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Our experience and review of the literature demonstrate that spinal epidural lipomatosis is a rare but serious neurologic complication of iatrogenic Cushing's syndrome. Most patients develop slowly progressive paraparesis over months, but a subgroup exists which presents with acute, irreversible paraplegia. We consider therapeutic options.
Multiple symmetric lipomatosis (MSL) is a rare disorder of middle life characterized by large nonencapsulated lipomas distributed around the neck, shoulders, and other axial regions. Neurologic involvement, particularly peripheral neuropathy, is frequent. The pathogenesis of the syndrome is still unknown, but ragged-red fibers are occasionally present in muscle of affected patients, suggesting a mitochondrial abnormality. We studied 11 unrelated patients with MSL by means of neurophysiology, muscle morphology, muscle biochemistry, Southern blot, and PCR analysis of mitochondrial DNA. All patients were men aged 41 to 63 years. Clinical or electrophysiologic signs of a sensorimotor polyneuropathy were present in nine patients, eight of whom had a history of alcoholism. In muscle biopsy specimens, the most prominent feature was pathologic subsarcolemmal aggregates of mitochondria. Biochemical analysis of respiratory chain enzymes revealed a moderate but significant decrease of cytochrome c oxidase activity as compared with age-matched controls. In one patient, Southern blot analysis showed multiple deletions of mitochondrial DNA. We conclude that mitochondrial dysfunction is common in MSL and may be based on identifiable defects in the mitochondrial genome.
Spinal epidural lipomatosis (SEL) is a rare complication of iatrogenic Cushing's syndrome (CS). There is only one case reported of symptomatic SEL in association with endogenous CS. We present a patient with compressive myeloradiculopathy due to SEL and Cushing's disease and suggest that in SEL of hypercortisolism, the excess of corticosteroids is the stimulus for the growth of adipose tissue in the spinal canal and that treatment should be aimed at correcting the endocrine abnormality.
In a nonalcoholic woman with multiple symmetric lipomatosis (MSL), muscle histochemistry showed ragged-red fibers and cytochrome c oxidase negative fibers. Southern blot analysis revealed a single deletion of mitochondrial DNA (mtDNA). We suggest that MSL is an uncommon manifestation of the wide clinical spectrum of mitochondrial disorders, in particular of those associated with single mtDNA deletions.
We describe familial multiple symmetric lipomatosis in a pedigree harboring the 8344 mutation in the tRNA(Lys) gene of mitochondrial DNA (mtDNA). The proband showed neuromuscular involvement but lacked the typical manifestations of myoclonic epilepsy and ragged-red fibers disease. The distribution of the mutation was unusual because the proportion of mutated genomes was higher in blood and lipomas than in muscle tissue.
In cases of Cushing's syndrome, unusual amounts of fat may accumulate in the spinal epidural space, similar to centripetal fat deposits elsewhere in the body. The mass of excessive epidural fat may be responsible for neurological symptoms referable to compression of the spinal cord or cauda equina. We report three cases illustrating this unusual phenomenon and call attention to three cases reported previously. Although it is an unusual complication of Cushing's syndrome, epidural lipomatosis should be considered in the differential diagnosis of any such case with neurological symptoms referable to the spinal cord or cauda equina. The diagnosis may be confirmed by computed tomographic scanning in conjunction with myelography. Therapeutic considerations are discussed.
Cowden disease (CD) is a genetic disease associated with multiple hamartomas and malignant neoplasms. During investigations for possible subnormal fertility, a series of eight males with CD underwent ultrasound scanning of their testes. Our findings detail the seven adult patients that were found to have multiple and bilateral testicular lesions on ultrasound. These lesions in Cowden's patients represent a newly described testicular pathology - lipomatosis of the testis. Here we detail the radiological findings. Ultrasound findings showed multiple (estimated 40+) discreet lesions randomly scattered thoughout the parenchyma of each affected testis. The lesions themselves had heterogeneous echo texture, but all being hyperechoic and with a variable size from 1 mm to 6 mm. One patient underwent MR examination of the lesions in which a high T(1) signal was seen, but no useful characteristic features were identifiable.
Spontaneous hemomediastinum is a rare event, occurring in association with bleeding disorders, intratumoral bleeding, or following an abrupt increase in intrathoracic pressure. We report the case of a patient with systemic lupus erythematosus, nephrotic syndrome, and renal failure, in whom mediastinal lipomatosis (ML) developed following increased corticosteroid therapy. Anticoagulant therapy likely precipitated a massive spontaneous hemomediastinum secondary to diffuse hemorrhage of mediastinal fat, which required emergency decompressive surgery. Steroid-induced ML is common and usually well tolerated, but clinicians should be aware of its potential risk of bleeding when associated with anticoagulant therapy. This case further emphasizes the bleeding complications of treatment with low-molecular-weight heparin in patients with renal failure.
A case of superior mediastinal widening due to unusual amounts of fat accumulations is reported in a patient with simple obesity, not associated with iatrogenic or primary Cushing's syndrome. The radiographic features included a smooth bilateral widening of the superior mediastinum, relative lucency, no definable mass in the lateral view, and no pressure effects on the trachea. Such mediastinal lipomatosis is a benign condition and may be related to general obesity. although rare, it should be considered in the differential diagnosis of mediastinal masses, particularly in obese individuals.
A case of diffuse lipomatosis is reported. A 54-year-old man presented with a large mass in the left gluteal region. Plain radiographs showed hyperostosis of the phalanx of the left third toe. The diagnosis was aided by magnetic resonance imaging and computed tomography which clearly demonstrated a diffuse overgrowth of fatty tissue in the left buttock and the extremity involving the subcutaneous and gluteal muscular tissue.
A 46-year-old man was diagnosed as having benign symmetric lipomatosis (BSL) based on the grotesque physical examination findings and subcutaneous fat tissue biopsy. Although markedly overweight, the glucose tolerance was normal and insulin levels indicated no remarkable insulin resistance on the 75 g oral glucose tolerance test. Furthermore his visceral fat tissue was very slight and the circulating adiponectin concentration was high those which suggesting a high insulin sensitivity. In addition, the relevance of alcohol in the onset of BSL is strongly suggested based on alcoholic hepatopathy and the history of the development of grotesque physical appearance associated with increased alcohol consumption.
We report a 29-year-old male with pelvic lipomatosis that presented with edema of the lower extremities. The patient visited our department because of the marked edema of the lower extremities with body weight gain. The pelvic region was generally radiolucent on plain abdominal radiogram. Excretory urogram and pelvic computed tomography scan revealed compression of the bladder by an adipose tissue mass. On lymphograms, lymph vessels were compressed from the inguinal area in the common iliac region. The edema of the lower extremities was thought to be caused by lymph vessel obstruction.
Pelvic lipomatosis is a rare condition characterized by excessive accumulation of fat in the pelvis. The symptoms and the course of the disease are related to compression of the lower urinary tract and rectum. The clinical findings and radiological characteristics are described.
The pathogenesis of multiple symmetric lipomatosis (MSL) is as yet unknown; however, some studies seem to indicate the neoplastic nature of this lesion. In this study we have examined the ultrastructural features of the adipocyte precursors of patients with MSL. The cells were isolated by collagenase, cultured, and then examined at the moment of their confluence in a monolayer. Their ultrastructural features were compared with those of cells of the abdominal subcutaneous adipose tissue taken from patients with nonsystemic, surgically treated illnesses. When examined immediately after isolation, the cells from normal tissue did not show significant differences from MSL cells. In culture, however, MSL grew more quickly into a monolayer and showed numerous nuclear pockets and cytoplasmic microfilaments, which were not seen in cells from normal tissue. These differences appear compatible with the neoplastic nature of the MSL adipocyte precursors.
A patient with benign symmetrical lipomatosis of the neck (Madelung's disease) was successfully treated with liposuction rather than classic surgical lipectomy. To our knowledge this is only the second such patient treated in this way.
A 47 year old man with a history of alcoholism presented with symmetrical lipomas on the shoulders, neck, and chest. He also had lipomatous areas on the trunk and thighs. He was treated with a combination of surgery and liposuction, and made an uneventful recovery. Histological examination confirmed the diagnosis of multiple symmetric lipomatosis, a rare disorder only about 200 cases of which have been reported. The aetiology is unknown, but is often seen in patients with a history of alcoholism.
A case of peroneal nerve entrapment is reported in a patient with scleroderma. Compression was due to a lipoma in the popliteal fossa and resulted in increasingly severe foot-drop. Complete recovery occurred after the lipoma was resected. A brief review of peroneal nerve palsies and lipomatosis is presented.
Pathological overgrowth of the epidural fat in the spine has been described and reported nearly exclusively in patients either with Cushing's syndrome or on chronic intake of glucocorticoids for a variety of clinical disorders. The authors report four patients with documented spinal lipomatosis (three pathologically and one radiologically). Only one of these patients received corticosteroids, and none had an underlying endocrinological abnormality. All four patients were adult males with a mean age at onset of symptoms of 43 years (range from 18 to 60 years). The symptoms ranged from simple neurogenic claudication and radicular pain to frank myelopathy. Myelography followed by computerized tomography were instrumental in the diagnosis of the first three patients; the fourth was diagnosed by magnetic resonance imaging. The thoracic spine was involved in two cases and the lumbosacral area in the other two. The different treatment modalities were tailored according to the symptomatology of the patients. These included weight reduction of an overweight patient with minimal neurological findings in one case and decompressive laminectomy and fat debulking to achieve adequate cord decompression in the remaining three cases. Two patients improved significantly, the condition of one stabilized, and the fourth required a second decompression at other spinal levels. The various modalities of treatment and their potential complications are discussed.