Abstract
Objectives
Sleep disorders, especially restless legs syndrome (RLS) and rapid eye movement sleep
behavior disorder (RBD), are common in spinocerebellar ataxia type 3 or Machado–Joseph
disease (MJD), and a possible underlying dopaminergic dysfunction is implicated. This
study assessed the relationship between sleep disorders in MJD and dopamine transporter
(DAT) densities.
Patients and methods
Twenty-two patients with MJD and twenty healthy subjects were enrolled in this study.
MJD patients underwent clinical sleep evaluation and polysomnography. SPECT with [99mTc]-TRODAT-1, was performed in all subjects.
Results
DAT densities were significantly reduced in MJD group when compared to controls. No
significant correlation was found between DAT densities and RLS or RBD in MJD.
Conclusion
Our study failed to demonstrate a clear correlation between sleep disorders and DAT
densities in MJD patients, hence suggesting that extrastriatal and non-presynaptic
dopamine pathways could be implicated in MJD-related sleep disorders.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.Lancet Neurol. 2004; 3: 291-304
- New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado Joseph disease).Curr Opin Neurol. 2008; 21: 111-116
- Caring for Machado–Joseph disease: current understanding and how to help patients.Parkinsonism Relat Disord. 2010; 16: 2-7
- Clinical correlates of olfactory dysfunction in spinocerebellar ataxia type 3.Parkinsonism Relat Disord. 2011; 17: 353-356
- Sleep disorders in cerebellar ataxias.Arq Neuropsiquiatr. 2011; 69: 253-257
- Sleep symptoms and their clinical correlates in Machado–Joseph Disease.Acta Neurol Scand. 2009; 119: 277-280
- Spinocerebellar ataxia type 1, 2 and 3 and restless legs syndrome: striatal dopamine D2 receptor status investigated by [11C]Raclopride positron emission tomography.Mov Disord. 2006; 21: 1667-1673
- REM sleep behavior disorder and vocal cord paralysis in Machado–Joseph disease.Mov Disord. 2003; 18: 1179-1183
- Sleep disorders in Machado–Joseph disease: frequency, discriminative thresholds, predictive values, and correlation with ataxia-related motor and non-motor features.Cerebellum. 2011; 10: 291-295
- Is neuropathy involved with restless legs syndrome in Machado–Joseph disease?.Eur Neurol. 2011; 66: 200-203
- Evaluation of patients with Clinically Unclear Parkinsonian Syndromes submitted to brain SPECT imaging using the technetium-99m labeled tracer TRODAT-1.J Neurol Sci. 2010; 291: 64-68
- Clinical acceptance of a molecular imaging agent: a long march with [99mTc] TRODAT.Nucl Med Biol. 2007; 34: 787-789
- Decreased dopamine transporter binding in Machado–Joseph disease.J Nucl Med. 2000; 41: 994-998
- Dopamine transporter concentration is reduced in asymptomatic Machado–Joseph disease gene carriers.J Nucl Med. 2002; 43: 153-159
- The REM sleep behavior disorder screening questionnaire — a new diagnostic instrument.Mov Disord. 2007; 22: 2386-2393
- Translation and validation into the Brazilian Portuguese of the restless legs syndrome rating scale of the International Restless Legs Syndrome Study group.Arq Neuropsiquiatr. 2008; 66: 832-836
- Restless Legs Syndrome Diagnosis and Epidemiology workshop at the National Institutes of Health; International Restless Legs Syndrome Study Group. Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology. A report from the restless syndrome diagnosis and epidemiology workshop at the National Institutes of Health.Sleep Med. 2003; 4: 101-119
- A manual of standardized terminology, techniques and scoring system for sleep stages of human subjects.Brain information service, Brain Research Institute, Los Angeles1968
- Recording and scoring leg movements.Sleep. 1993; 16 ([Guilleminault C, chairman]): 748-759
- Serial dopamine transporter imaging of nigrostriatal function in patients with idiopathic rapid-eye-movement sleep behaviour disorder: a prospective study.Lancet Neurol. 2011; 10: 797-805
- The dopamine transporter is decreased in the striatum of subjects with restless legs syndrome.Sleep. 2011; 34: 341-347
- Sleep disturbance in spinocerebellar ataxias: is the SCA3 mutation a cause of restless legs syndrome?.Neurology. 1998; 51: 1603-1607
- Movement disorders in spinocerebellar ataxias.Mov Disord. 2011; 26: 792-800
- Transcranial sonography findings in spinocerebellar ataxia type 3 (Machado–Joseph disease): a cross-sectional study.Neurosci Lett. 2011; 504: 98-101
- New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado–Joseph disease).Curr Opin Neurol. 2008; 21: 111-116
- Positron emission tomography (PET) in Machado–Joseph disease.J Neurol Sci. 1997; 145: 63-67
- Striatal dopamine function in a family with multiple SCA-3 phenotypes.J Neurol. 2011; 258: 308-310
Article info
Publication history
Published online: November 08, 2012
Accepted:
October 12,
2012
Received in revised form:
October 12,
2012
Received:
July 14,
2012
Footnotes
☆Protection of Human Research Subjects and Human Subject Informed Consent: The procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national). All subjects signed the approved informed consent form.
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
