CARD15/NOD2 mutations are not related to abdominal PFAPA.
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Case-Control Studies↗
Biomedical subjects
M Maschio
Publications and source records attributed to M Maschio.
Supportive therapy in neuro-oncology.
Patients with cerebral neoplasms often report pain or other kinds of symptoms which, even though not directly connected to the disease itself, can cause complications in the approach to therapy and worsen the quality of life of the patients. The therapies aimed at controlling these kinds of disturbances are referred to as supportive therapies as they do not cure the underlying disease. However, these therapies should not be underestimated, because, by controlling these disturbances, they are able not only to greatly improve the quality of life of the neuro-oncologic patients but also increase their survival. Based on this hypothesis, we will discuss and examine the drugs more frequently used for supportive therapy in neuro-oncologic patients. We assert, in fact, that the desire to offer neuro-oncologic patients better assistance guarantees not only adequate specialized input from the health operators involved, concerning the quality of life and the uniqueness of the 'person-patient', but also the ability to be able to listen to the patients, understand their choices and allow them to express their priorities.
Analgesia↗
Cerebellar atrophy and monoclonal gammopathy of undetermined significance: a possible correlation?
Central Nervous System involvement in Monoclonal gammopathies of undetermined significance has seldomly been reported and in all the cases a demyelinating disease was found. We report the case of a young man who had been suffering for five years of progressive cerebellar syndrome. MRI showed marked cerebellar vermis atrophy. An IgG lambda monoclonal gammopathy was revealed in the serum. Cerebrospinal fluid examination showed oligoclonal bands and elevated Link-Index. Serologic research for HBV, HCV, HIV, Lues, Rubella, Measles was negative, as also genetic analysis for SCA1, SCA2, SCA3, SCA7 and Friederich's ataxia. Nerve conduction studies were normal. Plasmatic vit.E was low, but treatment with high doses of tocopherol was ineffective. i.v. immunoglobulins and steroids obtained only transient clinical benefits. In conclusion, we hypothesize a pathogenetic role of the IgG in this cerebellar atrophy.
Adult↗
Demyelinating disease in monoclonal gammopathy of undetermined significance.
We describe herein the case of a 57 year old man who, over the last five years, has presented ataxic and spastic gait on the right side, a reduction in fine motor movement of the fingers mainly on the right side, superficial right side brachiocrural hypoesthesia and a marked dysarthria associated with internuclear ophthalmoplegia. The neurological picture, after an initial progressive worsening which lasted some months, remained relatively stable over the years. Repeated magnetic resonance imaging (MRI) of the brain and spinal cord documented the presence of demyelinating plaques spread in the white matter of the periventricular region and the semioval centres, and a right side paramedian plaque at the C4-C5 level, none of which were in the active phase. Oligoclonal bands were revealed in the cerebrospinal fluid (CSF). Monoclonal IgM/lambda gammopathy with anti-myelin and anti-nucleo reactivity, found with serum immunofixation, were confirmed several times in successive annual controls, not associated to myeloproliferative pathology. The lack of progression in the clinical picture would seem to contradict the diagnosis of late Multiple Sclerosis. The presence of antibody activity against the myelin might support the hypothesis of a pathogenetic role of the immunoglobulins at the onset of the demyelinating disease in this patient. However, in the end, there is the possibility of casual association with a poorly functioning immune system connected to age.
Brain↗
[Diabetic foot. Physiopathology, clinical aspects, and recent therapeutic approaches].
Diabetic foot is a complication of diabetes mellitus occurring in 15% of patients that is of specific surgical interest. Over the past few years, preventive measures and the use of new therapeutic resources has reduced the number of patients undergoing demolitive surgery. The authors present a concise but at the same time sufficiently detailed picture of modern knowledge of the physiopathology, clinical aspects and current therapeutic guidelines for diabetic foot. In particular, they analyse the validity of various forms of complementary treatment to surgery, including techniques to stimulate tissue repair processes, hyperbaric oxygen therapy and laser therapy, and they underline the importance of using a multidisciplinary approach to this pathology. To this end, they review all the articles on the subject reported on Medline from 1992 to June 1998, presenting and commenting on the most significant results.
Diabetic Foot↗
Cytomegalovirus encephalitis: diagnosis with clinical approach, EEG and PCR techniques.
Cytomegalovirus (CMV) encephalitis is particularly evident in immunodepressed patients. Often diagnosis is difficult and time-consuming because of the complex basic clinical picture of these patients. We describe the diagnostic steps taken in a case of cytomegalovirus encephalitis affecting an elderly patient, classified as immunodepressed and deceased on the thirty-fifth day of hospitalisation in the intensive care unit due to acute myocardial infarction. Treatment with ganciclovir, 30 mg/kg per day, begun at the time of diagnosis, appears to have had a positive effect on the neurologic symptoms.
Aged↗
Nocturnal sleep and daytime somnolence in untreated patients with temporal lobe epilepsy: changes after treatment with controlled-release carbamazepine.
PURPOSE: To define sleep disturbances in patients with temporal lobe epilepsy (TLE) and explore the association between carbamazepine (CBZ) therapy, sleep, and daytime somnolence. METHODS: We recorded nocturnal polysomnography and measured subjective and objective daytime somnolence in a group of newly diagnosed TLE patients, who had no evidence of anatomic brain lesion on neuroimaging and had never been treated before. Recordings were performed at baseline, after the initial administration of 400 mg CBZ-controlled release (CR) and after 1 month of treatment (400 mg twice daily b.i.d.). The findings were compared with those of a group of young healthy volunteers, both at baseline and after the first administration of CBZ. The chronic effect of CBZ-CR treatment was evaluated only in TLE patients. RESULTS: At baseline, nocturnal sleep patterns of TLE patients did not show marked alterations when the influence of seizures, cerebral lesions, and drugs had been ruled out. In both the TLE and the control groups, initiation of CBZ therapy provoked a reduction and a fragmentation of rapid eye movement (REM) sleep and an increase in the number of sleep stage shifts. In the TLE group, these effects were almost completely reversed after 1 month of treatment, and no significant difference was noted between baseline condition and long-term follow-up. With regard to daytime sleepiness, initial administration of the drug caused an increase in objective sleepiness only in the control group. Subjective sleepiness was higher in the control group than in the TLE group but was not modified by the drug. CONCLUSIONS: We conclude that CBZ-CR has negative effects on REM sleep during initial administration but chronic treatment does not significantly modify nocturnal sleep or daytime somnolence.
Adult↗
EEG changes induced by vigabatrin monotherapy in focal epilepsy.
The effect of vigabatrin (GVG) monotherapy on EEG interictal abnormalities and on background activity recorded at rest and during mental tasks was studied in 14 patients suffering from focal epilepsy. A long-term EEG monitoring was performed in each patient before and 3 months after the beginning of GVG therapy. Ictal and interictal EEG abnormalities (IEA) were quantified by specific computer programs. Background activity was evaluated by spectral analysis at rest with eyes closed (EC), during blocking reaction (BR) and during fixation of cartoons (FIX). During treatment, IEA was either decreased or unmodified independently from seizure occurrence, which clearly improved in the majority of patients. The only EEG modifications induced by GVG monotherapy were a more pronounced slowing of the background activity at rest with EC and a reduced responsiveness to BR. EEG data suggest a GVG monotherapy induced mild "sedative" action on attentive tasks rather than on cognitive function.
4-Aminobutyrate Transaminase↗
Effect of lamotrigine on EEG paroxysmal abnormalities and background activity: a computerized analysis.
1. Little information is available about the action of lamotrigine (LTG) on EEG paroxysmal abnormalities and background activity. On the contrary, several clinical trials have shown the therapeutic efficacy of the drug in preventing partial and generalized seizures. 2. We performed computerized EEG monitoring in 21 patients suffering from focal and generalized epilepsy before and 4 months after addition of LTG. The anticonvulsant modified the EEG ictal events by reducing their frequency and duration. A statistically significant decrease of the interictal spikes was observed. The decrease involved mainly the spreading component of the interictal events leading to a better spatial definition of the epileptic focus. 3. In the presence of LTG, generalized tonic-clonic attacks were completely controlled, whereas partial seizures were decreased. 4. The EEG background activity was not modified by the addition of the drug. 5. Our findings suggest a specific role for LTG in the generation and propagation processes of epileptiform activity without interfering with the EEG background activity.
Adolescent↗
Sleep in healthy elderly subjects: a 24-hour ambulatory polysomnographic study.
It is still debated whether the deterioration of the sleep pattern, frequently reported by elderly subjects, is due only to aging per se. Other factors associated with aging or modifications of biological rhythms could also be involved. Elderly subjects frequently complain of daytime sleepiness, but it is not clear whether this actually represents a return to a polyphasic structure of sleep, or only a consequence of a disturbed night sleep. Ten healthy, independent and active elderly subjects (age > 72 years) were elevated by means of 24-hour ambulatory polysomnography. Findings of nocturnal sleep were compared with sleep of the same group in the 24-hour period and with sleep of young healthy controls. We observed a fragmentation of nocturnal sleep, but a fairly good representation of stages and a preservation of cyclicity. Except for three cases, with early or late times of sleep onset and wake-up, sleep disruption did not seem to be related to modification of circadian rhythms. Only three subjects presented undesired daytime naps, whereas the others either did not show daytime sleep at all, or were used to having their siesta after lunch since their young adulthood. In normal aging, daytime sleep does not constitute a social problem. Ambulatory polysomnography is a valid alternative to laboratory recordings in the identification of daytime sleep.
Aged↗
Rapidly progressive form of Creutzfeldt-Jakob disease without dementia: a case report.
We describe a case of a patient with Creutzfeldt-Jakob disease (CJD) characterized by a rapid clinical course lasting one and a half months, by: presence of focal dystonic movements at onset, absence of mental deterioration in the period preceding the impairment of consciousness, ataxia, myoclonus and periodic EEG abnormalities. The autopsy confirmed subacute spongiform encephalopathy, but no evident neuronal loss was observed. An acute clinical course of CJD may explain this latter histological finding which, in turn, probably provides an explanation for the absence of intellectual impairment.
Aged↗
Cognitive performances in newly referred patients with temporal lobe epilepsy: comparison with normal subjects in basal condition and after treatment with controlled-release carbamazepine.
In order to verify whether the performance of patients with temporal lobe epilepsy (TLE) were different from those of the normal controls in various cognitive tasks, we conducted a neuropsychological study in the two groups, excluding the presence of other confounding factors in the group of TLE patients (newly referred, previously untreated, without cerebral lesions at neuroimaging, with low recurrence of seizures). In basal condition, no significant difference was found between the nine healthy controls and the eight TLE patients. The second aim of the study was to investigate the effect of the first administration of 400 mg controlled-release carbamazepine (CBZ-CR). The drug caused a significant decrease in verbal memory (short and long-term) in TLE patients, whereas, in the control group, the first administration of CBZ-CR caused only a less significant impairment of long-term verbal-memory. Finally, the cognitive effects of CBZ-CR were assessed after one month of therapy, only in the group of patients. Our data suggest that: 1) differences between patients and controls in basal conditions are not relevant, once the influence of seizures, of treatment and of anatomical lesions has been ruled out. In other words, our patients did not perform differently from controls simply because they had become epileptic 2) also in the controlled-release preparation, adverse effects of CBZ on cognitive function are minimal and limited to the beginning of treatment.
Adult↗
Development of myoclonus in patients with partial epilepsy during treatment with vigabatrin: an electroencephalographic study.
In the context of a study of the effects of gamma-vinyl-GABA (GVG) on seizure occurrence and on EEG abnormalities we present three cases with focal epilepsy in which new clinical and EEG paroxysmal manifestations were observed during GVG therapy. At that time, whereas an amelioration or no change in patients' habitual seizures were observed, myoclonic jerks appeared with related changes in the EEG paroxysmal abnormalities, represented by generalized polyspike and wave complexes. An electroclinical correlation was recorded in one case. These data indicate that, although occurring rarely, it is possible to have epileptic myoclonus during GVG treatment. Mechanisms underlying these manifestations are difficult to explain. Probably a shift in the anti/proconvulsant GABAergic balance towards the latter may compromise the therapeutic effect of GVG.
Adolescent↗
Quantitative EEG evaluation in normal elderly subjects during mental processes: age-related changes.
Computerized EEG study was performed on 39 healthy elderly subjects (50-90 years), divided into two cohorts of increasing age (old, older) and on a group of 21 young controls. The EEG was recorded at rest, with eyes closed (EC) and during blocking reaction (BR), fixation (FIX) and mental arithmetic (MA) tasks. At rest with EC, the only significant variation was an increase of beta 1 relative power in old subjects which was positively correlated with age. During the performance of the mental tasks, in the elderly population when compared to young controls, the slow activity decreased slightly or was not significantly modified, while the alpha reactivity progressively decreased, showing a negative correlation with age in the older group. Beta 2 increased significantly in elderly subjects during BR and FIX but such change was less consistent with increasing age. Data show that at rest, the EEG in the elderly population differs only from that of young controls by showing a more pronounced fast activity, while only during mental processes is it possible to evidence changes that can be utilized as physiological "markers" of normal aging.
Adult↗
Immune response after vaccination with recombinant hepatitis surface antigen in maintenance hemodialysis patients and healthy controls.
We evaluated anti-HBs titers 2 months after vaccination with recombinant hepatitis surface antigen (rDNA-HBsAg) in 43 maintenance hemodialysis patients (MHP). Of these, 34 had not undergone hepatitis B virus vaccination previously (NV-MHP) and 9 had shown negative response to vaccination with plasma-derived HBsAg (HEVAC Pasteur; V-MHP). 120 healthy workers from the same hospital undergoing rDNA-HBsAg immunization were used as controls. All low responders (LR) (anti-HBs less than 100 mIU/ml) and nonresponders (NR; anti-HBs less than 10 mIU/ml) were given a booster dose 3 months after the last dose of vaccine. Seroconversion rates were lower in NV-MHP (52.9%) than in controls (98.4%). V-MHP showed higher seroconversion rates (88.9%) than NV-MHP. In each group, the number of responders (R; anti-HBs greater than or equal to 100 mIU/ml), LR and NR was as follows: controls 101, 17, 2; NV-MHP 6, 12, 16; V-MHP 8, 0, 1. After booster dose, 17/17 controls LR and no NV-MHP LR showed a rise in anti-HBs titers over 100 mIU/ml. Six months after the last dose of vaccine or the booster dose, anti-HBs titer fell under 10 mIU/ml in 4/12 MHP LR and under 100 mIU/ml in 6/14 MHP R. To achieve high seroconversion rates and to avoid the decline of anti-HBs to nonprotective titers in MHP, a booster injection should be made at different dates after the first vaccination.
Adult↗
Results of therapy for Hodgkin's disease in childhood. The Argentine Group for Treatment of Acute Leukemia.
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Adolescent↗
Randomized trial of chemotherapy versus chemotherapy plus radiotherapy for stage I-II Hodgkin's disease.
A total of 277 patients with untreated Hodgkin's disease, clinical stages I-II, were randomized to cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP) alone for 6 monthly cycles or to CVPP plus radiation therapy (RT), 3,000 rad, to involved areas (CVPP plus RT). One or more of the following factors were considered as unfavorable prognosis: age greater than 45 years, more than two lymph node areas involved, or bulky disease. In the favorable group, disease-free survival (77% vs. 70%) or overall survival (92% vs. 91%) at 84 months for CVPP versus RT plus CVPP was similar. Patients with unfavorable prognosis treated with RT plus CVPP had longer disease-free survival (75% vs. 34%) (P = .001) and overall survival (84% vs. 66%) than patients treated with CVPP alone.
Adolescent↗
Plasma fibronectin in head-injured ICU patients.
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Adolescent↗