Bilateral periventricular nodular heterotopia with amniotic band syndrome

The amniotic (constriction) band syndrome is characterized by distal ring constrictions, intrauterine amputations, and acrosyndactyly. External constriction by amniotic bands is the generally accepted mechanism: early amniotic rupture leads to formation of mesodermal fibrous strands that constrict, entangle, and amputate distal portions of limbs. Etiology is heterogeneous. Anecdotal cases involving central nervous system abnormalities (e.g., acrania, anencephaly, polymicrogyria, congenital bilateral perisylvian syndrome, neuronal heterotopia, septo-optic dysplasia, and spinal cord tethering) have been reported. We describe a 9-year-old girl with typical features of constriction band syndrome localized to the lower limbs who had also profound mental retardation and drug-resistant epilepsy associated with bilateral periventricular nodular heterotopia (a brain malformation of neuronal migration and proliferation caused by mutations in the X-linked filamin 1 gene [FLN1] on chromosome Xq28). The karyotype was normal, as was mutational screening for FLN1. The occurrence of bilateral periventricular nodular heterotopia in the context of amniotic band syndrome is novel (chance occurrence of both: 0.000004%).     

Chronic treatment with the selective NOP receptor antagonist [Nphe1,Arg14,Lys15]N/OFQ-NH2 (UFP-101) reverses the behavioural and biochemical effects of unpredictable chronic mild stress in rats

Introduction

The present study was designed to assess the antidepressant effects of UFP-101, a selective nociceptin/orphanin FQ peptide (NOP) receptor antagonist, in a validated animal model of depression: the chronic mild stress (CMS).

Materials and methods and Results

UFP-101 (5, 10 and 20 nmol/rat; i.c.v., once a day for 21 days) dose- and time-dependently reinstated sucrose consumption in stressed animals without affecting the same parameter in non-stressed ones. In the forced swimming test, UFP-101 reduced immobility of stressed rats from day 8 of treatment. After a 3-week treatment, rats were killed for biochemical evaluations. UFP-101 abolished increase in serum corticosterone induced by CMS and reverted changes in central 5-HT/5-HIAA ratio. The behavioural and biochemical effects of UFP-101 mimicked those of imipramine, the reference antidepressant drug, administered at the dose of 15 mg/kg (i.p.). Co-administration of nociceptin/orphanin FQ (5 nmol/rat, from day 12 to 21) prevented the effects of UFP-101. Brain-derived neurotrophic factor mRNA and protein in hippocampus were not reduced by CMS nor did UFP-101 modify these parameters.

Discussion and Conclusion

This study demonstrated that chronic treatment with UFP-101 produces antidepressant-like effects in rats subjected to CMS supporting the proposal that NOP receptors represent a candidate target for the development of innovative antidepressant drugs.     

Functional reinnervation of the canine bladder after spinal root transection and genitofemoral nerve transfer at one and three months after denervation

ABSTRACT In the immediate management of patients with spinal cord injury (SCI), patients are typically observed for a period of time to determine whether voluntary control of bladder function returns. Therefore, bladder reinnervation surgeries are not likely to be performed immediately after the injury. We performed genitofemoral to pelvic nerve transfer (GF NT) surgery in canines at 1 and 3 months after bladder denervation (transection of S1 and S2 spinal roots) to determine whether this type of bladder reinnervation surgery has potential clinical feasibility. Nerve cuff electrodes were implanted on the genitofemoral nerves proximal to the pelvic nerve transfer site. Evidence for bladder reinnervation includes (1) increased bladder pressure and urethral fluid flow following electrical stimulation in four out of 20 nerve cuff electrodes implanted on the transferred GF nerves, (2) bilateral pelvic nerve stimulation induced bladder pressure and urethral fluid flow in three of four denervated animals with 1-month delay GF NT, and in five of six denervated animals with 3-month delay GF NT, and (3) abundant L1 and L2 spinal cord cell bodies (the origin of the GF nerve) retrogradely labeled with fluorogold injected into the bladder in all 10 of the GF NT animals, except one animal on one side. This study presents initial proof of concept that GF NT is a potentially viable clinical approach to reinnervation of the lower motor neuron-lesioned urinary bladder.     

Effects of using the posterior or anterior approaches to the lumbar plexus on the minimum effective anesthetic concentration (MEAC) of mepivacaine required to block the femoral nerve: a prospective, randomized, up-and-down study

BACKGROUND AND OBJECTIVES: To evaluate if psoas compartment block requires a larger concentration of mepivacaine to block the femoral nerve than does an anterior 3-in-1 femoral nerve block. METHODS: Forty eight patients undergoing anterior cruciate ligament repair were randomly allocated to receive an anterior 3-in-1 femoral block (femoral group, n = 24) or a posterior psoas compartment block (psoas group, n = 24) with 30 mL of mepivacaine. The concentration of the injected solution was varied for consecutive patients using an up-and-down staircase method (initial concentration: 1%; up-and-down steps: 0.1%). RESULTS: The minimum effective anesthetic concentration of mepivacaine blocking the femoral nerve in 50% of cases (ED(50)) was 1.06% +/- 0.31% (95% confidence interval [CI], 0.45%-1.68%) in the femoral group and 1.03% +/- 0.21% (95% CI, 0.6%-1.45%) in the psoas group (P = .83). The lateral femoral cutaneous and obturator nerves were blocked in 4 (16%) and 5 (20%) femoral group patients as compared with 20 (83%) and 19 (80%) psoas group patients (P = .005 and P = .0005, respectively). Intraoperative analgesic supplementation was required by 15 (60%) and 5 (20%) patients in the femoral and psoas groups, respectively (P = .01). CONCLUSIONS: Using a posterior psoas compartment approach to the lumbar plexus does not increase the minimum effective anesthetic concentration of mepivacaine required to block the femoral nerve as compared with the anterior 3-in-1 approach, and provides better quality of intraoperative anesthesia due to the more reliable block of the lateral femoral cutaneous and obturator nerves.     

The minimally invasive surgical approach in thyroid diseases

AIM: The targets of minimally invasive thyroidectomy could be summarised by: achievement of the same results as those obtained with traditional surgery, better postoperative course and improved cosmetic RESULTS: In minimally invasive surgical approach the skin incision should not exceed 30 mm in length. In our experience this limit may be extended of 5 mm for thyroid between 25 and 50 mL in volume. This way allows more patients, excluded before, to take the advantages of minimally invasive approach. The aim of this work has been to demonstrate that the central neck minimally invasive approach is safe, less painful, better for cosmetic results and easily reproducible in surgical practice. METHODS: From January 2003 to June 2007, 75 patients have been selected for minimally invasive thyroidectomy. The procedure was carried out through a central skin incision performed 'high' between the cricoid and jugular notch. Our 'modified Miccoli-procedure' consists in five-easily repeatable steps. In the postoperative stay, all patients were asked to evaluate the pain that feel and the cosmetic result by means of a numeric scale. RESULTS: The skin incision performed was from 25 to 30 mm (mean 27.39 +/- 2.6 mm). We obtained in all cases excellent results about patients cure rate and comfort, few postoperative pain and attractive cosmetic RESULTS: CONCLUSION: In this study we demonstrate that the central neck minimally invasive approach is safe, less painful, better for cosmetic results, with less paresthetic consequences and easily reproducible in surgical practice. In our opinion a longer incision (up to 35 mm), does not affect negatively the advantages of minimally invasive procedure. This way allows more patients to take the advantages of minimally invasive approach.     

Effect of desferrioxamine and hydroxypyridones on hemopoietic progenitors and neuroectodermal tumor cells

The iron chelator desferrioxamine (DFO) has been shown to inhibit the proliferation of hemopoietic progenitors and several tumor cell lines. We have compared the in viro hemopoietic inhibitory effect of desferrioxamine (DFO) and hydroxypyridones (HPOs) on hemopoietic progenitors and two human neuroectodermal (NE) tumor cell lines, NB 100 and SKNMC. Both DFO and HPOs showed a direct dose-related inhibitory effect on BFU-E and CFU-GM obtained from purified human non-T MNAC (T-Iymphocyte-depleted nonadherent mononuclear cells) and CD34+ cells. DFO and HPOs displayed both an inhibitory and a cytotoxic effect on NE cell lines. We calculated the ratio between NE cell and hemopoietic cell growth inhibition for a range of concentrations of chelators. DFO showed the most satisfactory ratio. This suggests that DFO is still the most preferable chelating agent for the treatment of neuroblastoma, since it combines the highest antineuroblastoma effect with the lowest hematopoietic toxicity. © 1994 Wiley-Liss, Inc.     

Combination of two different dopamine agonists in the management of Parkinson's disease

Neurological Sciences - An alternative approach to the symptomatic treatment of parkinsonian patients with and without motor fluctuations is to use dual dopamine agonists. The aim of this study was...     

Antagonist effect of terguride in Parkinson's disease.

The effects of the partial dopamine agonist terguride (9,10 transdihydrolisuride; THDL) on striatal dopamine receptors were studied by its i.v. administration to 13 patients with Parkinson's disease. Patients were maintained in a steadily mobile state with abnormal involuntary movements by a constant i.v. infusion of levodopa. Terguride showed dopamine antagonist properties in nine patients. In two of these nine patients, a decrease in dyskinesia score was observed without a concomitant worsening of parkinsonian symptoms, whereas in the remaining seven, full parkinsonian akinesia followed THDL administration. The subsequent i.v. injection of the dopamine agonist lisuride reversed THDL-induced akinesia in these seven patients. In the remaining four patients, no clinically significant motor effects were observed. These results show dopamine antagonist activity of terguride in patients with Parkinson's disease treated with Levodopa. Further studies using a wider dose titration are required to evaluate the possible role of dopamine partial agonists in the therapy of levodopa-induced dyskinesias.     

Non-random spatial relationships between mast cells and microvessels in human endometrial carcinoma

Mast cells (MCs) accumulate in the stroma surrounding tumors, where they secrete angiogenic cytokines and proteases, and an increased number of MCs have been demonstrated in angiogenesis associated with solid and hematological tumors. The aim of this study is to contribute to the knowledge of distribution of MCs in tumors, investigating the pattern of distribution of tryptase-positive MCs around the blood vessels in human endometrial carcinoma samples by introducing a quantitative approach to characterize their spatial distribution. The results have shown that in human endometrial cancer bioptic specimens the spatial distribution of MCs shows significant deviation from randomness as compared with control group in which, instead, the spatial distribution of MCs is consistent with a random distribution. These findings confirm that MCs enhance tumor angiogenesis and their preferential localization along blood vessels and sites of new vessel formation sustaining the suggestion for an association between MCs and angiogenesis. However, this spatial association between vessels and MCs might simply reflect migrating MCs from the blood stream at vessel growing sites.