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Biomedical subjects

A Quattrini

Publications and source records attributed to A Quattrini.

At least 73 records · Page 4Linked to original sources
PubMed1992

In vivo modulation of myelin gene expression by human recombinant IL-2.

We treated adult mice with human recombinant interleukin-2 (IL-2) and determined the expression of the genes encoding for the major central and peripheral myelin proteins. In the CNS, myelin basic protein (MBP) and myelin-associated glycoprotein (MAG) mRNA levels were the same both in IL-2-treated and in control mice. Proteolipid protein (PLP) transcript was decreased in IL-2-treated animals when compared to controls. In the PNS, the messages for the glycoprotein P0 and for MBP were markedly increased in IL-2-treated animals when compared to controls.

Animals↗
PubMed1992

A clinical, electrophysiological, morphological and immunological study of chronic sensory neuropathy with ataxia and paraesthesia.

We have observed 9 patients (8 men and 1 woman), 58 to 77 years of age with neuropathy with only sensory symptoms and insidious onset. Five of them (4 men and 1 woman) aged 65 to 77 years, had normal serum electrophoretic profiles, while the others (all men), 58 to 74 years, had IgM monoclonal gammopathy of undetermined significance (MGUS). Clinical data were consistent with a sensory neuropathy affecting predominantly the kinesthetic sense (position and vibration sensation). The electrophysiological data indicated predominant sensory axonal neuropathy. Morphological data confirmed the primary axonal damage. Western immunoblot showed that the IgG from a patient without MGUS reacted with a 55 kD protein of dorsal root ganglion homogenate. Three of four patients with IgM MGUS were serum reactive against chondroitin sulfate C (ChS-C) in double immunodiffusion. After absorption with ChS-C the monoclonal peak completely disappeared from two patients and was decreased in the third patient. Our data indicate that immunological abnormalities are part of the pathogenesis for a subgroup of chronic neuropathy with only sensory symptoms.

Aged↗
PubMed1991

Polyneuropathy associated with IgA monoclonal gammopathy: a hypothesis of its pathogenesis.

We describe three patients with chronic progressive polyneuropathy associated with IgA monoclonal gammopathy. Two patients had a prominent sensory neuropathy and one had a prominent motor neuropathy. Sural nerve biopsies showed axonal degeneration in all cases. In immunocytochemical studies patients' IgG immunostained axons. By Western immunoblot a band of IgG reactivity with an axonal protein of 66 kDa was found. No band of IgA and IgM were found. We suggest the possibility that the IgA monoclonal protein may act as a stimulating factor of preexisting B cell clones eliciting an immune reaction against nerve antigens.

Aged↗
PubMed1991

Early detection of skin and muscular involvement in Lafora disease.

Two siblings with Lafora disease (LD) are described: one with epilepsy, myoclonus, EEG abnormalities, severe dementia and many Lafora bodies (LBs) in muscle and skin tissue; the other with myoclonus, epilepsy, EEG abnormalities and LBs in muscle and in skin tissue, without dementia. The findings suggest that the diagnosis of LD by skin and muscular biopsy is possible in the early stage of the disease, when there are myoclonic epilepsy and EEG abnormalities, before the onset of dementia.

Adolescent↗
PubMed1991

Axonal neuropathy in a patient with monoclonal IgM kappa reactive with Schmidt-Lantermann incisures.

We report a patient with a progressive, predominantly sensory neuropathy and a IgM kappa M-protein that binds to Schmidt-Lantermann incisures. A sural nerve biopsy showed primary axonal damage and IgM deposits at Schmidt-Lantermann incisures were seen by direct immunoperoxidase. Serum from the patient injected into rat sciatic nerve reacts with the incisures as with those in the patient's nerve. The IgM kappa M-protein reacts with chondroitin sulfate C and binds to a broad nerve protein band with a mobility of between 170 and 118 kDa. Peripheral neuropathy may be related to the M-protein, which had immunocytochemical reactivity not previously described for patients with polyneuropathy and IgM monoclonal gammopathy.

Axons↗
PubMed1991

Morphological and functional evaluation of peripheral nerve regeneration in the rat using an expanded polytetrafluoroethylene (PTFE) microprosthesis.

The aim of our study was to evaluate in the rat the ability of a polytetrafluoroethylene microprosthesis (PTFE), to guide the peripheral nerve regeneration between the two extremities of a transected sciatic nerve. In 15 adult male Wistar rats, weighing 200 g, a segment of the right sciatic nerve was resected, leaving a gap of about 1 cm, bridged with microprosthesis, using our original microsurgical technique. Neurophysiological evaluations were performed at 6 and 9 months post-operatively to study the distal motor latency either in the right sciatic nerve or in the unoperated control side. In all the rats myoelectrical responses with an increased latency of the operated side were produced from the interosseous muscle of the foot. The animals were sacrificed 9 months post surgery. Histological sections at the level of the graft were done in all the rats, and in 10 animals biopsies of the tibialis anterior muscle (TA) of each side were performed. An active process of axonal regeneration was documented inside the graft, with no infiltration of nerve fibers through the wall of the prosthesis. A connective fibrous reaction was present around the external wall of the graft. Muscle biopsies showed definite signs of muscle reinnervation, with residual features of variable degree of denervation. These findings stress and confirm the ability of the PTFE graft to allow effective regeneration in a peripheral nerve gap in the rat.

Animals↗
PubMed1990

Callosotomy for the treatment of drug resistant generalized seizures.

Fifteen patients have been followed for more than one year following callosotomy having presented with long standing epilepsy, no well defined focus amenable to radical excision, and severely incapacitating atonic seizures that were refractory to anticonvulsant therapy. Atonic fits have been reduced by more than 80% in thirteen patients, with two patients suffering long term sequelae (slight dysarthria in one, and dyslexia with mild visuo-spatial disturbances in another). Anticonvulsant therapy was still required post-operatively.

Adolescent↗
PubMed1990

Evolution of neuropsychological changes after partial callosotomy in intractable epilepsy.

Early neuropsychological changes following partial anterior callosotomy were evaluated in 15 patients with epilepsy (10 secondary generalized and 5 Lennox-Gastaut syndromes) by comparing their performances 1 month before surgery and then 15-20 and 90-100 days postoperatively without modifying the anticonvulsant treatment. The following neuropsychological and motor functions were tested: memory, attention, visuo-motor ability, posture, motor dexterity, language, praxis and gnosis. Social behaviour was also investigated. The main results are as follows: (i) most cognitive functions showed no significant variation; (ii) motor organization was still slightly impaired at the second check-up after the surgery; (iii) improvements in social behaviour and posture were frequently observed at the final evaluation. The analysis of individual cases highlights the influence of the extent of commissurotomy, lesions related to the surgical procedure and preoperative brain damage in determining the post-surgical profile. On this basis, the short-term neuropsychological cost of partial callosotomy appears to be low and seems to depend mostly on surgical parameters and brain conditions before the operation.

Adolescent↗
PubMed1989

Callosotomy for the management of intractable non-focal epilepsy: a preliminary personal assessment.

After reviewing the data of the literature, the authors report their personal series composed of twelve cases. In evaluating the results of surgical treatment only the first ten patients are taken into account. As previously stressed in the literature, callosotomy (total in two cases and anterior in the remainder) has proved effective in abolishing or significantly reducing atonic seizures. Tonico-clonic seizures have also benefitted in some cases, while in complex partial and partial seizures the effects of callosotomy have been so far unpredictable, albeit in some patients all kinds of seizures have been abolished or definitely reduced. The most impressive EEG and neuropsychological features after callosotomy are briefly summarized. Anterior callosotomy seems to be a relatively safe procedure, its results appear to be, on the whole, gratifying even though in patients with severe mental retardation and Lennox-Gastaut syndromes remain controversial.

Adolescent↗
PubMed1989

[Remarks on callosotomy in the treatment of drug-resistant epilepsy].

In this report 17 patients with long-standing non-focal epilepsy underwent callosotomy (this was total in two patients and performed in two stages, and anterior-subtotal in the remaining patients). In all patients the atonic-hypertonic seizures with sudden falls were the most disabling epileptic fits. Callosotomy proved efficient in controlling atonic fits in 10 out of 15 patients in whom surgical results are evaluated. In 3 additional patients the frequency of atonic fits was reduced by more than 50%. In the remaining two patients, no therapeutic effect was observed. Callosotomy was less effective on seizures which were not atonic. Therefore, this procedure appears to be indicated in patients in whom atonic fits are predominant. The main effect of callosotomy is to transform drug-resistant seizures into drug-sensitive ones. Neuropsychological sequels are insignificant unless the splenium is severed. However, considerable psychic and behavioral improvement was nearly always observed after surgery. Despite the fact that on a therapeutic level results were often satisfactory, a number of practical problems still remain. These concern the full spectrum of indications for callosotomy, the extent of corpus callosum section, choice of methods in severely mentally retarded patients and, finally, the age at which the operation should be carried out.

Adolescent↗
PubMed1989

Functions of the corpus callosum: observations from callosotomy performed for intractable epilepsy.

The effects of complete and partial corpus callosotomy in 6 patients are reported. Only the 2 cases undergoing total callosotomy showed evidence of impaired interhemispheric sensory transfer, related to sectioning of the splenium. Only mild long-lasting neuropsychological deficits were detected. Post-commissurotomy mutism and akinesia appeared in 4 cases, 2 with total, and 2 with partial anterior callosotomy. The short-and long-term effects of corpus callosotomy appear to be related to the extent of the section the creation of lesions during the surgical procedure, and a peculiar organization of cognitive functions in chronic epileptic patients.

Adult↗
PubMed1988

Neuropsychological changes after callosotomy in drug-resistant epilepsy: a study of the short-term evolution.

With the increasing interest in callosotomy as treatment for intractable epilepsy, it seems to be important to define the neuropsychological consequences of the related surgical operation. 8 patients suffering from drug-resistant seizures underwent section of the corpus callosum, 6 in the anterior part only and 2 undergoing complete two-stage commissurotomy including the posterior part. Before the callosotomy the patients were studied using a cognitive, affective and behavioural battery which was repeated 15 and 90-100 days after the operation. The patients with sufficient I.Q. were described in more detail using the cognitive parameters; the social and motor behaviour of Lennox-Gastaut subjects are accurately reported. No disconnection syndrome was ever observed after the partial commissurotomy while it occurred in one of the two complete callosotomies. The patients showed longer reaction times and a mild impairment of linguistic, praxic, memory and motor functions in the former evaluation (15 days), but there was consistent improvement in the latter check up. At the 90-100 day follow-up the Lennox-Gastaut patients responded more readily to the environmental stimulations and their postural, motor and behavioural functions were unchanged or improved, with respect to presurgical performances. The social and emotional behaviour of all the patients had always improved by the time the long-term evaluation (90 days) was performed. Finally, by limiting the callosotomy to the anterior part only, the neurological and psychological consequences seem to be limited.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Cryoglobulinaemic neuropathy. A clinical, morphological and immunocytochemical study of 8 cases.

Clinical, pathological and immunocytochemical studies are described for 8 patients with cryoglobulinaemia and peripheral neuropathy: 5 had essential cryoglobulinaemia and 3 secondary cryoglobulinaemia. The cryoglobulins in 2 cases were type II (mixed cryoglobulins with a monoclonal component), type III (mixed polyclonal cryoglobulins) in 2 others, and were not characterized in 4. In all patients the neuropathy had an acute or subacute onset and in 7 it was initially asymmetric but, during the illness, in 3 it gradually progressed to a symmetric sensorimotor polyneuropathy. All patients had sural nerve biopsies. In 4 there was widespread vasculitis with necrosis of vessel walls and perivascular inflammatory cells. In another 2, thickening of vessel walls and luminal narrowing without inflammatory cell infiltration, together with loss of myelinated fibres from single fascicles or focally within fascicles, suggested that the neuropathy was of vascular origin. In all patients the main pathological damage was axonal degeneration. Signs of axonal regeneration were rare. The total number of myelinated fibres was reduced in all cases, from 24 to 95% below the lower limit of normal control values. Large diameter fibres were lost preferentially in 7 patients, being below 16.5% of the total number. Several possible pathogenetic mechanisms have been suggested for cryoglobulinaemic neuropathy, including immunologically mediated demyelination and ischaemic injury due to intravascular deposits of cryoglobulins or vasculitis. Our observations therefore confirm a major role for ischaemic factors, secondary to inflammatory vascular destruction, in the pathogenesis of peripheral neuropathy in most cases of types II and III cryoglobulinaemia.

Adult↗
PubMed1986

Ichthyosis accompanied by neurological symptoms with special reference to epilepsy.

5 patients with ichthyosis had a neurological accompaniment: epilepsy in 4, congenital palpebral ptosis, facial pain and neurosis in 1. In one patient epilepsy was combined with multiple malformations (normal dwarfism, prematurely old face, skeletal abnormalities) and oligophrenia. There was considerable variability genetically: 2 sporadic cases, 1 with X-linked transmission, 1 with autosomal dominant and 1 with apparent autosomal recessive heredity. In one case the co-existence of glucose-6-phosphate dehydrogenase deficiency provided proof of X-linked transmission. Further study of larger case-series is needed for a better definition of the nosographic and genetic aspects of non blastomatous neuroectodermatoses in which ichthyosis figures.

Adolescent↗