Idiocy amaurotic
Idiotia amaurotic (idiotia amaurotica; Greek idioteia - ignorance; amaurotica, Greek a- - particle of denial + mauros - dark; synonyms: amaurotic familial idiocy, cerebromacular degeneration) is a name that combines hereditary diseases from the group of accumulation diseases caused by a violation, characterized by a progressive decrease in vision and intelligence, and are transmitted in an autosomal recessive manner.
Types of amaurotic idiocy:
- congenital (congenita; synonym: Norman-Wood form of amaurotic idiocy): manifested soon after birth in the form of muscle hypotension, progressive hydrocephalus, seizures, arrest of neuropsychic development;
- late childhood (infantilis tarda; synonyms: Jansky - Bilshovsky form of amaurotic idiocy, Bilshovsky - Jansky's disease): manifested at the age of 3-4 years in the form of slowly growing organic dementia, ataxia, seizures, atrophy of the optic nerves;
- early childhood (infantilis praecox; synonym: Thea - Sachs disease): manifested in the first year of life in the form of mental retardation, progressive decrease in vision to complete blindness, hyperacusis, increasing central paralysis;
- late (tarda; synonym: Kufsa disease): arising in adulthood and manifested in the form of personality changes like organic psychosyndrome, retinitis pigmentosa, cerebellar disorders and progressive deafness;
- juvenile (juvenilis; synonyms: Spielmeyer - Vogt - Batten form of amaurotic idiocy, Spielmeyer - Vogt disease): manifested at the age of 6-10 years in the form of a decrease in intelligence, retinitis pigmentosa, memory impairment, lethargy, severe autonomic-endocrine disorders, inconstant movement …
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