71/85. Multiple symmetrical lipomatosis: no longer just a Mediterranean disease? Multiple symmetrical lipomatosis is a rare condition, of which the etiology remains unclear. Most reported cases have been from the Mediterranean countries, and it is generally thought of as a disorder characteristic of that region. However, there have been 11 cases reported in japan since 1978, suggesting that this condition is no longer confined to Mediterranean countries. ( info) |
72/85. Madelung disease: MR findings. Two cases of Madelung disease (benign symmetrical lipomatosis) are presented. The MR findings in this striking condition are demonstrated. Short-repetition-time/short-echo time sequences nicely show the relationship of the cervical lipomatous accumulations to the airway and major neurovascular structures in the carotid spaces. Fat-suppression techniques add no additional information in the radiologic evaluation of these patients. ( info) |
73/85. Symmetrical lipomatosis of the tongue presenting as macroglossia. Report of two cases. Symmetrical lipomatosis in the oral cavity is extremely rare. Two cases of symmetrical lipomatosis presenting as macroglossia are presented. glossectomy was performed in order to reduce the size of the tongue and for diagnosis. Because of their multiplicity, non-encapsulation and invasiveness, the lesions were diagnosed histopathologically as symmetrical lipomatosis. ( info) |
74/85. Unusual etiology of cough in a woman with asthma. A case of multiple symmetric lipomatosis (Madelung's disease) acquired through chronic use of corticosteroids is reported. Presumed symptoms of asthma, which consisted of a barking cough, were treated with escalating doses of steroids. We postulate that excessive use of steroids led to extensive mediastinal fatty infiltration with narrowing of the trachea and mainstem bronchi as noted both radiographically and bronchoscopically. When the steroids were tapered, there was marked improvement of the patient's symptoms and resolution of the upper airway intrathoracic obstruction. When patients with asthma are receiving optimal therapy and fail to improve, other causes for their symptoms should be considered. ( info) |
75/85. Madelung's disease: an uncommon disorder of unknown aetiology? A case of Madelung's disease (benign multiple symmetrical lipomatosis, Launois-Bensaude syndrome) is described. The characteristic clinical features, associations with chronic alcoholism and a review of the current literature is reported. ( info) |
76/85. 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. ( info) |
77/85. Symmetric lipomatoses in female patients. BACKGROUND: Symmetric lipomatoses are characterized by marked symmetric deposition of diffusely distributed fatty tissue. Though relatively common disorders, they are rather rarely reported in the literature, possibly being misdiagnosed as general obesity. While the differential diagnosis of symmetric lipomatosis versus general obesity may not appear difficult in males, it is obviously problematic in females. OBSERVATIONS: We describe the findings in 6 representative female patients with symmetric lipomatoses: 3 with benign (multiple) symmetric lipomatosis and 3 with female zonal obesity. The former disorder was characterized by massive, firm, symmetric fat deposition predominantly around the neck and shoulder girdle and was clearly associated with alcohol abuse and/or liver disease. There were no malignant tumors of the upper airways. In the latter case, fatty tissue had accumulated mainly at the buttocks and thighs but characteristically spared the feet and hands. Tenderness was a common symptom. This disorder showed familial predisposition. histology in both cases revealed normal fatty tissue which was neither encapsulated nor septally divided. CONCLUSIONS: We suggest that the term 'symmetric lipomatoses' refers to two separate disorders, benign (multiple) symmetric lipomatosis and female zonal obesity. ( info) |
78/85. Multiple familial lipomatosis with polyneuropathy, an inherited dominant condition. A 22-year-old man had polyneuropathy, facial dysmorphia, atopia and multiple lipomatosis. His mother had neuropathy but not lipomatosis as two of her first cousins. The proband's grandmother had multiple lipomatosis as her own mother and a sister of her mother, but they didn't have neuropathy. This family is an example of a dominant syndrome the principle features of which are polyneuropathy. Variable expression could account for the phenotypic differences, combined with multiple lipomatosis. ( info) |
79/85. 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. ( info) |
80/85. MR of laryngeal and scrotal involvement in multiple symmetrical lipomatosis. Multiple symmetrical lipomatosis is a rare disorder characterized by progressive anomalous deposition of fat typically located in the neck and shoulders. magnetic resonance imaging allows exact definition of the abnormal fatty tissue and the involvement of deep structures. We describe the MR findings in two patients with the typical fat deposition in the neck and upper thorax which also presented unusual location of abnormal fat. One patient had laryngeal involvement with fatty infiltration of true and false cords. The other patient had inguinal and scrotal large deposition of fat. ( info) |