Characteristics of the 6 patients with DNS
| Patients . | Age, y . | Sex . | Characteristics . | Chronology . | Previous treatment . | Symptoms . | Explorations . | Treatment . | Outcome/follow-up . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 28 | F | IgG κ, livedo | 1 y after diagnosis | HDIVig (stopped 3 mo before) | Flu-like syndrome, confusion, hemiparesis, epilepsy, and SE | cMRI: N. CSF: N. | ET: OTI, AEs, plasmapheresis, and HDIVig → slow CR. MT: lenalidomide + dexamethasone + HDIVig → CR. | Complete remission/1 y |
| 2 | 42 | M | IgG λ, carpal tunnel syndrome, smoldering myeloma | 5 y after diagnosis | HDIVig (spaced >6 wk apart) | Flu-like syndrome, fever, confusion, coma, and SE | cMRI: N. CSF: slight protein increase. | ET: OTI, AEs, plasmapheresis and HDIVig + CTs = CR. MT: lenalidomide + dexamethasone + HDIVig → CR. | Complete remission/2 y |
| 3 | 44 | M | IgG λ/GAL | 4 y after diagnosis | HDIVig | Flu-like syndrome, asthenia, fever, chorea, coma, epilepsy, and SE | cCT: N. | ET: OTI, AEs, plasmapheresis, HDIVIg → CR. MT: HDIVig → CR. | Complete remission/1 y |
| 4 | 68 | F | IgG λ/ GAL | 2 y before diagnosis (MGUS IgG λ) | — | Flu-like syndrome, fever, dysarthria, cerebellar syndrome, tetrapyramidal syndrome, coma, and SE | cMRI: N. EEG: N. CSF: N. | ET: OTI, AEs → PR. MT: HDIVig → CR. | Reduced psychomotor responses/4 y (complete skin response) |
| 5 | 57 | F | IgG λ and epilepsy ×3 (5 y before), purpura, skin necrosis, and vasculitis/GAL | 2 y after the first symptoms | — | Language dysfunction, epilepsy, and SE | cMRI: N, CSF: N, EEG: postcritical epilepsy. | ET: OTI, AEs, plasmapheresis, pulse methylprednisolone → CR. MT: bortezomib + dexamethasone. | Complete remission and epilepsy/2 y |
| 6 | 51 | M | IgG κ | 1 y after diagnosis (×5 episodes) | HDIVig | Confusion, asthenia, rotatory vertigo, convulsion, hemiparesis, hallucination psychosis, dysarthria, and SE | MRI: N ×3. cCT: N ×2. CSF: N ×5. EEG ×5: postcritical epilepsy. | ET: OTI, AEs → PR. MT: plasmapheresis and HDIVig = NR. Bortezomib/cyclophosphamide/dexamethasone and HDIVig → NR and relapse. Lenalidomide + bortezomib + dexamethasone → CR. | Complete remission (after the last episode) (after 5)/2 y (relapse after 2 y) |
| Patients . | Age, y . | Sex . | Characteristics . | Chronology . | Previous treatment . | Symptoms . | Explorations . | Treatment . | Outcome/follow-up . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 28 | F | IgG κ, livedo | 1 y after diagnosis | HDIVig (stopped 3 mo before) | Flu-like syndrome, confusion, hemiparesis, epilepsy, and SE | cMRI: N. CSF: N. | ET: OTI, AEs, plasmapheresis, and HDIVig → slow CR. MT: lenalidomide + dexamethasone + HDIVig → CR. | Complete remission/1 y |
| 2 | 42 | M | IgG λ, carpal tunnel syndrome, smoldering myeloma | 5 y after diagnosis | HDIVig (spaced >6 wk apart) | Flu-like syndrome, fever, confusion, coma, and SE | cMRI: N. CSF: slight protein increase. | ET: OTI, AEs, plasmapheresis and HDIVig + CTs = CR. MT: lenalidomide + dexamethasone + HDIVig → CR. | Complete remission/2 y |
| 3 | 44 | M | IgG λ/GAL | 4 y after diagnosis | HDIVig | Flu-like syndrome, asthenia, fever, chorea, coma, epilepsy, and SE | cCT: N. | ET: OTI, AEs, plasmapheresis, HDIVIg → CR. MT: HDIVig → CR. | Complete remission/1 y |
| 4 | 68 | F | IgG λ/ GAL | 2 y before diagnosis (MGUS IgG λ) | — | Flu-like syndrome, fever, dysarthria, cerebellar syndrome, tetrapyramidal syndrome, coma, and SE | cMRI: N. EEG: N. CSF: N. | ET: OTI, AEs → PR. MT: HDIVig → CR. | Reduced psychomotor responses/4 y (complete skin response) |
| 5 | 57 | F | IgG λ and epilepsy ×3 (5 y before), purpura, skin necrosis, and vasculitis/GAL | 2 y after the first symptoms | — | Language dysfunction, epilepsy, and SE | cMRI: N, CSF: N, EEG: postcritical epilepsy. | ET: OTI, AEs, plasmapheresis, pulse methylprednisolone → CR. MT: bortezomib + dexamethasone. | Complete remission and epilepsy/2 y |
| 6 | 51 | M | IgG κ | 1 y after diagnosis (×5 episodes) | HDIVig | Confusion, asthenia, rotatory vertigo, convulsion, hemiparesis, hallucination psychosis, dysarthria, and SE | MRI: N ×3. cCT: N ×2. CSF: N ×5. EEG ×5: postcritical epilepsy. | ET: OTI, AEs → PR. MT: plasmapheresis and HDIVig = NR. Bortezomib/cyclophosphamide/dexamethasone and HDIVig → NR and relapse. Lenalidomide + bortezomib + dexamethasone → CR. | Complete remission (after the last episode) (after 5)/2 y (relapse after 2 y) |
AE, antiepileptic; cCT, cerebral computed tomography; cMRI, cerebral MRI; CSF, cerebrospinal fluid; CT, corticosteroid; EEG, electroencephalography; ET, emergency treatment; GAL, granuloma annulare–like histology; MT, maintenance treatment; N, normal; OTI, orotracheal intubation; SE, status epilepticus.