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Biomedical subjects

J M Grau Junyent

Publications and source records attributed to J M Grau Junyent.

At least 19 recordsLinked to original sources

[Inclusion-body myositis: a familial report of 3 cases].

Inclusion body myositis (IBM) is a myopathy classified until now within the group of idiopathic inflammatory myopathies (IIM). Nevertheless, its clinical and histological features are specific and different from the other IIM. It is refractory to corticosteroid therapy. Recently, a few cases of IBM with familial transmission have been described, which is the first report in our country; previous reported cases in literature are reviewed. The similarities of some forms of IBM with muscle dystrophies, rather than with inflammatory myopathies are discussed.

Adolescent↗

[Myopathy and polyneuropathy caused by colchicine].

A forty-six year old man with chronic renal failure and a toxic chronic liver disease developed progressive muscle weakness after a long trial with colchicine. Physical exam revealed muscle weakness and proximal muscle atrophy with hyporeflexia. Serum levels of creatine kinase were high and signs of myopathy and axonal and demyelinating polyneuropathy was evidenced in electrophysiological studies. Muscle biopsy disclosed a vacuolar myopathy, disruption of myofibers and dilatation of sarcoplasmic reticulum. The clinical pictures was attributed to a toxic myopathy and polyneuropathy due to colchicine, thus this treatment was discontinued. Four week later, the patient was symptom free, the levels of seric creatine kinase were normal and a new muscle biopsy was normal, with disappearance of previous histological findings.

Colchicine↗

[Thyrotoxic hypokalemic periodic paralysis].

A case of palsy without family history associated to hyperthyroidism, is described. This is an rare entity in our environment (only two cases have been previously described) and may develop with hypo, normo or hyperkalemia. Its potential pathogenicity is discussed, which finally results in a disorder of the ionic interchange at the level of the muscle membrane. The need to identify these secondary forms of recurrent palsy is stressed, given that it may benefit from an adequate treatment. In the case presented here, the normalization of the thyroid function was followed by the sustained elimination of the muscle symptomatology.

Adult↗

[Myositis with inclusion bodies: a little-known variety of idiopathic inflammatory myopathy].

The authors report two male patients with inclusion body myositis (IBM) and review the features of this condition in the literature. This is an uncommon type of idiopathic myositis which involves males more often than females; it usually develops in elderly patients; its course is very slow; it is usually associated with distal weakness; neurophysiological studies show a mixed "myogenic" and "neurogenic" pattern; and it is usually unresponsive to corticosteroids. The diagnosis is basically made on the basis of the histological features, mainly consisting of vacuoles surrounded by a basophilic haze in histochemical stains of frozen tissue and, particularly, by the presence of characteristic microfilaments in ultrastructural studies.

Humans↗

[Still's disease of the adult: analysis of a series of 9 cases].

Characteristic features of 9 patients (5 male, 4 female) affected by Still's disease of adult (SDA) were analysed. All of them had fever and in 7 cases it was associated to arthritis and cutaneous rash. The most frequently affected joints were knees, ankles, and carpus. The sedimentation rate was increased in all patients and 6 of them had associated leucocytosis. 5 patients had mild inflammation synovial fluid. The histological changes found were: cutaneous perivascular inflammation, reactive lymph node enlargement, sinusoid dilatation and hepatic periportal inflammation. The NSA were active in 4 patients but 5 needed steroids to control the systemic clinical alterations. 3 patients relapsed and one was diagnosed as having sarcoidosis 3 years later, the possible association not having being described. We concluded that suspicion is the most important factor for an early diagnosis.

Adolescent↗