Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 488 children with central nervous system neoplasms, 43 (8.8%) had glioblastomas, 22 of which were in the cerebral hemispheres, 16 in the brain stem, two in the cerebellum, and three in the spinal cord. The male to female ratio was 3:2. Glioblastoma multiforme of the cerebral hemispheres occurred at a mean age of 12.7 years, and the frontal lobe was the most commonly involved. Main presenting symptoms included headache (85%), nausea or vomiting (65%), and seizures (35%). Papilledema (45%) was the most common physical finding. The longest survivals were achieved by a combination of operation and radiation (22 months). Brain stem glioblastomas occurred at a mean age of 6.7 years, with the pons as the most frequent site. Nausea or vomiting (50%) and headache (36%) were the main presenting symptoms; the major physical findings were ataxia (43%), cranial nerve palsies (28%), and paresis (28%). The length of survival was greatest with radiation alone (10.5 months). The period of survival of children with glioblastoma multiforme was significantly increased with steroid therapy. Glioblastoma multiforme behaves similarly in children and adults. Intracranial glioblastomas have a more rapidly fatal course than that of other similarly situated gliomas in childhood.
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PMID:Glioblastoma multiforme in children. 17 31

Brainstem tumors arise in portions of the rhombencephalon and mesencephalon. Some authorities include diencephalic tumors in this group. We have reviewed our clinical experience of 69 children (less than 21 years of age) with brainstem tumors evaluated and treated at Duke University Medical Center (DUMC) from 1960 to 1986. There were 19 patients with group 1 tumors (thalamus, third ventricle region, or midbrain) and 50 with group II tumors (pons, medulla oblongata). The common presenting signs and symptoms were ataxia, headache, motor loss, and cranial nerve palsies. The most commonly employed diagnostic imaging studies were air examinations and CT. Preradiotherapy confirmation of malignancy was obtained in five group I patients (astrocytoma, 4; germinoma, 1) and 8 group II patients (astrocytoma, 3; anaplastic astrocytoma, 2; glioblastoma multiforme, 3). All patients received radiotherapy. The 5-year survival rate for the entire population was 40%. The survival rate for group I patients was significantly better than that observed for group II patients. In the 50 group II patients neither patient sex nor age nor presence of cranial nerve palsies nor pretreatment CT scan findings nor field size influenced survival. A long duration of symptoms positively influenced survival. The vast majority of tumor recurrences were within the radiation field. Half of the patients had either stable or improved Karnofsky status 6 months following completion of irradiation. The management strategy for childhood brainstem tumors is discussed.
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PMID:Selection of a management strategy for pediatric brainstem tumors. 270 33

There have been conflicting opinions regarding the correct volume to be used in radiotherapy fields for brain stem tumors of childhood. Whereas many clinicians recommend limited fields designed to cover the tumor volume with a margin, some have advocated whole brain radiotherapy. Using our clinical experience at Duke University Medical Center, we have made an attempt to determine the proper irradiation volume in this group of tumors. We have evaluated 38 patients with brain stem tumors in children less than 18 years of age. The most common presenting symptoms were headache, ataxia, and hemiparesis. Thirteen patients had a histologic diagnosis made prior to treatment or post-mortem. All had either an anaplastic astrocytoma or a glioblastoma multiforme. Tumors were located in the thalamus, hypothalamus, or midbrain in 9 patients and in the pons or medulla oblongata in the remaining 29 patients. All patients received a course of radiotherapy. The mean minimum tumor dose was 52.6 +/- 5 Gy given at 1.7 to 2.0 Gy/fraction. Twenty-three patients received radiation to a limited field and 14 received whole brain irradiation. In one patient, the field size could not be ascertained. The five year survival of the total group was 39%. The survival of patients with thalamic, hypothalamic, or midbrain tumors was 73% compared with 28% for those with tumors of the pons or medulla oblongata (p = 0.0159). Eighty-eight percent of the tumor recurrences in evaluable patients (22/25) occurred within the radiotherapy fields. Patients were stratified for tumor location and no difference was observed in survival or relapse-free survival among those individuals treated with limited irradiation fields or whole brain irradiation fields. When our results are examined in conjunction with previously published data, the bulk of existing evidence supports the use of limited fields for irradiation of brain stem tumors of childhood.
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PMID:Pediatric brain stem tumors: patterns of treatment failure and their implications for radiotherapy. 298 30

A 74-year-old man was diagnosed with cerebellar glioblastoma multiforme (GBM). He initially presented with headache, nausea, vomiting, and truncal ataxia. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a round mass with ring enhancement in the vermis of the cerebellum. Subtotal removal of tumour was performed, and the pathological diagnosis was cerebellar glioblastoma multiforme. Subsequently, radiochemotherapy was performed. GBM of the cerebellum is rare and only accounts for a small fraction of all GBM. To our knowledge, there have only been 7 cases of senile cerebellar GBM reported. Our patient is one of the oldest cases recorded in the Asian literature.
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PMID:Glioblastoma multiforme of the cerebellum in an elderly man. 1536 8

Doppel (PRND) is a paralogue of the mammalian prion (PRNP) gene. It is abundant in testis and, unlike PRNP, it is expressed at low levels in the adult central nervous system (CNS). Besides, doppel overexpression correlates with some prion-disease pathological features, such as ataxia and death of cerebellar neurons. Recently, ectopic expression of doppel was found in two different tumor types, specifically in glial and haematological cancers. In order to address clinical important issues, PRND mRNA expression was investigated in a panel of 111 astrocytoma tissue samples, histologically classified according to the World Health Organization (WHO) criteria (6 grade I pilocytic astrocytomas, 15 grade II low-grade astrocytomas, 26 grade III anaplastic astrocytomas and 64 grade IV glioblastoma multiforme). Real-time PRND gene expression profiling, after normalisation with GAPDH, revealed large differences between low (WHO I and II) and high grade (III and IV) of malignancy (P<0.001). Extensive differences in PRND gene expression were also found within each grade of malignancy, suggesting that PRND mRNA quantitation might be useful to distinguish astrocytoma subtypes, and important in disease stratification and in the assessment of specific treatment strategies.
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PMID:Diagnostic value of PRND gene expression profiles in astrocytomas: relationship to tumor grades of malignancy. 1739 34

A 4-year-old Dutch warmblood mare was presented with a 10-month history of ataxia and proprioceptive deficits. Computed tomography defined a large, non-contrast enhancing mass in the left cerebral hemisphere. Necropsy examination revealed a tumour that effaced much of the piriform and temporal lobes. Microscopically the lesion was classified as a grade IV glioblastoma with an oligodendroglial component (GBM-O). The tumour was composed of highly pleomorphic cells organized in different patterns within a fibrillary stroma. There were multiple foci of necrosis. At the periphery of the tumour neoplastic oligodendroglioma-like cells were embedded in an extracellular mucinous matrix. Most neoplastic cells were strongly immunoreactive for glial fibrillary acidic protein; however, the oligodendroglioma cells did not express this marker. Cells forming microvascular proliferations were positively labelled for expression of factor VIII and smooth muscle actin. All neoplastic cells were negative for Neu-N and synaptophysin. The proliferation index was up to 5%. All neoplastic cells and normal brain tissue from the horse were uniformly negative for expression of epidermal growth factor receptor (EGFR), EGFR vIII mutant and the phosphatase and tensin homologue (PTEN) compared with positive control human GBM tissue. To our knowledge this is the first report of a GBM-O in the horse.
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PMID:A grade IV glioblastoma with an oligodendroglial component (GBM-O) in a horse. 1989 10

Multicentric Gliomas, both supratentorial and infratentorial, with varying histopathological picture is extremely rare. We report a unique occurrence of such a combination in a 50-year-old man who presented with features of elevated intracranial pressure, ataxia and vertigo. Magnetic resonance imaging showed a diffuse non-enhancing lesion in the temporal lobe and insula and another non-contigous well defined enhancing lesion in the cerebellum. Both the lesions had mass effect. The lesions were decompressed; first the temporal lesion and then the cerebellar lesion. Histopathology revealed grade II astrocytoma in the temporal lobe and glioblastoma multiforme in the cerebellum. He recieved whole brain radiotherapy with which he showed symptomatic improvement and survived for 1.5 years.
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PMID:Cerebellar glioblastoma multiforme with non-contiguous grade 2 astrocytoma of the temporal lobe in the same individual. 2073 16

We report the case of an 11 year old boy who presented with nausea, vomiting and ataxia. He was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI). Imaging demonstrated minimal enhancement and hemorrhage of a cerebellar mass. Cerebellar glioblastoma multiforme (GBM) is extremely rare in the cerebellum at any age but especially in children. The atypical findings of minimal enhancement, cerebellar location and hemorrhagic presentation combine to make the prospective diagnosis of GBM a difficult one. This rare combination of findings has not been previously reported.
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PMID:Pediatric cerebellar hemorrhagic glioblastoma multiforme. 2242 7

A 55-year-old gentleman with a left-sided glioblastoma multiforme (GBM) presented with palpitations which were shown to be atrial flutter (AFL) on ECG. Approximately 6 h later, he developed ataxia and expressive dysphasia. A brain CT scan showed no acute haemorrhage and no progression of the brain tumour. Ischaemic stroke was the top differential diagnosis. However, the authors could not perform thrombolysis due to the risk of intratumour haemorrhage. The AFL reverted to sinus rhythm with metoprolol and digoxin after 3 days. His neurological signs resolved within 24 h, and a diagnosis of transient ischaemic attack secondary to AFL was made. This case highlights the challenge of managing thrombo-embolic complications of arrhythmias in cancer patients.
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PMID:Atrial flutter in a patient with glioblastoma multiforme: problems in treating thromboembolic complications. 2269 88

Cerebellar glioblastoma multiforme (cGBM) is rare in adults, accounting for <1% of all patients with glioblastoma multiforme (GBM). The accurate diagnosis of cGBM is important for establishing a suitable therapeutic schedule. However, the diagnosis of cerebellar GBM is not usually suspected preoperatively because of its rarity. Generally, patients with cGBMs typically presented with symptoms of raised intracranial pressure, and infrequently cerebellar symptoms such as gait ataxia and disequilibrium. Nevertheless, the authors reported a cGMB patient, with his clinical presentations and imaging characteristics mimicking a vestibular schwannoma. To the best of our knowledge, this is the first reported patient with cGBM mimicking a vestibular schwannoma. Furthermore, the diagnosis, treatment, and prognosis for cGBM were broadly investigated.
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PMID:A Primary Cerebellar Glioblastoma Multiforme Mimicking Vestibular Schwannoma. 2751 87


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