Disease Information for Rasmussen's syndrome/encephalitis

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Clinical Manifestations
Signs & Symptoms
Adolescent
Unilateral symptoms/signs/findings
Weakness/one arm
Aphasia
Delayed speech/language development
Delirium
Delirium/Agitated delirium
Dysphasia
Frontal lobe signs
Hemiplegic Gait
Infant Seizures
Irritability/short tempered
Mental Deficiency Child
Mental Slowing Deteriation
Motor slowing
Myoclonus/Myoclonic jerks on exam
Seizures/Children/recurrent
Delayed speech development/impediment
Chronic Fatigue
Typical Clinical Presentation
Presentation/Epilepsy/several types/focal motor/child
Clinical Presentation & Variations
Presentation/Child Hemiplegia Encephalopathy Dementia Seizures
Disease Progression
Course/Progressive/slowly chronic illness
Onset/5-15 years old
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Adolescent
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
CSF abnormal
CSF Colloidal gold curve abnormal
Abnormal Lab Findings - Increased
CSF Cells
CSF Leukocytes
Diagnostic Test Results
Other Tests & Procedures
Lumbar puncture/Increased CSF pressure/LP test
Pathology
BX/Brain biopsy/Abnormal
PATH/Brain abnormal
PATH/Brain focal encephalopathy
PATH/Brain Neuronophagia microglial nodules motor frontal
PATH/Brain/Cellular infiltrate/chronic
PATH/Brain/Pyramidal cell loss
PATH/Encephalomalacia/inflammatory frontal lobe
Electrodiagnosis
EEG/Periodic lateralizing epileptiform discharge/PLED
EEG/Seizure activity abnormality
CT Scan
CT Scan/Head Brain Abnormality
MRI/Head Brain Abnormal
SPECT Head Scan/Thallium 201/abnormal
MRI/Chest abnormal
Associated Diseases & Rule outs
Rule Outs
Cortical dysplasia
MELAS/Encephalopathy
Russian Encephalitis
Tuberous Sclerosis
Associated Disease & Complications
Cerebral hemisphere/involvement lesion
CNS disease/involvement
Encephalitis
Encephalopathy/CNS effect
Epilepsy
Epilepsy partialis continua
Focal motor seizure
Hemiplegia
Meningitis, chronic
Mental retardation
Neurodevelopmental disorders
Partial complex seizure
Seizure disorder (epilepsy)
Status epilepticus
Todds paralysis/postictal
Disease Mechanism & Classification
Specific Agent
AGENT/Unclassified/unknown microbe (category)
Class
CLASS/Pediatric disorders (ex)
CLASS/Primary organ/system disorder (ex)
CLASS/Brain/CNS disorder (ex)
CLASS/Frontal lobe involvement/disorder (ex)
CLASS/Neurologic (category)
CLASS/Temporal lobe disorder (ex)
Pathophysiology
Pathophysiology/Autoimmune GluR3 glutamate receptors/CNS
Pathophysiology/Epileptic encephalopathy
Pathophysiology/Indolent hemispheric encephalitis
Process
PROCESS/Autoimmune disorder (ex)
PROCESS/Idiopathic/unclassified/unknown (category)
PROCESS/INCIDENCE/Esoteric disease (example)
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/Post-infectious disorder (ex)
Synonyms
Synonym
ENCEPH RASMUSSEN, Encephalitis Rasmussen, RASMUSSEN ENCEPH, Rasmussen encephalitis, Rasmussen syndrome, Rasmussen syndrome (disorder), Rasmussen's Syndrome, Synonym/Chronic encephalitis and epilepsy, Synonym/Chronic focal encephalitis (Rasmussen), Synonym/Chronic hemispheric encephalitis syndrome, Synonym/Chronic localized/focal encephalitis, Synonym/Epilepsy-Hemiplegia-mental retardation, Synonym/Rasmussen's disease, Synonym/Rasmussen's encephalitis/hemiplegia, Synonym/RE (Rasmussen Encephalitis]
Treatment
Drug Therapy - Indication
RX/Corticosteroid (Cortisone)
RX/Gamma globulin (intravenous, high dose)
SX/Hemispherectomy
Other Treatments
TX/Ketogenic diet
TX/Plasmapheresis/exchange.
Definition

Rare chronic mildly progressive encephalitis with epilepsy; hemispheric limit involves frontal lobe and maybe temporal;

focal cellular infiltrate; mental retarded regression; onset always before age 10; hemispherectomy has helped but as post viral post-infectious autoimmune disorder IVIG infusion or plasmaphoresis has helped; seizures are hard to treat; surgery seems to halt progression; signs are unilateral but cellular infiltrate may be bilateral; 5 yr course variable; possible post flu or measles onset; residuals occur but aphasia often clears

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Rasmussen"s encephalitis; Rasmussen"s disease ; Rasmussen"s syndrome; An extremely rare, progressive, central nervous system disorder, characterized by frequent and severe seizures, loss of motor skills, hemiparesis, inflammation of the brain, and mental deterioration; This disorder occurs almost exclusively in children under the age of 10;

----------------[whonamedit_com 2005]----------

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External Links Related to Rasmussen's syndrome/encephalitis
Google
Wikipedia
Merck
Images
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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