PubMed Health⌕ Search

Biomedical subjects

Robert A Zimmerman

Publications and source records attributed to Robert A Zimmerman.

At least 19 recordsLinked to original sources

Neuroimaging evaluation of cerebral palsy.

MRI can demonstrate and differentiate the various insults and anomalies that can be responsible for cerebral palsy. Recent advances have resulted in techniques and sequences that allow prompt detection of cytotoxic edema and evaluation of brain perfusion. MRI precisely demonstrates the various patterns of injury, distinguishing insults owing to profound asphyxia, partial prolonged asphyxia, and mixed partial prolonged and profound asphyxia. Infants and children can be studied with MRI, and ultrafast MRI permits evaluation of the fetal central nervous system. In the fetus, the cause of ventriculomegaly can be determined, such as cerebrospinal fluid flow obstruction, brain malformation, or brain destruction with or without hemorrhage. Results from fetal MRI have led to better understanding of many brain abnormalities.

Cerebral Palsy↗

3.0 T versus 1.5 T pediatric brain imaging.

This article represents a review of the authors' experience with two 3.0 T Siemens Trio Whole Body MR imaging units, with a cumulative experience of 12 months total imaging time on these scanners, over 1000 cases. The authors were able to identify and review numerous patients who had diagnostic studies both on 1.5 T and 3.0 T.

Adolescent↗

Fetal posterior fossa volume: assessment with MR imaging.

PURPOSE: To retrospectively determine the relationship between posterior fossa volume (PFV) and estimated gestational age (EGA) and/or femur length (FL) during pregnancy for the purpose of developing a normal growth curve. MATERIALS AND METHODS: Advance institutional review board approval was obtained for this HIPAA-compliant study, and the need for parent informed consent was waived. A cross-sectional retrospective study was performed to measure PFV on in vivo magnetic resonance (MR) images obtained in 76 fetuses of 18-36 weeks gestation who had a morphologically normal CNS. Because this was a retrospective series, MR imaging techniques varied slightly, but all fetuses underwent imaging at contiguous 3-5-mm intervals in at least two orthogonal planes, with repetition time msec/echo time msec, 5-12/62-95; number of signals acquired, one; flip angle, 150 degrees -180 degrees; and matrix, 128-192 x 256. Posterior fossa areas were manually traced on half-Fourier rapid acquisition with relaxation enhancement in utero fetal MR images by one observer. PFVs were then calculated by manually summing areas from the contiguous sections and multiplying the total area by the section thickness. An average PFV (APFV) across orthogonal planes was calculated for each fetus, and the relationship between APFV and EGA was mathematically modeled. Coronal, transverse, and sagittal views were compared with correlations and Bland-Altman plots. Two additional observers repeated the measurements for a small subset of fetuses (n = 5). Paired t test analyses were also performed to determine significant differences between sagittal, transverse, and coronal measurements, as well as to determine preliminary intraobserver and interobserver variability of measurements in a subset of cases. RESULTS: The relationship between APFV (in cubic centimeters) and EGA (in weeks) was well described by a single exponential function [APFV = 0.689 exp(EGA/9.10)]. APFV doubling time was 6.31 weeks. Root-mean-square variation of values around the model line was 1.63 cm(3). There was no statistically significant intra- or interobserver variation (P > .16 for all fetuses) at preliminary analysis. No correlation between APFV and FL could be found. CONCLUSION: The normal fetal PFV growth curve generated in this study may have potential as a model for clinical application.

Cranial Fossa, Posterior↗

Unusual diagnosis in a child suffering from juvenile Alexander disease: clinical and imaging report.

Alexander disease is a rare, sporadic leukoencephalopathy characterized by white-matter abnormalities with frontal predominance and, as a rule, clinically associated with megalencephaly, seizures, spasticity, and psychomotor deterioration. We describe a boy who was diagnosed as affected by anorexia nervosa because of his refusal to eat, progressive weight loss, and psychologic disturbances. The observation of a hyperintense lesion on T(2)-weighed magnetic resonance images (MRIs) was initially explained as a pontine and extrapontine myelinolysis related to malnutrition. Following MRI and DNA analysis, we diagnosed a juvenile type of Alexander disease. Therefore, we can affirm the importance of the history and clinical examination to look for brainstem dysfunction in patients presenting with atypical anorexia nervosa.

Adolescent↗

Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings.

Wilson disease (WD) that manifests solely with acute and severe neurological damage in the absence of hepatic disease and Kayser-Fleischer ring of the cornea is rare and difficult to diagnose at the acute setting. This report describes unusual diffusion and proton spectroscopic magnetic resonance (MR) imaging findings in a 12-year-old boy with WD who presented with hemichorea and subnormal copper metabolism. The MR imaging findings of lactate accumulation, decrease of N-acerylaspartate/creatinine (NAA/Cr) ratio and markedly increased apparent diffusion coefficient (ADC) value of the asymmetrical edematous putaminal lesions in the early stage were suggestive of acute necrosis with anaerobic metabolism of glucose leading to poor clinical outcome at follow-up.

Child↗

MRI/MRA evaluation of sickle cell disease of the brain.

Sickle cell disease is a major cause of pediatric stroke. Understanding the disease that affects the brain as infarctions, both clinically apparent and silent, requires an understanding of how the blood vessels are affected, the way in which both the brain and the blood vessels are imaged by MRI and MRA and the mechanism of injury.

Adolescent↗

Symptomatic Chiari I malformation in Kabuki syndrome.

Kabuki (Niikawa-Kuroki) syndrome is associated with a characteristic facial appearance, cleft palate, congenital heart defects, and developmental delay. Structural brain anomalies have only occasionally been described in Kabuki syndrome. Chiari type I malformation, characterized by caudal herniation of the cerebellar tonsils through the foramen magnum, has been described only infrequently in association with defined syndromes and has been reported once in association with Kabuki syndrome. We report three additional children with Kabuki syndrome who have Chiari I malformation. Two children presented with chronic headaches and the third patient presented with gait abnormalities in adolescence. The incidence of Chiari I malformation may be higher than previously reported in Kabuki syndrome since it may not be diagnosed until later in childhood, whereas most reports of Kabuki syndrome are of young children. Further, symptoms of Chiari I anomaly can be somewhat nonspecific. Thus, we suggest that Chiari type I be considered in patients with Kabuki syndrome who present with persistent headache, neck pain, or other symptoms suggestive of Chiari I anomaly.

Abnormalities, Multiple↗

PANDAS with catatonia: a case report. Therapeutic response to lorazepam and plasmapheresis.

This is a report of an 11-year-old, prepubertal boy with acute-onset urinary urgency and frequency, obsessions and compulsions related to urination, severe mood lability, inattention, impulsivity, hyperactivity, and intermittent periods of immobilization. Fever, cough, otitis, and sinusitis preceded neuropsychiatric symptoms. Antistreptolysin O and DNAse B antibody titers were elevated, and magnetic resonance imaging revealed bilateral diffuse caudate nuclei swelling. Plasmapheresis resulted in significant and rapid clinical improvement of obsessive-compulsive disorder symptoms and a simultaneous decrease in basal ganglia swelling, consistent with an immune-mediated pathophysiological process involving group A beta-hemolytic streptococci. Hyperactivity, impulsivity, and inattention improved with lorazepam, suggesting that the attention-deficit/hyperactivity disorder symptoms could be manifestations of catatonia.

Attention Deficit Disorder with Hyperactivity↗

Diffusion-weighted MR imaging of early methotrexate-related neurotoxicity in children.

BACKGROUND AND PURPOSE: Methotrexate is a major cause of treatment-related acute neurotoxicity in children with hematologic malignancies. The purpose of this study was to investigate whether diffusion-weighted MR imaging (DWI) detects acute methotrexate white matter neurotoxicity in this patient population. METHODS: Six children-three female and three male-with hematologic malignancies were studied at time of onset of neurologic dysfunction during the delayed intensification or consolidation phase of therapy, when intensive intrathecal methotrexate is given. MR imaging including DWI was performed on 1.5 T MR scanners. RESULTS: DWI demonstrated abnormal restriction of motion of water in the centrum semiovale in all six patients. This finding correlated to the acute onset of hemiparesis or aphasia. Fluid-attenuated inversion recovery imaging was not positive at this time, but it was positive in all five patients in whom follow-up imaging was performed. CONCLUSION: Early detection of methotrexate white matter injury by DWI has the potential to alert the oncologist to this event and provide a technique by which treatment of neurotoxicity can be monitored.

Adolescent↗

Choroid plexus carcinomas in children: MRI features and patient outcomes.

Choroid plexus carcinomas (CPC) are rare malignant intracranial neoplasms usually occurring in young children. The objectives of this study were to characterize the preoperative MRI features of CPC, determine the frequency of disseminated disease in the CNS at diagnosis, and assess patient outcomes. The preoperative cranial MR images of 11 patients with CPC were retrospectively reviewed for lesion location, lesion size, un-enhanced and enhanced MRI signal characteristics, and presence of disseminated intracranial tumor. Postoperative cranial and spinal MRI images were reviewed for residual, recurrent, and/or disseminated tumor. The study group included six male and five female patients ranging in age from 5 months to 5.3 years (median= 1.8 years). CPC were located in the lateral (n = 8), fourth (n = 1), and third (n = 1) ventricles, and foramen of Luschka (n = 1). Mean tumor size was 5.2 cm x 4.9 cm x 5.0 cm. On short-TR images, CPC had heterogeneous, predominantly intermediate signal with foci of high signal in 45% of lesions from areas of hemorrhage. On long-TR/long-TE images, solid portions of CPC typically had heterogeneous, intermediate-to-slightly-high signal. Small zones of low signal on long-TR/long-TE images were seen in 55% of the lesions secondary to areas of hemorrhage and/or calcifications. Tubular flow voids representing blood vessels were seen in 55% of the lesions. Zones of high signal comparable to CSF were seen in 64% of CPC secondary to cystic/necrotic zones. All CPC showed prominent contrast enhancement. Irregular enhancing margins suggesting subependymal invasion were seen in 73% of the lesions. Findings consistent with edema in the brain adjacent to the enhancing lesions were seen in 73% of CPC. CPC caused hydrocephalus in 82% of patients at diagnosis. Two patients died from hemorrhagic complications from surgical biopsies. Disseminated tumor in the leptomeninges was present in 45% of patients at diagnosis and was associated with a poor prognosis. The 1-year and 5-year survival probabilities were 55% and 45%, respectively. In conclusion, MRI features commonly associated with CPC include large intraventricular lesions with irregular enhancing margins; heterogeneous signal on long TR/long TE images and short-TR images; edema in adjacent brain; hydrocephalus; and presence of disseminated tumor. MRI evidence of disseminated tumor at diagnosis is associated with a poor prognosis.

Carcinoma↗

Periventricular leukomalacia is common after neonatal cardiac surgery.

OBJECTIVES: Periventricular leukomalacia is necrosis of the cerebral white matter adjacent to the lateral ventricles and results from injury to immature oligodendroglia. In infants without congenital heart disease, periventricular leukomalacia is associated with an increased incidence of developmental delay and attention deficit/hyperactivity disorder. The incidence of periventricular leukomalacia and the risk factors for development of periventricular leukomalacia after infant cardiac surgery are not known. METHODS: Magnetic resonance imaging of the brain was performed 6 to 14 days after cardiac surgery utilizing cardiopulmonary bypass with or without deep hypothermic circulatory arrest in 105 neonates and infants < or = 6 months of age. RESULTS: Median age at surgery was 6 days (range 1-178), with 82 neonates (age < or = 30 days). Periventricular leukomalacia was found in 44 of the neonates (54%) compared with 1 of 23 infants (4%). Forward logistic regression using age at surgery as a continuous variable identified a model containing longer total support time (cardiopulmonary bypass plus deep hypothermic circulatory arrest), lower systolic blood pressure at cardiac intensive care unit admission postoperatively, lower minimum diastolic blood pressure, and Po(2) in the first 48 hours after surgery. When age at surgery was considered as a dichotomous variable (neonate versus infant), younger age at surgery replaced systolic blood pressure, Po(2), and total support time in the model. Lower minimum diastolic blood pressure was a significant risk factor in both models. CONCLUSIONS: Periventricular leukomalacia was found in >50% of neonates after cardiac surgery but rarely in older infants. Hypoxemia and hypotension in the early postoperative period, particularly diastolic hypotension, may be important risk factors for periventricular leukomalacia.

Apolipoproteins E↗

Preoperative cerebral blood flow is diminished in neonates with severe congenital heart defects.

OBJECTIVE: Impaired neurodevelopmental outcome represents a major morbidity for survivors of infant heart surgery for congenital heart defects. Previous studies in these neonates have reported preoperative microcephaly, periventricular leukomalacia, and other findings. The hypothesis of this study is that preoperative cerebral blood flow is substantially diminished and might relate to preoperative neurologic conditions. METHODS: Preoperative brain magnetic resonance imaging was performed. Cerebral blood flow measurements in infants with congenital heart defects were obtained by using a novel noninvasive magnetic resonance imaging technique, pulsed arterial spin-label perfusion magnetic resonance imaging. Cerebral blood flow was measured before the operation under standard ventilation and repeated after increased carbon dioxide. RESULTS: A total of 25 term infants were studied. The average age at the time of the operation was 4.4 +/- 4.6 days. Congenital heart defects varied widely. Microcephaly occurred in 24% (6/25). Baseline cerebral blood flow was 19.7 +/- 9.2 mL . 100 g -1 . min -1 (8.0-42.2 mL . 100 g -1 . min -1 ). Five patients had cerebral blood flow measurements of less than 10 mL . 100 g -1 . min -1 . Mean hypercarbic cerebral blood flow increased to 40.1 +/- 20.3 mL . 100 g -1 . min -1 (11.4-94.0 mL . 100 g -1 . min -1 , P < .001). Pairwise analyses found that low hemoglobin levels were associated with higher baseline cerebral blood flow values ( P = .04). Periventricular leukomalacia occurred in 28% (7/25) and was associated with decreased baseline cerebral blood flow values ( P = .05) and a smaller change in cerebral blood flow with hypercarbia ( P = .003). CONCLUSIONS: Structural brain abnormalities are common in these neonates before surgical intervention. Preoperative cerebral blood flow for this cohort was low and drastically reduced in some patients. Low cerebral blood flow values were associated with periventricular leukomalacia. Carbon dioxide reactivity was preserved but might be compromised by some aspects of the cardiac anatomy. The full spectrum of cerebral blood flow measurements with this technique in congenital heart defects and their long-term significance require continued investigation.

Brain↗

MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation.

Maple syrup urine disease (MSUD) is an inborn error of amino acid metabolism, which affects the brain tissue resulting in impairment or death if untreated. Imaging studies have shown reversible brain edema during acute metabolic decompensation. The purpose of this paper is to describe the diffusion-weighted imaging (DWI) and spectroscopy findings during metabolic decompensation and to assess the value of these findings in the prediction of patient outcome. Six patients with the diagnosis of MSUD underwent conventional MR imaging with DWI during acute presentation with metabolic decompensation. Spectroscopy with long TE was performed in four of the six patients. Follow-up examinations were performed after clinical and metabolic recovery. DWI demonstrated marked restriction of proton diffusion compatible with cytotoxic or intramyelinic sheath edema in the brainstem, basal ganglia, thalami, cerebellar and periventricular white matter and the cerebral cortex. This was accompanied by the presence of an abnormal branched-chain amino acids (BCAA) and branched-chain alpha-keto acids (BCKA) peak at 0.9 ppm as well as elevated lactate on proton spectroscopy in all four patients. The changes in all six patients were reversed with treatment without evidence of volume loss or persistent tissue damage. The presence of cytotoxic or intramyelinic edema as evidenced by restricted water diffusion on DWI, with the presence of lactate on spectroscopy, could imply imminent cell death. However, in the context of metabolic decompensation in MSUD, it appears that changes in cell osmolarity and metabolism can reverse completely after metabolic correction.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗

Pediatric perfusion imaging using pulsed arterial spin labeling.

PURPOSE: To test the feasibility of pediatric perfusion imaging using a pulsed arterial spin labeling (ASL) technique at 1.5 T. MATERIALS AND METHODS: ASL perfusion imaging was carried out on seven neurologically normal children and five healthy adults. The signal-to-noise ratio (SNR) of the perfusion images along with T1, M(0), arterial transit time, and the temporal fluctuation of the ASL image series were measured and compared between the two age groups. In addition, ASL perfusion magnetic resonance (MR) was performed on three children with neurologic disorder. RESULTS: In the cohort of neurologically normal children, a 70% increase in the SNR of the ASL perfusion images and a 30% increase in the absolute cerebral blood flow compared to the adult data were observed. The measures of ASL SNR, T1, and M(0) were found to decrease linearly with age. Transit time and temporal fluctuation of the ASL perfusion image series were not significantly different between the two age groups. The feasibility of ASL in the diagnosis of pediatric neurologic disease was also illustrated. CONCLUSION: ASL is a promising tool for pediatric perfusion imaging given the unique and reciprocal benefits in terms of safety and image quality.

Adult↗

In vivo measurement of brain metabolites using two-dimensional double-quantum MR spectroscopy--exploration of GABA levels in a ketogenic diet.

A localized proton 2D double-quantum (DQ) spin-echo spectroscopy technique was implemented on 1.5 T clinical MRI scanners for the detection of gamma-aminobutyrate (GABA) in the brain. The 2D approach facilitates separation of peaks overlapping with GABA in 1D DQ-filtered (DQF) spectra. This technique was applied to four normal adult volunteers and four children with intractable epilepsy. The coefficient of variation of the level of GABA and overlapping macromolecules at F2 = 3.0 ppm and F1 = 4.8 ppm was 0.08 in normal subjects. Three patients received 2D MRS scans before and after initiation of the ketogenic diet (KD): one patient showed a trend of decreasing GABA throughout the study, and two patients showed low initial GABA levels that increased over time. In addition to major metabolites and GABA, low-level metabolites (valine, leucine, and glutathione) were also identified in the 2D spectra.

Adult↗

Clinical strategies in the management of biochemical recurrence after radical prostatectomy.

Prostate cancer is the most frequently diagnosed cancer and the second leading cause of cancer death in men, following lung cancer. Although radical prostatectomy continues to be a curative treatment for most patients diagnosed with prostate cancer, nearly 25% of patients undergoing radical prostatectomy will have biochemical recurrence as defined by an increase in serum prostate-specific antigen (PSA) level to >0.4 ng/mL after prostatectomy or a rapid doubling of the PSA over a 10-year follow-up period. The clinical challenges, an overview of available data, and a framework for the integration of this information for clinical management of biochemical recurrence postprostatectomy for prostate carcinoma are presented in this article. Therapeutic options, in addition to conservative management and watchful waiting, include radiation therapy and androgen deprivation. These options are discussed herein along with expected outcomes.

Androgen Antagonists↗

An MRI study of neurological injury before and after congenital heart surgery.

BACKGROUND: Neurological deficits are observed in patients with congenital heart disease (CHD) before and after neonatal surgery, the etiology being multifactorial. To understand the impact of preoperative events and to characterize the evaluation of neurological injury, we performed serial magnetic resonance imaging (MRI) studies of the brain in a cohort of neonates undergoing open-heart surgery. METHODS AND RESULTS: Twenty-four term neonates with CHD were studied prospectively with brain MRI: before surgery, within 2 weeks of surgery, and several months after surgery. Preoperative MRI examinations showed periventricular leukomalacia (PVL) in 4 patients (16%) and infarct in 2 subjects (8%). MR spectroscopy was performed in 19 subjects preoperatively and revealed elevated brain lactate in 53%. An early postoperative MRI (n=21) identified new PVL in 48%, new infarct in 19%, and new parenchymal hemorrhage in 33%. New lesions or worsening of preoperative lesions occurred in 67% of subjects. No patient- or procedure-related factors for the development of early postoperative lesions were identified. A late postoperative MRI (n=17) demonstrated resolution of early lesions in 8 and mild cerebral atrophy in 2. CONCLUSIONS: Mild ischemic lesions, primarily in the form of PVL, occur in a number of neonates with CHD before surgery and >50% of patients postoperatively. Resolution of these lesions is common 4 to 6 months after surgery. Longer-term follow-up is needed to determine the significance of perioperative ischemic lesions on functional outcome.

Brain↗