Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The brains of eight patient with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus. An orbital extraconal mass was noted in one child with exopthalmus. This decreased in a follow-up study after Vinblastine therapy. The cerebellum was abnormal in two patients; the area of the dentate nuclei exhibited abnormal signal in one asymptomatic child and the MRI of another boy with a ten year history of progressive cerebellar dysfunction showed cerebral and cerebellar atrophy. Three of the five patients with deficiency of antidiuretic hormone (ADH) were studied both before and after the administration of intravenous Gadolinium-DTPA. In all three, after administration of the contrast agent, there was enhancement of involved areas.
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PMID:Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA. 239 58

We report on 3 patients with intracranial manifestations of Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of the hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI.
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PMID:[Intracranial manifestations of Langerhans-cell histiocytosis. Nuclear magnetic resonance findings]. 784 76

Histiocytosis X is a disease of unknown etiology, characterized by a mass of proliferating histiocytes, plasma cells and inflammatory cells foaming a granuloma within the reticuloendothelial elements of any organ in the body. In the central nervous system (CNS), hypothalamic disorder of histiocytosis X is often found, but histiocytosis X in other regions is quite rare. We report a case of a 5-year-old girl with histiocytosis X of the zygoma presenting as a mass lesion in the tentorium cerebelli. A computed tomographic (CT) scan demonstrated a tumor at the left tentorial region, extending along the dura mater of the tentorium cerebelli. Magnetic resonance imaging (MRI) revealed a low signal intensity region on both T1 and T2-weighted images. MRI with Gd-DTPA showed a homogeneous enhanced mass extending to right and inferior sites with a thickened tentorium. As the thickened dura matter continued from the left middle fossa to the mass lesion, the tumor was considered to arise from the left zygoma and extend to the tentorium cerebelli. CNS extension of histiocytosis X is manifested either as (1) the cerebral type or (2) the dural type. Many cases of cerebral type histiocytosis X including hypothalamic disorder have been reported. Only 6 cases of the dural type of histiocytosis X have been described. Although the lesions of the cerebral type of histiocytosis X show prolonged T1 and T2 values on MRI, the MRI findings of the dural type have not been reported. The present case is the first report of the appearance of the lesion on MRI.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of dural type of histiocytosis X presenting as a mass lesion in the tentorium cerebelli]. 819 35

Two patients with spinal Langerhans cell granulomatosis were followed by magnetic resonance imaging, and the observed signal changes were detailed. Both patients had no neurologic abnormalities but back pain. They were treated by immobilization with a brace and bed rest. Their back pain disappeared after about a month. At the first visit, the tumorous lesions involving vertebrae showed mainly low signal intensity on T1-weighted imaging (T1WI) and high intensity on T2-weighted imaging (T2WI). They were clearly enhanced by gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA). Their original intensities changed to iso-intensities on both T1WI and T2WI without enhancement by Gd-DTPA after a year. These signal changes might indicate the healing stage of the involved vertebrae and recommend the removal of the spinal brace.
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PMID:Signal intensity changes on MRI during the healing process of spinal Langerhans cell granulomatosis: report of two cases. 1568 60

Eosinophilic granuloma is the localized form of Langerhans' cell histiocytosis. There are several reports of magnetic resonance (MR) imaging of eosinophilic granuloma of the skull, however there are few reports about dural enhancement. We report an operative case of eosinophilic granuloma of the skull with dural invasion. A 42-year old man was admitted to our hospital. He was neurologically intact and there were no other osseous or soft tissue lesions. CT showed an osteolytic lesion in the left parietal bone. MR images showed the lesion as isointense on T1-weighted, and high intense on T2-weighted images. T1-weighted images with Gd-DTPA demonstrated the mass which was enhanced with dural surface and subgaleal tissue. The angiogram demonstrated a tumor stain fed by the left occipital artery. Bone scintigraphy demonstrated a solitary lesion showing peripheral uptake with a central defect. The patient underwent craniectomy with removal of the dura and a subgaleal lesion. Histological examination revealed characteristic eosinophilic granuloma with dural invasion. No recurrence of the lesion was demonstrated 6 months after surgery.
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PMID:[Operative case of eosinophilic granuloma of the skull with dural invasion]. 1834 Oct 13