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Globus pallidus & kernicterus

Nidhi Prabhakar, Chirag K Ahuja, Niranjan Khandelwal
Bilateral basal ganglia lesions are a common non-specific finding seen in many diseases. One of the differential diagnoses for it, in a child, is kernicterus occurring due to hyperbilirubinemia. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common cause of severe hyperbilirubinemia. A 1-year old child presented to the hospital with history of generalized dystonia in the previous 3 days. MRI showed evidence of symmetrical lesions in bilateral globus pallidus, which were hyperintense on T2/FLAIR and isointense on T1...
April 2018: Annals of Neurosciences
Fu-Chen Yang, Julia Draper, Peter G Smith, Jay L Vivian, Steven M Shapiro, John A Stanford
Neonatal hyperbilirubinemia targets specific brain regions and can lead to kernicterus. One of the most debilitating symptoms of kernicterus is dystonia, which results from bilirubin toxicity to the globus pallidus (GP). Stem cell transplantation into the GP to replace lost neurons and restore basal ganglia circuits function is a potential therapeutic strategy to treat dystonia in kernicterus. In this study we transplanted human medial ganglionic eminence (MGE)-like neural progenitor cells (NPCs) that we differentiated into a primarily gamma-aminobutyric acid (GABA)ergic phenotype, into the GP of non-immunosuppressed jaundiced (jj) and non-jaundiced (Nj) rats...
January 1, 2018: Cell Transplantation
Janet M Rennie, Jeanette Beer, Michele Upton
We examined claims made against the National Health Service (NHS) involving neonatal jaundice in order to determine whether there were lessons that could be learnt from common themes.This was a retrospective anonymised study using information from the NHS Resolution database for 2001-2011.Twenty cases (16 males) had sufficient information for analysis. Fifteen had confirmed cerebral palsy and two young children had damage to the globus pallidus without confirmed CP. In three cases, the outcome was uncertain...
May 25, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
Guijing Jia, Jingshan Gong, Hui Ding, Anqi Li, Juan Wang, Jianmin Xu
OBJECTIVE: To determine risk factors of T1WI high signal intensity at globus pallidus and subthalamic nucleus (GP and STN) of neonates. METHODS: Brain MR images of 186 neonates with intact clinical files were retrospectively reviewed to identify whether there were T1WI high signal intensity at GP and STN. Among them, 15 neonates received followed-up MR imaging in 1-5 months after first MR examination. Statistic comparison of clinical features between neonates with and without T1WI high signal intensity at GP and STN were performed using univariate analyses...
April 21, 2015: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Yvonne W Wu, Michael W Kuzniewicz, Andrea C Wickremasinghe, Eileen M Walsh, Soora Wi, Charles E McCulloch, Thomas B Newman
IMPORTANCE: Exchange transfusion is recommended for newborns with total serum bilirubin (TSB) levels thought to place them at risk for cerebral palsy (CP). However, the excess risk for CP among these infants is unknown. OBJECTIVE: To quantify the risks for CP and CP consistent with kernicterus that are associated with high TSB levels based on the 2004 American Academy of Pediatrics exchange transfusion threshold (ETT) guidelines. DESIGN, SETTING, AND PARTICIPANTS: We enrolled 2 cohorts from a population of 525,409 infants in the Late Impact of Getting Hyperbilirubinemia or Phototherapy (LIGHT) birth cohort...
March 2015: JAMA Pediatrics
Jessica L Wisnowski, Ashok Panigrahy, Michael J Painter, Jon F Watchko
Infants with chronic bilirubin encephalopathy often demonstrate abnormal bilateral, symmetric, high-signal intensity on T2-weighted magnetic resonance imaging of the globus pallidus and subthalamic nucleus, consistent with the neuropathology of kernicterus. Early magnetic resonance imaging of at-risk infants, while frequently showing increased T1-signal in these regions, may give false-positive findings due to the presence of myelin in these structures. Advanced magnetic resonance imaging including diffusion-weighted imaging, magnetic resonance spectroscopy, and diffusion tensor imaging with tractography may shed new insights into the pathogenesis of bilirubin-induced brain injury and the neural basis of long-term disability in infants and children with chronic bilirubin encephalopathy...
November 2014: Seminars in Perinatology
Koichi Maruyama, Mizue Iai, Hiroshi Arai, Kenji Yokochi
OBJECTIVE: Persons with severe motor and intellectual disabilities (SMID) caused by injury to the developing brain sometimes present generalized hypertonia in a specific position with extreme muscle overactivity persisting for most of the time during wakefulness. This "persistent generalized muscle contraction" is often associated with bad humor, sleep disturbance, hyperhidrosis, wasting, elevation of serum creatine kinase levels, regular daytime use of hypnotic or sedative medication, and the necessity to maintain the neck or hip in a flexed position manually...
January 2014: No to Hattatsu. Brain and Development
Katie M Belz, Andrew J Specht, Victoria S Johnson, Julia A Conway
A severe increase in total bilirubin coincided with a decline in neurologic status to comatose in a 9 yr old spayed female mixed-breed dog being treated for immune-mediated hemolytic anemia. MRI of the brain was performed to investigate potential causes for the neurologic signs. MRI revealed bilaterally symmetrical hyperintensities within the caudate nuclei, globus pallidus, thalamus, deep cerebellar nuclei, and cortical gray matter on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences, which coincided with areas of bilirubin deposition and neuronal necrosis (kernicterus) identified on necropsy examination...
July 2013: Journal of the American Animal Hospital Association
Mark S Baron, Kunal D Chaniary, Ann C Rice, Steven M Shapiro
Classical rate-based pathway models are invaluable for conceptualizing direct/indirect basal ganglia pathways, but cannot account for many aspects of normal and abnormal motor control. To better understand the contribution of patterned basal ganglia signaling to normal and pathological motor control, we simultaneously recorded multi-neuronal and EMG activity in normal and dystonic rats. We used the jaundiced Gunn rat model of kernicterus as our experimental model of dystonia. Stainless steel head fixtures were implanted on the skulls and EMG wires were inserted into antagonistic hip muscles in nine dystonic and nine control rats...
2011: Frontiers in Systems Neuroscience
Virginia C Mendes, Fernando Costa, Antónia Silva, Maria José Rosas
Kernicterus is an encephalopathy resulting from the cerebral deposition of unconjugated bilirubin in the neonatal period. We report a case of kernicterus where MRI demonstrated bilateral symmetric high signal intensity and volume loss in the hippocampus in addition to globus pallidus and subthalamic nucleus hyperintensity onT2-weighted images. The authors believe that hippocampal sclerosis, plays a role in adding specificity to the imaging diagnosis of kernicterus.
July 2009: Journal of Pediatric Neurosciences
Umesh C Parashari, Ragini Singh, Rajesh Yadav, Pallavi Aga
The objective of the study was to establish the diagnosis of kernicterus as a cause of seizures and abnormal movements in a 1-year-old child. We performed an MRI of the brain of the child on our 1.5 T scanner. The MRI of the patient showed high signals on T2-weighted images in the globus pallidus bilaterally, with no evidence of mass effect. Because of an increased risk of hearing loss, the brain-stem evoked response examination was also performed. The brain-stem evoked response examination showed bilateral severe sensorineural hearing loss...
July 2009: Journal of Pediatric Neurosciences
Thor Willy Ruud Hansen, Lutz Nietsch, Elisabeth Norman, Jesper Vandborg Bjerre, Jean-Michel Hascoet, Khalaf Mreihil, Finn Ebbesen
AIM: To show the potential for reversing acute intermediate to advanced phase bilirubin encephalopathy. METHODS: Case studies. RESULTS: Six extremely jaundiced infants had symptoms of intermediate to advanced phase acute bilirubin encephalopathy. The infants were treated aggressively. Two patients had brain magnetic resonance imaging showing increased signals in the globus pallidus. On follow-up, all infants are neurologically normal. CONCLUSIONS: Intermediate-to-advanced stage acute bilirubin encephalopathy may occasionally be reversible...
October 2009: Acta Paediatrica
Akihisa Okumura, Hiroyuki Kidokoro, Hiromichi Shoji, Tomoyuki Nakazawa, Masakazu Mimaki, Katsunori Fujii, Hiroshi Oba, Toshiaki Shimizu
OBJECTIVE: We sought to clarify the features of kernicterus in preterm infants. METHODS: The subjects of this study were 8 preterm infants with athetoid cerebral palsy whose gestational ages were < or =34 weeks. We retrospectively investigated clinical, laboratory, MRI, and brainstem auditory evoked potential (BAEP) findings. RESULTS: Gestational age was < or =26 weeks in 6 of the 8 infants, and birth weight was <1000 g in 5 infants...
June 2009: Pediatrics
Wei-Hua Liao, Xiao-Yi Wang, Wu-Lin Wu, Xin-Ya Jiang, Yun-Hai Liu, Fang Liu, Run-Wen Wang
OBJECTIVE: To investigate the value of conventional magnetic resonance imaging (MRI) and diffusion weighed imaging (DWI) in the differentiation of hypoxic-ischemic encephalopathy (HIE) and acute bilirubin encephalopathy in neonates. METHODS: The MRI findings along with DWI characteristics in 15 neonates with HIE involving basal ganglia and in 18 neonates with acute bilirubin encephalopathy between November 2006 and June 2008 were retrospectively reviewed. RESULTS: On T1WI, only 5 patients presented hyperintensity in the globus pallidus in the HIE group, but 16 in the acute bilirubin encephalopathy group (p<0...
March 2009: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Xiaoyi Wang, Wulin Wu, Bob L Hou, Ping Zhang, Ashley Chineah, Fan Liu, Weihua Liao
INTRODUCTION: The purpose of this study was to evaluate the diagnostic value of conventional magnetic resonance imaging (MRI), proton magnetic resonance spectroscopy ((1)H-MRS), and diffusion-weighted imaging (DWI) for neonatal bilirubin encephalopathy. METHODS: We collected conventional MRI in 24 neonates with neonatal bilirubin encephalopathy. We performed (1)H-MRS and DWI sequences to nine of the 24 patients and seven age-matched healthy control subjects. Multiple-voxel (1)H-MRS data were acquired using PRESS pulse sequence with TE = 135 ms and TR = 1500 ms...
October 2008: Neuroradiology
Yasuo Hachiya, Masaharu Hayashi
Bilirubin encephalopathy (BE), which includes acute (kernicterus) and chronic (postkernicteric) forms, results from severe neonatal jaundice. In order to investigate neurodegenerative mechanisms in autopsy cases of BE, we immunohistochemically examined expressions of neurotransmitters, neuropeptides, and calcium-binding proteins in the basal ganglia; and deposition of oxidative products. Expression of tyrosine hydroxylase was reduced in the putamen in cases of acute BE, and in the globus pallidus in cases of acute and chronic postkernicteric BE...
April 2008: Brain & Development
Stephanie L Merhar, Donald L Gilbert
Chronic bilirubin encephalopathy, characterized clinically by extrapyramidal movement abnormalities, vertical gaze abnormalities, and hearing loss, results from neuronal injury after marked hyperbilirubinemia in term and preterm infants. In premature infants, bilirubin staining of specific brain structures has been described at autopsy after only moderate hyperbilirubinemia, but classic chronic bilirubin encephalopathy without marked hyperbilirubinemia has been reported only rarely. We report a case of a 7-year-old, former 29-weeks' gestation, gravely ill premature infant with a peak bilirubin level of 13...
November 2005: Pediatrics
Suad F AlOtaibi, Susan Blaser, Daune L MacGregor
OBJECTIVE AND BACKGROUND: Prevention of bilirubin encephalopathy relies on the detection of newborns who are at risk of developing serious hyperbilirubinemia. The objective of this study was to reassess the clinical syndrome of kernicterus as neurodiagnostic studies have become more readily available and can be used to evaluate these infants. METHODS: The study population was neonates born at term or near term admitted to The Hospital for Sick Children in Toronto, Ontario, Canada, between January 1990 and May 2000...
August 2005: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Wendy K Oakden, Aideen M Moore, Susan Blaser, Michael D Noseworthy
BACKGROUND AND PURPOSE: MR examination of infants with kernicterus shows abnormal changes in signal intensity in various parts of the brain, including the globus pallidus and subthalamic regions. The purpose of this study was to retrospectively analyze proton ((1)H) MR spectroscopic data to see if the MR spectroscopic profiles of infants with hyperbilirubinemia and symptoms of kernicterus provide new insights into the pathophysiology of bilirubin neurotoxicity. METHODS: Six patients aged 3 days to 3 weeks, with hyperbilirubinemia and symptoms of kernicterus underwent MR imaging and MR spectroscopy with a single-voxel point-resolved spectroscopic sequence...
June 2005: AJNR. American Journal of Neuroradiology
Steven M Shapiro
Kernicterus, currently used to describe both the neuropathology of bilirubin-induced brain injury and its associated clinical findings, is a complex syndrome. The neurobiology of kernicterus, including the determinants and mechanisms of neuronal injury, is discussed along with traditional and evolving definitions ranging from classical kernicterus with athetoid cerebral palsy, impaired upward gaze and deafness, to isolated conditions, for example, auditory neuropathy or dys-synchrony (AN/AD), and subtle bilirubin-induced neurological dysfunction (BIND)...
January 2005: Journal of Perinatology: Official Journal of the California Perinatal Association
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