Gelastic epilepsy and precocious puberty in hamartoma of the hypothalamus]

Four cases of hypothalamic hamartoma leading to gelastic epilepsy, precocious puberty and behavioural disorders are reported. Cerebral neuroradiologic examinations revealed a tumor-like mass attached to the hypothalamus in the region of the mamillary bodies in all cases. Precocious puberty developed in the two girls at 4 and 13 months but in neither of the two boys, who both suffered behaviour disturbances in the form of aggressive outbursts. A total resection of the tumors of both boys led to histologic confirmation of hamartoma. One boy was free of seizures upon follow-up, whereas seizure frequency in the other boy was reduced, while his aggressivity increased. The cases are discussed in context of current therapeutic conceptions of gelastic epilepsy and central precocious puberty.     

Adult-to-adult living donor liver transplantation using extended right lobe grafts.

OBJECTIVE: The authors report their experience with living donor liver transplantation (LDLT) using extended right lobe grafts for adult patients under high-urgency situations. SUMMARY BACKGROUND DATA: The efficacy of LDLT in the treatment of children has been established. The major limitation of adult-to-adult LDLT is the adequacy of the graft size. A left lobe graft from a relatively small volunteer donor will not meet the metabolic demand of a larger recipient. METHODS: From May 1996 to November 1996, seven LDLTs, using extended right lobe grafts, were performed under high-urgency situations. All recipients were in intensive care units before transplantation with five having acute renal failure, three on mechanical ventilation, and all with hepatic encephalopathy. The median body weight for the donors and recipients was 58 kg (range, 41-84 kg) and 65 kg (range, 53-90 kg), respectively. The body weights of four donors were less than those of the corresponding recipients, and the lowest donor-to-recipient body weight ratio was 0.62:1. The extended right lobe graft was chosen because the left lobe volume was <40% of the ideal liver mass of the recipient. RESULTS: Median blood loss for the donors was 900 mL (range, 700-1600 mL) and hospital stay was 19 days (range, 8-22 days). Homologous blood transfusion was not required. Two donors had complications (one incisional hernia and one bile duct stricture) requiring reoperation after discharge. All were well with normal liver function 5 to 10 months after surgery. The graft weight ranged from 490 g to 1140 g. All grafts showed immediate function with normalization of prothrombin time and recovery of conscious state of the recipients. There was no vascular complication, but six recipients required reoperation. One recipient died of systemic candidiasis 16 days after transplantation and 6 (86%) were alive with the original graft at a median follow-up of 6.5 months (range, 5-10 months). CONCLUSIONS: When performed by a team with experience in hepatectomy and transplantation, LDLT, using an extended right lobe graft, can achieve superior results. The technique extends the success of LDLT from pediatric recipients to adult recipients and opens a new donor pool for adults to receive a timely graft of adequate function.     

In vivo measurements show tensile axial strain in the proximal lateral aspect of the human femur

Two conflicting theories exist concerning the stress pattern for the proximal lateral aspect of the human femur. According to the classic theory of Pauwels, a bending moment on the femur leads to compression medially and to tension laterally. The alternative theory is that muscle forces contribute to a moment-free loading of the femur, with both the medial and lateral cortices subjected to compression. To examine these theories, we measured the strain at the external surface of the proximal lateral aspect of the femur of two female patients undergoing surgery for “snapping hip syndrome.” During the surgical procedure, a strain-gauge rosette was bonded to the lateral aspect of the femur and the cortical strains were monitored while the patient performed a series of activities. In both patients, principle tensile strain increased significantly during one-legged stance, walking, and stair climbing as compared with that during two-legged stance. During each loading situation, the principal tensile strain was aligned within 22° to the longitudinal femoral axis. Dynamic strain measurements consistently revealed tensile axial strain at the lateral aspect of the femur during each activity. The present studv supports the classic bending theory of Pauwels and demonstrates that the proximal lateral aspect of the femur is subjected to tension during the stance phase of gait.     

Immunomodulatory Effects of γ-HCH (Lindane) in Mice

Mice were fed for 24 weeks with three different subtoxic dosages of γ-HCH (0.012, 0.12 and 1.2 mg/kg) mixed in powdered feed. The immunological profile was assessed at an interval of one month during the entire exposure period. Both the cell mediated and humoral components of immunity showed a biphasic response characterized initially by stimulation followed by suppression in a dose dependent manner. However, γ-HCH did not affect the functional properties of peritoneal macrophages. Histological changes in lymphoid organs were in accordance with the biphasic immunomodulatory effects of γ -HCH.     

Age-related anticonvulsant activity of NMDA in the rat caudate-putamen.

Striatal pathways are important for modulating the threshold for seizures in the rat forebrain. N-Methyl-D-aspartate (NMDA), an excitatory amino acid derivative and powerful anticonvulsant agent, when injected into the brain, has been shown to protect adult rats against kindling and pilocarpine-induced seizures when injected into the caudate-putamen. The present study examines whether the anticonvulsant action of NMDA in the caudate-putamen varies with age. Bilateral striatal administration of NMDA was effective in suppressing bicuculline-induced seizures in rats older than 23 days of age. The results suggest that striatal pathways involved in the anticonvulsant activity of NMDA in the caudate-putamen are not functionally active in developing rats before the 4th week of life.     

The effects of 20 weeks of physical fitness training in female patients with fibromyalgia.

During a period of 20 weeks 18 female patients with fibromyalgia participated in a 60-minute exercise program twice a week. A control group, comprising 17 patients, was told not to change their physical activity level. Eleven patients in the training group and fourteen in the control group completed the study. The results at entry were compared to those after 20 weeks, as well as being compared to the results of the control group. No statistically significant changes or differences in general pain, pain coping and fatigue were seen after 20 weeks. Improved dynamic endurance work performance for the upper extremity was found, however, in the training group, measured as the strength of the first (p = 0.01) and the last repetition (p = 0.003). These results differed from the results of the control group (p = 0.02 and p = 0.003). It is concluded that fibromyalgia patients may undergo low-intensity dynamic endurance training without experiencing exacerbation of their general pain and fatigue symptoms.     

Leukocyte agglomeration reaction in the diagnosis of allergic diseases

This paper discusses the problem of how allergic responses to drug therapy can be obviated by using Flack's leukocyte agglomeration reaction (LAR). The sensitivity and reliability of the method were tested in patients who had drug allergic responses in their case histories. Altogether 112 patients were examined. The LAR data and history observations coincided in 68.7-83.3%. It is concluded that Flack's LAR is a reliable diagnostic method; it is simple, safe and can be widely used to diagnose drug allergy and to identify the drug allergen.     

Otogenic Fusobacterium necrophorum meningitis

A case of meningitis secondary to acute suppurative otitis media in a previously healthy child is reported. The only organism isolated from blood after aerobic and prolonged anaerobic culture was identified as Fusobacterium necrophorum. Complete recovery followed treatment with surgery and prolonged antibiotic therapy. The role of anaerobes in the development of meningitis, the isolation and identification of Fusobacterium necrophorum, the clinical presentations of F. necrophorum infection and the choice of antibiotics in the treatment of these infections are discussed.     

Prognostic factors in invasive squamous cell carcinoma of the vulva.

Histological malignancy grading and its correlation to prognosis were retrospectively investigated in 303 cases of squamous cell carcinoma of the vulva. A scoring system based on several variables is suggested. The scores and the individual histological variables were tested along with clinical factors with a view to predicting survival. Various histological variables including mode of invasion, depth of invasion and nuclear polymorphism, as well as the sum of the scores were good predictors of survival. The study revealed a significantly better survival rate in stage III if no metastases were suspected. An age factor was also found showing that cancer of the vulva has a worse prognosis in older women.