Unusual primary central nervous system lymphoma location involving the fourth ventricle and hypothalamus.

A previously healthy 65-year-old man presented with a two-week history of weight loss, headaches, blurred vision, asthenia and quickly worsening walking impairment. He denied photophobia, neck stiffness, fever, nausea or vomiting.Neurological examination showed global motor slowing, tendency to fall asleep during the clinical examination, generalized weakness against resistance to head and limbs, and osteotendon reflexes present in the upper limbs, but not evoked in the lower limbs. No sensitive deficit or focal neurologic sign was recognizable.Non-contrast multislice computed tomography (MSCT) of the head was performed in the emergency department, showing diffuse periventricular white matter and thalamic mild hyperdensity.Lumbar puncture, blood tests, including serology for HIV and other infections, were negative.On the third day the patient, showing decreased consciousness, underwent magnetic resonance imaging (MRI) with contrast medium injection. MRI revealed the presence of multiple pseudonodular avidly enhancing lesions, supra and infratentorial, crossing the midline, involving the ventricular system, including the fourth ventricle, with extension into the surrounding white matter, the corpus callosum, the thalamus and the hypothamalus.A stereotactic biopsy led to a diagnosis of diffuse large B-cell lymphoma, primarily located in the central nervous system (PCNSL).After the completion of the first phase of treatment (immunotherapy with intravenous Rituximab and corticosteroid), the MRI showed a marked regression of tumor masses.

Neuroimaging of plasmacytoma. A pictorial review.

This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to neuroradiology. Plasmacytoma is considered a counterpart of multiple myeloma that is described as a solitary and discrete mass of monoclonal neoplastic plasma cells. It may arise from osseous (medullary) or extramedullary sites. Isolated extramedullary plasmacytoma is very rare and comprises less than 4% of all plasma cellular diseases of which more than 80% are localized to the submucosal lymphoid tissue of the nasopharynx, nasal cavity and paranasal sinuses. We will demonstrate imaging findings in ten histopathologically proven plasmacytomas in different locations related to neuroradiology. Extramedullary and osseous plasmacytoma show nonspecific CT and MR imaging findings. MR is the preferred modality for evaluation due to better soft tissue contrast. Features that may suggest the diagnosis of plasmacytoma are bulky soft tissue mass and relatively isointense signal on T2-weighted MR images due to high cellularity.

Lhermitte-Duclos disease. A case report.

Lhermitte-Duclos disease is a rare pathologic condition consisting of a dysplastic gangliocytoma of the cerebellum. Its association with phacomatosis and an autosomal dominant neoplastic syndrome, Cowden's syndrome is also known. Modern neuroimaging contributes to a correct diagnosis and pre- and postoperative evaluation. Here we describe the morphologic and metabolic aspects of the disease as shown by conventional MRI, diffusion imaging and spectroscopy in a 31-year-old woman. In addition, the specific neuroradiologic characteristics are presented and discussed in the light of the main pathologic and clinical features, such as hypertrophy of the cerebellar folia associated with white matter atrophy.

Striatal necrosis in type 1 glutaric aciduria: Different stages in two siblings.

Two siblings born of a consanguineous marriage with history of neurologic deterioration were imaged. Imaging features are classical of glutaric aciduria type 1 (GA-1), acute (striatal necrosis) stage in younger sibling, and chronic stage in older sibling. GA-1 is an autosomal recessive disease with typical imaging features. Greater awareness about this condition among clinicians and radiologists is essential for early diagnosis and prevention of its catastrophic consequences. Striatal necrosis with stroke-like signal intensity on imaging correlates with clinical stage of patients.