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Table 2 Some systemic diseases that may lead to progressive cognitive impairment and dementia

From: Early-onset dementias: diagnostic and etiological considerations

System Examples Associated symptoms Diagnostic tests
CNS vascular disease Primary and secondary CNS vasculitis Headaches, focal neurological deficits MRI; cerebral angiography; CSF analysis; brain biopsy; connective tissue disease screen (for example, ANA, C3/C4, RF, c-ANCA and p-ANCA, ENA-4 screen, cryoglobulins)
Infection HIV-associated neurocognitive disorders Neurosyphilis Whipple's disease Variable, constitutional symptoms, with or without opportunistic infections Tabes dorsalis Gastrointestinal symptoms, weight loss, and polyarthralgia; oculomasticatory and oculofacial-skeletal myorhythmia in only 20%, but highly specific HIV screen; plasma HIV viral load; CD4 counts Serum RPR; MHA-TP; CSF VDRL Duodenal biopsy showing granular foamy macrophages with PAS-positive inclusions; CSF PCR for Tropheryma whipplei
Neoplastic Primary CNS lymphoma Headaches; focal neurological deficits CSF analysis; brain/meningeal biopsy
Autoimmune/paraneoplastic Hashimoto encephalitis Thyroid abnormalities Anti-microsomal antibody; anti-thyroglobulin antibody
  Limbic encephalitis Cancer (breast, lung, testicular, teratoma colon, gynecological) Anti-Hu, anti-Ri, anti-Yo, anti-Ma1, anti-Ma2, anti-VGKC, anti-CV2, anti-amphiphysin, anti-NMDAR, anti-AMPAR, anti-GAD antibodies
  Lupus with CNS involvement Multiple organ systems ANA; ENA; CSF analysis; MRI
Endocrine and nutritional Hypothyroidism Fatigue, weight gain, skin and hair changes, depression TSH; free T4
  B12 Deficiency Fatigue, macrocytic anemia, 'beefsteak' tongue, subacute combined degeneration of spinal cord B12
  1. AMPAR, 2-amino-3-(3-hydroxy-5-methyl-isoxazol-4-yl)propanoic acid receptors; ANA, antinuclear antibodies; ANCA, anti-neutrophil cytoplasmic antibodies; CNS, central nervous system; CSF, cerebrospinal fluid; ENA, extractable nuclear antigens; MHA-TP, microhemagglutination assay; MRI, magnetic resonance imaging; NMDAR, N-methyl-D-aspartate receptors; PAS, periodic acid-Schiff; RF, rheumatoid factor; RPR, Rapid Plasma Reagin; TSH, thyroid stimulating hormone; VDRL, Venereal Disease Research Laboratory test.