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Biomedical subjects

John B Bodensteiner

Publications and source records attributed to John B Bodensteiner.

At least 19 recordsLinked to original sources
PubMed2006

Ontogeny and physiology of the cavum septum pellucidum in premature infants.

We report the natural history of the closure of the cavum septum pellucidum in 47 premature infants. In this study, a cavum septum pellucidum was present in all patients at 25 to 26 weeks' postconceptual age, in keeping with previous reports. The data from this study suggest that premature delivery does not change the natural history of the normal closure of the cavum septum pellucidum in most infants by 36 to 40 weeks' postconceptual age. Although not statistically significant, there is a suggestion from these data that higher grades of intraventricular hemorrhage are more frequently associated with loss (early closure) of the cavum septum pellucidum. One particularly illustrative case with a grade 4 intraventricular hemorrhage and subsequent hydrocephalus suggests that increases in pressure and volume in the lateral ventricles can cause the laminae of the septum pellucidum to approximate and appear to fuse earlier than expected. However, the fact that the cavum septum pellucidum reappeared in this case after ventricular pressure was decreased (postventricular shunt) suggests that approximation is not the sole factor in definitive fusion of the laminae of the septum pellucidum.

Child Development↗
PubMed2006

Leukoencephalopathy with bilateral anterior temporal lobe cysts.

Recently, several reports describing patients with a nonprogressive clinical course, increased signal in the cerebral white matter, and cystic changes in the anterior temporal lobes on magnetic resonance imaging (MRI) have appeared. To date, 25 patients with this very distinctive condition have been described. We report four new cases of this newly recognized entity. All have been identified primarily because of the distinctive MRI features consisting of the very unusual anterior temporal lobe cystic changes. The clinical features are characterized by severe, disabling, but nonprogressive mental and motor retardation. Magnetic resonance spectroscopy has shown increased myo-inositol and decreased N-acetylaspartate in the cerebral white matter. This is a distinctive, probably genetic, condition with characteristic neuroimaging and clinical features. In the appropriate clinical situation, the neuroimaging features are diagnostic.

Adolescent↗
PubMed2006

Magnetic resonance imaging (MRI) findings in children surviving extremely premature delivery and extremely low birthweight with cerebral palsy.

To delineate the frequency, severity, and characteristics of the brain injury occurring in children surviving extremely premature birth, we reviewed brain magnetic resonance images (MRIs) of children with cerebral palsy whose birthweight was less than 1000 g and whose gestational age was less than 28 weeks. The patients were all enrolled in the state Children's Rehabilitative Services clinic, where cerebral palsy is an automatic qualifying condition. We tabulated the MRI findings with respect to the cerebellum, periventricular white matter, and corpus callosum. The inclusion criteria were met by 157 children; 94 had an MRI. The available scans were reviewed by the authors, and the findings were tabulated. Fifty scans were available for review. There were 4 totally normal scans, 18 scans had normal cerebellar imaging, and 8 scans were felt to have normal cerebral findings. The common cerebral abnormalities included decreased white-matter volume without gliosis (n = 36), periventricular leukomalacia (n = 16), and a thin corpus callosum (n = 18). Cerebellar abnormalities were found in 32. The cerebellar findings included destruction of major portions of the cerebellum (usually the inferior vermis and hemispheres) (n = 23) and focal or unilateral loss of cerebellar tissue (n = 4). The high incidence of injury to the cerebellum has not been previously appreciated. The most common cerebral injury is decreased volume of white matter in the periventricular regions without gliosis. The pattern of cerebellar injury suggests a vascular insult, and the deficient white matter without gliosis suggests immaturity of oligodendrogliocytes with limited response to injury. Both lesions are more or less unique to the age at which the insult occurred and represent an emerging, newly recognized type of cerebral palsy.

Arizona↗
PubMed2005

Neurologic complications associated with respiratory syncytial virus.

Encephalopathy has been demonstrated to be associated with respiratory syncytial virus bronchiolitis. In this study, the data on all patients less than 14 years of age hospitalized with respiratory syncytial virus bronchiolitis over the past 4 years was reviewed. Patients who had concomitant diagnoses consistent with neurologic disease underwent detailed chart review. There were 964 patients (age 0.1 to 13.6 years) with a diagnosis of respiratory syncytial virus bronchiolitis. Thirty-six of these patients had concurrent neurologic diagnoses. Twenty-four patients were excluded because of preexistent neurologic disorders, probable simple febrile seizures, or a history of epilepsy. Twelve respiratory syncytial virus-positive patients had definite neurologic complications without another recognized cause. Seven of these patients had seizures (predominantly generalized tonic-clonic and one with status epilepticus), three had generalized encephalopathy (marked hypotonia and decreased responsiveness) of whom two also developed esotropia. Two patients developed isolated esotropia. There was an incidence of neurologic complications of 1.2% (0.7% seizures) in a total of 964 patients with respiratory syncytial virus bronchiolitis. This percentage does not include presumed simple febrile seizures or exacerbations of preexisting seizure disorder (further 1.3%). Neurologic complications occur with respiratory syncytial virus bronchiolitis, and physicians and other caregivers should be aware of this entity as well as the favorable prognosis.

Acute Disease↗
PubMed2005

Frequency and nature of cerebellar injury in the extremely premature survivor with cerebral palsy.

We report on the frequency and variable nature of magnetic resonance imaging-documented injury to the cerebellum in children with cerebral palsy who had survived a birth with a weight under 1000 g and/or a gestational age under 28 weeks. Thirty of 67 patients who had magnetic resonance images were found to have injury to the cerebellum. Those with cerebellar injury were much more likely to be microcephalic and to be unable to walk or talk. They did not demonstrate a greater frequency of observed injury to the cerebrum. From a larger collection of children with known cerebellar injury and cerebral palsy who had a history of being extremely premature, we found that 35 of 47 patients had prominent injury to the inferior cerebellum, suggesting infarction, whereas the remainder demonstrated varying degrees of cerebellar atrophy with or without asymmetry and four also had enlarged 4th ventricles. Injury to the cerebellum in the extremely premature survivor who has cerebral palsy is common and associated with a more adverse clinical picture. The etiology of this injury is obscure.

Atrophy↗
PubMed2005

Cerebellar injury in the extremely premature infant: newly recognized but relatively common outcome.

Severe injury to the cerebellum as a complication of extreme prematurity with extremely low birthweight was recently described in 13 children with the clinical diagnosis of cerebral palsy. We report another 10 cases of this syndrome. The clinical features include striking motor impairment and variable degrees of ataxia and athetosis or dystonia, which represent a distinct clinical type of cerebral palsy. Most are severely damaged, with cognitive, language, and motor delays. All are microcephalic, except one with hydrocephalus. Neuroimaging studies demonstrate the absence of major portions of the cerebellum involving both the inferior vermis and hemispheres. Most also have injury of a less severe nature in the cerebrum. This report indicates that this is not an uncommon outcome of extremely low birthweight infants, and we hope to encourage further investigations into the relative frequency and likely etiologies of the condition.

Adolescent↗
PubMed2004

Cerebral palsy: defining the problem.

The term "cerebral palsy" has had varied meanings over the past century. This article presents the current consensus definition that best captures the core elements of this heterogeneous entity. Elements comprising the components of the consensus definition are elaborated upon to provide clarity. Areas of remaining lack of consensus, mostly reflecting the timing of diagnosis and the exclusion of various etiologic entities are highlighted.

Cerebral Palsy↗
PubMed2004

Treatment of refractory status epilepticus with hemispherectomy.

A 7-year-old boy with left hemiparesis secondary to right hemispheric cortical dysplasia was admitted to the hospital with increasing numbers of seizures. Magnetic resonance imaging showed a small dysplastic right hemisphere with abnormally thickened gyri and an apparently normal left hemisphere. Previous video-electroencephalogram (EEG) monitoring showed bilateral independent spikes and generalized slow spike-and-wave episodes on EEG and [18F]fluorodeoxyglucose (FDG) positron emission tomography scan demonstrated scattered areas of regional hypometabolism bilaterally; therefore hemispherectomy was not undertaken at that time. During this hospital stay, nonconvulsive status epilepticus developed and was refractory to multiple medical therapies including pentobarbital (PTB) coma. Burst-suppression pattern during PTB coma appeared to be generalized spike and wave, but when EEG was reviewed with increased time resolution spikes suggested a right hemisphere origin. The patient underwent bilateral intracarotid amobarbital spike-suppression test that showed only minimal suppression of epileptiform discharges with injection of the left carotid, but complete suppression of spike activity after right-sided carotid injection. A right hemispherectomy was performed with complete cessation of status epilepticus. Postoperative EEG showed no epileptiform discharges. Patient follow-up was limited to 12 months after surgery. The patient had regained the ability to walk unaided and was seizure free with a single antiepileptic medication. This case illustrates a potentially life-saving procedure for refractory status epilepticus and several techniques including a spike-suppression test to aid in prediction of cessation of seizures after hemispherectomy.

Amobarbital↗
PubMed2004

Agenesis of the corpus callosum is associated with feeding difficulties.

Our objective was to characterize the common occurrence of feeding and swallowing disorders noticed by our speech therapy department among patients with agenesis of the corpus callosum. All patients with suspected or presumed agenesis of the corpus callosum undergoing therapy for feeding and/or swallowing disorders, including oral and/or pharyngeal dysphagia and oral-sensory disorder, were identified. Their brain magnetic resonance imaging (MRI) studies and charts were reviewed in detail. Seven patients with striking oral-motor and oral-sensory disorder met the criteria for agenesis of the corpus callosum. Particular difficulties noted include oral-sensory defensiveness in five of these seven patients, oral dysphagia/weakness in all seven patients, and pharyngeal dysphagia with risk of aspiration in three of these seven patients. The oral-motor weakness observed in all of the patients affected the strength and coordination necessary for functional oral-phase swallowing. We conclude that feeding and swallowing disorders are significant in children with agenesis of the corpus callosum. Although the pathophysiologic mechanism is not known, recognition of this phenomenon is important because early detection and intervention can facilitate functional feeding and swallowing patterns earlier in these patients, therefore avoiding the adverse and more severe disorders that can arise when dysphagias and/or oral-sensory disorder persist over extended periods of time.

Agenesis of Corpus Callosum↗
PubMed2004

Naegleria fowleri hemorrhagic meningoencephalitis: report of two fatalities in children.

Two cases of hemorrhagic meningoencephalitis secondary to Naegleria fowleri infection confirmed by postmortem analysis are described. The first patient is a 5-year-old boy who presented with a severe headache, neck stiffness, and lethargy. His neurologic examination was significant for somnolence and nuchal rigidity. Cerebrospinal fluid studies and structural neuroimaging were consistent with hemorrhagic meningoencephalitis. Another 5-year-old boy presented to a different institution 2 miles away in the same week with similar complaints. Both patients declined rapidly and expired within 48 hours of admission secondary to transtentorial herniation caused by the mass effect of inflammation, edema, and hemorrhage with displacement of the brain stem. Histopathologic and immunochemistry analysis of brain tissue revealed the presence of Naegleria trophozoites in both cases.

Amebiasis↗
PubMed2003

Congenital myopathies/dystrophies.

The congenital myopathies and congenital muscular dystrophies are a group of relatively infrequent neuromuscular disorders. Ultimate understanding of these disorders, however, will undoubtedly shed considerable light on skeletal muscle development and function. Three classical congenital myopathies are central core disease, nemaline myopathy, and centronuclear myopathy. The congenital muscular dystrophies are often distinguished by whether or not they are associated with clinically evident cerebral involvement.

Child↗
PubMed2003

Hypotonia, congenital hearing loss, and hypoactive labyrinths.

The records of all patients attending a neurosensory genetics clinic over an 11-year period were reviewed. Of the 450 patients seen, 31 presented with sensorineural hearing loss, hypotonia, and delay in the acquisition of motor milestones. Of these, 4 children were found who did not have an etiologic diagnosis such as Down syndrome or cerebral palsy. Vestibular testing revealed hypoactive labyrinthine function in all 4 of the cases, and careful imaging of the temporal bone showed anomalous development of the cochlea, vestibule, and semicircular canals in 3 of the 4 cases. None of the patients had ataxia, tremor, or significant nystagmus. Over time, the hypotonia improved in all, and none were felt to have cognitive deficits. These cases demonstrate that hypoactive labyrinthine function may be associated with hypotonia that is severe enough to result in delayed acquisition of motor milestones. The patients followed the typical remitting course of "benign congenital hypotonia." The distinguishing clinical feature is the presence of moderate to profound sensorineural hearing loss in all of the patients.

Cochlea↗
PubMed2002

Episodic coma in a new leukodystrophy.

Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.

Aspartic Acid↗