Decreased platelet glutamate uptake in patients with amyotrophic lateral sclerosis.

Decreased glutamate uptake and a loss of the astrocytic glutamate transporter EAAT2 (GLT-1) have been shown in spinal cord and motor cortex of patients with ALS. Because platelets express the three major glutamate transporter subtypes, including GLT-1, and possess a high-affinity glutamate uptake, the authors investigated glutamate uptake in platelets from patients with ALS and controls. A 43% reduction of high-affinity glutamate uptake rate (p < 0.0001) was observed in patients with ALS compared with normal controls and chronic neurologic disorder patients, suggesting a systemic impairment of glutamate uptake in ALS.

An open-randomized clinical trial of selegiline in amyotrophic lateral sclerosis.

Based on the hypothesis that free radicals play a general role in the neurodegenerative process in motor neuron disease, we tested selegiline in a group of patients affected by amyotrophic lateral sclerosis (ALS) to examine whether it might modify the progression of the disease. Patients were admitted if they were 25-80 years old and had a confirmed diagnosis of ALS with symptoms lasting no longer than 24 months. Patients with familial ALS, pure progressive bulbar palsy, primary lateral sclerosis or progressive muscle atrophy were excluded; a total of 111 patients were recruited. Fifty-three patients were randomly assigned to receive the drug (selegiline 10 mg/day orally for 6 months) and the remaining 58 were considered ALS controls. Mortality was similar in the two groups (4 and 5 patients respectively), though the difference was not statistically significant. Among the survivors, mean MRC and Norris disability scores and forced vital capacity were fairly similar in the two groups at all times and no statistically significant difference between treated and untreated patients was found. The results did not change when the data were related to age, duration and characteristics of onset of the disease. The rate of progression was significantly more rapid in patients with bulbar symptoms in both groups. Our data do not show any significant effect of selegiline in modifying the progression of ALS.

The natural history and the effects of gabapentin in amyotrophic lateral sclerosis.

Glutamate excitotoxicity seems to play an important role in the aetiopathogenesis and progression of Amyotrophic Lateral Sclerosis (ALS). Gabapentin is a modulator of the glutamatergic system and has been shown to prolong survival in the transgenic model of familial ALS. It has also been demonstrated to slow the decline of arm strength in human sporadic cases. The aim of our study was to assess the effects of different dosages and duration of treatment of gabapentin on the natural history and survival of ALS patients. A total of 110 patients affected by definite ALS entered the study. After a 6-12 month period of observation, patients were randomly assigned to receive oral gabapentin 500 mg/day (Group A) or 1000 mg/day (Group B) for 6 months. In addition a group of patients received gabapentin 500 mg/day for 6 months and 1000 mg/day for a further 6 months (Group C). A group of 121 patients referred to our Institute, who received only symptomatic treatment, was considered as the control group (Group D). Each patient was seen at entry and every 3 months. All average slopes were negative but the comparison of all slopes showed a trend toward a slower rate of decline of muscle strength loss in all treated groups of patients compared with the control group. The differences were statistically significant. Analysis between the pretreatment and treatment period showed a statistically significant decrease of the decline of muscle strength and Norris score during the treatment period. Survival analysis showed a significantly longer survival in treated patients of Groups B and C. Our study suggests that gabapentin may be an effective drug for ALS; hence a controlled trial involving a sufficient large number of patients is warranted.

Effects of creatine supplementation on exercise performance and muscular strength in amyotrophic lateral sclerosis: preliminary results.

Creatine supplementation in humans has been reported to enhance power and strength both in normal subjects and in patients with various neuromuscular diseases. The purpose of this study was to examine the effects of supplementation on exercise performance and maximal voluntary isometric muscular contraction (MVIC) in Amyotrophic Lateral Sclerosis (ALS) patients. We report the results obtained in 28 patients with probable/definite ALS. In each patient we acquired the dynamometric measurement of MVIC in 10 muscle groups of upper and lower limbs and a measure of fatigue by means of an high-intensity intermittent protocol in elbow flexors and knee extensors muscles. All patients completed the protocols at the baseline and after supplementation of 20 g per day for 7 days and after supplementation of 3 g per day for 3 and 6 months. MVIC increased after 7 days of supplementation in 20 patients (70%) in knee extensors and in 15 (53%) of them also in elbow flexors. A statistically significant difference between pre and post-treatment mean values of MVIC was found both in elbow flexors (P<0.05) and knee extensors (p<0.04). The analysis of the slopes of fatigue test showed a statistically significant improvement after 7 days of supplementation in 11 patients (39%) in elbow flexors and in 9 patients (32%) also in knee extensors muscles. During the 6-month follow-up period all the examined parameters showed a linear progressive decline. In conclusion, our preliminary results have demonstrated that supplementation temporary increases maximal isometric power in ALS patients so it may be of potential benefit in situations such as high intensity activity and it can be proposed as a symptomatic treatment.

Treatment and long-term follow-up results of prolactin secreting pituitary adenomas.

The authors have reported data on a retrospective study, carried out in order to evaluate the short- and the long-term follow-up results of transsphenoidal microsurgery for prolactin-secreting pituitary adenomas; and to point out the indications for an eventual additional bromocriptine treatment. Prior to surgery, the patients were tested according to the study protocol. Patients were grouped differently according to their adenoma size. We found a good correlation between adenoma size and hormonal values. The surgical treatment led to an immediate endocrine cure in 47 patients (48.9%), with the majority occurring in microprolactinomas. Post-operatively, the cure rate was maintained in 45 patients. The Authors deem the transphenoidal microsurgery the therapy of first choice for microadenoma and mesoadenoma treatment, especially whenever there is bromocriptine intolerance. On the contrary, in invasive macroadenomas, bromocriptine is a necessary additional therapy both to shrink large tumors sufficiently for subsequent transsphenoidal approach and in incompletely removed tumors or persistent hyperprolactinemia.

Intraventricular supratentorial tumors in children.

Overall, intraventricular supratentorial tumors are rare in childhood. Classification can be based on the separation of lesions originating in intraventricular structures, such as choroid plexuses, from glial neoplasms of the ventricular wall which tend to infiltrate the ventricular cavities. Aim of the present study is to review the most common neoplasms of this region in childhood. Choroid plexus tumors (papillomas and carcinomas) and subependymal giant cell astrocytomas are dealt with, while for the other neoplasms which are rarer or more typic of other age ranges, specific reports should be consulted. Choroid plexus papillomas affect infants and are the most frequent oncological type among congenital tumors. The malignant variant (grade III-IV) is represented by the less frequent the neoplasms. The malignant variant (grade III-IV) is represented by the less frequent choroid plexus carcinoma which is markedly invasive with respect to adjacent nervous structures and has a high tendency to form metastases even at onset. Anaplastic papillomas are intermediate forms whose correct histopathology is still debated. Most frequent glial tumors are subependymal giant-cell astrocytomas. They are benign tumors (grade I) typically albeit not constantly associated to tuberous sclerosis. In this case the differentiation from subependymal nodules plays a major role. Contrast enhanced CT is fundamental in this assessment. Anaplastic variants, though rare, are well-known.

[Role of extracorporeal circulation in reversible acute cardiorespiratory insufficiency in neonatal and pediatric intensive care]. / Ruolo della circolazione extracorporea nell'insufficienza cardiorespiratoria acuta reversibile in terapia intensiva neonatale e pediatrica.

In the present study we try to analyze indications, contra-indication, side effects and limits of the use of extracorporeal circulation in neonatal and pediatric patients affected by reversible acute cardiorespiratory failure. The greatest experience on this technique has been achieved in the neonatal age (about 6000 newborns have been treated until now), while the employment in the pediatric age appears more recent (about 500 patients). Moreover, we focused on the drop-in criteria for neonatal and pediatric patients and on the clinical aspects and laboratory findings which can anticipate the surviving rate; the latter appears in any case much greater in the neonatal patient compared to the other pediatric ages.

[Teicoplanin therapy in neonatal and pediatric intensive therapy]. / La terapia con teicoplanina in terapia intensiva neonatale e pediatrica.

We administered teicoplanin as specific antibiotic therapy for nosocomial "ICU specific" infections with methicillin-resistant Staphylococcus aureus and epidermidis (MRSA-MRSE). The above mentioned drug has been given to 20 patients (15 newborns and 5 not-newborns) admitted into intensive care unit during the years 1988, 1989, 1990 with MRSA-MRSE localized and/or systemic infection, affected by severe disease (RDS, pulmonary edema, congenital cardiac disease, cystic fibrosis) undergoing invasive procedures which presented high nosocomial infective risk (tracheal intubation, mechanical ventilation, venous and arterial cannulation, total parenteral nutrition, etc.). Complete recovery from systemic or localized infection (sepsis, low respiratory tract infection, high respiratory tract infection) occurred in 19 out of 20 patients, with a rate of success of 95%. Teicoplanin treatment lasted from a minimum of nine days to a maximum of thirty days. The dose was 5-6 mg/kg/die in one administration for the first three days, then 4 mg/kg/die. The tolerability of teicoplanin has proven satisfactory, since we had no major side effects during treatment and follow up.

An overview of H-reflex studies in amyotrophic lateral sclerosis.

H-reflex methods were used to investigate motor neuron excitability and the basic physiology of segmental spinal reflexes. While many studies have been performed using these electrophysiological techniques, in both normal subjects and patients with different neurological diseases, few studies have focused on patients affected by amyotrophic lateral sclerosis (ALS). This article reviews the various electrophysiological techniques which use H-reflex to study the physiology of segmental spinal reflexes in motor neuron diseases. Their possible applications for elucidating pathophysiological mechanisms in ALS and for monitoring the disease during clinical trials are discussed.

Abnormalities of somatosensory evoked potentials in spasmodic torticollis.

This study examined the N20 and N30 waves of somatosensory evoked potentials (SEPs) from median nerve stimulation at three different stimulation rates (1, 3, and 6 Hz) in patients with idiopathic spasmodic torticollis (ST). The data were compared with those collected from a group of patients affected by Parkinson's disease (PD) and normal age-matched subjects. N30 amplitude was significantly decreased in both groups of patients with respect to the controls. The decrease was larger in patients with ST. The N20 wave remained stable. The latencies of both waves were unchanged. When the stimulus rate was increased, the N30 amplitude decreased significantly, with a similar trend observed in both patients and controls. The isolated abnormalities of the N30 wave in both ST and PD support the hypothesis of a common physiopathogenetic mechanism that involves the basal ganglia or their connections with the supplementary motor area.

H-reflex changes in the course of amyotrophic lateral sclerosis.

In this study the H-reflex and M-wave were evoked in a group of ALS patients, to correlate the findings with the clinical state, and to investigate whether a statistical approach for assessing H-reflex changes in the presence of a constant M-wave could be reproducible and helpful in monitoring the course of amyotrophic lateral sclerosis (ALS). The H-reflex and M-wave from the soleus muscle were evoked at different stimulus strengths in 35 patients with definite ALS during the course of their illness. The mean amplitude of the H-reflexes (H-mean) obtained in different sessions within an established range of mean M-response amplitude (M-mean) was calculated. For each patient, M-mean was made constant across sessions. H-mean showed high reproducibility and two different trends of changes which emerged in a 1 year follow-up within the population: a significant progressive increase and a steady decrease. When grouped on the basis of their H-mean trend, the patients did not differ in terms of any clinical variables considered. However, the group with progressive increase of H-mean showed a better prognosis. This study has shown that H-mean is effective in assessing the clinical course of ALS and could be useful in monitoring drug effects during clinical trials.

[Nosocomial infections caused by methicillin-resistant Staphylococcus aureus in a pediatric intensive care unit]. / Infezioni nosocomiali da stafilococco aureo meticillino resistente in un reparto di terapia intensiva pediatrica polivalente.

We performed an epidemiological investigation owing to the appearance between February and March 1988, of subsequent MRSA infections in our ICU; we also started a checking scheme. At the same time we performed a retrospective-perspective study of 701 patients (310 newborns 391 not newborns) admitted into ICU from 1-1-1987 to 30-6-1988. This study allowed us to settle the presence of MRSA in ICU and the manner in which they become infected. From our study the 4.9% of our patients contracted hospital infections and the 34.2% of these were MRSA infections. The 39.7% of our colonized patients were MRSA. It is really significant the frequency of MRSA colonization (p less than 0.0005) among newborn patients compared with other age patients; in the meantime there is not statistical significance in the frequency of MRSA hospital infections among the two groups. The results of our study show that infections were probably transmitted by the hands of the members of staff and that the reservoir of MRSA are tracheostomized hosts or long term ventilated patients.