Systemic Weber-Christian disease

A patient suffering from Weber-Christian panniculitis was found at necropsy to have similar lesions in the visceral adipose tissue. When the lesions occur internally, diagnosis during life is complicated by the difficulty of examining visceral adipose tissue and the disease may often go unrecognized, but patients suffering from Weber-Christian panniculitis should be investigated bearing in mind the possibility of internal lesions. Only 11 cases confirmed by necropsy appear to have been reported hitherto. The aetiology remains obscure. It is suggested that the name `systemic Weber-Christian disease' be applied when the lesions occur in adipose tissue other than the panniculus adiposus and that the term `Weber-Christian panniculitis' be used when the lesions are confined to the subcutaneous adipose tissue.     

Weber-Christian panniculitis and auto-immune disease: a case report.

A case is described of Weber-Christian panniculitis accompanied by a gammaglobulin disturbance which preceded by five years the diagnosis of an autoimmune hepatitis and pancytopenia. Also associated was the onset of diabetes mellitus, found at necropsy to be related to pancreatic islet amyloid deposition. This case reinforces the view that Weber-Christian panniculitis may be an adipose response to a variety of immunological stimuli.     

Clinico-anatomical analysis of the causes of death in ruptures of intracranial arterial aneurysms

Causes of lethal outcome in patients after subarachnoid hemorrhage due to cerebral aneurysm rupture were analysed. The major death cause in the operated patients was cerebral edema and dislocation, while in non-operated ones it was the penetration of blood into the ventricles of the brain. Cerebral edema and dislocation syndrome were mainly caused after surgery by ischemic changes in cerebral tissue. A relationship between the aneurysm localization and the extent of subarachnoid hemorrhage was revealed as well as between the hematoma localization and incidence of hemorrhage into the ventricles of the brain.     

The neuropathology of acquired immune deficiency syndrome

We reviewed the neuropathologic characteristics of 52 cases of acquired immune deficiency syndrome (AIDS) at autopsy. Histologically significant neuropathologic lesions were found in 38 cases. We believed that infection was the predominant pathologic process in 26 cases; occasionally, multiple infectious agents were present. This included Toxoplasma encephalitis (n = 16), fungal abscess (n = 1), tuberculous abscess (n = 1), progressive multifocal leukoencephalopathy (n = 2), cysticercosis (n = 1), and Escherichia coli meningoencephalitis (n = 1). Microglial nodules or perivascular inflammation suggested that encephalitis was the most likely cause in five cases. In two additional cases, a primary demyelinating process that was apparently related to cytomegalovirus was present. Vascular or hypoxic diseases were present in nine cases. The findings included intracerebral hemorrhage (n = 1), subarachnoid hemorrhage (n = 2), infarction (n = 2), diffuse hypoxic changes (n = 5), cerebral edema (n = 1), and rare thromboemboli with extravasation of RBCs (n = 1). One case of primary lymphoma was observed. The CNS lesions were the proximate cause of death in 15 patients. The CNS complications of AIDS are varied and often are the major manifestation of the syndrome.     

Weber-christian panniculitis with systemic cytophagic histiocytosis

Summary A 49-year-old woman suffered from Weber-Christian panniculitis with a typically periodic course. Subsequently, the attacks of the disease developed to a severe state: high fever, endotoxinemia, pancytopenia, and clotting disorder, in addition to the cutaneous manifestations. One and a half years after the outbreak of the disease the patient died in a septic shock. At autopsy all three stages of Weber-Christian panniculitis were found. In addition, an immense proliferation of benign cytophagic histiocytes could be observed in the bone marrow, spleen, lymph nodes and, less distinctly, in the fatty and interstitial tissues of the visceral organs. As a nosologic entity, the Weber-Christian disease is frequently questioned. Some authors consider the lobular, histiocytic, cytophagic panniculitis a unique syndrome. On the other hand, it must be emphasized that benign, cytophagic histiocytosis may exist associated with infections or may accompany different diseases. Furthermore, the Weber-Christian panniculitis is clinically and morphologically well defined.Abbreviations PAP method peroxydase-anti-peroxydase method     

Pathology of inverted Takotsubo cardiomyopathy

Myocardial dysfunction without coronary involvement may occur in acute cerebral diseases. The inverted Takotsubo pattern has been recently recognized as a novel heart neurologic stress-related syndrome. We report on the case of a 40-year-old woman presenting with massive subarachnoid hemorrhage and brain death. Echocardiography revealed an extensive left ventricular circumferential akinesis except at the apex. Histologic analysis of the heart confirmed the absence of myocardial infarction and revealed only sparse foci of myocyte necrosis with contraction bands in the akinetic areas.     

Weber-Christian panniculitis with immune complex glomerulonephritis

The etiology of Weber-Christian panniculitis is unknown. A case is presented in which an association is found between immune complex glomerulonephritis and a relapse of Weber-Christian panniculitis. This case adds to the literature, implicating immune system dysfunction in the pathophysiology of this disease.     

Lethal vertebral artery dissection in pregnancy: a case report and review of the literature

Subarachnoid hemorrhage represents a rare event in pregnancy with a high mortality rate. We present the case of a 39-year-old pregnant woman who developed right vertebral artery dissection with subsequent massive subarachnoid hemorrhage with fatal outcome. The macroscopic and microscopic autopsy findings are described. A review of the literature with a discussion of the varied predisposing factors for vertebral artery dissection and subarachnoid hemorrhage and the rarity of these events in pregnancy is provided.     

February 2001: A 74 year old man with a history over 3 months of increasing dyspnea and malaise.

The February COM: A man of 78 years with idiopathic late-onset cerebellar ataxia developed renal failure in association with a high ESR and positive pANCA. This was complicated by a subclinical spinal subarachnoid hemorrhage which was related to necrotizing inflammation of small leptomeningeal vessels. Renal cortical infarcts were due to similar inflammation in arcuate and interlobular arteries. Spinal subarachnoid hemorrhage is rare and usually due to rupture of an arteriovenous malformation. However, an immunogenic connective tissue disorder should be considered in the differential diagnosis. In this case, the histology and results of an autoantibody screen support a diagnosis of microscopic polyangiitis.     

Extracorporeal Membrane Oxygenation for Acute Life-Threatening Neurogenic Pulmonary Edema following Rupture of an Intracranial Aneurysm

Neurogenic pulmonary edema (NPE) leading to cardiopulmonary dysfunction is a potentially life-threatening complication in patients with central nervous system lesions. This case report describes a 28-yr woman with life-threatening fulminant NPE, which was refractory to conventional respiratory treatment, following the rupture of an aneurysm. She was treated successfully with extracorporeal membrane oxygenation (ECMO), although ECMO therapy is generally contraindicated in neurological injuries such as brain trauma and diseases that are likely to require surgical intervention. The success of this treatment suggests that ECMO therapy should not be withheld from patients with life-threatening fulminant NPE after subarachnoid hemorrhage.     

Type IV Ehlers-Danlos syndrome presenting as sudden infant death

A previously healthy 5-month-old female infant presented with sudden death due to spontaneous subarachnoid hemorrhage associated with minor multifocal visceral hemorrhages. The clinical diagnosis had been sudden infant death syndrome. Although the family history was noncontributory and other features of type IV Ehlers-Danlos syndrome (EDS) were absent, the pattern of hemorrhage was consistent with this type of connective tissue disorder. The diagnosis was confirmed after postmortem analysis of skin and aorta showed less than 5% type III collagen (normal greater than 15%). Extensive literature review failed to find any other reported cases of sudden death in infancy due to intracranial hemorrhage in patients with previously unsuspected type IV EDS. The authors suggest that collagen analysis should be performed in cases of unexplained multifocal spontaneous hemorrhage in infancy so that this rare diagnosis will not be missed.     

Dissecting aneurysms involving both anterior cerebral artery and aorta

Non-traumatic intracranial dissecting aneurysm (IDA) has been recently reported with increasing frequency and is recognized as a possible cause of subarachnoid hemorrhage. However, the pathogenesis of this disease is still unclear. Cystic medial necrosis (CMN) is known to be a cause of aortic dissection, especially in Marfan's syndrome. Presented herein is the case of a 49-year-old man who had IDA of the right anterior cerebral artery and abdominal aortic dissection without Marfan's syndrome. Histological examination showed medial degenerative changes with the accumulation of acid mucopolysaccharides in various intra- and extracranial arteries. Coexistence of dissecting aneurysms in the anterior cerebral artery and aorta suggests the presence of underlying pathogenesis that is common to these two dissection processes.     

Upper gastrointestinal bleeding in patients with liver cirrhosis

Patients with liver cirrhosis may develop upper gastrointestinal hemorrhage from a variety of lesions, which include those that arise by virtue of portal hypertension, namely gastroesophageal varices and portal hypertensive gastropathy and other lesions seen in the general population. Do patients with liver cirrhosis, hemorrhage from varices and other lesions equally, or are they more likely to bleed from varices? The aim of this study is to determine predominant causes of bleeding in patients with liver cirrhosis and upper gastrointestinal bleeding. PATIENTS AND METHODS: A retrospective review of 40 patients with liver cirrhosis based on the clinical and biochemical parameters of the Child-Pugh score, and upper gastrointestinal bleeding was carried out at an inner city hospital. Endoscopy diagnoses were documented. RESULTS: Of 40 patients, 38 patients had cirrhosis associated with alcohol consumption. Twelve of the above 38 patients who consumed alcohol also had hepatitis C virus (HCV) infection. Eleven patients had only varices on endoscopic examination, 17 had varices plus coexisting lesions. From these 17 patients, nine were found to have bled from varices, and eight were found to have bled from coexisting lesions. Twelve patients who had no varices bled from other lesions. Of 40 patients, 28 had varices, and 20 actually bled from varices. In this study there was no correlation between severity of liver cirrhosis as determined by the Child-Pugh score and the absence or presence of varices. CONCLUSION: Patients with liver cirrhosis and upper gastrointestinal bleeding hemorrhage from a variety of lesions. In this study of 40 patients, (70%) had gastroesophageal varices diagnosed at upper endoscopy, while 50% actually bled from varices.     

Marked calvarial thickening and dural changes following chronic ventricular shunting for shaken baby syndrome

We report the case of a 23-year-old woman who was abused at the age of 5 months. She suffered from complications frequently associated with shaken baby syndrome, such as hydrocephalus secondary to subarachnoid hemorrhage. The patient underwent a procedure to place a ventriculoperitoneal shunt 3 weeks after her presentation with signs of abuse. The ventricular shunt remained in place throughout her life, and the patient received multiple revisions. She also was noted to have a markedly thickened calvarium on both radiographs and computed tomographic scan at 6 years old. She died following an episode of grand mal status epilepticus. An autopsy was performed and her skull was found to be thickened circumferentially. Histologic examination revealed increased cancellous space with normal trabecular bone. It is hypothesized that intracranial hypotension resulting from chronic ventricular shunting lead to her thickened calvarium, a condition previously reported as hyperostosis cranii ex vacuo. Dural changes seen microscopically corroborate this hypothesis.     

Hemoglobin quantitation in subarachnoid hemorrhage

Summary The amount of hemoglobin in cerebrospinal fluid was quantitated in 15 patients with subarachnoid hemorrhage and in 35 subjects without subarachnoid hemorrhage (controls). The photometric determination of the hemoglobin content in cerebrospinal fluid indicated that patients with subarachnoid hemorrhage in contrast to controls showed a significant increase (P>0.01) of hemoglobin (Mann-Whitney test). In 5 of the patients with subarachnoid hemorrhage it was possible to determine the amount of hemoglobin in cerebrospinal fluid over a period of time. Possible correlations between clinical course and hemoglobin concentration as well as the use of hemoglobin quantitation in late diagnosis of subarachnoid hemorrhage are discussed.Abbreviations CSF Cerebrospinal fluid - Hb Hemoglobin - SAH Subarachnoid hemorrhage     

Granulomatous angiopanniculitis of the breast

The clinical and pathologic features of six examples of nonnecrotizing granulomatous angiopanniculitis (GAP) of the breast are reported. The patients presented with a solitary ill-defined breast mass causing clinical suspicion of carcinoma. Histopathologically, all lesions consisted of multiple nonnecrotic, noncaseous granulomas with a giant cell component predominantly involving the subcutaneous adipose tissue, extending into the underlying mammary tissue without affecting lobules or ducts. A nonleukocytoclastic lymphocytic angiitis involved small vessels and capillaries. None of the patients had a history of an autoimmune disorder or had previous diagnoses of erythema nodosum or multiforme, leukocytoclastic or nonleukocytoclastic vasculitis, or Weber-Christian disease. Treatment was limited to biopsy in all six patients. Studies for infectious agents on specimens were negative. Five of the six patients developed one or more recurrences in the breast or elsewhere on the body. Four patients experienced spontaneous regression of their recurrent lesions. GAP appears to be a self-limited disorder of uncertain etiology which involves the breast and other sites. It may represent a variant of Weber-Christian disease, as the two diseases share similar clinical and histologic features. GAP must be distinguished from causes of granulomatous inflammation of the breast for which specific medical therapy is available.     

小胶质细胞在蛛网膜下腔出血后脑损伤中作用机制的研究进展

小胶质细胞是位于中枢的单核巨噬细胞系统成员,对维持中枢神经系统的稳态有重要意义。蛛网膜下腔出血（SAH）是脑卒中的一种亚型,其发生后会造成72 h内早期脑损伤,以及SAH后3～15 d迟发性脑损伤。以往认为,血管痉挛在SAH后脑损伤中发挥重要作用,但脑损伤中的炎症反应近年逐渐受到重视,其中小胶质细胞可在SAH后早期和延迟相脑损伤的炎症反应中向不同方向极化,既可发挥促炎作用,也可产生神经保护作用。我们综述近几年小胶质细胞在SAH后脑损伤中发挥的作用及相关治疗靶点的研究进展及相关热点,为临床处理该类疾病提供潜在治疗靶点。     

Aneurysms of coronary arteries in a patient with adult polycystic kidney disease: arteriosclerosis or involvement by the primary disease?

Summary Adult polycystic kidney disease is frequently associated with gastrointestinal and cardiovascular abnormalities. These include hypertension, mitral valve prolapse, mild dilation of the aortic root, abdominal aneurysms, and predisposition to aortic, mitral, and tricuspidal valve regurgitation reminiscent of Marfan's syndrome. Although tho exact molecular mechanisms of adult polycystic kidney disease are not well established, a generalized defect of collagen structure is hypothesized. The most severe vascular problems, however, are typical intracranial aneurysms with a high incidence of subarachnoid hemorrhage and a high mortality rate. We report a case of dilated coronary arteries found incidentally in a patient with adult polycystic kidney disease and stress-induced angina pectoris. The typical angina pectoris of the patient is explained by left ventricular hypertrophy and coronary heart disease. Multiple liver cysts, mitral valve prolapse, and the coronary aneurysms in this patient with adult polycystic kidney disease appear to reflect the manifestation of a generalized connective tissue disorder in this syndrome.Abbreviation APKD adult polycystic kidney disease Dedicated to Prof. Dr. N. Zöllner on the occasion of his 70th birthday     

A 38-year old woman with a dural based lesion

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare.We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38-year-old female. Xanthogranulomas are benign non-Langerhans cell histiocytic lesions. They are frequently described in the skin of infants and children. Extracutaneous manifestations especially in the central nervous system are highly uncommon. Dural xanthogranulomas usually arise in association with familial hypercholesterolemia, with Erdheim Chester disease (ECD), and with Weber-Christian disease. Our case however, had no such associations. In this report, the authors describe the clinical, radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma.     

Preeclampsia of pregnant women complicated by HELLP syndrome

One case of a Hell syndrome in a female of 22 with gestational eclampsism is described. The syndrome seemed to develop 2 weeks before hospitalization and manifested with brain hemorrhage followed by obstruction of liquor spaces. Morphologically, HELL syndrome was characterized by multilobular liver necrosis, hemoglobinurea nephrosis and pronounced hemorrhagic diathesis.