Disease Information for Alpers cerebral degeneration of infancy

Ads
Clinical Manifestations
Signs & Symptoms
Central Facial Paralysis
Equinus Posture/Ankles
Infant Seizures
Seizures
Seizures/Children/recurrent
Spastic paralysis/signs
Spasticity/Spastic gait
Clinical Presentation & Variations
Presentation/Progressive dementia Child Infant
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Prognosis bad/usually
Course/Progressive
Onset/1-5 years old
Onset/Infancy
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Family History
Family history/Childhood CNS degenerating disease/death
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings - Increased
Lactic acid/Lactate (Lab)
pH, arterial blood (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
Acidosis
Acidosis, metabolic
Alpers cerebral degeneration of infancy
Ascites in Children
Chronic liver disease
Cirrhosis
Cirrhosis in childhood
Cirrhosis/complication/secondary
Cognitive/learning disability
Congenital cataracts
Convulsions (grand mal)
Dementia
Lactic acidosis
Microcephaly/oligophrenia
Poliodystrophy/congenital
Spinal cord lesion/dysfunction
Upper GI Bleeding in children
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Liver involvement/disorder (ex)
CLASS/Brain/CNS disorder (ex)
CLASS/Neurologic (category)
CLASS/Spinal cord disorder (ex)
Pathophysiology
Pathophysiology/Gene locus 15q25
Pathophysiology/Gene locus Chromosome 15
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Congenital Brain malformation
Process
PROCESS/Hereditary mitochondrial disorder (ex)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Mitochondrial disorders (ex)
Synonyms
Synonym
Alper Disease, ALPER SYNDROME, Alpers, ALPERS DIFFUSE DEGENERATION OF CEREBRAL GRAY MATTER WITH HEPATIC CIRRHOSIS, ALPERS DIS, Alper's Disease, Alpers' Disease, Alpers' disease or gray matter degeneration, ALPERS HUTTENLOCHER SYNDROME, ALPERS PROGRESSIVE INFANTILE POLIODYSTROPHY, Alpers Syndrome, Alper's Syndrome, Alpers' Syndrome, degeneration gray matter, degeneration grey matter, Disease Alpers', gray matter degeneration, grey matter degeneration, NEURONAL DEGENERATION OF CHILDHOOD WITH LIVER DISEASE PROGRESSIVE, PNDC, Poliodystrophy, Prog neur degen liver cirrho, Progressive degeneration cerebral gray matter, Progressive neuronal degeneration with liver cirrhosis, Progressive sclerosing poliodystrophy, Progressive sclerosing poliodystrophy (disorder), Spongy glioneuronal dystrophy, Syndrome Alpers, Synonym/Alpers Diffuse cerebral grey matter/hepatic cirrhosis, Synonym/Alpers disease variant, Synonym/Alpers Progressive infantile Poliodystrophy, Synonym/Diffuse cerebral degeneration in infancy/Alpers, Synonym/Hereditary mitochondrial disease/Alpers type, Synonym/Poliodystrophia Cerebri Progressiva, Synonym/Progressive Cerebral Poliodystrophy
Definition

Alpers Disease; Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis; Alpers Progressive Infantile Poliodystrophy; Diffuse Cerebral Degeneration in Infancy; Poliodystrophia Cerebri Progressiva; Progressive Cerebral Poliodystrophy.

Discussion: Alpers Disease is a progressive neurologic disorder that begins during childhood; Symptoms include increased muscle tone with exaggerated reflexes (spasticity), seizures, loss of cognitive ability (dementia) and, in many cases, liver disease; [NORD 2005]

(Edit)

External Links Related to Alpers cerebral degeneration of infancy
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)
Ads