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

The majority of pediatric neoplasias of the brain are midline growths in the posterior fossa. These mass lesions lead to obstruction of cerebrospinal fluid circulation and cause increased intracranial pressure. Affected children typically present with insidious complaints of headache and vomiting. Ataxia, cranial nerve palsies, or pyramidal tract signs may be present at the time the diagnosis is entertained. In the reports describing pathognomonic clinical features of posterior fossa tumors, an accelerated presentation with minimal prodromal events has not been emphasized. This report details the case of a child with a cerebellar medulloblastoma who presented with abrupt onset of fever, nuchal rigidity, and altered mental status. Emergency department misdiagnosis occurred.
 ...
PMID:Erroneous diagnosis within the cranial vault. 140 86

Two siblings in a family--a 5-year-old boy and an 8-year-old girl--suffered from progressive headache and gait disturbance in an interval of 1 year, consecutively. Neurologic manifestations were papilledema and truncal ataxia. Both of their computed tomography scans showed a large, well-enhanced tumor located in the cerebellar vermis with secondary hydrocephalus. Both had surgical resection followed by craniospinal irradiation and then chemotherapy. The pathologic findings confirmed the diagnosis of medulloblastomas. The family pedigree disclosed some other cancer in close relatives. These findings suggested a possible role of heredity in the oncogenesis of this tumor. To our knowledge, our cases are the seventh report of familial medulloblastoma occurring in nontwin siblings in the world.
 ...
PMID:Familial medulloblastoma in siblings: report in one family and review of the literature. 218 31

A case of a neuroblastoma occurring in the cerebellum of a 73-year-old man is reported. The patient presented with progressive truncal ataxia and was found to have an enhancing tumor mass in the cerebellar vermis. By light microscopy, the tumor was a small cell neoplasm and was similar to medulloblastoma, with areas showing structures suggestive of Homer-Wright pseudorosettes. By electron microscopy and immunoperoxidase techniques, however, the tumor showed convincing evidence of neuronal differentiation. The absence of previous reports of this tumor in the posterior fossa of adults suggests that immunoperoxidase techniques and/or electron microscopy of such small cell tumors may be required for accurate diagnosis.
 ...
PMID:Posterior fossa neuroblastoma occurring in an elderly man. 334 12

The case of a child presenting with Hodgkin's disease (stage I A, mixed cellular type) at the age of 23 months is reported. Chemotherapy (six treatments with MOPP) enabled a clinical remission, but an in situ relapse with the same histological pattern was observed at the age of 3 years and 9 months. After local irradiation, a second complete remission was observed. At the age of 6, a clinical picture of intraskull hypertension with ataxia, lead to the diagnosis of posterior cavity tumor. Complete excision was performed and the pathological examination showed a typical medulloblastoma. Adjuvant radiotherapy was carried out. Eight months later, a fatal plurifocal medullar relapse was observed. Second tumors in Hodgkin's disease are well known. However, this new case appeared to be the first report of a medulloblastoma after Hodgkin's disease.
 ...
PMID:[Medulloblastoma of the posterior cranial fossa. A second tumor after Hodgkin's disease]. 361 42

We analyze a series of 44 medulloblastomas in patients aged 20 and older, listed in the Connecticut Tumor Registry. Average age was 34.1 years. Incidence was 0.058 per 100,000 per year. Headache and ataxia were the most frequent presenting complaints. Findings at diagnosis most often were papilloedema, cranial nerve palsies, and cerebellar dysfunction. Treatments included operation (15%), radiation (7%), and operation plus radiation (60%). Probability of survival at one year was 0.62; at five years, 0.26. Survival with operation and radiation was superior to survival with other courses of treatment. Extracranial metastases to the spinal cord, the bones, and the pleura occurred in 27% of cases. For the first two years from diagnosis, survival in this series was significantly better than survival in the contemporary series of children with medulloblastoma. At five years and ten years, survival in adults and children was comparable.
 ...
PMID:Adult occurrence of medulloblastoma. 361

Among 100 childhood brain tumors treated at Kobe Children's Hospital from May 1970 to June 1985, 18 of the children presented with symptoms during the first year of life. This paper analyzes these 18 cases. Supratentorial tumors (78%) were more common than infratentorial ones, and 67% of all the tumors were located in the central neural axis. Initial symptoms were cranial enlargement (56%), vomiting (17%), cranial deformity (11%), blepharoptosis, respiratory distress, and ataxia. Histological diagnosis of the tumors was as follows: teratoma (3 cases), medulloblastoma (3), glioblastoma (2), astrocytoma (2), ependymoma (2), craniopharyngioma (1), choroid plexus papilloma (1), hamartoma (1), lipoma (1), melanotic progonoma (1), and an undetermined type, probably medulloblastoma (1). Seventeen of the patients underwent craniotomy for tumor resection (4 total, 4 subtotal and 7 partial removal, and 2 biopsies). Additional therapeutic methods used separately and in various combinations included ventriculoperitoneal shunt, subduralperitoneal shunt, ventricular drainage, radiotherapy and chemotherapy. Nine patients died (average 98 days) after surgery. Of the 9 survivors, 6 are still alive after more than 5 years. Five of the 6 are mentally retarded and 4 are physically handicapped to some degree.
 ...
PMID:Intracranial tumors in the first year of life. 377 67

A report is given on 113 tumours of the 4th ventricle in children (up to the age of 18 years) observed among a total of 1028 cerebellar operations. Of these, 87 (= 77%) tumours were exclusively tumours of the 4th ventricle, in 26 cases the tumour also encroached on adjacent structures (cerebellar hemisphere and vermis, pons and Medulla oblongata). Medulloblastomas were predominant (55%), they mainly occurred in school age. Leading clinical symptom of the tumours of the 4th ventricle - especially in child age - is the increase in intracranial pressure which can often be demonstrated radiologically in this age group. Besides there are coordination disturbances and disturbances of the cerebral nerves; relatively frequently one sees a "vermis syndrome". The initial symptoms, which are in most cases non-characteristic (nausea, headache, also ataxia), are more often than not misinterpreted. Surgically treatment aims at a radical removal of the tumour whenever of possible, which is frequently problematic in children, and the restoration of the pathways the cerebrospinal fluid. Microsurgical techniques clearly contributed to a decrease in the mortality rate which, however, still is very high. This stresses the importance of an improvement of early recognition of these tumours.
 ...
PMID:[Special diagnostic and surgical problems in tumors of the 4th ventricle in childhood]. 663 59

Brain tumors are, after leukemias, the most frequent fatal neoplasms of infancy. The clinical features and symptoms are often markedly different from those observed in the adult forms, according to the peculiar anatomy and behaviour of the child. Persistent headache, vomiting, astenia , behavioral alterations may be the precocious findings. Later, some more specific and suggestive signs such as strabismus, dyplopia , fast head size increase, funduscopic alterations, ataxia, paresis and nystagmus may be observed. On their appearance a prompt diagnostic work-up should be performed. The tumors of the posterior fossa (cerebellar astrocytoma and medulloblastoma, brainstem glyoma , hependimoma in decreasing order of frequency) generally cause precocious symptoms because of the small dimension of the subtentorial space; the presence in this region of several fundamental nuclei and pathways may explain how also small tumors may cause severe deficits. Supratentorial tumors (astrocytoma, malignant glyoma , hependimoma , craniofaringioma ) often show a more prolonged latency and may begin with signs of endocranial hypertension, seizures, or sometimes with hormonal troubles according to the involved anatomic structures. Hypothalamic astrocytoma is responsible of an extreme weight loss as far as to a cachetic status, due to the hyperincretion of GH. Finally, plexus papilloma, dermoid, optic nerve glyoma , oligodendroglyoma , germinoma, teratoma are responsible of a small number of child brain tumors, with different localization and symptomatology.
 ...
PMID:[Clinical course of brain tumors in childhood]. 673 95

A 3-year-old girl received conventional-dose external beam posterior fossa irradiation (5400 cGy in 30 fractions over 40 days) for good-risk medulloblastoma. Soon thereafter, she experienced an extraneural (occipital scar, cervical lymph nodes) and central nervous system (CNS) recurrence. Intensive cisplatin and cyclophosphamide chemotherapy led to rapid disappearance of the extraneural disease. Methotrexate was administered via a ventricular reservoir. After 2 months of chemotherapy, CNS toxicity progressed rapidly from ataxia to paraplegia to quadriplegia to central respiratory failure. Radiographic scans and autopsy material revealed brain stem necrosis. This unusual toxicity raises concern about the safety of aggressive systemic chemotherapy and intrathecal therapy, when given after conventional radiotherapy.
 ...
PMID:Fatal brain stem necrosis after standard posterior fossa radiation and aggressive chemotherapy for metastatic medulloblastoma. 850 76

A 4-year-old female, spayed Border Collie Dog was brought to the Veterinary Medical Teaching Hospital for evaluation of a progressive head tilt and ataxia that were unresponsive to therapy. Neurologic examination localized a right-sided lesion. The owner refused additional diagnostic tests, and necropsy was performed after euthanasia. Gross findings included atrophy of the temporal muscles and a moderately well delineated, 2.5- x 1.5- x 1.0-cm, gray soft-tissue mass compressing the right cerebellar hemisphere and dorsal hindbrain, resulting in massive dilatation of the lateral, third, and fourth ventricles and hydrocephalus. Histologic examination revealed two distinct features: undifferentiated, primitive, polygonal to fusiform cells with typical morphologic characteristics of medulloblastoma and interspersed areas containing myelinated axons and cells with glial and neuronal differentiation. Immunohistochemical examination confirmed the presence of primitive neuroepithelium and cells with glial and neuronal differentiation.
 ...
PMID:Cerebellar medulloblastoma with multiple differentiation in a dog. 982 98


1 2 3 4 5 Next >>