Measuring Neurological Impairment and Functional Visual Assessment In Spinocerebellar Ataxias

NCT00654251 | Completed

• 1 other identifier: 2007-0228
• Study Type: observational
• Target: 20
• Locations: 1 country
• Sites: 2

Brief Summary

Measuring the various difficulties patients with spinocerebellar ataxias (SCA) report in an accurate manner is important to be able to test any therapy that may be developed. As basic research identifies some therapy of this type, clinicians are planning studies that can either prove or disprove that such treatments actually have an effect. Walking problems and problems with eye movements that can give rise to visual complaints are common in the SCA's. Existing neurological scales such as the "SARA" are based on the usual neurological examination items that can carry a degree of subjective bias. Also the intervals between numbers on such scores often do not carry the same "weight" so that the difference between a score of 1 and 2 may not be equal to difference between 2 and 3. Lastly, such scales done in the clinic setting capture only a brief period of a patient's day. We propose that examination of home based gait monitoring, timed tests of motor function and quantitative measures of visual problems in patients with SCA are more useful in measuring the disability in these patients.

Conditions

Spinocerebellar Ataxia

Keywords

Spinocerebellar ataxia; Machado-Joseph disease; Gait monitoring; Functional vision assessment

Outcome Measures

Primary Outcomes (1)

• Home based gait data vs SARA index

  0, 6, 12, 24 months

Secondary Outcomes (3)

• Barthel Index

  0, 6, 12, 24 months

• Visual function- acuity, contrast, stereo, color

  0, 6, 12, 24 months

• Ocular Motility defects- phoria/tropia, vergence amplitudes

  0, 6, 12, 24 months

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients attending the Ataxia Clinic

You may qualify if:

• Patients with the diagnosis of Spinocerebellar Ataxia (Type 1,2,3,6,7,8 and 10) will be selected from those attending the ataxia clinic in the Neurology Department at University of Mississippi Medical Center
• Patients will be 18years or older in age
• All patients are able to walk with or without assisted devices

You may not qualify if:

• Patients less than 18years.
• Patients who are wheelchair bound

Sponsors & Collaborators

• University of Mississippi Medical Center: lead
• University of Texas: collaborator

Study Sites (2)

University of Mississippi Medical Center

Jackson, Mississippi, 39211, United States

Location

University of Texas Medical Branch

Galveston, Texas, 77555, United States

Location

Related Publications (6)

• Schols L, Bauer P, Schmidt T, Schulte T, Riess O. Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol. 2004 May;3(5):291-304. doi: 10.1016/S1474-4422(04)00737-9.

  PMID: 15099544 BACKGROUND

• Duenas AM, Goold R, Giunti P. Molecular pathogenesis of spinocerebellar ataxias. Brain. 2006 Jun;129(Pt 6):1357-70. doi: 10.1093/brain/awl081. Epub 2006 Apr 13.

  PMID: 16613893 BACKGROUND

• Schmitz-Hubsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schols L, Szymanski S, van de Warrenburg BP, Durr A, Klockgether T, Fancellu R. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006 Jun 13;66(11):1717-20. doi: 10.1212/01.wnl.0000219042.60538.92.

  PMID: 16769946 BACKGROUND

• Subramony SH, May W, Lynch D, Gomez C, Fischbeck K, Hallett M, Taylor P, Wilson R, Ashizawa T; Cooperative Ataxia Group. Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale. Neurology. 2005 Apr 12;64(7):1261-2. doi: 10.1212/01.WNL.0000156802.15466.79.

  PMID: 15824358 BACKGROUND

• Busse ME, Pearson OR, Van Deursen R, Wiles CM. Quantified measurement of activity provides insight into motor function and recovery in neurological disease. J Neurol Neurosurg Psychiatry. 2004 Jun;75(6):884-8. doi: 10.1136/jnnp.2003.020180.

  PMID: 15146006 BACKGROUND

• Fahey MC, Corben LA, Collins V, Churchyard AJ, Delatycki MB. The 25-foot walk velocity accurately measures real world ambulation in Friedreich ataxia. Neurology. 2007 Feb 27;68(9):705-6. doi: 10.1212/01.wnl.0000256037.63832.6f. No abstract available.

  PMID: 17325285 BACKGROUND

MeSH Terms

Conditions

Spinocerebellar Ataxias; Machado-Joseph Disease

Condition Hierarchy (Ancestors)

Cerebellar Ataxia; Cerebellar Diseases; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Spinocerebellar Degenerations; Spinal Cord Diseases; Heredodegenerative Disorders, Nervous System; Neurodegenerative Diseases; Ataxia; Dyskinesias; Neurologic Manifestations; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• James J Corbett, MD

  University of Mississippi Medical Center

  STUDY CHAIR

• S H Subramony, MD

  University of Texas

  STUDY DIRECTOR

• Sachin Kedar, MD

  University of Mississippi Medical Center

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: April 1, 2008
• First Posted: April 7, 2008
• Study Start: February 1, 2008
• Primary Completion: February 1, 2009
• Study Completion: February 1, 2009
• Last Updated: April 29, 2009

Record last verified: 2009-04

Locations

US (2)