Familial motor neuron disease with Lewy body-like inclusions in the substantia nigra, the subthalamic nucleus, and the globus pallidus.
In a familial case of motor neuron disease (MND), 2 unusual features were noted in the necropsy. The first was a pallidoluysonigral degeneration, observed in only 4 other cases of MND and which was here asymptomatic. The second was the presence in degenerated spinal cord anterior horns and in degenerated basal ganglia of neuronal Lewy body-like inclusions stained by antibodies against ubiquitin.
Tranchant
C
C
Service de Neurologie II, C.H.U., Strasbourg, France.
Dugay
M H
MH
Mohr
M
M
Wasser
P
P
Warter
J M
JM
D005799:Genes, Dominant; D005911:Gliosis / Q000209:etiology / Q000473:pathology; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D016631:Lewy Bodies / Q000737:chemistry; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000235:genetics* / Q000473:pathology; D009410:Nerve Degeneration; D010375:Pedigree; D013116:Spinal Cord / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology*; D014452:Ubiquitins / Q000032:analysis
D014452:Ubiquitins