---
title: "Impulsivity, Compulsivity, and Social Inappropriateness in Cerebellar Langerhans Cell Histiocytosis"
id: "pubmed-42768142"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42768142"
content_type: "clinical_feed_article"
specialty: "Critical Care"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42768142/"
doi: "10.1007/s12311-026-02085-0"
published_at: "2026-09-22T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Impulsivity, Compulsivity, and Social Inappropriateness in Cerebellar Langerhans Cell Histiocytosis
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42768142
- **Specialty:** [Critical Care](https://medichelpline.com/clinical-feed/critical-care.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42768142/)
- **DOI:** [10.1007/s12311-026-02085-0](https://doi.org/10.1007%2Fs12311-026-02085-0)
- **Published At:** 2026-09-22T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- This case series reports six patients with **Langerhans cell histiocytosis (LCH)** who developed neurological and neuropsychiatric disturbances associated with **cerebellar** and brainstem lesions on MRI. - The authors focused on underrecognized behavioral features: **impulsivity**, **compulsivity**, and **social inappropriateness**, alongside cognitive impairment and ataxia. - Patients with more extensive cerebellar and brainstem involvement exhibited more prominent impulsive, compulsive, and socially inappropriate behaviors; those with isolated dentate abnormalities had milder attentional and impulsive symptoms. - Reported behavioral manifestations included hyperactivity, poor impulse control, hypersexuality, pathological gambling, substance abuse, aggression, and social inappropriateness. - Serial imaging was reviewed when available; in one patient a temporal parallel was documented between psychiatric exacerbation and progression of cerebellar lesions, with later clinical and radiological improvement after **immunomodulatory treatment**. - A systematic literature review was performed to identify previously reported neuropsychiatric manifestations in cerebellar LCH, expanding the recognized phenotype. - The findings support a possible role for cerebello-limbic and broader **cerebellar networks** in these behaviors and underscore the need for systematic behavioral assessment, longitudinal follow-up, and further study of targeted therapies. - The authors conclude these features are underrecognized in cerebellar LCH and recommend attention to impulsive-compulsive symptoms in clinical evaluation and research.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliations * 1 Departamento de Neurología, Fleni, Sección de Movimientos Anormales, Buenos Aires, Argentina. mrossi@fleni.org.ar. * 2 Instituto Fleni-CONICET (INEU), Buenos Aires, Argentina. mrossi@fleni.org.ar. * 3 Departamento de Diagnóstico por Imágenes, Fleni, Buenos Aires, Argentina. * 4 Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina. * 5 Fleni, Buenos Aires, Argentina. * 6 Department of Neurology, Baylor College of Medicine, Houston, TX, USA. * 7 Department of Neurology, Columbia University Irving Medical Center and the New York Presbyterian Hospital, New York, NY, USA. * 8 Initiative for Columbia Ataxia and Tremor, Columbia University Irving Medical Center, New York, NY, USA. * 9 Departamento de Neurooncología, Fleni, Buenos Aires, Argentina. * 10 Servicio de Neurooncología, Fleni, Buenos Aires, 2325, 1428, Montañeses, Argentina. * PMID: **42768142** * DOI: [ 10.1007/s12311-026-02085-0 ](https://doi.org/10.1007/s12311-026-02085-0) Item in Clipboard Case Reports # Impulsivity, Compulsivity, Social Inappropriateness, and Other Neuropsychiatric Features in Cerebellar Histiocytosis Malco Rossi et al. Cerebellum. 2026. Show details Display options Display options Format Abstract PubMed PMID Cerebellum Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Cerebellum%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Cerebellum%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) . 2026 Sep 22;25(5):142. doi: 10.1007/s12311-026-02085-0. ### Authors [Malco Rossi](https://pubmed.ncbi.nlm.nih.gov/?term=Rossi+M&cauthor_id=42768142)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-1 "Departamento de Neurología, Fleni, Sección de Movimientos Anormales, Buenos Aires, Argentina. mrossi@fleni.org.ar.")[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-2 "Instituto Fleni-CONICET \(INEU\), Buenos Aires, Argentina. mrossi@fleni.org.ar."), [Hernan Chaves](https://pubmed.ncbi.nlm.nih.gov/?term=Chaves+H&cauthor_id=42768142)[ 3 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-3 "Departamento de Diagnóstico por Imágenes, Fleni, Buenos Aires, Argentina."), [Veronica Navarrine](https://pubmed.ncbi.nlm.nih.gov/?term=Navarrine+V&cauthor_id=42768142)[ 4 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-4 "Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina.")[ 5 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-5 "Fleni, Buenos Aires, Argentina."), [Santiago Volmaro](https://pubmed.ncbi.nlm.nih.gov/?term=Volmaro+S&cauthor_id=42768142)[ 4 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-4 "Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina.")[ 5 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-5 "Fleni, Buenos Aires, Argentina."), [Chi-Ying R Lin](https://pubmed.ncbi.nlm.nih.gov/?term=Lin+CR&cauthor_id=42768142)[ 6 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-6 "Department of Neurology, Baylor College of Medicine, Houston, TX, USA."), [Sheng-Han Kuo](https://pubmed.ncbi.nlm.nih.gov/?term=Kuo+SH&cauthor_id=42768142)[ 7 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-7 "Department of Neurology, Columbia University Irving Medical Center and the New York Presbyterian Hospital, New York, NY, USA.")[ 8 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-8 "Initiative for Columbia Ataxia and Tremor, Columbia University Irving Medical Center, New York, NY, USA."), [Blanca Diez](https://pubmed.ncbi.nlm.nih.gov/?term=Diez+B&cauthor_id=42768142)[ 9 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-9 "Departamento de Neurooncología, Fleni, Buenos Aires, Argentina.")[ 10 ](https://pubmed.ncbi.nlm.nih.gov/42768142/#short-view-affiliation-10 "Servicio de Neurooncología, Fleni, Buenos Aires, 2325, 1428, Montañeses, Argentina.") ### Affiliations * 1 Departamento de Neurología, Fleni, Sección de Movimientos Anormales, Buenos Aires, Argentina. mrossi@fleni.org.ar. * 2 Instituto Fleni-CONICET (INEU), Buenos Aires, Argentina. mrossi@fleni.org.ar. * 3 Departamento de Diagnóstico por Imágenes, Fleni, Buenos Aires, Argentina. * 4 Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina. * 5 Fleni, Buenos Aires, Argentina. * 6 Department of Neurology, Baylor College of Medicine, Houston, TX, USA. * 7 Department of Neurology, Columbia University Irving Medical Center and the New York Presbyterian Hospital, New York, NY, USA. * 8 Initiative for Columbia Ataxia and Tremor, Columbia University Irving Medical Center, New York, NY, USA. * 9 Departamento de Neurooncología, Fleni, Buenos Aires, Argentina. * 10 Servicio de Neurooncología, Fleni, Buenos Aires, 2325, 1428, Montañeses, Argentina. * PMID: **42768142** * DOI: [ 10.1007/s12311-026-02085-0 ](https://doi.org/10.1007/s12311-026-02085-0) Item in Clipboard Cite Display options Display options Format Abstract PubMed PMID ## Abstract Langerhans cell histiocytosis (LCH) may present with cerebellar and brainstem lesions associated with ataxia, cognitive impairment, and behavioral symptoms. While neuropsychiatric manifestations have been reported in LCH, little attention has been paid to impulsivity, compulsivity, and social inappropriateness in the context of cerebellar involvement. We describe six patients with multisystem LCH who developed neurological and psychiatric disturbances in association with cerebellar abnormalities on magnetic resonance imaging. Clinical, neuropsychological, and neuroimaging findings were reviewed, with a particular focus on impulsive-compulsive symptoms and social behavior. When serial imaging was available, the temporal relationship between symptom severity and cerebellar lesion evolution was reviewed. In addition, a systematic literature review was performed to identify previously reported neuropsychiatric manifestations associated with cerebellar LCH. Patients with more extensive cerebellar and brainstem involvement showed more prominent impulsive, compulsive, and socially inappropriate behaviors, whereas patients with restricted dentate abnormalities showed milder attentional and impulsive symptoms. Behavioral manifestations included hyperactivity, poor impulse control, hypersexuality, pathological gambling, substance abuse, aggression, and social inappropriateness. A notable finding was the temporal clinical-radiological association documented in one patient, the only case with neuroimaging obtained during and soon after psychiatric exacerbation. In this patient, psychiatric worsening paralleled progression of cerebellar lesions and later improved clinically and radiologically after immunomodulatory treatment. These cases expand the neuropsychiatric phenotype of cerebellar LCH by highlighting impulsivity, compulsivity, and social inappropriateness as underrecognized manifestations. Our findings support a possible contribution of cerebellar networks, particularly cerebello-limbic circuitry, to these behaviors and underscore the need for systematic behavioral assessment, longitudinal follow-up, and further studies on targeted therapeutic approaches. **Keywords:** Cerebellum; Compulsive Behavior; Impulsive Behavior; Langerhans Cell Histiocytosis; Neurodegenerative Langerhans Cell Histiocytosis; Neuropsychiatric Symptoms. © 2026. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. [PubMed Disclaimer](https://pubmed.ncbi.nlm.nih.gov/disclaimer/) ## Conflict of interest statement Declarations. Competing interests: The authors declare no competing interests. ## References 1. 1. Rossi M, Stephen CD, Damásio J, Pedroso JL, Kuo SH, Lin CYR, et al. Unravelling the global tapestry of genetic ataxias: Epidemiology and genetic testing approaches. Mov Disord. 2025;40(9):1805–20. - [PubMed](https://pubmed.ncbi.nlm.nih.gov/40682316/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/12291621/) 2. 1. Lin CYR, Kuo SH. Ataxias: Hereditary, acquired, and reversible etiologies. Semin Neurol. 2023;43(1):48–64. - [PubMed](https://pubmed.ncbi.nlm.nih.gov/36828010/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/10354687/) 3. 1. Kuo SH, Ataxia. Continuum (Minneap Minn). 2019;25(4):1036–54. - [PubMed](https://pubmed.ncbi.nlm.nih.gov/31356292/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/7339377/) 4. 1. A multicentre retrospective. survey of Langerhans’ cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans’ Cell Histiocytosis Study Group. Arch Dis Child. 1996;75(1):17–24. 5. 1. Sconocchia T, Foßelteder J, Sconocchia G, Reinisch A. Langerhans cell histiocytosis: current advances in molecular pathogenesis. Front Immunol. 2023;14:1275085. - [PubMed](https://pubmed.ncbi.nlm.nih.gov/37965340/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/10642229/) Show all 69 references ## Publication types * Case Reports Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Case+Reports%22%5Bpt%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Case+Reports) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Systematic Review Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Systematic+Review%22%5Bpt%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Systematic+Review) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) ## MeSH terms * Adolescent Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Adolescent%22%5BMeSH%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Adolescent) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Adult Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Adult%22%5BMeSH%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Adult) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Cerebellar Diseases* / complications Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Cerebellar+Diseases%2Fcomplications%22%5BMAJR%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Cerebellar+Diseases) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Cerebellar Diseases* / diagnostic imaging Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Cerebellar+Diseases%2Fdiagnostic+imaging%22%5BMAJR%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Cerebellar+Diseases) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Cerebellar Diseases* / pathology Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Cerebellar+Diseases%2Fpathology%22%5BMAJR%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Cerebellar+Diseases) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42768142/) * Cerebellar Dis
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