Intracerebral vascular occlusion in familial erythrophagocytic lymphohistiocytosis: a case report of two siblings

Neuropathological findings in two siblings with familial erythrophagocytic lymphohistiocytosis (FEL) are reported. Case 1 showed the typical neuropathological findings of FEL with lymphohistiocytic infiltration of the leptomeninges and perivascular spaces. A characteristic erythrophagocytosis was detected in inguinal lymph nodes, lung and bone marrow. Case 2 revealed calcification and necrotic lesions in the brain. In the necrotic areas, parenchymal calcification, vascular medial calcification, and occlusion of many vessels due to subendothelial fibrosis were detected. The areas of necrosis correlated with the distribution of occluded vessels. These changes were most prominent in putamen, internal capsule, thalamus and dentate nucleus. Hypercytokinemia is suspected to be the underlying mechanism for the clinical and laboratory findings in patients with FEL, although the relationship to the vascular pathology is unclear.     

Kinesthetic sense of the shoulder in patients with impingement syndrome

A proprioceptive deficit is an important determinant of disability in various shoulder disorders, such as instability and osteoarthrosis. In 15 patients with impingement syndrome stage II (Neer 1983), who were treated by arthroscopic subacromial decompression, we measured movement sense by determining threshold levels for the perception of motion of the shoulder. The patients were placed in a specially designed chair allowing continuous passive motion of the shoulder joint, while avoiding cutaneous, auditory and visual stimuli. To assess movement detection thresholds, passive abduction movements of the shoulder were performed at a starting angle of 60°, an amplitude of 10° and an angular velocity of 1.3°/s. Before surgery, all patients had higher threshold levels for the perception of motion in their affected shoulders then in the other side. After decompression, proprioception had improved on the decompressed side, but was unchanged on the other side.     

Diffuse prolonged depression of cerebral oxidative metabolism following concussive brain injury in the rat: a cytochrome oxidase histochemistry study.

Utilizing a lateral fluid percussion injury as a model of cerebral concussion, rats were studied histochemically measuring the degree of cytochrome oxidase activity present within different structures at different times following injury. After concussion, the cerebral cortex ipsilateral to the site of injury exhibited a diffuse decrease in its level of chromotome oxidase (CO) activity beginning at as soon as one day and lasting for up to 10 days after the insult. The ipsilateral dorsal hippocampus also exhibited an injury-induced decrease in CO activity, however, it was not as severe as in the cortex. These results indicate that oxidative metabolism is depressed primarily within the cerebral cortex and hippocampus for several days following a cerebral concussion. We propose that this period of metabolic depression may delineate a period of time during which the injured brain is unable to function normally and thus would be vulnerable to a second insult.     

Cerebriform intradermal naevus (a rare form of secondary cutis verticis gyrata)

BACKGROUND: Acquired cerebriform intradermal naevus (CIN) is a rare form of pseudo cutis verticis gyrata. CASE REPORT: A case of acquired CIN of the scalp in a 46-year-old male patient is presented. The clinical and histopathological presentations of CIN are described and the therapeutic possibilities are discussed. CONCLUSIONS: In each individual case the physician must decide whether to do surgery or follow a wait-and-see policy. There is little risk of malignant change of CIN, and surgical excision of such lesions often involves evident mutilation.     

Neuropsychological, psychosocial and vocational correlates of the Glasgow Outcome Scale at 6 months post injury: a study of moderate to severe traumatic brain injury patients

Traumatic brain injury (TBI) subjects at Glasgow Outcome Scale levels 3 (severe disability), 4 (moderate disability), 5 (good recovery), and an other injury control group (OIC) were compared in terms of neuropsychological, psychosocial, and vocational functioning 6 months after injury. Subjects were a sample of 100 patients with a moderate to severe traumatic brain injury (TBI) and a matched sample of 30 other injury control subjects (OIC) enrolled in the UCLA Brain Injury Research Center study of TBI outcome. Overall, the results showed a systematic decrease in mean neuropsychological test performance as a function of increasing GOS severity, as well as an increased prevalence of symptoms of depression and lower ratings on measures assessing employability and capacity for self care. TBI patients in the 'severe' and 'moderate disability' groups were distinctly inferior to the 'good recovery' and 'OIC' groups, who were quite similar to each other in terms of cognitive, psychosocial, and vocational outcomes. The results demonstrate overall support for the predictive and concurrent validity of the GOS 6 months post injury. Despite these results, which strengthen the utility and appeal of the GOS for multicentre studies, concerns still remain regarding GOS category 4 (moderate disability), which was shown to lack sufficient discriminability in this study.     

Endotracheal suctioning causes right upper lobe collapse in intubated children

Objective: Right upper lobe collapse is a common radiographic finding in intubated children. We hypothesized that deep suctioning and uncontrolled negative pressures during endotracheal tube suctioning were significant contributory factors. Methods : The incidence of right upper lobe (RUL) collapse in intubated, ventilated children on a paediatric cardiac intensive care unit was determined over a 3-month period ( n = 102). Graduated suction catheters and suction vacuums of < 165 cm H₂O were then introduced. Another prospective audit was carried out 3 months later ( n = 60). Results : We found that 24% developed RUL collapse and 4 developed an apical pneumothorax. Following the introduction of graduated catheters and controlled vacuums pressures, a significant reduction in the incidence of RUL collapse, to 7%, was observed ( p < 0.05). Conclusions : We conclude that high negative pressure and deep-suctioning causes RUL collapse in children. Any lobar collapse not only prolongs the child's stay in intensive care, but can be associated with further morbidity which may have a serious implication. By improving suctioning technique this morbidity can be significantly reduced.     

Cardiomyopathies with particular reference to the diagnostic relevance of endomyocardial biopsies

A distinction is made between heart muscle diseases with a known cause, or occurring as part of a systemic disease, and those of unknown cause. The first category is termed "specific heart muscle disease". The term "cardiomyopathy" is then limited to the latter category. Cardiomyopathies are categorized as dilated cardiomyopathy, hypertrophic cardiomyopathy and restrictive cardiomyopathy. The pathology of dilated cardiomyopathy depends on the clinical stage of the disease. Basically, the histology shows hypertrophic myocytes, often with degenerative signs, and interstitial fibrosis. Lymphocytes may be observed, particularly in cases studied shortly after the onset of symptoms. Endocardial thickening may occur in time. The role of endomyocardial biopsies in the clinical setting is limited and relates mainly to excluding other diseases, such as myocarditis. The pathology of hypertrophic cardiomyopathy is characterized by myocardial wall thickening, either asymmetric or symmetric, and disorganization of the normal myocardial texture. The latter phenomenon should be distinguished from disarray, since it may occur as a natural phenomenon. The differentiation between these two may be extremely difficult on the basis of only small tissue samples, as with endomyocardial biopsies. This, therefore, poses serious limitations in the use of endomyocardial biopsies. The pathology of restrictive cardiomyopathy also depends on the stage of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)