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)

A 37-year-old man presented with recurrent secondary generalised seizures from right focal onset. An MRI scan of the brain revealed multiple contrast enhancing lesions mainly involving the frontal, parietal and temporal regions. The left frontotemporal lesion was biopsied and histopathology confirmed it to be a rare case of adult Langerhans cell histiocytosis. He was given a short course of oral corticosteroids. Two years later his clinical condition was much improved on antiepileptic therapy alone, and repeat MRI brain and MR spectroscopy showed significant resolution of the lesions.
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PMID:Langerhans cell histiocytosis presenting as adult onset epilepsy. 1462 91

Eosinophilic granuloma (EG) is a rare disease but is more common in adults than children. It's often self-limiting. Spinal involvement is rare. It is the localized and most benign form of Langerhans' cell histiocytosis (previously known as histiocytosis X), characterised by lytic lesions in one or more bones. Spontaneous resolution of vertebral body lesions is very rare. In this case, the patient had one EG in a cervical vertebra and a similar lesion in a lumbar vertebra. This case is important because it featured a symptomatic lesion in the cervical spine accompanied by an asymptomatic lesion in a lumbar vertebra. We treated the cervical lesion by surgical fusion and followed the lumbar lesion up conservatively, with the patient in a corset. After 8 years of follow-up, control MRI showed that the lumbar lesion had spontaneously resolved.
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PMID:Spontaneous resolution of lumbar vertebral eosinophilic granuloma. 1496 50

The study was performed to compare whole-body short time inversion recovery (STIR) MR imaging and (99m)Tc-methylene diphosphonate planar scintigraphy in the examination of children with suspected multifocal skeletal malignant lesions. Sixteen patients with known or suspected malignant skeletal disease underwent both whole-body STIR MR imaging and bone scintigraphy. The lesions were described and numbered according to scintigraphic evaluation criteria. Thus, 16 regions were analyzed in each patient for the comparison between the two modalities. Histology was proven in the primary malignant regions. Follow-up MRIs were registered. Scintigraphy and MRI follow-up were evaluated as gold standard. A total of 139 different lesions was observed by both modalities. Baseline whole-body MRI revealed 119 bone lesions in 256 possible sites (46.5%); scintigraphy revealed only 58 lesions (22.6%). Congruence was observed in only four patients (25%). According to the location of the lesion, correlation was observed in 39/139 lesions (28%). In all, 57.5% of the lesions were detected only by MRI and 14.5% of the lesions were detected only by scintigraphy. Whole-body MRI was more sensitive (P<0.001). Of all lesions numbered which could be separated in the initial MRI, whole-body MRI detected 178 lesions in the patients. The results suggest that whole-body MRI using a STIR sequence is an effective radiation free method for examination of children with suspected multifocal bone lesions. MRI showed more lesions than conventional (99m)Tc-methylene diphosphonate scintigraphy. Therefore, whole-body MRI may be feasible as a screening modality for metastatic and skip lesions in osteosarcoma, PNET, Ewing sarcoma and Langerhans cell histiocytosis in children.
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PMID:Comparison of whole-body STIR-MRI and 99mTc-methylene-diphosphonate scintigraphy in children with suspected multifocal bone lesions. 1524 16

Langerhans' cell histiocytosis (LCH) describes a group of conditions affecting the reticuloendothelial system. It includes the subtypes of Letterer-Siwe disease, Hand-Schuller-Christian disease and eosinophilic granuloma and most often presents in childhood. Intracranial involvement in LCH is usually restricted to the hypothalamic-pituitary axis or involves extra-axial extension from skull vault lesions. Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described and are not included in the magnetic resonance based classification system of neurological LCH. We present the MRI of a patient with multisystem LCH with spontaneous resolution of enhancing temporal lobe lesions.
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PMID:Spontaneous regression of supratentorial intracerebral Langerhans' cell histiocytosis. 1532 50

We present the case of a 43-year-old man diagnosed of insipid diabetes and hypogonadotropic hypogonadism in whom a right temporal bone lesion was observed in the control MRI, thus leading to his admission. A bone scintigraphy with 99mTc-HMDP showed increased uptake in the right temporal region with sphenoidal extension, coinciding with the MRI. In addition, increased uptake having less intensity and size was observed in the left temporal region. A biopsy on the newly appearing mouth ulcers in the jugal mucous was performed and showed Langerhan's cells, the diagnosis of histiocytosis X being confirmed. Six months later a new MRI indicated disease progression with bilateral temporal involvement. The bone scan findings in this case provided a more exact extension diagnosis of the disease than the MRI.
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PMID:[Usefulness of bone scintigraphy for staging in a case of histiocytosis of the temporal bone]. 1570 46

CNS involvement in Langerhans cell histiocytosis (LCH) is a rare but potentially devastating disorder. Different types of involvement have been described by MRI. CNS changes can have space-occupying or degenerative character. Little is known about the underlying neuropathology and pathophysiology. In our study we reviewed brain samples from 12 patients with LCH. The neuropathology findings were correlated with the MR morphology and the clinical presentation. By neuropathology, three types of lesions were distinguished. (i) Circumscribed granulomas within the brain's connective tissue space corresponded to tumorous lesions in the meninges or choroid plexus on MRI. They showed a composition similar to Langerhans granulomas in peripheral organs, with variable presence of CD1a-reactive cells and pronounced CD8-positive (+) T-cell infiltration. (ii) Granulomas occur within the brain's connective tissue spaces with partial infiltration of the surrounding CNS parenchyma by CD1a-reactive histiocytes. This was associated with profound T-cell-dominated inflammation and severe neurodegeneration, characterized by a nearly complete loss of neurons and axons, and gliosis. (iii) Neurodegenerative lesions lacking infiltration of CD1a+ cells, mainly affecting the cerebellum and brainstem, exhibited a profound inflammatory process dominated by CD8-reactive lymphocytes, associated with tissue degeneration, microglial activation and gliosis. Patients with such lesions showed different stages of neurological deterioration. This study indicates that neurodegeneration in LCH occurs on the background of a T-cell-dominated inflammatory process and is characterized by neuronal and axonal destruction with secondary demyelination, resembling paraneoplastic encephalitis.
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PMID:Neuropathology of CNS disease in Langerhans cell histiocytosis. 1570 14

CNS complications of LCH include "space occupying" lesions corresponding to histiocytic granulomas and "neurodegenerative" presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.
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PMID:MRI features of neurodegenerative Langerhans cell histiocytosis. 1662 52

Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (DI) due to infiltrative processes. We present a 4-year-old boy who was initially diagnosed as having central DI. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic DI. Since central DI and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X-ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis.
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PMID:Magnetic resonance imaging of thickened pituitary stalk proceeding to Langerhans cell histiocytosis in a child. 1663 38

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient.
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PMID:Liver involvement in Langerhans cell histiocytosis. 1681 98

Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.
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PMID:Endocrine disorders in pediatric - onset Langerhans Cell Histiocytosis. 1711 2


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