Biomedical subjects

Nicole J Ullrich

Publications and source records attributed to Nicole J Ullrich.

6 recordsLinked to original sources

PubMed2006

Molecular genetics of pediatric central nervous system tumors.

Recent advances in molecular biology have enhanced our understanding of the pathogenesis of brain tumors, particularly in children. The use of molecular diagnostic tools is quickly becoming a standard component in the diagnosis and classification of brain tumors in children, in addition to providing insight leading to treatment stratification and improved outcome prediction. All new protocols involving treatments for brain tumors in children include studies of biomarkers and biologic correlates as a means to identify new targets for therapeutics and possible intervention strategies.

Astrocytoma↗

PubMed2006

Transverse myelitis after therapy for primitive neuroectodermal tumors.

Traditional therapy for malignant primitive neuroectodermal tumors in children includes surgery, multi-agent chemotherapy, and radiation. Given the poor prognosis with conventional therapy alone, newer treatment approaches have incorporated high-dose chemotherapy followed by autologous stem cell rescue. Treatment with chemotherapy and radiation is not without unanticipated and unwanted side effects. Specifically, radiation-induced damage to the central nervous system can occur, though the frequency is thought to be acceptably low. This report describes two cases of treatment-related transverse myelitis in patients who received induction chemotherapy and craniospinal irradiation followed by high-dose chemotherapy with autologous stem cell rescue. Other patients treated with a similar strategy but different sequence and timing of treatment did not experience symptoms of myelitis, suggesting that the specific timing of radiation in relationship to the chemotherapy may be of critical importance.

Adolescent↗

PubMed2005

Craniopharyngioma therapy: long-term effects on hypothalamic function.

BACKGROUND: Craniopharyngiomas are the most common intracranial tumor of extraneural origin in childhood. REVIEW SUMMARY: In this review, we discuss the presentation, diagnosis, and treatment of craniopharyngioma. As the survival prognosis of patients with craniopharyngioma is quite optimistic, long-term side effects of both the tumor and its treatment are now better appreciated. Aside from well-recognized hormonal deficiencies and visual deficits related to tumor location, patients are now acknowledged to experience pathologic obesity and deficits of higher cortical function, memory, and behavior. The combination of these deficits can have profoundly detrimental effects on quality of life. CONCLUSIONS: Careful attention to issues related to hormonal balance, visual field defects, cognitive function, and mood disorders is essential to optimize long-term outcome of patients with craniopharyngioma.

Craniopharyngioma↗

PubMed2004

Electroencephalographic correlate of juvenile Huntington's disease.

The spectrum of clinical disease in juvenile Huntington's disease differs from that seen in adults. Younger patients often present with seizures, dystonia and rigidity. The mechanism and type of seizures, timing of onset and electrographic features have not been well characterized in either adults or children. We describe the electroencephalographic findings observed in a young child with Huntington's disease who presented with motor regression and seizures. Recordings demonstrated bilateral posterior quadrant epileptiform discharges and occipital intermittent rhythmic delta activity, generally considered a nonspecific abnormality. These findings have not been reported in adult or juvenile Huntington's disease. Its presence in proximity to bilateral posterior spikes suggests that occipital intermittent rhythmic delta activity is an epileptiform abnormality, on a continuum with the posterior spike-and-wave discharges. Intermittent rhythmic delta activity can occur secondary to either the subcortical gray-matter disease or the associated seizure disorder. Huntington's disease should be considered in the differential diagnosis of young children who present with seizures and developmental regression.

Age of Onset↗

PubMed2003

Pediatric brain tumors.

Brain tumors in children vary with regard to histology, location, gender distribution, and age of onset. The past several decades have witnessed substantial improvements in the diagnosis and treatment. As a result, the number of long-term survivors also has increased continuously. Recent advances in neuroimaging facilitate tumor localization and mapping of brain function by noninvasive techniques and are becoming important in preoperative assessment for brain tumors. Surgery remains the mainstay of treatment of many tumor types. A role for chemotherapy is emerging, particularly for nonresectable tumors and in infants, for whom the adverse effects of radiation therapy are severe. Despite the improved treatments and prognostic data, however, many long-term survivors experience significant neurocognitive and developmental deficits.

Brain Neoplasms↗

PubMed2003

Transient global amnesia in a young adult with cyanotic heart disease.

The etiology of transient global amnesia is poorly understood, particularly in children and young adults. Transient global amnesia may follow a wide range of precipitating events. Proposed causes have included vascular event, seizure, and migraine. A young man with cyanotic congenital heart disease experienced an episode of transient global amnesia in the setting of polycythemia. Differential diagnosis of acute confusional episodes in children should include transient global amnesia, as well as confusional migraine, and should include evaluation for underlying coagulation abnormalities and polycythemia.

Adult↗