Biomaterial Database

[Organic dementia as a first symptom of infantile Huntington's chorea (author's transl)]. 1976

E Maida, and G Schnaberth

A loss of intellectual capacity at the age of 10 was the first symptom in a case of infantile Huntington's chorea. Subsequently a rigid hypokinetic syndrome occurred only at the age of 20. The dementia syndrome was confirmed in psychological tests. Auxiliary findings such as a diffuse abnornal EEG, as well as a dilatation of the lateral ventricles and an extension of the subarachnoid spaces in the PEG corresponded to a positive diagnosis of Huntington's chorea.

UI	MeSH Term	Description	Entries
D009461	Neurologic Manifestations	Clinical signs and symptoms caused by nervous system injury or dysfunction.	Neurologic Deficits,Neurologic Signs and Symptoms,Focal Neurologic Deficits,Manifestations, Neurologic,Manifestations, Neurological,Neurologic Dysfunction,Neurologic Findings,Neurologic Manifestation,Neurologic Signs,Neurologic Symptoms,Neurological Manifestations,Deficit, Focal Neurologic,Deficit, Neurologic,Deficits, Focal Neurologic,Deficits, Neurologic,Dysfunction, Neurologic,Dysfunctions, Neurologic,Finding, Neurologic,Findings, Neurologic,Focal Neurologic Deficit,Manifestation, Neurologic,Manifestation, Neurological,Neurologic Deficit,Neurologic Deficit, Focal,Neurologic Deficits, Focal,Neurologic Dysfunctions,Neurologic Finding,Neurologic Sign,Neurologic Symptom,Neurological Manifestation,Sign, Neurologic,Signs, Neurologic,Symptom, Neurologic,Symptoms, Neurologic
D010375	Pedigree	The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.	Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D003951	Diagnostic Errors	Incorrect or incomplete diagnoses following clinical or technical diagnostic procedures.	Diagnostic Blind Spots,Errors, Diagnostic,Misdiagnosis,Blind Spot, Diagnostic,Blind Spots, Diagnostic,Diagnostic Blind Spot,Diagnostic Error,Error, Diagnostic,Misdiagnoses
D004569	Electroencephalography	Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain.	EEG,Electroencephalogram,Electroencephalograms
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006816	Huntington Disease	A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)	Huntington Chorea,Juvenile Huntington Disease,Akinetic-Rigid Variant of Huntington Disease,Chorea, Chronic Progressive Hereditary (Huntington),Chronic Progressive Hereditary Chorea (Huntington),Huntington Chronic Progressive Hereditary Chorea,Huntington Disease, Akinetic-Rigid Variant,Huntington Disease, Juvenile,Huntington Disease, Juvenile-Onset,Huntington Disease, Late Onset,Huntington's Chorea,Huntington's Disease,Juvenile-Onset Huntington Disease,Late-Onset Huntington Disease,Progressive Chorea, Chronic Hereditary (Huntington),Progressive Chorea, Hereditary, Chronic (Huntington),Akinetic Rigid Variant of Huntington Disease,Chorea, Huntington,Chorea, Huntington's,Huntington Disease, Akinetic Rigid Variant,Huntington Disease, Juvenile Onset,Huntington Disease, Late-Onset,Juvenile Onset Huntington Disease,Late Onset Huntington Disease
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths