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 |
| Â | 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 |
| Â | 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 |
| Â | B12 Deficiency | Fatigue, macrocytic anemia, 'beefsteak' tongue, subacute combined degeneration of spinal cord | B12 |