Langerhans' cell histiocytosis and the nervous system

Burn, David J.; Watson, J. D. G.; Roddie, Mary E.; Chu, Anthony; Legg, N. J.; Frackowiak, R. S. J.

doi:10.1007/bf00867593

Cited by 20 publications

(8 citation statements)

References 36 publications

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“…The slow processing speed observed for JK, and to a lesser extent for AB, is also consistent with observations of other LCH patients [13]. Difficulties with short-term memory, formally assessed here, have been described by others, who have found that these difficulties did not resolve even when treatment seemed to be effective in other domains [14]. These difficulties will almost certainly impact the LCH child's ability to learn new material as well as to demonstrate knowledge and ability when assessed.…”

Section: Discussionsupporting

confidence: 91%

Neuropsychologic deficits in children with Langerhans cell histiocytosis

Whitsett

Kneppers

Coppes

et al. 1999

Med. Pediatr. Oncol.

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Background Manifestations of Langerhans cell histiocytosis (LCH) in children range from only a rash, to bony lesions accompanied by pain, to major organ disease. When the central nervous system (CNS) is affected, the LCH patient may exhibit signs and symptoms of hypothalamic and pituitary dysfunction (most often resulting in diabetes insipidus or other endocrinopathies) or more global neurologic and neuropsychologic sequelae. Surprisingly, researchers have only recently begun to examine the neuropsychologic manifestations of the disease, but early findings suggest that they may, in fact, be significant in a small percentage of children with LCH. Procedure We evaluated two CNS‐positive patients with LCH and long‐term intermittent treatments, using extensive neuropsychologic assessments, including intellectual functioning, memory, visual‐motor functioning, attention and concentration, sensory and motor performance, and gross academic achievement. Objective measures of behavior were obtained through parental report. Neuroradiologic imaging was obtained concurrently with the neuropsychologic evaluations. Results The neuropsychologic assessments indicated significant deficits in a number of the measured areas of functioning. Global cognitive deficiencies in full‐scale IQ were identified, as were deficits in memory, attention/concentration, and perceptual‐organizational capabilities. Similarities were noted in the patterns of deficits obtained with both patients, despite differences in the pathophysiology of their disease. Behavioral functioning in both children had suffered, presumably in relation to the neuropsychologic deficits. There were radiologic findings of gross cerebellar white matter damage in one patient, in addition to focal (e.g., hypothalamic) lesions in the other. Conclusions LCH has an adverse impact on cognitive functions in some children with evidence of CNS involvement, and further study into the etiology, incidence, and means of remedial intervention is needed. Med. Pediatr. Oncol. 33:486–492, 1999. © 1999 Wiley‐Liss, Inc.

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Section: Discussionsupporting

confidence: 91%

Neuropsychologic deficits in children with Langerhans cell histiocytosis

Whitsett

Kneppers

Coppes

et al. 1999

Med. Pediatr. Oncol.

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“…Progressive neurologic abnormalities, such as subtle cerebellar ataxia and nystagmus, also have been shown to precede clinical diabetes insipidus by many years. [25][26][27] Similarly, pulmonary symptoms and spontaneous pneumothorax most likely are late symptoms that reflect more advanced disease. 21,28 Pulmonary LCH generally displays an insidious and often progressive natural history that makes determination of its time of onset very difficult.…”

Section: Natural History Of the Diseasementioning

confidence: 99%

Langerhans cell histiocytosis

Howarth

Gilchrist

Mullan

et al. 1999

Cancer

566

125

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“…The most prominent abnormality was an abnormal eating pattern and obesity; fi ve patients developed morbid obesity, which was diffi cult to control 6 . Further abnormalities, such as disturbances in thermoregulation and adipsia, can make DI diffi cult to treat and complicate the overall management of these problematic cases 14 . No such abnormalities were noted in our subject.…”

Section: Histiocytosis: An Uncommon Presentation With Hypopituitarismmentioning

confidence: 99%

Histiocytosis: An Uncommon Presentation with Hypopituitarism

Kota

Jammula

Tripathy

et al. 2012

J. Nepal Paedtr. Soc.

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Langerhans cell histiocytosis is a multi system disorder with a certain predilection for involving hypothalamic pituitary axis. We hereby report a 7 year old girl presenting with polyuria, polydipsia and growth retardation. The girl had a past history of pain in right hip joint and nodular region over chest. Water deprivation test confirmed the diagnosis of central diabetes inspidus. Other investigations revealed Growth hormone deficiency and central hypothyroidism. X-ray and MRI hip revealed absent right inferior pubic ramus with bone marrow biopsy confirming the diagnosis of histiocytosis. Patient was treated with nasal Arginine Vasopressin spray, subcutaneous growth hormone and oral thyroxine.

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Langerhans' cell histiocytosis and the nervous system

Cited by 20 publications

References 36 publications

Neuropsychologic deficits in children with Langerhans cell histiocytosis

Neuropsychologic deficits in children with Langerhans cell histiocytosis

Langerhans cell histiocytosis

Histiocytosis: An Uncommon Presentation with Hypopituitarism

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