Neurological involvement and characterization in acquired hemophagocytic lymphohistiocytosis in adulthood
| Autor: | Sean Gratton, Carlo Tornatore, Brett Theeler, Tasha R. Powell, Faria Amjad, Jason S. Hawley |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Pediatrics Encephalopathy Lymphohistiocytosis Hemophagocytic Young Adult Non-Langerhans cell histiocytosis Cerebrospinal fluid Neuroimaging Seizures Edema medicine Humans Aged Retrospective Studies Brain Diseases Hemophagocytic lymphohistiocytosis business.industry Brain Middle Aged medicine.disease Hemiparesis Neurology Spastic tetraparesis Female Neurology (clinical) medicine.symptom business |
| Zdroj: | Journal of the Neurological Sciences. 357:136-142 |
| ISSN: | 0022-510X |
| DOI: | 10.1016/j.jns.2015.07.017 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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