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Biomedical subjects

W E Bell

Publications and source records attributed to W E Bell.

At least 19 recordsLinked to original sources

Bacterial meningitis in children. Selected aspects.

Accumulating clinical experience has gradually outlined the epidemiology of acute bacterial meningitis, including the epidemic and the sporadic forms, the customary clinical signs related to different age groups and causative organisms, and methods of rapid diagnosis by laboratory examinations. Effective treatment, which continues to evolve, emerged in the 1940s with the development of antibacterial antimicrobials, first with the sulfonamides and then with the penicillins. The literature relative to these aspects of the disease has been abundant in the past few years. This article is directed to a variety of topics that have direct bearing on the disorder but are less often addressed to those who deal with infants and children.

Acute Disease

Epidermal nevus syndrome: subgroup with neuronal migration defects.

Epidermal nevus syndrome is one of the sporadic congenital hamartoses in which neurologic abnormalities have been frequently reported. We report two cases with severe primary brain involvement, seizures, mental retardation, and facial hemihypertrophy. We emphasize the superiority of magnetic resonance imaging over other radiographic studies in outlining the primary central nervous system anomalies associated with this syndrome. Although attempts were made to distinguish between several variants of epidermal nevus syndrome, it is clear that these are one entity. Proteus syndrome, encephalocraniocutaneous lipomatosis, and epidermal nevus syndrome have several overlapping phenotypic features. We suggest that they represent a phenotypic continuum, which in turn suggests a common pathogenetic process. While the cause of these syndromes is unknown, observations point to a somatic mutation leading to variable patterns of mosaicism.

Abnormalities, Multiple

Etiology of lateral rectus palsy in infancy and childhood.

The etiologies of lateral rectus palsy in 132 infants and children seen over a period of 22 years in a university medical center were retrospectively reviewed and compared with similar reports in the literature. Unlike most reports, which lump children with adults, this study focuses on childhood etiologies of lateral rectus palsy. In contrast to the only two studies in the literature on childhood lateral rectus palsy, which reflect a purely ophthalmologic perspective, this study reflects experiences of pediatric neurology and pediatric neurosurgery, as well as ophthalmology. Most lateral rectus palsies were unilateral, almost equally distributed between right and left sides. Trauma, tumor, and congenital etiologies were the most prevalent. The relative frequency of each of these etiologies varies by service (neurology, neurosurgery, ophthalmology). In 10.6% of patients, etiology could not be determined. The majority of patients had an isolated lateral rectus palsy. Association of lateral rectus palsy with other cranial nerve palsies and/or long tract signs characterized trauma and tumor. The majority of tumors were primary and infratentorial. A small number of patients had benign recurrent lateral rectus palsy.

Abducens Nerve

Brain tumors occurring before 1 year of age: a retrospective reviews of 22 cases in an 11-year period (1977-1987).

Congenital brain tumors have been reported infrequently and their management remains ill defined. An 11-year review (1977-1987) of all children with brain tumors with the onset of symptoms before 1 year of age was completed. Twenty-two children with the following histological diagnoses were treated: astrocytoma (7 patients), primitive neuroectodermal tumor (6 patients), papilloma or carcinoma of the choroid plexus (3 patients), malignant teratoma (2 patients), dermoid tumor (2 patients), embryonal rhabdomyosarcoma (1 patient), and chloroma (1 patient). Fifteen tumors were supratentorial in location, and 7 were infratentorial. Initial symptoms were hydrocephalus (32%), focal neurological deficit (23%), asymptomatic increase in head circumference (18%), failure to thrive (14%), and seizures (4.5%). The goal of treatment was a radical excision when possible, with primary chemotherapy in the last 6 years of the review period. Radiation therapy was the adjunct to surgery in the initial 5-year period. All patients with papillomas of the choroid plexus and dermoid lesions underwent a total resection with no recurrence. All 7 astrocytomas were supratentorial, with 6 occurring in the diencephalon. Five of the seven patients with astrocytomas survived more than 5 years. The 6 primitive neuroectodermal tumors were located equally between the supra- and infratentorial spaces. Four of the 6 infants with these tumors received chemotherapy (2 received chemotherapy alone; 2 received chemotherapy and radiation therapy) and are tumor free 2 to 9 years later. A fifth child received radiation therapy alone early in the series and survived only 4 months. The family of the other child refused adjunctive treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Astrocytoma

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

Allescheria (Petriellidium) boydii brain abscess in a child with leukemia.

A 3-year-old boy with acute lymphoblastic leukemia developed a right frontal lobe abscess from which Allescheria (Petriellidium) boydii was cultured. The mycotic infection complicating his underlying disease appeared to be confined to the brain. Surgical drainage and therapy with amphotericin B were followed by eventual recovery with no neurologic deficits.

Amphotericin B