Risk factors for idiopathic cerebellar ataxia of late onset

In an attempt to identify risk factors for the development of idiopathic cerebellar ataxia (IDCA) we performed a case-control study of 59 IDCA patients. Hypertension and medicine intake were less frequent in IDCA than in neurological controls. Multiple logistic regression yielded an odds ratio (OR) for hypertension of 0.13 (95% confidence interval: 0.00–1.02, P=0.0527) and medicine intake of 0.10 (95% confidence interval: 0.00–0.72, P=0.0157). In contrast, we did not identify an association of IDCA with a number of medical diseases, head trauma, smoking, alcohol intake, rural living and well-water drinking. Some of these factors have been previously shown to be associated with other neurodegenerative diseases. In addition, serum antibody titers against neurotropic viruses were not elevated in IDCA.     

Detection of occult micrometastases in the bone marrow of patients with prostate carcinoma

A panel of three monoclonal antibodies that recognize membrane and cytoskeletal antigens expressed by epithelial cells (T₁₆′ C₂₆′ and AE-1) was used in a sensitive immunohistochemical assay to detect tumor cells in bone marrow aspirates from 20 patients with prostate cancer. Bone marrow aspirates from 2/9 (22%) patients with localized prostate cancer (stage B, 0/5; Stage C, 2/4), and 4/11 (36%) patients with metastatic prostate cancer (Stage D₁′ 0/7 patients; Stage D₂′ 4/4 patients) had antigen-positive cells in their bone marrow. The patients with localized disease had conventional examinations for metastases, including radioisotope bone scans and examination of bone marrow cytology, which were negative. The serum prostatic specific antigen (PSA) level appeared to correlate with the presence of micrometastases. Those patients with localized disease and antigen-positive cells in the bone marrow had an average serum PSA level of 26.6 ng/ml, while the average serum PSA level in patients without antigen-positive cells was 12.3 ng/ml. In addition, the number of antigen-positive cells detected appeared to correlate with the stage of disease; patients with Stage C prostate cancer had an average of 10 antigen-positive cells per one million bone marrow elements, while patients with Stage D2 disease had an average of 25 antigen-positive cells per one million bone marrow elements. We have demonstrated that immunohistochemical staining of bone marrow aspirates can detect occult bone marrow metastases in patients with apparently localized prostate cancer. Further follow-up of these and a larger number of patients will be required to determine the potential clinical significance of this finding. © 1994 Wiley-Liss, Inc.     

CT in autosomal dominant and idiopathic cerebellar ataxia

Signs of atrophy on cranial CT were investigated in 35 patients diagnosed as suffering from autosomal dominant (n=21) or idiopathic (n=14) cerebellar ataxia. Thirteen patients with a pure cerebellar syndrome were examined after at least 4 years of disease (mean duration 10.5 years) and were classified as cerebellar atrophy (CA). Twenty-two patients with additional non-cerebellar signs were classified as olivo-ponto-cerebellar atrophy (OPCA). Four (30%) of the patients with CA had atrophy of the brain stem in addition. Of the 22 patients with OPCA, 9 (40%) had atrophy of the cerebellum only. In patients with CA or OPCA correlation of clinical signs with severity of atrophy on CT was poor. Atrophy on CT often fails to differentiate autosomal dominant or idiopathic cerebellar ataxias in CA or OPCA: patients with CA can also have atrophy of the brain stem and patients with OPCA do not necessarily show brain stem atrophy.     

Esophageal carcinoma associated with neurofibromatosis—A case report

An unusual occurrence of esophageal carcinoma in a 58-year-old man with neurofibromatosis is reported. The patient underwent radical surgery, but died of multiple metastases to the bones, lungs, liver kidneys, adrenal glands and heart shortly after the operation. This paper contributes new information to documented cases of the association of malignancies to neurofibromatosis.     

Breast carcinoma metastatic to eyelids: case report and review of the literature

Violaceous, indurated plaques around both eyes were found to be cutaneous metastases and the initial clinical presentation of a lobular carcinoma of the breast in an 80-year-old woman. Available literature indicates that breast carcinoma is the most common metastatic tumor of the eyelid and the onset of a palpebral mass may precede the diagnosis of the primary tumor in the breast.     

A simple operative technique for chronically severing te cerebellar peduncles in neonatal rats

The method of anaesthesia and a new operative approach to allow chronic cutting of the cerebellar peduncles in neonatal rats by a relatively atraumatic method is described in detail. The operation allows a very quick postoperative recovery time, does not cause necrosis of the cerebellum, allows choice of partial or complete pedunclectomy, and there is no incidence of infection. A postoperative regime which has yielded a very low incidence of savaging by the mother is also described.     

Treatment of medulloblastoma in children: Long-term results following surgery,radiotherapy and chemotherapy

Summary Forty-seven children with histologically confirmed medulloblastoma are considered. Forty-five cases were surgically treated by direct approach to the tumour, while two cases were treated only with a shunt. A shunt was inserted preoperatively in 14 cases, postoperatively in 4 cases. Surgical resection was total or subtotal in 52% of cases, partial in 35%, and limited to a biopsy in 13%. Radiation treatment to the entire neuraxis was done in 37 cases: 10 of these cases received additional chemotherapy—mostly with CCNU—as primary treatment for medulloblastoma.Ten patients died within 30 days after surgery. Twenty-two patients died months after treatment, mainly from tumour recurrence (19 cases). One patient was lost to follow-up. Thirteen patients are survivors from 10 months to 20 years after treatment. As a whole, the one year survival rate has been 67%, 3 year survival 43%, and 5 year survival 27%. Complications affecting prognosis have been presented by tumour recurrence and metastases. CSF shunting and lack of prophylactic irradiation to the cerebral hemispheres have been considered responsible for the high incidence of supratentorial metastases in our series.Factors influencing prognosis have been the extent of tumour resection and association of primary chemotherapy with radiotherapy. Within 3 years after surgery survival has been 52% in cases with total resection against 31% in cases with partial resection of tumour. As regards chemotherapy, 3 year survival has been 60% for patients with combined treatment (chemo- and radiotherapy) against 37% in patients with radiotherapy alone. It is concluded that the best results in children with medulloblastoma are achieved by a radical resection, associated with a combined primary treatment of radiotherapy and chemotherapy.     

Symptomatic management and imaging of brain metastases

Summary Brain metastasis is the most common malignancy of the nervous system. Survival is short and the majority of patients die within 5 months after diagnosis. In this review, clinical and pathophysiological aspects of brain metastases are described, including novel radiological methods as triple-dose gadolinium-enhanced MRI. Recursive partitioning analysis is a powerful tool to analyse prognosis, and recent studies contribute to subgroup division. Subsequently, treatment choices can be made, based on prognostic characteristics of the individual patient. Commonly, symptomatic therapy starts with the administration of corticosteroids, often resulting in improvement of neurological deficit. Anticonvulsants are administered in patients with symptomatic epilepsy. The risk on vascular complications in patients with brain metastases is increased and needs special attention. Treatment of psychiatric complications e.g. delirium or depression may also improve quality of life.     

Assessing perfusion changes during whole brain irradiation for patients with cerebral metastases

Purpose: To assess perfusion changes within brain and correlate these with clinical symptoms during whole brain radiotherapy (WBRT) for cerebral metastases. Materials and methods: Fourteen patients with cerebral metastases underwent dynamic CT perfusion scans during palliative whole brain irradiation. Perfusion scans were performed on Day 1 prior to initial radiotherapy treatment, then on Day 2, and on Day 5 immediately after completion of radiotherapy. Measurements of cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT) and capillary surface area permeability product (PS) were performed for each perfusion scan, and daily symptom assessment was taken prior to initial perfusion scan and thereafter prior to each daily radiation treatment. Results: Utilizing Day 1 as baseline, a 19% increase (P=0.033) was noted in PS at Day 2, (median 1.47 ml/100 g/min), which returned to Day 1 range at Day 5 (median 1.31 ml/100 g/min). When symptoms were correlated with perfusion parameters, a statistically significant association between change in MTT with change in headache scores was observed, baseline to Day 2 (P=0.019), and a trend between change in nausea scores with change in CBV (P=0.059) as well as change in MTT (P=0.098), baseline to Day 5. Conclusion: This study has demonstrated the feasibility of a non-invasive technique to assess changes occurring within the human brain during a course of radiation treatment. Dynamic perfusion tomography provides insight into the pathophysiological processes taking place and allows correlation with patient symptomatology.     

Treatment of recurrent cerebellar hemangioblastoma with external radiotherapy in a patient with von Hippel-Lindau disease: a case report and review of the literature

Von Hippel-Lindau Disease, a multisystem familial cancer syndrome, is inherited as an autosomal-dominant trait. Common manifestations of the disease are retinal, cerebellar and medullary hemangioblastomas; renal cysts and carcinomas; pancreatic cysts; pheochromocytoma; and papilllary cystadenoma of the epididym. We report the case of a 40-year-old man with type I von Hippel-Lindau disease treated with external radiotherapy for recurrent cerebellar hemangioblastoma.