MR differential diagnosis of acute deep grey matter pathology in paediatric patients.

A high metabolic demand, rich vascularization and high concentrations of ionic elements leading to the generation of oxygen free radicals, give to the deep grey matter (DGM) nuclei specific susceptibility to both acute and chronic insults, especially in paediatric patients. Reaching a diagnosis in the early stages of acute diseases in many patients is crucial for instigating prompt specific therapy leading to a favourable outcome. On the basis of a review of a 10-year in-house database and a review of the literature on CNS pathology involving the DGM nuclei in paediatric patients, we summarize the MR findings and clinical clues that may help the radiologist in the difficult differential diagnosis process. The terms "acute" and "chronic" refer to the clinical onset of the disease. MR imaging allows the detection of an injury, determination of its precise anatomical location and characterization of the signal changes. This, combined with a knowledge of specific MRI patterns, may be a roadmap to a definite diagnosis. Clinical history, physical and laboratory findings, timing of the MR examination and advanced MR imaging techniques (diffusion-weighted imaging and (1)H-MR spectroscopy), are crucial in some patients.

Encephalocraniocutaneous lipomatosis (ECCL): neuroradiological findings in three patients and a new association with fibrous dysplasia.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome characterized by involvement of tissues of ectodermal and mesodermal origin such as skin, eye, adipose tissue, and brain. Since 1970, when Haberland and Perou had described the first patient, 54 cases of ECCL have been reported in literature. We report on three new boys with ECCL. In addition to their typical dermal, ocular and central nervous system anomalies, one of them had a spheno-ethmoidal osseous lesion. Histopathological evaluation confirmed the benign nature of the lesion and was consistent with fibrous dysplasia. The aim of our study is to review clinical records and brain imaging studies of these three new patients with ECCL and compare these findings with those reported in literature to better define the phenotypic spectrum and radiological findings in ECCL.

Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features.

Parry-Romberg syndrome (PRS) is a sporadic disease of unknown etiology with typical onset in childhood or in young adults. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. The neurological symptoms usually include focal epilepsy, migraine, and unilateral brain lesions on the same side as the atrophy. A common neuroimaging finding of the syndrome is white matter high signal intensity on brain magnetic resonance (MR) imaging. Rasmussen encephalitis (RE) is a rare and chronic inflammatory disease of the brain that begins in the first decade of life and more rarely in adolescents and adults. It usually involves one hemisphere with focal cortical inflammation. Neurologic symptoms are intractable seizures and progressive hemiplegia. Both PRS and RE are often associated with other inflammatory or autoimmune disorders and only 1 case of both syndromes has been reported in literature. We report the clinical and neuroradiological findings in a 6-year-old boy, presenting with focal hemifacial and arm motor seizures and progressive facial hemiatrophy. Serial MR imaging studies revealed progressive brain hemispheric signal alterations and atrophy. This would thus suggest acoexistence of PRS and RE.

Paracoccidioidomicose pulmonar: manifestações clínicas, diagnóstico e tratamento / Pulmonary paracoccidiodomycosis: clinical manifestations, diagnostic and treatment

Relata-se caso de paciente adulta, sexo feminino, residente em Belo Horizonte, com quadro de infecção respiratória, evoluindo há seis meses. Submetida à propedêutica que incluiu biópsia transbrônquica, foi diagnosticada paracoccidioidomicose. Foi instituído tratamento com itraconazol, com melhora clínica da paciente. Esta descrição alerta para o diagnóstico desta micose, que pode ocorrer também no sexo feminino, apesar de pouco freqüente, e ser decorrente de reativação de foco antigo, quando já não existe contato com a área endêmica ou atividade rural.

A female adult living in Belo Horizonte had a respiratory infection for the last sixth months. A transbronchial biopsy revealed pulmonary paracoccidioidomycosis. Treatment with itraconazol improved the patient's clinical condition. It is important to be aware of the occurrence of this disease in urban areas far from the endemic region.