Book contents
- Frontmatter
- Contents
- List of contributors
- Preface
- 1 Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology
- 2 The diagnostic histopathology of Langerhans cell histiocytosis
- 3 Histiocyte function and development in the normal immune system
- 4 The immunological basis of Langerhans cell histiocytosis
- 5 The genetics of Langerhans cell histiocytosis
- 6 Langerhans cell histiocytosis: a clinical update
- 7 Histiocytosis of the skin in children and adults
- 8 Langerhans cell histiocytosis of bone
- 9 Special aspects of Langerhans cell histiocytosis in the adult
- 10 Adult lung histiocytosis
- 11 Central nervous system disease in Langerhans cell histiocytosis
- 12 The treatment of Langerhans cell histiocytosis
- 13 Treatment of relapsed and/or refractory Langerhans cell histiocytosis
- 14 Late effects of Langerhans cell histiocytosis and its association with malignancy
- 15 Uncommon histiocytic disorder: the non-Langerhans cell histiocytoses
- 16 The histopathology of hemophagocytic lymphohistiocytosis
- 17 Genetics and pathogenesis of hemophagocytic lymphohistiocytosis
- 18 Clinical aspects and therapy of hemophagocytic lymphohistiocytosis
- 19 Secondary haemophagocytic syndromes associated with rheumatic diseases
- 20 Malignancies of the monocyte/macrophage system
- 21 Psychosocial aspects of the histiocytic disorders: staying on course under challenging clinical circumstances
- Index
- Plate section
11 - Central nervous system disease in Langerhans cell histiocytosis
Published online by Cambridge University Press: 27 August 2009
- Frontmatter
- Contents
- List of contributors
- Preface
- 1 Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology
- 2 The diagnostic histopathology of Langerhans cell histiocytosis
- 3 Histiocyte function and development in the normal immune system
- 4 The immunological basis of Langerhans cell histiocytosis
- 5 The genetics of Langerhans cell histiocytosis
- 6 Langerhans cell histiocytosis: a clinical update
- 7 Histiocytosis of the skin in children and adults
- 8 Langerhans cell histiocytosis of bone
- 9 Special aspects of Langerhans cell histiocytosis in the adult
- 10 Adult lung histiocytosis
- 11 Central nervous system disease in Langerhans cell histiocytosis
- 12 The treatment of Langerhans cell histiocytosis
- 13 Treatment of relapsed and/or refractory Langerhans cell histiocytosis
- 14 Late effects of Langerhans cell histiocytosis and its association with malignancy
- 15 Uncommon histiocytic disorder: the non-Langerhans cell histiocytoses
- 16 The histopathology of hemophagocytic lymphohistiocytosis
- 17 Genetics and pathogenesis of hemophagocytic lymphohistiocytosis
- 18 Clinical aspects and therapy of hemophagocytic lymphohistiocytosis
- 19 Secondary haemophagocytic syndromes associated with rheumatic diseases
- 20 Malignancies of the monocyte/macrophage system
- 21 Psychosocial aspects of the histiocytic disorders: staying on course under challenging clinical circumstances
- Index
- Plate section
Summary
Introduction
Central nervous system (CNS) involvement has been a well-known feature of Langerhans cell histiocytosis (LCH) since the early descriptions of the disease. Around the turn of the twentieth century, Hand–Schüller–Christian reported on patients with diabetes insipidus (DI), the clinical symptom of the most frequent type of CNS involvement (Schüller, 1915; Christian, 1920; Hand, 1921). Some years later, several authors described cases with ‘generalized xanthomatosis of Schüller–Christian type’ with cerebral involvement other than the infundibular region including isolated or multiple tumours, or demyelination, nerve cell destruction, and gliosis associated with a plethora of neurological symptoms (Chester and Kugel, 1932; Chiari, 1933; Davison, 1933; Feigin, 1956). During the last decade, in parallel to the more frequent routine use of modern imaging techniques, more and more LCH patients have been detected with CNS changes, some even in the absence of clinical symptoms (Greenwood et al., 1981; Graif and Pennock, 1986; Burn et al., 1992; Breidahl et al., 1993; Grois et al., 1993; Barthez et al., 2000). Magnetic resonance imaging (MRI) (or sometimes computed tomography, CT) is usually performed to monitor craniofacial lesions, or to evaluate patients with clinical endocrine deficiencies such as DI, growth retardation or pubertal abnormalities, or those with neurological or psychological problems.
As discussed later, histopathologically CNS involvement takes two forms, granulomas which morphologically and immunohistochemically are typical LCH lesions, and a neurodegenerative form in which no active LCH can be found.
- Type
- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 208 - 228Publisher: Cambridge University PressPrint publication year: 2005
- 5
- Cited by