Object The origin and long-term outcome of cerebellar mutism syndrome (CMS), a postoperative syndrome of diminished speech, hypotonia, and ataxia that affects approximately 25% of patients with medulloblastoma, is poorly elucidated. The current study was undertaken to determine factors associated with development of CMS, a means to determine its severity or cause, and outcomes of patients with this syndrome. Methods The study included 28 children with medulloblastoma who either underwent an operation or were referred to the authors' institution soon after surgery. Eleven (39%) of these children had CMS. The preoperative, immediate postoperative, and 1-year postoperative MR images were reviewed by a neuroradiologist blinded to diagnosis of CMS. The severity of mutism and neurological and neurocognitive outcomes were examined. Results Preoperative MR images showed no differences in tumor size, hydrocephalus, or peritumoral edema in patients with and without CMS. An association with brainstem invasion was significant (p < 0.05), and there was a trend toward an association with involvement of the cerebellomedullary angle (p = 0.08). Images obtained immediately postoperatively showed cerebellar edema in 92% of all patients; there were trends for more middle and superior cerebellar peduncle edema in patients with CMS (p = 0.05 and 0.07, respectively). At 1 year postoperatively, patients with CMS showed more moderate to severe atrophy/gliosis of total cerebellum (p < 0.01), vermis (p < 0.01), and brainstem (p < 0.05). Mean IQ was 16 points lower in patients with CMS (IQ = 84.2 ± 15.8) compared with those without CMS (IQ = 100.4 ± 17.4), with a trend toward significance (p = 0.07). Conclusions This study demonstrates that CMS is associated with postoperative damage to the cerebellum and brainstem, damage not predicted by immediate postoperative MR imaging, and with poorer associated functional outcome.

The postoperative cerebellar mutism syndrome (CMS), consisting of diminished speech output, hypotonia, ataxia, and emotional lability, occurs after surgery in up to 25% of patients with medulloblastoma and occasionally after removal of other posterior fossa tumors. Although the mutism is transient, speech rarely normalizes and the syndrome is associated with long-term adverse neurological, cognitive, and psychological sequelae. The clinical, neuroradiographic, and neuropsychological findings associated with CMS as well as possible mechanisms of injury are reviewed. Theories about the pathophysiology of CMS have evolved along with our understanding of the cerebellum as an important structure in the distributive neurocircuitry underlying complex speech, cognition, and behavior. CMS shares many similarities with the cerebellar cognitive affective syndrome, more commonly described in adults and consisting of disturbances of executive function, visuospatial skills, nonmotor language, and affect regulation. Future directions include more thorough neuropsychological characterization, functional and diffusion tensor imaging studies, and investigations into the underlying differences that may make some patients more vulnerable to CMS.

Although detection of cervical spine injuries by MRI does not discriminate between accidental and abusive head trauma, it can help to distinguish a traumatic from non-traumatic intracranial subdural hemorrhage. Cervical MRI should be considered in children with acute intracranial bleeds and otherwise non-contributory history, physical examination and ophthalmological findings. There is a statistically significant relationship between diffuse hypoxic-ischemic brain injury patterns and abusive head trauma. The high incidence of cervical injuries in children with hypoxic-ischemic injuries suggests a causal relationship. Overall, increased utilization of brain and spine MRI in children being evaluated for abusive head trauma can be helpful.

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Choroid plexus carcinomas (CPC) are rare malignant intracranial neoplasms usually occurring in young children. The objectives of this study were to characterize the preoperative MRI features of CPC, determine the frequency of disseminated disease in the CNS at diagnosis, and assess patient outcomes. The preoperative cranial MR images of 11 patients with CPC were retrospectively reviewed for lesion location, lesion size, un-enhanced and enhanced MRI signal characteristics, and presence of disseminated intracranial tumor. Postoperative cranial and spinal MRI images were reviewed for residual, recurrent, and/or disseminated tumor. The study group included six male and five female patients ranging in age from 5 months to 5.3 years (median= 1.8 years). CPC were located in the lateral (n = 8), fourth (n = 1), and third (n = 1) ventricles, and foramen of Luschka (n = 1). Mean tumor size was 5.2 cm x 4.9 cm x 5.0 cm. On short-TR images, CPC had heterogeneous, predominantly intermediate signal with foci of high signal in 45% of lesions from areas of hemorrhage. On long-TR/long-TE images, solid portions of CPC typically had heterogeneous, intermediate-to-slightly-high signal. Small zones of low signal on long-TR/long-TE images were seen in 55% of the lesions secondary to areas of hemorrhage and/or calcifications. Tubular flow voids representing blood vessels were seen in 55% of the lesions. Zones of high signal comparable to CSF were seen in 64% of CPC secondary to cystic/necrotic zones. All CPC showed prominent contrast enhancement. Irregular enhancing margins suggesting subependymal invasion were seen in 73% of the lesions. Findings consistent with edema in the brain adjacent to the enhancing lesions were seen in 73% of CPC. CPC caused hydrocephalus in 82% of patients at diagnosis. Two patients died from hemorrhagic complications from surgical biopsies. Disseminated tumor in the leptomeninges was present in 45% of patients at diagnosis and was associated with a poor prognosis. The 1-year and 5-year survival probabilities were 55% and 45%, respectively. In conclusion, MRI features commonly associated with CPC include large intraventricular lesions with irregular enhancing margins; heterogeneous signal on long TR/long TE images and short-TR images; edema in adjacent brain; hydrocephalus; and presence of disseminated tumor. MRI evidence of disseminated tumor at diagnosis is associated with a poor prognosis.

Children with sickle cell disease (SCD) have high risk of neurologic morbidity and mortality, such as strokes, silent infarcts and TIA's. A retrospective review of magnetic resonance imaging and magnetic resonance angiography identified eight children with radiological and clinical characteristics of reversible posterior encephalopathy (RPLS). These patients had no evidence of previous cerebral infarcts or vasculopathy. Three have died during the 5-year follow up; one developed a stroke and one a conditional TCD. RPLS needs to be considered in the differential diagnosis of children with SCD that present with acute neurological changes, especially if they are already been hospitalized.