Biomedical subjects
Publications and source records attributed to C L Truwit.
Rhombencephalosynapsis is an unusual disorder characterized predominantly by agenesis/hypogenesis of the cerebellar vermis and fusion of the cerebellar hemispheres. Three cases are reported with emphasis on the MRI and clinical findings. Discussion of the relative importance of the cerebellar anomalies and associated supratentorial abnormalities is included.
A combined MR suite and operating room (MR-OR) has been developed and extensively assessed for its use in a wide spectrum of therapeutic applications. Equipped with a 1.5 T short bore clinical MR scanner and standard neurosurgical OR equipment, in this MR surgical suite, surgeons can obtain intraoperative planar and volumetric MR images with superior soft tissue contrast and spatial resolution for surgical planning, guidance, and monitoring. Besides MR morphologic imaging capability, blood oxygen level-dependent functional MRI and proton MR spectroscopic imaging have been demonstrated intraoperatively in the same MR-OR to aid in surgical planning and guide tumor resections. A perspective surgical navigation device and remotely operated instrument have been developed and successfully used to assist surgeons in aligning and introducing biopsy needles under fluoroscopic MRI in brain biopsy procedures. Furthermore, surgical complications can be assessed immediately before the closure. There are numerous advantages offered by this unprecedented MR-guided surgical approach, most of which are demonstrated and presented herein. Since 1997, >270 neurosurgical cases (42% brain biopsies, 25% tumor resections, 11% functional neurosurgeries, 10% cyst drainages and shunt placements, and 12% others) have been performed in the MR-OR with a <1% overall complication rate. The tumor recurrence rate for the MR-guided surgical approach is significantly less than that of the conventional one. Exemplary neurosurgical cases that have been performed in the MR-OR suite within the last 24 months are included. Overall, this high magnetic field approach to the MR-guided minimally invasive surgical procedures has been shown to be practical and acceptable to neurosurgeons as well as to neuroradiologists for a wide range of neurosurgical and neuroradiologic applications.
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A 4-month-old boy with polydactyly and bifid epiglottis was found to have a large sellar and suprasellar mass. When the diagnosis of Pallister-Hall syndrome was made, conservative management was elected. When the patient was 2 years old, the tumor had grown proportionally with the patient, and he was developing appropriately. Although rare, this entity is important to recognize not only for clinical diagnosis but also for appropriate management and genetic counseling.
PURPOSE: We used MR to retrospectively analyze the brains of patients suffering from cerebral palsy, our aim being to determine MR's role in the assessment of brain damage and the relationship of pre-, peri-, and post-natal events to cerebral palsy. METHODS: Forty patients (aged 1 month to 41 years) underwent MR scanning and findings were correlated with clinical histories in all cases. RESULTS: Review of MR scans of 11 patients who had been born prematurely revealed findings of periventricular white matter damage, indicative of hypoxic-ischemic brain injury (82%), the chronology of which was difficult to determine. Among 29 patients who had been born at term, three major patterns emerged: (1), gyral anomalies, suggestive of polymicrogyria, consistent with mid-second trimester injury; (2), isolated periventricular leukomalacia reflecting late second- or early third-trimester injury; and (3), watershed cortical or deep gray nuclear damage, consistent with late third-trimester, perinatal or postnatal injury. In 16 (55%) of 29 patients born at term, MR findings of intrauterine brain damage were observed; in over half of these cases MR revealed developmental anomalies, which is nearly twice the rate reported in prior studies employing CT. CONCLUSIONS: Our results support a growing consensus that cerebral palsy in term infants is often the result of prenatal factors, and less commonly related to the perinatal period.
Neurotoxicity is a recognized complication of cyclosporin A (CsA) therapy in patients undergoing organ transplantation. It is most commonly manifested by fever, seizures, and altered mental status. Cortical blindness and speech and motor disturbances can also occur. Changes seen in cerebral white matter on imaging studies are nonenhancing areas of hypoattenuation on CT and T2 prolongation on MR. We report three cases of CsA-induced neurotoxicity in which reversible changes were observed in the cerebral white matter. In the first patient, CsA neurotoxicity occurred 1 week following orthotopic liver transplantation. In the second patient, CsA neurotoxicity coincided with an episode of severe systemic hypertension 4 weeks after cardiac transplantation. The third patient experienced seizures 1 month after heart/lung transplantation for cystic fibrosis. A current theory postulates a relationship between diminished serum cholesterol and CsA neurotoxicity. This theory, however, does not satisfactorily address all cases of CsA neurotoxicity. In particular, serum cholesterol measurements were normal in cases 2 and 3 and probably were normal in case 1, despite diminished cholesterol levels preoperatively. Although the matter of CsA-induced neurotoxicity remains unresolved, we suggest that endothelin, a newly described neuropeptide that causes intense vasoconstriction and that has been implicated in cerebral vasospasm, may potentiate CsA-induced damage to endothelium and promote CsA neurotoxicity.
We describe the clinical and MR findings in three cases of rhombencephalosynapsis, a rare congenital malformation of the posterior fossa consisting of vermian agenesis or severe hypogenesis, fusion of the cerebellar hemispheres, and apposition or fusion of the dentate nuclei. Associated anomalies include hydrocephalus, fusion of the inferior colliculi, deficiency or absence of the septum pellucidum, and hypoplasia of the anterior commissure. Fourteen previous cases of rhombencephalosynapsis have been reported including Obersteiner's first report in 1914. The clinical presentation is variable, ranging from early death to variable degrees of cerebellar dysfunction and developmental delay. Patients may reach young adulthood. We report three additional cases and provide radiographic (MR) images of this unusual anomaly detected during life. Diagnoses in three children with rhombencephalosynapsis were made on the basis of MR findings. To our knowledge, this is the first report of this disorder being diagnosed in living patients.
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MR scans of 25 patients who suffered asphyxia at known gestational ages were reviewed retrospectively. The gestational ages of the patients at the time of asphyxia ranged from 24 to 46 weeks. The MR pattern of brain damage in patients with prolonged partial asphyxia was seen to evolve in a predictable manner corresponding to the known maturation of the brain and its vascular supply. Patients at 24- and 26-weeks gestational age had irregularly enlarged ventricular trigones with minimal periventricular gliosis. Patients at 28-34 weeks had variably dilated ventricles with periventricular gliosis. The 36-week neonate had mild cortical and subcortical atrophy and gliosis superimposed on deep white matter and periventricular gliosis. Term neonates had significant cortical and subcortical gliosis and atrophy in the parasagittal watershed areas. Postterm neonates (44-46 weeks) showed cortical and subcortical watershed gliosis and atrophy with sparing of the immediate periventricular region. Two children suffered cardiocirculatory arrest; their scans revealed a different pattern of brain damage, demonstrating primarily brainstem, thalamic, and basal ganglia involvement. MR appears to be a powerful tool in the assessment of brain damage resulting from perinatal asphyxia that gives important clues to the time and nature of the asphyxia.
PURPOSE: We report the MR findings in nine patients with clinical and laboratory evidence of Kallmann syndrome (KS), a genetic disorder of olfactory and gonadal development. In patients with KS, cells that normally express luteinizing hormone-releasing hormone fail to migrate from the medial olfactory placode along the terminalis nerves into the forebrain. In addition, failed neuronal migration from the lateral olfactory placode along the olfactory fila to the forebrain results in aplasia or hypoplasia of the olfactory bulbs and tracts. Patients with KS, therefore, suffer both reproductive and olfactory dysfunction. METHODS: Nine patients with KS underwent direct coronal MR of their olfactory regions in order to assess the olfactory sulci, bulbs, and tracts. A 10th patient had MR findings of KS, although the diagnosis is not yet confirmed by laboratory tests. RESULTS: Abnormalities of the olfactory system were identified in all patients. In particular, the anterior portions of the olfactory sulci were uniformly hypoplastic. The olfactory bulbs and tracts appeared hypoplastic or aplastic in all patients in whom the bulb/tract region was satisfactorily imaged. In two (possibly three) patients, prominent soft tissue in the region of the bulbs suggests radiographic evidence of neurons that have been arrested before migration. CONCLUSIONS: Previous investigators of patients with KS used axial MR images to demonstrate hypoplasia of the olfactory sulci but offered no assessment of the olfactory bulbs. In the present study we used coronal images to show hypoplasia of both olfactory sulci and bulbs. In addition, we found what we believe to be the radiologic correlate of arrested neuronal migration in KS.