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Helmut Prosch

Publications and source records attributed to Helmut Prosch.

11 recordsLinked to original sources
PubMed2007

Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis.

BACKGROUND: Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined. PROCEDURE: In this single center study, 25 LCH patients observed for a median time of 10 years 3 months underwent a uniform thorough follow-up program including neuropsychological testing and electrophysiological evaluation. RESULTS: Overall permanent consequences were seen in 9 of 25 patients. Intracranial abnormalities were the most frequent including diabetes insipidus (DI) in seven patients, anterior pituitary deficiencies in five patients, and neurodegenerative CNS disease in five patients. No patient had overt neurological symptoms upon neurological evaluation, but psychological testing revealed subtle deficits in short-term auditory memory (STAM) in 14 patients. Brain stem evoked potentials showed abnormalities in four of nine tested patients, all of these four had neurodegeneration on MRI. CONCLUSION: Psychoneuroendocrine sequelae were found in an unexpectedly high number of patients in this single center study. Long-term follow-up focusing on such sequelae are important in LCH survivors, in order to detect early deficits, to monitor the evolution of the disease, and to provide specific support.

Adolescent↗
PubMed2006

Central diabetes insipidus: Is it Langerhans cell histiocytosis of the pituitary stalk? A diagnostic pitfall.

Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases. In pediatric and adolescent patients the most common causes for CDI are Langerhans cell histiocytosis (LCH) and germinoma. To avoid a potentially hazardous biopsy of the hypothalamic pituitary region it is recommended to evaluate patients with CDI carefully to identify potential extracranial lesions. Since LCH is the most common systemic disease that may cause CDI, special focus is paid to the identification of LCH lesions. We report on a 9(1/2) year old girl who presented with central diabetes insipidus and a thickening of the pituitary stalk on magnetic resonance imaging. Diagnostic workup revealed a history of recurrent ear infections and a compressed 6th thoracic vertebral body on radiographs. Based on these findings LCH was anticipated. Upon growth of the pituitary stalk lesion the patient was treated with LCH standard chemotherapy. After an initial shrinkage of the lesion, a further growth of the pituitary stalk lesion was observed and the tumor was resected. Histopathology revealed germinoma. This case underscores the importance of a istopathologically proven diagnosis in patients with HPR tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive.

Biopsy↗
PubMed2006

TB or not TB?

Explore the source record for details and available documents.

Adolescent↗
PubMed2006

Diagnosis of thoracic splenosis by ferumoxides-enhanced magnetic resonance imaging.

We report on a 56-year-old woman with a history of splenectomy and surgery of the left hemidiaphragm, who presented with multiple pleural and mediastinal mass lesions. On the basis of the patient's history of splenectomy and missing Howell-Jolly bodies in peripheral blood smears, splenosis was suspected. To confirm the presumptive diagnosis, ferumoxides-enhanced magnetic resonance was performed. As all lesions showed a pronounced signal loss on T2* sequences, the diagnosis of splenosis was made and was further confirmed by scintigraphy. The reported case illustrates that ferumoxides-enhanced magnetic resonance imaging may be a helpful tool for the evaluation of suspected splenosis.

Contrast Media↗
PubMed2006

Management of patients with small pulmonary nodules: a survey of radiologists, pulmonologists, and thoracic surgeons.

OBJECTIVE: The objective of our study was to survey recommendations for the management of small pulmonary nodules found on helical CT from radiologists, pulmonologists, and thoracic surgeons. MATERIALS AND METHODS: We surveyed 774 radiologists, 623 pulmonologists, and 101 thoracic surgeons. All are members of an associated Austrian scientific society and were asked for their recommendations in 13 hypothetical cases. Statistical analysis was focused on possible differences between medical specialities, residents and fellows, and male and female doctors and on a possible influence of the number of years in training or in medical practice. RESULTS: Complete surveys were returned from 91 radiologists, 74 pulmonologists, and 12 thoracic surgeons. The most frequent recommendation from radiologists was follow-up, whereas the most frequent recommendation from pulmonologists and surgeons was biopsy. The most frequently advised CT follow-up period was 3 months. Whereas thoracic surgeons preferred video-assisted thoracoscopy, radiologists and pulmonologists recommended less invasive procedures (bronchoscopy, CT-guided biopsy) to gain a specific diagnosis. CONCLUSION: There are significant differences in the management of small pulmonary nodules among radiologists, pulmonologists, and thoracic surgeons. Whereas radiologists tend to recommend short-term follow-up, pulmonologists and thoracic surgeons prefer a more aggressive approach, especially in patients with a higher likelihood of malignancy.

Austria↗
PubMed2005

Neuropathology of CNS disease in Langerhans cell histiocytosis.

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.

Adolescent↗
PubMed2005

Modern imaging methods for the assessment of Langerhans' cell histiocytosis-associated neurodegenerative syndrome: case report.

Langerhans' cell histiocytosis-associated neurodegenerative syndrome is an enigmatic manifestation, most often localized in the cerebellum and the basal ganglia. Its pathophysiologic basis is poorly understood, and effective treatment strategies are currently missing. Modern imaging modalities offer the possibility of shedding further light on this puzzling disease in a noninvasive way. We report on a 12-year-old boy with a Langerhans' cell histiocytosis-associated neurodegenerative syndrome who underwent a thorough evaluation with different modern imaging methods in addition to routine brain magnetic resonance imaging (MRI) to analyze their informative value for this condition. Additional imaging included positron emission tomography using [18F]fluorodeoxyglucose (FDG-PET), single photon emission computed tomography using [123I]2beta-carbomethoxy-3beta-(4-iodophenyl)tropane and [123I]iodobenzamide, and magnetic resonance spectroscopy. The potential relevance of each method for neurodegenerative Langerhans' cell histiocytosis is discussed based on the results obtained, and a review of the literature is made. The case underlines the fact that MRI undoubtedly possesses the major role in the diagnostic evaluation and monitoring of Langerhans' cell histiocytosis-associated neurodegenerative syndrome. FDG-PET and magnetic resonance spectroscopy findings were in good correlation with the MRI results. In particular, magnetic resonance spectroscopy could provide a valuable diagnostic tool in addition to MRI in the early detection and evaluation of the neurodegenerative component of this disease.

Brain↗
PubMed2004

Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis.

BACKGROUND: Diabetes insipidus (DI) is the most frequent sequela in Langerhans cell histiocytosis (LCH). The clinical relevance and therapeutic impact of brain magnetic resonance imaging (MRI) findings in LCH patients with LCH during the disease course is unclear. PROCEDURE: In this retrospective study, we reviewed 113 brain MRIs from 59 DI patients, in 17 of these serial follow up MR-examination findings were correlated to the clinical course and therapy. RESULTS: At DI diagnosis, 71% patients showed a thickened stalk, in 24% the stalk was still thickened on MRIs done more than 5 years after DI onset, and in two patients the stalk was already thickened several months before DI onset. The changes of the pituitary stalk thickness were highly variable and did not clearly correlate with the treatment. Regression of pituitary thickness on MRI did not concur with clinical recovery of DI, which persisted in all but one patient with initially partial DI. The occurrence of anterior pituitary hormone deficiencies appeared to be linked to a thickening of the stalk at DI diagnosis. LCH-lesions in the craniofacial bones were seen in 75% DI patients, and 76% of DI patients with follow up MRIs done 5 years or longer after DI diagnosis had parenchymal neurodegenerative brain changes. CONCLUSIONS: Our study indicates that repeated MR-examinations in DI patients are of limited value for assessing a response to therapy in the pituitary stalk, but are important for the monitoring of craniofacial bone lesions and for the detection of parenchymal CNS disease.

Adolescent↗
PubMed2004

Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis.

BACKGROUND AND OBJECTIVES: Central diabetes insipidus (CDI) is a rare disorder associated with various underlying diseases. Among the systemic diseases that may cause CDI, Langerhans cell histiocytosis (LCH) is the most common. Therefore, in patients with endocrinologically proven CDI, a comprehensive diagnostic evaluation is crucial to identify possible extracranial sites of LCH. The goal of the diagnostic evaluation is to yield histopathological proof of the underlying disease. If possible, this histopathological proof should be provided by a biopsy of extracranial lesions to avoid a potentially hazardous biopsy of the pituitary stalk. STUDY DESIGN: In this retrospective study we included 54 patients registered at the LCH study reference center in whom the onset of CDI preceded the diagnosis of LCH, and we investigated their presentation and course to define a clinical pattern characteristic for LCH. RESULTS: In 49/54 patients (91%) the detection and biopsy of extracranial lesions led to the diagnosis of LCH. The most frequently involved organs were bones, skin, and lungs; 86% of the patients with bone lesions had skull lesions. In 18% of the patients extracranial lesions were already found at presentation of CDI, in another 51% of the patients extracranial lesions were found within 1 year from onset of CDI. CONCLUSIONS: These observations underline that a comprehensive search for extracranial lesions at presentation and during the first year thereafter may help to achieve a specific diagnosis without a pituitary stalk biopsy.

Adolescent↗
PubMed2004

MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis.

BACKGROUND AND PURPOSE: Intracranial manifestations of Langerhans cell histiocytosis (LCH) are underestimated in frequency and diversity. We categorized the spectrum of MR imaging changes in LCH. METHODS: We retrospectively reviewed 474 MR images in 163 patients with LCH and 55 control subjects. Lesions were characterized by anatomic region and signal intensity. Brain atrophy was assessed. RESULTS: We noted osseous lesions in the craniofacial or skull bones in 56% of patients, meningeal lesions in 29%, and choroid-plexus involvement in 6%. In the hypothalamic-pituitary region, infundibular thickening occurred in 50%; pronounced hypothalamic mass lesions in 10%; and infundibular atrophy in 29%. The pineal gland had a cystic appearance in 28%, and pineal-gland enlargement (>10 mm) was noted in 14%. Nonspecific paranasal-sinus or mastoid opacifications were seen in 55% of patients versus 20% of controls, and accentuated Virchow-Robin spaces occurred in 70% of patients versus 27% of controls (P <.001). Intra-axial, white-matter parenchymal changes resulted in a leukoencephalopathy-like pattern in 36%. Enhancing lesions in a vascular distribution were noted in 5%. Gray-matter changes suggestive of neurodegeneration were identified in the cerebellar dentate nucleus in 40% and in the supratentorial basal ganglia in 26%. All patients with neurodegenerative lesions had lesions in the extra-axial spaces. Cerebral atrophy was found in 8%. CONCLUSION: In LCH, cranial and intracranial changes at MR imaging include 1) lesions of the craniofacial bone and skull base with or without soft-tissue extension; 2) intracranial, extra-axial changes (hypothalamic-pituitary region, meninges, circumventricular organs); 3) intracranial, intra-axial changes (white matter and gray matter); and 4) cerebral atrophy.

Adolescent↗