title: Neurocognitive features distinguishing primary central nervous system lymphoma from other possible causes of rapidly progressive dementia.
abstract: OBJECTIVE
  Define the neurocognitive features of primary central nervous system lymphoma (PCNSL) presenting with dementia, and compare with other causes of rapidly progressive dementia (RPD).
  
  
  BACKGROUND
  PCNSL can present as an RPD. Differentiating PCNSL from other RPDs is critical because lymphomatous dementia may be reversible, and untreated PCNSL is fatal.
  
  
  METHODS
  We performed a meta-analysis of case reports of dementia from PCNSL (between 1950 and 2013); 20 patients (14 with lymphomatosis cerebri) met our criteria. We compared these patients to a case series of patients with RPD from Creutzfeldt-Jakob disease and other non-PCNSL etiologies (Sala et al, 2012. Alzheimer Dis Assoc Disord. 26:267-271).
  
  
  RESULTS
  Median age was 66 years (range 41 to 81); 70% were men. Time from symptom onset to evaluation was <6 months in 65%. No patients had seizures; 5% had headaches; 45% had non-aphasic speech difficulty. There was significantly more memory impairment in patients with PCNSL than other RPDs and significantly less myoclonus and parkinsonism. Behavioral changes and cerebellar signs were not significantly different. Significantly more patients with PCNSL than other RPDs had white matter changes; significantly fewer had atrophy. Elevated CSF protein and pleocytosis were more frequent in PCNSL; patients with other RPDs tended to have normal CSF±14-3-3 protein.
  
  
  CONCLUSIONS
  Unlike patients with RPD from other causes, those with PCNSL commonly present with impaired memory, apathy, and abnormal speech and gait, without headache, seizure, or myoclonus. White matter changes and CSF abnormalities predominate. Improved clinical awareness of PCNSL can prompt earlier diagnosis and treatment.
authors: Departments of *Neurology ‡Psychiatry and Biobehavioral Sciences, David Geffen School of Medicine, University of California, Los Angeles, California †Neurobehavior Unit, Veterans Affairs Greater Los Angeles Healthcare System, Los Angeles, California.
authors: Mariel B
authors: MB
authors: Deutsch
authors: Mario F
authors: MF
authors: Mendez
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D057565:Apathy; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000523:psychology; D016543:Central Nervous System Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology*; D003071:Cognition; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000523:psychology; D003704:Dementia / Q000209:etiology*; D003937:Diagnosis, Differential; D018450:Disease Progression; D004569:Electroencephalography; D005260:Female; D005684:Gait; D006801:Humans; D008223:Lymphoma / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008569:Memory Disorders / Q000209:etiology; D008875:Middle Aged; D012189:Retrospective Studies; D013064:Speech Disorders / Q000209:etiology; D013997:Time Factors; D014057:Tomography, X-Ray Computed