunohistochemistry and electron microscopy. The lesion presented macroscopically as a sharply defined, black pigmentation that was restricted to the dentate nucleus of the cerebellum. Other deep nuclei were uninvolved. Similarly, other areas of the cerebellum, brainstem, and supratentorial regions were macroscopically free of pigment. Microscopically, however, the pigment was noted to be present, albeit in microscopic deposits, within layers of the cerebellar cortex. Additionally, immunohistochemistry and electron microscopy defined an intracellular component within astrocytes. X-ray analysis of the pigment showed it to consist almost entirely of sulfur, an element known to be prominent in neuromelanin. This report also describes an association of the pigment with astrocytes by ultrastructural examination. We discuss the results of our findings in the context of etiopathogenetic considerations, seeking to gain a better understanding of this abnormal pigmentation and its relationship to neuromelanin.
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U2 - 10.1093/jnen/nlx084
DO - 10.1093/jnen/nlx084
M3 - Article
C2 - 29044415
AN - SCOPUS:85032168761
SN - 0022-3069
VL - 76
SP - 949
EP - 956
JO - Journal of neuropathology and experimental neurology
JF - Journal of neuropathology and experimental neurology
IS - 11
ER -