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The occurrence of personality change due to traumatic brain injury (PC), and its clinical and neuroimaging correlates were investigated. Ninety-four children, ages 5 through 14 at the time of hospitalization following traumatic brain injury (TBI; severe TBI N = 37; mild–moderate TBI N = 57), were assessed. Standardized psychiatric, adaptive functioning, cognitive functioning, family functioning, family psychiatric history, severity of injury, and neuroimaging assessments were conducted. The Neuropsychiatric Rating Schedule (NPRS) was used to establish a diagnosis of PC. Approximately 40% of consecutively hospitalized severe TBI participants had ongoing persistent PC an average of 2 years postinjury. An additional approximately 20% had a history of a remitted and more transient PC. PC occurred in 5% of mild–moderate TBI but was always transient. Interrater reliability for the diagnosis of PC was good (Kappa = .70). In severe TBI participants, persistent PC was significantly associated with severity of injury, particularly impaired consciousness over 100 hr, adaptive and intellectual functioning decrements, and concurrent diagnosis of secondary attention deficit hyperactivity disorder, but was not significantly related to any psychosocial adversity variables. These findings suggest that PC is a frequent diagnosis following severe TBI in children and adolescents, but is much less common following mild–moderate TBI. (JINS, 2000, 6, 279–289.)

Shprintzen-Goldberg syndrome is one of a group of disorders characterized by craniosynostosis and marfanoid habitus. Eleven cases were reported previously. We present 4 new patients and review one of the patients of the original report of Shprintzen and Goldberg [1982: J Craniofac Genet Dev Biol 2:65-74], 15 years later. The clinical and radiologic findings on our patients are compared with those of the previously reported patients and also with those of Furlong et al. [1987: Am J Med Genet 26:599-604] and Lacombe and Battin [1993: Clin Dysmorphol 2: 220-224], who share many of the characteristics of Shprintzen-Goldberg syndrome. Some of the clinical data are helpful in determining if the patients of Furlong et al. [1987: Am J Med Genet 26:599-604] and Lacombe and Battin [1993: Clin Dysmorphol 2: 220-224] have a separate syndrome or represent a variant of Shprintzen-Goldberg syndrome. However, radiologic investigations appear to be more specific, since an abnormality of the first and second cervical vertebrae, hydrocephalus, dilatation of the lateral ventricles, and a Chiari-I malformation of the brain were found only in the patients with Shprintzen-Goldberg syndrome. The apparently diagnostic findings of the 15 patients with this syndrome may be helpful in differentiating between Shprintzen-Goldberg syndrome and other syndromes with craniosynostosis and marfanoid habitus.

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