[Pediatric multiple sclerosis: is it different from the adult form?]. / La SEP de l'enfant: est-elle différente de celle de l'adulte ?

INTRODUCTION: Multiple sclerosis (MS) is not uncommon in children. The aim of this study was to compare early onset MS (EOMS) with adult onset MS (AOMS). METHODS: A retrospective study including MS cases between 1997 and 2010. EOMS was defined by age at MS onset<18years. Data were collected using the EDMUS database (European Database of Multiple Sclerosis) including: sex, age at onset, disease duration, EDSS, score after relapse. The MSSS and the Progression Index were calculated. Patients with disease duration less than one year were excluded. MS symptoms at onset and at further relapses were also noted. These parameters were compared between the EOMS and the AOMS groups. RESULTS: Two hundred fifty-nine cases were included including 31 EOMS (11.96%). The mean follow-up was 96months. The relapsing-remittent form was significantly more frequent in the pediatric group (94% vs 79%). Mean EDSS and MSSS scores and the percentage of fast progressors (MSSS>5) were lower in the EOMS group. Analysis of neurological symptoms at the first MS attack and further neurological events showed a lower frequency of gait disturbances, motor symptoms and bladder symptoms in the EOMS group compared with the AOMS group. The 10-year mean EDSS score was 1.9 for EOMS and 4.1 for AOMS, after 25years it was 4.5, and 7.27 respectively. CONCLUSION: This study highlights the relative frequency of EOMS in our MS population. However, different severity scores showed less disability progression in EOMS patients compared with AOMS patient; irreversible disability was reached at an early age.

[Paroxysmal dystonia and multiple sclerosis]. / Dystonie paroxystique et sclérose en plaques.

INTRODUCTION: Movement disorders are uncommon in multiple sclerosis, except for tremor. Patients rarely have paroxysmal dystonia (or tonic spasm), which can be the presenting manifestation of the disease. OBSERVATIONS: Two videotaped observations are presented. The first patient was a 27-year-old woman, treated for relapsing-remitting multiple sclerosis, who presented daily several short (<1minute) paroxysms of right hemibody dystonia. Brain MRI revealed several areas of cerebral demyelination, including the posterior limb of the left internal capsule with gadolinium enhancement. These events disappeared 7 days after corticosteroid infusion. The second patient was a 62-year-old man who presented brief episodes (<1minute) of daily painful left hemibody dystonia. Three months later, similar paroxysms affecting the right hemibody including the face occurred. At times, the two hemibodies were affected simultaneously. The brain MRI showed multiple areas of white matter hyperintensity, including two symmetrical areas in the posterior limb of the internal capsules. Multiple sclerosis was diagnosed on clinical, MRI and biological data. Four days after starting corticosteroids, these paroxysmal phenomena disappeared totally. CONCLUSION: Dystonia is an under-recognized aspect of paroxysmal events during multiple sclerosis. It might involve ephaptic transmission among abnormal demyelinated neurons; this ectopic excitation can arise at variable levels of the corticospinal tract, but the analysis of reported cases and those described in this study shows that impairment of the posterior limb of the internal capsule seems to be a prevalent topography. Inflammation is likely to play a role because steroids often improve these phenomena. In this article, we review the clinical aspects, pathophysiology and outcome of paroxysmal dystonia in multiple sclerosis.

Doppler ultrasound in aortic dissections: a study of cephalic and peripheral arteries.

We investigated the role of continuous-wave Doppler ultrasound in predicting the presence of an aortic dissection prospectively in 28 patients whose diagnosis was confirmed either by arteriography or surgery (26 cases) or at autopsy (two cases). We hypothesized that dissections, by creating two channels for flow, would produce velocity disturbances detectable in accessible arteries such as the carotid, brachial, and femoral arteries. Of the 28 patients, 20 had Type I, two Type II, and six Type III dissections. Two abnormalities of the Doppler signals were found: in 18 of Type I dissections, notching was found in the systolic upslope of the velocity tracing from those arteries that were either involved in or distal to the dissection sites. Notching was much less frequent for Type II and III dissections: only one patient with Type II and two patients with Type III dissections showed notching. In addition diastolic backflow with marked aortic valvular insufficiency was present. Notching in brachiocephalic artery continuous-wave Doppler signals appears to have a high positive predictive value for Type I dissections.

[Sensitivity and specificity of temporal Doppler in Horton's disease]. / Sensibilité et spécificité du Doppler temporal dans la maladie de Horton.

170 patients with suspected temporal arteritis underwent Doppler investigation before temporal artery biopsy. Doppler study included the large cervical trunks, the ophthalmic system and analysis of the terminal branches of the external carotid with recording of the temporal territory on 3 points. Biopsy confirmed the diagnosis of temporal arteritis in 48 cases; it was negative in 122 cases. Doppler study and histological examination agreed in 137 patients (80%). There was disagreement in 33 cases (20%). The Doppler signal was pathological in 43 of the 48 cases of temporal arteritis (90%). There were 5 false negatives (10%). In subjects free from temporal arteritis, the Doppler examination was normal in 94 cases (77%), and was pathological in 28 cases (23%). The sensitivity of Doppler examination in the diagnosis of temporal arteritis was 90%, its specificity was 77%. The predictive value of a positive Doppler vis-a-vis the condition was only 61%. On the other hand, the predictive value of a normal examination was very high (95%). The high level of false positives is explained by technical problems, but especially by a high incidence of sometimes very stenosing senile arteritis. Finally, 3 of the 28 false positives (11%) subsequently developed authentic inflammatory arteritis. The authors conclude that a positive Doppler examination does not allow reliable prediction of the presence of temporal arteritis. On the other hand, a normal examination almost always allows the diagnosis of temporal arteritis to be excluded with a risk of error of less than 5%.

[Role of Doppler in the diagnosis of ophthalmic complications of Horton's disease]. / La place du Doppler dans le diagnostic des complications ophtalmiques de la maladie de Horton.

A study of the eyeball was carried out using a double frequency Doppler transducer in 31 patients suffering from histologically confirmed temporal arteritis. These patients were divided into 3 groups depending on the results of the ophthalmological examination: group A: 20 patients; normal ophthalmological exam, group B: 5 patients; non specific ocular anomalies, group C: 6 patients; ocular lesions specific to temporal arteritis (ischemic optic neuropathy with amaurosis, dysoric nodules). Doppler examination demonstrated normal curves in the patients in groups A and B. The mean amplitude of systolic peaks was 7.9 mm in group A and 10.3 mm in group B (P = NS). These data did not differ from those of a control population of 22 subjects not suffering from temporal arteritis. On the other hand, group C demonstrated important anomalies on Doppler examination: lack of ophthalmic signal in one case; marked dampening of curves in 4 cases; zones of turbulence in one patient. The mean amplitude of systolic peaks was drastically decreased (1.8 mm). After steroid treatment, a significant increase in blood velocity was seen leading to a normalization of tracings in the majority of cases, including the patients in group C. Doppler examination would appear capable of reliably assessing the risk of ophthalmic complications of temporal arteritis when blood flow anomalies exist. On the other hand, patients with normal Doppler curves may be considered to be at little risk. Subjects at high risk should be urgently treated with high doses of steroids and can be regularly monitored by repeated Doppler examination.

Iatrogenic cardiac tamponade after central venous catheter.

Myocardial perforations with a central venous catheter are rare in adults (67 cases published since 1968). These accidents are fatal in more than two-thirds of the cases owing to confusion caused by misleading symptoms which suggest pulmonary embolism. The perforation is generally localized in the right atrium (29 cases), less frequently in the right ventricle (18 cases). The superior vena cava is rarely affected (3 cases). The site of the perforation was not found in the other published cases. Clinical symptoms are signs of tamponade with disorders of cardiac rhythm. An enlargement of the cardiac shadow and an abnormal position of the catheter, buckled or openly intrapericardial, make the diagnosis radiologically. Echocardiography provides some information, but this is often too late for practical application. The diagnosis is made with right catheterization when it shows an equalization of the diastolic pressures. This allows the patient to be watched closely following the pericardial tap, after which a surgical approach may be indicated and performed. Prevention of these iatrogenic accidents must be systematic and strictly controlled for.

[Malignant lymphoma of the heart. Contribution of modern technics of medical imaging]. / Lymphome malin de localisation cardiaque. Apport des techniques modernes d'imagerie médicale.

Although rarely encountered, tumours of the heart are now more easily and frequently diagnosed owing to the availability of non-invasive imaging methods, notably echocardiography. With echocardiography the cardiac extension of the tumour is perfectly visualized, but its connections with the mediastinum are less readily accessible. The case presented here of a female patients with a malignant lymphoma involving the heart (right ventricle and atrium, pericardium) and the mediastinum illustrates the complementary nature of echocardiography, computerized tomography and magnetic resonance imaging.

[Value of continuous Doppler velocimetry for the diagnosis of patent ductus arteriosus]. / Intérêt de la vélocimétrie Doppler continu pour le diagnostic de la persistance du canal artériel.

A study of the curve of wave Doppler ultrasonography of 3 arterial axes (carotid, subclavian and femoral) was performed in 32 children in whom the diagnosis of patent ductus arteriosus was suspected. A continuous 8 MHz wave directional ultrasonic Doppler apparatus was used. At the level of subclavian and femoral arteries, patent ductus arteriosus is associated with a negative holodiastolic wave on the curve. At the carotid level, disappearance of the physiologic diastolic elevation may be the only change observed. Three groups were studied (1) 15 premature neonates with isolated murmur, (2) 15 premature neonates with respiratory distress and (3) 2 older children. In group 2, diagnosis of patent ductus arteriosus relies on clinical signs. A good correlation between Doppler and clinical data and/or TM ultrasonography was observed in 93% of cases.

[Arterial stenoses of the arms in Horton's temporal arteritis. Apropos of 3 cases. A review of the literature]. / Sténoses des artères des membres supérieurs dans l'artérite temporale de Horton. A propos de 3 observations. Revue de la littérature.

The authors describe three histologically documented cases of temporal arteritis complicated by arteritis of the upper limbs confirmed by arteriography. The evolution during treatment was followed up until recovery. A review of the literature underlines the frequency of upper limb arteritis in Horton's disease. It may be the presenting feature or, more commonly, occur during the evolution of the condition. It may give rise to ischaemic symptoms and/or an arterial bruit and/or asymmetric blood pressure readings which should be looked for routinely. Doppler ultrasound may be helpful. Arteriography shows that the lesions are usually bilateral, on the subclavian and axillary arteries. Giant cell arteritis has been found on arterial biopsy. Upper limb arteritis necessitates renewed or increased steroid therapy which may be beneficial in the absence of thrombosis. These arterial lesions have also been reported in some cases of polymyalgia rheumatica. Several cases of isolated upper limb arteritis without temporal arteritis or polymyalgia rheumatica have also been described.

[Dysmorphic syndrome and vascular dysplasia: an atypical form of type IV Ehlers-Danlos syndrome]. / Syndrome dysmorphique et dysplasie vasculaire: une forme atypique d'Ehlers-Danlos type IV.

A 29 year old woman was hospitalized for the successive onset of extremely serious vascular accidents: rupture of the gastro-duodenal artery, aneurysm of the posterior tibial artery, discovery of bilateral carotid and vertebral aneurysm with development of a carotid-cavernous fistula. The patient had a very unusual morphotype with dwarfism, white hair and alopecia. Histological investigations failed to reveal atheromatous lesions and by contrast showed involvement of the skin (dermal atrophy) and in the blood vessels fibro-dysplasia of the media. This picture was suggestive of a vascular form of Ehlers-Danlos disease (Sack's syndrome or type IV Ehlers-Danlos disease). This syndrome is characterized by minor skin or joint manifestations replaced by arterial accidents (arterial rupture or development of aneurysms). The etiology is faulty maturation of procollagen III and the diagnosis is based upon fibroblast culture.

The carotid pulse in dissecting aneurysm of the aorta.

In five patients with acute aortic type I dissection a deep early systolic notch was recorded on the carotid pulse tracing. This peculiar feature of the carotid pulse was quite comparable to the aspect of the continuous wave Doppler ultrasound recorded along the carotid arteries. It can be explained by the temporary interruption of blood flow caused by false lumen in the artery. Therefore the carotid pulse can provide to the clinician a good help to the diagnosis of aortic dissection especially in case of nontypical presentation.

[Trapped popliteal arteries. Incidence, epidemiology, therapeutic considerations]. / Artères poplitées piégées. Incidence, épidémiologie, considérations thérapeutiques.

The trapped popliteal artery syndrome is an extrinsic dynamic compression of the vascular structures in the popliteal fossa by the surrounding fibromuscular structures. The condition mainly affects the popliteal artery resulting in atypical intermittent claudication because it often occurs in young and active patients. The arterial lesions are initially purely extrinsic and dynamic; sometimes they progress to thrombosis, embolism or aneurysm due to jet lesions. The diagnosis is obtained by radiology in the uncomplicated forms by showing compression or complete occlusion of the artery during contraction of the gastrocnemius and quadriceps muscles. Doppler studies of the posterior tibial artery may also demonstrate the condition during these manoeuvres. The epidemiological study was performed in 199 medical students and 80 athletes. A positional abolition of the posterior tibial artery was demonstrated in 39 students (19%) and 12 athletes (15%). The entrapment was commoner in females (24%) than males (13%). It was observed more frequently in dancers, cyclists and basket ball players: 12 control digitised intravenous angiographies were performed; the diagnosis was confirmed in 11 cases. Several anatomical anomalies of the popliteal fossa may give rise to this condition. The commonest is a deviation of the artery within the internal head of the gastronemius muscle; compression by the soleus or fibro muscular bands arising from the semi-tendinous or semi-membranous have been described. The treatment is surgical in very symptomatic or complicated cases; uncrossing the artery associated with venous grafting in cases of thrombosis are the procedures of choice.

[Iatrogenic internal mammary arteriovenous fistula. Apropos of 2 cases]. / Les fistules artério-veineuses mammaires internes iatrogènes. A propos de deux cas.

Two fairly similar cases of internal mammary arterio-venous fistula were observed in two patients involved in serious road accidents, following which a catheter was inserted into the subclavian vein for the purposes of resuscitation. The clinical diagnosis was made in both cases on routine examination, which revealed a continuous right subclavian bruit with extensive radiation three years after the accident. There were no functional implications. Further investigations revealed a fistula located between the internal mammary artery and the right brachiocephalic venous trunk and demonstrated the haemodynamic consequences: normal intra-cardiac pressures, but a pulmonary blood flow which was twice that of the systemic blood flow. Both cases were treated by means of surgical cure via thoracotomy, which confirmed the nature of the arterio-venous fistula and which defined the relations with the phrenic nerve, which is the most exposed element. The results of this operation were excellent in both cases.

[Aortocaval fistulas. Apropos of 5 cases]. / Les fistules aorto-caves. A propos de 5 observations.

UNLABELLED: 5 cases of aorto-iliac aneurysms which fistulised into the inferior vena cava are described. They illustrate the problems encountered in this condition. - DIAGNOSIS: although a classical diagnosis, it is only recognised in 27 p. 100 of cases because the clinical presentation can be very variable. A pulsating, expansive, abdominal mass very suggestive of an aneurysm of the abdominal aorta should alert to the possibility of a fistula within the inferior vena cava when the patient has unexplained cardiac failure, shock, lower limb deep vein thrombosis, urinary or psychological problems. In the absence of a continuous abdominal murmur, the diagnosis may be confirmed by complementary investigations; the investigations of choice are non-invasive. Abdominal ultrasonography showing an aneurysm of the abdominal aorta associated with dilatation of the inferior vena cava is very suggestive of the diagnosis. - The only treatment is surgery. The operative risk depends on the presence of absence of associated retroperitoneal rupture of the aneurysm, the respective mortalities being 83 p. 100 and 15 p. 100. - The principal causes of death are pulmonary embolism and renal failure which necessitate special prophylactic measures (surgical and anaesthetic).

[Pulsatile echography (ultrasonic cerebral tomosphygmography) in carotid and vertebral pathology. Surgical applications]. / L'échographie pulsatile (tomosphygmographie cérébrale ultrasonore) en pathologie carotidienne et vertébrale. Applications chirurgicales.

Ultrasonographic cerebral tomosphygmography (U.C.T.S.) is a method for determining cerebral pulsatility by centimeter-thick sections. The prototype uses a 2 MHz ultrasound wave emitted in the direction of cerebral structures. The reflected wave is captured by centimeter-thick sections, the mean determined and a pulsatile index defined. This index varies according to the regions studied (cortico-subcortical, superficial sylvian, deep sylvian and vertebrobasilar). Physiologic pulsatility was determined from a series of 150 healthy subjects. This technique was used to evaluate effects of carotid and vertebral circulation revascularization in 21 patients. Findings showed lack of relation between anatomic lesion and cerebral pulsatility, absence of significant modification of the index after release of carotid occlusion except when postoperative thrombosis occurred, and heterogeneous individual behavior after revascularization distinguishing subgroups of indifferent (40%), improved (27%), hyperpulsatile (27%) and worsened (13%) subjects. Two patients presented worsening on the contralateral side compatible with a steal syndrome. Results suggest that U.C.T.S. is a promising method of preoperative investigation and postoperative surveillance of carotid artery stenosis.

[Septicemia on endocavitary sounds for cardiac stimulation]. / Septicémies sur sondes endocavitaires de stimulation cardiaque.

Two cases of septicaemia on endocavitary cardiac pacemaker probes lead the authors to discuss the aetiological and therapeutic aspects of this type of infection. In both cases, the pacemaker had been in situ for a long time and had been replaced on several occasions, there was infection in the site of implantation and an attempt at removal of all of the pacemaker material failed, leaving a probe incarcerated in the right ventricle by one of its extremities with the other extremity floating free. The endocarditis associated with this septicaemia can, theoretically, be due to two mechanisms: metastatic implantation of infection form a distant site which must be detected or infection developing in contact with the pacemaker and propagated along the probe. The treatment of this type of septicaemia consists of appropriate antibiotics, which are only rarely sufficient to sterilize the infection. In most cases, all of the pacemaker material must be removed, which is easily achieved in the majority of cases. After cleansing of the site, the pacemaker has been successfully replaced. Sometimes, the probe is buried in the myocardium: surgical removal by cardiotomy, with or without extracorporeal circulation, seems to be preferable to the techniques of continuous traction which carry certain risks (tricuspid and myocardial lesions). Persistent floating probes must be removed surgically.