Motor neuron disease (MND) can be associated with frontotemporal dementia (FTD) and circumscribed lobar atrophy of frontal and temporal lobes. Neuropathological correlations were sought in postmortem studies from a woman with these associated conditions. Cerebral blood flow imaging and autopsy showed cortical abnormalities in the frontal and temporal lobes, but typical argyrophilic Pick's bodies were absent and ubiquitin-positive neuronal cytoplasmic inclusions were rare. However, hematoxylin and eosin staining revealed eosinophilic, granular, central cytoplasmic 'clearing' in most neurons of the substantia nigra and in cervical anterior horn cells. Electron microscopy showed that these neurons contained a central cytoplasmic zone cleared of neuromelanin and normal cytoplasmic organelles containing small mitochondria with matrix inclusions and randomly oriented filamentous material. A mitochondrial dysfunction or defective transport of mitochondria into axonal processes needs to be studied as a potential cause for the coassociation of MND and FTD.

Atrophy↗

PubMedDate not supplied

A case of relapsing polychondritis associated with hemolytic anemia.

Relapsing Polychondritis (RPC) is a rare disorder of unknown etiology which affects mucopolysaccharide-rich tissues such as cartilage. A 64-year-old man developed auricular and nasal chondritis with complaints of arthralgia of the hands and cervical pain. The auricular biopsy established the diagnosis of RPC. The hematological data revealed normocytic, slightly hypochromic anemia, a persistently elevated reticulocyte count, slightly increased bilirubin, and decreased haptoglobin. The presence of hemolytic anemia was confirmed by the shortened half-life of erythrocytes and erythroid hyperplasia of the bone marrow. This case illustrates the coexistence of RPC and hemolytic anemia which has been only rarely reported. The pathogenesis of RPC is also discussed may elucidate the pathogenesis of this disease.

Anemia, Hemolytic, Autoimmune↗

PubMedDate not supplied

Thalamic extrapontine lesions in central pontine myelinolysis.

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Adult↗

PubMedDate not supplied

Adult cerebellar medulloblastoma: imaging features with emphasis on MR findings.

PURPOSE: To describe the MR imaging features of cerebellar medulloblastoma in the adult. MATERIALS AND METHODS: The neuroimages and records of 15 adults with proved cerebellar medulloblastoma were retrospectively evaluated. In 12 patients, preoperative MR scans were reviewed; nine had Gd-DTPA-enhanced scans. RESULTS: Of the 12 tumors evaluated preoperatively, eight were hemispheric, two hemispheric-vermian, and two vermian. Tumor margins were well demarcated, except in three cases, two of which had large infiltrative tumors. In 10 cases, tumor extended to the brain surface, and in five of these, contiguity with the tentorium or cerebellopontine angle cistern was such than an extraaxial tumor was considered. The tumors were typically hypointense on T1 but a spectrum was seen on T2-weighted images. Enhancement ranged from minimal and patchy to marked. One tumor became isointense after Gd-DTPA. Other features included cystic changes, hemorrhage, exophytic invasion at the cerebellopontine angle, spinal cerebrospinal fluid seeding, intraventricular seeding, and bone metastasis. CONCLUSION: Although there is no pathognomonic MR appearance of adult cerebellar medulloblastoma, the finding of a well-demarcated, mild to moderately enhancing hemispheric mass involving the brain surface in a young adult is suggestive of medulloblastoma. Awareness that this tumor may resemble meningioma may avoid misdiagnosis and aid surgical planning.

Adolescent↗