title: Familial motor neuron disease with Lewy body-like inclusions in the substantia nigra, the subthalamic nucleus, and the globus pallidus.
abstract: 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.
authors.affiliation: Service de Neurologie II, C.H.U., Strasbourg, France.
authors.forename: C
authors.initials: C
authors.lastname: Tranchant
authors.forename: M H
authors.initials: MH
authors.lastname: Dugay
authors.forename: M
authors.initials: M
authors.lastname: Mohr
authors.forename: P
authors.initials: P
authors.lastname: Wasser
authors.forename: J M
authors.initials: JM
authors.lastname: Warter
mesh_terms: 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
chemical_list: D014452:Ubiquitins