Sudden bilateral blindness in children. Selected cases are presented and differential diagnosis is discussed.
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Astrocytoma↗
Biomedical subjects
P K Duffner
Publications and source records attributed to P K Duffner.
Calcification in brainstem gliomas.
Calcification in brainstem gliomas is an unusual histologic and and radiologic finding. To date, radiologic evidence of calcification in these tumors has not been reported. The two patients described here had evidence of calcification on either computerized tomography (CT) or plain skull films. Increasing availability of CT scans may provide a more accurate estimate of the incidence of calcification in these tumors.
Adolescent↗
Infantile spasms associated with histidinemia.
A case of infantile spasms associated with histidinemia is presented. Histidinemia was well-documented through biochemical assays. The patient was treated with the standard anticonvulsant regimen for infantile spasms, as well as an elimination diet for histidinemia. Despite low levels of histidine and adequate anticonvulsant therapy, the child continues to have seizures and is markedly retarded. The natural history of infantile spasms and its possible association with histidinemia is discussed.
Amino Acid Metabolism, Inborn Errors↗
Treatment of infants with malignant gliomas: the Pediatric Oncology Group experience.
Although survivals of infants with malignant brain tumors are worse than any other age group, one possible exception to this rule are the malignant gliomas. Eighteen children less than 3 years of age with malignant gliomas (glioblastoma multiforme, anaplastic astrocytoma and malignant glioma) were treated on the Pediatric Oncology Group regimen of prolonged postoperative chemotherapy and delayed irradiation, (1986-1990). Of 10 children evaluable for neuroradiologic response, 6 had partial responses (> 50% reduction) to two cycles of cyclophosphamide and vincristine. Progression free survivals at 1,3 and 5 years were 54.25% +/- 12, 43% +/- 16 and 43% +/- 23 respectively. Survivals at 5 years were 50% +/- 14. Four children were not irradiated after 24 months of chemotherapy due to parental refusal and none have developed recurrent disease. Neither degree of surgical resection, presence or absence of metastases, nor pathology influenced survival but this may reflect small sample size. This study suggests that some malignant gliomas in infants are chemotherapy sensitive and may be associated with a good prognosis. Why infants with these high-grade gliomas fare better than adults is not clear. It is likely that there is something intrinsically different about them that cannot be identified on routine pathologic examination.
Antineoplastic Combined Chemotherapy Protocols↗
The role of neurologists in pediatric neuro-oncology.
Because of advances in the diagnosis and management of brain tumors, the opportunity for neurologists to participate in the care of children with central nervous system neoplasms has greatly increased. Data developed by various study groups has allowed a more systematic approach to childhood brain tumors. Much more information will become available. New data must be reviewed and recommendations for therapy made by individuals with clinical neuroscience experience and training. The training needs of such individuals must be met in neurology training programs.
Brain Neoplasms↗
Unusual biochemical and clinical features in a girl with ornithine transcarbamylase deficiency.
A girl, ultimately diagnosed as having profound ornithine transcarbamylase (OTC) deficiency, presented as a neonate with feeding intolerance, irritability, and seizures without concurrent hyperammonemia. Developing normally until ten months of age, the girl subsequently experienced two episodes of hyperammonemia, which were associated with focal seizures and residual hemiparesis. She continued to have profound neurologic impairment and seizures and died at 26 months of age, despite appropriate dietary protein restriction, sodium benzoate, and arginine supplementation. Symptomatic OTC deficiency has not been previously reported unassociated with hyperammonemia. The recurrent cerebrovascular episodes are distinctly uncommon in patients with urea cycle enzymopathies.
Amino Acid Metabolism, Inborn Errors↗
Long-term consequences of CNS treatment for childhood cancer, Part II: Clinical consequences.
Survival of children with brain tumors has improved over the past 20 years due in part to advances in surgery, radiation, and most recently chemotherapy. The long-term adverse effects of radiation and chemotherapy on these children is the subject of this report. In Part I, we reviewed the pathologic consequences of radiation, including leukoencephalopathy, radiation necrosis, and radiation myelopathy as well as the oncogenic effects of both radiation and chemotherapy. Part II addresses the long-term consequences of radiation and chemotherapy on intellectual and endocrine function. Risk factors for the development of both endocrinopathies and intellectual dysfunction include age at the time of radiation, volume and dose of radiation, site of tumor, and use of adjuvant chemotherapy, in particular methotrexate. Early recognition of these complications and treatment, where indicated, will measurably improve the quality-of-life of children treated for brain tumors. The national cancer groups are currently attempting to limit these long-term adverse effects by taking risk factors into account when formulating new treatment regimens.
Brain Neoplasms↗
Long-term consequences of CNS treatment for childhood cancer, Part I: Pathologic consequences and potential for oncogenesis.
The pathologic changes associated with the treatment of cancer of the nervous system are reviewed. Computed tomographic, magnetic resonance imaging, and positron emission tomographic findings of these abnormalities are described, followed by discussion of the known histopathologic features. For the most part, pathologic effects are primary vascular and/or demyelinating. We review each of these effects at all levels of the neural axis. This review concludes with a discussion of the risk of developing second malignancies. Although this complication is infrequent, the likelihood that survivors of childhood cancer will develop a second malignancy is 10 times that of age-matched controls. This phenomenon in part relates to genetic predisposition, environmental factors, and host susceptibility. These qualifications not withstanding, most studies implicate central nervous system radiation with and without chemotherapy as the primary etiology for second malignancies.
Antineoplastic Agents↗
Treatment of brain tumors in babies and very young children.
Approximately 13% of brain tumors in childhood occur in children less than 2 years of age. Although the survivals of older children with certain forms of brain tumors have increased over the past 20 years, this treatment has been least effective in very young children with brain tumors. These poor survival rates may be due in part to the highly malignant nature of the neoplasms; the delay in diagnosis because of low index of suspicion; the large bulk of tumor found at presentation, and the reduction in radiation dosage to the central nervous system. Since standard treatment has produced both poor results and severe neurotoxicity, new approaches have been sought. A conservative approach is recommended in some children with optic gliomas and low-grade supratentorial astrocytomas. Radiation is deferred until the child reaches 3-4 years of age when he can better tolerate its effects on the CNS. Current therapy of malignant brain tumors of infancy is not only toxic, but inadequate. There are several centers which currently use postoperative chemotherapy and delayed radiation. Early results have been encouraging and neurotoxicity has been low.
Adolescent↗
Pediatric brain tumors: an overview.
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Adolescent↗