[Rigid spine syndrome and its nosological borders. 2 cases].

The rigid spine syndrome is characterized clinically by rigidity due to deficient extensor muscles of the spine (chiefly cervical) and radiologically, by a normal spinal image. Electromyography demonstrates abnormalities suggestive of a myogenic process, and histology shows alterations of muscular fibres. From the case-records of two male patients aged 14 and 15 years respectively, a relationship could be established between various diseases having in common a rigid spine syndrome.

[Tendon areflexia in congenital myopathies accompanied by atrophy of type I fibers. Electrophysiologic study].

A predominance and/or an atrophy of type I fibers and a loss of deep tendon reflexes are often observed in different types of congenital myopathy. Various data indicate that both findings can be linked: dysfunction of the myotatic reflex can induce predominant involvement of type I fibers. In order to specify the mechanism of the loss of tendon reflex, an investigation of the Hoffmann reflex (H reflex) was performed in one case of centronuclear myopathy and in one case of congenital type fiber disproportion with type I hypertrophy.

[Chronic X-linked recessive bulbospinal amyotrophy (Kennedy-Stefanis type). Apropos of a case].

A new observation of chronic X-linked recessive spinal amyotrophy is reported. This series of cases fits the description of Kennedy-Stefanis chronic spinal amyotrophy, whose main characteristics are: sex-linked recessive inheritance, weakness and bulbo-spinal amyotrophy with proximal predominance, facio-lingual fasciculations, areflexia, gynecomastia in half of cases, very slowly progressive course, neurogenic EMG with normal nerve conduction velocities, and neurogenic muscle biopsy.

Peripheral neuropathy induced by amiodarone chlorhydrate. A clinicopathological study.

Four cases of amiodarone neuropathy are reported. Patients presented a sensorimotor neuropathy with distal predominance. Improvement occurred after drug discontinuation.

[Late post-poliomyelitic muscular atrophy. Apropos of 14 cases].

The authors report 14 cases of late post-poliomyelitis muscular atrophies. The atrophy appears many years after the acute episode of anterior poliomyelitis. Several types of disorders were observed: extension of weakness and wasting; chronic spinal amyotrophy; amyotrophic lateral sclerosis without bulbar atrophy, coincidental muscular dystrophy.

[Chronic spinal amyotrophy with paralysis of the vocal cords: Young-Harper syndrome].

A 59 year old man had a 20 year history of proximal muscle weakness and proximal and distal amyotrophy with areflexia and vocal cords paralysis. The EMG and muscular biopsy were compatible with a chronic spinal atrophy. The sister of this patient was said to be suffering from a similar syndrome.

[Chronic distal spinal amyotrophy localized to the both upper limbs (O'Sullivan and McLeod type)].

A case of sporadic spinal muscular atrophy affecting the upper limbs and with a chronic course over 19 years is reported, features of the affection being similar to the rare cases reported in the literature. Distinguishing aspects of these cases are those of a particular topographical form of chronic spinal muscular atrophies: onset in adolescents or young adults, symmetrical weakness and atrophy of the small muscles of the hands sometimes extending to the forearms, a progressive and slow course, absence of sensory or pyramidal signs, normal findings on cervical cord examinations neurogenic electromyogram recordings and normal nerve conduction rates.

[3 cases of scapulo-peroneal neurogenic amyotrophy (Dawidenkow's syndrome). Nosological situation in relation to Charcot-Marie-Tooth disease].

Three cases of neuropathic scapulo-peroneal syndrome are reported. One case was of the hypertrophic type, two were of the neuronal type. The nosological situation of the Dawidenkow syndrome is discussed.

[Clinical study of Bi-Profenid in rheumatologic practice].

The effectiveness and tolerance of Bi-Profenid as 150 mg tablets were analyzed in a multicenter study. Each of the 288 patients entered in the study had one of the main conditions found in rheumatologic practice: arthrosis of the hip or knee, rheumatoid arthritis or ankylosing spondylarthritis. Each patient was given one 150 mg Bi-Profenid tablet twice daily for 30 days.

CT of primary muscle diseases.

Seventy-five patients with a variety of muscular dystrophies were studied using computed tomography (CT). At least 11 slices were taken in each patient, from the forearm to the lower leg. Sufficient information was obtained to provide some CT characteristics of several dystrophies, including Duchenne muscular dystrophy, facioscapulohumeral syndrome, limb-girdle muscle myopathies, and myopathic dystrophies.

Complete monitoring of the purification of the plasma membrane from rabbit skeletal muscle.

A fast and reproducible purification procedure for rabbit skeletal muscle plasma membrane is described. Each step was monitored by determination of tetrodotoxin, ouabain and insulin receptors. A ouabain-sensitive K+-stimulated and a Ca2+-dependent phosphatases, probably identical to, respectively the (Na+-K+) and Ca2+-ATPases, were also evaluated.

[The joint in algodystrophy. Joint fluid, synovium, cartilage].

The joint fluid in cases of algodystrophy was studied in 17 samples. It is poor in cells (275/mm3 with a mean of 15% polymorphs). The protein level was studied 4 times and was equal to or greater than 40 g/l, i.

[Bone and phosphoro-calcium metabolism in reflex sympathetic dystrophy].

A combined study organised by the French Society of Rheumatology was devoted to the investigation of bone and phosphoro-calcium metabolism in cases of reflex sympathetic dystrophy. The following observations were made: the usual phosphoro-calcium parameters are not altered, apart from a slight elevation of the urinary calcium in multifocal forms of the disease, during the 3rd and 4th months; the level of PTH, studied in 11 patients, was normal in each case; the examination of 8 bone biopsies, one performed in the 7th week and six others performed during the 3rd and 4th months of the disease, showed, initially, invasion of the spongy tissue by oedema, signs of marrow stress and bone stress, with a reduction in the number of osteoblasts, without any marked alteration of bone remodelling. At a later stage, the biopsy shows intense bone remodelling with hyperosteoclastosis and hyperosteoblastosis and the formation of irregular bone tissue which later becomes lamellar.

[Myotonia with muscular weakness corrected by exercise. The therapeutic effect of mexiletine].

A case of generalized myotonia with autosomal recessive transmission related to Becker's type is reported. A muscular weakness improved by exercise was combined to myotonia. In addition to abundant myotonic discharges, electromyography showed alteration of the voluntary pattern during sustained contraction.

[A forgotten muscular dystrophy: Ullrich's disease].

The muscular disease described by Ullrich (1930) is no longer included in classifications of neuromuscular disorders. A typical case is reported and 15 documented cases have been found in the German and Japanese literature. The clinical picture associates, paradoxically, retraction of proximal muscles and hyperlaxity of distal muscles and joints.

[Quadriceps myopathy or amyotrophic quadriceps syndrome. Nosologic study apropos of 10 cases].

Quadricipital myopathies are often mentioned but have been described in only about 10 papers. Based on a review of the literature and 10 personal cases, the term "quadricipital amyotrophic syndrome" is proposed for a group of affections which can currently be divided into: 1) primary muscular dystrophies: pure quadricipital myopathy, quadricipital myopathy "plus", lumbopelvifemoral myopathy though it is doubtful whether the latter should be included; 2) metabolic muscular disorders; 3) chronic polymyositis localized to the quadriceps; 4) spinal amyotrophy localized to the quadriceps.

[Algodystrophy: predisposition and pathogenic factors. Results of a multicentric survey concerning 765 cases].

The authors analyze the results of a cooperative and retrospective survey of seven hundred and sixty five cases of reflex dystrophies of all localizations. Most of the etiologies usually described were present. As for predisposing factors, some, such as psychic or vascular factors and alcoholism, seem to have been overestimated.