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A family genealogy, comprising four cases of familial multiple lipomatosis, making their appearance one after the other in three generations is reported. Two cases with impressive clinical features are presented in detail. This report contributes to the opinion that the disease is transmitted by the autosomal dominant route of inheritance.
Adenylate cyclase activity and the number of alpha- and beta-adrenergic receptors (studied by radioligand binding techniques) were compared to adipocyte membranes prepared from lipomatous and normal adjacent adipose tissue of a patient with multiple symmetric lipomatosis. No difference could be detected between the normal and lipomatous adipose biopsies. These data strongly suggest that the impairement of catecholamine-induced lipolysis often reported in lipomas is related to a defect in the enzymatic steps localized beyond rather than before cyclic AMP synthesis.
The authors report three cases of abdominal lipomatosis, presenting as an abdominal mass. The problems of diagnosis caused by this rare entity are discussed. The value of the CT scan is emphasized. Indeed, only this examination is able to give all the informations necessary for the diagnosis. Ultrasonography seems to be particularly useful in the assessment of the evolution of this disease, especially for the study of the urinary complications. Before apparition of these complications, no treatment is required.
Pelvic lipomatosis is characterised by an excessive amount of adipose tissue in the pelvis. Although there are usually few clinical symptoms, the radiological changes are characteristic: pear-shaped bladder, straightened and displaced rectosigmoid junction. However, these changes are not specific and some workers advise histological confirmation with or without surgical exploration. The authors describe a case illustrating the diagnostic value of computerised axial tomography, as has previously been reported. This investigation seems to give reliable results in the diagnosis of pelvic tumours, especially those of adipose tissue.
We report on clinical and metabolic studies of a newly delineated lipomatosis, characterised by an abnormal mediastinal and abdominal accumulation of fat, without obesity. The clinical features, which occurred in all the patients studied, are: Exertional dyspnoea due to a space-occupying mediastinal accumulation of fat, without evidence of cardiac or pulmonary disease. A pseudo-ascitic abdominal enlargement, due to intra- and retroperitoneal accumulation of fatty tissue. Insulin-independent diabetes mellitus. Type IV hyperlipidaemia and elevated levels of plasma uric acid were observed in four patients. Intra-abdominal lipomatous tissue, obtained during laparoscopy from four patients, demonstrated a reduced lipolytic response to beta-adrenergic stimulation. Thus, fat deposition in the abdominal and mediastinal areas could be causally related to defective lipid mobilization in lipomatocytes. Lipoprotein lipase activity in abdominal adipose tissue were normal in two patients (10.0 and 10.6 nmol/g/min) and markedly elevated in another two patients (37.3 and 49.9 nmol/g/min), as compared with controls (12.7 +/- 2.1 nmol/g/min). When expressed on per cell basis, LPL activity in lipomatous tissue was significantly higher than in control tissue (3.21 +/- 1.1 nmol/10(5) cell/min vs 0.92 +/- 0.16 nmol/10(5) cell/min). Lipoprotein fractionation did not demonstrate consistent modification of the serum lipoprotein pattern. HDL and HDL2 cholesterol values were reduced, even in patients with elevated LPL activity in adipose tissue.
Pelvic lipomatosis is a rare disease characterized by an abnormal accumulation of adipose tissues around the pelvic organs. The symptoms are vague and non-specific. The diagnosis is facilitated by typical radiological features. CT scanning is extremely useful and typical. The course of the disease is usually benign in older men, but may be dangerous in the young; it may require diversion.
Two patients with encephalocraniocutaneous lipomatosis have been studied. The main features of the syndrome are unilateral cutaneous and ophthalmologic lesions with ipsilateral cerebral malformations. Seizures beginning in infancy and varying degrees of mental retardation are present in affected patients. The involved tissues are derived from the embryonic ectodermal and mesodermal layers.
Case of unusual diffuse segmental lipomatosis of a part of the small bowel is reported. Other cases from the literature are compared to various pathological deposits of fat in the intestine. Aetiology, clinical signs, diagnosis and differential diagnosis, as well as surgical treatment, are discussed.
Two cases of segmental, small-intestinal lipomatosis are described. In both, the patients had crampy abdominal pain secondary to partial intestinal obstruction. A review of the literature uncovered reports of 16 similar cases, in some of which virtually the entire small intestine was involved. Diverticulosis and intussusception are associated lesions. Diagnosis may be made by an abnormal roentgenographic pattern. Provided the bowel is not so extensively involved that complete resection is impractical, surgical removal of the affected segment results in cure.
Lipoprotein lipase activity in lipomatous tissue, post-heparin lipoprotein lipase activity in plasma, and the composition and concentration of serum lipoproteins were studied in 15 patients with Multiple Symmetric Lipomatosis (MSL). Extremely elevated lipoprotein lipase activity in adipose tissue was found in MSL patients. Total and hepatic post-heparin plasma lipolytic activity was normal, while a moderate but statistically significant increase of extrahepatic lipolytic activity was present. An abnormal composition of serum lipoproteins, characterized by a significant increase in high density lipoproteins, namely HDL2 subfraction, and apoprotein A-I, was demonstrated. A concomitant decrease in and abnormal composition of low density lipoproteins were found. This lipoprotein pattern is consistent with a newly recognized type of hyperalphalipoproteinemia. Significant correlations were found between serum HDL2 cholesterol values and lipoprotein lipase activity in adipose tissue (as well as between serum VLDL-triglyceride and HDL2 cholesterol values). These observations confirm the role of adipose tissue lipoprotein lipase in triglyceride-rich lipoprotein catabolism. The elevated levels of lipoprotein lipase activity in adipose tissue, in addition to a previously demonstrated decrease in adrenergic-stimulated lipid mobilization, could account for both the abnormal fat accumulation in lipomatous fat cell and for hyperalphalipoproteinemia in MSL patients. The occurrence of MSL in two brothers suggests an inherited enzymatic defect, indicating MSL as a "triglyceride storage disease in adipose tissue".
It is reported on a 44-year-old patient who has a peculiar form of the disturbance of the distribution of fatty tissue. Apart from clear superficial hypertrophies at upper arms, shoulders, thorax and hypogastrium as well as plum-sized nodes at the left femor and retroauricularly discrete atrophies are found in the face, at forearms and lower legs. In very inconspicuous paraclinical findings special forms of the progressive lipodystrophy and of the indolent symmetrical lipomatosis are discussed.
The case of a 57-year-old woman is described with a two months history of proximal muscle weakness and pain, marked hypotrophy and brisk reflexes. Clinical investigation demonstrated normal serum CK, myopathic EMG and osteomalacia. Muscle biopsy showed type II fibre atrophy and mitochondrial alterations without inclusions. Further examinations including a jejunal biopsy revealed malabsorption accounting for osteomalacia. At autopsy diffuse nodular lipomatosis of the small bowel was detected (Acta neurol. belg., 1982, 82, 65-71).
The authors report the ultrasonographic findings in a case of pelvic lipomatosis presenting with pathognomonic radiographic features. Ultrasound alone may exclude other lesions but cannot make a specific diagnosis because the sonographic appearance of fat is often conflicting. Ultrasonography should be of value as a follow-up examination in patients with definite risk of progressive ureteral obstruction.
Case report on a case of pelvic lipomatosis, the 21. case published in the world literature. The typical radiographic findings, such as deformity of the urinary bladder and the abnormal course of the sigma, were seen. Computed tomography confirms the diagnosis.
The authors report 3 observations of cervico-facial lipomatosis with mediastinal localization. In the 3 cases the rather unusual localization of Launois and Bensaude's disease produced respiratory troubles which worsened a chronic respiratory insufficiency due to another cause. Once a tracheotomy had to be done because of tracheo-malacia. Another time there was a pharynged localization. This disease etiology always remains obscure, usually appearing in alcoholic addicts of about 50. Mediastinal localizations have been unfrequently described but must be searched for systematically. The evolution is chronic, without specific treatment. If there is already a chronic respiratory insufficiency in relation with a chronic obstructive bronchopneumopathy or a cardiac insufficiency, then the mediastinal localization becomes an aggravating factor.
The relatively rare disease of symmetrical lipomatosis is described on the basic of six own observations. For many years five patients worked with asphalt and one was a coal-miner. Because of this professional exposition oil and cole products are considered to be reason for this disease. The still unknown aetiology and pathogenesis are discussed and finally the surgical therapy is presented.
The cases of two female patients with lipomatosis of the ileocecal valve inducing episodes of intestinal obstruction are presented. A barium enema with air contrast was performed in patient 1; patient 2 was operated on to treat ileus. Resection of the fatty tissue from the ileocecal valve was performed at operation, after histologic diagnosis on frozen section. These cases suggest that limited resection removing fatty tissue is effective and preferable to more radical resection.
We describe an unusual case of multiple symmetric lipomatosis (MSL) that presented with severe polyneuropathy, and review the literature. To the best of our knowledge, this is the first description of polyneuropathy as the presenting sign of MSL and the first report of this syndrome in Israel. MSL has to be included in the differential diagnosis of unexplained polyneuropathy.