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At least 19 recordsLinked to original sources
PubMed1979

Syndromes of compression of the median nerve in the proximal forearm (pronator teres syndrome; anterior interosseous nerve syndrome).

Entrapment of the median nerve in the proximal forearm is seen in two forms: the pronator teres syndrome, and the anterior interosseous nerve (or Kiloh-Nevin) syndrome. Both syndromes are rare, and they comprised approximately 1% of the compression syndromes of the upper limb which were treated operatively by the authors. The symptoms, signs, etiologies, and intraoperative findings are discussed. It is pointed out that certain of the clinical features may resemble those of irritation of the median nerve by a supracondylar process or Struthers' ligament. Although both proximal median entrapment syndromes have a favorable prognosis when treated non-operatively, the authors recommend operative treatment in cases in which there is no perceptible improvement following 8 weeks of non-operative treatment, since this is likely to speed and enhance recovery. Nine cases of the pronator teres syndrome (8 treated successfully by operation, 1 failure) and 2 cases of the anterior interosseous nerve syndrome (both fully recovered) are added to the cases reported previously in the literature.

Adult↗
PubMed1975

The C.C.A. syndrome (congenital contractural arachnodactyly): a new differential syndrome for Marfan's syndrome and homocystinuria.

The first case in the dental literature of congenital contractural arachnodactyly (C.C.A. syndrome) is presented. This newly delineated syndrome is an autosomal dominant heritable disorder of connective tissue. Its similarities to Marfan's syndrome and homocystinuria, as well as other syndromes, are discussed. The lack of cardiovascular disease, specific ocular anomalies, and mental retardation are presented in the differential diagnosis of the C.C.A syndrome with Marfan's syndrome and homocystinuria.

Arthrogryposis↗
PubMed1977

Sjogren's syndrome in SLE: Part 2. An examination of the clinical significance of Sjogren's syndrome by comparison of its frequency in typical and atypical forms of SLE, overlap syndromes and scleroderma.

The clinical features of Sjogren's syndrome were found in 24 percent of Glasgow patients with SLE without other atypical features. Sjogren's syndrome was found in one (13 percent) of a group of eight patients with features of both SLE and scleroderma, one of three patients with features of both polymyositis and scleroderma and in three of 12 (25 percent) patients with scleroderma. The highest frequency of Sjogren's syndrome was seen in six patients with features of SLE and an erosive polyarthritis, five of whom (83 percent) had Sjogren's syndrome. In patients satisfying diagnostic criteria for SLE no relationship between the presence of Sjogren's and the presence or absence of renal disease was found.

Adolescent↗
PubMed1979

[Styloid syndrome; Sluder's syndrome; Charlin's syndrome].

The styloid syndrome is caused by an irritation of the glossopharyngeal nerve from an excessive development of the styloid apophysis. Treatment consists of the resection of the same. The Sluder syndrome represents a nevralgy with origin in the sphenopalatine ganglion and a dysfunction of the parasympathetic system. The Charlin syndrome, much less frequent, manifests itself by pains in the territory of the nasociliar nerve and the ciliar ganglion.

Bone Diseases↗
PubMed1976

Prognosis of the Lennox syndrome-long-term clinical and electroencephalographic follow-up study, especially with special reference to relationship with the West syndrome.

The long-term clinical and electroencephalographic follow-up studies were carried out for more than three years, up to 14 years in the longest, on 116 cases with Lennox syndrome. And the results of s-ystematic study on changing patterns and out come have been reported. The follow-up examination was performed also on West syndrome which is closely related with Lennox syndrome; especially the relationship between both syndromes regarding prognosis has been clarified. (1) According to the long-term follow-up on 116 cases with Lennox syndrome, there were 98 cases (84.5%) having mes (61.2%) and persisted as Lennox syndrome except for one case. (2) Generally speaking, the cases with age of onset before two years old showed unfavorable outcome. (3) There were 42 cases (36.2%), which were converted from West syndrome and showed markedly unfaborable prognosis in regard to intelligence as well as the remainging of seizure. (31 cases, 77.5%) (4) On thehand, in 23 idiopathic cases, which showed no developmental retardation before onset of seizure, had favorable outcome, and the remaining of seizure was observed in eight cases (34.8%). However, even in such cases, it was noticed that those displaying mental defect at the follow-up attained 14 cases (60.9%). That is to say, it was clarified that persistence of even minor seizures induced mental deterioration. (5) The cases with favorable prognosis showed usually a typical slow spike-and-wave pattern electroencephalographically, whereas those with poor prognosis showed mostly an asymmetric or disorganized slow spike-and-wave pattern. (6) In many cases displaying signs of brain atrophy with pneumoencephalograpm and accompanying overt neurological signs at the initial examination, prognosis is obviously poor. (7) From the follow-up examination on 94 cases with West syndrome for three to 15 years transformed into Lennox syndrome Among them, those cases with the remaining of seizure at the time of follow-up were 44 (46.8%) out 94 cases, of which 37 cases (83.8%) had remaining seizure as Lennox syndrome. (8) From the above results, it is emphasized that Lennox and West syndromes show close relationship with each other and that a study should be done on the interrelation between their prognosis.

Adolescent↗
PubMed1975

The short arm deletion syndrome of chromosome 4 (4p- syndrome).

Partial deletion of the short arm of chromosome 4 (4p-) represents another (rare) cause of cleft lip and cleft palate. Further characteristic manifestations of the syndrome (also called Wolf or Wolf-Hirschhorn syndrome) are growth failure, microcephaly, prominent glabella, hypertelorism, beaked nose, poorly differentiated and low set ears, cardiac and renal malformation and hypospadias. Life expectancy is often shortened. The 4p- syndrome has many features in common with another deletion syndrome, the cri-du-chat syndrome, and also with the Smith-Lemli-Opitz syndrome. The latter is a hereditary condition with normal karyotype. The cri-du-chat syndrome is characterized by a peculiar high-pitched, mewing cry and can be differentiated from the Wolf syndrome by the different staining characteristics (banding) of chromosomes 4 and 5.

Abnormalities, Multiple↗
PubMed1975

Investigation of a pain syndrome of spinal origin (on the concept of the generator mechanism of the pain syndrome).

A pain syndrome was induced in rats by means of a microinjection of purified tetanus toxin into the posterior horns of gray matter of the lumbosacral segments of the spinal cord. The toxin was used as a means of disturbing inhibitory mechanisms. Investigation showed that a pain syndrome can be reproduced if afferent stimulation from the periphery is blocked (by division of the nerves of the hind limbs or division of the dorsal lumbosacral roots on the side of injection of the toxin). Under these conditions the latent period of onset of the syndrome was lengthened and the degree of its development weakened a little in the initial stages by comparison with animals with intact afferentation. In many animals with blocked afferentation from the hind limb general manifestations (restlessness, aggressiveness, crying, etc.) were accompanied by a localized response in the form of increased licking, biting, or even chewing the tissues of the deafferented limb at the site of projection of the pain (the phantom syndrome). In some animals only the general reaction was observed without localization of the pain (protopathic pain). In all cases the attacks of pain arose paroxysmally. In animals with intact limb innervation the zones of licking were trigger zones of facilitated induction of an attack of pain. Injection of glycine into the affected posterior horns of the spinal cord abolished the pain syndrome during the time of action of the glycine. It is concluded that the pain syndrome is based on the formation of a generator of pathologically intensified excitation, as a result of disturbance of inhibitory processes, in the system of neurons connected with pain sensation. These mechanisms are evidently those principally concerned in the pathogenesis of all pain syndromes.

Animals↗
PubMed1975

The feedback effects of sex steroid hormones on pituitary gonadotropin release in Turner's syndrome and Klinefelter's syndrome.

The present study was designed to elucidate the feedback relationship between the release of pituitary gonadotropins and sex steroid hormones in Turner's syndrome and Klinefelter's syndrome. LH-RH stimulation test was employed to evaluate the effects of sex steroids on the release of gonadotropins. The release of gonadotropins in response to LH-RH as well as in baseline level was suppressed after the treatment with estrogen (mestranol 0.08 mg/day) for 10 days, followed by the treatment of the same period with estrogen (mestranol 0.08 mg/day) and progesterone (chlormadinone acetate 2.0 mg/day) in combination in both syndromes. The inhibitory effect of the combined treatment was greater than that of the treatment with estrogen alone. Administration of testosterone propionate (25 mg/day) for 3 days resulted in suppression of the release of both gonadotropins in baseline level and in response to LH-RH in both syndromes, but the suppressive effect appeared to be less complete as compared with that of estrogen or estrogen-progesterone. It was thus verified that the feedback interaction between the pituitary gonadotropin release and sex steroids such as estrogen, estrogen-progesterone or testosterone was operative in the same fashion in the patients with Turner's syndrome and Klinefelter's syndrome.

Adolescent↗
PubMedDate not supplied

[Noonan syndrome: differential diagnosis with Turner's syndrome].

Clinical and laboratory evidences assure an unequivocal identity to the syndrome described by Noonan. We believed that the terminology used by many authors has contributed to maintain confusion with Turner's syndrome from which it is clearly differenciated. The signology of both syndromes was confrontated in order to delineate the syndrome. Emphasis was made to point out the signs which are proper to each syndrome and the signs which are common to both of them stressing those that, occur with equal or significant difference. Two new signs are described in Noonan syndrome: alopecia of the hund portions of the eyebrows and keratosis rubra pilaris (Ulerythema ophriogenes).

Adolescent↗