Regulation of transcription of the human T cell antigen receptor delta chain gene. A T lineage-specific enhancer element is located in the J delta 3-C delta intron.

We have defined transcriptional enhancing sequences inside the TCR-delta gene locus, using transient transfections with constructs containing DNA fragments cloned upstream to a reporter gene fused to a heterologous promoter. A 14-kb DNA region extending from the J delta 3 segment to 6 kb 3' to C delta was analyzed. We show the presence of positive regulatory sequences inside the J delta 3-C delta intron and have localized these sequences to two DNA fragments of approximately 300 and 258 bp. Analysis of cell specificity of the activation of such sequences demonstrates a T cell pattern for one of the two fragments. The nucleotide sequence of the T cell-specific element shows motifs sharing homology with previously described core enhancers.

The V gamma locus of the human T cell receptor gamma gene. Repertoire polymorphism of the first variable gene segment subgroup.

Southern blot analysis using a genomic probe of the human TCR-gamma chain first variable gene subgroup (V gamma I) was performed on DNA samples from both parents of 36 healthy Caucasian families. Two types of polymorphisms were found in these 72 unrelated DNA samples: three repertoire polymorphisms and two restriction fragment length polymorphisms (RFLP). In all cases, Mendelian inheritance of these polymorphisms was demonstrated. The most frequent repertoire polymorphism consists in the lack of the V gamma 4 and V gamma 5 segments. In 16% of chromosomes, the Eco RI and Taq I restriction fragments corresponding to V gamma 4 and V gamma 5 were lacking, with no additional bands. In these cases, a decrease of 10 kb was observed in the Bam HI fragment containing all V gamma I segments as compared with samples containing V gamma 4-V gamma 5 segments. To better understand this polymorphism, which takes place in a previously incompletely defined region, the central part of the V gamma I region, including the polymorphic V gamma 4-V gamma 5 segments, was cloned. This allowed us to localize precisely the V gamma 5 segment and thus complete the description of the V gamma I region. A striking homology of DNA and deduced amino acid sequences is present between V gamma 2 and V gamma 4 and between V gamma 3 and V gamma 5, much higher than that observed between V gamma 2 and V gamma 3 and between V gamma 4 and V gamma 5. The differences in nucleotide sequence occur mainly in the intron and three hypervariable regions. These results strongly suggest a gene duplication relationship between the segments V gamma 2-V gamma 3 and the segments V gamma 4-V gamma 5. The most frequent RFLP documented in this study is due to the combined absence of the Eco RI and the Taq I sites located in the noncoding region between V gamma 3 and V gamma 4. The haplotypic frequence of this RFLP is 6.9% of the general population. As the gamma/delta receptor may play an important role in immunological response, the biological relevance of the high degree of polymorphism occurring in the V gamma I region, as well as its possible association with some immune disturbances, should be further explored.

MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity.

We report the MR studies of the cervical cord in seven patients presenting juvenile muscular atrophy of distal upper extremity. This illness, also known as monomelic amyotrophy or benign focal amyotrophy, is distinct from the other motor neuron diseases. Seen in young males, it is characterized by muscular atrophy of the hand, and usually of the forearm, most often unilateral. The underlying process, of unknown origin, affects the anterior horn cells in the lower cervical cord. The gradual onset of purely motor disturbances may mimic early amyotrophic lateral sclerosis. This latter diagnosis may be excluded because of clinical stabilization and lack of pyramidal tract involvement. In our series, five MR studies were positive. In three cases we were able to demonstrate focal and unilateral atrophy in the lower cervical cord limited to the anterior horn region. Morphologic MR findings correlated with clinical and electromyographic features. In two other cases the MR-clinical correlation was more complex. No pathologic MR signal was detected on either T1- or T2-weighted images. Although the diagnosis of monomelic muscular atrophy is based on neurologic and neurophysiologic data, MR provides confirmatory evidence as well as useful information contributing to an understanding of this disease.

Comparison study of CT and positron emission tomographic data in recent cerebral infarction.

To better understand the pathophysiologic correlates of the computed tomographic (CT) scan changes seen in recent cerebral infarction, 17 patients (20 studies) underwent both x-ray transmission and positron emission CT investigations within 18 days after clinical onset of complicated ischemic stroke in the internal carotid artery territory. The density changes before and after contrast study measured within the CT lesion were correlated to the local cerebral blood flow (CBF), oxygen utilization (CMRO2), and oxygen extraction fraction (OEF) measured with the oxygen-15 steady-state positron technique. Statistically significant linear correlations were found between hypodensity and CBF, hypodensity and CMRO2, and contrast enhancement and CBF, such that the more CBF and CMRO2 were depressed, the more marked was the hypodensity; and the more CBF was elevated, the more marked was the contrast enhancement. Although marked contrast enhancement was associated with decreased OEF (luxury perfusion), it was only rarely associated with increased CBF. Various hypotheses are discussed to explain these findings.

MR imaging of spinal cord arteriovenous malformations at 0.5 T: study of 34 cases.

Thirty-four patients with angiographically proved arteriovenous malformations of the spinal cord were studied between May 1986 and July 1987. Examinations were performed on a CGR 5000 Magniscan 0.5-T scanner with a surface coil in all cases, and multislices in both T1- and T2-weighted sequences were obtained in sagittal and axial planes. The results showed that MR can accurately diagnose all cases of intramedullary arteriovenous malformations, since transverse images were able to precisely locate the nidus within the spinal cord. Other types of spinal cord arteriovenous malformations were also studied (perimedullary fistulae and dural arteriovenous fistula), and the findings confirmed the value of MR in the characterization of these lesions as well.

Treatment of active gastroesophageal variceal bleeding with terlipressin or hemostatic balloon in patients with cirrhosis. A randomized controlled trial.

Gastroesophageal variceal bleeding due to portal hypertension should be treated by endoscopic sclerotherapy. This procedure, however, has some limitations. It has been established that vasoactive drugs are effective for controlling active variceal bleeding. We report the results of a randomized controlled trial comparing terlipressin to hemostatic tube (Linton-Michel tube) for the treatment of bleeding gastroesophageal varices in cirrhotic patients. Thirty-seven cirrhotic patients with a total of 40 episodes of gastroesophageal variceal bleeding were included in this trial. Patients were randomly assigned to intravenous terlipressin or Linton-Michel tube (LM tube), for 24 h. During this period, hemostasis was defined as obtaining of hemodynamic and hematocrit stabilization and/or absence of hematemesis or melena. Bleeding recurrence was assessed during a 1-month period after treatment. Twenty bleeding episodes were treated with terlipressin (Group I) and 20 with LM tube (Group II). Both groups of patients were similar in age, sex distribution, etiology of cirrhosis and degree of hepatic insufficiency. Bleeding was controlled in 70% of patients from Group I and in 95% from Group II (p < 0.05) during treatment. Bleeding recurred in 14% of patients in Group I vs. 36% in Group II 1 week following the treatment (p > 0.05) and in 16.6% in Group I vs. 83.3% in Group II 1 month after treatment (p < 0.05). Complications were more frequent in Group II than in Group I (65 vs. 15%, p < 0.05). Mortality rate was similar in both groups 1 month after treatment. In conclusion, hemostatic tubes were superior to terlipressin for the control of active gastroesophageal variceal bleeding within the first 24 h. Complications and bleeding recurrence were more frequent in patients treated by hemostatic tube within a period of 1 month after treatment. Mortality rate was similar in both groups of patients.

[Loss of a stent in the left main coronary artery and secondary migration into the left circumflex artery needing surgical extraction. Apropos of a case]. / Perte dans le tronc commun de la coronaire gauche d'une endoprothèse et migration secondaire dans l'artère circonflexe ayant conduit à une extraction chirurgicale. A propos d'un cas.

The authors report a case of accidental loss of a coronary stent in the coronary arteries and its migration into the circumflex artery. This complication occurred during revascularisation of the left anterior descending artery. In view of a dissection at the site of angioplasty and the migration of the stent, emergency surgery was undertaken comprising bypass grafting of the left anterior descending and arteriology of the left circumflex arteries to recover the stent. This is a rare complication, the frequency is probably underestimated. The authors discuss the factors predisposing to failure of implantation and the means of recovering the stents. The consequences of loss of a stent in the coronary or systemic circulations are also commented.

[Value of computer assisted tomography in the exploration of the sellar and supra sellar structures (author's transl)]. / Apport de la tomodensitométrie a l'exploration de la région sellaire et supra-sellaire.

The results obtained in 145 cases of space-occupying lesions in the sellar and suprasellar structures, after exploration with computer assisted tomography, were used to assess the technical and diagnostic value of this procedure. Precise details of the localization and extent of the lesion can be obtained, but not the nature of the lesion in many cases.

[Attempted treatment of a voluminous tympano-jugular and retropharyngeal glomus tumor by embolization]. / Tentative de traitement par embolisation d'une volumineuse tumeur glomique tympano-jugulaire et rétropharyngée.

The authors report a case of glomus tumor effectively treated by embolisation. The very large size of this tumor, the age and very poor general condition of the patient due to swallowing difficulties represented a contraindication to classical methods of treatment. Elective devascularisation was carried out by embolisation of the tumour itself using non-resorbable inert material by way of selective catheterisation of the vessels supplying it, almost all of which arose from the external carotid. Rapid and durable disappearance of part of the left cranial nerve involvement, in particular swallowing difficulties, reflected the effectiveness of the method. 18 months after this attempt at palliative treatment, the patient's condition remains satisfactory, with no detectable evidence of any recurrence or progression of the tumour.

[Meningeal arterio-venous fistulas with cortical venous drainage]. / Les fistules artério-veineuses méningées pures à drainage veineux cortical

Pure dural arteriovenous fistulas are still not properly understood, and pose problems wich are far from being resolved. Among these, dural fistulas with cortical venous drainage represent a very special entity since they alone are expressed clinically by a neurological brain syndrome. Diagnosis is difficult and mistakes can be made as it is only by means of hyperselective arteriography of the branches of the external carotid that an accurate diagnosis can be made. However, their diagnosis assumes considerable importance since surgical or radiological treatment of hese fistulas brings about complete disappearance of clinical symptoms.

[Non-lethal brain stem hematomas in hypertensive patients]. / Les hématomes bénins du tronc cérébral chez les hypertendus artériels.

Brain stem hemorrhages (peduncular, pontine, medullary) were demonstrated by CT scan in hypertensive patients, the outcome being favorable without surgical intervention. Such lesions are considered as being usually massive and fatal. A review of the literature show that hemorrhages in the brain stem represent 5 to 9 p. cent of intraparenchymatous hemorrhages, and are usually located in the pons. A favorable course was known to occur before the use of computed tomography: the rare cases described were often related to subacute hematomas in young normotensive subjects which could be treated by surgery with or without ventricular shunting. Clinical diagnosis is based on the rapid progressive course of the disorder and the location of the lesion. Computed tomography provides an immediate correlation between anatomical and clinical findings, and allows a better evaluation of semiological and prognostic features that were previously considered well established. A major element appears to be the degree to which the hematoma is tolerated. As far as possible neurosurgical procedures should be avoided in hypertensive patients.

[Pseudotumoral spinal cord dilatation during multiple sclerosis. 2 cases with magnetic resonance imaging]. / Dilatation médullaire pseudo-tumorale au cours de la sclérose en plaques. Deux cas avec imagerie par résonance magnétique.

We report 2 patients presenting with signs and symptoms of a medullary lesion. Spinal magnetic resonance imaging (MRI) using surface coils showed a volume increase of the spinal cord. Multiple sclerosis (MS) was suspected on the clinical evolution, the cerebrospinal fluid oligoclonal pattern in one case and the return of the cervical cord to an almost normal calibre on successive MRI. This diagnosis was further supported by the results of encephalic MRI. Such a pseudotumoral aspect of the spinal cord in MS has rarely been reported, and probably has been underestimated. MS should be considered in the differential diagnosis of localised enlargement of the spinal cord.

[Medical treatment of inoperable malignant glioma. Non-superselective intra-arterial chemotherapy with HECNU followed by conventional radiotherapy]. / Traitement médical des gliomes malins inopérables. Chimiothérapie intra-artérielle non hypersélective par HECNU suivie de radiothérapie conventionnelle.

Forty-six cases of non-surgical malignant glioma were treated by several repeated infusion of non-superselective intra-arterial chemotherapy using HECNU, followed by conventional radiotherapy. Chemotherapy was well tolerated immediately. Good responses rate was 46% for the whole group, but was higher in anaplastic astrocytomas (71%) than in glioblastomas (43%). The median survival for responders was 17 months, and clinical improvement was observed in a large majority of them. Later, neurological or ophthalmological ipsilateral complications of varying severity were observed in 8.7% and 15% of the cases respectively. Despite these complications the treatment was generally well tolerated and gave in responders a longer survival with better comfort.

Intracerebral stenosing arteriopathies. Contribution of three radiological techniques to the diagnosis.

Fifty-one patients with intracerebral stenosing arteriopathy were studied by computerized tomography (CT), magnetic resonance imaging (MRI) and cerebral arteriography. Clinical symptoms were varied, included impaired cognitive functions, progressive neurological deficit, headache and vomiting, and were sometimes not suggestives of the diagnosis. CT scans of the brain were normal in 25 percent of the cases or they were not probative because of various and non-specific abnormalities (hypodensity of various types or haemorrhagic hyperdensity). MRI always showed abnormalities but in many cases the lesions observed on T2-weighted images consisted of non-specific focal areas of high-intensity signal in the white matter. High-intensity signals in both white matter and cortex seemed to be more suggestive of the diagnosis. In this as in other studies, arteriography therefore remains the reference examination for stenosing arteriopathies. Inflammatory, infectious and atheromatous processes are the main causes of these arterial lesions. The aetiological value of radiological examinations is poor, and in most cases the morphology and distribution of the lesions does not point to any specific origin. However, herpes zoster arteritis usually affects the proximal segments of the anterior and middle cerebral arteries and spares the carotid siphon.

[Indications of arteriography and of emergency embolization in gastrointestinal tract and abdominal hemorrhages (author's transl)]. / Place de l'artériographie et de l'embolisation en urgence dans les hémorragies digestives et abdominales.

A propos 24 cases of gastro-intestinal tract's embolization of which eleven were performed in emergency for bleeding, the principle indications are described and illustrated. In some cases embolization gracefully replaces the ligation by a rapid and simple procedure at the time of diagnostic arteriography, preparing with safety the operative cure. In other cases embolization alone is curative: arteriovenous fistulas, telangiectases in 'Osler-'s disease, radiotherapic bleeding lesions, inoperable cancers. This implies the presence of trained radiologic teams and a tight collaboration with intensive care, endoscopy and surgery in large medical center. In this view the existence of radio-surgical rooms is useful.

[Iohexol in studies of the spinal canal. Multicenter study]. / L'Iohexol dans les explorations du canal rachidien. Etude multicentrique.

The author presents the results of a multicentric study of Iohexol in investigation of the spinal canal undertaken at the request of Winthrop Laboratories in 32 Radiological departments. The study involved 329 adults of both sexes. It confirmed the excellent quality of results obtained with this preparation in the literature and its excellent tolerance. On the basis of these results Iohexol may be considered to be definitely one of the best currently available preparations for investigation of the spinal canal.

[Ischemic accident caused by thrombosis of a venous angioma. Apropos of a case]. / Accident ischémique par thrombose d'un angiome veineux. A propos d'un cas.

Authors report one case of cerebral ischemic accident by thrombosis of a venous angioma of brain. This kind of malformation is quite unusual and was considered by most authors as unable to involve supratentorial ischemic complications. This case seems to be the first reported in the literature and demonstrates that venous brain ischemic accidents can result from venous angioma of the brain.

[Closed injuries to the extracranial carotid artery]. / Traumatismes fermés de la carotide extra-crânienne.

A series of 26 cases of injury to the extracranial carotid artery included 3 patients with false aneurysms, 21 dissecting aneurysms, and 3 aneurysms following direct trauma. Clinical and angiographic findings, and clinical course in the 26 patients are described, together with changes observed on angiography in the 11 cases where this was repeated. Prognosis in this series appeared to be better than that usually described.

[Preoperative embolization in spinal pathology. Apropos of 21 cases]. / Embolisation préopératoire en pathologie rachidienne. A propos de 21 cas.

Problems encountered during pre-operative embolization in 21 patients with spinal affections could be differentiated as a function of the 3 main regions affected: cervical, dorsolumbar, and lumbosacral. The distinction between the totally different problems raised was based on histologic and angiographic studies of the vascularization of the spinal cord and vertebral column. Whenever possible, and particularly when it can be complete, embolization markedly facilitates the surgical procedure.

[Computed tomography in the diagnosis and surveillance of cerebrovascular accidents]. / Apport de la scanographie au diagnostic et à la surveillance des accidents vasculaires cérébraux.

In cerebral hemorrhage, it provides an easy and early diagnosis, by demonstrating hyperdensity indicative of the presence of coagulated blood in the cerebral tissue. During the course of the illness, this hyperdensity progressively lessens, to disappear within a varying interval of time. The sequelae are seen as a cavity, the density of which is close to that of the CSF. In cerebral infarctions, the scan remains normal for the first few hours, and its only value at this stage is to eliminate cerebral hemorrhage before anticoagulants are prescribed. It is only after 24 to 48 hours that the fundamental sign is seen, that is hypodensity of the ischemic territory. This hypodensity may be accompanied by a mass effect and, above all, the uptake of contrast, which may be of great diagnostic importance, and the significance of which has been widely discussed. The course takes the form of the progressive development of the sequelary cystic appearance.