TY - JOUR
T1 - Behr syndrome
T2 - A clinicopathologic report
AU - Horoupian, Dikran S.
AU - Zucker, David K.
AU - Moshe, Solomon
AU - Peterson, Hart De C.
PY - 1979/3
Y1 - 1979/3
N2 - Two sisters had Behr syndrome; autopsy was performed on one. The autopsy revealed central atrophy of the optic nerves and total disarray of the normal laminar pattern of the lateral geniculate nucleus, dropout of neurons, and gliosis. There were numerous axonal spheroids in the neuropil. Similar spheroids with cell loss and gliosis were also observed in other thalamic nuclei and, rarely, in the pallida. We raise the possibility that Behr syndrome is a manifestation of a heterogeneous group of disorders, and suggest relationship of this particular case to infantile neuroaxonal dystrophy.
AB - Two sisters had Behr syndrome; autopsy was performed on one. The autopsy revealed central atrophy of the optic nerves and total disarray of the normal laminar pattern of the lateral geniculate nucleus, dropout of neurons, and gliosis. There were numerous axonal spheroids in the neuropil. Similar spheroids with cell loss and gliosis were also observed in other thalamic nuclei and, rarely, in the pallida. We raise the possibility that Behr syndrome is a manifestation of a heterogeneous group of disorders, and suggest relationship of this particular case to infantile neuroaxonal dystrophy.
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U2 - 10.1212/wnl.29.3.323
DO - 10.1212/wnl.29.3.323
M3 - Article
C2 - 571977
AN - SCOPUS:0018382833
SN - 0028-3878
VL - 29
SP - 323
EP - 327
JO - Neurology
JF - Neurology
IS - 3
ER -