[Differential diagnosis of metastatic and primary cerebellar neoplasms].
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.
A pigmented neoplasm of the cerebellar vermis in a four year old child was typical of differentiating medulloblastoma with islands of epithelial-like cells containing melanin pigment. There have been several previous reports of such melanotic cerebellar neoplasms. Reported cases have had a clinically malignant behavior with dissemination in the central nervous system. They appear to be variants of medulloblastoma and not pigmented neuroectodermal tumors of infancy (melanotic prognomas or retinal anlage tumors). Ultrastructurally the neoplasm was compatible with medulloblastoma with focal poorly differentiated cells which contained melanin pigment. The pigment resembled neural crest (cutaneous or ocular) melanin rather than neuromelanin.
Photoelectric eye movement recording in 9 patients with cerebellar disorders defined three features of saccadic overshoot dysmetria: (i) saccades were hypermetric and successively diminished in amplitude; (ii) saccadic initiation interval averaged 173 ms: and (iii) eye position was constant during the intersaccadic period. These characteristics indicated that the visually evoked saccades subserving foveation had increased gain, and were modelled by computer simulations using a sampled-data control model with increased feed-forward gain. Eight patients with saccadic overshoot dysmetria had cerebellar neoplasms, vermis-splitting surgical procedures and mid-line cerebellar signs. This clinical evidence suggests that vermian dysfunction is responsible for saccadic overshoot dysmetria. Normally, the cerebellar vermis appears to play an adaptive role by continuously adjusting gain of the direct visual motor pathway. When cerebellar disorder exists, adaptive gain modulation is lost, and, if gain then increases, saccadic overshoot dysmetria is a result.
Seven patients with histologically confirmed cerebellar hemangioblastomas are reviewed in order to establish computed tomographic (CT), angiographic and clinical characteristics and correlations. Noncontrast CT scans demonstrated most cystic lesions but did not always depict nodules on the cyst wall and small solid tumors. Contrast-enhanced CT scans demonstrated the solid and cystic types of tumors but in some cases angiography better delineated them and the presence of nodules. The CT appearance reflected the pathologic characteristics, which included vascular channels, cyst formation and nodules. Solid tumors enhanced homogeneously and could not be definitively distinguished from other cerebellar neoplasms by CT alone. One atypical undifferentiated tumor appeared as a solid mass with a necrotic center. The CT appearance of associated retinal angiomatosis is also described. Hydrocephalus, gait or appendicular ataxia was the initial clinical presentation in these patients.
A cerebellar neoplasm of an 18-month-old boy was examined with both the light and electron microscopes. The diagnosis of neuroblastoma was made on the basis of the presence of numerous synaptic vesicles in the great majority of cell processes and the occasional complete synapses within the tumor tissue.
Eye movements were studied with electro-oculography in a patient with primary position, large amplitude, upbeat nystagmus. The upbeat nystagmus increased in amplitude on upward gaze, decreased on downward gaze, and was not altered by loss of fixation. The patient could not produce smooth pursuit movements upward or to the left, but had normal saccadic and vestibular induced eye movements in all directions. At necropsy, a low grade glioma was found involving primarily the medulla and caudal pons. The inferior olives and prepositus hypoglossal nuclei were diffusely infiltrated with tumor. These results suggest (1) primary position upbeat nystagmus is due to a defect in the upward smooth pursuit system, (2) the lower brain stem at the level of the inferior olives and nucleus prepositus hypoglossi is important in the mediation of vertical pursuit, and (3) primary position upbeat nystagmus can result from damage to several nuclei and interconnecting pathways in the caudal brain stem and midline cerebellum involved in control of vertical smooth pursuit.
As the most frequent solid tumor occurring in childhood, brain tumors constitute an important segment of pediatric oncology. Neurologic manifestations may be deceptively mild and easily overlooked or misinterpreted, particularly in the very young, because of the remarkable resiliency of the immature central nervous system and the skull's ability to expand throughout the pre-adolescent years. The majority of childhood tumors produce increased intracranial pressure, usually the consequence of obstructive hydrocephalus. Specific neurologic deficits correspond to the tumor's location. The posterior fossa harbors two-thirds of childhood tumors, and each of the four common tumors in this location produces a characteristic syndrome. Supratentorial tumors occupy the cerebral hemisphere, the suprasellar area, and the pineal gland. Diagnostic studies have reached a state of great sophistication and precise anatomical localization. Surgery, either alone or with adjuvant radiotherapy, cures no more than one-third of all tumors; for the remainder, it has a diagnostic and palliative role. The introduction of operative microsurgery has advanced the art, particularly in the surgical treatment of craniopharyngiomas and pinealomas, but any significant improvement in the treatment of brain tumors as a group seems unlikely to be achieved by surgery alone.
One hundred forty-seven patients with astrocytoma were treated between 1942 and 1967. There were 25 postoperative deaths. The 14 patients in whom the tumor was thought tohave been completely removed were not irradiated and all survived 5 years or longer. Seventy-one of the 108 patients with imcompletely excised lesions received radiation therapy. The 5-year survival rate for those with imcomplete resection alone was 19%, compared to 46% when irradiation was given. Based on observations up to 20 years, after incomplete removal postoperative irradiation significally prolonged useful life and may have lead to permanent control in some. There was no evidence of radiation damage. Most of these tumors were fibrillary astrocytomas, and the results apply particularly to this histologic type. Only 1 of 11 patients with gemistocytic astrocytoma survivied 5 years. The survival rate for Grade i tumors was appreciably greater than for Grade iilesions; in both grades, it was improved by irradiation.
A case of multiple primary tumors in a 12-year-old girl is described. The tumors included a right optic nerve glioma, a pilocytic astrocytoma in the right globus pallidus, a ganglioglioma in the left globus pallidus and a medulloblastoma in the cerebellum. To our knowledge no such case has been reported so far in literature.
A case of primary intracranial melanoma in a 65-year-old female is described. A complete postmortem examination revealed multiple metastases in the liver, and eliminated the possibility of an extraneural primary melanoma. A review of the literature confirmed that such cases are highly exceptional, and that an exhaustive autopsy including particularly the eyes is indispensable to substantiate the diagnosis. When these conditions are fulfilled, there is no reason not to consider the eventuality of visceral deposits from a primary melanoma of the central nervous system.
Six patients with epithelial ovarian cancer had clinical diagnoses of central nervous system (CNS) metastases during a four-year period. A thorough review of the literature shows that involvement of the CNS by ovarian cancer is a rare phenomenon. Four of the six patients developed parenchymal brain metastases whereas carcinomatous meningitis was detected in the other two. These patients were younger and survived longer from initial diagnosis than the majority of women with ovarian cancer. Five of the six patients had previously received chemotherapy and had developed metastatic spread to extraperitoneal sites. Following extensive surgical resection, chemotherapy may provide intraperitoneal and systemic control, thereby extending survival and permitting occult CNS metastases to become apparent.
Multifocal pontine lesions were found at postmortem examination in four patients with various types of malignancy. The patients had undergone extensive evaluation and treatment with multiple chemotherapy regimens as well as radiotherapy to the central nervous system. The histologic character and striking anatomic distribution of the pontine lesions are described and their possible pathogenesis discussed.
Explore the source record for details and available documents.
CT has considerably facilitated the diagnosis of intracranial tumors, especially in infants and children. The authors review their experience with a series of 260 children with proven brain tumors. In most cases CT is so conclusive that other diagnostic techniques are no longer often needed. Pneumoencephalography in particular has largely been replaced by CT in recent years in the authors' departments, and the frequency of central ventriculography with contrast material has decreased substantially. Particular diagnostic difficulties with regard to tumors near to or within the caudal brain stem, some posterior fossa tumors, small vermian tumors, and tumors of the cerebral hemispheres are discussed.
The clinical course of a patient with mycosis fungoides, which could be confirmed histologically in the premycotic stage, 2 years after the onset of the disease is reported. The disease led to death after a further 3 years, during which the infiltrative and tumorous stage had appeared. At autopsy, apart from the skin changes typical for mycosis fungoides, involvement of the pia mater, plexus chorioideus, cerebrum and cerebellum was established. This constituted the only manifestation of the disease in the internal organ systems. There were no detectable neurological defects.
The accuracy of CAT in the diagnosis of infratentorial expanding lesions is analysed on the basis of 224 personal cases. The posterior fossa lesions are classified in four groups according to the reliability of CAT as the only pre-operative examination. The present indications for conventional neuroadiological procedures are also outlined. Particular emphasis is laid on the information supplied by CAT in postoperative follow up to detect both immediate and delayed complications.
Even after the introduction of the computerized tomogram, midline tumours in infants may give diagnostic problems. In the present study 41 children with tumours in the midline were investigated by both computerized tomography and encephalotomography. The results could be divided into two groups, according to the method and according to the localization of the space-occupying lesion. The diagnostic procedure may be restricted to computerized tomography in cases with direct signs (density differences, contrast enhancement). In cases showing indirect signs on the computerized tomogram (dislocation of ventricles or cisterns or both), further investigation would appear to be valuable. Encephalotomography is the method of choice in patients with negative or uncertain CT findings and contradictory clinical symptomatology.