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NEUROLOGY 2004;63:1258-1263
© 2004 American Academy of Neurology

Damage to the reticulotegmental nucleus of the pons in spinocerebellar ataxia type 1, 2, and 3

U. Rüb, MD, K. Bürk, MD, L. Schöls, MD, E. R. Brunt, MD, R. A.I. de Vos, MD, G. Orozco Diaz, MD, K. Gierga, E. Ghebremedhin, MD, C. Schultz, MD, D. Del Turco, PhD, M. Mittelbronn, MD, G. Auburger, MD, T. Deller, MD and H. Braak, MD

From the Institute for Clinical Neuroanatomy (Drs. Rüb, Ghebremedhin, Schultz, Del Turco, Deller, and Braak, K. Gierga), J.W. Goethe University, Frankfurt am Main, Germany; Department of Neurology (Dr. Bürk), University of Ulm, Germany; Department of Neurology (Dr. Schöls), St. Josef Hospital, Ruhr-University Bochum, Germany; Department of Neurology (Dr. Brunt), University Hospital, Groningen, The Netherlands; Laboratorium Pathologie Oost Nederland (Dr. de Vos), Enschede, The Netherlands; Department of Neurology (Dr. Orozco Diaz), V.I. Lenin Hospital, Holguín, Cuba; Institute of Brain Research (Dr. Mittelbronn), University of Tübingen, Germany; and Section Molecular Neurogenetics (Dr. Auburger), Department of Neurology, J.W. Goethe University, Frankfurt am Main, Germany.

Address correspondence and reprint requests to Dr. Udo Rüb, Institute for Clinical Neuroanatomy, J.W. Goethe University, Theodor-Stern-Kai 7, D-60590 Frankfurt am Main, Germany; e-mail: U.Rueb{at}em.uni-frankfurt.de

Background: The reticulotegmental nucleus of the pons (RTTG) is among the precerebellar nuclei of the human brainstem. Although it represents an important component of the oculomotor circuits crucial for the accuracy of horizontal saccades and the generation of horizontal smooth pursuits, the RTTG has never been considered in CAG repeat or polyglutamine diseases.

Methods: Thick serial sections through the RTTG of 10 patients with spinocerebellar ataxias (SCAs) assigned to the CAG repeat or polyglutamine diseases (2 SCA-1 patients, 4 SCA-2 patients, and 4 SCA-3 patients) were stained for neuronal lipofuscin pigment and Nissl material.

Results: The unconventionally thick tissue sections revealed the hitherto overlooked involvement of the RTTG in the degenerative processes underlying SCA-1, SCA-2, and SCA-3, whereby in one of the SCA-1 patients, in two of the SCA-2 patients, and in all of the SCA-3 patients, the RTTG underwent a conspicuous loss of its nerve cells.

Conclusions: Neurodegeneration may not only affect the cranial nerve nuclei (i.e., oculomotor and abducens nuclei) of SCA-1, SCA-2 and SCA-3 patients integrated into the circuits, subserving accuracy of horizontal saccades and the generation of horizontal smooth pursuits, but likewise involves the premotor networks of these circuits. This may explain why the SCA-1, SCA-2, and SCA-3 patients in this study with a heavily damaged reticulotegmental nucleus of the pons developed dysmetric horizontal saccades and impaired smooth pursuits during the course of the disease.

Received January 8, 2004. Accepted in final form June 1, 2004.






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