Article

The transformation of pediatric gliomatosis cerebri to cerebellar glioblastoma multiforme presenting as supra- and infratentorial acute disseminated encephalomyelitis. Case report.

Department of Neurological Surgery, Columbia College of Physicians and Surgeons, Neurological Institute of New York, New York 10032, USA.
Journal of Neurosurgery (Impact Factor: 3.23). 02/2005; 102(1 Suppl):72-7. DOI: 10.3171/ped.2005.102.1.0072
Source: PubMed

ABSTRACT Cerebellar glioblastoma multiforme (GBM) is a rare entity in adults and an extremely rare entity in children. Approximately 30 cases have been reported in the literature. The authors report the case of a histologically confirmed cerebellar GBM presenting initially as supra- and infratentorial gliomatosis cerebri. Acute disseminated encephalomyelitis had been diagnosed in the patient and that diagnosis remained until near the end of his treatment. This case underscores the need for recognizing the clinical presentation of gliomatosis cerebri and multifocal GBM in the pediatric subpopulation thought to harbor demyelinating disease.

0 Bookmarks
 · 
104 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Dolichoectasia (DE) in cerebral arteries is a poorly understood arteriopathy that has been associated with increased risk of vascular morbidity and mortality. Dolichoectasia tends to affects older individuals with vascular risk factors, but it can also be secondary to specific conditions related with extracellular matrix health. The range of methods used to study DE and the biases inherent to hospital-based samples weaken the generalizability of DE study results to the general population. Within the context of these limitations, there is growing evidence that DE is a serious condition that can increase the risk of vascular death. Recurrent strokes and compressive symptoms are among the major causes of morbidity, but cardiac ischemic disease and aortic aneurysms are not uncommon in populations with DE. The devastating outcomes of patients with DE are a call to action aimed at improving the quality of research on the topic and discovering therapies that can palliate the burden of DE in the population.
    Current Cardiology Reports 09/2014; 16(9):525. DOI:10.1007/s11886-014-0525-0
  • [Show abstract] [Hide abstract]
    ABSTRACT: OPINION STATEMENT: Dolichoectasia is a dilatation and elongation of the arteries, usually affecting intracranial basilar and vertebral arteries. It may cause posterior circulation stroke or transient ischemic attacks independent of atherosclerotic disease. Compression of cranial nerves or brainstem leads to hemifacial spasm, trigeminal neuralgia, or brainstem dysfunction (temporary or permanent). Intracranial bleeding is another possible manifestation. In general, the mortality and morbidity is higher in patients with dolichoectasia. Progression of arterial dilatation or elongation is an ominous sign for poor prognosis. Optimal treatment for vertebrobasilar dolichoectasia is uncertain. Adequate control of blood pressure may prevent ischemic or hemorrhagic stroke. In case of ischemic stroke, antiplatelet treatment is indicated for the prevention of recurrent stroke. However, the risk of hemorrhagic stroke should be considered in patients with severe forms of dolichoectasia. Close observation and follow-up neuroimaging is recommended to monitor the progression of the disease and appearance of new ischemic or hemorrhagic lesions. Surgical decompression may be reserved for the medically intractable patients with trigeminal neuralgia or hemifacial spasm.
    Current Treatment Options in Cardiovascular Medicine 03/2011; 13(3):261-7. DOI:10.1007/s11936-011-0123-z
  • [Show abstract] [Hide abstract]
    ABSTRACT: Gliomatosis cerebri (GC) is an intriguing disease for several reasons. First, it is difficult to draw the border between GC and diffuse gliomas. In this regard, GC could represent the most invasive form of diffuse gliomas. Second, both in terms of histologic grading and clinical course, GC is a heterogeneous disease, ranging from rapidly evolving to slowly and somewhat indolent forms. Because of the extensive spread of the disease, surgery-outside a biopsy for diagnosis-is rarely indicated in gliomatosis cerebri. Therapeutic options include radiotherapy, generally involving the whole brain, and chemotherapy with temozolomide or nitrosoureas. Because of the rarity of the disease, no trial comparing these two modalities has been undertaken so far. Decision is, therefore, based on small retrospective noncomparative studies and expert opinions. On one hand, there is a rationale to postpone the whole brain radiotherapy because of late neurotoxicity, but on the other hand, there is also the risk that an aggressive disease evolves to intracranial hypertension making the radiotherapy hazardous or even impossible. As a consequence, the patient would lose the opportunity to receive a potentially effective treatment. In this decision, the evaluation of histologic data together with clinical and radiologic features, performance status, and molecular profile may be of help. Because radiotherapy usually involves large volumes of the brain, chemotherapy is generally preferred up front in patients with a slowly evolving disease. Conversely, in patients with rapidly (ie, over few weeks) evolving disease with neurologic deficits or when histologic features of glioblastoma are evident, whole brain radiotherapy (45 Gy with 1.8 Gy fractions), alone or associated with concomitant temozolomide, is often preferred. The value of advanced of magnetic resonance imaging and positron emission tomography techniques to predict outcome and monitoring the treatment still remains to be defined.
    Current Treatment Options in Neurology 02/2014; 16(2):273. DOI:10.1007/s11940-013-0273-2 · 2.18 Impact Factor

Full-text (2 Sources)

Download
50 Downloads
Available from
Jun 3, 2014