Predominantly Cerebral Forms of Histiocytosis-X

Kepes, John J.; Kepes, Magda

doi:10.1097/00005072-197001000-00084

Cited by 7 publications

(13 citation statements)

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“…Histologically, the histiocytes showed a tendency to ingest plasmatocytes. thus reflecting their 'normal' function as debris-removing cells [14], Some atypical hyper trophic astrocytes in our material could lead to a suspicion of glioma, as also pointed out by Kepes and Kepes [14], The histio-and lymphocytic infiltration of the choroid plexuses found in our case did not match the massive xanthomatous infiltration described by Morello et a i [18].…”

Section: Discussioncontrasting

confidence: 37%

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Histiocytosis ‘X’ of the Central Nervous System

Iraci¹,

Chieco‐Bianchi²,

Giordano³

et al. 1979

Pediatr Neurosurg

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The case is presented of a child with early manifestations of Hand-Schüller-Christian disease limited to exophthalmos, and rapid final progression towards a clinical picture dominated by intracranial hypertension and cerebeliar involvement, caused by massive histiocytic infiltration of the cerebellum, combined with discrete subdural deposits of xanthomatous tissue and minor deposits in other parts of the central nervous system and other organs.

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Section: Discussioncontrasting

confidence: 37%

“…According to Kepes anti Kepes [14], there is a strict resemblance of cerebral HX with a predominant hypo thalamic involvement to Gegel's hypothalamic granuloma and related entities of the European literature. Müller and Orthner [20] found 19 cases of intra cerebral 'lipoid granulomatosis' in the literature.…”

Section: Discussionmentioning

confidence: 99%

Histiocytosis ‘X’ of the Central Nervous System

Iraci¹,

Chieco‐Bianchi²,

Giordano³

et al. 1979

Pediatr Neurosurg

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“…The histiocytic granulomatous tissue in these patients first described by G agel [5], is localized mainly in the hypo thalamus; patients therefore exhibit diabetes insipidus [7].…”

Section: Introductionmentioning

confidence: 78%

“…The neurological manifestations of histiocytosis X have been summarized by K epes and K epes [7], CNS involvement in histiocytosis X is generally easily recognized if the patients are also known to suffer from other mani festations of this disease such as visceral and skeletal. Diagnostic difficulties, however, arise if the process is entirely or predominantly localized in the CNS.…”

Section: Discussionmentioning

confidence: 99%

“…Diagnostic difficulties, however, arise if the process is entirely or predominantly localized in the CNS. It has been shown that the most common localization in the CNS is the hypothalamic region, especially the pituitary gland and the hypothalamus [7], This region is involved either by direct extension from a histiocytosis X focus in the sphenoid bone or it is the place of onset of the histiocytic in filtration. The characteristic clinical symptom of these patients is diabetes insipidus [7], Cranial nerve symptoms (n. opticus, n. acusticovestibularis) can also be observed during the course of this disease.…”

Section: Discussionmentioning

confidence: 99%

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Histiocytosis X in an Adult with Skin and Uncommon Central Nervous System Involvement

Amirdjazil¹,

Esca²,

Konrad³

1977

Dermatology

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CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH‐associated neurodegeneration and mass lesions

McClain

Picarsic

Chakraborty

et al. 2018

Cancer

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BACKGROUND: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. METHODS: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Additionally, the presence of BRAFV600E was tested in peripheral mononuclear blood cells (PBMCs) as well as brain biopsies from LCH-ND patients, and the response to BRAF-V600E inhibitor was evaluated in 4 patients with progressive disease. RESULTS: Osteopontin was the only consistently elevated CSF protein in patients with CNS-LCH compared with patients with other brain pathologies. BRAFV600E DNA was detected in CSF of only 2/20 (10%) cases, both with LCH-ND and active lesions outside the CNS. However, BRAFV600E+ PBMCs were detected with significantly higher frequency at all stages of therapy in LCH patients who developed LCH-ND. Brain biopsies of patients with LCH-ND demonstrated diffuse perivascular infiltration by BRAFV600E+ cells with monocyte phenotype (CD14+CD33+CD163+P2RY12−) and associated osteopontin expression. Three of 4 patients with LCH-ND treated with BRAF-V600E inhibitor experienced significant clinical and radiologic improvement. CONCLUSION: In LCH-ND patients, BRAFV600E+ cells in PBMCs and infiltrating myeloid/monocytic cells in the brain is consistent with LCH-ND as an active demyelinating process arising from a mutated hematopoietic precursor from which LCH lesion CD207+ cells are also derived. Therapy directed against myeloid precursors with activated MAPK signaling may be effective for LCH-ND.

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Predominantly Cerebral Forms of Histiocytosis-X

Cited by 7 publications

References 0 publications

Histiocytosis ‘X’ of the Central Nervous System

Histiocytosis ‘X’ of the Central Nervous System

Histiocytosis X in an Adult with Skin and Uncommon Central Nervous System Involvement

CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH‐associated neurodegeneration and mass lesions

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