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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pontine gliomas have been considered to be out of indication for operative treatment. However, in case of a cystic type, evacuation of the cyst alone can possibly extend the survival time of the patient. Since the advent of high resolution CT the nature of the pontine tumor, whether cystic or solid, can be easily differentiated, and cystic ones subjected to operation will be increasing in number. We report a case of cystic pontine glioma associated with von Recklinghausen's disease. The patient showed a remarkable improvement in her neurological status after evacuation of the cyst. A 16-year-old girl was admitted to our clinic with complaints of tinnitus and hearing difficulty of the left ear, progressive gait disturbance and double vision. Neurological examination revealed a sensory disturbance on the left side of the face, left abducens palsy, left facial paresis, left deafness, left cerebellar ataxia, right hemiparesis and right hemisensory disturbance excluding the face. Signs and symptoms of increased intracranial pressure were absent. There were many cafe-au-lait spots and several subcutaneous nodules. CT scan demonstrated a cystic lesion with a mural nodule in the left cerebello-pontine angle. The patient underwent left suboccipital craniectomy, and a puncture of the cyst between the trigeminal and facial-acoustic nerves which were displaced dorsally yielded yellowish fluid. The content of the cyst was evacuated and its wall was widely opened. After the operation the patient showed a remarkable improvement in her neurological deficits only with left deafness remained unchanged. Histological examination showed anaplastic astrocytoma. She was discharged after irradiation of 5000 rads.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cystic pontine glioma associated with von Recklinghausen's disease--report of a case]. 643 42

A case of cryptococcosis simulating brain tumor was reviewed. A 66-year-old female was admitted to our hospital with chief complaint of vertigo, gait disturbance and dysarthria. These symptoms started about one year before admission and worsened. Vomiting and urinary incontinence appeared. Neurological examination revealed left cerebellar ataxia and dysarthria. In plain CT (computerized tomography) irregular ill-defined low density area was noted in the cerebellar vermis and bilateral cerebellar hemispheres. And slight ventricular dilatation was found. Irregular shape of ring-like enhancement corresponding to capsule and patchy or mottled enhancement inside the tumor were seen. Suboccipital craniectomy was performed and yellowish necrotic tumor with hard capsule was removed. Histological diagnosis was not neoplasm or tuberculoma. Postoperatively liver function progressively worsened. She died due to disseminated intravascular coagulation. Autopsy revealed typical liver cirrhosis without malignant change. 3.0 X 2.5 cm sized, slightly hard, yellowish lesion was found on upper part of cerebellar hemispheres. This had extremely necrotic tissue and a great number of cryptococcus neoformans were found. And other intracranial lesion was not confirmed. Finding of pulmonary cryptococcosis was not gained. Our case is very rare because of solitary cerebellar abscess and absence of meningitic episode or pulmonary cryptococcosis. There are three types of inflammation in cerebral cryptococcosis. The commonest manifestation is the meningitic type, the second mode is granulomatous lesion and the third and the least presentation is intracranial abscess formation. CT reveals various findings according to clinical stage. CT findings are those of meningitis, meningoencephalitis, granuloma and abscess. Cryptococcal granuloma or abscess often simulates brain abscess, glioma and metastatic brain tumor.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of cerebral cryptococcosis, with special reference to computerized tomography findings]. 646 65

Three children with myoclonic encephalopathy (Kinsbourne's disease) are described, in which one of them was shown to have ganglioneuroblastoma. Symptoms were opsoclonus, polymyoclonia of the striated muscles and cerebellar ataxia. Treatment consisted in corticosteroids and adrenocorticotropic hormone respectively in all patients, the patient with ganglioneuroblastoma also had a resection of the tumor. All patients responded to therapy, however recurrence of myoclonia and of the opsoclonus were seen after discontinuation or reduction of the corticosteroid dose, as well as following the course of intercurrent viral infections. Neurologic symptoms eventually disappeared after 3 1/2-5 1/2 years, however in two children behavioural abnormalities and disorders of speech and cognitive development remained.
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PMID:[Myoclonic encephalopathy (Kinsbourne syndrome)]. 647 68

A 29-year-old man developed acute cerebellar ataxia following Epstein-Barr virus infection. Serum IgG and IgM antibodies reacted with both nuclear and cytoplasmic elements of neurons. Western blot revealed IgG binding to the 34- and 29-kd bands and IgM binding to the 44-, 37-, and 29-kd bands. The IgM reactivity gradually reduced. There was no identifiable neoplasm and the ataxia gradually improved. These findings suggest a role for autoimmune mechanisms in the pathogenesis of acute cerebellar ataxia.
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PMID:Antineuronal antibodies in acute cerebellar ataxia following Epstein-Barr virus infection. 805 57

Dermoid cysts in the central nervous system are often associated with various congenital disorders, especially dermal sinus and spina bifida. We report a case of dermoid cyst in the fourth ventricle associated with Klippel-Feil syndrome. A 47-year-old man with a long history of headache had been known to have a cystic lesion in the posterior fossa for 12 years. When he was referred to our hospital with complaints of transient tetraparesis, he showed bilateral cerebellar ataxia and minimal left hemiparesis. Furthermore, he was noted to have a webbed neck with a low hairline and facial asymmetry. CT and MRI showed multiple cerebral infarctions as well as a mass lesion in the posterior fossa. Cervical roentgenogram showed a fusion of C 2 and C 3 vertebrae. The tumor was totally removed via a suboccipital approach, and the diagnosis was a dermoid cyst. The present patient had not only dermoid cyst and Klippel-Feil syndrome but also hypertrophy of the zygomatic bone. The pathogenesis of the Klippel-Feil syndrome is presumed to be an intrauterine defect, with a failure of segmentation of mesodermal somites. The zygomatic bone is also derived from the mesoderm somites at early fourth week, too. From these points of view, the disturbance in the mesoderm before the fourth week of gestation might have played an important role in causing a dermoid cyst.
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PMID:[Dermoid cyst in the fourth ventricle associated with Klippel-Feil syndrome]. 821 99

We reported a case of subacute sensory neuropathy. A 78-year-old woman was admitted to Kenwakai Hospital because of progressive numbness in her hands and feet. Four months before admission, numbness and tingling sensations appeared in her hands and feet, and subsequently she felt difficulty in skilled finger movement. On general examination, she was found to have a mass in the right lower abdomen. Neurological examination revealed marked loss of position sense and vibratory sense in the distal extremities, and mild reduction of sensation to pinprick and light touch in the distal extremities. Stretch reflexs were depressed in the upper limbs and absent in the lower limbs. Her gait was unsteady and Romberg's sign was positive. She showed no cranial nerve dysfunction, cerebellar ataxia, muscle weakness, and autonomic dysfunction. Blood examination revealed high TTT (11.3Kunkel U), high ZTT (16.4Kunkel U) titer. Tumor markers were normal except for CA125 (93 U/ml). The cerebrospinal fluid showed 48 mg/dl of protein, 10.6 mg/dl of IgG. and an almost normal cell count (5.3/mm3). Serum was tested by immunohistochemical staining. Only the cytoplasm of neurons in the dentate nucleus and brain stem was stained on a rat's brain. Sural nerve biopsy showed a severe loss of large myelinated fibers. An exploratory laparotomy revealed a peritoneal tumor, 5 cm in diameter, and it was removed. During the surgery, other than a few rice-sized nodules in the cul-de-sac, there was no evidence of tumor in bilateral ovaries. The tumor was proven to be a serous papillary adenocarcinoma with psammoma bodies resembling ovarian cancer.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of subacute sensory neuropathy associated with extraovarian serous papillary adenocarcinoma of the peritoneum]. 852 39

Ataxia-telangiectasia (A-T) is a multisystem recessive disease characterized clinically by cerebellar ataxia, oculocutaneous telangiectasias, immunodeficiency, sensitivity to radiomimetic agents, and cancer predisposition. This pleiotropic disorder is caused by mutations in the ATM (mutated in A-T) gene, which is located in the human chromosomal region 11q22-q23. The ATM gene product is a member of a novel family of large proteins implicated in the regulation of the cell cycle and response to DNA damage. Heterozygosity for A-T was previously suggested to be associated with an increased risk of tumors, particularly female breast cancer. Because of loss of constitutional heterozygosity at 11q22-q23 is a frequent event in breast and other tumors, suggesting the presence of a tumor suppressor gene(s) in this region, we screened blood DNA samples from 88 unrelated breast cancer patients of Swedish cancer families for ATM mutations using single-strand conformation polymorphism analysis. All patients had a family history of tumors previously associated with A-T heterozygosity or homozygosity. We demonstrate the first three germ-line mutations in ATM identified by screening of breast cancer patients. Two mutations were previously found in A-T homozygotes and one mutation was a 1-bp insertion. All mutations were found in families with a large number of tumors, however, they did not cosegregate with malignancies. Although the proportion of A-T carriers in this sample seems to be higher than expected by chance, larger studies and pooled data sets will be required to establish that an A-T allele confers cancer susceptibility in heterozygotes.
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PMID:ATM mutations in cancer families. 879 79

We report a 63-year-old man with progressive gait disturbance and dysarthria. The patient was apparently well until the age of 62 (February, 1990) when he noted unsteadiness of gait. Two months later, dysarthria appeared. He was admitted to Juntendo Izunagaoka Hospital on April 23, 1990. Neurologic examination revealed a mentally sound man with normal higher cerebral functions. Cranial nerves were unremarkable except for scanning speech. His gait was ataxic with positive Romberg sign. No motor weakness was noted, however, he had hypotonia and cerebellar ataxia. Deep tendon reflexes were retained and the plantar response was flexor. Pain, touch and vibration senses were diminished in the distal parts of the lower extremities. Laboratory examination revealed a 2.5 cm mass in the left lung field. Cranial MRI revealed a small T1-low and T2-high signal intensity lesion in the left temporal lobe. Abdominal CT scan revealed multiple low density lesions in the liver. His subsequent course was complicated by progressive deterioration in his gait and loss of deep tendon reflexes. He expired on November 24, 1990. The patient was discussed in the neurological CPC and the chief discussant arrived at the conclusion that the patient had anti-Hu associated paraneoplastic encephalomyelitis and sensory neuropathy. Some other participants thought that the patient had carcinomatous cerebellar degeneration. Postmortem examination revealed a 4x4 cm mass lesion involving the left S4-S5 segments. Histologic examination of the tumor was small cell carcinoma. Many metastatic foci were found in the liver. The cerebral hemispheres were unremarkable except for a small wedge-shaped tissue defect in the left temporal lobe which appeared to have been caused by old head trauma which the patient had received. The cerebellar vermis showed slight enlargement of cortical sulci, however, the cerebellar hemispheres appeared unremarkable. Upon histologic examination, marked loss of Purkinje cells was noted, particularly in the cerebellar anterior lobe. The dentate nucleus showed slight cell loss with increase in fat granule cells. The inferior olive was normal. The histologic characteristics were consistent with the pathologic diagnosis of carcinomatous cerebellar degeneration. No evidence of limbic encephalitis was seen. The peripheral nerve was not examined.
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PMID:[A 63 year-old man with progressive gait disturbance and dysarthria]. 888 38

A 39-year-old man was admitted with right hearing loss, tinnitus and vertigo. Neurological examination on admission revealed right facial palsy, right acoustic nerve disturbance and cerebellar ataxia. CT scan demonstrated a mass with intra-and extracranial extension in the pyramid bone concomitant with enlarged jugular foramen. MRI showed a ring-like enhanced, extra-axial mass in the right CP angle. Cerebral angiography showed no tumor stain. Venous phase of VAG revealed lateral displacement of the right sigmoidal sinus and obstruction of the internal jugular vein. Three dimensional CT was very useful to reveal enlarged jugular foramen. The tumor was resected totally and was approached through a right suboccipital craniectomy and mastoidectomy on July, 1994. Surgery confirmed that the tumor was a neurinoma originating from the glossopharyngeal nerve. After the operation, right facial palsy developed and transient fugitive CSF leakage was observed, but the patient is doing well. There was no amelioration of right hearing loss. JFN originating from the glossopharyngeal nerve is rare. Twenty-five cases of glossopharyngeal neurinoma are reviewed.
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PMID:[A case of jugular foramen neurinoma originating from glossopharyngeal nerve]. 899 Apr 67

We report a 29-year-old man with diabetes insipidus and cerebellar ataxia who developed spinal cord swelling 15 years after the onset. He was well until 14 years of the age when he noted dizziness. Two years after there was an onset of gait disturbance and slurred speech. He also noted polydipsia and polyuria. He was evaluated at the neurosurgery service of our hospital when he was 17 years of the age. Neurologic examination at that time revealed memory loss, horizontal nystagmus, cerebellar ataxic gait, dysmetria and decomposition more on the left. Cranial CT scan revealed a mass lesion involving the left subthalamic region and the head of the caudate area. Spinal fluid was unremarkable, however, human chorionic gonadotropin was increased to 27 mIU/ml. He was treated by radiation therapy (3,000 rads for total brain area and 5,460 rads for focal region). His CT scan and memory loss improved, however, cerebellar ataxia was unchanged. Three years after the radiation, he started to show choreic movement in his neck and left upper extremity. He was admitted to our service in August 14, 1995 when he was 29 years of the age. On admission, he was alert but disoriented to time; calculation was also poor. Higher cerebral functions were intact. The optic fundi were normal without papilledema. Visual field appeared intact. Gaze nystagmus was observed in all the directions, but more prominent in the horizontal direction. Speech was slurred. Otherwise, cranial nerves were unremarkable. Motor wise, he showed marked truncal and gait ataxia; he was unable to walk because of ataxia. Muscle atrophy and marked weakness was noted in both upper extremities more on the left side. Deep tendon reflexes were diminished in the upper extremities but active in the lower extremities. He was polyuric; urinary specific gravity was low. Spinal fluid contained 6 cells/cmm and 113 mg/ dl of protein; Queckenstedt was positive. MRI revealed swelling of the cervical cord; in addition, the entire cervical region and the medullar oblongata appeared as high signal intensity areas. No mass lesion was noted in the supratentorial structures but the third ventricle was markedly enlarged. Surgical biopsy was performed on the cervical lesion. The patient was discussed in neurologic CPC, and the chief discussant arrived at the conclusion that the patient had germinoma with syncytiotrophoblastic giant cells in the diencephalic region which appeared to have been cured by radiation therapy; he thought that the cervical lesion was the seeding of germinoma. Cerebellar ataxia was ascribed to the remote effect of germinoma. Most of the participants thought that the original tumor was germinoma and the cervical lesion was its spread. Some participants thought that his ataxia was caused by germinoma cells involving the medulla and the inferior cerebellar peduncles. Histologic observation of the biopsied tissue from the spinal cord revealed the typical two cell patterned germinoma. Most of the tumor cells were not stained for an antibody against HCG, but some tumor cells were positively stained. Germinoma is very radio-sensitive; this patient showed T2 high signal lesion involving the medulla oblongata and cervical cord continuously. Probably, tumor cells in the lower brain stem escaped radiation, and gradually spread to the spinal cord over many years. At the time of operation, the surface of the spinal cord was free from tumor cells. Therefore, tumor cells invaded the spinal cord continuously from the medulla oblongata. He was treated with cervical radiation, and his neurologic as well as radiologic findings showed marked improvement.
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PMID:[A 29-year-old man with diabetes insipidus and cerebellar ataxia and development of spinal cord swelling 15 years after the onset]. 916 63


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