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J Motte

Publications and source records attributed to J Motte.

67 records · Page 4Linked to original sources
PubMed1985

[Fetal torsades de pointe and the long QT syndrome].

The authors report a case of pregnancy interrupted by caesarian section at 38 weeks' gestation for attacks of fetal ventricular tachycardia. The recording of a long QT interval at birth indicated a diagnosis of fetal torsades de pointe. These attacks recurred 2 hours after birth leading to cardiac arrest. The arrhythmia was immediately controlled by betablockers therapy. The diagnostic, epidemiological and physiopathological features of the long QT syndrome are reviewed. The links with maturation of the sympathetic nervous system are recalled. Finally, a diagnostic and therapeutic strategy is proposed, based on the results of fetal echocardiography.

Arrhythmias, Cardiac↗
PubMed1985

[Extradural hematoma in children under 1 year of age].

The authors report 3 cases of epidural hematoma in infants under one year of age. The trauma had been minimal with a long symptom-free interval preceding the first clinical symptoms: anemia and its consequences, pallor, bradycardia, and finally shock. CT scan revealed the hematoma. In one case, it showed an asymptomatic hematoma which posed a therapeutic problem.

Craniocerebral Trauma↗
PubMed1985

[Benign intracranial hypertension (17 cases) and cerebral venous thromboses (49 cases). Comparative study].

A series of 49 cases with cerebral venous thrombosis (CVT) and a series of 17 cases with benign intracranial hypertension (BIH) without CVT, both observed between 1973 and 1983, have been compared. Papilledema has been found in 100% of BIH and 28.5% of CVT, visual acuity loss in 52% of BIH and 6% of CVT, obnubilation in 0% of BIH and 65% of CVT. Cerebrospinal fluid, EEG, cerebral angiography and Scanner were normal in all cases of BIH, while in CVT the same investigations were abnormal in respectively 90%, 100%, 100% and 54% of cases. Prognosis was excellent in BIH with only one residual mild loss of visual acuity. Recurrences occurred in 18% of BIH. Among CVT 18% died, 23% developed neurological sequelae, 59% totally recovered. There was no recurrence. On the basis of this comparative study we emphasize the following points: 6% of CVT only follow a course of BIH from end to end, according to Johnston and Paterson's diagnostic criteria of BIH. These cases concern only thrombosis of lateral sinuses. Other cases of CVT rather deal with patients admitted in neurosurgical units on emergency. Diagnosis of CVT is a difficult one and prognosis is still serious in spite of the use of Heparin. 85% of BIH are irrelevant to CVT. BIH is a "quiet" intracranial hypertension without trouble of vigilance. Prognosis is really good if diagnosis is early made, in spite of possible multiple recurrences. Treatment consists of CSF subtraction, preferably through external lumbar drainage, and Acetazolamide prescription. Head injuries and hormonal imbalance in women are two common etiologies of BIH and CVT.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1981

[Choroid plexus papilloma (author's transl)].

The authors report a new case of choroid plexus papilloma. Hydrocephalus produced by this tumour has special features. Diagnosis is made by CAT scan. Despite surgery and the begnignity of such tumours, prognosis is generally poor. In the reported case two radiological pictures over a 5 year period did not any increasing of the size of the tumour.

Cerebral Ventricle Neoplasms↗
PubMedDate not supplied

Neurofibromatosis type one and West syndrome: a relatively benign association.

Fifteen patients with neurofibromatosis type one (NF1) and West syndrome (WS) were studied. The evolution of WS was clearly different from that seen in the other neurocutaneous syndromes, especially tuberous sclerosis. Steroids were efficacious and spasms disappeared. There was no relapse after discontinuation of steroids and antiepileptic drugs were successfully stopped several years later. Mental outcome was also very good. The association of both conditions does not seem to be a coincidence. WS with NF1 seems remarkably benign and resembles idiopathic WS more than symptomatic WS.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

[Cutaneous granulomatous lesions disclosing ataxia-telangiectasia].

INTRODUCTION: Idiopathic cutaneous granulomatous lesions are exceptionally described in the course of congenital immunodeficiency, including ataxia-telangiectasia. CASE REPORT: We describe a new case of a 28-month girl who presented granulomatous skin lesions revealing a previously unknown ataxia-telangiectasia in the absence of typical neurologic signs, telangiectasia and infectious complications. The clinical aspect showed infiltrated erythemato-squamous plaques and nodules predominating on the face and limbs. These lesions increased in number without remission. Histological examination revealed a nodular, lymphohistiocytic infiltration with granulomatous tendency in the deep dermis and the hypodermis. Before the onset of skin treatment, the child developed an Epstein-Barr-virus related lymphoproliferation. Immunoglobulins and oral corticosteroids associated with chemotherapy permitted the regression of the granulomatous lesions but not of the fatal spread of the lymphoproliferative syndrome. DISCUSSION: These rare cutaneous manifestations are important to know because they can be the initial sign of an immunodeficiency. Clinical and histological aspects are characteristic. They are eventually associated with visceral granulomatous lesions. Physiopathology remains hypothetical. An abnormal immune response to an undetermined antigenic stimulation could be suspected in this particular context. The question of a correlation between these lesions and a proliferative syndrome remains open.

Ataxia Telangiectasia↗