1/26. Intracranial lipoma with extracranial extension through foramen ovale in a patient with encephalocraniocutaneous lipomatosis syndrome.We present CT and MR features of a large intracranial lipoma located in the left sphenocavernosal region in a patient with a history of spinal intradural lipoma excision. The lipomatous lesion surrounded the internal carotid artery and trigeminal nerve and extended extracranially into the infratemporal region through a smoothly dilated foramen ovale.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/26. Idiopathic symptomatic epidural lipomatosis of the lumbar spine.BACKGROUND: Symptomatic spinal epidural lipomatosis (SEL) of the lumbar spine is a rare disease, often associated with steroid overload. Idiopathic lipomatosis is even much less frequent. signs and symptoms depend upon the level and degree of nerve root compression. diagnosis is best based on MRI. Weight reduction can be curative, however after failure of medical treatment or in severe cases surgical decompression should be performed. METHOD: Four patients with severe symptoms of lumbar spinal epidural lipomatosis were treated by surgical decompression. Patient history and neurological examination are described, diagnostic imaging is demonstrated, surgical treatment and outcome are documented. Different surgical techniques including laminectomy, interlaminar fenestration and lateral recess decompression were applied and are discussed. FINDINGS: All four patients improved after surgery. No surgical complications were observed. Even though limited to four cases this is the second largest series of operated idiopathic spinal epidural lipomatosis. INTERPRETATION: Surgical decompression was effective in improving symptoms in severe lumbar idiopathic spinal epidural lipomatosis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/26. Encephalocraniocutaneous lipomatosis: a case with unilateral odontomas and review of the literature.We report a 7-year-old boy with the typical features of encephalocraniocutaneous lipomatosis (ECCL) including unilateral skin, eye, bone, and asymptomatic central nervous system involvement. The presenting sign were numerous ipsilateral odontomas which have been described in only two previous patients so far. We reviewed all 32 cases reported to date and focus on the dermatological hallmark of the condition, namely a fatty tissue naevus of the scalp for which the term naevus psiloliparus was recently coined. Sporadic occurrence, patchy type of organ involvement and roughly balanced sex ratio in ECCL are compatible with the concept of a lethal autosomal mutation only surviving in a mosaic state. CONCLUSION: In children presenting with a congenital smooth hairless lesion on the scalp, the diagnosis of naevus psiloliparus should be considered and other signs of encephalocraniocutaneous lipomatosis should be looked for.- - - - - - - - - - ranking = 0.01233777651358keywords = organ (Clic here for more details about this article) |
4/26. Fibrolipomatous hamartoma in the foot: atypical MR imaging findings.lipomatosis of a nerve is a well-known but uncommon entity mostly seen in the median nerve. magnetic resonance imaging (MRI) has been shown to provide pathognomonic features, obviating the need for diagnostic biopsy. We present a case of lipomatosis of a branch of the medial plantar nerve with an atypical appearance on magnetic resonance imaging.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
5/26. Presurgical evaluation and cognitive functional reorganization in Fishman syndrome.Fishman syndrome, also known as encephalocraniocutaneous lipomatosis (ECCL), is a rare, congenital neurocutaneous syndrome characterized by unilateral skin, eye, and brain abnormalities. Epileptic seizures and developmental delay are usually present. We report the clinical, radiological, and, for the first time, neurophysiological findings in a 24-year-old woman diagnosed with ECCL who was evaluated for epilepsy surgery. Functional magnetic resonance imaging revealed transfer of memory and language functions to the nonaffected hemisphere, providing evidence that functional reorganization and restoration of cognitive functions may occur in the context of extensive malformations, such as neurocutaneous syndromes.- - - - - - - - - - ranking = 0.061688882567902keywords = organ (Clic here for more details about this article) |
6/26. Epidural spinal lipomatosis with acute onset of paraplegia in an hiv-positive patient treated with corticosteroids and protease inhibitor: case report.STUDY DESIGN: Case report. OBJECTIVE: To report a case of hiv-related lipodystrophy with a rapid onset of symptoms from epidural lipomatosis in the wake of protease inhibitor and steroid treatment. SUMMARY OF BACKGROUND DATA: Symptomatic spinal epidural lipomatosis is considered to be a rare condition usually presenting with slowly progressive cord or nerve root compression. Only 2 cases of spinal lipomatosis in hiv-related lipodystrophy have been reported. methods: We describe the case of a 41-year-old male with hiv who received protease inhibitor medication and had neurologic deficits rapidly develop. RESULTS: The patient had complete paraplegia develop within 12 hours from admission following a 1-day history of unsteady gait and a 3-day history of leg numbness. After diagnosis of epidural lipomatosis on magnetic resonance imaging, the patient underwent decompressive thoraco-laminectomy. He recovered well and was able to walk by postoperative day 4. CONCLUSION: It is important to maintain an awareness for the possible association between hiv lipodystrophy and symptomatic epidural lipomatosis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/26. Infantile congenital parotid lipomatosis: a rare case report.A case of congenital parotid lipomatosis in an infant is reported and literature is reviewed. This rare condition in children presents as gradually increasing parotid swelling, which is difficult to diagnose preoperatively as this condition is not considered in the differential diagnosis of a parotid mass. Complete excision with superficial or total parotidectomy with preservation of facial nerve is the treatment of choice.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/26. A neglected case of macrodystrophia lipomatosa of the foot in an elderly man.Macrodystrophia lipomatosa is a rare disorder characterized by three-dimensional enlargement of one or more fingers or toes with predominantly fibroadipose tissue. Radiographically, it appears as hypertrophy of soft tissues and bones. The pathologic findings are infiltration and hypertrophy of adipose tissue in subcutaneous tissue, nerve sheaths, and periosteum. Macrodystrophia lipomatosa is usually diagnosed during childhood. The case presented here involves the most elderly patient with the condition ever reported, to our knowledge. As such, it may advance current knowledge of macrodystrophia lipomatosa. Special emphasis is given to the unique "bridge" formation seen radiographically in this case.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/26. Hamartomatous polyps of the colon: ganglioneuromatous, stromal, and lipomatous.Intestinal ganglioneuromas comprise benign, hamartomatous polyps characterized by an overgrowth of nerve ganglion cells, nerve fibers, and supporting cells in the gastrointestinal tract. This polyposis has been divided into 3 subgroups, each with a different degree of ganglioneuroma formation: polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. The ganglioneuromatous polyposis subgroup is not known to coexist with systemic disorders that often have an associated intestinal polyposis, such as multiple endocrine neoplasia type IIb, neurofibromatosis type I, and Cowden syndrome. We report a case of ganglioneuromatous polyposis plus cutaneous lipomatosis in a 41-year-old man with no established systemic disease. However, he possessed unique anatomic findings in addition to his ganglioneuromatosis, suggesting that the ganglioneuromatosis-lipomatosis in our patient may represent an unrecognized syndrome. This case report and brief review of the literature provide an overview of intestinal ganglioneuromatosis in relation to the hereditary polyposis syndromes and describe the individual ganglioneuromatosis subgroups.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
10/26. 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.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
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