title: Neurological presentation of hemophagocytic lymphohistiocytosis.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory disorder arising from defects in critical regulatory pathways responsible for termination of inflammatory response. We are presenting a case report of a 20-year-old male, admitted to the Department of Neurology because of left lower limb weakness and balance disturbances. After a few days of hospitalization, fever occurred. Laboratory tests revealed anemia, neutropenia, lymphopenia, and thrombocytopenia. The clinical course and laboratory tests results confirmed the diagnosis of HLH. In our opinion, the disorder in the presented case occurred due to severe chronic active Epstein-Barr virus infection syndrome. We are presenting the case of pure neurological onset of hemophagocytic lymphohistiocytosis in an adult patient. Hemophagocytic lymphohistiocytosis, initially presenting with neurological symptoms, can occur in adult patients with irrelevant family history. It is a life-threatening but potentially curable condition requiring proper diagnostic and treatment management.
authors.affiliation: Department of Neurology, Jagiellonian University Medical College, Krakow, Poland. Electronic address: kamilaanna@op.pl.
authors.forename: Żur-Wyrozumska
authors.initials: ŻW
authors.lastname: Kamila
authors.affiliation: Department of Neurology, Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Rog
authors.initials: R
authors.lastname: Teresa
authors.affiliation: Department of Hematology, Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Mensah-Glanowska
authors.initials: MG
authors.lastname: Patrycja
authors.affiliation: Department of Neurology, Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Szczudlik
authors.initials: S
authors.lastname: Andrzej
mesh_terms: D000914:Antibodies, Viral / Q000134:cerebrospinal fluid; D001766:Blindness / Q000209:etiology; D016572:Cyclosporine / Q000627:therapeutic use; D004279:DNA, Viral / Q000134:cerebrospinal fluid; D003907:Dexamethasone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004844:Epistaxis / Q000209:etiology; D005047:Etoposide / Q000627:therapeutic use; D004854:Herpesvirus 4, Human / Q000276:immunology / Q000302:isolation & purification; D006801:Humans; D007244:Infectious Mononucleosis / Q000134:cerebrospinal fluid / Q000150:complications; D051359:Lymphohistiocytosis, Hemophagocytic / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000209:etiology*; D004856:Postural Balance; D012678:Sensation Disorders / Q000209:etiology*; D055815:Young Adult
chemical_list: D000914:Antibodies, Viral; D004279:DNA, Viral; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine
keywords: Epstein–Barr virus infection; Hemophagocytic lymphohistiocytosis; Polyneuropathy