Sean M Gratton1, Tasha R Powell2, Brett J Theeler3, Jason S Hawley4, Faria S Amjad5, Carlo Tornatore6. 1. Medstar Georgetown University Hospital, Department of Neurology, PHC, 7th Floor, 3800 Reservoir Road, NW, Washington, DC 20007, United States. Electronic address: seangratton@gmail.com. 2. 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. 3. 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. 4. 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. 5. 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. 6. 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.
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.
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: Matthew R Woodward; Margaret S Ferris; Guillermo Rivell; Laura Malone; Tara M Dutta; Haroon Ahmad; Nicholas A Morris Journal: Neurohospitalist Date: 2021-09-09
Authors: Laura J Janke; Denise M Imai; Heather Tillman; Rosalinda Doty; Mark J Hoenerhoff; Jiajie J Xu; Zachary T Freeman; Portia Allen; Natalie Wall Fowlkes; Ilaria Iacobucci; Kirsten Dickerson; Charles G Mullighan; Peter Vogel; Jerold E Rehg Journal: Vet Pathol Date: 2020-11-19 Impact factor: 2.221