Child s Nerv Syst (1997) 13: 285 – 288 © Springer-Verlag 1997 CASE REPORT Joachim K. Krauss Joseph Jankovic Hemidystonia secondary to carotid artery gunshot injury Received: 8 October 1996 J. K. Krauss (½)1 Department of Neurosurgery, Baylor College of Medicine, 6560 Fannin, Suite 944, Houston, TX 77030, USA Tel.: (713) 798-5366 Fax: (713) 798-3739 J. Jankovic Department of Neurology, Baylor College of Medicine, Houston, Texas, USA Present address: 1 Department Neurochirurgie, Inselspital, Universitätsspital Bern, CH-3010 Bern, Switzerland Fax: (41) 31-3 82 24 14 Abstract A 9-year-old boy was accidentally shot at close range with a pistol. The bullet entered through the left anterior neck and severed the left common carotid artery. Emergency surgery was performed with an endto-end anastomosis. He recovered gradually from severe right-sided hemiparesis. CT scans demonstrated left parietal infarction. Within months he developed right hemidystonia, which progressed over the next few years. The movement disorder was refractory to medical therapy. MR scans showed a large demarcated defect in the left parietal Introduction Dystonia can be secondary to a variety of causes [8, 15]. Trauma is increasingly recognized as a cause of dystonic movement disorders [7, 11, 13]. We have previously described patients who developed dystonia secondary to craniocerebral or peripheral injury [9, 11, 12, 15]. Here we present an unusual cause of secondary hemidystonia in a child who suffered gunshot injury to the common carotid artery. Case report The patient was referred to the Movement Disorders Clinic at the age of 17 years for further evaluation and treatment of severe rightsided hemidystonia. This young man had had an unremarkable childhood until age 9, when he sustained a gunshot wound to the left upper mediastinum. He was accidentally shot at close range with a 22-caliber pistol by lobe extending to the occipital lobe, to the insula and to the posterior ventral putamen. At age 18 the patient underwent a staged left-sided thalamotomy. The hemidystonia improved postoperatively but later partially recurred Key words Gunshot injury · Hemidystonia · Posttraumatic movement disorder · Stereotactic surgery his older brother. The bullet entered through the anterior aspect of his neck to the left of the midline above the sternal notch. The injury resulted in severe bleeding. Angiographic studies disclosed severance of the left common carotid artery with the bullet lodged in the left upper mediastinum. An emergency neck exploration was performed, which also revealed severance of the thoracic duct. The severed common carotid artery was repaired by an end-to-end anastomosis. Postoperative CT scans demonstrated a left parietal hemorrhagic infarction with a mass effect resulting in midline shift to the right. The patient developed flexor posturing and the pupils became dilated, particularly on the right. He recovered gradually with highdosed dexamethasone therapy and hyperventilation. Two weeks after the injury, he was able to follow commands. When referred for neurological rehabilitation 4 weeks after the injury, he was drowsy and confused and his ability to cooperate was limited. He was lethargic, but he followed simple commands. He did not respond to questions. A right homonymous quadrantanopsia was noted on finger-perimetric testing. He had severe right-sided hemiparesis, with his arm being more involved than his leg. His right arm was flaccid and minimal spasticity was noted in his right leg. Deep tendon reflexes were increased on the right side, and a positive Babinski sign was elicited on the right. When he was discharged 3 months after the injury, the hemiparesis had improved to the point that he was able to walk, but spasticity of his right limbs increased. Within the next few months, he developed right-sided hemidystonia. 286 Although the dystonic movement disorder gradually worsened over the next few years, the diagnosis of dystonia was not made until he was referred to our clinic. When first seen at our Movement Disorders Clinic at age 17, the patient was alert and fully oriented. He had poor abstractive abilities, and his short-term memory was impaired. An incongruous right homonymous hemianopia was found. He had severe right-sided hemidystonia with sustained abduction of his right arm at the shoulder, extension of the elbow, and flexion of his wrist and fingers. His right leg displayed a dystonic posture with extension of the knee, inversion of the foot and flexion of the toes. The dystonic posture was superimposed by spasmodic muscle contractions producing repetitive abduction and adduction of his shoulder and flexion and extension of his elbow. He could voluntarily suppress these movements for a brief period of time. When he walked his right arm became flexed and he tended to hold it behind his neck. Sensory examina- tion revealed right upper extremity hypoesthesia. His deep tendon reflexes could not be assessed on the right side due to the severe dystonia. X-ray examination of the thorax showed a bullet in the left upper mediastinum below the clavicle and smaller metallic fragments projected over the medial aspect of the first rib (Fig. 1). Magnetic resonance imaging of his head revealed a large demarcated defect in the left parietal lobe, extending to the occipital lobe and to the posterior aspect of the insula and the posterior ventral putamen (Figs. 2, 3). A small paraventricular lesion was also found involving the body of the caudate and the superior knee of the internal capusule. No lesions were detected in the brainstem, the thalamus, the pallidum, or the anterior putamen. The left lateral ventricle was enlarged. The hemidystonia did not improve with trihexyphenidyl at a daily dose of 6 mg. At age 18, the patient underwent a left ventrolateral thalamotomy. Postoperatively, he showed remarkable improvement. However, the hemidystonia partially recurred within the next 3 months. He underwent a second functional stereotactic operation 6 months after the first procedure to enlarge the lesion in the left ventrolateral thalamus. Postoperatively, his right-sided hemidystonia was further relieved. There was sustained improvement of the residual hemidystonia over the following months. Within the next few years the hemidystonia again recurred. The residual dystonia was treated with botulinum toxin injections in the right deltoid muscle, right forearm, wrist and finger flexors, and the right gastrocnemius muscle, which yielded additional symptomatic and functional benefit. He received further botulinum toxin injections at 3- to 6-month intervals. Five years after stereotactic surgery there was mild improvement of the hemidystonia compared to the preoperative status. Discussion Fig. 1 AP upper thoracic X-ray showing a 22-caliber bullet lodged in the left upper mediastinum. The bullet had severed the left common carotid artery Fig. 2 Coronal SE 550/15 MR images 8 years after the carotid injury show a large demarcated defect of the left parietal lobe extending to the posterior aspect of the insula Dystonia is characterized by involuntary, sustained, patterned, and often repetitive muscle contractions of opposing muscles, causing twisting or spasmodic movements or abnormal postures [8]. Hemidystonias most often are secondary dystonias and are associated with distinct cerebral lesions. In our patient the dystonic movement disorder developed after a latency of several months and progressed 287 Fig. 3 Axial SE 2500/80 MR images demonstrate extension of the lesion to the left posterior ventral putamen and the occipital lobe over the next few years. Such a course is common in patients with “posthemiplegic dystonia” [5]. The delay of onset can be longer in children than in adults [17]. In our experience, dystonia is often not recognized in these patients, who may present with accompanying spasticity and residual hemiparesis, so-called spastic dystonia [11]. Carotid artery injuries account for only about 5% of arterial injuries [16]. Penetrating carotid artery injuries with hemorrhage present challenging management problems, and they must be treated with great urgency. There has been considerable controversy regarding the optimal treatment [2, 16]. Restoration of cerebral blood flow may result in conversion of an ischemic infarction in a hemorrhagic infarction. The overall mortality rate after carotid artery injury was 21% according to the literature review by Unger et al. [18]. Their review showed that follow-up status at 1 year after trauma was available only in 40 out of 722 patients reported earlier [18]. The frequency and severity of neurological deficits after carotid injury varies [2, 16, 18]. In those patients who suffer neurological symptoms, contralateral hemiparesis is a common finding [16, 18]. Movement disorders have been described only exceptionally after carotid injury. Andrew et al. reported a patient who de- veloped a rest and kinetic tremor after blunt carotid artery trauma sustained in a motorcycle accident [1]. The tremor developed with a latency of 3 months. Initially the patient had a hemiparesis, which was associated with a white matter lesion in the corona radiata. The tremor was greatly reduced after a contralateral thalamotomy. Lesions of the basal ganglia or the brainstem are frequently identified in patiens with secondary dystonia. The caudate and the putamen are most frequently involved, as demonstrated by neuroimaging studies [8, 11, 13 – 15]. It is unclear why some patients with vascular basal ganglia lesions develop dystonic movement disorders and others do not [6]. In a previous report on patients with dystonia secondary to craniocerebral trauma, contralateral subcortical or cortical frontoparietal hemiatrophy was noted in addition to basal ganglia lesions [11]. Hemidystonia has also been noted in patients with cerebral hemiatrophy without basal ganglia lesions [14]. In the patient described here, there was only minor involvement of the posterior ventral putamen and the body of the caudate nucleus, but extensive damage to the parietal lobe. It is conceivable that this large lesion contributed to the development of the disorder. Rarely, parietal lesions have been associated with dystonic movement disorders [3, 10]. Medication is often ineffective in secondary dystonia [11, 15]. Patients with secondary dystonia, however, may benefit from functional stereotactic surgery [4, 11]. Hemidystonia was improved in 50% of patients after contralateral thalamotomy at a mean of 41 months in a recent study [4]. Sustained mild to moderate improvement was observed in 3 out of 6 patients with posttraumatic hemidystonia after long-term follow-up of 18 years (range, 9 – 24 years) [11]. Botulinum toxin injections are also helpful in controlling the residual dystonia [8]. This report draws attention to the rare association between carotid artery injury and subsequent hemidystonia. Since the onset of dystonia may be delayed by several years, the movement disorder may not be attributed to the underlying cause. Proper recognition of the posttraumatic movement disorder is important, as only this makes it possible to recommend appropriate treatment. Acknowledgement The authors wish to thank Monica L. Domingue for preparation of the manuscript. References 1. Andrew J, Fowler CJ, Harrison MJG, Kendall BE (1982) Posttraumatic tremor due to vascular injury and its treatment by stereotactic thalamotomy. J Neurol Neurosurg Psychiatry 45:560–567 2. Barros D’Sa AAB (1996) Brachiocephalic artery injury. In: Yao JST, Pearce WH (eds) Arterial surgery. Appleton & Lange, Stamford, pp 459–477 3. Botenelli MD, Mendilaharsu C, Garcia Mullin R (1967) Trastornos locomotores en la lesión parietal. Acta Neurol Latinoam 13:249–261 4. Cardoso F, Jankovic J, Grossman RG, Hamilton WJ (1995) Outcome after stereotactic thalamotomy for dystonia and hemiballismus. Neurosurgery 36:501–508 5. Factor SA, Sanchez-Ramos J, Weiner WJ (1988) Delayed-onset dystonia assiciated with corticospinal tract dysfunction. Mov Disord 3:201–210 288 6. Hawker K, Lang AE (1990) Hypoxicischemic damage of the basal ganglia. Mov Disord 5:219 –224 7. Jankovic J (1994) Post-traumatic movement disorders: central and peripheral mechanisms. Neurology 44:2006–2014 8. Jankovic J, Fahn S (1993) Dystonic disorders. In: Jankovic J, Tolosa E (eds) Parkinson’s disease and movement disorders, 2nd edn. Williams & Wilkins, Baltimore, pp 337 –374 9. Jankovic J, Van der Linden C (1988) Dystonia and tremor induced by peripheral trauma: predisposing factors. J Neurol Neurosurg Psychiatry 51:1512–1519 10. Krauss JK, Mohadjer M, Nobbe F, Scheremet R (1991) Hemidystonia due to a contralateral parieto-occipital metastasis: disappearance after removal of the mass lesion. Neurology 41:1519–1520 11. Krauss JK, Mohadjer M, Braus DF, Wakhloo AK, Nobbe F, Mundinger F (1992) Dystonia following head trauma – a report of nine patients and review of the literature. Mov Disord 7:263–272 12. Krauss JK, Tränkle R, Kopp KH (1996) Posttraumatic movement disorders in survivors of severe head injury. Neurology 47:1488–1492 13. Lee MS, Rinne JO, Ceballos-Baumann A, Thompson PD, Marsden CD (1994) Dystonia after head trauma. Neurology 44:1374–1378 14. Marsden CD, Obeso JA, Zarranz JJ, Lang AE (1985) The anatomical basis of symptomatic hemidystonia. Brain 108:463–483 15. Pettigrew LC, Jankovic J (1985) Hemidystonia: a report of 22 patients and a review of the literature. J Neurol Neurosurg Psychiatry 48:650–657 16. Rich NM (1986) The management of trauma to the carotid-vertebral system. In: Robicsek F (ed) Extracranial cerebrovascular disease. Macmillan, New York, pp 319–329 17. Scott BL, Jankovic J (1996) Delayedonset progressive movement disorders after static brain lesion. Neurology 46: 68–74 18. Unger SE, Tucker WS, Medeza AN, Wellons HA, Chandler JG (1980) Carotid arterial trauma. Surgery 97:477–487