Tumor-related epilepsy in children.

A 10-year retrospective review of 15 children with cerebral tumors and seizures was conducted to study the factors responsible for delay in the diagnosis of tumors and to assess outcome following surgery. Mean duration of seizures prior to surgery was 37 months. Ninety-three percent had no focal neurologic deficits. Head computed tomography was abnormal in 64%, whereas magnetic resonance imaging was abnormal in all patients. Electroencephalography showed focal abnormalities ipsilateral to the tumor in 73%. There was no surgical mortality. Eighty percent were seizure free or had rare seizures following surgery. Factors contributing to a delayed diagnosis of the brain tumor included a nonfocal neurologic examination and delay in obtaining an appropriate neuroimaging study. We believe that head magnetic resonance imaging should be the investigation of choice in partial epilepsies.

Phase II evaluation of interferon-alpha-2a for progressive or recurrent craniopharyngiomas.

OBJECT: Craniopharyngiomas originate from the same cells as squamous cell skin carcinoma, which can be treated successfully with interferon-alpha (IFNalpha)-2a. The authors evaluated the activity and toxicity of systemic IFN in young patients with craniopharyngiomas. METHODS: Fifteen patients between the ages of 4.2 and 19.8 years who had progressive or recurrent craniopharyngiomas were enrolled in this study. Nine of these patients had never received external-beam radiation therapy. Therapy consisted of 8,000,000 U/m2 IFNalpha-2a administered daily for 16 weeks (induction phase) followed by the same dose three times per week for an additional 32 weeks (maintenance phase). Of the 12 patients who could be evaluated, radiological studies demonstrated a response to treatment in three with predominantly cystic tumors (one minor response, one partial response, and one complete response); one of these patients also showed improvement in visual fields. The size of the cystic component of the tumors often increased temporarily during the first several months of therapy. Three patients met the criteria for progressive disease during therapy. The median time to progression was 25 months. The need for radiation therapy in patients treated with IFN was delayed for 18 to 35 months (median 25 months) in six patients. All patients developed transient flulike symptoms shortly after receiving the first dose of IFN. Other toxicities (predominantly hepatic, neurological, and cutaneous) were seen in nine (60%) of the 15 patients during the first 8 weeks of treatment but resolved after temporary discontinuation and/or dose reduction. CONCLUSIONS: Interferon-alpha-2a is active against some childhood craniopharyngiomas; its toxicity precludes administration of high daily doses, and the optimum dose level and schedule remain to be defined.

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.

Chiari III malformation treated with CSF diversion and delayed surgical closure.

Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malformation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. Instead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the brainstem and cerebellum to regress into the cervical spinal canal as the dilated cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospinal fluid diversion with delayed closure may be an option for large lesions.

Spontaneous spinal epidural hematoma in children.

Spontaneous spinal epidural hematoma is rare in children. It is usually confined to the dorsal epidural space. Ventral spontaneous spinal epidural hematoma (SSEH) is rarer, with only two previous reports. The authors present three children, two with dorsal and one with ventral spinal epidural hematoma, and review the literature. No etiology of the hematoma was found in the authors' patients and there was no history of trauma. A review of 24 patients of children younger than 18 years of age reported in the literature and the authors' three patients revealed that the cervicothoracic region was the most common site of SSEH, the mode of onset was frequently subacute, and there was no male preponderance as has been reported in adults. We found that the initial symptoms were often nonspecific, leading to a delay in diagnosis, especially in younger children. Follow-up data revealed that 15 of the 27 patients recovered completely, 11 had residual neurologic deficits, and one patient died. Irritability and neck pain with restricted movements of the cervical spine in an afebrile child may be early signs of SSEH and often precede onset of neurologic deficits by several hours to days. These signs should alert the clinician to consider spinal epidural hematoma and the need for urgent magnetic resonance imaging (MRI) of the spine for early diagnosis and treatment to minimize morbidity.

Chiari I malformation: association with hypophosphatemic rickets and MR imaging appearance.

PURPOSE: To evaluate for an association between familial hypophosphatemic rickets (FHR) and Chiari I malformation (CM1). MATERIALS AND METHODS: Sixteen patients with FHR underwent magnetic resonance (MR) imaging of the cervicomedullary junction. Images were analyzed by three radiologists for cerebellar tonsillar ectopia, syringohydromyelia, calvarial bone thickening, a flat posterior fossa, and cervical spinal stenosis. Final diagnoses were made by means of consensus. Tonsillar ectopia of 4 mm indicated CM1. Subjects underwent neurologic examination and completed a questionnaire. Medical records were retrospectively reviewed. A two-sided Fisher exact test was used to test for independence between CM1 and bone thickening or ventriculomegaly. RESULTS: Seven subjects (44%) had CM1. The more severe the bone thickening, the more likely that a CM1 was present. Four subjects (25%) had cervical spinal stenosis. CONCLUSION: Findings indicate that CM1 is associated with FHR and that the primary abnormality in patients with CM1 is a small posterior fossa caused by a bony malformation.

Primary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor.

Malignant rhabdoid tumor was originally described and is most commonly reported in the kidney [1-5]. A case of primary central nervous system malignant rhabdoid tumor is presented. The clinical and pathologic findings are presented and the computed tomography and magnetic resonance imaging findings are described.

Magnetic resonance imaging diagnosis of a cerebral aneurysm in an infant. Case report and review of the literature.

A posterior cerebral artery aneurysm presented as a seizure disorder in a 7-week-old infant. A small hemorrhage in the posterior thalamus was seen on CT scan. However, magnetic resonance imaging (MRI study) demonstrated an aneurysm and appropriate therapy was carried out. The incidence, natural history, etiology, and surgical treatment of these lesions are reviewed, and the usefulness of MRI in their diagnosis is emphasized.

Role of fructose 2,6-bisphosphate in the regulation of glycolysis and gluconeogenesis in chicken liver.

Glucagon and dibutyryl cyclic AMP inhibited glucose utilization and lowered fructose 2,6-bisphosphate levels of hepatocytes prepared from fed chickens. Partially purified preparations of chicken liver 6-phosphofructo-1-kinase and fructose 1,6-bisphosphatase were activated and inhibited by fructose 2,6-bisphosphate, respectively. The sensitivities of these enzymes and the changes observed in fructose 2,6-bisphosphate levels are consistent with an important role for this allosteric effector in hormonal regulation of carbohydrate metabolism in chicken liver. In contrast, oleate inhibition of glucose utilization by chicken hepatocytes occurred without change in fructose, 2,6-bisphosphate levels. Likewise, pyruvate inhibition of lactate gluconeogenesis in chicken hepatocytes cannot be explained by changes in fructose 2,6-bisphosphate levels. Exogenous glucose caused a marked increase in fructose 2,6-bisphosphate content of hepatocytes from fasted but not fed birds. Both glucagon and lactate prevented this glucose effect. Fasted chicken hepatocytes responded to lower glucose concentrations than fasted rat hepatocytes, perhaps reflecting the species difference in hexokinase isozymes.