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Biomedical subjects

A Sevely

Publications and source records attributed to A Sevely.

At least 19 recordsLinked to original sources
PubMed2002

[Optic pathway gliomas in neurofibromatosis type I. Longitudinal study of 30 cases in two multidisciplinary practices].

UNLABELLED: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol. PATIENTS AND METHODS: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination. RESULTS: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis. CONCLUSION: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.

Adolescent↗
PubMed2002

[Superficial siderosis of the central nervous system: a case report].

Superficial siderosis of the central nervous system is secondary to chronic subarachnoid bleeding. However, the underlying etiology is not always detected. Superficial siderosis can be diagnosed at MRI by the presence of a hypointense rim along the brain surface on gradient echo T2W images. The authors report a case of superficial cerebral siderosis where the underlying etiology remained undiagnosed in spite of extensive neuroradiological work up.

Central Nervous System Diseases↗
PubMed2002

CTM brace effect on scoliotic intervertebral discs using MRI method.

MRI has been clinically only used for investigation of intervertebral disc disorders. In this study, MR images were used and a new 3D modelling of the intervertebral discs was proposed. MRI examination had been performed on fourteen girls presenting an idiopathic scoliosis and wearing a first CTM brace. Using an in-house image processing software and the pre-post processing software Patran, geometrical models were obtained with and without brace for each patient. These models included the outline of the intervertebral high intensity zone, composed of the nucleus and a part of the annulus. The shift forward between disc high intensity zone centres and body centres was found to be varying from 0 to 8mm. The sagittal and coronal shifts forward appeared in the curvature convexity and were maximum at the curvature apex. The intervertebral disc wedging was found to be varying from -10 degrees to +10 degrees. On these fourteen analysed patients, the CTM brace decreased the coronal shift forward between disc high intensity zone centres and body centres, and increased the sagittal intervertebral wedging. The intervertebral disc informations obtained represented new data in the scoliotic deformation description. But this method was not adapted for a clinical use. The qualitative and quantitative data obtained will help the orthopaedist in the brace design and also the clinician in the scoliosis comprehension.

Adolescent↗
PubMed2000

Pure sensorineural hearing loss and otosclerosis. An imaging case report.

Pure sensorineural hearing loss is not a rare finding in otological practice. Numerous aetiologies could be at the origin of such a deficit. However, otosclerosis is very rarely cited as a cause of pure sensorineural hearing loss. We present one such case of pure sensorineural hearing loss linked to otosclerosis in a 30-year old caucasian male and underline the high contribution of computed tomography to confirm such a diagnosis. Pure sensorineural hearing loss due to otosclerosis is a rare event and can be misdiagnosed. The clinical diagnosis of such a disease may be difficult. In these cases, CT-Scan is the exam of choice to confirm the diagnosis.

Adult↗
PubMed2000

Functional magnetic resonance imaging may avoid misdiagnosis of cochleovestibular nerve aplasia in congenital deafness.

OBJECTIVE: To investigate a narrow internal auditory canal (IAC) syndrome using functional magnetic resonance imaging (fMRI) of the auditory cortex. STUDY DESIGN: The study design was a case report. The follow-up period lasted 18 months. SETTING: The study was carried out in the audiology clinic of an ear, nose, and throat department and in the department of pediatric neuroradiology at a university hospital. MAIN OUTCOME MEASURES: Age-appropriate observational audiometry, objective audiovestibular tests, computed tomography (CT), magnetic resonance imaging (MRI), and (fMRI) of the auditory cortex were performed to analyze in detail the profound deafness of a young child. RESULTS: Audiovestibular examination demonstrated both measurable hearing and normal vestibulo-ocular reflex, and CT showed narrow IACs combined with normal labyrinths. Axial MR images completed by sagittal sections perpendicular to the IAC delineated a single nerve that was initially supposed to be the facial nerve. No cochleovestibular nerve was identified. However, fMRI performed with the patient under general anesthesia demonstrated activation of the primary auditory cortex during 1-kHz monaural stimulation on the left side. CONCLUSIONS: The absence of cochleovestibular nerve on MR studies cannot exclude connections between the inner ear and the central auditory pathways. This might be caused by a lack of spatial resolution of anatomical MR studies. The single nerve delineated within the IAC might also carry both facial and cochleovestibular fibers. Functional MRI can assess the cortical response to acoustic stimuli when aplasia of the cochleovestibular nerve is suspected. This case study illustrates a novel and atypical presentation of cochlear nerve dysplasia.

Acoustic Stimulation↗
PubMed1998

Diffusion- and magnetisation transfer-weighted MRI in childhood moya-moya.

MRI in two children with moya-moya demonstrated low signal on T2-weighted images in the acute and subacute phases of ischaemia. Gradient-echo sequences, more sensitive to magnetic susceptibility, demonstrated these abnormalities better. Signal loss, due to temporary accumulation of iron, decreases progressively and disappears in the chronic stage of the disease. Diffusion-weighted MRI allows early detection of ischaemic lesions and can be used to monitor progressive spreading of the lesions. Magnetisation transfer maps provide sharp contrast, useful for demonstrating cortical atrophy.

Acute Disease↗
PubMed1998

Spinal cord abscess in a heroin addict: case report.

Spinal cord abscesses are extremely rare, even in intravenous drug abusers. They usually have a poor prognosis unless diagnosed and treated promptly. MRI is the best imaging modality for diagnosis and follow-up. We report a 42-year-old man, an active intravenous drug user, HIV negative, who developed subacute tetraplegia from an intramedullary abscess caused by Staphylococcus aureus. Immediate decompressive surgery and antibiotic treatment led to progressive recovery.

Abscess↗
PubMed1998

[Acute orbital myositis and idiopathic inflammatory pseudotumor in children: three cases].

UNLABELLED: Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM). CASE REPORTS: A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma. CONCLUSION: The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.

Adolescent↗
PubMed1998

[Pituitary stalk transection syndrome].

BACKGROUND: Pituitary stalk transection is a non-negligible cause of growth hormone (GH) deficiency. POPULATION AND METHODS: We studied 22 children (13 boys, nine girls) aged at the first clinical manifestations from 2 days to 10 years (average = 5.33 +/- 2 years). Pituitary stalk transection was assessed by the means of magnetic resonance imaging (MRI). The children's past history showed fetal distress in 12 cases (54.5%), cranial trauma in three (13%) and a midline anomaly in three (13%). The first clinical manifestations were neonatal hypoglycemia (two cases), decreased growth velocity (18 cases) and diabetes insipidus (two cases). RESULTS: GH deficiency was complete, present from the onset in 19 of 22 cases and isolated in four. Fifteen of 22 cases had adreno-corticotrophic hormone (ACTH) and thyroid stimulating hormone (TSH) deficiency. Diabetes insipidus was present in six cases and revealed the syndrome in two. All children older than normal age of puberty (n = 10) had gonadotropin deficiency. In our study, these hormonal anomalies progressed from isolated GH deficiency to multiple hormonal deficiencies. CONCLUSION: The recently described stalk transection syndrome is relatively frequent and should be suspected after cranial trauma or fetal distress syndrome. The outcome is progressive evolution towards panhypopituitarism and these patients require regular clinical survey and hormonal controls.

Adrenocorticotropic Hormone↗
PubMed1997

[Pseudotumor forms of neurosarcoidosis in children. Diagnostic difficulties and therapeutic management].

In systemic sarcoidosis, neurologic manifestations are possible and occurred in 5 p. 100 of cases. The diagnosis is easier when sarcoiddosis is known, but in half case neurologic symptoms are inaugural. In these cases, involvement of other sites (liver, lung, eyes or skin) by the disease is needed to confirm sarcoidosis. Main manifestations in neurosarcoidosis are cranial nerve palsy, diabetes insipidus or chronic aseptic meningitis, but sometimes sarcoidosis can present as an intracranial mass. The diagnosis of neurosarcoidosis may be difficult and could be confused with infection (such tuberculosis or mycosis), with inflammatory diseases (such multiple sclerosis) or with neoplasm, particularly in case of oedema. Brain biopsy may be necessary in isolated neurosarcoidosis if no peripheral histology evidence of noncaseating granulomas has been obtained. Treatment with steroids alone is the best choice for initial therapy but must be prolonged. It is difficult to predict accurately the response to steroids and relapse may be possible after a long time. Other immunosuppressive therapeutics or radiotherapy have been used in patient with refractory neurosarcoidosis. Sequential magnetic resonance imaging is the useful mean for the follow-up of neurosarcoidosis.

Adolescent↗
PubMed1996

[Association of venous angioma and cavernoma of the posterior fossa].

BACKGROUND: Association of venous angioma to cavernous malformation is rare. If bleeding occurs, it must be assigned to the cavernous malformation. CASE REPORT: A 4 year-old boy suffered from an acute ataxia. Investigation showed a venous angioma and a cavernoma malformation in the posterior fossa. The patient was admitted again at the age of 8 years for a new episode of acute ataxia due to cerebellar hematoma. A conservative treatment was settled because of the risk of venous infarction. CONCLUSION: Venous angiomas are rarely symptomatic and are considered as normal venous variants. Indeed, the cavernomas are true malformations with a high bleeding potential. When both lesions are associated and bleeding occurs, only the cavernous malformation has to be removed if surgically accessible.

Cerebellar Neoplasms↗
PubMed1996

[Multiple cerebral telangiectasias. Apropos of an anatoma-radioclinical case].

UNLABELLED: Telangiectasias are vascular malformations corresponding to dilated capillary separated by nervous parenchyma. They are generally asymptomatic and discovered during autopsy. CASE REPORT: We describe the case of a 2-year-old girl who was admitted for drowsiness and vomitings with craniomegaly and right hemiparesis. The cerebral imaging demonstrated large amounts of calcium in the region of pons and left thalamus with a contrast enhancement throughout these calcifications. The thalamic lesion was cystic with obstruction of the third ventricle and hydrocephalus. There were multiple calcifications scattered throughout the basal ganglia and the white matter. The latter had a low density on CT and high signal intensity on MRI (T2). The cerebral angiographic study showed a blush in the pons and the thalamus. Clinical course was fluctuating or intermittently progressive. Finally, in spite of radiotherapy and oral corticosteroid treatment, the child became quadriparesic with oculomotor palsy. She suddenly died at age 4 years and 6 months from a hemorrhage of the pons. Neuropathological study showed two clusters of telangiectasias with calcifications in the pons and the thalamus. Other calcified vascular malformations were seen in the white matter and the basal ganglia. Near the lesions, astrocytar gliosis and edema were observed. The white matter was malacic with isolated calcifications. CONCLUSION: Cerebral telangiectasias may be multiple and may produce severe neurologic disorders, occurring in infancy with fluctuating clinical course. Arteriography may show these usually cryptic vascular malformations. This diagnostic should be kept in mind in view to the neuroradiological aspect we report here.

Basal Ganglia↗
PubMed1993

[Prenatal diagnosis of congenital cerebral tumors. Apropos of 3 cases].

BACKGROUND: The prognosis for congenital brain tumors is usually poor, so that their management during pregnancy is difficult. CASES REPORTS: Case 1. A large cystic mass was revealed by ultrasound at the 37th week of pregnancy; it was located near the brain stem and was associated with ventricular dilation. The newborn was delivered by cesarean section because of an abnormally enlarged head. The CT scan confirmed the presence of this mass containing a fluid that was found to be normal after needle aspiration. Surgical shunting of excess fluid was ineffective, and progressive deterioration prevented further exploration and/or treatment. The child is still living, confined to his bed, at the age of 5 years. Case 2. Ultrasonography at the 30th week of pregnancy showed a tumor located near the brain stem with dilation of the entire ventricular system. Post mortem examination after abortion revealed a capillary and cavernous hemangioma. Case 3. Ultrasonography at the 34th week of pregnancy showed dilation of the ventricular system. As a result, birth was induced. CT scan and MRI of the newborn showed a mixed, solid and liquid, mass in the posterior fossa. Post mortem examination showed a papilloma of the choroid plexus. CONCLUSION: These rare congenital tumors are usually revealed by ultrasonography showing ventricular dilation. Advances in imaging techniques, especially MRI of fetal brain, should help in the management of such tumors.

Brain Neoplasms↗
PubMed1991

Doppler ultrasound study of the anterior cerebral artery in neonates.

In neonates doppler ultrasound of the anterior cerebral artery (ACA) enables cerebral haemodynamics to be studied in a non-invasive manner at the patient's bedside. The signal of the pericallosal artery is recorded through the fontanelle with a 7.5 mHz real-time probe. Two parameters are monitored: Pourcelot's resistance index and mean velocity which reflects blood flow rate. Having established normal values according to the gestational and post-natal ages, the authors describe the changes in cerebral circulation observed in such pathological situations as intraventricular haemorrhage, apnoea, patent ductus arteriosus, hydrocephalus and post-asphyxia syndrome.

Apnea↗
PubMed1991

[Severe neurologic course of galactosemia. Default of myelisation caused by deficient synthesis of UDP-galactose?].

We describe the unfavourable neurological outcome of a galactosemic patient who however received an appropriate diet from the neonatal period. The magnetic resonance imaging showed significantly abnormal myelinization of the brain. The biochemical hypotheses at the origin of this abnormal cerebral myelinization are discussed in our patient. The 1-14C incorporation in the TCA precipitable glycoproteins reached 21% of the average value observed in 6 controls; it ranged from 37% to 59% for galactosemic patients without neurological disturbances. These results, which have to be confirmed, suggest that some galactosemic patients have a very low UDP-galactose synthesis.

Galactosemias↗
PubMed1989

[Predictive value of skull hypodensities in x-ray computed tomography in perinatal asphyxia in full-term newborn infants].

Prediction of risk of sequelae in 78 asphyxiated full-term newborns was evaluated during the 15 first days of life by clinical, electroencephalographic and cranial tomodensitometric (CT) data. The predictive value of CT hypodensity is poor (sensitivity: 87.7%; specificity: 63%), while its negative predictive value appears more interesting (97%). Combined evaluation of clinical, electroencephalographic and CT data allows the most accurate prognosis. These findings agree with other studies in the literature.

Asphyxia Neonatorum↗
PubMed1982

[Haemorrhages of the posterior fossa in full-term newborns (author's transl)].

Four cases of non-traumatic haemorrhage of the posterior fossa in full-term newborns are reported and compared with the 30 cases already published. Computerized tomography is the only method that permits both a diagnostic and a therapeutic approach. Surgery is indicated in the presence of brain stem compression but should be carefully discussed in cases of intracerebellar haemorrhage with secondary extension to the posterior fossa. Early hydrocephalus is common and requires shunting in about 25% of the cases

Cerebellar Diseases↗