Clin Neuropathol
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Down Syndrome (DS) is the most common of the chromosomal disorders and manifests abnormalities in several organ systems. While mental retardation, skull and brain anomalies, and the development of Alzheimer-type neuropathological changes in patients greater than age 40 years are well recognized by neurologists and neuropathologists, less appreciated are the various cervical spine abnormalities that can occur. ⋯ We present a case of 49-year-old DS patient whose triplegia, subacute progressive respiratory failure, and death could be attributed to severe degenerative joint disease of the cervical spine with osteophyte formation and severe spinal canal stenosis. We provide the first detailed correlation study between pre-mortem magnetic resonance imaging (MRI) and extensive autoptic dissection in an adult DS patient with cervical spine abnormalities, as well as a review of the literature.
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The injured brain may be damaged by primary impact, secondary injury from secondary damage due to initiation of destructive inflammatory and biochemical cascades by the primary injury or secondary ischemic injury following secondary insults that initiate or augment these immunological and biochemical cascades. Cerebral ischemia will arise whenever delivery of oxygen and substrates to the brain fall below metabolic needs. Many factors lead to the development of secondary insults to the injured brain during initial resuscitation, transport, surgery, and subsequent intensive care. ⋯ After brain trauma, systemic hypotension, compromised CPP, raised ICP, elevated temperature, hypoxemia, and jugular bulb venous desaturation are associated with poor prognosis. Clinical trials of moderate hypothermia following brain trauma are ongoing. Following adult brain trauma maintenance of CPP above at least 65 mmHg (probably > 40 mmHg in children below 8 years) seems important to improve outcome indicating the need for continuous ICP monitoring during intensive care of brain-injured patients.
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Creutzfeldt-Jakob disease (CJD) is a transmissible neurodegenerative disorder characterized by the accumulation of proteinase-resistant prion protein (PrP) in the brain. Pathological changes in the cerebellum are common and include atrophy of the granular layer, spongiform change in the molecular layer, and astrocytic gliosis of the cerebellar cortex and white matter. In most cases of sporadic CJD immunohistochemistry for PrP shows widespread granular deposits of the scrapie isoform of the prion protein (PrPSc) in the cerebellar cortex. ⋯ No significant difference in allele frequency between the 2 groups was found. Plaques or plaque-like PrPSc deposits were found in 9 cases of CJD and were associated with the presence of valine at codon 129 on at least 1 allele of PRNP. CJD patients homozygous for valine (V/V) were on an average more than 5 years younger than patients with M/M or M/V at codon 129.
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Cerebral amyloid angiopathy accounts for approximately 10% of spontaneous intracerebral hemorrhage, typically in the cortex and subcortical white matter. Its contribution to primary pontine hemorrhage is not known. The present study was designed to determine if amyloid angiopathy occurs in the pons and whether primary pontine hemorrhages are associated with amyloid infiltration of nearby vessels. ⋯ A congo red stain and an A4 immunohistochemical technique were used. Only 1 patient from group A and none from group B had amyloid angiopathy in the pons. It is concluded that pontine angiopathy is rare and an exceptional cause of primary pontine hemorrhage.
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Two completely intradural extraosseous tumors with the histologic appearance of chordoma or ecchordosis physaliphora caused symptoms and, in 1 case, ultimately the death of a 44-year-old man. The literature contains 5 similar cases, 2 of which were associated with intratumoral and subarachnoid hemorrhage. In view of the circumscribed nature of such tumors and their lack of osseous involvement, they lend themselves to surgical resection. These features may justify distinguishing "intradural chordoma" from classic chordoma.