The movement disorder of adult opsoclonus.

We present three cases of the adult opsoclonus-myoclonus syndrome in patients with systemic carcinoma. In addition to opsoclonus and myoclonus, other clinical components of the syndrome can include ataxia, tremor, gait and stance dysfunction, altered mental status, and head and face dyskinesias. The most common etiologies are idiopathic, paraneoplastic, and infectious encephalitis. Radiographic and pathological studies suggest brain-stem dysfunction with associated cerebellar and/or cerebellar pathway dysfunction. In many cases, there is evidence for the involvement of immunologic and/or inflammatory processes in the pathogenesis of this syndrome. The timely recognition of this syndrome is important because of its implications for the underlying etiology and prognosis. The appearance of this syndrome should prompt the search for an occult malignancy.

Supratentorial pilocytic astrocytomas. A clinicopathologic, prognostic, and flow cytometric study of 51 patients.

BACKGROUND: Supratentorial pilocytic astrocytomas are uncommon tumors, the prognosis and management of which have been controversial. METHODS: The authors retrospectively studied the clinical and pathologic aspects of 51 patients with supratentorial pilocytic astrocytomas, with particular reference to their response to treatment. RESULTS: The study group consisted of 25 male and 26 female patients (median age, 15 years). The extent of surgical removal was gross total or radical subtotal in 16 patients and subtotal removal or biopsy in 35. At a median follow-up of 14.9 years, 41 patients were alive. Overall survival was 82% at 10 and 20 years; 89% of surviving patients were fully active. Survival at 10 years was 100% for the 16 patients who underwent gross total or radical subtotal removal and 74% for the 35 who had subtotal tumor removal or biopsy. No association was found between pathologic features such as histologic grade or flow cytometric characteristics and patient survival. CONCLUSIONS: Supratentorial pilocytic astrocytomas behave differently from low-grade astrocytomas of the diffuse or fibrillary type in that they have a favorable prognosis. Radiation therapy appears not to be needed for patients undergoing gross total removal. In patients undergoing subtotal resection, close follow-up with additional surgery or radiation therapy at the time of tumor progression is recommended. Patients who have biopsy only should receive radiation therapy. Histologic grading and flow cytometric analysis add little to clinical decision making with regard to patients with pilocytic astrocytoma.

Preliminary communication--treatment of primary brain tumors recurrent after irradiation with aziridinylbenzoquinone (AZQ;NSC-182986).

Twenty-nine patients with primary brain tumors recurrent or progressive after cerebral irradiation were treated with AZQ. Twenty of the 29 patients had also failed prior chemotherapy. CT scan-documented tumor regressions were noted in 17.2% (5/29) and ranged from 15.0% (3/20) in patients with prior chemotherapy to 22.2 (2/9) in patients without prior chemotherapy. Myelosuppression was the only significant toxicity noted. AZQ is worthy of further studies in patients with primary brain tumors.

Phase II studies of dianhydrogalactitol-based combination chemotherapy for recurrent brain tumors.

The drug combinations of dianhydrogalactitol and VP-16 and dianhydrogalactitol, VP-16, and triazinate were used in patients with primary brain tumors, principally astrocytoma, recurrent following cranial irradiation. Tumor regressions were noted in 40% of patients treated with the 2-drug regimen and in 33% of patients treated with the 3-drug regimens. Regression were noted in all grades of tumor. Poor performance score on the patients' part did not seem to effect regression rates. Myelosuppression was the principal toxicity encountered. Dianhydrogalactitol-based combination chemotherapy seems as active as nitrosourea therapy and presents an alternative to nitrosourea therapy.

Dianhydrogalactitol and radiation therapy. Treatment of supratentorial glioma.

Dianhydrogalactitol was the most active of 177 agents tested against a mouse ependymoblastoma tumor. We conducted a prospectively randomized trial comparing whole-brain irradiation alone vs identical irradiation plus dianhydrogalactitol in 42 patients with grade 3 and 4 supratentorial astrocytomas. Patients receiving dianhydrogalactitol in addition to irradiation had a significantly longer median survival time (67 vs 35 weeks) than did patients receiving only irradiation. The major toxic effect of dianhydrogalactitol is hematologic suppression of a cumulative nature. Dianhydrogalactitol may play an important role (in conjunction with radiation therapy) in the initial treatment of patients with supratentorial glioma. Our data may indicate that the mouse ependymoblastoma system is a useful screen for agents to be used in the treatment of human glioma.

The diagnosis and natural history of spinal cord arteriovenous malformations.

The present study of 71 patients shows that the initial symptoms often cannot differentiate spinal cord arteriovenous malformation from other lesions causing cord dysfunction, but the picture at the time of presentation may suggest the diagnosis. Most patients are males with neurologic findings referable to the thoracolumbar area who present with gradually progressive pain, weadness, sensory distubance, and disturbance of micturition. Early impairment of micturition may help suggest this lesion because it is less likely to be an early complaint in patients with disk disease or tumor affecting the spinal cord. Symptoms occasionally vary with posture and exercise and menses. Most commonly there are combined upper motor neuron and lower motor neuron manifestations with nonradicular sensory deficit. The cerebrospinal fluid is abnormal in more than 75% of cases. The myelogram is positivie in 75 to 90% of cases and the angiogram is almost always diagnostic.

Controlled study of CCNU and radiation therapy in malignant astrocytoma.

The authors report 63 patients with biopsy-proved malignant (Grades 3 and 4) astrocytomas who were randomly placed in one of three treatment schedules within 2 weeks of surgery. One group (22 patients) received radiation therapy alone; the second group (22 patients) received 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) orally at intervals of 8 weeks; and the third group (19 patients) received combined radiation and drug therapy. Patients who received radiation therapy, with or without the drug, had a significantly longer survival than did those who received the drug alone. There was no difference in survival between the two groups who received radiation. The nitrosourea derivative CCNU does not seem to be an effective agent in the therapy of primary malignant brain tumors.

An unusual compressive syndrome of the cauda equina.

An unusual case of a capillary hemangioma of the filum terminale is presented. The successful removal returned the patient to an asymptomatic state.