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 Scopus citations


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
Issue number6
StatePublished - Aug 1 2012


  • 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

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