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Biomedical subjects

W E Bell

Publications and source records attributed to W E Bell.

At least 37 records · Page 2Linked to original sources

Computed tomography and magnetic resonance imaging of the brain in Hurler's disease.

Computed tomographic (CT) and magnetic resonance imaging (MRI) scans of the brain in five patients with Hurler's disease are described and compared to the few available reports in the literature. Computed tomographic scans revealed low attenuation areas in the centrum semiovale and peritrigonal white matter. Ventriculomegaly was not a prominent feature in our patients, compared to those previously reported. In two patients, CT were normal. The most prominent magnetic resonance imaging abnormalities were the presence of radially oriented cystic areas in the centrum semiovale, peritrigonal white matter, corpus callosum, and pericallosal region. Magnetic resonance imaging abnormalities were present in all patients, even when CT scans were normal. Abnormalities on CT and MRI scans tended to be more prevalent in the posterior regions. Magnetic resonance imaging proved to be a more reliable imaging method in Hurler's disease. T1-weighted images delineated the cystic areas more clearly, whereas T2-weighted images were more sensitive in detecting small white-matter abnormalities. Magnetic resonance imaging abnormalities correlated well with known neuropathologic alteration in this disease. It is suggested that the cystic areas seen on MRI correspond to perivascular lacunae seen in histopathologic material.

Brain↗

Aqueductal stenosis and neurofibromatosis: a rare association.

Three cases with rare association of neurofibromatosis, hydrocephalus, and aqueductal stenosis seen at the University of Iowa during the past 20 years are presented. The literature on nontumoral hydrocephalus and aqueductal stenosis in neurofibromatosis is reviewed. Possible explanations for the association of aqueductal stenosis and neurofibromatosis are discussed. It is concluded that aqueductal stenosis, though not common, should nevertheless be considered in the differential diagnosis of hydrocephalus in neurofibromatosis.

Adolescent↗

Cerebral hemiatrophy, hypoplasia of internal carotid artery, and intracranial aneurysm. A rare association occurring in an infant.

A 4-month-old child with a very rare association of primary (congenital) left cerebral hypoplasia, hypoplasia of the left internal carotid artery, and giant anterior communicating artery aneurysm was seen. The aneurysm was detected and treated before rupture. We briefly reviewed the literature on agenesis (hypoplasia) of the internal carotid artery, intracranial aneurysms in childhood, and primary cerebral hypoplasia. It is proposed that cerebral arteriography, cranial computed tomography, or magnetic resonance imaging be performed in all children with primary cerebral hemihypoplasia to establish an etiologic diagnosis and, more importantly, to detect and treat possible associated intracranial aneurysms before they become symptomatic.

Atrophy↗

Correspondence between MMPI PTSD measures and clinical diagnosis.

Correspondence of the Minnesota Multiphasic Personality Inventory (MMPI) posttraumatic stress disorder (PTSD) subscale and the clinical scale decision rules reported by Keane, Malloy, and Fairbank (1984) with clinical diagnoses of PTSD was measured on a sample of 595 veterans. The measures demonstrated good sensitivity and selectivity, but the false-positive rate was high. It is suggested the MMPI measures be used to rule out, but not to establish, the diagnosis of PTSD. The construct validity of the PTSD subscale was supported by the finding of a higher mean score in combat than noncombat veterans.

Combat Disorders↗

Hypomelanosis of Ito. Wood's light and magnetic resonance imaging as diagnostic measures.

Hypomelanosis of Ito is an uncommonly reported neurocutaneous syndrome with a peculiar pattern of swirling hypopigmentation. The skin markings in the lightly pigmented individual are not easily visualized in regular light but can be readily seen with the use of Wood's light. We describe a 7-month-old female infant with this disorder who presented with intractable convulsions. Computed tomography showed enlargement of the left cerebral hemisphere but was otherwise not definitive. Abnormalities of neuronal migration were demonstrated with magnetic resonance imaging that could not be shown by computed tomography.

Brain↗

Magnetic resonance imaging of the spine in children.

We reviewed the magnetic resonance imaging (MRI) scans of the spine of 42 children who had neurologic signs compatible with lesions of the spinal cord. Twenty-three of the children had abnormalities identified by MRI. The spectrum of abnormalities included posttraumatic lesions, tumor, tethered cord, and syringohydromyelia. Tethered cord with or without lipoma and syringohydromyelia were the most common findings, affecting six and four children, respectively. Thirteen children studied by MRI also underwent conventional metrizamide myelography and/or computed tomography. In 12 cases, the findings of MRI were comparable to those of myelography and computed tomography. These observations indicate that MRI effectively detects lesions of the spinal cord in children. Because MRI can be performed on an outpatient basis and avoids the risks of metrizamide myelography, we conclude that MRI should be considered to be the preferred screening technique for children with suspected spinal cord disorders.

Adolescent↗

MRI in children with postinfectious disseminated encephalomyelitis.

Acute disseminated encephalomyelitis, an inflammatory and demyelinating disorder of central nervous system white matter, typically occurs following childhood viral infections. Although CT may demonstrate abnormalities, many children have normal CT studies in spite of widespread neurologic abnormalities. We report a series of five patients with the typical clinical presentation of disseminated encephalomyelitis who were studied using magnetic resonance imaging (MRI). In each case the children presented with progressive subacute neurologic abnormalities including headache, diplopia, ataxia, hemiparesis, seizures, dysarthria, and/or coma. CT was nondiagnostic. MRI clearly demonstrated multifocal white matter lesions of the cerebrum, brainstem, and cerebellum which corresponded to clinical signs. The patients improved dramatically with corticosteroid therapy. MRI showed progressive resolution of multifocal lesions in conjunction with clinical improvement.

Acute Disease↗

Detection of heterotopic gray matter in children by magnetic resonance imaging.

Heterotopic gray matter results from abnormal brain development and is a recognized focus of seizures. It may be associated with mental retardation and/or severe malformations of the brain. Three patients with heterotopia of gray matter were identified by magnetic resonance imaging (MRI). CT failed to detect the heterotopic gray matter in each case. One child was referred for removal of a neoplasm based on CT studies until MRI demonstrated the developmental nature of his condition. One infant had severely dysplastic left cerebral hemisphere associated with heterotopic gray matter and the syndrome of Hypomelanosis of Ito. All three children suffered from seizures and/or mental retardation. MRI provided important information in the management of each case and appears to be the imaging method of choice in evaluating children with seizures or retardation for heterotopic gray matter in the brain.

Brain Neoplasms↗

CSF lactate levels in neonates. Effect of asphyxia, gestational age, and postnatal age.

During a six-month period, lactate measurements were performed on all samples of CSF obtained by lumbar puncture from infants of varying gestational ages between birth and 32 days of age. The normal levels of lactate in the CSF of newborns were demonstrated to be higher than those previously reported in older children or adults. A steady decrease in CSF lactate levels was demonstrated as postnatal age increased, so that after the first ten days of life, CSF lactate levels were within the range reported for older children and adults. We could not demonstrate that neonatal asphyxia or gestational age significantly altered these values.

Age Factors↗

Posterior fossa hemorrhage in the term neonate.

Posterior fossa hemorrhage (PFH) in the newborn term infant has been infrequently reported, and before computed tomographic (CT) scanning most were identified at postmortem examination. The origin and causes of PFH in the term neonate differ from those in adults. The main possible causes are: (a) tentorial and falx disruption with avulsion of bridging veins due to mechanical compression and distortion of the head during the birth process, resulting in posterior fossa subdural collections and vermis hematoma, and (b) occipital osteodiastasis associated with cerebellar hemisphere laceration, with intracerebellar hematoma and subdural collections. Three full-term neonates with PFH were operated on with good outcome. Both of the possible causes contributed to the hemorrhage. Review of the literature to date has led to the following conclusions: (a) PFH in the term infant is frequently associated with breech delivery, forceps instrumentation, and prolonged labor with cranial molding. (b) Massive PFH is associated with dural sinus laceration, rupture of the vein of Galen, or rupture of hematoma into the subdural space. Symptoms may be delayed up to 96 hours in cases where there is a small continuing hemorrhage. (c) CT scanning with reconstructions is essential to provide precise localization of the hemorrhage and thus avoid errors in interpretation. (d) Prompt removal of PFH improves survival and decreases sequelae. (e) Shunting procedures are necessary in 36% of the cases.

Adolescent↗

Communicating hydrocephalus secondary to venous complications following intraatrial baffle operation (mustard procedure) for d-transposition of the great arteries.

A 39-month-old male had macrocephaly and communicating hydrocephalus secondary to superior vena cava (SVC) obstruction which was a postoperative complication of an intraatrial baffle operation (Mustard procedure) performed at age 10 months. Computerized tomography revealed progressive hydrocephalus. Cardiac catheterization showed an SVC pressure of 24 mm Hg. IVC and systemic vein atrium pressures were 5 mm Hg. Cineangiograms showed huge azygos collaterals from SVC to IVC. The operation was revised using a Dacron baffle instead of the original pericardial baffle. Nine months postoperatively, the head circumference was 55.0 cm and there was no clinical evidence of SVC obstruction.

Child, Preschool↗

Treatment of bacterial infections of the central nervous system.

Antimicrobial agents for the treatment of bacterial meningitis and other pyogenic intracranial infections have now been in use for approximately 45 years. The last decade, however, has been a period of tremendous advances in knowledge of the pharmacokinetics and other characteristics of the available drugs. The identification of factors that affect their in vivo metabolism and excretion, the importance of drug interactions, and the development of methods for estimation of serum and cerebrospinal fluid levels have added both complexity and sophistication to the clinical utilization of the antimicrobial agents. The number of available antimicrobials has expanded greatly during recent years, although the penicillins, chloramphenicol, and the aminoglycosides remain the mainstay of antibiotic treatment in the majority of cases of bacterial infection of the central nervous system.

Adolescent↗

Treatment of fungal infections of the central nervous system.

The recent development of new antifungal antimicrobials that can be administered in combination with amphotericin B or as alternatives to it has expanded the dimensions of treatment for fungal infections of the central nervous system. These disorders have acquired increasing importance as patients with malignant and other illnesses associated with immunosuppression survive longer and as renal transplantation is more widely applied. Amphotericin B has remained the most effective therapeutic preparation for most types of neurological fungal disease, although important roles for 5-fluorocytosine, miconazole, and, more recently, ketoconazole are being recognized.

Amphotericin B↗

Arachnoid cysts in children.

Twenty-seven cases of intracranial arachnoid cysts in children aged 1 to 16 years have been studied with attention to diagnosis, pathologic findings, and surgical treatment. In nine patients, the cyst was located in the posterior fossa. In eight patients, there were other CNS or other organ system abnormalities. No specific symptom complex can be ascribed to arachnoid cysts in that the clinical manifestations are the result of their size and location, secondary complications, and remote effects. This is illustrated in the ten cases reported.

Adolescent↗