Behr syndrome: A clinicopathologic report

Dikran S. Horoupian, David K. Zucker, Solomon L. Moshe, Hart De C Peterson

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)323-327
Number of pages5
JournalNeurology
Volume29
Issue number3
StatePublished - 1979

Fingerprint

Gliosis
Autopsy
Neuroaxonal Dystrophies
Geniculate Bodies
Thalamic Nuclei
Neuropil
Optic Nerve
Atrophy
Neurons
Behr syndrome
Nucleus
Syndrome
Neuron
Nerve
Manifestation
Drop out
Sister
Cells
Optics

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

Cite this

Horoupian, D. S., Zucker, D. K., Moshe, S. L., & Peterson, H. D. C. (1979). Behr syndrome: A clinicopathologic report. Neurology, 29(3), 323-327.

Behr syndrome : A clinicopathologic report. / Horoupian, Dikran S.; Zucker, David K.; Moshe, Solomon L.; Peterson, Hart De C.

In: Neurology, Vol. 29, No. 3, 1979, p. 323-327.

Research output: Contribution to journalArticle

Horoupian, DS, Zucker, DK, Moshe, SL & Peterson, HDC 1979, 'Behr syndrome: A clinicopathologic report', Neurology, vol. 29, no. 3, pp. 323-327.
Horoupian DS, Zucker DK, Moshe SL, Peterson HDC. Behr syndrome: A clinicopathologic report. Neurology. 1979;29(3):323-327.
Horoupian, Dikran S. ; Zucker, David K. ; Moshe, Solomon L. ; Peterson, Hart De C. / Behr syndrome : A clinicopathologic report. In: Neurology. 1979 ; Vol. 29, No. 3. pp. 323-327.
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