Angiopathie amyloïde cérébrale ou atteinte des microvaisseaux cérébraux en rapport avec l’HTA : un challenge clinique

Cerebral amyloid angiopathy (CAA) is a entity characterized by degenerative Amyloïd deposits in the walls of the meningeal and cortical vessels. It is considered as the second cause of primitives cerebral hemorrhage in elderly. The differential diagnosis between AAC and hypertension-related cerebral small vessel diseases is difficult and represent a true challenge for the clinician. We report two cases of cerebral small vessel diseases revealed by malignant hypertension.     

Perinatal lung development following maternal exposure to methylmercuric chloride

Mercury ingested from dietary sources has potent neurotoxic and teratogenic effects. Initial studies have shown that mercury may also affect fetal lung development. Since these pulmonary effects may play a role in subsequent neonatal morbidity and mortality due to compromising of the development of the lung, mercury effects in fetal and neonatal lung were investigated. Methylmercuric chloride (MMC), 1,000 ppm (15 mg/kg of body weight), was administered via an intragastric tube to timed-pregnant Swiss/Webster mice on day 9 of gestation. Lungs from fetuses on gestational day 18 and from neonates on days 1, 5, or 10 after birth were studied. Significant changes in MMC-exposed lungs compared to controls occurred at postnatal day 1. At this time, lung weight per gram body weight increased, phospholipid content per gram of lung or per microgram of DNA decreased, while DNA per gram of lung increased. Methylmercury appears to have delayed lung maturation. Cuboidal epithelial cells in alveolar tubules contained conspicuous glycogen deposits, and differentiation of alveolar type II cells was adversely affected. These results suggest that prenatal exposure to methylmercury may be detrimental to lung development, specifically to the initiation of surfactant synthesis, by delaying the normal pattern of maturation of the alveolar type II cells within the lungs. Pediatr Pulmonol. 1994; 17:11–21 . © 1994 Wiley-Liss. Inc.     

Pathogenesis of Mucocutaneous Lesions in Behçet's Disease

Behcet's disease (BD) is characterized by recurrent oral aphthae, skin lesions, eye lesions, and genital ulceration. To determine the pathogenesis of BD, we performed histological and immunohistochemical studies of these mucocutaneous lesions, an assay of neutrophil activity, and HLA typing. Dense dermal or subcutaneous infiltrations of polymorphonuclear cells (PMN) without leukocytoclastic vasculitis were found in 28 of 57 lesions. Immunohistochemically, deposits of C3 on the vessels were found in 12 of 31 lesions. Deposits of immunoglobulin were not found except for one of IgM. C3 deposits and PMN infiltrations were significantly related (p<0.05). PMN activity by polarization was enhanced; however, the results did not show a significant relationship with the PMN infiltrations or the C3 deposits. The incidence of HLA-B51 was significantly high in BD, but no significant relationship was found between HLA-B51 and the results of other examinations. These results suggest that the pathogenesis of BD lesions differs from that of collagen diseases and that C3 deposits on the vessels may play an important role in the development of mucocutaneous lesions where PMN have mainly infiltrated.     

中医综合疗法对假性近视患者黄斑处脉络膜厚度及微血流密度的影响

[目的] 研究中医综合疗法对青少年假性近视患者黄斑处脉络膜厚度及微血流密度的影响,为中医疗法在青少年假性近视的治疗方面提供依据。[方法] 使用SPSS 21.0软件产生随机数字,将51例（102只眼）假性近视青少年患者随机分成对照组和治疗组。对照组患者27例（54只眼）,单纯使用复方托吡卡胺滴眼液点眼治疗;治疗组患者24例（48只眼）,在对照组治疗的基础上配合中药雾化、中药眼贴和耳穴埋籽等中医综合治疗。两组患者疗程均为8周,治疗前后检测两组患者裸眼远视力、电脑验光屈光度数、黄斑中心凹下脉络膜厚度及黄斑区浅层血流密度,并评价两组患者疗效。[结果] 1）治疗组1例因中药雾化过敏导致脱落,最终纳入23例（46只眼）。2）治疗8周后,治疗组总有效率为92.3%,对照组总有效率为75.0%,治疗组疗效优于对照组,差异有统计学意义（P＜0.05）。3）治疗后,治疗组患者平均视力较治疗前提高,差异具有统计学意义（P＜0.05）;对照组患者平均视力较治疗前无明显改善,差异无统计学意义（P＞0.05）。组间比较方面,治疗组治疗后平均视力较对照组有一定提高,差异有统计学意义（P＜0.05）。4）经治疗,治疗组患者平均假性近视度数较治疗前下降,差异有统计学意义（P＜0.05）;对照组患者平均假性近视度数较治疗前略有下降,差异无统计学意义（P＞0.05）。组间比较方面,治疗组治疗后平均假性近视度数较对照组有一定下降,差异有统计学意义（P＜0.05）。5）治疗组患者黄斑中心凹下脉络膜厚度较治疗前增厚,差异有统计学意义（P＜0.05）;对照组黄斑中心凹下脉络膜厚度较治疗前无明显改变,差异无统计学意义（P＞0.05）;组间比较方面,治疗组治疗后黄斑中心凹下脉络膜厚度较对照组增厚,两组比较差异有统计学意义（P＜0.05）。6）治疗后,治疗组黄斑区浅层血流密度较治疗前增加,差异有统计学意义（P＜0.05）;对照组黄斑区浅层血流密度较治疗前增加,但差异无统计学意义（P＞0.05）;组间比较方面,治疗组治疗后黄斑区浅层血流密度较对照组明显增加,两组比较差异有统计学意义（P＜0.05）。[结论] 采用中医综合疗法对青少年假性近视治疗效果较好,并且可以增加黄斑中心凹下脉络膜厚度及黄斑区浅层血流密度,短期观察无不良反应,值得临床推广运用。     

Vascular lesions in testes associated with male infertility in Cameroon

Summary Testicular biopsies in 40 of 41 infertile males with severe oligospermia in Cameroon presented massive subendothelial fibrinoid deposits in the small and medium sized vessels. Fibrinogen, complement and IgM were demonstrated in these deposits by immunofluorescence. Evidence strongly suggestive of parasitic testicular involvement was also observed in 2 cases.It is postulated that the fibrinoid deposits are the result of repeated formation and deposition of circulating immune complexes by reaction of antibodies with antigens. These antigens could be of various origins and in the cases described here they could be derived from living or dying parasites in the region. The accumulation and incorporation of the fibrinoid deposits may lead to vascular stenosis resulting in chronic ischaemia, tubular atrophy and fibrosis, and finally oligospermia.     

Immunotactoid glomerulopathy with fingerprint immune deposits

Summary Immunotactoid glomerulopathy is a distinct clinico-pathological entity which has recently been defined. The term immunotactoid refers to highly organized immune depositions appearing as rod-like microtubular structures in ultrastructural examination. We describe a patient with mixed connective tissue disease who demonstrates characteristic features of immunotactoid glomerulopathy. The diagnosis was made after excluding amyloidosis, cryoglobulinaemia and lupus nephritis. In addition to immunotactoid microtubules, ultrastructural examination also demonstrated presence of fingerprint depositions which were intimately mixed with immunotactoid structures. Fingerprint deposits have been described in lupus nephritis and cryoglobulin-related nephropathy, but rarely in other glomerulonephritis. These unique findings in our patient may suggest a previously unsuspected relationship between the syndrome of immunotactoid glomerulopathy and systemic lupus erythematosus.     

Amyloid deposits in aortic and mitral valves

Summary The material from 100 consecutive aortic and mitral valve operations has been studied histologically with particular reference to the presence of amyloid deposits. Sixty seven per cent were positive (aortic 88%, mitral 45%).The simultaneous occurrence of calcification of the valves and amyloid degeneration as well as of calcification and hyalinization was significant. Similarly there was significantly more amyloid in the older age groups, as well as a significant correlation between the degree of hyalinization of the valve and amyloid.]Thirty-two patients had previously suffered from rheumatic fever. The heart valves of these patients did not differ histologically from the others, whereas significantly more amyloid was observed in the stenotic mitral valves than in the mitral valves which were insufficient.     

Immunohistological study of senile brains by using a monoclonal antibody recognizing β amyloid precursor protein: significance of granular deposits in relation with senile plaques

Summary Immunochemical analyses revealed that a monclonal antibody Am-3 recognized amyloid precursor protein (APP) in senile plaques extracted from Alzheimer's brain, but did not recognize amyloid protein. Immunohistochemically, however, the staining pattern of Am-3 in frozen section of Alzheimer's brain was almost the same with that of rabbit polyclonal antibody to amyloid peptide which could recognize both amyloid protein and APP. In other words, APP was present in senile plaques of various types, cerebrovascular amyloid and granular deposits. The granular deposits were 5–10 m in size and laminarily distributed in the 1st, 3rd and 4th layers of cerebral cortex. They were especially abundant in 1st and 4th layers where senile plaques were usually fewer in number. Although the distribution in the cerebral cortex was different between the senile plaques and the granular deposits, the number of the granular deposits was well correlated with that of senile plaques. The granular deposits were negative in Congo-red birefringence, but contained amyloid protein as well as APP fragment judging from positive staining by both Am-3 and polyclonal antibody to synthetic amyloid peptide. Thus, they could be regarded as pre-amyloid.     

Occurrence of diffuse amyloid deposits in the presubicular parvopyramidal layer in Alzheimer's disease

Summary Silver staining by a modified Bielschowsky's technique and immunostaining for -amyloid protein BAP have revealed the occurrence of diffuse amyloid deposits bilaterally in the presubiculum in each of fourteen Alzheimer's disease cases examined. Observations on serial blocks show these deposits to be localized in the parvopyramidal layer of the presubiculum proper and the transsubiculum. They are also observed in the cellular islands within the molecular layer of the subiculum but not in the parasubiculum. These amyloid deposits are not accompanied by neurofibrillary tangles, neuropil threads, or the aggregated microglial reaction which is characteristically associated with classic senile plaques. Convergence of input from limbic and cortical areas might play a significant role in the formation of these diffuse amyloid deposits.Supported by grants from the MRC of Canada, the Alzheimer's Society of B.C., the American Health Assistance Foundation and the McLean Foundation