[A case of neuro-Behçet's disease with numerous hypertrophied and proliferated dendrites in the inferior olivary nucleus].
A 32-year-old man suffering from iritis, erythema, and recurrent oral aphthae showed signs of involvement of the central nervous system for 8 years before his death. Behçet disease was diagnosed. He showed neurologic symptoms, such as facial nerve palsy, gait disturbance, and pathological reflexes, and psychiatric symptoms, such as euphoria, disinhibition, and irritability. Characteristic neuropathologic findings were hypertrophy of the bilateral olivary nuclei with marked hypertrophy and proliferation of dendrites, which have been extremely rare in the reported autopsy cases of neuro-Behçet disease. Most changes were observed in the brainstem. Many microspongionecrotic foci were fused to from glial scars with marked lymphocytic perivascular infiltration. In addition, lesions were found throughout the spinal cord; degenerative changes were found in the lateral corticospinal tract, posterior colum (especially the fasciculus gracilis), and anterior and posterior spinocerebellar tracts. We believe, on the basis of the following neuropathologic findings, that the cause of the olivary pseudohypertrophy with dendritic hypertrophy and proliferation was a denervational process: (1) bilateral red nuclei were intact and (2) bilateral tegmental tracts had degenerated from the lower portion of the pons to the periphery of olivaly nuclei.
Inagaki
T
T
Department of Psychiatry, Shimane Medical University, Izumo, Japan.
Ishino
H
H
Seno
H
H
Iijima
M
M
Fujimoto
A
A
Manou
M
M
Harada
T
T
D001528:Behcet Syndrome / Q000473:pathology*; D003712:Dendrites / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D006984:Hypertrophy; D008297:Male; D008875:Middle Aged; D009847:Olivary Nucleus / Q000473:pathology*; D013681:Tegmentum Mesencephali / Q000473:pathology