PubMed Health⌕ Search

Biomedical subjects

M Bergui

Publications and source records attributed to M Bergui.

At least 37 records · Page 2Linked to original sources

Interferon-beta dose and efficacy: the OPTIMS study.

Interferon beta (IFN-beta) reduces exacerbation rates in patients with relapsing-remitting multiple sclerosis (MS), but some patients do not respond to treatment. Recent studies have shown a clear dose-response effect on the reduction of exacerbation rates, and on burden of disease accumulation and active lesion frequency seen on MRI. During treatment with 8 MIU IFN-beta we noticed a 30% rate of treatment failure. We then treated non-responders with 12 MIU IFN-beta and observed significant improvement in the clinical signs of disease activity. In order to compare the efficacy of two different doses of IFN-beta-1b, a multicenter study for the optimization of interferon for MS (OPTIMS) has been organized. The design of the study is presented here.

Dose-Response Relationship, Drug↗

De novo cavernoma case report and review of literature.

BACKGROUND: De novo cavernoma, reported with the familial form of disease, is rare in cases with a negative family history. Cranial radiation, coexistent vascular malformation, genetic and hormonal factors, previous surgery for intracranial lesions, or other apparently unrelated intracranial lesions have been reported as risk factors. METHODS: We report a case of de novo cavernoma without a family history and without previous irradiation or any other known risk factors. The genesis of this lesion is discussed. RESULTS: To our knowledge, this is the first case, based on two separate magnetic resonance imaging (MRI) studies, demonstrating evidence of de novo cavernous malformations in the absence of familial history, brain radiation therapy, or other apparently unrelated intracranial tissue lesions. Based on previous negative computed tomography scans, other cases have been presented as de novo cavernous angiomas; thus it is possible that the newly discovered cavernoma existed previously but had been missed on previous poorer quality or lower resolution imaging studies. CONCLUSIONS: Cavernoma can arise even without an associat family history; in our case, a previous head injury could have set off either a genetic cascade with attendant endothelial proliferation or a latent virus.

Adult↗

Restricted dissociated sensory loss in a patient with a lateral medullary syndrome: A clinical-MRI study.

BACKGROUND: Various sensory syndromes in lateral medullary infarctions are described. A small variation in the location of a lesion may lead to very different clinical features, owing to the complex anatomy of the medulla oblongata. MRI may identify the location and extent of the ischemic lesions, allowing a clear clinical-anatomical correlation. CASE DESCRIPTION: We describe a man with an ischemic lesion in the right portion of the lower medulla that presented a contralateral impairment of spinothalamic sensory modalities and an ipsilateral impairment of lemniscal modalities with a restricted distribution (left forearm and hand, right hand and fingers, respectively). The restricted and dissociated sensory abnormalities represent the only permanent neurological consequence of that lesion. CONCLUSIONS: The atypical sensory syndrome may be explained by the involvement of the medial portion of spinothalamic tract and the lateral portion of archiform fibers at the level of the lemniscal decussation.

Arm↗

Transforming growth factor beta1 (TGFbeta1) mRNA level correlates with magnetic resonance imaging disease activity in multiple sclerosis patients.

Eight relapsing-remitting multiple sclerosis (MS) patients were tested for the level of transforming growth factor beta1 (TGFbeta1) mRNA in peripheral blood mononuclear cells every 15 days for 6 months. Disease activity was evaluated every 4 weeks by magnetic resonance imaging (MRI) and neurological examination. An inverse correlation was found between the level of TGFbeta1 mRNA and MRI disease activity. The level of TGFbeta1 mRNA predicted the presence of disease activity in the scans performed 2-4 weeks later with high sensitivity (88%) and specificity (87.5%) suggesting that TGFbeta1 mRNA quantification could be an indicator of disease activity in MS.

Adult↗

Brain lesions due to cerebral venous thrombosis do not correlate with sinus involvement.

Cerebral venous thrombosis may be well tolerated or lead to a brain lesion; availability of collateral venous pathways may explain the great variability of the lesions. This collateral circulation involves mainly medullary and cortical veins. These are difficult to assess neuroradiologically, particularly if thrombosed. Cerebral venous thrombosis is diagnosed usually based on thrombosis of dural sinuses and of the deep venous system. We tried to correlate the site and extent of dural sinus thrombosis with the location and the size of brain lesions in 26 consecutive patients with cerebral venous thrombosis, to investigate whether a simple causal relationship exists. No significant correlation between the extent and site of thrombosis in dural sinuses and the extent and location of brain lesions was found. In some cases a clear relationship between thrombosis of cortical and medullary veins and the lesions was evident. These data suggest that sinus thrombosis alone may be well tolerated in many cases, while involvement of cortical and medullary veins leads to a worse clinical situation.

Adolescent↗

A case of meningioma with exceptionally extensive oedema.

We report a meningioma with very extensive surrounding oedema. This finding, along with the age of the patient and other clinical features, made correct diagnosis difficult. The pathophysiology of oedema in general and its exceptional extent in this case are discussed.

Aged↗

MRI and CT in an autosomal-dominant, adult-onset leukodystrophy.

We report MRI findings in a family with an autosomal-dominant, adult-onset neurological disorder. The clinical picture, the white matter changes detected on MRI and the absence of any laboratory abnormality suggested the diagnosis of leukodystrophy with an unknown biochemical defect. Autosomal-dominant inheritance is extremely rare in this kind of disease, and most reported families have not undergone MRI. We performed MRI and clinical examination of 17 members of our family; 9 affected subjects, at different stages of the disease, were detected. The most characteristic MRI findings were initially symmetrical areas of signal change in the white matter of the trigonal region; demyelination extending thereafter to the frontal and parietal regions, partially involving subcortical white matter; the temporal lobe and optic radiations were less involved; the internal capsule and corpus callosum were involved later, in a dorsoventral direction; patchy demyelination was evident in the late stages in the brain stem; the cerebellum was spared even in the latest stages of the disease. While pathological examination is essential to characterise and classify these kinds of diseases, MRI can make substantial contributions to understanding their natural history, and to detect early signs of the disease.

Adolescent↗

False-negative angiograms in subarachnoid haemorrhage due to intracranial aneurysms.

Of 440 patients with spontaneous subarachnoid haemorrhage in whom an aneurysm was suspected, 60 had a negative angiogram. A second angiogram performed 1-4 weeks later revealed an aneurysm in 5 of 40 cases. Of these patients, 3 had a second haemorrhage. In all cases, diffuse bleeding, with involvement of the anteroinferior interhemispheric fissure, was present on CT. There were three aneurysms of the anterior communicating artery and two of the carotid siphon. The reasons for the false-negative angiograms and the usefulness of repeated angiography are discussed.

Adult↗

Autosomal dominant late-onset leukoencephalopathy. Clinical report of a new Italian family.

The adult-onset autosomal dominant leukoencephalopathies are rare disorders. Very few pedigrees have been extensively described and no biochemical or genetic marker has been identified so far. The present study was aimed to characterized an autosomal dominant late-onset leukoencephalopathy occurring in a large Italian kindred. A genealogic method was adopted to ascertain 51 affected individuals among nearly 400 subjects in 8 generations. Medical records were obtained from 11 deceased patients. We personally examined 8 symptomatic and 9 asymptomatic at-risk individuals who underwent a standardized clinical, biochemical, radiological and neurophysiological study. The mean age at onset of the disease was 46.6 years and the mean duration of disease 9.9 years. The clinical picture was characterized by progressive pyramidal and pseudobulbar signs, urinary incontinence and, sometimes, action tremor of the head and/or hands. No relevant mental deterioration was noted. In all the symptomatic and in 1 asymptomatic subject, brain MRI showed marked symmetrical hyperintensity on T2-weighted images of the white matter of the cerebral hemispheres, with constant sparing of the cerebellum. In these subjects, evoked potentials revealed altered central neural conduction. Nerve conduction velocity, biochemical (including lysosomal enzymatic activities) and biopsy (peripheral tissue specimens) examination were normal. The clinical and neuroradiological data are consistent with an autosomal dominant adult-onset leukoencephalopathy whose features are unusual when compared to those previously reported.

Adult↗

Uncommon symptomatic cerebral vascular malformations.

We describe three cases of unusual vascular malformations in which the most relevant angiographic findings were the presence of a pathologic arteriovenous shunt through multiple small direct arteriovenous fistulas and the lack of a clearly identifiable nidus. All malformations were symptomatic. Such lesions are relatively rare, but they should be taken into consideration in the differential diagnosis of cerebrovascular malformations.

Adult↗

Long term recombinant interferon alpha treatment in MS with special emphasis to side effects.

Twenty relapsing-remitting (RR) clinically definite MS patients were treated with 9 MIU intramuscular recombinant interferon alpha-2a (rIFNA) (Roferon-A, Roche) (n = 12) or placebo (n = 8) every other day for 6 months and followed up for a further 6 months after stopping treatment. Numbers of active lesions at MRI and of patients with clinical-MRI signs of disease activity and lymphocyte interferon gamma production, which were decreased during treatment, returned to values similar to baseline and placebos after stopping treatment. rIFNA chronic therapy seems therefore needed in order to maintain drug efficacy. Side effect profile was monitored, too, for over 1 year in the same 20 patients plus 25 additional RR MS patients. Besides the typical side effects of type I interferon therapy (fever, fatigue, depression, lymphopenia, hepatic enzyme elevation), occurrence of serum autoAbs was noted in 30% patients (in 60% antinuclear and in 80% antithyroid autoAbs). In two patients rIFNA treatment was stopped, in one case for antithyroid autoAbs and hypothyroidism, in the other for antinuclear autoAbs and a five-fold increase of ALT. A careful monitoring of serum autoAbs and of signs of thyroid or liver damage must always precede and accompany longterm type I IFN therapy.

Autoantibodies↗

Interferon alpha-2a treatment of relapsing-remitting multiple sclerosis: disease activity resumes after stopping treatment.

We evaluated the long-lasting effects of systemic high-dose recombinant interferon alpha-2a (rIFNA) in relapsing-remitting (RR) MS after discontinuing treatment in a single-blind randomized placebo-controlled trial with 20 RR clinically definite MS patients using either nine million IU intramuscular rIFNA (n = 12) or placebo (n = 8) every other day for 6 months. Follow-up continued for a further 6 months without IFN treatment. In rIFNA-treated patients, main outcome measures, significantly different from placebo during treatment, returned, after discontinuing treatment, to values similar to placebo or baseline. Active MRI lesions per patient increased from 0.08 +/- 0.08 to 1.2 +/- 0.4 (p < 0.02), number of patients with clinical MRI signs of disease activity from 2 of 12 to 8 to 12 (P < 0.04), lymphocyte IFN gamma production from 3.0 +/- 0.7 to 12.4 +/- 2.2 IU/mL (p < 0.01), lymphocyte tumor necrosis factor alpha production from 5.8 +/- 0.9 to 18.9 +/- 6.3 pg/mL (p < 0.05). All side effects of rIFNA treatment disappeared after discontinuing the drug. The reduction of clinical MRI signs of disease activity and the immunologic effects were temporary and restricted to the period of rIFNA administration. The depression of many immunologic and clinical MRI responses during drug administration and their simultaneous return to baseline after discontinuing the drug strongly argue all observed changes were related to drug administration.

Adult↗

Propofol analgesia in central pain: preliminary clinical observations.

Propofol, an intravenous general anaesthetic, has been reported to relieve some forms of pruritus at subhypnotic doses. We assessed its effectiveness in 32 patients with several kinds of non-malignant chronic pain, in a placebo-controlled, double-blind study. We found that central pain, but not neuropathic pain, is at least partially controlled by propofol at subhypnotic doses, without major side-effects. In particular, allodynia associated with central, but no neuropathic, pain has been completely controlled. Propofol analgesia leads to renormalization of brain metabolism as seen on single photon emission computed tomography. We conclude that propofol may help in the diagnosis of central pain, particularly in unclear cases, and also in treatment. Possible mechanisms of action are discussed.

Adult↗

Early modification of stretch reflex in Parkinson's disease.

We stretched quadriceps femoris in healthy subjects and in patients with a recent diagnosis of PD in order to assess whether modifications of the long-latency component of the stretch reflex is an early event in the course of Parkinson's disease (PD). We found a modified mechanical and electromyographic (EMG) behavior in stretching relaxed muscles of patients while voluntary activation greatly reduced differences between normal and Parkinsonian subjects, suggesting that a lower threshold of the response is an early sign in PD.

Adult↗

Stretch reflex of quadriceps femoris and its relation to rigidity in Parkinson's disease.

Much evidence suggests that parkinsonian rigidity is due to hyperactivity of a reflex arc. While tendon jerk and H reflex are not modified in Parkinson's disease (PD), the long-latency component of stretch reflex (LLR) shows an increased size in PD. It has been proposed that this modification could account for rigidity. We studied in 14 PD patients and 8 normal subjects the stretch reflex of the quadriceps femoris. The muscle was stretched by a torque motor in two experimental sets: at rest and with voluntary background activity. Latency, duration and size of the reflex were compared in two groups; correlation between size of the reflex and rigidity was investigated. A lower threshold for the reflex was found in PD patients in trials at rest, and LLR showed increased size and duration in trials with background activity. No clear relationships between these data and rigidity were demonstrated.

Adult↗

Stretch reflex of quadriceps femoris in normal man: methodological considerations and normative data.

Short- and long-latency responses to stretching have been evoked in various muscles in the healthy subject. The quadriceps femoris, however, has never been investigated in this connection, despite its functional importance. We studied its stretch reflex in 17 healthy subjects with a torque motor to determine its morphological features in relation to the level of background EMG activity, the stretching velocity and duration and the instructions given to the subject. Two bursts of EMG activity were constantly evoked when the muscle was voluntarily activated. Response magnitude was proportional to the background activity and displacement velocity. Short stretching evoked only one EMG burst. These findings are similar to those from other muscles. Intra-individual variability was evaluated repeating the experiment in the same session without changing position of the electrodes, and then after two months. The long-latency response showed a good intra-individual reproducibility.

Adult↗