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C Raybaud

Publications and source records attributed to C Raybaud.

At least 19 recordsLinked to original sources
PubMed2002

[Central alveolar hypoventilation syndrome and cerebral venous thrombosis: fortuitous association?].

UNLABELLED: Central hypoventilation syndrome is defined as the failure of automatic control of breathing. Secondary central hypoventilation syndrome should distinguish from congenital central hypoventilation syndrome by brainstem abnormalities, place of respiratory control. CASE REPORTS: We report two clinical cases characterized by late onset central hypoventilation syndrome (three years--six months, and five years old): in the first case the diagnosis was made after general anesthesia and the second one presented with acute nocturnal comatose state. Neuroradiologic investigations showed bilateral cerebral sinus veinous thrombosis without any brainstem lesions. Moreover these children had severe behavior disorders: psychomotor instability, alterations of social relations, language dysfunction, and neurocognitive deficit. This symptomatology seems independent from central hypoventilation syndrome and cerebral venous thrombosis. CONCLUSION: Late onset central hypoventilation syndrome may be associated with cerebral venous thrombosis. Ischemia of central chemoreceptors or integration of their informations could be one of mechanism.

Child, Preschool↗
PubMed2002

[MRI: an essential examen for the management of sacrococcygeal chondromas].

Chordomas are rare tumors, involving mainly the sacrococcygeal area and the clivus. Although a significant radiological semiology exists (osteolytic, lobulated, calcified tumor), the definite diagnosis is histological. Five cases of sacral and/or coccygeal chordomas were studied by CT scan and MRI. Clinical, radiological (tumoral topography and extension) and histological data were noted in all cases. At CT scan, the tumor was median, osteolytic, often calcified, of intermediate density, sparing iliac bones. MRI showed, in all the cases, a tumoral lobulation making easier the diagnosis of chordoma. Futhermore, as compared to CT scan MRI brought new information for the study of the tumoral extension, revealing a frequent involvement of adjacent muscles and spinal canal. It also showed that the tumor most often spared the rectal wall. These data are fundamental in the management of patients: the therapeutical aim must be, if possible, a complete removal of the tumor. Thus, besides its interest in the diagnosis, MRI appears to be essential in the pre-therapeutical assessment of sacro-coccygeal chordomas.

Aged↗
PubMed2002

[The presurgical evaluation of epilepsies].

In this article, we present an overview of the principles, practices and procedures of the presurgical evaluation of the epilepsies in use in our center and in the majority of French teams. Surgery for epilepsy is offered to patients presenting with severe epilepsy with partial seizures. Its aim is to stop the seizures, or to significantly reduce their frequency. To do that, the epileptogenic zone should theoretically be removed and/or the propagation pathways of the seizures should be cut. Discussion of these indications inevitably includes prior assessment of the functional sequels (sensory, motor, cognitive or behavioral) which surgery is liable cause. The presurgical evaluation involves a multidisciplinary approach involving epileptologists, neurophysiologists, neuroradiologists, neuropsychologists and neurosurgeons and is carried out in two phases. The phase I is based on non-invasive investigations, including functional and structural neuroimaging, neuropsychological assessment, source localization of interictal spike and video-EEG recordings of seizures. The phase II is often required and is aimed to precisely define the anatomical localization of the epileptogenic zone and the relationships with a structural lesion. This invasive phase is mainly based on stereoelectroencephalography (SEEG). Finally, the surgical procedure must be adapted according to the distribution and dynamics of the anatomical and functional abnormalities which individually define each case of epilepsy.

Age Factors↗
PubMed2002

[Craniopharyngioma in children: MRI study of 43 cases].

BACKGROUND AND PURPOSE: Craniopharyngiomas are intra-cranial tumors, relatively frequent in children, expanding in the pituitary stalk axis, from the third ventricle to the sphenoid body. Plain films and CT scan generally show a calcified lesion, deforming the sella turcica. MRI improves tumor description and topographic and structural analysis of the lesion. The aim of this study is to analyze the MRI aspect of craniopharyngiomas in a pediatric population and to correlate findings with surgical data. METHODS: MR and CT studies of 43 pediatric cases of histology-proven craniopharyngiomas were reviewed retrospectively. Tumor emergence, extensions and signal on different sequences were recorded. We searched for radio-surgical correlations. RESULTS: Craniopharyngiomas can be classified into two groups: intra-sellar tumors and third ventricle floor tumors (infundibulum and tuber cinereum). Preferential routes of extension are observed in each group correlated with consistency (cystic and/or solid). Surgical data confirmed these results. CONCLUSIONS: MRI is crucial for the pre-therapeutic evaluation of craniopharyngiomas allowing not only a detailed description of the tumor but also guiding therapeutic decisions. This series demonstrated that craniopharyngiomas exhibit two different types of localization and behavior. Embryonic development of the tumor explains the topographical differences.

Adolescent↗
PubMed2001

Neurocutaneous melanosis: radiological-pathological correlation.

We report the case of a young patient with neurocutaneous melanosis (NCM) who presented with temporary aphasia and right hemiparesis followed by progressive coma and death. To our knowledge, this is the first case of this disease examined by CT, MRI, angiography and in which an autopsy was performed to assert the diagnosis with histology. Besides, we discuss differential diagnoses and interest of MRI for early diagnosis.

Adult↗
PubMed2001

Schizencephaly: correlation between the lobar topography of the cleft(s) and absence of the septum pellucidum.

The bipolar defects observed in schizencephalies-clefts in the hemispheric mantle on the one hand, absent septum pellucidum on the other--without any anatomic or functional continuity suggest that there is some sort of common specific vulnerability of both structures. A study of the correlation between lobar location of the clefts and involvement of the septum pellucidum was undertaken, considering the hypothesis that the septum pellucidum is the portion of a "medial medullary velum" that corresponds to the frontal lobe, while the psalterium would correspond to the parieto-occipital lobe and the fimbria corresponds to the temporal lobe. This retrospective study of 16 cases of schizencephaly properly investigated by MR discloses a perfect correlation, all cases with absent septum pellucidum having clefts into the frontal lobe, all cases with present septum pellucidum having clefts in the parietal, temporal, and occipital lobes, and only the few instances (3 cases) of overlapping findings being characterized by clefts in the central area, where the distinction between posterior frontal and anterior parietal lobes is uncertain because of the cortical dysplasia related to the clefts. Partial defects of the septum also proved to correlate closely, topographically, with the location of the clefts. Therefore, the facts confirm a segmental organization of the mantle and septal defects, suggesting a developmental rather than a destructive mechanism, which could at least be related to a segmental pattern of selective vulnerability.

Brain Damage, Chronic↗
PubMed2001

[Psychological and behavioral disorders with good outcome in neurosarcoidosis].

INTRODUCTION: Neurological involvement is observed in 5% of cases of sarcoidosis and includes impairment of the central nervous system, the meninges, and the cranial and peripheral nerves. Besides neurological defects, cognitive impairment may be encountered ranging from isolated memory defect to dementia. EXEGESIS: We report a case of neurosarcoidosis occurring in a 40-year-old woman, a native of Reunion Island, with initial meningeal and hypophyseal involvement. Three years later, while treated with low dose prednisolone and methotrexate, she presented a paranoid state associated with cognitive impairment of frontal type and severe behavioral disturbances. After 2 years of high dose steroid treatment associated with hydroxychloroquine, her behavioral status improved, allowing social and familial reinsertion. CONCLUSION: In our observation, sarcoidosis was revealed through a central neurological impairment, with chronic meningitis, facial nerve palsy, and, finally, through psychiatric symptoms and severe behavioral disturbances. A slow favorable outcome was obtained using high dose methylprednisolone and hydroxychloroquine with total regression of behavioral disturbances but with persisting cognitive alteration.

Adult↗
PubMed2001

[Mayer-Rokitansky-Kuster-Hauser syndrome: associated pathologies].

UNLABELLED: MRKH syndrome (Mullerian structures agenesis in woman) is often associated with other anomalies: kidney and bone anomalies, deafness, ovarian tumors. Functional ovarian anomalies have not been previously reported. CASE REPORTS: Five new cases of MRKH syndrome are reported. They were associated with deafness in two cases, with kidney anomaly in three cases, vertebral anomalies in one case, and four suffered from obesity and ovarian dysfunction (ovarian polycystics syndrome, advanced puberty, androgenic excess). All of them had ovarian ectopy, which was responsible for false ultrasonographic diagnosis. CONCLUSION: An MRKH syndrome diagnosis implicates a search for other anomalies. Ovarian functional trouble should not let MRKH syndrome remain unrecognized.

Abnormalities, Multiple↗
PubMed2001

Fetal brain MR imaging.

In the past 10 years, improvements in MR imaging and faster imaging techniques have dramatically increased the use of in-utero fetal brain MR imaging. Challenging abnormalities now can be diagnosed prenatally through a careful analysis of morphology and signal changes. Using illustrations of normal brain development as a starting point, this article illustrates and discusses major brain malformations and specific morphologic changes, destructive lesions, and isolated ventriculomegalies, and the advantages and disadvantages of T1 and T2 sequences are provided.

Brain↗
PubMed2001

Neuroimaging of epilepsy in children.

Epilepsy is, above all, a pediatric disease. The electroclinical expression of epilepsy depends on several factors, including the presence of a subjacent brain anomaly and the age of the child. Genetic, congenital, and perinatal disorders account for most of the cases. MR imaging should identify any abnormality of the brain, in order to understand the condition and, in selected cases, to be able to treat the patient surgically.

Brain↗
PubMed2001

[Epileptic seizures, hemiplegia and hyperglycemia: late discovery of a localized cortical dysplasia].

A 56-year-old woman was hospitalized with a right hemiplegia and aphasia evoking a cerebral infarction. In fact the neurologic deficits were of post-ictal origin, secondary to a partial epilepsy which began a few weeks before, at the same time as a polyuria-polydipsia syndrome revealing diabetes mellitus. This case illustrates the possibility for a partial epilepsy to occur in relation with a nonketotic hyperglycemia. If in most of those cases there is no underlying cortical lesion, in some observations the hyperglycemia is associated with an infarction. In our case the MRI revealed another type of lesion: a cortical dysplasia in form of a unilateral micropolygyria with a perisylvian distribution centered around the insula. The discovery of a cortical dysplasia at such an age is very unusual.

Cerebral Cortex↗
PubMed2000

Structural brain lesions do not influence the prognosis of juvenile myoclonic epilepsy.

OBJECTIVES: In juvenile myoclonic epilepsy (JME) neuroimaging procedures are usually unnecessary but are often performed following the first generalized tonic-clonic seizure before a specific diagnosis has been reached. Our aim was to evaluate the influence of abnormal findings on the clinical presentation and prognosis of JME. MATERIAL AND METHODS: We retrospectively analyzed all medical records of JME patients first referred in two epilepsy centers (Marseilles, Nice) between 1981 and 1998. RESULTS: Among 170 consecutive unselected newly diagnosed JME patients, neuroimaging was performed in 82 cases (MRI: 22, CT: 75, including MRI+CT: 15). In 4 subjects, specific neurological abnormalities had justified neuroimaging: severe head trauma after the onset of JME (2, both with lesions related to the trauma), multiple sclerosis with onset after JME (1, with normal MRI before the onset of multiple sclerosis and abnormal, specific findings later), severe mental retardation (1, with diffuse MRI changes but a typical electro-clinical presentation and evolution). In the other 78 cases, 9 had abnormal findings: common arachnoid cyst (3), mild diffuse cerebral atrophy and ventricular enlargement (1), mild ventricular enlargement only (3), septum lucidum cavum (1), mildly increased T2 signal in the left temporal lobe (1). Abnormal neuroimaging did not influence therapeutic decisions or prognosis. CONCLUSIONS: These data confirm that neuroimaging procedures should not be routinely performed in patients with typical JME, unless additional neurological problems, that are unrelated to the diagnosis of epilepsy, occur.

Adolescent↗
PubMed2000

Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation.

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.

Adolescent↗
PubMed2000

Is it juvenile myoclonic epilepsy?

A 21-year old man with marked developmental delay was referred for the diagnosis of myoclonic jerks (MJ), which were sometimes responsible for sudden falls without loss of consciousness, that had begun 2 years before, and for a recent generalized tonic-clonic seizure preceded by a cluster of MJ. Physical examination revealed a small stature, bilateral pyramidal signs, severe mental retardation, and retinis pigmentosa. Etiological factors for this encephalopathy were not found (muscle and skin biopsies, karyotype and extensive blood chemistry). Waking interictal EEG showed a normal background activity and generalized poly-spike-and wave (PSW) discharges. Photic stimulation disclosed a marked photoparoxysmal response, sometimes associated with myoclonic jerks. Three spontaneous jerks accompanied by a burst of generalized PSW were recorded on awakening from a nap. The MRI disclosed wide ventricles, a thin corpus callosum, brainstem atrophy and a so-called "redundant gyration"; these changes were evocative of acquired perinatal damage. Juvenile myoclonic epilepsy (JME) was diagnosed and valproate was started resulting in complete control of seizures. During a 5-year follow-up, the patient has remained seizure-free and the EEG consistently normal. In our opinion, JME can be diagnosed in very uncommon settings, including patients with significant brain damage, as long as all the other criteria for the diagnosis are present.

Adult↗
PubMed1999

The pitfall of silent neurosarcoidosis.

Sarcoidosis in childhood is seldom reported. Most cases are observed in older children and preadolescents as bilateral pulmonary disease and eye lesions. Arthritic features are more likely to be observed in infants and children younger than 4 years of age who do not develop pulmonary disease. Neurosarcoidosis is exceptional in this age group and seldom suspected when the neurologic symptoms are present. The authors report a pediatric patient with systemic sarcoidosis who developed a severe but silent neurologic involvement. Numerous masslike lesions were discovered on systematic cranial magnetic resonance imaging. The authors recommend a complete screening of extrapulmonary manifestations in children with sarcoidosis. The proper management of patients with incidentally discovered neurosarcoidosis has yet to be established.

Brain Diseases↗
PubMed1999

Benign childhood epilepsy with centrotemporal spikes and hippocampal atrophy.

A boy without significant family or personal history had three consecutive nocturnal seizures at 1-month intervals at age 10 years, all simple focal seizures with motor and sensory symptoms, the last with secondary generalization. Waking and napping EEG showed focal sharp changes typical of benign epilepsy with centrotemporal spikes (BECTS). A magnetic resonance imaging documented a marked right hippocampal atrophy (HA). After valproic acid (VPA) therapy, there were no more seizures, and there were fewer EEG changes. An EEG performed in the younger, fully asymptomatic 8-year-old sister documented unilateral right focal sharp waves. This case shows that HA, as well as other central nervous system lesions, can be found fortuitously in patients with BECTS.

Atrophy↗
PubMed1999

Late-onset epilepsy associated with regional brain cortical dysplasia.

RATIONALE: Cortical dysplasia (CD) designates a diverse group of malformations resulting from one or more abnormalities in the development of the cerebral cortex. The clinical manifestations of CD are varied, probably depending on the type, location and extent of CD. Epilepsy is a potential late manifestation of any cortical malformation. To our knowledge, however, no study has focused specifically on late onset of epilepsy in patients with localized CD. MATERIAL AND METHODS: We studied patients with localized CD confirmed by MRI. Patients were divided into 2 groups according to age at onset of epilepsy. Group 1 included patients in whom the first seizure occurred up to the age of 12 (early-onset group) and group 2 included patients in whom the first seizure occurred after the age of 12 (late-onset group). The two groups were compared with regard to the type of CD, clinical findings and EEG findings. RESULTS: Thirty-three patients with various forms of CD were studied. Onset of epilepsy occurred in adolescence or adulthood in 9 cases (37%). In 6 of these (17% overall), the first seizure occurred in adulthood. CD were posterior bilateral pachygyria (1), unilateral polymicrogyria (3), focal dysplasia with subcortical gray matter heterotopia (1), perisylvian bilateral polymicrogyria (1), bioccipital polymicrogyria (1) and bilateral nodular periventricular gray matter heterotopia (2). The incidence of neurological signs was lower in the late-onset group. Mental retardation was moderate or absent, thus allowing a fairly normal lifestyle. All patients presented partial seizures with a lower incidence of drug resistance (p < 0.01). EEG demonstrated preservation of background activity and absence of diffuse or multifocal abnormalities. CONCLUSION: Onset of epilepsy with various forms of CD may be delayed until adolescence or adulthood. Prognosis of epilepsy is usually more favorable in these cases.

Adolescent↗