OBJECTIVE: To review the clinical research progress of spinal epidural lipomatosis (SEL). METHODS: The clinical studies on SEL at home and abroad in recent years were extensively reviewed, and the pathogenesis, clinical and imaging manifestations, and treatment status of SEL were summarized and analyzed. RESULTS: SEL is a disease characterized by compression of the spinal cord and nerve roots due to abnormal accumulation of epidural adipose tissue in the spinal canal. Its prevalence and diagnosis rate are low and the pathogenesis is not fully understood. MRI is the most sensitive and specific diagnostic test for SEL. Surgical decompression and removal of excess adipose tissue are the only options for patients with acute SEL or those who have failed conservative management, and conservative management should be considered for other patients. CONCLUSION SEL is a rare disease and related research still needs to be improved. In the future, high-quality, multi-center and large-sample studies will be of great significance for evaluating the choice of treatment methods and effectiveness of SEL patients.
Humans ; Decompression, Surgical/methods* ; Epidural Space/surgery* ; Lipomatosis/surgery* ; Magnetic Resonance Imaging ; Spinal Cord Diseases/surgery*

Lipoma arborescens or synovial lipomatosis is a rare disorder that is characterized by mature fat infiltration of the hypertrophic synovial villi, most frequently affecting the supra-patellar pouch of the knee. This paper presents a case of lipoma arborescens of the ankle joint bilaterally in an adult patient with involvement of both the intra-articular synovium and the synovial sheath of the tendons around the ankle.
Adult ; Ankle Joint ; Ankle ; Humans ; Knee ; Lipoma ; Lipomatosis ; Magnetic Resonance Imaging ; Synovial Membrane ; Tendons

Congenital infiltrating lipomatosis of the face is a rare disorder resulting from overgrowth of adipose tissues. This condition presents gradually with swelling along with age, hypertrophy of adjacent bones, and tooth abnormalities. This study reports a case of congenital infiltrating lipomatosis of face with seizures and reviews relevant literature on the etiology, clinical symptom, diagnosis, and treatment of this condition.
Adipose Tissue ; Face ; Humans ; Lipomatosis ; complications ; Seizures ; complications

Lipedematous alopecia (LA) is a rare disease entity that manifests as increased thickness of subcutaneous fatty tissue in the scalp with a variable degree of acquired, non-scarring alopecia. Although the pathogenesis of alopecia in LA is not clear, it is assumed that the increased thickness of subcutaneous fat retards hair growth by disturbing the peripheral microenvironment of the hair bulb. LA is clinically distinguishable from lipedematous scalp (LS) in that LS is not associated with any hair abnormalities, while LA is characterized by variable degree of hair loss. However, both LA and LS share increased scalp thickness. Here, we describe a rare case of LA on the frontal scalp of an Asian woman, further characterize the clinical and histologic features, and suggest applying an integrated diagnosis as lipedematous diseases of the scalp.
Adipose Tissue ; Alopecia* ; Asian Continental Ancestry Group* ; Diagnosis ; Female ; Hair ; Humans ; Lipomatosis ; Rare Diseases ; Scalp* ; Subcutaneous Fat

Madelung's disease, also known as multiple symmetric lipomatosis (MSL) is a rare disease characterized by the presence of multiple, symmetric, loose adipose tissues distributed around the neck, shoulder, back or chest. MSL is different from simple obesity, which is characterized by the presence of well-distributed total body fat. It is rarely reported in Asia and most commonly seen among middle-aged men. Although the etiology of MSL is unknown, the disease is associated with alcoholism in 60% to 90% of patients. The diagnosis is usually made on the basis of the history, and clinical appearance. Also, imaging such as CT or MRI could confirm the diagnosis. Herein, we experienced an uncommon case of MSL in middle-aged women with alcoholism.
Adipose Tissue ; Alcoholism* ; Asia ; Diagnosis ; Female ; Humans ; Lipomatosis, Multiple Symmetrical* ; Magnetic Resonance Imaging ; Male ; Neck ; Obesity ; Rare Diseases ; Shoulder ; Thorax

OBJECTIVE: This study aimed to unravel the putative mechanism underlying the neurologic deficits contralateral to the side with lumbar disc herniation (LDH) and to elucidate the treatment for this condition. METHODS: From January 2009 to June 2015, 8 patients with LDH with predominantly contralateral neurologic deficits underwent surgical treatment on the side with LDH with or without decompressing the symptomatic side. A retrospective review of charts and radiological records of these 8 patients was performed. The putative mechanisms underlying the associated contralateral neurological deficits, magnetic resonance imaging (MRI), electromyography (EMG), and the adequate surgical approach are discussed here. RESULTS: MRI revealed a similar laterally skewed paramedian disc herniation, with the apex deviated from the symptomatic side rather than directly compressing the nerve root; this condition may generate a contralateral traction force. EMG revealed radiculopathies in both sides of 6 patients and in the herniated side of 2 patients. Based on EMG findings and the existence of suspicious lateral recess stenosis of the symptomatic side, 6 patients underwent bilateral decompression of nerve roots and 2 were subjected to a microscopic discectomy to treat the asymptomatic disc herniation. No specific conditions such as venous congestion, nerve root anomaly or epidural lipomatosis were observed, which may be considered the putative pathomechanism causing the contralateral neurological deficits. The symptoms resolved significantly after surgery. CONCLUSION: The traction force generated on the contralateral side and lateral recess stenosis, rather than direct compression, may cause the contralateral neurologic deficits observed in LDH.
Constriction, Pathologic ; Decompression ; Diskectomy ; Electromyography ; Humans ; Hyperemia ; Intervertebral Disc Displacement ; Lipomatosis ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Radiculopathy ; Retrospective Studies ; Traction

No abstract available.
Lipomatosis* ; Neoplasm Metastasis* ; Small Cell Lung Carcinoma*

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.
Adipose Tissue ; Anophthalmos ; Anticonvulsants ; Basal Ganglia ; Brain ; Brain Neoplasms ; Cerebellum ; Child ; Drug Resistant Epilepsy ; Female ; Follow-Up Studies ; Hippocampus ; Humans ; Lipoma ; Lipomatosis* ; Magnetic Resonance Spectroscopy ; Methionine ; Neoplasms, Neuroepithelial* ; Neurocutaneous Syndromes ; Neurologic Manifestations ; Positron-Emission Tomography ; Seizures* ; Thalamus ; White Matter

No abstract available.
Brachial Plexus Neuropathies* ; Lipomatosis, Multiple Symmetrical*

Spinal epidural lipomatosis (SEL) is a rare condition that presents as a back pain with progressive neurologic symptoms. Most affected patients are obese and receiving steroid therapy, or have an endocrinopathies. We report a rare case of cauda equina syndrome caused by SEL in a non-obese healthy young man without any evident traumatic episode. A healthy 19-year-old man, who had experienced lower back pain for two months, visited our emergency room because of the sudden development of motor weakness and voiding difficulty. Lumbar magnetic resonance image revealed extradural fat compressing the cauda equina. Urgent decompression via posterior laminectomy and excision of excess epidural fat resulted in an immediate symptom improvement.
Back Pain ; Cauda Equina* ; Decompression ; Emergency Service, Hospital ; Humans ; Laminectomy ; Lipomatosis* ; Low Back Pain ; Neurologic Manifestations ; Polyradiculopathy* ; Young Adult