Spinal cord atrophy in spinocerebellar ataxia type 3 and 6 : impact on clinical disability.

OBJECTIVE: To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS: Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched healthy control subjects (n = 24,) corresponding to the two SCA groups, were studied. Images were post-processed by a semi-automated volumetry method combining a marker based segmentation and an automatic histogram method facilitating highly reliable quantification and morphometry of the upper cervical cord in vivo. RESULTS: We found a significant reduction of normalized mean crosssectional area of the spinal cord in SCA3 (p < 0.0005), whereas in SCA6 patients normalized mean crosssectional area was in the normal range (p = 0.379). No correlation was found between spinal cord atrophy and disease duration as well as CAG repeat length in both subtypes. In SCA6 a negative dependency between clinical disability, as expressed by the International Cooperative Ataxia Rating Scale as a well established ataxia score, and the mean cross-sectional area was found (p = 0.02). A similar correlation was observed in SCA3 but did not reach statistical significance. CONCLUSION: Our results quantify for the first time in vivo spinal cord atrophy as a non-cerebellar neurodegenerative process in SCA3. Our results suggest MR volumetry of the upper cervical cord as a marker of functional importance in SCA3 and SCA6.

Sensitivity and reproducibility of a new fast 3D segmentation technique for clinical MR-based brain volumetry in multiple sclerosis.

Fast, reliable and easy-to-use methods to quantify brain atrophy are of increasing importance in clinical studies on neuro-degenerative diseases. Here, ILAB 4, a new volumetry software that uses a fast semi-automated 3D segmentation of thin-slice T1-weighted 3D MR images based on a modified watershed transform and an automatic histogram analysis was evaluated. It provides the cerebral volumes: whole brain, white matter, gray matter and intracranial cavity. Inter- and intra-rater reliability and scan-rescan reproducibility were excellent in measuring whole brain volumes (coefficients of variation below 0.5%) of volunteers and patients. However, gray and white matter volumes were more susceptible to image quality. High accuracy of the absolute volume results (+/-5 ml) were shown by phantom and preparation measurements. Analysis times were 6 min for processing of 128 slices. The proposed technique is reliable and highly suitable for quantitative studies of brain atrophy, e.g., in multiple sclerosis.