Hydrocephalus and Bobble-Head Doll Syndrome

Abstract

Hydrocephalus and Bobble-Head Doll SyndromeA 4-month-old female child presented to a neurosurgeon for evaluation of macrocephaly and abnormal head movements. The mother's pregnancy had no complications or known perinatal infections. The parents were unrelated, and there was no family history of congenital anomalies. Ultrasonography performed at 14 and 24 weeks of gestation had revealed moderate hydrocephalus. The patient was born at 38 weeks of gestation by cesarean delivery, with a birth weight of 4100 g, a length of 50 cm, and an occipitofrontal circumference (OFC) of 40 cm.Despite the macrocephaly, the patient's developmental milestone dates were normal. At age 3 months, she had an OFC of 48 cm and was already able to sit and support her neck, but on physical exam she demonstrated repetitive, rhythmic, vertical head movements (Video 1; available at www.jpeds.com). Hydrocephalus was evident on head computed tomography scan (Figure). After placement of a ventriculoperitoneal shunt, she showed an improvement in involuntary head movements (Video 2; available at www. jpeds.com).

Bobble Head Doll Syndrome

“…These movements are rhythmic, may intensify during stress, but are partially controllable by the patient and disappear during sleep [11]. At least 81 other cases have already been described before our cases [2,3,5,8,14,15,17,20] which have a particular clinical character due to the predominance of ‛‛non-non laterality movements''. Since the first description in 1966 [1], the consistency of intracerebral cystic abnormalities has been noticed but the direct causal link remains for the moment subject to controversies.…”