Disease Information for Encephalopathy, myoclonic, infantile (Kinsbourne)

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Clinical Manifestations
Signs & Symptoms
Infant/loss of sucking response
Abnormal movements/involuntary
CNS symptoms/signs
Cognitive defect
Delayed speech/language development
Infant Seizures
Involuntary movements
Myoclonus/Myoclonic jerks on exam
Newborn Poor Sucking response/reflex
Obtunded/poorly responsive status/signs
Polymyoclonus
Seizures Newborn
Seizures/Children/recurrent
Opsoclonus/chaotic eye movements
Clinical Presentation & Variations
Presentation/Infant Jerky Eye Movements Ataxia MR
Presentation/Movement Disorder Ataxia Dementia Progressive
Presentation/Progressive dementia Child Infant
Presents/Myoclonus Epilepsy Mental Decline <20
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Circadian rhythm, same time event/flair
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Infant
Associated Diseases & Rule outs
Associated Disease & Complications
CNS disease/involvement
Coma in Children
Coma/Unconscious
Developmental neurologic degeneration/child
Encephalopathy, myoclonic infantile (Kinsbourne)
Encephalopathy, myoclonic, infantile
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Brain/CNS disorder (ex)
CLASS/Neurologic (category)
Process
PROCESS/Congenital/developmental (category)
Synonyms
Synonym
Kinsbourne Syndrome, Myoclonic encephalopathy of infants, Opsoclonus myoclonus, Synonym/Dancing eyes syndrome, Synonym/Infantile polymyoclonia, Synonym/Kinsbourne's disease, Synonym/Opsoclonus syndrome, Synonym/Opsoclonus-myoclonus encephalopathy
Treatment
Other Treatments
TX/Therapeutic Hypothermia
Definition

Synonyms of Kinsbourne Syndrome; Myoclonic Encephalopathy, Kinsbourne Type; Opsoclonic Encephalopathy

Opsoclonus-Myoclonus; Kinsbourne Syndrome is a rare neurological disorder that usually affects infants and young children; The disorder is characterized by the sudden onset of brief, repeated, shock-like spasms of several muscles within the arms, legs, or entire body (polymyoclonus), an impaired ability to control voluntary movements (ataxia), and continual, involuntary, rapid eye movements in both horizontal and vertical directions (opsoclonus); In approximately 50 percent of affected individuals, a malignant tumor, usually a tumor of embryonic nerve cells (neuroblastoma), is responsible for the symptoms associated with Kinsbourne Syndrome; In most other cases, Kinsbourne Syndrome may be due to a viral infection such as Coxsackievirus B3, poliovirus, or St Louis encephalitis virus; Rarely, the disorder may result due to other underlying causes such as a tumor within the skull (intracranial tumors) or hydrocephalus, a condition in which inhibition of the normal flow of cerebrospinal fluid (CSF) and abnormal widening (dilatation) of the cerebral spaces of the brain (ventricles) causes accumulation of CSF in the skull and potentially increased pressure on brain tissue----------[NORD 2005]----------

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External Links Related to Encephalopathy, myoclonic, infantile (Kinsbourne)
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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