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Biomedical subjects

C Scoppetta

Publications and source records attributed to C Scoppetta.

At least 37 records · Page 2Linked to original sources
PubMed1985

[Alzheimer's presenile dementia transmitted in an extended kindred].

Forty-three patients affected with Alzheimer's disease were identified in a kindred of Italian origin, emigrated in part to the U.S.A. and France. Thirteen were known by history, 21 by medical record, and 9 by personal examination, of whom 5 were confirmed histopathologically. The clinical picture was fairly uniform: the first symptom was memory loss beginning around age 40. Psychotic-like symptoms often followed, with rapid evolution into profound dementia, and death around age 50. Akinesia was prominent at a late stage, often with myoclonus. Grand mal seizures sometimes occurred, with occasional interictal spike and wave discharge; repetitive paroxystic periodic discharges were never recorded. A genealogical study, as far as possible free from line bias, has been conducted mainly by analysis of municipal records. 1 435 subjects in 10 generations, linked to affected subjects through ascent/descent or marriage, were listed in a computer file; the corresponding genealogical tree or selected part thereof are generated by computer. Application of Bayesian techniques to demographic data makes possible an estimation of disease probability in subjects for which no clinical data were available: such an estimate was confirmed by the later discovery of a living patient in descent of a subject with 0.7 estimated disease probability. No patient was found in descent from an inbred union known as such. Patients are the only transmitters. The sex ratio is not significantly different from 1. There is no detectable maternal effect. The segregation ratio, as calculated from extensively known sibships, lies in the range 0.65 to 0.89; the lower value itself is significatively higher than the 0.5 value expected in an autosomal dominant monogenic Mendelian transmission. An environment factor is ruled out by the diversity of locations and circumstances in kindred members. Such a kindred may represent an useful model for fundamental studies in Alzheimer's disease and senile dementia of the Alzheimer type.

Adolescent↗
PubMed1984

Myocardial damage due to hypokalaemia and hypophosphataemia.

A case of severe hypokalaemia with stupor, skeletal muscle and heart muscle damage is reported. An initial infusion of glucose-insulin and potassium (GIK) produced a temporary clinical improvement with reduction of creatine kinase (CKMB) and elevation of serum K+. On the 4th day of treatment, neuromuscular and cardiovascular deterioration occurred accompanied by a further rise of CKMB. This deterioration was coincident with a serum phosphate of 0.26 mmol/l. The impaired left ventricular (LV) function was measured using echocardiography and detecting the ejection fraction (EF). GIK was stopped and a potassium phosphate infusion commenced. As the phosphate and potassium deficiencies were corrected, the neuromuscular and cardiac abnormalities resolved, CKMB fell to normal and LVEF rose from 40% to 72%. We suggest that additional cardiac damage due to hypophosphataemia may have occurred in this patient, who already had cardiac impairment as a result of profound hypokalaemia. Possible mechanisms are discussed.

Echocardiography↗
PubMed1984

Adverse interaction between acetazolamide and anticholinesterase drugs at the normal and myasthenic neuromuscular junction level.

At skeletal neuromuscular junction level in vivo and in vitro experiments have revealed an adverse reversible interaction between acetazolamide and anticholinesterase drugs. Acetazolamide (500 mg, i.v.) prevented the increase in amplitude induced by edrophonium (5 mg, i.v.) on the action potentials derived by surface electrodes from the opponens pollicis muscle of patients affected by myasthenia gravis, when the median nerve was stimulated at the wrist by low frequency repetitive pulses (5/s). Similarly, acetazolamide significantly reduced the contractile force potentiation induced by neostigmine on the rat phrenic-diaphragm preparation, indirectly stimulated by means of low frequency repetitive pulses on the motor nerve. Under such experimental conditions acetazolamide did not show any significant action of its own, but it counteracted the effects of anticholinesterase drugs only when tested before them. It is hypothesized that the effect of acetazolamide on the skeletal neuromuscular junction may occur at presynaptic and/or postsynaptic sites by a mechanism only partly ascribable to the well-known carbonic anhydrase inhibitory activity of this drug.

Acetazolamide↗
PubMed1983

Myasthenia gravis in the elderly: report of 37 cases.

The treatment of myasthenia gravis in the elderly is controversial. Thirty-seven myasthenic patients with onset of the disease after the age of 60 were followed for a period of 14 years. All of the 37 patients received anticholinesterase drugs during this period, ten underwent thymectomy, and 24 were treated with corticosteroids. At present, one patient is in remission, 28 are improved, one is unchanged, and seven have died. Only one death was directly related to myasthenia. In the authors' experience thymectomy can be an effective treatment of myasthenia gravis in elderly patients; corticosteroid therapy can also be useful in addition to or as an alternative to surgery. Using a "personalized" schedule the authors obtained good results in 78 per cent of their patients.

Aged↗
PubMed1983

[Myasthenia and sleep].

A Central Nervous System (CNS) acetylcholine receptors involvement in Myasthenia Gravis (MG) has been suggested but never fully demonstrated. On the other hand, although the question concerning the neurotransmitters connected to REM sleep is still unsettled, the importance of acetylcholine as a neurotransmitter involved in the maintenance of this sleep stage has been outlined. The spontaneous night sleep organization has been studied in 9 male subjects with MG and results are compared with those obtained in normal subjects matched for age and sex. Statistically significant differences have been found: 1) slow-waves sleep better represented in MG patients; 2) average REM period length shorter in MG patients; 3) instability of the EEG consisting in a tendency toward lightening of sleep. These findings may confirm the presence of a disturbance in the CNS cholinergic activity of MG patients.

Adult↗
PubMed1982

When is there a full recovery for a myasthenia gravis patient?

A myasthenia gravis (MG) patient who seems to have recovered can later have recurrence of myasthenic signs. Clearly clinical remission does not always correspond to the normalization of all the factors involved in the pathogenesis of the disease. In ten patients who had apparently recovered from MG, electromyographic tests of repetitive supramaximal stimulation were performed and the anti-acetylcholine receptor (anti-AChR) antibody was assessed. In two of the ten patients all these tests were normal, thus showing lack of electromyographic myasthenic fatigability and the absence of circulating anti-AChR antibodies. Our hypothesis is that for these two subjects the risk of a recurrence of MG is lower than for the others.

Adolescent↗
PubMed1981

Sporadic distal myopathy.

The sporadic distal myopathies are uncommon primary muscle diseases, the pathogenesis of which is still unclear. The inclusion body myositides are inflammatory myopathies, the distal form of which presents some features resembling those of sporadic distal myopathy. A case is reported of a patient showing features of both the first and the second forms.

Adult↗
PubMed1981

Clinical features of amyotrophic amyotrophic lateral sclerosis.

The clinical features and course of amyotrophic lateral sclerosis are discussed. The data on a series of 116 patients are compared with those of the literature. The following points emerge: 1) when the disease starts before the age of 50, the prognosis is often less poor than usual; 2) the forms with spinal, and especially cervical, onset appear to be less rapid than bulbar forms; 3) in 20% of the patients survival is over 5 years. There may be some unknown factor that increases the resistance of some subject to the disease.

Adult↗
PubMed1981

Circulating immune complexes in myasthenia gravis: a study in relation to thymectomy, clinical severity and thymus histology.

Circulating immune complexes were assayed employing the method recently described by Barnett and Chia in a group of patients with myasthenia gravis. The subjects were classified according to clinical severity and immune complexes were sought before and after thymectomy. The operated subjects were further divided into those with thymoma or thymic hyperplasia. Antigen-antibody complexes were higher before thymectomy than after, in hyperplasias than in thymomas, and in severe myasthenia gravis than in mild disease. Circulating immune complexes and anti-acetylcholine receptor antibodies did not correlate.

Antigen-Antibody Complex↗
PubMed1981

Waldenström's macroglobulinemia and myasthenia gravis.

Until now, three patients with IgG and only one with IgM class monoclonal gammopathy (without the classical features of Waldenström's macroglobulinemia) have been reported in subjects with myasthenia gravis. A case of Waldenström's macroglobulinemia which occurred before thymectomy in a myasthenic patient is described in this paper. Both neuromuscular disease and the lymphocyte dyscrasia worsened after operation and before starting steroid treatment. No evidence of circulating immune complexes nor of anti-acetylcholine receptor antibodies belonging to the IgM class was found. The patient's HLA type shared A2 and B15 antigens with an IgG-lambda monoclonal gammopathy previously reported in a myasthenic woman, and his genotype included the Bw15 specificity which has been described to be frequent in Waldenström's macroglobulinemia. It appears that a persistent thymic abnormality, responsible for myasthenia gravis, may be associated with a lymphoreticular neoplasm.

Adult↗
PubMed1980

Myasthenia gravis, thymectomy, and antiacetycholine receptor antibody.

The antiacetylcholine receptor antibody was titered in the serum of 63 patients with myasthenia gravis (MG) and 20 control healthy subjects. The titer was significantly high in 92% of MG patients in contrast with none of the controls and no correlation was found with the thymus pathology and the severity of the disease. The titer decreased after thymectomy almost steadily with the improvement of the myasthenic signs. The role of the antibody in the pathogenesis of the disease is discussed.

Acetylcholine↗