Neuropsychological aspects of benign childhood epilepsy with centrotemporal spikes

PurposeTo establish whether the disability in benign epilepsy with centrotemporal spikes (BECTS) is the result of the number of seizures, the anti-epileptic therapy or is an inherent characteristic of the syndrome itself.MethodsThirty-six children with BECTS were tested for cognitive functions prior to commencing treatment with anti-epileptic drugs, and the findings were compared with those in 15 children with normal electroencephalograms, performed for unrelated reasons. The data in the study group were further correlated with the laterality of the epileptic focus and the number of seizures.ResultsScores for verbal functioning on neuropsychological tests were significantly lower in the study group than the control group. There was no relationship between the neuropsychological scores in the patients and either lateralization of the epileptic focus or number of seizures.DiscussionChildren with BECTS have an impaired ability to process verbal information. The deficiency is apparently a result of the pathological electrical discharges that are part of the syndrome and are not dependent on the epileptic focus laterality, the number of seizures, or the anti-epileptic treatment.     

Benign angiopathy of the central nervous system presenting with intracerebral hemorrhage

BACKGROUND: Primary central nervous system vasculitis has traditionally been described as an aggressive condition, with significant morbidity and mortality. A subgroup of patients has been identified who have a similar clinical presentation, but with a benign course. This syndrome of BACNS is successfully treated with low-dose steroids and calcium-channel blockers. Histologic confirmation, when performed, is normal. METHODS: Intracerebral hemorrhage is a rare presenting finding in the setting of BACNS. We present 2 patients with acute onset of headache and neurologic impairment secondary to an ICH. RESULTS: Cerebral angiography showed characteristic findings of diffuse vasculitis. Both patients were subjected to biopsy and both failed to reveal evidence of vasculitis. CONCLUSION: This report is the first to document the normal histologic features of BACNS in the setting of an ICH. Although these angiographic changes are similar to vasculitis, these processes can be differentiated on clinical grounds, which is of vital importance as the treatment and clinical course of BACNS is more benign. Furthermore, the presence of an ICH in the setting of vasoconstriction seen on angiography may represent a novel feature in some patients with BACNS and is not necessarily a harbinger of the more malignant PCNSV.     

Blood transfusion and lung function in children with thalassemia major.

Pulmonary function tests were performed at rest and following blood transfusion on 17 patients with thalassemia major. At rest, diffusion capacity was significantly reduced (mean of 57 percent), and forced vital capacity was mildly reduced (mean of 85 percent). In two of the patients, arterial oxygen tension was low. Other pulmonary function results were in the normal range. Following blood transfusion, a significant drop was seen in arterial oxygen tension (PaO2 of 22 mm Hg) and in forced vital capacity (FVC) of 32 percent. These changes were dramatic and require further explanation.     

Tuftsin–phosphorylcholine (TPC) equally effective to methylprednisolone in ameliorating lupus nephritis in a mice model

The role of helminth treatment in autoimmune diseases is growing constantly. Systemic lupus erythematosus (SLE) is a multi‐system autoimmune disease with challenging treatment options. Tuftsin–phosphorylcholine (TPC) is a novel helminth‐based compound that modulates the host immune network. This study was conducted to evaluate the potential value of TPC in ameliorating lupus nephritis in a murine model and specifically to compare the efficacy of TPC to the existing first‐line therapy for SLE: corticosteroids (methylprednisolone). Lupus‐prone NZBxW/F₁mice were treated with TPC (5 µg/mouse), methylprednisolone (MP; 5 mg/body weight) or phosphate‐buffered saline (PBS) (control) three times per week once glomerulonephritis, defined as proteinuria of grade > 100 mg/dl, was established. Levels of anti‐dsDNA autoantibodies were evaluated by enzyme‐linked immunosorbent assay (ELISA), splenic cytokines were measured in vitro and the kidney microscopy was analysed following staining. TPC and MP treatments improved lupus nephritis significantly and prolonged survival in NZBxW/F₁ mice. TPC‐treated mice showed a significantly decreased level of proteinuria (P < 0·001) and anti‐dsDNA antibodies (P < 0·001) compared to PBS‐treated mice. Moreover, TPC and MP inhibited the production of the proinflammatory cytokines interferon IFN‐γ, interleukin IL‐1β and IL‐6 (P < 0·001) and enhanced expression of the anti‐inflammatory cytokine IL‐10 (P < 0·001). Finally, microscopy analysis of the kidneys demonstrated that TPC‐treated mice maintained normal structure equally to MP‐treated mice. These data indicate that the small molecule named TPC hinders lupus development in genetically lupus‐prone mice equally to methylprednisolone in most of the cases. Hence, TCP may be employed as a therapeutic potential for lupus nephritis.     

Comparison of PET Imaging with 64Cu-Liposomes and 18F-FDG in the 7,12-Dimethylbenz[a]anthracene (DMBA)-Induced Hamster Buccal Pouch Model of Oral Dysplasia and Squamous Cell Carcinoma

Purpose

Currently, 2-deoxy-2-[¹⁸F]fluoro-d-glucose (¹⁸F-FDG) is the gold standard radiotracer for staging of head and neck cancer; however, the low sensitivity of this tracer can impede detection of early lesions. ⁶⁴Cu-liposomes accumulate in various cancers and provide both a sensitive tracer and an indication of the biodistribution of nanotherapeutics. Here, the accumulation of ⁶⁴Cu-liposomes in early and established cancers is assessed and compared with ¹⁸F-FDG in a head and neck cancer model.

Methods

Lesions ranging from mild dysplasia to squamous cell carcinoma were induced in a hamster model of head and neck cancer by topical application of 7,12-dimethylbenz[a]anthracene to the buccal pouch. The hamsters were imaged with micro-positron emission tomography using ¹⁸F-FDG and ⁶⁴Cu-liposomes.

Results

At 24 h postinjection, ⁶⁴Cu-liposome accumulation exceeded the accumulation of ¹⁸F-FDG in every pathologic grade. The lesion-to-cheek pouch (background) ratio and lesion-to-brain ratio were also higher for ⁶⁴Cu-liposomes than for ¹⁸F-FDG.

Conclusion

Imaging of a nanotracer such as ⁶⁴Cu-liposomes can improve the visualization of head and neck tumors. Accumulation of liposomal particles in head and neck tumors over various pathologic grades averaged 3.5 %ID/cc demonstrating the potential for liposomal therapy with targeted chemotherapeutic agents.     

Combined effect of nifedipine and diltiazem on methacholine-induced bronchoconstriction in asthmatic patients.

Calcium blockers alter airway hyperreactivity. We studied the effect of either 60 mg diltiazem or 20 mg nifedipine and their combination on the airway response to methacholine-induced bronchoconstriction. Compared with placebo or diltiazem, nifedipine significantly raised the PC20. When diltiazem was given alone, it had no effect on the airway but, when given with nifedipine, it significantly raised the PC20 to methacholine. The effect was superior to any of the other treatments given. This study supports the concept of combining two different calcium blockers in the treatment of bronchial asthma.     

Phenytoin pharmacokinetics in catamenial epilepsy

It is not known why the frequency of seizures sometimes increases in the perimenstrual period (catamenial epilepsy). We have examined the possibility that changes in anticonvulsant pharmacokinetics may be responsible. Seventeen women with seizures who were taking phenytoin (DPH) and whose seizures were more frequent perimenstrually were examined twice each, once on the first or second menstrual day and again after 2 weeks. Mean serum DPH levels were lower during the menses. In seven women with seizures unrelated to menses, the fall of DPH levels was much smaller. In catamenial epilepsy, the fall was due to increased DPH clearance. At the time of ovulation, hepatic DPH metabolism may be slowed by competition from steroid hormones.