| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the Department of Neurology (Drs. Gilman and Junck, M. Heumann, and K.J. Kluin), Department of Biostatistics (Dr. Little, J. Johanns, and H. An), Department of Physical Medicine and Rehabilitation (K.J. Kluin), and Division of Nuclear Medicine, Department of Internal Medicine (Dr. Koeppe), University of Michigan, Ann Arbor, Michigan.
Address correspondence and reprint requests to Dr. Sid Gilman, Department of Neurology, University of Michigan Health System, 1500 E. Medical Center Dr., Ann Arbor, MI 48109-0316
OBJECTIVE: To determine the percentage of sporadic olivopontocerebellar atrophy (sOPCA) patients who later develop multiple system atrophy (MSA).
METHODS: Observations of the course of 51 sOPCA patients 20 years of age or older initially evaluated in an ataxia clinic over 14 years and followed at 3- to 6-month intervals for 3 months to 10 years (median 2.5 years, interquartile range 5 months to 4 years).
RESULTS: Seventeen patients evolved to develop MSA, whereas the remaining 34 manifested only progressively worsening cerebellar ataxia. The features of the MSA cases included autonomic failure and parkinsonism in 10 patients, autonomic failure without parkinsonism in six, and parkinsonism without autonomic failure in one. Using survival analysis methods, the authors estimated that 24% of subjects in this population will evolve to MSA within 5 years of the onset of sOPCA symptoms (95% CI 10% to 36%). An older age at onset of symptoms and a shorter time from onset of symptoms to first presentation in a neurology specialty clinic were both highly predictive of evolution to MSA. Six of the 17 patients who evolved to MSA died 4 months to 5 years after they had met diagnostic criteria for MSA. The estimated median survival time from time of transition was 3.5 years. In contrast, death occurred in only one of the 34 patients with sOPCA who did not evolve to MSA. Autopsy examination of all six patients with MSA who died confirmed the diagnosis.
CONCLUSIONS: Approximately one-fourth of sporadic olivopontocerebellar atrophy patients will evolve to multiple system atrophy within 5 years, and this transition carries a poor prognosis for survival. Older age at onset of ataxia and earlier presentation in a neurologic specialty clinic predicted transition to MSA.
This article has been cited by other articles:
|
|
 |
|
  G. Bensimon, A. Ludolph, Y. Agid, M. Vidailhet, C. Payan, P. N. Leigh, and for the NNIPPS Study Group Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study Brain, January 1, 2009; 132(1): 156 - 171. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. F. Abdo, B.P.C. van de Warrenburg, M. Munneke, W. J.A. van Geel, B. R. Bloem, H. P.H. Kremer, and M. M. Verbeek CSF analysis differentiates multiple-system atrophy from idiopathic late-onset cerebellar ataxia Neurology, August 8, 2006; 67(3): 474 - 479. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Burk, U. Buhring, J. B. Schulz, C. Zuhlke, Y. Hellenbroich, and J. Dichgans Clinical and Magnetic Resonance Imaging Characteristics of Sporadic Cerebellar Ataxia Arch Neurol, June 1, 2005; 62(6): 981 - 985. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M B Muzaimi, J Thomas, S Palmer-Smith, L Rosser, P S Harper, C M Wiles, D Ravine, and N P Robertson Population based study of late onset cerebellar ataxia in south east Wales J. Neurol. Neurosurg. Psychiatry, August 1, 2004; 75(8): 1129 - 1134. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K Deguchi, I Sasaki, T Touge, M Tsukaguchi, H Takeuchi, and S Kuriyama Abnormal baroreceptor-mediated vasopressin release as possible marker in early diagnosis of multiple system atrophy J. Neurol. Neurosurg. Psychiatry, January 1, 2004; 75(1): 110 - 115. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Burk, C. Globas, T. Wahl, U. Buhring, K. Dietz, C. Zuhlke, A. Luft, J. B. Schulz, K. Voigt, and J. Dichgans MRI-based volumetric differentiation of sporadic cerebellar ataxia Brain, January 1, 2004; 127(1): 175 - 181. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Nicholson, A. C. Pereira, and G. M. Hall Parkinson's disease and anaesthesia Br. J. Anaesth., December 1, 2002; 89(6): 904 - 916. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. A. Brunberg, S. Jacquemont, R. J. Hagerman, E. M. Berry-Kravis, J. Grigsby, M. A. Leehey, F. Tassone, W. T. Brown, C. M. Greco, and P. J. Hagerman Fragile X Premutation Carriers: Characteristic MR Imaging Findings of Adult Male Patients with Progressive Cerebellar and Cognitive Dysfunction AJNR Am. J. Neuroradiol., November 1, 2002; 23(10): 1757 - 1766. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Abele, K. Burk, L. Schols, S. Schwartz, I. Besenthal, J. Dichgans, C. Zuhlke, O. Riess, and T. Klockgether The aetiology of sporadic adult-onset ataxia Brain, May 1, 2002; 125(5): 961 - 968. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Watanabe, Y. Saito, S. Terao, T. Ando, T. Kachi, E. Mukai, I. Aiba, Y. Abe, A. Tamakoshi, M. Doyu, et al. Progression and prognosis in multiple system atrophy: An analysis of 230 Japanese patients Brain, May 1, 2002; 125(5): 1070 - 1083. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
