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Imaging in epidural lipomatosis.

This paper reviews the literature concerning symptomatic epidural lipomatosis. The different diagnostic procedures (conventional myelography, computed tomography, and magnetic resonance imaging) and therapeutic approaches (including medical treatment, surgery, and dietary influence) are discussed.

Journal Article↗

Impairment of cardiovascular autonomic reflexes in multiple symmetric lipomatosis.

Cardiovascular autonomic reflexes were evaluated in 13 male subjects affected by Multiple Symmetric Lipomatosis (MSL) and the results were compared with those obtained in 13 age-matched male controls and in 16 male heavy drinkers matched with the MSL group for age and alcohol intake. Valsalva Manoeuvre (VR), Heart Rate variations on Deep Breathing (DB) and R-R intervals 30/15 ratio during Lying-to-Standing (LS) (tilting), were used as primarily parasympathetic tests. As primarily sympathetic tests, we assessed the increment of diastolic blood pressure in Sustained Handgrip (SHG) and the fall of systolic blood pressure on standing (Postural Hypotension = PH). Mean values of VR and DB were significantly (P less than 0.001) lower in MSL subjects than in controls and heavy drinkers. Mean LS values were significantly lower in MSL subjects (P less than 0.001) and in heavy drinkers (P less than 0.01) in relation to controls. No significant differences were observed in the 3 groups of subjects regarding the mean values of PH and SHG. The results suggest that MSL is characterized by an impairment of autonomic function. This impairment seems to be prevalently parasympathetic and not related to a high alcohol intake.

Adult↗

Case report: replacement lipomatosis of the kidney--unusual CT features.

A case of replacement lipomatosis of the kidney (RLK) diagnosed on computed tomography (CT) is presented. CT demonstrated an infiltrating fatty mass surrounding a scarred kidney with a central staghorn calculus. Infiltration of the retroperitoneal muscles and an abscess within the erector spinae were also seen. The most important feature of this case is a mass effect causing displacement of the aorta and spleen, which is enlarged. This is usually only seen with malignant masses and has not been previously reported with RLK. The literature and radiological features of RLK are reviewed.

Aged↗

Benign symmetric lipomatosis (Madelung's disease).

We report on a 50-year-old man who presented with benign, symmetric lipomatosis and chronic alcoholic liver disease. The characteristic clinical features and a brief review of the current literature are presented.

Journal Article↗

Lipomatosis of the scalp and macrocephaly.

In summary, a case of macrocephaly and lipomatosis of the scalp and forehead has been presented. The phenotypic and clinical features found in our patient are in accordance with previous reports. The differential diagnosis with other hamartoneoplastic syndromes has been evaluated and discarded, concluding that the present report is in accordance with Bannayan-Zonana syndrome. However, as in other inherited traits, finding a genetic test to diagnose this entity remains a challenge.

Child, Preschool↗

Symptomatic spinal epidural lipomatosis induced by a long-term steroid treatment. Review of the literature and report of two additional cases.

Spinal epidural lipomatosis associated with Cushing's syndrome is an uncommon complication (11 reported cases). Two additional symptomatic cases with neurologic deficit are described. Steroid treatment was systemic in the first case and local with epidural injections in the second. The second case is unique because no similar observations have yet been reported. In most cases, a preoperative computed tomographic scan establishes the diagnosis by demonstrating dural compression by an adipose mass. Myelography is far less specific. In some cases, the exact diagnosis is made at the time of surgery. The treatment is primarily surgical, with laminectomy over the length of the compression and the removal of the compressing fat. Neurologic recovery is dependent on two factors: the level of the compression and the adequacy of decompression.

Epidural Neoplasms↗

[Encephalocraniocutaneous lipomatosis and linear naevus sebaceus].

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous dysgenetic syndrome, which is characterized by unilateral lipomas in the subcutis of the face, skalp and skull, porencephalic cyst of the ipsilateral hemisphere, epilepsy, and severely delayed development. Furthermore a variety of skin lesions (choristomas) has been described. In the present case the skin lesions had the form of a linear nevus sebaceus. This condition is frequently combined with epilepsy and neurological disorders (Schimmelpenning-Feuerstein-Mims-syndrome). The latter seems not to be a very rare syndrome. The coincidence of characteristics of both syndromes in the same child supports the suggestion of a pathogenetical relationship.

Brain↗