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Biomedical subjects

Philippe Maeder

Publications and source records attributed to Philippe Maeder.

At least 19 recordsLinked to original sources

Trilateral retinoblastoma with suprasellar tumor and associated pineal cyst.

Trilateral retinoblastoma (TRb) is a well-known syndrome associating hereditary retinoblastoma (Rb) with an intracranial neuroblastic tumor arising usually in the pineal region, rarely at the suprasellar or parasellar site. It develops in most cases after diagnosis of Rb. The outcome is usually fatal because of secondary spinal dissemination. Pineal cysts have recently been reported as a benign variant of TRb. We report the unusual presentation of a TRb in a 12-month-old boy with extensive bilateral Rb, a voluminous suprasellar tumor, pineal cyst, and leptomeningeal disease. The special features of this "quadrilateral" Rb are discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Interhemispheric integration at different spatial scales: the evidence from EEG coherence and FMRI.

The early visual system processes different spatial frequencies (SFs) separately. To examine where in the brain the scale-specific information is integrated, we mapped the neural assemblies engaged in interhemispheric coupling with electroencephalographic (EEG) coherence and blood-oxygen-level dependent (BOLD) signal. During similar EEG and functional magnetic resonance imaging (fMRI) experiments, our subjects viewed centrally presented bilateral gratings of different SF (0.25-8.0 cpd), which either obeyed Gestalt grouping rules (iso-oriented, IG) or violated them (orthogonally oriented, OG). The IG stimuli (0.5-4.0 cpd) synchronized EEG at discrete beta frequencies (beta1, beta2) and increased BOLD (0.5 and 2.0 cpd tested) in ventral (around collateral sulcus) and dorsal (parieto-occipital fissure) regions compared with OG. At both SF, the beta1 coherence correlated with the ventral activations, whereas the beta2 coherence correlated with the dorsal ones. Thus distributed neural substrates mediated interhemispheric integration at single SF. The relative impact of the ventral versus dorsal networks was modulated by the SF of the stimulus.

Adult↗

Spinal cord ischemia: clinical and imaging patterns, pathogenesis, and outcomes in 27 patients.

BACKGROUND: The natural history and pathogenesis of ischemic spinal cord infarction remain largely unknown because most clinical studies have included mostly patients with ischemic lesions associated with aortic surgery or prolonged arterial hypotension. OBJECTIVE: To assess the pathogenetic mechanisms and outcomes of these cord infarctions based on clinical findings and spinal vascular anatomy. DESIGN: Retrospective review. PATIENTS: We analyzed the clinical, laboratory, imaging, and outcome data for 27 patients with acute spinal cord infarction admitted between 1990 and 2003. There were 11 men and 16 women (age range, 19-80 years [mean age, 56 years]). RESULTS: Ten patients had anterior spinal artery patterns, 4 each had anterior and posterior unilateral patterns, 3 had central patterns, and 2 each had posterior spinal artery patterns, transverse syndromes, and unclassifiable clinical pictures. Twenty patients had no identifiable etiology. Patients with a central or transverse infarct showed a high frequency of peripheral vascular disease, and all transverse infarcts occurred following prolonged arterial hypotension. The onset of all other infarcts was associated with mechanical triggering movements (P = .02), and these patients frequently had diseases of the spine (P = .003) at the level of the spinal lesion, with the clinical data suggesting root involvement at the level of the spinal cord lesion and pointing to mechanical injury of a radicular artery. The outcomes were favorable, with only 13 patients showing significant gait impairment on leaving the hospital. CONCLUSIONS: There are 2 main types of spinal cord ischemia: (1) radicular artery territory infarct (bilateral anterior or posterior spinal artery infarcts and unilateral infarcts) and (2) extensive spinal cord hypoperfusion (central and transverse infarcts). Each type has characteristic clinical, imaging, pathogenetic, and prognostic features.

Adult↗

Hand preference and sex shape the architecture of language networks.

In right-handed subjects, language processing relies predominantly on left hemisphere networks, more so in men than in women, and in right- versus left-handers. Using DT-MRI tractography, we have shown that right-handed men are massively interconnected between the left-hemisphere language areas, whereas the homologous in the right hemisphere are sparse; interhemispheric connections between the language areas and their contralateral homologues are relatively strong. Women and left-handed men have equally strong intrahemispheric connections in both hemispheres, but women have a higher density of interhemispheric connections.

Adult↗

Benign pineal cysts in children with bilateral retinoblastoma: a new variant of trilateral retinoblastoma?

PURPOSE: Patients with hereditary retinoblastoma (Rb) develop in 4%-8% a malignant midline tumor called trilateral Rb (TRb). We report in this study on benign pineal cysts observed in patients investigated for TRb. PATIENTS AND METHODS: Between September 1990 and December 2001, 172 patients were screened for TRb. Ninty-five had bilateral, 77 unilateral disease. The median age at diagnosis of Rb was 7 months (range 1-26). Treatment included enucleation, local treatment with cryotherapy or photocoagulation, first-line chemotherapy (CT), thermo-chemotherapy (TCT), Ruthenium plaque, and, rarely, external beam radiation (EBR). RESULTS: TRb was found in 5/95 patients (5.3%) with bilateral disease. Interestingly, five other patients (5.3%) presented a pineal cyst on magnetic resonance imaging (MRI). No cysts were recorded in the 77 patients with unilateral disease. This difference was statistically significant (P < 0.05). The median age at diagnosis of the pineal cyst was 26 months (range 16-80), much younger than reported in literature for healthy children. Four of five patients with TRb died of the disease, while all the patients with pineal cysts remained stable and asymptomatic during a median follow-up of 41 months (range 37-54). CONCLUSIONS: This report describes benign cystic lesions of the pineal gland in patients with hereditary Rb, suggesting a benign variant of TRb. Underlying possible pathogenetic mechanisms are discussed.

Brain Neoplasms↗

A cross validation study of deep brain stimulation targeting: from experts to atlas-based, segmentation-based and automatic registration algorithms.

Validation of image registration algorithms is a difficult task and open-ended problem, usually application-dependent. In this paper, we focus on deep brain stimulation (DBS) targeting for the treatment of movement disorders like Parkinson's disease and essential tremor. DBS involves implantation of an electrode deep inside the brain to electrically stimulate specific areas shutting down the disease's symptoms. The subthalamic nucleus (STN) has turned out to be the optimal target for this kind of surgery. Unfortunately, the STN is in general not clearly distinguishable in common medical imaging modalities. Usual techniques to infer its location are the use of anatomical atlases and visible surrounding landmarks. Surgeons have to adjust the electrode intraoperatively using electrophysiological recordings and macrostimulation tests. We constructed a ground truth derived from specific patients whose STNs are clearly visible on magnetic resonance (MR) T2-weighted images. A patient is chosen as atlas both for the right and left sides. Then, by registering each patient with the atlas using different methods, several estimations of the STN location are obtained. Two studies are driven using our proposed validation scheme. First, a comparison between different atlas-based and nonrigid registration algorithms with a evaluation of their performance and usability to locate the STN automatically. Second, a study of which visible surrounding structures influence the STN location. The two studies are cross validated between them and against expert's variability. Using this scheme, we evaluated the expert's ability against the estimation error provided by the tested algorithms and we demonstrated that automatic STN targeting is possible and as accurate as the expert-driven techniques currently used. We also show which structures have to be taken into account to accurately estimate the STN location.

Algorithms↗

Understanding diffusion MR imaging techniques: from scalar diffusion-weighted imaging to diffusion tensor imaging and beyond.

The complex structural organization of the white matter of the brain can be depicted in vivo in great detail with advanced diffusion magnetic resonance (MR) imaging schemes. Diffusion MR imaging techniques are increasingly varied, from the simplest and most commonly used technique-the mapping of apparent diffusion coefficient values-to the more complex, such as diffusion tensor imaging, q-ball imaging, diffusion spectrum imaging, and tractography. The type of structural information obtained differs according to the technique used. To fully understand how diffusion MR imaging works, it is helpful to be familiar with the physical principles of water diffusion in the brain and the conceptual basis of each imaging technique. Knowledge of the technique-specific requirements with regard to hardware and acquisition time, as well as the advantages, limitations, and potential interpretation pitfalls of each technique, is especially useful.

Body Water↗

Intraparenchymal migration of an intrathecal catheter three years after implantation.

Intrathecal drug administration using implanted catheter and pump systems has been used in routine clinical practice for more than 20 years to treat chronic refractory pain or spasticity. Complications associated with the use of these systems include drug related adverse events as well as technical problems, most of which are related either to the catheter or the procedure. Although traumatic neural damage is exceedingly rare it is usually associated with significant neurological impairment. We present a case of asymptomatic intraspinal migration of an intrathecal catheter three years after an uneventful implantation. To the best of our knowledge, this complication has never been reported before.

Catheterization↗

Imaging of a synchronous neuronal assembly in the human visual brain.

Perception, motion, and cognition involve the formation of cooperative neuronal assemblies distributed over the cerebral cortex. It remains to explore what characterizes the assemblies, their location, and the structural substrate of assembly formation. In this EEG/fMRI study, we describe the response of the visual areas of the two hemispheres in subjects who viewed bilateral iso-oriented (IG) or orthogonally-oriented (OG) moving gratings projected in the two hemifields. The IG stimulus synchronized activity across the hemispheres, as shown by an increased EEG coherence. The increase was restricted to the occipital electrodes and to the beta band. Compared with OG, IG increased the BOLD signal in a restricted territory corresponding to area VP/V4. Within this territory, a linear relation was found between the increased interhemispheric EEG coherence and BOLD. Thus, the increased BOLD localized a trans-hemispheric, synchronous neuronal assembly probably achieved by a callosal cortico-cortical connection. This assembly might reflect an early stage of perceptual grouping since the IG stimulus conforms to Gestalt psychology principles of collinearity and common fate.

Adult↗

Sporadic Creutzfeldt-Jakob disease: a comparison of pathological findings and diffusion weighted imaging.

To investigate a possible relationship between the severity of pathological and radiological lesions in diffusion-weighted MRI (DWI) we compared DWI findings from 6 sequential brain MRI scans with pathological features of numerous tissue blocks of different cortical and subcortical regions in a case of autopsy-proven sporadic CJD. Whereas DWI and pathological examination revealed multifocal, cortical and deep hyperintensities at corresponding localizations, no correlation between the degree of severity of radiologically visible and pathological damage was found. The characteristic focal involvement and extension of lesions of the cortex and the basal ganglia bilaterally shown by DWI may be an argument for the spreading of the disease per contiguitatem.

Cell Death↗

Cross validation of experts versus registration methods for target localization in deep brain stimulation.

In the last five years, Deep Brain Stimulation (DBS) has become the most popular and effective surgical technique for the treatent of Parkinson's disease (PD). The Subthalamic Nucleus (STN) is the usual target involved when applying DBS. Unfortunately, the STN is in general not visible in common medical imaging modalities. Therefore, atlas-based segmentation is commonly considered to locate it in the images. In this paper, we propose a scheme that allows both, to perform a comparison between different registration algorithms and to evaluate their ability to locate the STN automatically. Using this scheme we can evaluate the expert variability against the error of the algorithms and we demonstrate that automatic STN location is possible and as accurate as the methods currently used.

Deep Brain Stimulation↗

Acute brain perfusion disorders in children assessed by quantitative perfusion computed tomography in the emergency setting.

OBJECTIVE: Perfusion computed tomography (CT) is a simple imaging technique that allows accurate quantitative assessment of brain perfusion. Perfusion CT is an ideal imaging technique to be used in the emergency setting and has thus gained recognition in the early management of adult acute stroke patients. Perfusion CT can be applied to children successfully by using adequate imaging protocols. The goal of this article is to provide a pictorial essay of the perfusion CT features of diseases that affect brain perfusion as depicted in a population of children who were evaluated in the emergency CT unit of our institution. METHODS: During the period of September 2001 to October 2002, all the children, who were evaluated in the emergency CT unit of our institution and who were prescribed with a cerebral CT and an intravenous administration of iodinated contrast material, underwent a perfusion-CT examination. Perfusion-CT maps were reviewed in the patients diagnosed as abnormal on the basis of follow-up clinical/radiological examinations and correlated with the results of these tests. RESULTS: Brain perfusion-CT examinations have been performed in 77 children. Fifty-three patients were considered as normal, based on normal conventional cerebral CT and normal clinical/radiological follow-up. Perfusion-CT results showed major abnormalities in 14 cases among the 24 remaining patients, related to brain ischemia in 2, head trauma in 9, brain infection in 2, and sickle cell disease in 1. These abnormalities consisted in low regional cerebral blood flow and volume values, and in high mean transit time values. They demonstrated typical anatomical distribution, depending on the considered pathological condition. CONCLUSIONS: Perfusion CT provides quantitative assessment of child brain perfusion disorders. Its ability to be easily performed upon admission makes it an ideal emergency tool that advantageously competes with other imaging techniques such as perfusion-weighted magnetic resonance imaging, despite its limited spatial coverage. Its usefulness with respect to the impact on treatment and outcome, however, remains to be established in further studies.

Adolescent↗

Spinal cord lesion after long-term intrathecal clonidine and bupivacaine treatment for the management of intractable pain.

Long-term intrathecal drug administration using implanted pumps is increasingly used in the treatment of chronic refractory pain [Anderson and Burchiel 1999, Neurosurgery 44 (1999) 289; Krames 2002, Best Pract Res Clin Anaesthesiol 16 (2002) 619; Wallace 2002, Neurology 59 (2002) S18]. Extensive clinical experience over the last 15 years suggests that in selected cases the technique is safe, although infections, system malfunction and drug-related complications have been reported. In most cases, drug-related spinal cord injuries have resulted from the compression of a spinal inflammatory mass or abcess rather than from a direct neurotoxic effect. We report on a case of toxic spinal cord lesion occurring after more than 3 years of uneventful continuous infusion of a mixture of bupivacaine and clonidine.

Analgesia, Epidural↗

Relationship between brain perfusion computed tomography variables and cerebral perfusion pressure in severe head trauma patients.

OBJECTIVE: To compare brain perfusion-computed tomography (CT) results with invasive cerebral perfusion pressure (CPP) monitoring in severe head trauma patients. DESIGN: Prospective cohort study. SETTING: Emergency room and surgical intensive care unit of our hospital. PATIENTS: Sixty-one severe head trauma patients. INTERVENTIONS: We prospectively collected 103 perfusion-CT examinations with simultaneous measurement of mean arterial pressure and intracranial pressure, affording calculation of CPP. The statistical relationship between perfusion-CT results and the corresponding CPP values was evaluated using Wilcoxon (Mann-Whitney) and generalized F-tests. The functional outcome of the 61 patients was evaluated 3 months after trauma on the basis of the Glasgow Outcome Scale score and compared between groups using Fisher's exact tests. MEASUREMENTS AND MAIN RESULTS: Perfusion-CT enabled us to distinguish between two groups of patients. Within each group, a significant correlation (p <.001) between the CPP values and the corresponding perfusion-CT results was demonstrated. There was also a significant correlation (p <.001) between the CPP values and the extent of the abnormal perfusion-CT areas (R up to.817). The first group was characterized by a weak dependence of perfusion-CT results on the corresponding CPP values (low slope) and the second group by a strong dependence (steep slope). These groups were interpreted as having preserved (or pseudo) and impaired cerebral vascular autoregulation, respectively. The functional outcome was better in the second group of patients. CONCLUSIONS: Intermittent perfusion-CT measurements plus continuous CPP measurement provide more information than continuous CPP alone. Perfusion-CT gives unique information regarding regional heterogeneity of brain perfusion. It might allow clinicians to distinguish between patients with preserved auto-regulation (or pseudoautoregulation) and those with impaired autoregulation and could therefore guide interpretation of CPP measurements and therapy.

Adult↗

Admission perfusion CT: prognostic value in patients with severe head trauma.

PURPOSE: To assess the prognostic value of admission perfusion computed tomography (CT) in patients with severe head trauma. MATERIALS AND METHODS: This prospective study included 130 patients with severe trauma, aged 19-86 years, admitted with a Glasgow Coma Scale score of 8 or less. They underwent perfusion CT as part of their admission CT survey. Clinical data, unenhanced cerebral CT findings, and perfusion CT scans were evaluated with respect to the Glasgow Outcome Scale (GOS) score at 3 months. Perfusion CT features were evaluated in patients with intracranial hypertension, cerebral contusions, and juxtadural hematomas. Ordered logistic regression was used to determine risk factors for an unfavorable GOS score at 3 months. RESULTS: Perfusion CT was more sensitive than conventional unenhanced CT in the detection of cerebral contusions. Perfusion CT featured specific patterns with respect to patient outcome, with normal brain perfusion or hyperemia in patients with favorable outcome, and oligemia in patients with unfavorable outcome. The number of arterial territories with low regional cerebral blood volume at perfusion CT was an independent prognostic factor (P =.008), as were mean arterial pressure at the scene of accident (P =.083), base excess at admission (P =.002), presence of skull fractures (P =.041), and signs of herniation (P =.013) at admission unenhanced cerebral CT. Perfusion CT also showed a range of brain perfusion alterations in patients with juxtadural collections, cerebral edema, or intracranial hypertension. CONCLUSION: Perfusion CT in patients with severe head trauma provides independent prognostic information regarding functional outcome.

Adult↗

Brain perfusion in children: evolution with age assessed by quantitative perfusion computed tomography.

OBJECTIVE: The objective of this study was to assess the age-related variations of brain perfusion through quantitative cerebral perfusion computed tomography (CT) results in children without brain abnormality. METHODS: Brain perfusion CT examinations were performed in 77 children, aged 7 days to 18 years. These patients were admitted at our institution for both noncontrast and contrast-enhanced cerebral CT. Only children whose conventional cerebral CT and clinical/radiologic follow-up, including additional investigations, were normal were taken into account for this study (53 of 77). RESULTS: The average regional rCBF amounts to 40 (mL/100 g per minute) for the first 6 months of life, peaks at approximately 130 (mL/100 g per minute) at approximately 2 to 4 years of age, and finally stabilizes at approximately 50 (mL/100 g per minute) at approximately 7 to 8 years of age, with a small increase of rCBF values at approximately 12 years of age. The rCBF in the gray matter averages 3 times that in the white matter, except for the first 6 months of life. The global CBF represents 10% to 20% of the global cardiac output for the first 6 months of life, peaks at approximately 55% by 2 to 4 years of age, and finally stabilizes at approximately 15% by 7 to 8 years of age. Specific age-related evolution patterns were identified in the different anatomic areas of the cerebral parenchyma, which could be related to the development of neuroanatomic structures and to the emergence of corresponding cognitive functions. CONCLUSIONS: Quantitative perfusion CT characterization of brain perfusion shows specific age variations. Brain perfusion of each cortical area evolves according to a specific time course, in close correlation with the psychomotor development.

Adolescent↗

Sound recognition and localization in man: specialized cortical networks and effects of acute circumscribed lesions.

Functional imaging studies have shown that information relevant to sound recognition and sound localization are processed in anatomically distinct cortical networks. We have investigated the functional organization of these specialized networks by evaluating acute effects of circumscribed hemispheric lesions. Thirty patients with a primary unilateral hemispheric lesion, 15 with right-hemispheric damage (RHD) and 15 with left-hemispheric damage (LHD), were evaluated for their capacity to recognise environmental sounds, to localize sounds in space and to perceive sound motion. One patient with RHD and 2 with LHD had a selective deficit in sound recognition; 3 with RHD a selective deficit in sound localization; 2 with LHD a selective deficit in sound motion perception; 4 with RHD and 3 with LHD a combined deficit of sound localization and motion perception; 2 with RHD and 1 with LHD a combined deficit of sound recognition and motion perception; and 1 with LHD a combined deficit of sound recognition, localization and motion perception. Five patients with RHD and 6 with LHD had normal performance in all three domains. Deficient performance in sound recognition, sound localization and/or sound motion perception was always associated with a lesion that involved the shared auditory structures and the specialized What and/or Where networks, while normal performance was associated with lesions within or outside these territories. Thus, damage to regions known to be involved in auditory processing in normal subjects is necessary, but not sufficient for a deficit to occur. Lesions of a specialized network was not always associated with the corresponding deficit. Conversely, specific deficits tended not be associated predominantly with lesions of the corresponding network; e.g. deficits in auditory spatial tasks were observed in patients whose lesions involved to a larger extent the shared auditory structures and the specialized What network than the specialized Where network, and deficits in sound recognition in patients whose lesions involved mostly the shared auditory structures and to a varying degree the specialized What network. The human auditory cortex consists of functionally defined auditory areas, whose intrinsic organization is currently not understood. In particular, areas involved in the What and Where pathways can be conceived as: (1) specialized regions, in which lesions cause dysfunction limited to the damaged part; observed deficits should be then related to the specialization of the damaged region and their magnitude to the extent of the damage; or (2) specialized networks, in which lesions cause dysfunction that may spread over the two specialized networks; observed deficits may then not be related to the damaged region and their magnitude not proportional to the extent of the damage. Our results support strongly the network hypothesis.

Acute Disease↗