Basal ganglia alterations and brain atrophy in Huntington's disease depicted by transcranial real time sonography

Abstract

Objectives and methods-Transcranial real time sonography (TCS) was applied to 49 patients with Huntington's disease and 39 control subjects to visualise alterations in the echotexture of the basal ganglia. For comparison T1 weighted, T2 weighted, and fast spin echo MRI was performed in 12 patients with Huntington's disease with and in nine patients without alterations of the basal ganglia echotexture as detected by TCS and T1 weighted, T2 weighted, and fast spin echo MRI. Furthermore, the widths of the frontal horns, third ventricle, and the lateral ventricles were depicted in TCS examinations and correlations examined with corresponding CT slices. Results-Eighteen out of 45 (40%) of the patients with Huntington's disease with adequate insonation conditions showed hyperechogenic lesions of at least one basal ganglia region. In 12 patients TCS depicted hyperechogenic lesions of the substantia nigra; in six patients the head of the caudate nucleus was aVected. The lentiform nucleus (n=3) and the thalamus (n=0) were less often aVected or spared. Hyperechogenic lesions were significantly more frequent in patients with Huntington's disease than in 39 control subjects, who had alterations of the echotexture in 12.8% (4/39) of the examinations. The number of CAG repeats and the clinical status correlated with the identification of hyperechogenic lesions of the substantia nigra (p<0.01). Hyperechogenic lesions of the caudate nucleus were associated with an increased signal intensity in T2 weighted MR images (p<0.05). All TCS parameters indicating brain atrophy correlated with CT findings (p<0.0001). Conclusions-TCS detects primarily abnormalities of the caudate nucleus and substantia nigra in Huntington's disease. These changes in the echotexture may represent degenerative changes in the basal ganglia matrix and are partially associated with CAG repeat expansion and the severity of clinical findings. (J Neurol Neurosurg Psychiatry 1999;67:457-462)

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

“…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.…”

“…10 The maximal width of the frontal horns of the side ventricles, the distance between the septum pellucidum and the head of caudate nucleus and the minimal transverse diameter of the third ventricle were measured on a standardized diencephalic examination plane. 16 The findings were stored, so that a second experienced TCS investigator (J.E. ), who was also blinded to the clinical ratings, performed a second independent "off-line" evaluation.…”

“…[11][12][13][14][15] Up to now, there has been only 1 TCS study evaluating basal ganglia alterations in patients with Huntington disease. 16 The echogenicity of the brain stem raphe was not assessed in this study. This is of particular interest since depression is a frequent psychiatric symptom in patients with Huntington disease.…”

Sinais em neurorradiologia: parte 2

“…Huntington's disease causes muscles discoordination and cognitive and behavioral alterations. The finding of ventricular dilatation, as well as basal ganglia atrophy, is very sensitive, but poorly specific (26,27) .…”

Transcranial Sonography in Movement Disorders

“…Postert et al 48 published the fi rst results of TCS evaluation of patients with Huntington's disease in 1999. In 40 % of their patients, a hyperechogenic SN (in 27 %) or caudate nucleus (in 13 %) was detected.…”