Bobble Head Doll Syndrome

Abstract

Introduction. Bobble head doll syndrome is a neurological syndrome characterized by abnormal involuntary movements of the head combining repetitive or episodic movements of 2 to 3 Hz back and forth in the anterior-posterior direction that can be assimilated to approval movements (yes-yes) and occasionally lateral rotations that can be assimilated to disapproval signs (no-no). It is a rare entity first described by Benton[1]. We describe three cases managed at the Yalgado Ouédraogo University Hospital in Burkina Faso. Observations. Three children, one girl and two boys, aged respectively 5, 9 and 14 years were seen for involuntary abnormal head movements associating lateral rotations assimilated to signs of disapproval (no-no) in two cases, and back and forth movements of the head in one case. In the 14-year-old adolescent, there was also a picture of intracranial hypertension. Clinical examination revealed ataxia and macrocrania in both cases and a syndrome in 1 case. Imaging revealed triventricular hydrocephalus on aqueductal stenosis in all cases with an associated supra sellar cyst in 2 cases. The treatment consisted of endoscopic treatment in 2 cases and ventriculoperitoneal shunt in 1 case. Surgery allowed a considerable regression of involuntary movements of the head in the immediate postoperative period and a complete recovery in the long term. Conclusion. The bobble head doll syndrome is a rare entity related to the consequences of chronic hydrocephalus responsible for abnormal movements whose management done well and early leads to favourable results.