Reversible posterior leukoencephalopathy syndrome associated with left ventricular assist device.

Reversible posterior leukoencephalopathy syndrome (RPLES), previously known as posterior reversible encephalopathy syndrome (PRES), is characterized by the presence of bilateral lesions located within the posterior border zones of the cerebral hemisphere and cerebellum. This condition commonly presents with headache, nausea, vomiting, decreased vision and level of consciousness, and seizures. RPLES has been associated with hypertensive encephalopathy, eclampsia, renal failure, and immunosuppressive therapy following transplant. We report the development of RPLES in a boy following implantation of a left ventricular assist device (LVAD). To our knowledge, this is the first report of RPLES in association with the use of LVAD.

Treatment of posttransplant lymphoproliferative disease in the central nervous system of a lung transplant recipient using allogeneic leukocytes.

Posttransplant Epstein-Barr virus-related lymphoproliferative disease (PT-LPD) is a common and often fatal complication following solid organ and hematopoietic stem cell transplantation. PT-LPD following solid organ transplantation generally occurs in B cells of recipient origin in contrast to PT-LPD in marrow transplant recipients, which is exclusively of donor origin. The efficacy of adoptive immunotherapy using donor leukocytes to treat PT-LPD in bone marrow transplant recipients has recently been reported. Because PT-LPD in solid organ transplant recipients is generally of recipient origin, the potential application of adoptive immunotherapy of PT-LPD in solid organ recipients obligates the use of either autologous or allogeneic HLA identical leukocytes, with the attendant risk of organ rejection if cells mismatched with the transplanted organ are used. Nonirradiated allogeneic mononuclear cells from an Epstein-Barr virus (EBV)-seropositive, HLA-identical normal sibling were used to treat a monoclonal EBV lymphoma of recipient origin in the central nervous system of a child who had undergone an HLA-mismatched cadaveric lung transplant. The patient received three separate mononuclear cell infusions over a 9-month period, each containing 1 x 10(6) CD3+ mononuclear cells per kilogram. Complete clinical, radiological, and pathological remission was achieved with this treatment regimen. The response correlated with in vivo reconstitution of normal EBV-specific cytotoxic activity and cytotoxic T lymphocyte precursor frequency. Use of allogeneic HLA-compatible mononuclear cells may thus offer an additional mode of therapy for EBV-related lymphoproliferative disease in selected solid organ transplant recipients refractory to conventional therapies.

Intraosseous hematoma in a newborn with factor VIII deficiency.

We present an unusual case of an intraosseous hematoma in a newborn with a known bleeding disorder. This cephalohematoma was diagnosed shortly after birth, was entirely within the bony skull, and was in fact determined to be an intraosseous hematoma. The initial CT scans showed the unusual appearance and location of the lesion; later scans showed a significant amount of remodeling, with resolution of the hematoma. Although the coagulopathic diagnosis was independent of this finding, a bleeding disorder might be considered in other patients with similar CT findings.

Familial hypophosphatemic rickets causing ocular calcification and optic canal narrowing.

In a case of familial hypophosphatemic rickets, marked bone thickening caused narrowing of the optic canals, resulting in bilateral optic atrophy. The case also showed metastatic calcification in the walls of both globes.

Central nervous system cryptococcosis: parenchymal calcification and large gelatinous pseudocysts.

In an 11-year-old immunocompetent girl with protracted cryptococcal infection of the central nervous system, CT showed multiple areas of parenchymal calcification. MR imaging showed large gelatinous pseudocysts around the brain stem. These imaging features and the child's age are unusual for intracranial cryptococcosis.

Clinical correlates of white-matter abnormalities on head magnetic resonance imaging.

We undertook this study to investigate the relationship between white-matter abnormalities (seen on brain magnetic resonance imaging [MRI]) and muscle tone and muscle stretch reflexes on clinical examination. We identified all patients less than 5 years of age who had undergone cranial MRI studies at Riley Hospital for Children between June 30, 1999, and July 1, 2000, whose scans were read as showing white-matter abnormalities. We measured two ratios and the thickness of the corpus callosum as indicators of the quantity of cerebral white matter. The ratios were R1, the ratio of the thickness of the white matter at the level just above the body of the lateral ventricle compared with the width of the hemisphere, and R2, the ratio of the thickness of the white matter to the width of the hemisphere at the level of the trigone of the lateral ventricle. The thickness of the corpus callosum was measured at the junction of the anterior two thirds and the posterior third. We also evaluated the signal intensity of the cerebral white matter by reviewing the fluid-attenuated inversion-recovery images and grading the signal as normal to severely abnormal depending on the degree and extent of high signal intensity seen (0 = normal to 4+). Thirty-eight children less than 5 years of age who underwent MRI scans between June and August 2000 and who were found to have normal tone prospectively and normal MRI scan on review served as a control group. We identified 215 patients who had white-matter abnormalities; of these, only 142 (66%) had documented tone assessments in their medical record. Our study group was divided into three groups: increased (n = 35), decreased (n = 53), and normal tone (n = 54). All three measurements of white matter in each of the three study groups were significantly below values for control children. The children with white-matter abnormalities and decreased tone had significantly less signal intensity abnormality than the other study groups. Children with white-matter abnormalities and increased tone had a greater frequency of increased reflexes and tended to have more signal abnormalities than the other groups. The group of children with white-matter abnormalities and normal tone had the least amount of cerebral white-matter deficiency of the three study groups. In patients with strikingly decreased quantities of cerebral white matter, those with normal signal-intensity white matter are likely to be hypotonic with normal reflexes and those with increased signal intensity in the white matter are likely to be spastic.

Vertebral artery compression due to head rotation in thalamic stroke.

Vertebral artery compression from head turning has been described in adults, especially in those who have spondylosis of the spine and osteophytes. There are no reports that demonstrate vertebral artery compression due to head turning in children without vertebral abnormalities. A child is reported with thalamic stroke in whom cerebral arteriography revealed vertebral artery compression on head turning. Blood flow in the vertebral artery, however, could be demonstrated by Doppler ultrasonography with the head in the midline. It is concluded that the vertebral artery is vulnerable to mechanical compression in the neck even in the absence of bony abnormalities.

Midwest experience with moyamoya disease.

A review of the cases of Moyamoya disease at two large Mid-Western United States Universities was undertaken for the purpose of assessing the epidemiology of Moyamoya disease. A total of 51 cases of Moyamoya disease were identified, with 12 cases classified as akin Moyamoya disease, nine cases of probable Moyamoya disease, and 30 cases of classic or definite Moyamoya disease. The conditions associated with akin Moyamoya were sickle cell disease, Down's syndrome, trauma, radiation, and neurofibromatosis. The mean age of presentation for probable and classic Moyamoya disease was 22 years. The sex predilection was approximately equal, with a slight female predominance. The racial background was identified in 22 of the definite cases, and included six patients with oriental inheritance, three with American Indian inheritance, one black, and the remainder Caucasian. Of some interest, there were five Caucasian patients with names identifiable as Eastern European in origin. The mean age of presentation of the definite Moyamoya disease was 14 years, the probable Moyamoya disease was 4 years, and the akin Moyamoya disease was 5 years.

Hydrocephalus in children: neurosurgical and neuroimaging concerns.

The neuroimaging of hydrocephalus from the perspective of the pediatric neurosurgeon is discussed. Processes with new developments in therapy and imaging are described, including congenital causes of hydrocephalus, unilateral hydrocephalus, trapped fourth ventricle, and benign external hydrocephalus.

Imaging the central nervous system effects of radiation and chemotherapy of pediatric tumors.

New developments in radiotherapy and chemotherapy have resulted in significant improvements in survival from childhood tumors. Stereotactic radiosurgery and other conformed field radiotherapy treatments allow precise localization of tumors. Through small beam sizes, these new radiation techniques deliver steep dose gradients at the field edges permitting the prescribed dose to be given to the tumor while avoiding vital structures only millimeters away. Newer chemotherapy regimens allow radiotherapy to be postponed until the child's brain can better tolerate radiation. The MR imaging and CT scans appearance of treated childhood brain tumors and the new developments in radiation and chemotherapy are discussed.

Livedo reticularis, developmental delay and stroke-like episode in a 7-year-old male.

Livedo reticularis is a vascular abnormality of the skin resulting in an erythematous reticular rash. The combination of livedo reticularis and stroke-like episodes in adults is known as Sneddon syndrome [Sneddon, IB (1965). Br J Dermatol 77:180-188]. A similar combination of stroke-like episodes and livedo reticularis has been reported to occur in children [Baxter P et al. (1993). Dev Med Child Neuro 35:917-926]. We present here a 7-year-old male with congenital livedo reticularis, obesity, developmental delay, stroke-like episode, hypertension and cystic kidneys. We summarize our patient's findings and family history, and compare his disorder to other possibly related conditions.

Arachnoid cyst rupture with concurrent subdural hygroma.

Arachnoid cysts (ACs) are relatively common intracranial mass lesions, which occur most often in the middle cranial fossa. While these lesions can present as a mass lesion, many are asymptomatic. Rarely, posttraumatic or spontaneous rupture of ACs can result in intracystic hemorrhage, subdural hematoma or subdural hygroma. We have encountered two cases of ruptured arachnoid cysts that resulted in subdural hygromas. Both patients harbored middle cranial fossa cysts and suffered mild closed head injuries. The presentation, radiographic findings and surgical management of these patients as well as the association between ACs and subdural hygromas are described.

Klippel-Trenaunay-Weber syndrome with hemimegalencephaly.

There are many documented cases of Klippel-Trenaunay-Weber syndrome characterized by the triad of port wine stain, varicose veins, and hypertrophy of bones and overlying tissue. A few cases have even included the additional findings of hemimegalencephaly. We report a case of Klippel-Trenaunay-Weber syndrome with hemimegalencephaly and calvarial hypertrophy, but no evidence of limb or trunk hypertrophy.