Table 1.
Encephalopathy phenotypes: cancers, immunological findings, and paraclinical data.
| Pt no. Age/Gender | Syndrome | Symptoms, signs | Cancer, infection, autoimmune | NIF, CSF | MRI | CSF WCC/Pro/OCB/Ab | Neurophysiology, neuropsychology | |
|---|---|---|---|---|---|---|---|---|
| 1. M/77 | ENC | AMS, auditory halluci, dysarthria | URI | HL | Br: deep WM T2 abn + enhancement | 35/N/0/0 | EEG: triphasic waves | |
| 2. F/47* | ENC | Anxiety, agitation, AMS, confusion, dyskinesias, coma | SCLC | AL | Br: hazy deep WM ↑T2 FLAIR | N/42/4/NMDA‐R | EEG: gen delta slowing, seizures | |
| 3. M/52* | ENC | Depression, anxiety, AMS | ‐ | AHL | Br: limbic & anterior temporal T2 FLAIR Abns | 16/72/8 | EEG: N | |
| 4. F/74 | ENC | AMS, AMS, visual disturbance (ophthalmitis) | Merkel Cell ca, pembrolizumab | AHL | Br: N | 11/150/5/CRMP5 | ‐ | |
| 5. M/76 | ENC | Rapid‐onset amnesia, gait apraxia | Prostate adenoca | H | Br: N | N | ‐ | |
| 6. M/40 | ENC | Headache, meningism, blurred vision, seizures | NHL | H | Br: fronto‐parietal T2 WM changes | 8/64/N | EEG: seizures | |
| 7. M/41 | ENC | AMS, depressed mood | Enterovirus | H | Br: N | 14/68/N/0 | ‐ | |
| 8. F/66 | ENC | AMS, apraxia eyelid opening & gait, coma, | HIV CD4 > 200 | H | Br: N | N | ‐ | |
| 9. M/73 | ENC | AMS, cognitive decline | Lung adenoca | AH | Br: N | N/49/N/0 | ‐ | |
| 10. M/55 | ENC | Cognitive decline, motor apraxia | ‐ | A | Br: N | N | ‐ | |
| 11. M/47 | ENC | AMS, focal dyscognitive seizures | Symptoms post‐Ehrlicosis pneumonia; RA | A | Br: diffuse WM ↑ T2 | N/56/N | EEG: gen seizure discharges | |
| 12. M/63* | ENC & brach plexitis | HA, diplopia, nausea, meningism, AMS, R&L shoulder pain | Papillary renal cell ca; membranous glomerulonephritis | A | Br: L&R periventricular, hazy WM ↑T2.Thick , T2 signal abns L brach plexus | 11/41/4 | EMG: bilateral suprascapular neuropathies w/o re‐innervation | |
| 13. F/75 | ENC | AMS, amnesia,, diplopia, dysarthria, dysphagia, weakness | Merkel cell ca, pembrolizumab | AHL | Br: N | 12/58/N/0 | ‐ | |
| 14. F/60* | ENC | Visual disturbance, hemianopsia, delirium, confusion, VII CN palsy | Pernicious anemia | A | Br: hemispheric deep WM ↑T2; R parieto‐occipital, periventricular, & centrum semiovale regions, enhancement | N/79/N | EEG, N. NP: Global deficits, executive dysfunction, frontosubcortical. | |
| 15. M/79 | ENC | AMS, coma, dysarthria, dysphagia | Hepatocellular ca | A | ‐ | N/93/N/0 | EEG: generalized slowing | |
| 16. F/73* | ENC | Neck & body pain, AMS, delusions, & auditory hallucinations (mild asymptomatic neuropathy at F/U | Ehrlichosis: URTI, ↓Plts, ↑ LFTs. Tick bite 2 weeks prior. | HL | Br C & T SC, N; LS spine: enhancing anterior thecal sac & LS nerve roots | 55/150/N/0, ↑IgG index | EEG: focal L temporal slowing; EMG mild polyrad | |
| 17. M/69 | ENC & ataxia | AMS, gait apraxia, dysarthria, cerebellar signs | ‐ | AHL | Br: diffuse atrophy | N/N/4/0 | ‐ | |
| 18. F/43 | ENC & ataxia | AMS, dysarthria, dysphagia, tremor, cerebellar ataxia | HIV, medication noncompliant | A | Br: hazy ↑T2 WM, R frontal, deep WM, BS, cerebellar hemispheres/ peduncles, (frontal enhancement) | 199/N/P/0 | ‐ | |
| 19. M/88* | ENC & ataxia | Delirium, pan‐cerebellar ataxia, large amplitude rubral tremor, dysphagia, dysarthria | Pancreas neuroendocrine ca | AHL | Br: hazy ↑T2 brain & cerebellar WM | N/46/N/0 | ‐ | |
| 20. F/74* | ENC | Headache, vertigo, nausea, vomiting, deep body aching, spasticity | SCLC | AHL | Br: Hazy ↑T2 (deep WM, brainstem, SC corticospinal tracts), enhancing L VII CN | 11/77/9/0 | ‐ | |
| 21. F/48 | Encephalomyelopathy | Vision disturbance, multimodal cognitive decline, seizures weakness | RA, hypothyroid history | AHL | Br: R&L hemispheric WM & full SC T2 changes with enhancement (brain & conus) | 11/94/P/GFAP | ‐ | |
| 22. M/57 | Encephalomyelopathy | Headache, nausea, visual disturbance, AMS, hemianopsia, weakness | Lung rhabdoid adenoca, ipilimumab & novolumab | AL | Br: hypophysitis | 20/93/NA | ‐ | |
| 23. M/21* | Encephalomyelopathy | Visual disturbance, AMS; relapse with myelopathy | ‐ | A | Br: R&L hemispheric hazy WM T2 FLAIR changes. Spine: N | 36/NA/9 | ‐ | |
A, alpha‐internexin; Ab, antibody; Abn, anormal; AMS, altered mental status; brach, brachial; Ca, carcinoma; CRMP5, collapsin response‐mediator protein‐5 EEG, electroencephalogram; EMG, electromyography; ENC, encephalopathy; F, female; FLAIR, fluid‐attenuated inversion recovery; F/U, follow‐up; gen, generalized; GFAP, glial fibrillary acidic protein; H, heavy chain; Halluci, hallucinations; HIV, human immunodeficiency virus; L, left; Li, light chain; LS, lumbosacral; M, male; Mo, months; N, normal; NA, Not available; NMDA‐R, N‐methyl‐D‐aspartate receptor; NP, neuropsychometric test; OCBs, oligoclonal bands (CSF‐exclusive); Polyrad, polyradiculoneuropathy; Pro, protein; Pt no., patient number; R, right; RA, rheumatoid arthritis; SCLC, small cell lung cancer; URI, upper respiratory tract infection; WCC, white cell count; WM, white matter.
Patient evaluated in person at Mayo Clinic; ↑ = increased/hyperintense; ↓ = reduced.