Maria Teresa Carvalho de Lacerda

Leukoencephalopathy, cerebral calcifications, and cysts: New observations

We describe three cases of the rare syndrome of leukoencephalopathy, brain calcifications, and cysts. Conventional MRI, proton spectroscopy, and diffusion-weighted imaging yielded additional information on the disease. Imaging findings favor increased water content rather than a demyelinating process in the pathophysiology of this disease. Clinical features of Coats disease and consanguinity were also encountered.

Leukoencephalopathy with vanishing white matter: report of four cases from three unrelated Brazilian families.

Four patients with leukoencephalopathy with vanishing white matter from three unrelated Brazilian families are reported. In all cases the initial symptoms occurred in the three first years of life. In three cases the onset was acute and at least in two patients the involvement of the white matter preceded the clinical symptoms. Only cerebellar and pyramidal signs were present and persisted throughout the evolution. An episodic course with worsening of the symptoms during febrile illnesses was noted in one patient. In three patients a significant deceleration of the head growth was noted. In one family, brother and sister were affected but the twin brother of the boy was free from the disease. In another family, the patient had a sister who died at 13 years of age from an identical disease not diagnosed at that time. In one family, the parents were first cousins. In all patients, serial magnetic resonance imaging and magnetic resonance spectroscopy showed the characteristic picture of the involvement of the white matter with increasing signal intensity close to that of the cerebrospinal fluid.

Avaliação textural por ressonância magnética dos tumores da fossa posterior em crianças

OBJECTIVE: To distinguish healthy from pathological tissues in pediatric patients with posterior cranial fossa tumors using calculated textural parameters from magnetic resonance images. MATERIALS AND METHODS: We evaluated 14 pediatric patients with posterior cranial fossa tumors using the software MaZda to define the texture parameters in selected regions of interest representing healthy and pathological tissues based on T2-weighted magnetic resonance images. RESULTS: There was a statistically significant difference between normal and tumoral tissues as well as between supposedly normal tissues adjacent and distant from the tumoral lesion. CONCLUSION: Magnetic resonance textural evaluation is an useful tool for determining differences among various tissues, including tissues that appear apparently normal on visual analysis.

Duplicação da hipófise e da haste hipofisária: relato de um caso e revisão da literatura

The authors report a case of a female patient with chronological age of 7 years and bone age of 11 years, presenting with bilateral breast stage Tanner III and gonadotrophin-dependent precocious puberty. Magnetic resonance imaging of the sellar region demonstrated duplication of the pituitary gland and stalk associated with a hypothalamic hamartoma.