TY - JOUR
T1 - Neuropathologic findings in an aged albino Gorilla
AU - Pumarola Batlle, Marti
AU - Fernández Bellon, Hugo Manuel
AU - Serafin Canals, Anna Maria
AU - Márquez, M.
AU - Serrat, S.
AU - Ferrer-Admetlla, A.
AU - Bertranpetit, J.
AU - Ferrer, I.
PY - 2008/7/1
Y1 - 2008/7/1
N2 - 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 life from progressive tetraparesis, nystagmus, and dyskinesia of the arms, hands, and neck, with accompanying abnormal behavior. The postmortem neuropathologic examination revealed, in addition to aging-associated changes in the brain, numerous corpora amylacea in some brain areas, especially the substantia nigra, and large numbers of axonal spheroids associated with iron accumulation in the internal globus pallidus. Sequencing of the gorilla PANK2 gene failed to detect any mutation. The clinical, neuropathologic, and genetic findings in this gorilla point to an age-related pallido-nigral degeneration that presented PKAN-like neurologic deficits.
AB - 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 life from progressive tetraparesis, nystagmus, and dyskinesia of the arms, hands, and neck, with accompanying abnormal behavior. The postmortem neuropathologic examination revealed, in addition to aging-associated changes in the brain, numerous corpora amylacea in some brain areas, especially the substantia nigra, and large numbers of axonal spheroids associated with iron accumulation in the internal globus pallidus. Sequencing of the gorilla PANK2 gene failed to detect any mutation. The clinical, neuropathologic, and genetic findings in this gorilla point to an age-related pallido-nigral degeneration that presented PKAN-like neurologic deficits.
KW - Axonal dystrophy
KW - Gorilla
KW - Iron
KW - Neurodegeneration with brain iron accumulation
KW - Nonhuman primate
KW - Pallido-nigral degeneration
KW - Pantothenate kinase-associated neurodegeneration
U2 - https://doi.org/10.1354/vp.45-4-531
DO - https://doi.org/10.1354/vp.45-4-531
M3 - Article
VL - 45
SP - 531
EP - 537
JO - Veterinary Pathology
JF - Veterinary Pathology
SN - 0300-9858
ER -