title: Neurological involvement and characterization in acquired hemophagocytic lymphohistiocytosis in adulthood.
abstract: OBJECTIVE
  To describe the neurological and neuroradiological features of acquired hemophagocytic lymphohistiocytosis (HLH) in adulthood by reporting a series of cases.
  
  
  METHODS
  Ten consecutive patients who were diagnosed with HLH at Medstar Georgetown University Hospital and Walter Reed National Military Medical Center were evaluated for neurological involvement. All underwent clinical neurological evaluation, and when indicated CSF analysis and MR imaging of the brain. Data were gathered and analyzed retrospectively.
  
  
  RESULTS
  Seven of the ten patients with HLH had neurological involvement. Mean age at onset was 50 (range: 21 to 73). Four patients were males. Prominent clinical features included mild to severe encephalopathy and seizures. Other findings included hemiparesis and spastic tetraparesis. Neuroimaging revealed a wide spectrum of abnormalities including cortical and subcortical edema, gadolinium enhancement, hemorrhage, and diffusion restriction. Basal ganglia involvement was present in four out of seven patients. Three patients died due to multisystem organ failure, and the other patients displayed varying degrees of recovery.
  
  
  CONCLUSIONS
  The neurological features of acquired HLH in adults have not been previously reported. These seven patients demonstrate the spectrum of neurological involvement that can occur. The diagnosis of HLH should be considered in patients who are systemically ill with unexplained fevers and hyperferritinemia who have evidence of inflammation in the CNS.
authors.affiliation: Medstar Georgetown University Hospital, Department of Neurology, PHC, 7th Floor, 3800 Reservoir Road, NW, Washington, DC 20007, United States. Electronic address: seangratton@gmail.com.
authors.forename: Sean M
authors.initials: SM
authors.lastname: Gratton
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Building 19, 8901 Rockville Pike, Bethesda, MD 20889, United States. Electronic address: Tasha.R.Powell2.mil@health.mil.
authors.forename: Tasha R
authors.initials: TR
authors.lastname: Powell
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Building 19, 8901 Rockville Pike, Bethesda, MD 20889, United States; Walter Reed National Military Medical Center, John P. Murtha Cancer Center, Building 19, 8901 Rockville Pike, Bethesda, MD 20889, United States. Electronic address: btheeler@gmail.com.
authors.forename: Brett J
authors.initials: BJ
authors.lastname: Theeler
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Building 19, 8901 Rockville Pike, Bethesda, MD 20889, United States. Electronic address: Jason.S.Hawley.mil@health.mil.
authors.forename: Jason S
authors.initials: JS
authors.lastname: Hawley
authors.affiliation: Medstar Georgetown University Hospital, Department of Neurology, PHC, 7th Floor, 3800 Reservoir Road, NW, Washington, DC 20007, United States. Electronic address: Faria.S.Amjad@gunet.georgetown.edu.
authors.forename: Faria S
authors.initials: FS
authors.lastname: Amjad
authors.affiliation: Medstar Georgetown University Hospital, Department of Neurology, PHC, 7th Floor, 3800 Reservoir Road, NW, Washington, DC 20007, United States. Electronic address: tornatoc@gunet.georgetown.edu.
authors.forename: Carlo
authors.initials: C
authors.lastname: Tornatore
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012640:Seizures / Q000150:complications / Q000175:diagnosis; D055815:Young Adult
keywords: Basal ganglia; Central nervous system; Ferritin; Hemophagocytic lymphohistiocytosis; Neuroimaging; Neuroimmunology; Non-Langerhans cell histiocytosis