Prominent neurological involvement in Dercum disease

“Based on the present findings and data from the literature, neurological and neuropsychiatric manifestations, although not specific, are a relevant and probably under-estimated component of DD. The episode of dizziness followed by left side sensory-motor deficit might have been of vascular origin (namely, a transient ischemic attack), although it is still unclear whether the stroke-like events described in few reports of DD are caused by the condition itself or if their occurrence is merely coincidental. Notably, painful cutaneous lipomatosis has been related to microangiopathy and increased tissue fluid (lipedema),inflammatory reactions and fibrosis. Pain in lipedema is thought to result from hypoxia, inflammation, and necrosisof adipocytes; on the other hand, hyperbaric compression reduces tissue fluid and improves oxygen saturation, resulting in decreased pain in DD. Accordingly, these changes might represent a risk factor for lipohyalinotic andmicroatheromatous degeneration of the small penetrating cerebral vessels, given their limited caliber. The final result may be a modification of the local functioning of the striothalamic arterioles in the basal ganglia, eventually leading to ischemic lesions and enlarged perivascular spaces characteristic of microvascular disease. Systemic features of DD also include early onset spondy-loarthropathy, a finding which is in line with our report and with the occurrence of spine degeneration in DD. Although the etiology of DD is unknown, an inflammatory pathomechanism has been proposed, possibly leading to the release of pain-related neuropeptides, as suggested forother chronic pain syndromes. Finally, DD must be differentiated from other conditions including lipomas. Multiple lipomatosis is principally distinguished by large subcutaneous fatty masses, usually not painful, distributed in a symmetrical fashion, more prevalent in non-obese males and often associated with alcoholism. Sometimes,multiple lipomatosis is inherited as an autosomal-dominant trait, then referred to as FML. Benign adipose tissue tumors (solitary or multiple) are isolated subcutaneous tumors of well-differentiated adipocytes, some of which are painful; conversely, malignant cutaneous adipose tissue neoplasms are exceptionally rare and typically not painful.”

PDF: https://tinyurl.com/yd86s2dt
http://link.springer.com/article/10.1007/s00415-017-8415-1

 

 

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