Observations on Myopathy

Familial muscular dystrophy of late onset

“…However, these authors agree that not all cases can be fitted into such a classification and suggest that further case andfamilial studies are needed in these instances. We describe here two cases of muscular dystrophy with a strong family history which appears clinically closer to the 'limited quadriceps myopathy' first reported by Bramwell (1923) than any of the above categories.…”

Quadriceps myositis: an appraisal of the diagnostic criteria of quadriceps myopathy

“…The term quadriceps myopathy is not a strict anatomical definition because, as in the present case, many of the previous reports indicate that patients may have lesser degrees of weakness in other muscles of the pelvic girdle or even in the upper limbs (Denny-Brown, 1939;Bramwell, 1922;Espir and Matthews, 1973), while in some other patients the upper limbs may be clinically normal but show myopathic changes on electromyography (Mumenthaler et al, 1958;Wijngaarden et al, 1968).…”

“…Selective wasting and weakness of the quadriceps muscles has been reported in two patients with myositis (Denny-Brown, 1939;Turner and Heathfield, 1961), two patients with unspecified myopathy (Bramwell, 1922), twelve patients with muscular dystrophy (Erb, 1891;Walton, 1956;Mumenthaler et al, 1958;Wijngaarden et al, 1968;Espir and Matthews, 1973) and one patient with chronic spinal muscular atrophy (Boddie and Stewart-Wynne, 1974). The latter report suggested that quadriceps myopathy is a clinical syndrome with various underlying pathologies.…”

Hereditary quadriceps myopathy

“…Myopathy clinically limited to the quadriceps muscles was first described by Bramwell (1923) and there have been a few later reports of sporadic cases (Denny-Brown, 1939;Walton, 1956;Turner and Heathfield, 1961). Bacon and Smith (1971) described a genetically determined muscular dystrophy, clinically predominant in the proximal limb muscles, but involvement of the arms was a prominent and early feature in their family.…”