The authors report one case of amyloidosis associated with muscular pseudohypertrophy in a 46-year-old woman, who developed weakness, macroglossia and muscle hypertrophy associated with primary systemic amyloidosis. Electromyography showed a myopathic pattern and bilateral carpal tunnel syndrome. The muscle biopsy presented with a type I and II fiber hypertrophy and infiltration of amyloid material in the interstitious space and artery walls. She underwent bone marrow transplantation with stabilization and subjective improvement of the clinical picture.KEY WORDS: muscular hypertrophy, muscle biopsy, amyloidosis.Pseudo-hipertrofia muscular associada com amiloidose: relato de caso RESUMO -Descreve-se um caso de pseudo-hipertrofia muscular associada a amiloidose primária em uma paciente do sexo feminino, com 46 anos, que apresentava astenia e macroglossia. O estudo eletromiográfico mostrou padrão miopático e síndrome do túnel do carpo, bilateral. A biópsia muscular revelou hipertrofia de fibras tipo I e II, com infiltração de material amilóide no interstício e parede dos vasos, principalmente arteriais. A paciente foi submetida a transplante autólogo de medula óssea, evoluindo com estabilização do quadro e um sentimento subjetivo de melhora.

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The intravenous pamidronate in a group of postmenopausal women with predominant high risk of fracture promoted an isolated gain in the spine BMD, even though, clinical randomized trials are needed to confirm its anti-fracture efficacy.

Background Subtle cognitive impairments have been described in children with congenital hypothyroidism (CH) detected by neonatal screening (NS), even with early and adequate treatment. Patients with CH may present brain cortical thickness (CT) abnormalities, which may be associated with neurocognitive impairments. Aims To evaluate the CT in adolescents with CH detected by the NS Program (Paraná, Brazil), and to correlate possible abnormalities with cognitive level and variables of neurocognitive prognosis. Methods Review of medical records followed by psychometric evaluation of adolescents with CH. Brain magnetic resonance imaging with analysis of 33 brain areas of each hemisphere was performed in 41 patients (29 females) and in a control group of 20 healthy adolescents. CT values were correlated with Full-scale Intelligence Quotient (FSIQ) scores, age at start of treatment, pretreatment thyroxine levels and maternal schooling. Results No significant difference in CT between patients and controls. However, there was a trend toward thinning in the right lateral orbitofrontal cortex among patients and in the right postcentral gyrus cortex among controls. CT correlated significantly with FSIQ scores and with age at start of treatment in one area, and with hypothyroidism severity in five brain areas. Maternal schooling level did not correlate with CT but was significantly correlated with FSIQ. Cognitive level was within average in 44.7% of patients (13.2% had intellectual deficiency). Conclusions There was a trend toward morphometric alterations in the cerebral cortex of adolescents with CH compared with healthy controls. The correlations between CT and variables of neurocognitive prognosis emphasize the influence of hypothyroidism on cortical development. Socioeconomic status exerts a limiting factor on cognitive outcome.