Table 1.
Demographic, clinical features, immunologic data and treatment of the patients with DPPX antibodies
| Patient 1 | Patient 2 | Patient 3a | Patient 4 | Patient 5 | Patient 6b | Patient 7 | Patient 8 | Patient 9 | |
|---|---|---|---|---|---|---|---|---|---|
| Demographic | |||||||||
| Sex | F | F | F | M | M | F | F | M | F |
| Age at onset, y | 65 | 47 | 62 | 35 | 14 | 51 | 35 | 54 | 52 |
| Months to onset | 12 | 0.5 | 3 | 0.5 | 0.5 | 0.5 | 4 | 1 | 6 |
| Clinical Features | |||||||||
| Prodromal symptoms | Headache | Fever, diarrhea | Weight loss (10 kg), diarrhea, headache; | Headache | Fever, gastroparesis and constipation | Fever, headache, weight loss (5 kg) | Vertigo, diarrhea | Headache | Fever |
| Main symptoms | Memory loss, executive dysfunction, delusions, hyperreflexia | Delirium in 11 days to cognitive disorder, aphasia, limb paralysis, RBD, vertigo | Bradykinesia, tremor, memory loss, RBD, hyperreflexia, nystagmus | Stiffness, sleep disorder (insomnia), generalized seizure | Confusion, cognitive disorder, limb paralysis, respiratory failure, hyperreflexia | Memory loss, insomnia, ataxia, nystagmus | Generalized seizure, insomnia, RBD | Somnolence, cognitive disorder, myoclonus, insomnia | Delirium, cognitive disorder, recurrent generalized seizures, |
| Brain MRI | Bilateral hippocampus T2/FLAIR increased signals | Bilateral temporal lobe and amygdala T2/FLAIR increased signals | Nonspecific white matter changes | Left hippocampus and basal ganglia T2/FLAIR increased signals | Extensive abnormal signals in the brainstem, basal ganglia and bilateral white matter | Extensive abnormal signals in the frontal lobe, basal ganglia, hippocampus and cerebellum | Normal | Nonspecific white matter changes | Bilateral temporal lobe, hippocampus and left frontal lobe T2/FLAIR increased signals |
| Immunologic data | |||||||||
| CSF | WBC: normal Protein: normal | WBC: normal Protein: normal | WBC: normal Protein: normal | December 2018 WBC: 370, Protein: 852 January 2019 WBC: 20, Protein 549 | March 2019 WBC: 220, Protein:2096 May 2019 WBC: 10, Protein: 469 | WBC: normal Protein: normal | WBC: normal Protein: normal | WBC: normal Protein: 958 | November 2018 WBC (−), Protein: 609 December 2018 WBC (−), Protein (−) |
| DPPX antibody | Serum: 1:100 CSF: ND | Serum: 1:10 CSF: ND | Serum: 1:32 CSF: ND | Serum: 1:32 CSF: 1: 3.2 | Serum: 1:320 CSF (−) | Serum: 1:100 CSF: 1:1 | Serum: 1:10 CSF: (−) | Serum: 1:1000 CSF: 1:10 | December 2018 Serum:1:10 CSF: (−) October 2019 Serum:1:32 CSF: ND |
| Treatment/response | IVMP, oral steroids/ marked improvement | IVMP, oral steroids/ marked improvement, but relapsed with steroid taper | Oral steroids/ marked improvement | IVMP, Tacrolimus/ mild, but steady improvement | IVMP, IVIg/ incomplete improvement | IVMP, Rituximab/ marked improvement | IVMP, oral steroids/ marked improvement | IVMP, oral steroids/ marked improvement | IVMP, oral steroids/ marked improvement, but relapsed with steroid taper |
Abbreviations: DPPX dipeptidyl-peptidase-like protein-6, ND not done, IVMP intravenous methylprednisolone, IVIg intravenous immunoglobulins
a Micropapillary carcinoma of thyroid was found during the course of the disease
b Non-Hodgkin’s lymphoma was found during the examinations