Evidence for shoulder girdle dystonia in selected patients with cervical disc prolapse

Abstract

Some patients with cervical disc herniation suffer from persistent nuchal pain and muscle spasms after decompressive surgery despite the lack of clinical and radiological signs for actual spinal root compression. Sonographic examination of the brain in some of these patients showed increased echogenicity of the lentiform nuclei as described in patients with idiopathic dystonia. This has been linked to an altered Menkes protein level and copper metabolism. We suggest a relationship between persistent nuchal pain after adequate cervical disc surgery and dystonic movement disorders. Thirteen patients with persistent nonradicular nuchal pain after at least one cervical disc surgery and without evidence of continuing spinal root compression and 13 age-matched controls were included. All patients had a complete neurological examination, ultrasound, and MRI scan of the brain. In addition, Menkes protein mRNA levels of leucocytes were analyzed in patients and controls. All patients with persistent nuchal pain exhibited a constant tonic unilateral shoulder elevation associated with an ipsilateral hypertrophy of the trapezius muscle. Ultrasound examination showed an increased echogenicity of the lentiform nucleus in one patient unilaterally and in 10 patients bilaterally but in none of the controls. On MRI the T2-values of the lentiform nuclei were found to be higher in patients exhibiting a hyperechogenicity of the lentiform nuclei compared to controls (P = 0.01). In addition, Menkes protein mRNA levels were decreased in patients with cervical disc herniation (P = 0.03). Clinical, neuroimaging, and biochemical findings of this selected patient sample with chronic nuchal pain and muscle spasms after cervical disc surgery resemble alterations in patients with idiopathic cervical dystonia. This suggests a link between both disorders. A peripheral trauma to the nerve roots may precipitate dystonic movements in susceptible patients and chronic dystonic muscle contraction would account for the persistent nuchal pain.

Milestones in magnetic resonance imaging and transcranial sonography of movement disorders

“…The same finding is frequently observed in patients with upper limb dystonia (83%), but less often in patients with facial dystonia (31%) and only rarely in healthy controls (12%) 116. Areas of hyperechogenicity in idiopathic dystonia are larger contralateral to the clinically affected side, and the intensity of hyperechogenicity seems to correlate with the severity of the dystonic symptoms 116. Interestingly, a strikingly high percentage of patients with herniated cervical discs and nuchal pain show hyperechogenicity of the LN 117.…”

“…Increased echogenicity of the lentiform nucleus (LN) has been observed in up to 75% of patients with cervical dystonia 114, 115. The same finding is frequently observed in patients with upper limb dystonia (83%), but less often in patients with facial dystonia (31%) and only rarely in healthy controls (12%) 116. Areas of hyperechogenicity in idiopathic dystonia are larger contralateral to the clinically affected side, and the intensity of hyperechogenicity seems to correlate with the severity of the dystonic symptoms 116.…”

Reduced Serum Ceruloplasmin Levels in Cervical Dystonia

“…Previous studies have repeatedly demonstrated disturbances of copper metabolism in primary dystonia by transcranial ultrasound [3,4,5], leukocyte analysis [6], and neurochemical analysis of trace metals and proteins in the brain tissue [7,8]. The findings were also reproduced in post-surgical secondary dystonia [9], implying the presence of subjects susceptible to dystonia, although the copper gene might be irrelevant by itself to the pathogenesis of dystonia [10]. …”

Sonographic Alteration of Lenticular Nucleus in Focal Task-Specific Dystonia of Musicians

“…While standard structural magnetic resonance imaging (MRI) studies have not revealed any gross pathology in patients with primary dystonia, transcranial sonography (TCS) and voxel-based morphometry MRI detected changes in the globus pallidus of patients with primary spontaneous dystonia [4,5] . Increased echogenicity ('hyperechogenicity'), mainly in the medial part of the lenticular nucleus (LN), was detected on TCS in more than 75% of patients with spontaneous cervical or upper-limb dystonia [4,6] . To find out whether basal ganglia changes are also present in task-specific dystonia, we studied musicians with focal task-specific hand dystonia, unaffected musicians and unaffected nonmusicians with TCS.…”