Clinical presentation of primary cerebral involvement of rheumatic diseases and opportunistic CNS infections61723245365–67
| Clinical presentation | NPSLE | Primary CNS vasculitis | Wegener’s granulomatosis | Neuro-Behçet | Neurosarcoidosis | PML | HSV encephalitis | Bacterial meningitis | Brain abscess |
|---|---|---|---|---|---|---|---|---|---|
| NPSLE, neuropsychiatric systemic lupus erythematosus; PML, progressive multifocal leucoencephalopathy; HSV, herpes simplex virus; ?, no data from clinical trials available. | |||||||||
| Onset of CNS disease | Acute, subacute, chronic | Acute, subacute, chronic | Subacute, chronic | Acute, subacute, chronic | Acute, subacute, chronic | Subacute (weeks) | Acute, subacute (days-weeks) | Acute (days) | Subacute (days-weeks) |
| Seizures | 13–35% | 15–20% | Rare | 4% | 10% | 5% | Frequent | 20–30% | 20–30% |
| Headache | 34–57% | 30–64% | Frequent | 95% | 30% | 5% | Frequent | 90% | 70–90% |
| Motor weakness/paresis | 20% | 50% | ? | 10–21% Paresis, 24% pyramidal signs | 5–10% | 33% | Frequent | 10–15% | 20–50% |
| Impaired conciousness | 5–10% | 29% | Rare | Rare | Rare | <1% | Frequent | 30–50% | 20–30% |
| Cognitive disorders | 12–66% | 40–50% | Rare | 88% | 10% | 36% | Frequent | Frequent | ? |
| Psychosis | 4–6% | Rare | Rare | 2% | Rare | Rare | Rare | Rare | Rare |
| Mood disorder | 10–30% | Rare | Rare | Rare | Rare | Reactive | Rare | Rare | Rare |
| Visual deficits | 5% (vasculitis, neuropathy, amaurosis fugax) | 10–15% | Frequent (cranial neuropathy, ocular motor deficits) | 1% Optic neuropathy | 5–38% Optic neuritis | 35% e.g. homonymous hemianopsia | Frequent | Rare | Hemianopsia |
| Sensory deficits | 6–20% | 15–20% | ? | Up to 27% | 5–10% | 17% | Rare | Rare | 30% |
| Cerebellar disorders | Rare (infarction) | Rare | Rare | 33% | 21% | 13–32% | Rare | Rare | Ataxia 10% |
| Cranial nerve involvement | 5–35% | Frequent | Frequent | 25% Ophthalmoplegia, 10–15% bulbar paresis | 50–72% Cranial nerve palsies | Rare | Rare | 10 (N. III, VI, VII, VIII) | Rare |
| CRP | Normal | Normal | Frequently ↑ | Frequently ↑ | Normal ↑ | Normal ↑ | normal - ↑ | ↑ | ↑ |
| Other | <1% Transverse myelitis, 2–5% chorea | 30% Aphasia | Chronic meningitis, myelopathy | 8% Meningo-encephalitis, 6% movement disorders | 5–12% Meningitis, 10–28% myelitis, 10% hypothalamic and pituitary dysf. | Impaired speech | Wernicke aphasia, dysphasia | Meningism, fever, erythema | 50% Fever, 25–30% meningism |