Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH)

Phillip Ruppert, Emily C. Edmonds, Michael Brook, Suzanne Musil, S. Duke Han*

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

We present a case of an individual diagnosed with hemophagocytic lymphohistiocytosis (HLH), an extremely rare and commonly fatal disorder characterized by rapid dysregulation of immune system processes. Typical age of onset is in childhood, with adult-onset occurring less frequently. The pathophysiology of this condition is characterized by a hyperin-flammatory response with infiltration of visceral organs, lymph nodes, bone marrow, and the central nervous system. The clinical presentation has been documented in the extant medical literature. However, there appear to be no published reports of neuropsychological functioning in HLH patients. The patient we present here is a 28-year-old woman with 16 years of education who developed HLH subsequent to systemic lupus erythematosus flare-up. She was initially comatose for 3 weeks. Acute MRI reported multiple subcortical abnormalities, including the brainstem. The patient underwent chemotherapy, immunosup-presant, and steroid treatments. She underwent a neuropsychological evaluation at 2.5 and 7 months post initial presentation. Preliminary neuropsychological evaluation found impairments in motor abilities and aspects of executive functions. Subsequent evaluation showed improved executive function and relative sparing of higher-order cognitive abilities, but continued impairment on motor tests. To our knowledge this is the first study to report neuropsychological data for an adult diagnosed with HLH.

Original languageEnglish (US)
Pages (from-to)1038-1052
Number of pages15
JournalClinical Neuropsychologist
Volume26
Issue number6
DOIs
StatePublished - Aug 1 2012

Fingerprint

Hemophagocytic Lymphohistiocytosis
Aptitude
Executive Function
Immune System Phenomena
Multiple Abnormalities
Coma
Age of Onset
Systemic Lupus Erythematosus
Brain Stem
Central Nervous System
Lymph Nodes
Bone Marrow
Steroids
Education
Drug Therapy
Onset
Evaluation
Impairment

Keywords

  • Executive functions
  • HLH
  • Hemophagocytic lymphohistiocytosis
  • Motor abilities

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Developmental and Educational Psychology
  • Clinical Psychology
  • Arts and Humanities (miscellaneous)
  • Psychiatry and Mental health

Cite this

Ruppert, Phillip ; Edmonds, Emily C. ; Brook, Michael ; Musil, Suzanne ; Duke Han, S. / Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH). In: Clinical Neuropsychologist. 2012 ; Vol. 26, No. 6. pp. 1038-1052.
@article{6ee165d9ae444c0cb2bf22a5cd37da55,
title = "Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH)",
abstract = "We present a case of an individual diagnosed with hemophagocytic lymphohistiocytosis (HLH), an extremely rare and commonly fatal disorder characterized by rapid dysregulation of immune system processes. Typical age of onset is in childhood, with adult-onset occurring less frequently. The pathophysiology of this condition is characterized by a hyperin-flammatory response with infiltration of visceral organs, lymph nodes, bone marrow, and the central nervous system. The clinical presentation has been documented in the extant medical literature. However, there appear to be no published reports of neuropsychological functioning in HLH patients. The patient we present here is a 28-year-old woman with 16 years of education who developed HLH subsequent to systemic lupus erythematosus flare-up. She was initially comatose for 3 weeks. Acute MRI reported multiple subcortical abnormalities, including the brainstem. The patient underwent chemotherapy, immunosup-presant, and steroid treatments. She underwent a neuropsychological evaluation at 2.5 and 7 months post initial presentation. Preliminary neuropsychological evaluation found impairments in motor abilities and aspects of executive functions. Subsequent evaluation showed improved executive function and relative sparing of higher-order cognitive abilities, but continued impairment on motor tests. To our knowledge this is the first study to report neuropsychological data for an adult diagnosed with HLH.",
keywords = "Executive functions, HLH, Hemophagocytic lymphohistiocytosis, Motor abilities",
author = "Phillip Ruppert and Edmonds, {Emily C.} and Michael Brook and Suzanne Musil and {Duke Han}, S.",
year = "2012",
month = "8",
day = "1",
doi = "10.1080/13854046.2012.702788",
language = "English (US)",
volume = "26",
pages = "1038--1052",
journal = "Clinical Neuropsychologist",
issn = "0920-1637",
publisher = "Swets & Zeitlinger",
number = "6",

}

Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH). / Ruppert, Phillip; Edmonds, Emily C.; Brook, Michael; Musil, Suzanne; Duke Han, S.

In: Clinical Neuropsychologist, Vol. 26, No. 6, 01.08.2012, p. 1038-1052.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH)

AU - Ruppert, Phillip

AU - Edmonds, Emily C.

AU - Brook, Michael

AU - Musil, Suzanne

AU - Duke Han, S.

PY - 2012/8/1

Y1 - 2012/8/1

N2 - We present a case of an individual diagnosed with hemophagocytic lymphohistiocytosis (HLH), an extremely rare and commonly fatal disorder characterized by rapid dysregulation of immune system processes. Typical age of onset is in childhood, with adult-onset occurring less frequently. The pathophysiology of this condition is characterized by a hyperin-flammatory response with infiltration of visceral organs, lymph nodes, bone marrow, and the central nervous system. The clinical presentation has been documented in the extant medical literature. However, there appear to be no published reports of neuropsychological functioning in HLH patients. The patient we present here is a 28-year-old woman with 16 years of education who developed HLH subsequent to systemic lupus erythematosus flare-up. She was initially comatose for 3 weeks. Acute MRI reported multiple subcortical abnormalities, including the brainstem. The patient underwent chemotherapy, immunosup-presant, and steroid treatments. She underwent a neuropsychological evaluation at 2.5 and 7 months post initial presentation. Preliminary neuropsychological evaluation found impairments in motor abilities and aspects of executive functions. Subsequent evaluation showed improved executive function and relative sparing of higher-order cognitive abilities, but continued impairment on motor tests. To our knowledge this is the first study to report neuropsychological data for an adult diagnosed with HLH.

AB - We present a case of an individual diagnosed with hemophagocytic lymphohistiocytosis (HLH), an extremely rare and commonly fatal disorder characterized by rapid dysregulation of immune system processes. Typical age of onset is in childhood, with adult-onset occurring less frequently. The pathophysiology of this condition is characterized by a hyperin-flammatory response with infiltration of visceral organs, lymph nodes, bone marrow, and the central nervous system. The clinical presentation has been documented in the extant medical literature. However, there appear to be no published reports of neuropsychological functioning in HLH patients. The patient we present here is a 28-year-old woman with 16 years of education who developed HLH subsequent to systemic lupus erythematosus flare-up. She was initially comatose for 3 weeks. Acute MRI reported multiple subcortical abnormalities, including the brainstem. The patient underwent chemotherapy, immunosup-presant, and steroid treatments. She underwent a neuropsychological evaluation at 2.5 and 7 months post initial presentation. Preliminary neuropsychological evaluation found impairments in motor abilities and aspects of executive functions. Subsequent evaluation showed improved executive function and relative sparing of higher-order cognitive abilities, but continued impairment on motor tests. To our knowledge this is the first study to report neuropsychological data for an adult diagnosed with HLH.

KW - Executive functions

KW - HLH

KW - Hemophagocytic lymphohistiocytosis

KW - Motor abilities

UR - http://www.scopus.com/inward/record.url?scp=84871878389&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84871878389&partnerID=8YFLogxK

U2 - 10.1080/13854046.2012.702788

DO - 10.1080/13854046.2012.702788

M3 - Article

C2 - 22784400

AN - SCOPUS:84871878389

VL - 26

SP - 1038

EP - 1052

JO - Clinical Neuropsychologist

JF - Clinical Neuropsychologist

SN - 0920-1637

IS - 6

ER -