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Biomedical subjects

Tena Rosser

Publications and source records attributed to Tena Rosser.

9 recordsLinked to original sources
PubMed2008

Acute transient encephalopathy following paclitaxel treatment in an adolescent with a recurrent suprasellar germinoma.

Paclitaxel is an antineoplastic agent that is used in the treatment of a variety of solid tumors. Dose-limiting side effects of myelosuppression and peripheral neuropathy are well known. Paclitaxel has minimal penetration of the blood-brain barrier and central nervous system side effects are rare. However, transient encephalopathy following paclitaxel infusion has been described in adults but not in children. We present the case of a 14-year-old female with a recurrent suprasellar germinoma who developed an acute encephalopathy 4-6 hr following paclitaxel infusion.

Adolescent↗
PubMed2006

The diverse clinical manifestations of tuberous sclerosis complex: a review.

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous disorder. TSC results in hamartomatous lesions primarily involving the skin, central nervous system, kidneys, eyes, heart, and lungs. The clinical findings and severity of TSC are highly variable. Recent advances in our understanding of the complexities of the TSC1 and TSC2 genes are making genotype-phenotype correlations possible. While managing seizures, cognitive dysfunction, and behavioral abnormalities are the primary responsibility of the neurologist, familiarity with all aspects of this disease helps provide better comprehensive care for affected individuals.

Central Nervous System↗
PubMed2005

Postural headache in a child with Marfan syndrome: case report and review of the literature.

The case of a 10-year-old female with Marfan syndrome and postural headache secondary to spontaneous intracranial hypotension is described. The patient was found to have multiple dural ectasias and a cerebrospinal fluid leak at the left cervicothoracic junction. Her presentation, diagnostic work-up, and management are reviewed, and the relevant literature is discussed. Spontaneous intracranial hypotension secondary to cerebrospinal fluid leaks from dural ectasia should be recognized as a potential complication in children with Marfan syndrome and other connective tissue diseases.

Child↗
PubMed2004

Epstein-Barr virus-associated smooth muscle tumor of the basal ganglia in an HIV+ child: case report and review of the literature.

We describe the clinicopathologic features of an Epstein-Barr virus (EBV)-associated smooth muscle tumor arising in the basal ganglia of a 10-year-old human immunodeficiency virus (HIV)-positive child. Only a few cases of intracranial smooth muscle tumors are reported in the literature and virtually all of these have been extra-axial, involving the dura or sinuses in HIV+ adults. Our case underscores the need to include an EBV-associated smooth muscle tumor in the differential diagnosis when evaluating intracranial mass lesions in immunodeficient children.

Basal Ganglia↗
PubMed2003

Aicardi syndrome.

The astute observations of Aicardi and colleagues led to the first description of Aicardi syndrome as a triad of infantile spasms, absence of the corpus callosum, and chorioretinal lacunae. Still diagnosed clinically, we now recognize an expanded version of this probable X-linked dominant disorder that predominantly affects females. In addition to the classic findings, patients typically experience intractable epilepsy of multiple seizure types, profound mental retardation, and costovertebral anomalies. Associated cerebral and ophthalmologic malformations are numerous. This article highlights several seminal citations involving the history of the initial description and the characteristic ophthalmologic and electroencephalographic features of Aicardi syndrome.

Agenesis of Corpus Callosum↗
PubMed2002

Neurofibromas in children with neurofibromatosis 1.

Neurofibromatosis 1 is a common autosomal dominant disease reported in approximately 1 in 3000 individuals. Although some features of neurofibromatosis 1, such as café-au-lait spots and Lisch nodules, are clinically silent, neurofibromas cause a significant degree of morbidity, mortality, and cosmetic disfigurement. Childhood through early adulthood is a vulnerable period for the growth of these lesions. Neurofibromas are a heterogeneous group of benign tumors that grow from intraneural and extraneural tissues. These tumors take on different morphology, grow at variable rates, and occur in multiple locations. Symptoms arise as neurofibromas enlarge, compressing and distorting local structures. The unpredictable nature of neurofibromas has a serious impact on the quality of life of patients with neurofibromatosis 1, and their management is challenging for physicians. Surgical removal remains the mainstay of treatment. However, advances in the understanding of the genetics and pathogenesis of neurofibromatosis 1 have led to the development of promising new biologically directed therapies. The purpose of this review is to summarize the defining characteristics, incidence, clinical course, management options, and outcome of neurofibromas in children with neurofibromatosis 1.

Adolescent↗
PubMed2002

Intracranial neoplasms in children with neurofibromatosis 1.

Neurofibromatosis 1 is associated with an increased risk for the development of benign and malignant tumors involving neural and non-neural tissues. Children as well as adults with neurofibromatosis 1 are affected. Central nervous system neoplasms represent a significant portion of these malignancies and appear primarily in children less than 10 years of age. Optic pathway gliomas and brainstem gliomas are the most common intracranial neoplasms found in neurofibromatosis 1, although there also is an increased incidence of other brain tumors in this population. The majority of these intracranial neoplasms are benign pilocytic astrocytomas, which may behave in a less aggressive manner than histologically identical tumors in non-neurofibromatosis 1 patients. Owing to the indolent nature of these tumors, conservative management with close follow-up is recommended. When intervention is required, conventional treatment with surgery, radiation, or chemotherapy has been used with variable results. The current challenge lies in understanding the pathogenesis of gliomas in neurofibromatosis 1, which may lead to the development of biologically directed therapies with less associated morbidity and mortality for neurofibromatosis 1 as well as non-neurofibromatosis 1 children.

Brain↗
PubMed2002

Therapy for plexiform neurofibromas in children with neurofibromatosis 1: an overview.

Plexiform neurofibromas are one of the most common and disabling features of neurofibromatosis 1. Treatment options for patients with plexiform neurofibromas have been limited, with surgery being the primary option for patients with progressive lesions causing significant morbidity. Trials have evaluated other treatment approaches, including the use of antihistamines, maturation agents, and antiangiogenic agents. The design of such trials and entry criteria have been quite variable, and results have been difficult to interpret. As more is understood concerning the molecular genetic underpinnings of plexiform neurofibromas, new avenues of treatment are being explored. Evaluation of clinical trials is challenging because of the unpredictable nature of plexiform neurofibromas and difficulties in measuring objective responses. The use of innovative neuroimaging techniques and other outcome measures may greatly improve the design of trials and evaluation of potential effective agents.

Child↗