Intrathoracic Malignancy Mimicking Axial Dystonia

Abstract

A 28-year-old woman was referred to the movement disorders clinic at the National Hospital for Neurology and Neurosurgery for investigation of lateral spinal flexion deformity of the trunk and a working diagnosis of a functional (psychogenic) movement disorder. She had a 5-year history of persistent right-sided chest pain, which started during her final university exams, followed by the gradual development of a fixed abnormal posture of the trunk, tilting her to the right. MRI brain and spine were normal. Abnormal posture and pain worsened over time. She had an examination under anesthetic in an orthopedic unit where there was an almost complete correction of her scoliotic deformity. She was placed in a plaster cast to maintain the corrected posture; however, it was immediately removed because of severe breathlessness. The patient was otherwise fit and well. She had no other medical illnesses, including no history of mental health problems. Her only medication was paracetamol for pain relief. There was no family history of dystonia.Clinical examination revealed a marked deformity of her posture with truncal shift to the left and a large thoracic curve (convex to the left) without rotational component. Her left shoulder was elevated and her pelvis was higher on the right (Fig. 1). Lateral flexion was minimal, likely owing to the S-shaped nature of the curve. There was tenderness on palpation over the right chest wall and right shoulder tip with hyperesthesia and allodynia. She was able to self-correct the posture with guidance, however was unable to maintain a normal posture. There were no jerky movements, palpable contractions of back or abdominal muscles, and no sensory geste. Pain and spinal curvature continued when she lay in bed at night. She did not have fixed knee or hip flexion contractures, and her posture did not change with position (supine, sitting, standing, or walking). There was anisocoria (dark: right, 7 mm; left, 6 mm; bright light: right, 4 mm; left, 3 mm) and hyperhidrosis of the right hand and axilla. The remainder of the neurological examination was entirely normal (Video 1).Needle electromyography was normal. Thermoregulatory sweat testing revealed profuse general sweating, but in the face this was observed more on the right side. The presence of chest pain, anisocoria, and unilateral hyperhidrosis prompted a CT chest investigation. A pericardial mass indenting the right atrium and distal superior vena cava (SVC) was identified. There were also a right middle lobe nodule, a number of lung parenchymal nodules extending into the pleural, including the right posterior

Globus Pallidus Internus Deep Brain Stimulation for Dystonic Opisthotonus in Adult-Onset Dystonia: A Personalized Approach

“…In terms of etiology, ATD is highly heterogeneous ( Jabbari et al, 1992 ; Bhatia et al, 1997 ; Benecke and Dressler, 2007 ; Shaikh et al, 2014 ; Selikhova et al, 2015 ). It has been observed in genetic, idiopathic and acquired dystonic syndromes ( Jabbari et al, 1992 ; Bhatia et al, 1997 ; Shaikh et al, 2014 ; Selikhova et al, 2015 ; Lizarraga and Fasano, 2019 ), and can have central and peripheral etiologies.…”

“…In terms of etiology, ATD is highly heterogeneous ( Jabbari et al, 1992 ; Bhatia et al, 1997 ; Benecke and Dressler, 2007 ; Shaikh et al, 2014 ; Selikhova et al, 2015 ). It has been observed in genetic, idiopathic and acquired dystonic syndromes ( Jabbari et al, 1992 ; Bhatia et al, 1997 ; Shaikh et al, 2014 ; Selikhova et al, 2015 ; Lizarraga and Fasano, 2019 ), and can have central and peripheral etiologies. It may be associated with parkinsonism and neuromuscular disorders ( Stamelou et al, 2013 ; Lizarraga and Fasano, 2019 ) as well as with neuroleptic-induced acute and tardive dystonia ( Bhatia et al, 1997 ; Benecke and Dressler, 2007 ; Barone et al, 2016 ).…”