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kernicterus dystonia

Fu-Chen Yang, Sean M Riordan, Michelle Winter, Li Gan, Peter G Smith, Jay L Vivian, Steven M Shapiro, John A Stanford
High levels of bilirubin in infants can cause kernicterus, which includes basal ganglia damage and dystonia. Stem cell transplantation may be an effective treatment for this disease. In this study, we transplanted human neural progenitor cells differentiated toward propriospinal interneurons into the striatum of 20-day-old spontaneously jaundiced (jj) Gunn rats and nonjaundiced (Nj) littermates. Using immunohistochemical methods, we found that grafted cells survived and grew fibers in jj and Nj brains 3 weeks after transplantation...
April 13, 2017: Cell Transplantation
Ayumi Yoshimura, Tetsuya Kibe, Kenji Yokochi
Os odontoideum is a separate ossicle from the odontoid process from the body of the axis by a variable transverse gap. A boy with cerebral palsy probably due to prematurity and kernicterus, was treated with botulinum toxin for continuous dystonic movements at the age of 3.5 years. Although botulinum toxin appeared to be remarkably effective for relaxing hypertonia, abnormal frequent anterior flexion of the neck remained. Because of feeding difficulty and frequent aspiration episodes, additional botulinum toxin therapy was discontinued...
July 2014: No to Hattatsu. Brain and Development
Luigi Bassani, David H Harter
OBJECT: Techniques for the placement of intrathecal baclofen (ITB) systems have been described in detail, with special consideration given to complications from hardware placement. Risks including catheter kinking and migration, hardware erosion through the skin, and lumbar CSF leak are elevated given the often-low body mass index and poor nutritional status of this patient population. The bulk of a spinal catheter and fascial connector within the lumbar wound may increase the potential for the aforementioned risks, leading to potential risks for wound infection and breakdown...
January 2012: Journal of Neurosurgery. Pediatrics
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
M Netravathi, P K Pal, B Indira Devi
BACKGROUND AND PURPOSE: Studying patients with secondary movement disorders (SMD) provides a unique opportunity to determine the correlation between the etiology and phenomenology of movement disorders. METHODS: This was a prospective study of 103 patients (43 women, 60 men; age=28.7±17.3 years; range=2-70 years) with SMD, in a tertiary hospital over 4 years. RESULTS: The mean age of onset of SMD was 24.3±19.7 years, and duration of symptoms was 4...
February 2012: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Allison Przekop, Terence D Sanger
"Athetosis," from the Greek athetos, meaning "without fixed position," is a movement disorder first described by Hammond in 1871. The term described slow, irregular continual movements of the distal extremities. In 1983, Foley defined the athetoid syndrome as "a nonprogressive but evolving disorder due to damage to the basal ganglia of the full-term brain … [with] impairment of postural reflexes, arrhythmical involuntary movements, and dysarthria, [but] sparing … sensation, ocular movements and … intelligence...
2011: Handbook of Clinical Neurology
Kunal D Chaniary, Mark S Baron, Ann C Rice, Paul A Wetzel, Viswanathan Ramakrishnan, Steven M Shapiro
Spontaneously jaundiced Gunn rats exposed to sulfadimethoxine develop bilirubin encephalopathy (kernicterus) with hearing loss and dystonia, closely resembling the human syndrome. We recently characterized the electromyographic activity in this animal model supporting our clinical impression of dystonia. The objective of this study was to develop a simple, non-invasive method to quantify the motor deficits in dystonic rodents. On postnatal day 16, Gunn rats were treated with 100mg/kg of sulfadimethoxine or saline...
June 15, 2009: Journal of Neuroscience Methods
Kunal Chaniary, Mark Baron, Ann Rice, Paul Wetzel, Steven Shapiro
Kernicterus is known to produce damage to the auditory system and the basal ganglia in humans. Although the Gunn rat model of kernicterus has been extensively used to characterize the auditory features, this model has not been similarly utilized to systematically investigate the movement disorder. In the present study, spontaneously jaundiced (jj) 16 day old Gunn rat pups were treated with sulfadimethoxine to exacerbate bilirubin toxicity and compared to saline treated jjs and non-jaundiced (Nj) littermates...
June 15, 2008: Movement Disorders: Official Journal of the Movement Disorder Society
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
Yahya Paksoy, Hasan Koç, Bülent Oğuz Genç
Two children with mental retardation, choreoathetosis, dystonia, and muscle rigidity are reported. They had a history of severe hyperbilirubinemia after birth as a result of Rh isoimmunization. The history and clinical picture suggested the diagnosis of kernicterus. The magnetic resonance imaging examination showed a bilateral signal intensity increase in the globus pallidus on T2-weighted images. Additionally, our patients showed symmetric bilateral hyperintensity and volume loss in the hippocampus, which is known to be another characteristic area of bilirubin deposition in kernicterus...
March 2004: Journal of Computer Assisted Tomography
No abstract text is available yet for this article.
August 1964: Developmental Medicine and Child Neurology
Jaishri Blakeley, Joseph Jankovic
PxD are sudden, episodic, involuntary movement disorders that may include any combination of dystonia, chorea, athetosis, or ballism. The majority of reported cases are familial or idiopathic; however, there have been several reports of secondary PxD. We report 20 new cases of secondary, non-psychogenic PxD, and review 130 cases reported in the literature. The results suggest that although PxD is a rare disorder, secondary forms may be more common than previously recognized, accounting for 26% of all cases in our series...
2002: Advances in Neurology
M V Johnston, A H Hoon
Magnetic resonance imaging and neuropathologic studies have demonstrated remarkably selective patterns of injury to subregions of the basal ganglia in children. Examples are kernicterus and certain mitochondrial encephalopathies, which cause selective injury to the globus pallidus, and near-total perinatal asphyxia, which causes lesions in the putamen and thalamus. To explain the differential vulnerability of nuclei within millimeters of each other, we hypothesize that their locations within the neurotransmitter-specific circuitry of the basal ganglia motor loop are important...
September 2000: Journal of Child Neurology
M Steinborn, K C Seelos, A Heuck, H von Voss, M Reiser
We report on a 2.5-year-old boy with severe mental retardation, choreoathetosis, dystonia, muscle rigidity, opisthotonus and severe hearing impairment. He had history of severe hyperbilirubinaemia immediately after birth presumably due to ABO incompatibility. The history and the clinical picture suggested the diagnosis of Kernicterus. The MR imaging examination upon admission revealed bilateral signal intensity increase in the globus pallidum on T2-weighted sequences. Additionally, our patient showed signal intensity changes within the subthalamic nuclei, which is known to be another characteristic area of bilirubin deposition in Kernicterus...
1999: European Radiology
B L Scott, J Jankovic
We studied 53 patients (64% females) with static brain lesions who developed progressive movement disorders. Of these, 50 (94%) had dystonia, 17 (32%) tremor, eight (15%) parkinsonism, seven (13%) myoclonus, and three (6%) chorea. The precipitating insults included perinatal hypoxia/ischemia in 22 (42%), stroke in 12 (23%), head injury in eight (15%), encephalitis in eight (15%), and carbon monoxide poisoning, kernicterus, and radiation necrosis in one patient (2%) each. Among the 30 patients with initial insult occurring at age 2 years or younger (Infant group), distribution of dystonia at follow-up was focal in three (10%), segmental in eight (27%), unilateral in 10 (33%), and generalized in nine (30%)...
January 1996: Neurology
M Aramideh, L J Bour, J H Koelman, J D Speelman, B W Ongerboer de Visser
We report on four patients with involuntary eyelid closure and eye movement disorders. Three were healthy until the onset of their illness and one had a mild generalized choreoathetosis and dystonia due to kernicterus. Electromyographic recording revealed solely blepharospasm in two patients and blepharospasm in combination with involuntary levator palpebrae inhibition in the other two. The eye movement abnormalities were clinically characterized by inability to fix gaze and short or prolonged episodes of uncontrollable eye deviations accompanied, in two patients, by diplopia in horizontal or vertical directions...
December 1994: Brain: a Journal of Neurology
J Foley
Two hundred and nineteen cases of the dyskinetic and dystonic forms of cerebral palsy which were seen in the course of three decades at a single clinic have been analysed. Fifty-seven patients had kernicterus. In the remaining 162, 71% of whom were born at term, birthweight was below the expected mean in two-thirds. There was no relationship between birth weight, or abnormal birth, or asphyxia, and the ultimate clinical severity of the children. We conclude that abnormal birth and asphyxia are not direct causes of the cerebral damage, but are expressions of a pre-existing condition resulting in susceptibility to the stress of birth, whether it is normal or abnormal...
January 1992: Acta Paediatrica
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