Table.
Clinical Summary of Patients With Neuronal Antibodies Against Surface Antigens Initially Suspected to Have Creutzfeldt-Jakob Disease
| Characteristic | Antibody Specificity | |||||
|---|---|---|---|---|---|---|
| CASPR2 | LGI1 | NMDAR | AQP4 | Tr (DNER) | Unidentified NSA | |
| Sex/age at onset, y | M/68 | M/79 | M/58 | M/73 | F/59 | F/59 |
| Instaurationa | Insidious | Acute | Subacute | Acute | Acute | Subacute |
| First symptoms | Confusion, abnor- mal behavior |
Myoclonic-like movementsb |
Depression and disorientation |
Acute confusional state |
Gait instability | Depression and memory impairment |
| Cognitive decline | Yes | No | Yes | Yes | No | Yes |
| Myoclonus | No | No | No | No | No | Yes |
| Pyramidal signs | Yes | No | No | Yes | Yes | No |
| Cerebellar signs | No | No | No | No | Yes | No |
| Depression | Yes | No | Yes | No | No | Yes |
| Hallucinations | No | No | Yes | No | No | Yes |
| Psychosis | Yes | No | No | No | No | No |
| Seizures | Yes | Yesb | Yes | No | No | No |
| MRI results | Medial temporal lesions on FLAIR sequences |
Normal | Normal | Periventricular and hippocampal lesions on FLAIR sequences |
Normal | Normal |
| CSF cell count | Normal | Normal | Normal | Normal | 23 WBCs | 14 WBCs |
| EEG results | Diffuse slowing | Normal | Diffuse slowing | FIRDA | Normal | Normal |
| Cancer | No | No | No | No | No | Yes (SCLC) |
| Treatment | Corticosteroids | Corticosteroids | ECT, venlafaxine, quetiapine |
Corticosteroids, plasmapheresis, rituximab |
Corticosteroids, rituximab, cyclophosphamide |
Chemotherapy, radiotherapy |
| Improvement (final mRS) | Yes (3) | Yes (1) | Yes (2) | Yes (4) | No (4) | Yes (1) |
Abbreviations: AQP4, aquaporin 4; CASPR2, contactin-associated protein-like 2; CSF, cerebrospinal fluid; ECT, electroconvulsive therapy; EEG, electroencephalogram; FIRDA, frontal intermittent rhythmic delta activity; FLAIR, fluid-attenuated inversion recovery; LGI1, leucine-rich glioma-inactivated 1; MRI, magnetic resonance imaging; mRS; modified Rankin scale; NMDAR, N-methyl-d-aspartate receptor; NSA, neuronal surface antigen; SCLC, small cell lung carcinoma; Tr(DNER), δ/notch-like epidermal growth factor–related receptor; WBCs, white blood cells.
Time from first symptom to first medical evaluation: acute (<1 wk), subacute (1 wk–3 mo), and insidious (>3 mo).
Initially considered as myoclonus, retrospectively considered as faciobrachial dystonic seizures.