Clinical feature | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 |
---|---|---|---|---|---|---|
Age of onset | 16 months | 15 months | 14 months | 9 months | 18 months | 16 months |
Sex | Female | Male | Female | Female | Male | Male |
Clinical manifestations | ||||||
Opsoclonus | Yes | Yes | Yes | Yes | Yes | Yes |
Myoclonus | Yes | Yes | Yes | Yes | Yes | Yes |
Ataxia | Yes | Yes | Yes | Yes | Yes | Yes |
Irritability | Yes | Yes | Yes | Yes | Yes | Yes |
Sleep disturbance | Yes | Yes | Yes | Yes | Yes | Yes |
Abnormal behavior | Yes | Yes | Yes | Yes | Yes | Yes |
Feeling frightened | Yes | No | No | No | Yes | Yes |
Preceding manifestations | None | Vaccination of varicella | None | None | None | None |
Etiology (neuroblastoma or infectious) | Unknown | Unknown | Neuroblastoma | Unknown | Unknown | Neuroblastoma |
OMS score at presentation | 18 | 18 | 15 | 18 | 14 | 14 |
Investigations | ||||||
Evidence of pathogenic infection after screening | No | No | No | No | No | No |
CSF analysis (antibodies) | Not available | Negative | Not available | Not available | Negative | Negative |
Serum analysis (antibodies) | Negative | Negative | Not available | Negative | Negative | Negative |
Neuron-specific enolase | Elevated | Elevated | Elevated | Elevated | Elevated | Elevated |
Electroencephalograph | Normal at the beginning, slow background later. | Normal at the beginning, slow background later. | Slow background | Slow background then normal. | Normal | Normal |
Targeted/whole-body MRI/CT for neuroblastoma | Normal | Normal | CT found neuroblastoma in the posterior mediastinum. | Normal | Normal | CT found neuroblastoma in the posterior mediastinum |
Urine catecholamine (Vanillylmandelic acid ) | Negative | Negative | Not available | Elevated | Negative | Negative |