Lhermitte-Duclos disease: 11C-methionine positron emission tomography data in 4 patients.

BACKGROUND: Lhermitte-Duclos disease is a cerebellar lesion, characterized by an overgrowth of cerebellar ganglion cells, which replace granular cells and Purkinje cells. Lhermitte-Duclos disease may be a manifestation of Cowden syndrome (multiple hamartoma-neoplasia syndrome). The nature of LDD, whether neoplastic, dysplastic, or hamartomatous, is still not exactly understood. Metabolic imaging of the amino acid metabolism using PET could be useful for noninvasive characterization of these lesions. METHODS: To define the Meth-PET imaging characteristics of these lesions, we undertook a Meth-PET study in 4 patients with LDD after obtaining informed consent. All 4 patients had clinical signs of Cowden syndrome. In 2, the diagnosis was made with MRI; in 2, it was confirmed histologically. RESULTS: Using Meth-PET, the cerebellar lesions had a high methionine uptake, except in the subtotally resected lesion. The uptake of the lesions was markedly higher than that of the contralateral normal regions. The mean L/C ratio was 2.07. CONCLUSION: 11C-methionine positron emission tomography visualizes the lesion of Lhermitte-Duclos disease as a high uptake area. This amino acid hypermetabolism may be related to the slow growth of the lesions, and is an argument to suggest that patients with LDD should be followed up carefully to detect progression of the cerebellar lesion.

Early endovascular treatment of ruptured cerebral aneurysms in patients in very poor neurological condition.

OBJECTIVE: In patients in very poor neurological condition (World Federation of Neurosurgical Societies Grade V) with aneurysmal subarachnoid hemorrhage, early surgery to prevent rebleeding and to allow appropriate treatment of complications is often difficult. The aim of the present study was to evaluate whether early endovascular treatment followed by aggressive proactive treatment of complications (prophylactic hypervolemic hemodilution, hypertensive treatment in the event of systemic hypotension, and appropriate treatment of intracranial hypertension) is an acceptable management strategy for these patients. METHODS: We prospectively studied 11 consecutive patients who presented with acutely ruptured aneurysms and were in very poor neurological condition after resuscitation (World Federation of Neurosurgical Societies Grade V) but did not have a significant intracerebral hemorrhage. These patients received endovascular treatment with Guglielmi detachable coils (Boston Scientific/Target, Fremont, CA). Follow-up consisted of a clinical evaluation based on the Glasgow Outcome Scale. A control angiogram was obtained after 6 months in patients with favorable outcomes to evaluate the occlusion of the aneurysm. RESULTS: There were no deaths or complications directly related to the procedure. Two patients died as a consequence of increased intracranial pressure. The mean follow-up of the surviving patients was 12 months. Two patients had early rebleeding after the coiling and required further treatment. Four patients had good outcomes, two patients were moderately disabled, and three patients were severely disabled. CONCLUSION: This study demonstrates that early endovascular treatment of acutely ruptured cerebral aneurysms in patients evaluated as World Federation of Neurosurgical Societies Grade V allows for aggressive treatment of intracranial hypertension and vasospasm. More than half of the patients had favorable outcomes. Therefore, early endovascular treatment seems to be a valuable alternative to early surgery in patients who present with a very poor clinical grade after subarachnoid hemorrhage. The results of this study are promising but must be interpreted with caution, because a small number of patients were studied.

Transient local response and persistent tumor control in a child with recurrent malignant glioma: treatment with combination therapy including dendritic cell therapy. Case report.

Treatment of malignant glioma is difficult and discouraging. Even after resection and maximal adjuvant therapy, the prognosis remains poor. The authors sought a novel form of treatment, such as stimulating the patient's own immune response against the tumor, and developed a protocol of tumor vaccination in which autologous dendritic cells (DCs) were used in patients with recurrent malignant glioma. A 4-year-old girl was treated by means of biopsy sampling and radiotherapy for a rolandic low-grade glioma. Ten years later, a Grade III recurrence was discovered and treated with subtotal resection, interstitial radiation, six courses of oral temozolomide, and 12 courses of oral VP 16. At the end of the chemotherapy cycle, a new rapidly growing recurrence was diagnosed. A macroscopically complete resection was performed. Afterward, the girl was vaccinated with autologous DCs that had been pulsed ex vivo with the homogenate of the resection specimen. She received six vaccines in total. The efficacy of immunization was checked by a positive delayed-type hypersensitivity skin reaction after the second injection. After the fifth vaccine, a transient contrast enhancement without mass effect was visualized on magnetic resonance imaging. Simultaneously, positron emission tomography imaging revealed a transient increase of metabolic activity around the resection cavity, but the metabolic uptake ratio remained below 1.8. The patient's disease is still in complete remission 24 months after the last surgery. She is clinically well with minor and stable left hemiparesis. This case report illustrates the potential of vaccination with DCs loaded with crude tumor homogenate as adjuvant therapy to induce prolonged tumor control of malignant glioma and the objective noninvasively monitored immune response against infiltrating tumor cells.

Controlled study of nimodipine in treatment of patients with diffuse axonal injury.

OBJECTIVE: To determine the efficacy and safety of early intervention with nimodipine treatment in diffuse axonal injury. METHODS: Based on the characteristic radiological signs and criteria for diffuse axonal injury (DAI), 89 patients with the diagnosis of DAI were enrolled in this randomized, double-blind, placebo-controlled trial. RESULTS: Nimodipine proved to be safe and well tolerated. With TCD sonography we found that there was a higher incidence of cerebral vasospasm in this series (38.2%). Overall, Nimodipine produced a better clinical result than placebo, but there was no statistically significant difference in favorable outcome at 3 months after injury (P=0.11) between the two groups. A trend toward a favorable effect was suggested by the analyses in two small subgroups, either in the patients suffering from clinical Grade III DAI (P=0.04), or in those with the TCD-evidence of cerebral vasospasm during clinical observation (P=0.049). CONCLUSIONS: We postulate that a clinically valuable benefit is possible with nimodipine treatment in DAI patients. However, the effects on outcome should be verified by further controlled study.

Anatomy of the anterior temporal lobe and the frontotemporal region demonstrated by fiber dissection.

OBJECTIVE: The white matter structure of the anterior temporal lobe and the frontotemporal region is complex and not well appreciated from the available neurosurgical literature. The fiber dissection method is an excellent means of attaining a thorough knowledge of the three-dimensional structure of the white matter tracts. This study was performed to demonstrate the usefulness of the dissection technique in understanding the white matter anatomy and the effects of current surgical approaches on the subcortical structure of the region. METHODS: Seventeen brain specimens obtained at routine autopsy were dissected by use of Klingler's fiber dissection technique after preparation by fixation and freezing. The dissections were performed with an operating microscope and followed a stepwise pattern of progressive white matter dissection. RESULTS: The dissection is described in an orderly fashion showing the white matter tracts of the anterior temporal lobe and the frontotemporal region. An insight is gained into the three-dimensional course of the anterior loop of the optic radiation, the temporal stem, the anterior commissure, and the ansa peduncularis. CONCLUSION: The anterior temporal lobe and the frontotemporal region contain several important white matter tracts that can be uniquely understood by performing a white matter dissection of the region. Surgical procedures on the anterior temporal lobe differ substantially as to their repercussions on the subcortical white matter tract anatomy, as shown by the findings in this study.