- Email: [email protected]
The above letter was shown to Dr. Ono and coworkers, whose reply is printed below
Journal of the Neurological Sciences, 1988, 85:239-240
239
Elsevier
Letters to the Editors
Dear Editors,
The article by Ono et al. (1987) concludes that the intracytoplasmic eosinophilic neuronal inclusion bodies observed in various areas of the brain represent a specific feature of the disease. It is our experience, based on a systematic study of a large number of non-pathologic brains, that such inclusion bodies are found in the neurons of the thalamus of virtually all adults (Pefla 1980), as well as in the substantia nigra, and to a lesser extent in the locus ceruleus and anterior horns of the spinal cord (Pefia 1981). These observations agree with those of Schochet et al. (1970) for the substantia nigra. The inclusions have a rather characteristic free structure and are composed of thin filaments measuring 5.5-6.0 nm in thickness, arranged in a crystalloid pattern. These observations suggest that the inclusion bodies are a constant finding in the adult human brain and are not specific for any one disease. Their significance is not completely clear at the present time but it is suggested that they represent an adaptation of neuronal cytoskeletal proteins.
REFERENCES Ono, S., K. Inoue, T. Mannen, F. Kanda, K. Jinnai and K. Takahashi (1987) Neuropathologieal changes of the brain in myotonic dystrophy - some new observations. J. Neurol. Sci., 81: 301-320. Pefia, C.E. (1980) Intracytoplasmic neuronal inclusions in the human thalamus. Light-microscopic,histochemical, and ultrastructural observations.Acta Neuropathol. (Berl.), 52: 157-159. Pefia, C. E. (1981)Intracytoplasmicneuronal inclusions in the central nervous system:an unrecognizedform of neuronal protein storage. Lightmicroscopic,histochemicaland ultrastructural study,d. Neuropathol. Exp. Neurol., 40:311 (Abstract). Schochet, S.S., R.B. Wyatt and W.F. McCormick (1970) Intraeytoplasmie aeidophilie granules in the substantia nigra. A light and electron microscopic study. Arch. Neurol., 22: 550-555. Department of Pathology, School of Medicine, University of Pittsburgh, 3600 Ridgewood Drive, Pittsburgh, PA 15235 (U.S.A.)
CARLOSE. PE~A
(Received 30 December, 1987) (Accepted 22 January, 1988)
The above letter was shown to Dr. Ono and coworkers, whose reply is printed below: We appreciate the interest of Dr. Pe~ta in our recent paper (Ono et al. 1987). We regret the inadvertent omission of a reference to his paper (Peila 1981).
240 Our paper (Ono et al. 1987) emphasized two points: (1)intracytoplasmic inclusion bodies of the thalamus (Culebras et al. 1973; Wi~niewski et al. 1975; Pefla 1980) and the substantia nigra (Schochet et al. 1970), and Marinesco bodies, observed in controls, were encountered with a far higher frequency in patients with myotonic dystrophy (MyD) than in controls; (2) the presence, in combination, of these inclusion bodies in cerebral cortex, putamen, caudate nucleus, thalamus and substantia nigra, the first three of which have not yet been described in the literature and were not observed in controls, and of Marinesco bodies was the most conspicuous finding in our cases. Therefore, we do not state that these inclusion bodies observed in various areas of the brain in our cases represent a specific feature of the disease. We entirely agree with the claim by Dr. Petla that such inclusion bodies in the neurons of the thalamus and the substantia nigra are a constant fmding in the adult brain and are not specific for any one disease. Although we are uncertain whether there is any relationship between the pathological findings and the etiology and pathogenesis of MyD, we believe that further study of the findings noted in our work may yield information on the characteristics of pathology of the brain in MyD.
REFERENCES Culebras, A., R.G. Feldman and F.B. Meak (1973) Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. A light and electron microscope study. J. Neurol. Sci.~ 19: 319-329. Ono, S., K. Inoue, T. Mannen, F. Kanda, K. Jinnai and K. Takahashi (1987) Neuropathological changes of the brain in myotonic dystrophy - some new observations. J. Neurol. Sci., 81: 301-320. Pefia, C.E. (1980) Intracytoplasmic neuronal inclusions in the human thalamus. Light-microscopic, histochemical, and ultrastructural observations. Acta NeuropathoL (Berl.), 52: 157-159. Pefia, C. E. (1981) Intracytoplasmic neuronal inclusions in the central nervous system: an unrecognized form of neuronal protein storage. Light microscopic, histoehemical and ultrastruetural study. J. Neuropathol. Exp. Neurol., 40:311 (Abstr.). Schochet, S.S., R.B. Wyatt and W.F. McCormick (1970) Intracytoplasmic acidophilic granules in the substantia nigra. A light and electron microscopic study. Arch. NeuroL, 22: 550-555. Wi~niewski, H.M., K. Berry and A.J. Spiro (1975) Ultrastrueture of thalamic neuronal inclusions in myotonic dystrophy. J. Neurol. Sci., 24: 321-329.
IDepartment of Neurology, Teikyo University School of Medicine, Ichihara Hospital 3426-3, Anesaki, Ichihara City, Chiba 299-01 (Japan), and 2Department of Neurology, Institute of Brain Research, Faculty of Medicine, University of Tokyo, 7-3-l, Hongo, Bunkyo-ku, Tokyo 113 (Japan) (Received 22 January, 1988) (Accepted 25 January, 1988)
S. ONO I, K. INOUE2 AND T. MANNEN2
239
Elsevier
Letters to the Editors
Dear Editors,
The article by Ono et al. (1987) concludes that the intracytoplasmic eosinophilic neuronal inclusion bodies observed in various areas of the brain represent a specific feature of the disease. It is our experience, based on a systematic study of a large number of non-pathologic brains, that such inclusion bodies are found in the neurons of the thalamus of virtually all adults (Pefla 1980), as well as in the substantia nigra, and to a lesser extent in the locus ceruleus and anterior horns of the spinal cord (Pefia 1981). These observations agree with those of Schochet et al. (1970) for the substantia nigra. The inclusions have a rather characteristic free structure and are composed of thin filaments measuring 5.5-6.0 nm in thickness, arranged in a crystalloid pattern. These observations suggest that the inclusion bodies are a constant finding in the adult human brain and are not specific for any one disease. Their significance is not completely clear at the present time but it is suggested that they represent an adaptation of neuronal cytoskeletal proteins.
REFERENCES Ono, S., K. Inoue, T. Mannen, F. Kanda, K. Jinnai and K. Takahashi (1987) Neuropathologieal changes of the brain in myotonic dystrophy - some new observations. J. Neurol. Sci., 81: 301-320. Pefia, C.E. (1980) Intracytoplasmic neuronal inclusions in the human thalamus. Light-microscopic,histochemical, and ultrastructural observations.Acta Neuropathol. (Berl.), 52: 157-159. Pefia, C. E. (1981)Intracytoplasmicneuronal inclusions in the central nervous system:an unrecognizedform of neuronal protein storage. Lightmicroscopic,histochemicaland ultrastructural study,d. Neuropathol. Exp. Neurol., 40:311 (Abstract). Schochet, S.S., R.B. Wyatt and W.F. McCormick (1970) Intraeytoplasmie aeidophilie granules in the substantia nigra. A light and electron microscopic study. Arch. Neurol., 22: 550-555. Department of Pathology, School of Medicine, University of Pittsburgh, 3600 Ridgewood Drive, Pittsburgh, PA 15235 (U.S.A.)
CARLOSE. PE~A
(Received 30 December, 1987) (Accepted 22 January, 1988)
The above letter was shown to Dr. Ono and coworkers, whose reply is printed below: We appreciate the interest of Dr. Pe~ta in our recent paper (Ono et al. 1987). We regret the inadvertent omission of a reference to his paper (Peila 1981).
240 Our paper (Ono et al. 1987) emphasized two points: (1)intracytoplasmic inclusion bodies of the thalamus (Culebras et al. 1973; Wi~niewski et al. 1975; Pefla 1980) and the substantia nigra (Schochet et al. 1970), and Marinesco bodies, observed in controls, were encountered with a far higher frequency in patients with myotonic dystrophy (MyD) than in controls; (2) the presence, in combination, of these inclusion bodies in cerebral cortex, putamen, caudate nucleus, thalamus and substantia nigra, the first three of which have not yet been described in the literature and were not observed in controls, and of Marinesco bodies was the most conspicuous finding in our cases. Therefore, we do not state that these inclusion bodies observed in various areas of the brain in our cases represent a specific feature of the disease. We entirely agree with the claim by Dr. Petla that such inclusion bodies in the neurons of the thalamus and the substantia nigra are a constant fmding in the adult brain and are not specific for any one disease. Although we are uncertain whether there is any relationship between the pathological findings and the etiology and pathogenesis of MyD, we believe that further study of the findings noted in our work may yield information on the characteristics of pathology of the brain in MyD.
REFERENCES Culebras, A., R.G. Feldman and F.B. Meak (1973) Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. A light and electron microscope study. J. Neurol. Sci.~ 19: 319-329. Ono, S., K. Inoue, T. Mannen, F. Kanda, K. Jinnai and K. Takahashi (1987) Neuropathological changes of the brain in myotonic dystrophy - some new observations. J. Neurol. Sci., 81: 301-320. Pefia, C.E. (1980) Intracytoplasmic neuronal inclusions in the human thalamus. Light-microscopic, histochemical, and ultrastructural observations. Acta NeuropathoL (Berl.), 52: 157-159. Pefia, C. E. (1981) Intracytoplasmic neuronal inclusions in the central nervous system: an unrecognized form of neuronal protein storage. Light microscopic, histoehemical and ultrastruetural study. J. Neuropathol. Exp. Neurol., 40:311 (Abstr.). Schochet, S.S., R.B. Wyatt and W.F. McCormick (1970) Intracytoplasmic acidophilic granules in the substantia nigra. A light and electron microscopic study. Arch. NeuroL, 22: 550-555. Wi~niewski, H.M., K. Berry and A.J. Spiro (1975) Ultrastrueture of thalamic neuronal inclusions in myotonic dystrophy. J. Neurol. Sci., 24: 321-329.
IDepartment of Neurology, Teikyo University School of Medicine, Ichihara Hospital 3426-3, Anesaki, Ichihara City, Chiba 299-01 (Japan), and 2Department of Neurology, Institute of Brain Research, Faculty of Medicine, University of Tokyo, 7-3-l, Hongo, Bunkyo-ku, Tokyo 113 (Japan) (Received 22 January, 1988) (Accepted 25 January, 1988)
S. ONO I, K. INOUE2 AND T. MANNEN2