MONGOLISM. - M. or Langdon-Down syndrome (1866) is a state of marked retardation of physical and mental development, congenital but not hereditary; and it owes its name to the fact that the face of affected children resembles the Mongol type; Curschmann (1938) distinguished two varieties of it: Malayan and Eskimo.
At birth, m. occurs in a proportion of about 1 per thousand, without distinctions of race or social class or sex; in the ancestors, syphilis and tuberculosis are frequent, while mental and nervous taints seem rarer. The influence of the mother's relatively advanced age is well known; often the mongoloid is the last of a numerous offspring. The cause of this disease of growth (ausopathy) may be said to be [a matter of] hypotheses presented.
Anatomo-clinical aspects: small cerebral hemispheres, with simple and coarse convolutions and a wide sylvian fissure (thin cortex, poor in cells, scanty development of tangential fibers); thyroid often normal; the hypophysis poor in eosinophil cells; almond-shaped eyes with narrow palpebral fissures and with epicanthus (vertical skin fold) at the inner angle (with frequent blepharitis, strabismus, myopia); flattened cheekbones; small, half-open mouth, with thick lips; voluminous and wrinkled tongue, teeth altered in shape and in development; ogival palatal vault; broad and flattened root of the nose; upper part of the auricle strongly flattened; frequent microbrachycephaly; stature below normal; during infancy a marked muscular hypotonia with great laxity of the joints, regressing with age; umbilical hernias frequent; hypogenitalism pronounced, while hypothyroidism is observed only in mixed types (m. + myxedema); delayed walking. Mentally: oligophrenia generally remaining within the limits of imbecility, that is, at a mental age below 9 years; speech always defective, agrammatic, poor; affectivity fairly well developed, attention very labile; memory weak. In general the patient remains little accessible to discipline and instruction; exceptionally the development of intelligence can reach the degree of "mental weakness."
Mortality is high: 50% of mongoloids die in the first year of life, barely a quarter reach puberty and only 10% reach the 25th year; death is most often due to an affection of the respiratory tract, especially tuberculosis, to which the mongoloid seems particularly disposed. M. does not respond, or responds only to a very limited extent, to medical and pedagogical treatment. The capacity to understand and to will, and, correspondingly, legal and moral responsibility, are proportionate to its degree of mental impairment (v. FRENASTENIA).
Bibliography5 works
Bruno Callieri