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At least 109 records · Page 6Linked to original sources

PubMed1996

Multiple symmetrical lipomatosis (Madelung's disease): a case report.

Multiple symmetrical lipomatosis (Madelung's disease) is a rare disease of undetermined cause characterized by symmetrical deposits of non-encapsulated fat on the suboccipital area, cervical area, shoulders and trunk. The patients are usually middle-aged male alcoholics. The treatment is palliative surgical removal of excess fat from the neck and paracervical regions or any other lesion sites. Oriental report about multiple symmetrical lipomatosis (MSL) is very rare. Here a case of multiple symmetrical lipomatosis in a 43 year-old man is described. We describe his clinical course and review the literature.

Adult↗

PubMed2002

A case of idiopathic spinal epidural lipomatosis presented with radicular pain caused by compression with enlarged veins surrounding nerve roots.

OBJECTIVE: To report a case of idiopathic spinal epidural lipomatosis (SEDL) presented with unique radicular pain most likely caused by enlarged veins surrounding nerve roots. PATIENT: A 26-year-old male presented with radicular pain of the right T6-T7 area. He also showed Becker's nevus in the corresponding area. CT myelography and magnetic resonance imaging revealed epidural lipomatosis posterior to T4-T8 of the spinal cord. Surgical removal of adipose tissue and a hemilaminectomy of T4-T7 were performed and resulted in relief of the radicular pain. CONCLUSIONS: Lipomatosis was histologically confirmed and surrounded by enlarged veins. These abnormally enlarged veins compressed the nerve roots and were thought to cause radicular pain. Also, Becker's nevus of this case seems to have some relationship with SEDL.

Adult↗

PubMed1998

[Lipohyperplasia or intestinal lipomatosis].

Lipohyperplasia or intestinal lipomatosis is an infrequent disease characterised by anomalous infiltration of adipose tissue in the intestinal submucosa. Localised forms are generally asymptomatic, whereas diffuse forms may lead to intestinal subocclusion, digestive hemorrhage or diarrhoea. Although benign, the differential diagnosis of intestinal lipomatosis with malignant pathologies of the colon or appendix often prompts the need for surgical exploration and the histological analysis of biopsy material. Surgical exeresis of the lesion is generally associated with the normalisation of clinical symptoms. The authors report the onset and clinical evolution of two cases of intestinal lipomatosis referred to their attention.

English Abstract↗

PubMed1987

Benign symmetric lipomatosis Launois-Bensaude. Report of ten cases and review of the literature.

Benign symmetric lipomatosis Launois-Bensaude is a disease rarely reported in the American literature and not mentioned in the standard English textbooks of dermatology. It seems, however, to be relatively common in Europe. Between 1981 and 1985 we examined twelve patients, and a number of case reports have been published in French and German literature. We describe ten typical cases and review the literature. The disease is characterized by massive symmetric fat deposits predominantly in the neck and shoulder girdle area. Pathogenetically, the increase in fatty tissue is assumed to result from a localized defect in catecholamine-induced lipolysis. The disease is frequently associated with alcoholism, hepatopathy, glucose intolerance, hyperuricemia, and malignant tumors of the upper airways, requiring thorough clinical evaluation of all patients. Dietary treatment and weight loss are of limited value in the management of benign symmetric lipomatosis. Surgical removal of lipomatous tissue is frequently followed by recurrence and should be restricted to decompression in patients with functional impairment.

Adult↗

PubMed1986

Lipomatosis of the neck (Madelung's neck).

Benign symmetrical lipomatosis of the neck is a rare disease that has to be differentiated from goiter, sialadenitis, obesity or a lymphatic tumor. Most patients are severe alcoholics, but they may have other endocrine disorders, such as diabetes mellitus, hyperuricemia, or hyperlipidemia. Aside from the cosmetic disfigurement and consequent psychological stress, respiratory distress may be the indication for surgical treatment. Excision of the lipomatosis requires technical skill because the extensive and sometimes infiltrative growth makes dissection of muscle and nerves difficult. The computer tomogram provides good information on the extent of the disease. Three of our 5 patients died 2 1/2 to 6 years after the first operation because of their primary disease.

Adult↗

PubMed1989

A case of multiple symmetric lipomatosis (Madelung's disease).

A case of multiple symmetric lipomatosis in a 61-year-old man is described. The patient had the striking appearance that characterizes multiple symmetric lipomatosis. He had a history of alcohol abuse. Before admission to our institution, he underwent surgical treatment three times at other hospitals. We describe his clinical course and review the literature.

Alcoholism↗

PubMed1990

Familial multiple symmetric lipomatosis with peripheral neuropathy.

We describe coexisting peripheral neuropathy and multiple symmetric lipomatosis in 4 of 7 siblings. The absence of either condition in 3 other generations of this family suggests autosomal recessive inheritance. None of the affected siblings were alcoholic, a factor some have proposed to explain the frequent occurrence of peripheral neuropathy in sporadic multiple symmetric lipomatosis. Serum lipid studies, including apoprotein A levels, were normal. Sural nerve biopsy from 1 patient showed nerve fiber loss, predominantly affecting large myelinated fibers. The relationship between myelin sheath thickness and axon diameter was normal, arguing that this neuropathy is not due to primary axonal atrophy.

Adult↗

PubMedDate not supplied

Insulin sensitivity and metabolic clearance rate of insulin in familial multiple lipomatosis.

Intolerance to glucose in certain kinds of lipomatosis is well documented. This article describes a euglucaemic hyperinsulinaemic clamp study of alterations in glucose and/or insulin metabolism in four members of a single family with familial multiple lipomatosis. Fifteen normal subjects were studied as controls. The four patients exhibited no alteration in tolerance to orally administered glucose. When a Biostator Glucose-Controlled Insulin Infusion System (GCIIS) was used to clamp glycaemia at 4.44 mmol/L with successive insulin infusion rates of (a) 0.5 (b) 1.0 or (c) 5.0 mU/kg/min, there was no difference between patients and controls as regards the value of M, the rate of glucose infusion, but the concentrations of immunoreactive insulin recorded during the last 40 minutes of each phase of the clamp were greater in patients than in controls (45 +/- 2 vs 27 +/- 2 uU/mL (p less than 0.01), 83 +/- 2 vs 60 +/- 5 uU/mL (p less than 0.05) and 537 +/- 48 vs 377 +/- 25 uU/mL (p less than 0.05) for insulin infusion rates (a), (b) and (c) respectively), and the ratio M/IRI was consequently smaller for patients than controls (1.92 +/- 0.41 vs 3.06 +/- 0.19 (p less than 0.05) for an insulin infusion rate of 5 mU/kg/min). The metabolic clearance rate of insulin was likewise slower in patients than controls (p less than 0.01). It is concluded that the four patients studied (all members of the same family) have sub-normal sensitivity to insulin secondary to a sub-normal metabolic clearance rate for insulin.

Blood Glucose↗

PubMed1988

[Multiple symmetrical lipomatosis. A retrospective study of 14 cases and review of the literature].

"Multiple symmetric lipomatosis" is an accumulation of fatty tissue in upper areas of the body mainly effecting middle aged men. It is a rare benign disease connected with differing disorders. We report 14 male patients suffering from "multiple symmetric lipomatosis" and focus on the etiology and on associated disorders. Differential diagnosis, pathohistological considerations, clinical behaviour, and operative treatment are discussed. A review of the literature is presented.

Adult↗

PubMed1997

Mitochondrial dysfunction with myoclonus epilepsy and ragged-red fibers point mutation in nerve, muscle, and adipose tissue of a patient with multiple symmetric lipomatosis.

We report a 64-year-old man presenting with multiple symmetric lipomatosis (MSL) and mitochondrial encephalomyoneuropathy. The diagnosis of a mitochondrial cytopathy was based on the typical clinical symptoms and signs, including chronic progressive external ophthalmoplegia, hearing impairment, cerebellar ataxia, proximal myopathy, and polyneuropathy, and on molecular genetic and histological examinations. As a unique finding, the A-->G(8344) myoclonus epilepsy and ragged-red fibers point mutation was found in peripheral nerve, muscle, and adipose tissue. Muscle biopsy revealed multiple ragged-red fibers and other morphological signs of a mitochondrial myopathy. Sural nerve biopsy demonstrated a mixed axonal and demyelinating neuropathy with extensive loss of myelinated fibers and conspicuous onion bulb formations, as well as structural mitochondrial abnormalities on electron microscopy. These findings clearly demonstrate mitochondrial dysfunction in muscle, adipose tissue, and for the first time also in nervous tissue of an MSL patient, and strongly support the concept of mitochondrial cytopathy as one of the possible causes of multiple symmetric lipomatosis.

Adipose Tissue↗

PubMed2000

A t(2;19)(p13;p13.2) in a giant invasive cardiac lipoma from a patient with multiple lipomatosis.

Cardiac lipomas occur infrequently but account for a significant portion of rare cardiac tumors. Common cutaneous lipomas have previously been associated with rearrangements of chromosome band 12q15, which often disrupt the high-mobility-group protein gene HMGIC. In this report, we describe the cytogenetic analysis of an unusual giant cardiac lipoma that exhibited myocardial invasion in a patient with a history of multiple lipomatosis (cutaneous lipoma, lipomatous gynecomastia, lipomatous hypertrophy of the interatrial septum, and dyslipidemia). Cytogenetic studies of cells derived from the cardiac lipoma demonstrated no abnormalities of chromosome 12, but did reveal a t(2;19)(p13;p13.2). A liposarcoma-derived oncogene (p115-RhoGEF) previously mapped to chromosome 19 and the low-density lipoprotein receptor gene (LDLR) previously mapped to chromosome band 19p13 were evaluated to determine whether they were disrupted by this translocation. Fluorescence in situ hybridization analyses assigned p115-RhoGEF to chromosome 19 in bands q13.2-q13.3 and mapped the LDLR to chromosome arm 19p in segment 13.2, but centromeric to the t(2;19) breakpoint. Thus, these genes are unlikely to be involved in the t(2;19)(p13;p13.2). Further studies of the regions of chromosomes 2 and 19 perturbed by the translocation in this unusual infiltrating cardiac lipoma will identify gene(s) that participate in adipocyte growth and differentiation and may provide insight into syndromes of multiple lipomatosis.

Chromosomes, Human, Pair 19↗

PubMed1980

Analysis of a large pedigree with elliptocytosis, multiple lipomatosis, and biological false-positive serological test for syphilis.

Elliptocytosis, multiple lipomatosis, and biological false-postive serological test for syphilis (BFPSTS) were found in a single individual. One hundred eighty relatives were tested for the three diseases: 74 were typed for seven blood group antigens, and 58 were typed for four electrophoretic enzyme markers. Likelihood analysis of the pedigree data confirmed independent dominant inheritance for elliptocytosis and lipomatosis. BFPSTS appears dominant, but the analysis was inconclusive. No linkages were found between any disease gene and any marker gene. Two female pedigree members with BFPSTS developed systemic lupus erythematosus, a finding in agreement with the previously described association. The analysis did not lead to any conclusions about the causal relationship between the two traits.

Adult↗

PubMed1991

Mitochondrial dysfunction in multiple symmetrical lipomatosis.

Multiple symmetrical lipomatosis is a striking clinical finding associated with a variety of peripheral and central nervous system abnormalities. We describe 4 unrelated patients with evidence of mitochondrial dysfunction in skeletal muscle. Multiple symmetrical lipomatosis is an additional, albeit unusual, manifestation of the expanding clinical spectrum of mitochondrial diseases.

Adult↗

PubMed1988

Spinal cord compression by epidural lipomatosis in juvenile rheumatoid arthritis.

Two cases of spinal cord compression secondary to steroid-induced epidural lipomatosis in systemic juvenile rheumatoid arthritis (JRA) patients are reported. This complication of prolonged corticosteroid therapy has not been described previously in children with JRA. Epidural lipomatosis should be considered in the differential diagnosis of JRA patients receiving high-dose and/or prolonged corticosteroid therapy who present with neurologic signs and symptoms referable to the spinal cord.

Adrenal Cortex Hormones↗

PubMed1997

Epidural lipomatosis with lumbar radiculopathy in one obese patient. Case report and review of the literature.

Idiopathic epidural spinal lipomas are rare: only 13 cases have been described in the literature. We report a further case in an obese patient without known etiological factors. Diagnosis of epidural lipomatosis was performed by MRI. Weight reduction was obtained by conservative treatment, reserving surgery in case symptoms did not disappear. Of the 8 obese patients with idiopathic epidural spinal lipomatosis described in the literature, 3 were treated conservatively, with complete regression of symptoms in 2 cases, partial in the other one. In our patient, the radicular symptomatology disappeared once he had lost weight.

Adult↗

PubMed1988

Mediastinal lipomatosis: a complication of high dose steroid therapy in children.

Mediastinal lipomatosis has been described in adults and is a well recognized cause of mediastinal enlargement. Fatty accumulation in the mediastinum has been observed following extended usage of large doses of steroids, generally greater than 60 mg daily. Little documentation of this entity exists in pediatric patients. A 9-year-old male who received large doses of steroids for graft-versus-host disease following bone marrow transplantation for acute myelogenous leukemia developed mediastinal lipomatosis. Early qualification of this diagnosis is especially important in this group of patients, who are also at risk for a second neoplasm.

Child↗

PubMed1988

Pelvic lipomatosis: diagnosis and characterization by magnetic resonance imaging.

While the number of reported cases of pelvic lipomatosis has been relatively small, this entity's prevalence is probably underestimated. Disease progression can cause hydroureteronephrosis and renal failure, and clinical follow-up is mandated after diagnosis. Diagnosis is based on detection of characteristic findings in conventional radiographs and computed tomographic (CT) images. We report here the magnetic resonance imaging (MRI) features of pelvic lipomatosis in 5 male patients, 2 of whom ultimately required placement of a ureteral stent to alleviate mechanical obstruction. Multiplanar MRI not only allows diagnostic confirmation comparable to that possible with CT but also provides delineation of cephalad displacement of the bladder base, elongation of the bladder neck and posterior urethra, and elevation of the prostate gland. The MR images show characteristic medial and superior displacement of the seminal vesicles and show fatty tissue separating the prostate gland from the rectum. The noninvasive nature and low biological risk of MRI are desirable features, particularly when serial evaluation of pelvic anatomic distortion is necessary.

Adult↗

PubMed2005

Lipomatosis of the sciatic nerve: typical and atypical MRI features.

Lipomatosis of nerve, also known as fibrolipomatous hamartoma, is a rare condition of nerve, usually affecting the median nerve. The MRI appearance is characteristic. We describe two cases of lipomatosis of nerve involving the sciatic nerve, an extremely unusual location for this lesion, in patients with sciatic neuropathy. These cases share the typical features previously described in the literature for other nerves, but also contain atypical features not previously highlighted, relating to the variability in distribution and extent of the fatty deposition. Recognition of the MRI appearance of this entity is important in order to avoid unnecessary attempts at surgical resection of this lesion.

Adult↗