In a study of 138 patients with thalassemia major (homozygous \g=b\-thalassemia, Cooley's anemia), 94 presented typical cephalofacial deformities (CFDs) on clinical inspection. The patient's age and duration of clinical symptoms, the degree of anemia, and the age at onset of transfusions are important in determining the development of CFD and are correlated positively with the degree of such changes. Splenectomy performed no later than the age of four years had some beneficial effect against CFD. Cephalofacial deformities irrespective of degree implied no intellectual impairment in the patients studied. The high incidence of a proximal type of muscular disorder, which occurred only in patients with CFD suggests possible common underlying mechanisms for skeletal and muscular abnormalities. Degree of CFD was proportional in severity to several systemic abnormalities characterizing homozygous \g=b\-thalassemia.Osseous changes in thalassemia major (Cooley's anemia, homo¬ zygous /S-thalassemia) with widening of medullary cavities, atrophy and trabeculation of the spongiosa, and cortical thinning follow hyperplasia of the red marrow. This occurs in re¬ sponse to marrow overstimulation
Twenty patients with Parkinson disease (PD) and twenty normal control subjects (NC) matched on age, sex, education and socio-economic status (SES) were tested for comprehension of four types of relative clauses with complex thematic roles (syntax) and no semantic and pragmatic constraints (reversible) in a sentence-picture matching task. The results show a clear language impairment for PD patients compared to NC. Additional evidence from testing school children in grade 1 (G1) and grade 6 (G6) indicates that G1 children perform similar to PD patients and G6 children perform as high as NC. The overall picture of the findings suggests: (1) PD patients process sentences with complex thematic roles and semantic reversibility on a heuristic and not on an algorithmic basis, a type of behavior assumed to be associated with frontal lobe dysfunction; (2) PD patients display some patterns of language behavior similar to those observed in aphasics. Similarities in language behavior between PD patients and G1 children are discussed with regard to the "regression hypothesis" (Jacobson, 1968).
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