TY - JOUR
T1 - Ultrastructure of thalamic neuronal inclusions in myotonic dystrophy
AU - Wiśniewski, Henryk M.
AU - Berry, Kenneth
AU - Spiro, Alfred J.
N1 - Funding Information:
This investigation was supported in part by NIH grants NS 08180, NS 02255 and NS 03356, a grant from the Alfred P. Sloan Foundation, and a Clinical Service Grant from the Muscular Dystrophy Associations of America, Inc. Reprint requests to: Dr. Henryk M. Wi~niewski, Department of Pathology (Neuropathology), Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, N.Y. 10461, U.S.A. * Present address: Medical Research Council, Demyelinating Diseases Unit, General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, Great Britain.
PY - 1975/3
Y1 - 1975/3
N2 - Recently, the presence of thalamic neuronal cytoplasmic inclusions in patients with myotonic dystrophy has been reported. At the ultrastructural level, the inclusions were described as "containing a fibrillar material within a limiting membrane studded on its outer surface with ribosomes". We have studied the brain of a 48-year-old woman with myotonic dystrophy. Many neuronal inclusion bodies were found within the thalamus, and examined in the electron microscope. Inclusions were found to have an electron density resembling that of nuclear chromatin, were not membrane-bound, and had an internal structure composed of parallel alternating dark and pale lines. We confirm the previously-reported existence of cytoplasmic thalamic neuronal inclusions in myotonic dystrophy, but differ in our observations of their ultrastructural appearance, and note that these inclusions bear no resemblance to previously-described inclusion bodies.
AB - Recently, the presence of thalamic neuronal cytoplasmic inclusions in patients with myotonic dystrophy has been reported. At the ultrastructural level, the inclusions were described as "containing a fibrillar material within a limiting membrane studded on its outer surface with ribosomes". We have studied the brain of a 48-year-old woman with myotonic dystrophy. Many neuronal inclusion bodies were found within the thalamus, and examined in the electron microscope. Inclusions were found to have an electron density resembling that of nuclear chromatin, were not membrane-bound, and had an internal structure composed of parallel alternating dark and pale lines. We confirm the previously-reported existence of cytoplasmic thalamic neuronal inclusions in myotonic dystrophy, but differ in our observations of their ultrastructural appearance, and note that these inclusions bear no resemblance to previously-described inclusion bodies.
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U2 - 10.1016/0022-510X(75)90252-X
DO - 10.1016/0022-510X(75)90252-X
M3 - Article
C2 - 46919
AN - SCOPUS:0016606842
SN - 0022-510X
VL - 24
SP - 321
EP - 329
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 3
ER -