Table 2.
Clinical information and additional findings
| Disease | Sex | Age (years) | Symptoms and signs | Corticosteroid responsiveness | CSF | Additional tests/imaging/information |
|---|---|---|---|---|---|---|
| Neurosarcoidosis | F > M | 20–40 | Symptoms of increased intracranial pressure due to hydrocephalus and cranial nerve palsy, and systemic symptoms | + | Non-specific | Chest CT, BAL |
| CNS vasculitis | M > F | Average 50, wide range | Throbbing headache, stroke-like | + | Non-specific | DSA |
| CNS lymphoma | M > F | Middle age or elderly (> 50) | Headache, symptoms of increased intracranial pressure due to hydrocephalus | + | Malignant cells, increased protein concentration | PET-CT |
| ECD | M > F | Middle age or elderly (> 50) | Bone pain, neuroendocrine disorders | − | Non-specific | X-rays of long bones |
| Lyme disease | M = F | Two peaks: 2–15 and 30–55 | Erythema migrans, cranial nerve palsy | − | Non-specific | Dermatological exam, serology |
| Vitamin B12 deficiency | M = F | All ages | Anaemia, peripheral neuropathy | − | ↓ vitamin B12 (uncommon, usually tested in serum) | Spine MRI |
| CLIPPERS | M = F | Average 50 (wide range) | Gait ataxia, diplopia, dizziness, nausea | + | Rarely oligoclonal bands | Spine MRI, PET-CT |
| Candidiasis | M = F | Preterm neonates and immunocompromised adults | Meningism, (multi)organ failure | − | CSF culture | Echocardiogram, DSA |
| Cryptococcosis | M = F | Immunocompromised adults | Meningism, focal neurological deficit | + | CSF culture | Chest CT |
| PML-IRIS | M = F | Immunocompromised adults | New and/or worsening of existing clinical symptoms of PML (cognitive, motor, sensorial) | + | PCR JC virus | – |
| GFAP | M = F | Average 40 (wide range) | Meningoencephalomyelitis | + | IgG anti-GFAP | Serum GFAP-IgG |
| NMOSD | F > M | Average 35 (adolescence − 60) | Unilateral or bilateral optic neuritis, area postrema syndrome, longitudinally extensive myelitis | + | AQP4-IgG (uncommon, usually tested in serum) | Spine MRI |
| Behçet’s disease | M > F | 10–50 | Pyramidal signs, behavioural changes | + | Non-specific | DSA |
| Neurotuberculosis | M = F | Any age, commonly < 30 | Focal neurological signs, intracranial hypertension, behavioural changes, seizures | − | PCR, CSF culture | Chest CT |
| Histoplasmosis | M = F | Immunocompromised adults or at any age in endemic areas | None, cough | − | Antibodies anti-H. capsulatum (uncommon, usually tested in serum) | Chest CT |
| Susac’s syndrome | F > M | 20–40 | Encephalopathy, hearing loss and visual disturbance | + | Non-specific | Ophthalmological and audiological exam |
| Metastases | M = F | Elderly | Headache, focal neurological deficit | +/− | Malignant cells | PET-CT |
BAL bronchoalveolar lavage, DSA digital subtraction angiography, PCR protein chain reaction, PML-IRIS progressive multifocal leukoencephalopathy–immune reconstitution inflammatory syndrome, ECD Erdheim–Chester disease, CLIPPERS chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, JC John Cunningham, GFAP glial fibrillary acidic protein, NMOSD neuromyelitis optica spectrum disorders