OBJECTIVEInitial studies applying deep brain stimulation (DBS) of the posteromedial hypothalamus (PMH) to patients with pathological aggressiveness have yielded encouraging results. However, the anatomical structures involved in its therapeutic effect have not been precisely identified. The authors’ objective was to describe the long-term outcome in their 7-patient series, and the tractography analysis of the volumes of tissue activated in 2 of the responders.METHODSThis was a retrospective study of 7 subjects with pathological aggressiveness. The findings on MRI with diffusion tensor imaging (DTI) in 2 of the responders were analyzed. The authors generated volumes of tissue activated according to the parameters used, and selected those volumes as regions of interest to delineate the tracts affected by stimulation.RESULTSThe series consisted of 5 men and 2 women. Of the 7 patients, 5 significantly improved with stimulation. The PMH, ventral tegmental area, dorsal longitudinal fasciculus, and medial forebrain bundle seem to be involved in the stimulation field.CONCLUSIONSIn this series, 5 of 7 medication-resistant patients with severe aggressiveness who were treated with bilateral PMH DBS showed a significant long-lasting improvement. The PMH, ventral tegmental area, dorsal longitudinal fasciculus, and medial forebrain bundle seem to be in the stimulation field and might be responsible for the therapeutic effect of DBS.

Context: Pegvisomant is an effective treatment for somatostatin analogue-resistant acromegaly, but the determinants defining the response to this treatment are largely unknown. Objective: To investigate the efficacy of pegvisomant treatment in resistant acromegalic patients (e.g. serum IGF1 at least 1.25!upper normal limit) in a clinical setting and the factors conditioning this response. Design and setting: A retrospective cross-sectional study performed in six Spanish University hospitals from 2004 to 2007. Patients: Forty-four acromegalic patients (61.4% female, mean age: 49G14), 95% of whom had undergone pituitary surgery and 61% having received pituitary radiotherapy. The mean follow-up was 22.7G11.2 months. Main outcome measures: IGF1 levels reflected treatment efficacy, and the influence of gender, age, weight, previous radiotherapy and duration of treatment was assessed. Results: IGF1 normalisation was achieved in 84% of the patients. Male gender (P!0.05), previous irradiation (P!0.05) and the treatment duration (rZ0.364, P!0.02) were associated with a better response to pegvisomant therapy. There was a significant decrease in HbA1c (P!0.001) and in the mean insulin dose (P!0.01) in acromegalic diabetic patients. Although 25% of patients experienced mild adverse events, pegvisomant was only withdrawn in four patients due to side effects (two cases of tumour growth, one liver dysfunction and one headache). Conclusions: Long-term pegvisomant is a very effective therapy in resistant acromegaly. Male gender and prior radiotherapy influence the therapeutic response rate.

No abstract

No abstract

Solitary primary melanocytic tumors of the central nervous system (CNS) represent a spectrum of lesions ranging from well-differentiated melanocytoma to melanoma. The association of a meningeal melanocytoma with an ipsilateral nevus of Ota is extremely rare, with only six cases reported in the literature to date. Only a minority of melanocytic tumors correspond to lesions of intermediate-grade malignancy, whose biological behavior and outcome remain undetermined. We report a new case of a 25-year-old man with a giant fronto-temporal intracranial meningeal melanocytoma associated with a congenital nevus of Ota, who suffered an acute neurological deterioration requiring an emergent surgical procedure. Despite total removal of the lesion, the patient did not recover from the operation due to a malignant infarct of the right hemisphere. The lesion showed no histological criteria of malignancy but did exhibit an elevated (8%) MIB-1/Ki-67 cell proliferative index, which suggested an intermediate-grade melanocytoma. In addition, its metabolic profile, determined by in vivo proton magnetic resonance spectroscopy (MRS), was similar to that observed in most high-grade gliomas. To our knowledge, this is the first case of a meningeal melanocytoma of intermediate grade associated with a nevus of Ota reported in the literature. Only two additional intermediate-grade melanocytomas showing an elevated MIB-1/Ki-67 labeling index have been previously reported. The MIB-1/Ki-67 labeling index may have potential prognostic value in helping the clinician to predict an aggressive clinical behavior and/or malignant progression for primary melanocytic neoplasms of the CNS.