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Biomedical subjects

L Mazzini

Publications and source records attributed to L Mazzini.

33 records · Page 2Linked to original sources
PubMed1995

Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis.

Bulbar involvement in amyotrophic lateral sclerosis (ALS) is often related to a worse prognosis on account of the higher risk of pulmonary aspiration and undernutrition due to dysphagia. The aim of our study was to assess the effects of enteral feeding by percutaneous endoscopic gastrostomy (PEG) in a long-term follow-up of ALS patients. We report the results of PEG in 31 ALS patients with bulbar involvement. The patients were observed at 3-monthly intervals over a period of 2 years after PEG. All the data were compared with those obtained from a control group of 35 ALS patients who refused PEG. Mortality did not differ significantly between the two groups of patients during the first 6 months of observation, whereas after this period it was lower in the PEG group. In the patients who had had PEG, the body mass index showed a mild but statistically significant improvement after tube insertion while in the control group it decreased significantly. The findings of this study demonstrate that PEG can improve survival in elderly and young ALS patients with bulbar involvement; it enhances their quality of life and helps their integration in their social and family surroundings. We think that PEG should be included symptomatic treatment of all ALS patients with bulbar involvement from the onset of symptoms.

Adult↗
PubMed1994

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.

Adult↗
PubMed1994

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.

Adult↗
PubMed1994

Short-latency neck muscle responses to vertical body tilt in normal subjects and in patients with spasmodic torticollis.

EMG responses in the sternocleidomastoid (SCM) and dorsal neck muscles (DNM) to vertical head acceleration were studied in normal subjects and in patients with spasmodic torticollis, standing on a platform that could be tilted upwards. The vertical body displacement and the induced changes in the head-neck angle (a flexion-extension sequence) were recorded. Excitatory responses, symmetrical on the two sides, were elicited in normal subjects in both muscle groups, at a latency of about 60 msec (DNM) and 90 msec (SCM). With the head initially extended, the latency of DNM response increased, leaving that of SCM unchanged. During an isometric rotatory effort, an early inhibitory period was recorded in the active muscles at a latency of about 40 msec. Downward tilt did not evoke the responses. The DNM excitatory responses appeared to be related to muscle stretch, while those in SCM, as well as the inhibitory responses in both muscles, were thought to originate in the vestibular receptors. During active head rotation the response increased in amplitude in the active SCM and decreased in the lengthened antagonist; decreased responses in the lengthened muscle persisted during passive head rotation. This was attributed to an influence from the tonic neck receptors. In the patients, SCM responses had normal latency, but were reduced in amplitude or absent in the dystonic muscle, in spite of tilt-induced head movements comparable to those recorded in normals. The diminution was even bigger if compared to normal subjects with the head actively rotated to a similar extent. It persisted when the head was returned to normal position by the "geste antagoniste." The inhibitory responses were unaffected in the active normal and dystonic muscles. The possible role of a deficit of the central vestibular connections in the decreased excitatory SCM response in dystonic patients is considered.

Adult↗
PubMed1992

Activation of the neck muscles from the ipsi- or contralateral hemisphere during voluntary head movements in humans. A reaction-time study.

Reaction times (RTs) of EMG onset of agonist right and left sternocleidomastoid (SCM) and splenius (SPL) muscles in response to acoustic (AC) or unilateral somatosensory (SS) stimulation were measured in normal subjects, during head rotation (to the right or to the left) and flexion. No significant difference was present in the AC-RT of SCM muscles of the two sides between rotations or flexion. The same was true for the SPL. Under the tactile condition, in which the stimulus was delivered to one index finger, the RTs of both agonist muscles were shorter during head rotation toward the stimulus than away from it: the SCM contralateral to the stimulated finger was faster than the ipsilateral SCM, while the reverse was true for the SPL. During flexion, the SS-RTs of the SCM of both sides were similar, and similar in turn to the SCM-RT during rotation away from the stimulus. When the stimulus was delivered to the shoulder, the RT difference between the agonist SCMs disappeared. The delay in the activation of the SCM ipsilateral to the stimulated finger is compatible with the interhemispheric transmission time in the absence of callosal connection between the hand areas of the primary somatosensory cortex. The data favour the hypothesis that SCM activation during voluntary head rotation is controlled by the ipsilateral hemisphere.

Adult↗
PubMed1992

Methylbromide intoxication: a case report.

This work focuses on the neurophysiological features in a patient with action myoclonus and mental deterioration following methylbromide intoxication. The patient is a 28-year-old man, without respiratory distress or exposure to other toxics. Myoclonus improved with polytherapy (clonazepam, 5-HT, carbidopa, GABA). The neurophysiological and neuropsychological evidence in this patient suggests a possible double site of action of methylbromide at cortical and subcortical levels.

Adult↗
PubMed1991

Amyotrophic lateral sclerosis: a possible example of autosomal recessive inheritance.

A family is reported in which three out of four siblings of a consanguineous healthy couple developed adult onset Amyotrophic Lateral Sclerosis (ALS). All patients showed a similar clinical course with regard to disease progression and absence of cognitive deterioration. Laboratory findings included modification of Spinal Evoked Potential (SEP) and normal value of thiamine and thiamine monophosphate in cerebrospinal fluid (CSF). These data suggest an autosomal recessive mode of inheritance of ASL in this family.

Adult↗
PubMed1990

Post-traumatic extrapyramidal syndrome: case report.

We report the case of a young man with possible post-traumatic extrapyramidal syndrome. Some features of this case have a bearing on the controversy surrounding the existence of post-traumatic Parkinson disease.

Adolescent↗
PubMed1986

Motor and psychomotor functions in amyotrophic lateral sclerosis evaluated by tests of motor ability.

18 patients with typical sporadic Amyotrophic Lateral Sclerosis (ALS) were investigated by the Motor Accuracy and Speed Test (MAST) and 18 healthy age- and-sex-matched volunteers, acted as controls. All performed each of the five tests 10 times with both the right and the left hands and repeated the experiment after one week by the same procedure. Motor performances were better in the controls than in the ALS patients only in the first three tasks. At retest, one week later, the controls generally improved while ALS patients did not. The mean percentages of changes showed a statistically significant difference in the fifth task. Analysis of the results suggests the possibility that, among the other mechanisms, a disturbance of motor learning ability could be operating in ALS patients.

Adult↗
PubMed1986

The identification of neuroapraxia, axonostenosis and trigger zone in facial nerve pathology.

The classical electromyographic investigations and simultaneous recordings of voluntary activity and M responses from muscles of both the upper and lower branch of the facial nerve were carried out in 5 patients with Bell's palsy. R1 responses were also recorded. All investigations were extended to the healthy side. With the aim of localizing the point of axonostenosis with axonal atrophy we investigated the following parameters: conduction velocity (c.v.) in the fallopian canal, c.v. in the external facial nerve, side difference of R1-M. In one case of amyotrophic lateral sclerosis with previous Bell's palsy signs of collateral sprouting and of ephaptic transmission were detected.

Adult↗
PubMed1986

Neuropsychological measures in amyotrophic lateral sclerosis and their relationship with CT scan-assessed cerebral atrophy.

Twenty-one male patients affected by ALS have been given a short neuropsychological battery and the results have been compared with those obtained by 21 male subjects affected by other non-dementing neurological diseases. Only two ALS patients had definitely low scores on WAIS (performance scale) and on learning tasks, both verbal and spatial, but the ALS group did not differ, on the whole, from the controls. No relationship was observed between cognitive impairment and cerebral atrophy assessed by computerized tomography. The conclusion is suggested that the cognitive impairment in ALS be a discrete, seldom occurring event.

Adult↗
PubMed1984

Somatosensory evoked potentials in amyotrophic lateral sclerosis.

Forty five patients with amyotrophic lateral sclerosis were investigated, by means of somatosensory evoked potentials, in order to detect the presence of subclinical sensory changes. Cervical SEPs from the median nerve and cortical SEPs from the median and tibial nerve were recorded, showing a delay of N13 and subsequent components; the latency of the first constant cortical potential was also increased in many patients. Only the SEPs from the tibial nerve showed a decrease of amplitude. These results suggest a pathological slowing of conduction along the central sensory pathways in amyotrophic lateral sclerosis.

Adult↗
PubMedDate not supplied

Sleep-related breathing disorders in amyotrophic lateral sclerosis.

Sleep-related breathing events in patients with amyotrophic lateral sclerosis (ALS) have been reported in small case series, but the association with the clinical presentation--with (B) or without (nonB) bulbar symptoms--or the relevance for prognosis have not been investigated. We retrospectively analyzed sleep studies of 114 (46 nonB) ALS patients, aged 54 +/- 11 years. Respiratory function was better in nonB patients: forced vital capacity was 76 +/- 20% vs 55 +/- 23% in the bulbar group (p < 0.001); PaCO2 41 +/- 5 vs 44 +/- 6 mm Hg p < 0.05. The mean apnea/hypopnea index (AHI) was higher in nonB patients (22 +/- 12 vs 15 +/- 16 events per hour- p < 0.05); in this group 21 out of 46 patients (46%) had more than 20 events/hour versus 14 out of 68 (21%) in the nonB group (p < 0.005). On the contrary the oxygen desaturation index (ODI) was similar (10 +/- 11 vs 9 +/- 12 events per hour, p = NS). Most events had a central genesis and obstructive events were usually erratic, except in 7 patients (6 in group B) who had more than 10 obstructive events/hour. Data were stratified in three groups: with a disease duration below 1 year (< 1 yr), between 1 and 2 years (1-2 yr), and more than 2 years (> 2 yr). The occurrence of sleep-related respiratory disorders decreased with the increase of disease duration (23 +/- 15; 18 +/- 14; and 16 +/- 15 events per hour respectively), the decrease being significantly lower in the > 2 yr group than in the < 1 yr (p < 0.05). Again ODI was similar in the three groups. In conclusion the present study shows that sleep-related breathing events are more common than previously described in ALS patients, particularly in the first year following onset of the disease. Obstructive events occur rarely, although the prevalence of obstructive sleep apnea is higher than predicted, particularly when bulbar symptoms are present. Patients without bulbar signs show a higher prevalence of central events. The progressive decrease of events with the increase of disease duration could be due to a progressive weakness of respiratory muscles, but it could also suggest an independent role for nocturnal events which could be linked to a worse prognosis or to a more rapid decay of clinical status.

Adult↗