Lenticular nucleus lesion in idiopathic dystonia detected by transcranial sonography

Abstract

We report the transcranial sonography (TCS) findings of the basal ganglia in 86 patients with dystonic disorders including idiopathic dystonia (facial, cervical, upper limb, and generalized dystonia), drug-induced tardive dystonia, dopa-responsive dystonia, and kinesigenic dystonia. The TCS was focused on alterations of the lenticular, caudate, raphe nuclei, and the thalamus. Seventy-five percent of patient with idiopathic cervical and 83% of those with idiopathic upper limb dystonia had a hyperechogenic lesion of the middle segment of the lenticular nucleus on the side opposite to the clinical dystonic symptoms. The ipsilateral side was also affected in 20%. In facial dystonia, only one-third of the patients revealed lenticular nucleus lesions. The mean area of the lenticular nucleus lesion opposite to the clinically affected side was 30 mm2 in cervical dystonia, 17 mm2 in upper limb dystonia, and 7.5 mm2 in facial dystonia. These lenticular abnormalities were significantly more frequent (p < 0.001) and their areas were significantly greater (p < 0.001) compared with a control group of 50 patients afflicted with radiculopathy. There was a significant correlation of the severity of symptoms with the intensity of lenticular nucleus hyperechogenicity in patients with cervical and upper limb dystonia (p < 0.05). Increased caudate nucleus echogenicity was present in 20% of patients with cervical and upper limb dystonia, mostly contralateral to the clinically affected side and raphe abnormalities were present in 7% of all patients with idiopathic dystonia. In contrast, there were no abnormalities of the lenticular nucleus or thalamus in nonidiopathic dystonias. We conclude that idiopathic dystonia is associated with lesions in the basal ganglia, particularly the lenticular nucleus, that can be visualized by TCS. An alteration of the basal ganglia matrix may be the pathologic basis of idiopathic dystonia with secondary affliction of striatopallidothalamic pathways.

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

“…Third-ventricle dilatation typically found already at early stages of progressive supranuclear palsy (Richardson syndrome) supports its discrimination from PD [31]. Hyperechogenicity of the lenticular nucleus is a characteristic finding in patients with generalized, segmental, focal and taskrelated forms of idiopathic dystonia [41,42]. However, the specificity of this TCS finding has been challenged by one study [44], and further studies are needed to assess its diagnostic value, e. g. for the discrimination of idiopathic dystonia from psychogenic movement disorders.…”

“…The diagnosis of basal-ganglia hyperechogenicity is usually made visually [2]. In addition, hyperechogenic areas may be measured planimetrically, similar to measurements of SN hyperechogenicity [2,41]. Recently, the echo-intensity of basal ganglia was reliably quantified using digitized image analysis [12,42].…”

Spontaneous dyskinesia and familial liability to schizophrenia

“…For example, lesions involving the caudate nucleus, putamen, globus pallidus, subthalamic nucleus, thalamus, and cerebellum have been associated with particular spontaneous dyskinesias in several studies using a variety of methodological techniques (e.g., Antonini et al, 1998;Dooling & Adams, 1975;Kim et al, 2002;Krystkowiak et al, 2000;Krystkowiak et al, 1998;LeDoux & Brady, 2003;Lehericy et al, 1996;Lenz et al, 2002;Lozza et al, 2002;Naumann et al, 1996;Perlmutter et al, 1997). Moreover, an array of structural and functional abnormalities have been observed in several of these regions in individuals with schizophrenia, although substantial variation in findings exists (e.g., Danos et al, 2002;Gunduz et al, 2002;Jernigan et al, 1991;Keshavan et al, 1998;Lang et al, 2001;Marcelis et al, 2003;McCreadie et al, 2002;Menon et al, 2001;Muller et al, 2002;Sigmundsson et al, 2001;Staal et al, 2000;Staal et al, 2001;Young et al, 2000).…”

Transcranial Sonography in Movement Disorders

“…Dystonia is characterized by involuntary prolonged muscle contractions that distort the body into typical postures 52,53 . The pathogenesis of idiopathic dystonia and their origin is still unknown.…”

“…52 ). Subsequent studies 53 demonstrated that hyperechogenic LN is present in patients with torticollis or dystonic writer's cramp on the contralateral side of rotation of the head or aff ected hand.…”