Table 1 Main clinical features, examination, treatment and prognosis in 6 children with anti-mGluR5 encephalitis
Patient sex, age (y) | Prodromal features | Main clinical features | Tumor | CSF analysis | MRI | Treatment | Last follow-up, mo; outcome; |
|---|---|---|---|---|---|---|---|
Patient1 M,15 | Headache, nausea | Confusion, auditory and visual hallucinations, decreased verbal output, attention deficit, status epilepticus | Hodgkin's lymphoma | 114 WBC, OCB | Bilateral (left > right) posterior cortical diffusion restriction | Chemotherapy, RT | 72; Complete recovery |
Patient2 F,16 | Headache | Psychosis, hallucinations, poor sleep, dystonia, generalized seizures | Hodgkin's lymphoma | 31 WBC, OCB | Normal | Steroids, chemotherapy, PE | 48; Symptoms of Neurological symptom and tumor recurrence |
Patient3 F,6 | Rash, headache, flulike symptoms | Status epilepticus, dLOC, memory loss, poor sleep with altered sleep–wake cycle, followed by dystonia and oculogyric crisis, psychomotor slowness, ataxia, speech and motor regression, hypoventilation | None | 21WBC OCB negative | Bilateral frontal (left > right) and right occipital lobes, cerebellum | Steroids, IVIg, RTX | 19; Partial, improved aphasia, cannot walk unassisted |
Patient4 M,15 | None | Facial paralysis, then developed altered behavior, memory loss, anxiety, irritability, visual hallucinations, insomnia | Hodgkin's lymphoma | 45 WBC, OCB | Normal | Steroids, IVIg, chemotherapy | 12; Moderate memory problems |
Patient5 F,15 | loss of appetite | Psychobehavioral abnormalities, seizures, generalized myoclonus | None | Normal | Mild cerebral atrophy | Steroids, IVIg | 3; Lethargy |
Patient6 M,12 this case | headache, intermittent fever | hallucination and auditory hallucination, mental disorders, sleep disorder | gangliocytoma | 90.0 WBC, OCB | encephalitis in limbic | Steroids, IVIg resection of gangliocytoma | 3;occasional hallucinations mentioned |