Neuropsychologic deficits in children with Langerhans cell histiocytosis

Abstract

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.

Cerebral White Matter

“…An earlier report of a patient with LCH involving cerebellar WM also reported significant deficits in global cognitive scores, memory, attention and concentration, and perceptual-organizational capabilities, along with substantial emotional and behavioral problems. 183 These behaviors fall within the domain of the cerebellar cognitive affective syndrome and its neuropsychiatric manifestations, 182,184,185 and they probably reflect involvement of the nonmotor region of cerebellum in the posterior lobe.…”

Langerhans cell histiocytosis and human herpes virus 6 (HHV‐6), an analysis by real‐time polymerase chain reaction

“…1,2 The disease most commonly involves bone, [3][4][5][6][7][8][9][10] but it is also known to affect a variety of other organ systems. 4,[6][7][8][9][10][11][12][13][14][15][16] Orthopedic surgeons are likely to encounter patients with this disease because bone involvement is seen in 80-100% of cases. [3][4][5][6][7][8]10,14 Although the most common presentation is a solitary lesion of bone, multisystem disease can present in a variety of ways across a wide clinical spectrum.…”

Recovery of cognitive function in neuropsychiatric Langerhan’s cell histiocytosis

“…Marked deficits were also identified in attention, visual memory, and managing verbal interference, whilst basic language and other academic abilities were intact in the CNS‐affected group. The only report of longitudinal neuropsychological function in LCH, of an adolescent with significant cerebellar involvement assessed at 12 and 15 years, showed a significant decline in verbal and performance IQ, with full‐scale IQ dropping from 112 to 86 over the 3 year interval 8 …”

“…It has been suggested that LCH sufferers with cerebellar changes generally show a progressive clinical and neurological decline, 3,8–10 and LCH has been described as ‘a progressive neuropsychiatric disease’ 9 . The evidence for cognitive decline is, however, lacking, with a single case report constituting the evidence base.…”