Neuropsychiatric Manifestations in Langerhans’ Cell Histiocytosis Disease: A Case Report and Review of the Literature

Abstract

The spectrum of the possible manifestations of Langerhans' cell histiocytosis in children is very wide, ranging from a simple rash to major multiorgan disease. There may be hypothalamic and pituitary dysfunction or more global neurological and neuropsychiatric manifestations when the central nervous system is affected. The cerebellum is preferentially affected for yet undetermined reasons. The clinical presentation includes motor dysfunction as well as cognitive, behavioral, and psychiatric expression of the ongoing neurodegeneration. We report a young patient with Langerhans' cell histiocytosis who underwent an unusual course of psychiatric deterioration.

Recognition and Treatment of Concurrent Active and Neurodegenerative Langerhans Cell Histiocytosis

“…CCAS has been described secondary to various cerebellar insults and is characterized by deficits in executive functioning, impaired visual-spatial abilities, emotional and behavioral changes, and speech/language problems. [18][19][20] Our case strengthens the need for consideration of CCAS in patients with cerebellar LCH. 20 …”

“…[18][19][20] Our case strengthens the need for consideration of CCAS in patients with cerebellar LCH. 20 …”

Histiocitose das células de Langerhans no sistema nervoso central: relato de caso incomum

“…A literatura mostra que existem muitas manifestações cranianas da HCL, incluindo espessamento do talo infundibular, comprometimento do plexo coroide, anormalidade da glândula pineal e alterações acentuadas da substância branca 10 . O espectro de manifestações neurológicas na HCL está relacionado com disfunção motora ou cerebelar e transtornos psiquiátricos 11 .…”

Neurodegeneration in the course of Langerhans cell histiocytosis

“…Behavioural, cognitive and mood dysfunctions have been depicted during several cerebellar disorders leading to the hypothesis of a cerebellar cognitive affective syndrome [3,9]. The cognitive deficits resulting from cerebellar pathology may be related to the disturbance of cerebellar connections to prefrontal regions.…”

“…Apart from the hypothalamic-pituitary region, the central nervous system is involved approximately in 4% of patients with LCH [2]. Clinically it usually presents with ataxia, nystagmus, dysarthria, spastic tetraparesis, pseudobulbar palsy or cognitive/behavioral alterations [3]. Cerebellum is involved in a specific but poorly understood way.…”