Transcranial sonography in spinocerebellar ataxia type 3

Postert, Thomas; Eyding, Jens; Berg, Daniela; Przuntek, H.; Becker, Georg; Finger, Michael; Schöls, Lüdger

doi:10.1007/978-3-7091-0579-5_15

Cited by 39 publications

(27 citation statements)

References 18 publications

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“…23,30 Although these findings are characteristic for idiopathic Parkinson disease, less frequently, hyperechogenicities of the substantia nigra were reported in other conditions, such as spinocerebellar ataxia, depression and Huntington disease. 15,16,31 In a study of 45 patients with Huntington disease, Postert and colleagues 16 observed hyperechogenicites of the substantia nigra in 12 (27%) patients, of the caudate nuclei in 6 (13%) patients and of the lentiform nuclei in 4 (9%) patients. These authors reported that the number of CAG repeats and the severity of the disease correlated with the identification of hyperechogenicities of the substantia nigra.…”

Section: Discussionmentioning

confidence: 99%

Depression in patients with Huntington disease correlates with alterations of the brain stem raphe depicted by transcranial sonography

Krogias

Strassburger

Eyding

et al. 2011

jpn

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Background: Transcranial sonography (TCS) has become a new diagnostic tool in the evaluation of extrapyramidal disorders. Studies of TCS report alterations of the mesencephalic raphe in patients with depression. The aim of this study was to evaluate TCS findings in patients with Huntington disease in correlation with their neurologic and psychiatric status. Methods: We recruited patients with genetically confirmed Huntington disease. The neurological and psychiatric status of participants was assessed by independent physicians. Echogenicities were investigated according to examination protocol for extrapyramidal disorders using a Siemens Sonoline Elegra system. The sonography examiner was blinded for clinical data. Results: We included 39 patients in our study; 21 patients (53.8%) showed symptoms of depression at the time of evaluation and, of those, 15 (71.4%) had hypoechogenic raphe structures. Thirty patients (76.9%) had a history of depressive episodes, 19 (63.3%) of them with hypoechogenic raphe structures. All 9 patients without a history of depressive episodes showed normal echogenicity of raphe structures (sensitivity 63.3%, specificity 100%). Twelve (70.6%) of the 17 patients with Huntington disease who showed psychiatric disturbances prior to the occurrence of motor symptoms exhibited pathological raphe echogenicity (sensitivity 70.6%, specificity 68.2%). Limitations: Most of the patients were taking antichoreatic medication, which particularly influences neurologic status. Thus, a meaningful interpretation of the correlation between TCS findings and neurologic features was limited. Conclusion: As a novel finding, a relation between mesencephalic raphe echogenicity and depressive state could be identified in patients with Huntington disease. An alteration of the serotonergic brain stem raphe might be involved in the pathogenesis of depression in these patients.

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Section: Discussionmentioning

confidence: 99%

Depression in patients with Huntington disease correlates with alterations of the brain stem raphe depicted by transcranial sonography

Krogias

Strassburger

Eyding

et al. 2011

jpn

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“…On this plane the 4 th ventricle can be seen in dorsal vicinity to the midbrain. In children and young adults the 4 th ventricle often cannot be recognized due to its tightness [39]. However, in elderly subjects and particularly in patients with cerebellar atrophy it is usually visible.…”

Section: Visualization and Assessmentmentioning

confidence: 99%

“…There is currently no standard for the assessment of the cerebellum. Reported abnormal findings are 4 th ventricle dilatation, a coarse appearance of the cerebellar sulci, and increased echogenicity of the dentate nuclei [39,40]. Fourth-ventricle measures are the width and cross-sectional area on a semi-coronal plane.…”

Section: Visualization and Assessmentmentioning

confidence: 99%

“…Abnormal TCS findings of the 4 th ventricle and cerebellum have been demonstrated in patients with various forms of hereditary spinocerebellar ataxia (Friedreich's ataxia, SCA-3, SCA-17) [39,40]. So far there are no reports showing a diagnostic specificity of abnormal cerebellum TCS findings, neither alone nor in combination with other TCS findings.…”

Section: Diagnostic Relevancementioning

confidence: 99%

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Transcranial Sonography (TCS) of Brain Parenchyma in Movement Disorders: Quality Standards, Diagnostic Applications and Novel Technologies

Walter

Školoudík

2014

Ultraschall in Med

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Transcranial B-mode sonography (TCS) of brain parenchyma is being increasingly used as a diagnostic tool in movement disorders. Compared to other neuroimaging modalities such as magnetic resonance imaging (MRI) and computed tomography, TCS can be performed today with portable machines and has the advantages of noninvasiveness and high resistance to movement artifacts. In distinct brain disorders TCS detects abnormalities that cannot be visualized or can only be visualized with significant effort with other imaging methods. In the field of movement disorders, TCS has been established mainly as a tool for the early and differential diagnosis of Parkinson?s disease. The postoperative position control of deep brain stimulation electrodes, especially in the subthalamic nucleus, can reliably and safely be performed with TCS.?The present update review summarizes the current methodological standards and defines quality criteria of adequate TCS imaging and assessment of diagnostically relevant deep brain structures such as substantia nigra, brainstem raphe, basal ganglia and ventricles. Finally, an overview is given on recent technological advances including TCS-MRI fusion imaging and upcoming technologies of digitized image analysis aiming at a more investigator-independent assessment of deep brain structures on TCS.

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“…The results of a recent TCS study 50 confirm that patients with genetically-proven spinocerebellar ataxia subtype 3 (SCA3) show hyperechogenicity of various brain structures, as do subtypes SCA1 and SCA2. Hyperechogenic white matter of the cerebellum was detected in 57 %, hyperechogenic dentate nucleus in 54 %, hyperechogenic SN in 40 %, hyperechogenic putamen in 40 % and hyperechogenic pallidum in 40 % patients.…”

Section: Spinocerebellar Ataxiamentioning

confidence: 78%