Table 1.
No. of case Age at investigation Sex Hospital stay |
Diagnosis | Etiology | Spasms' duration | Other seizure types | Brain MRI findings | Treatment | Follow-up |
---|---|---|---|---|---|---|---|
Case 1 4 years Male 30 days |
Mycoplasma-pneumoniae-related encephalopathy | Mycoplasma pneumoniae infection | Lasting 13 days, burst suppression both in sleep and awake | Focal seizures, originated from right frontal or parietal areas | At Day 15: atrophy of both cerebrum and cerebellum | IVIG, MP, 8 AEDs, KD; at investigation: TPM, LTG, CZP, LEV | At 9 years, walking, but not speaking, could not recognize parents, short stature |
Case 2 5 years and 2 months Female 37 days |
Influenza-virus-type-one-related acute encephalopathy | H1N1 infection | Lasting 6 months, burst suppression, gradually alleviated | Focal seizures, originated bilaterally | Generalized atrophy after decompressive craniectomy | IVIG, MP, KD, prednisone, 4 AEDs | At 5 years and 1 month, walking, mental retardation, no language |
Case 3 5 years and 7 months Male 16 days |
Anti-NMDAR encephalitis | Autoimmune encephalitis | Lasting 8 months, burst suppression EEG | Focal seizures originated right hemisphere, convulsive SE | At 10 months: MRI showed atrophy of cerebrum, and hippocampi improved | IVIG, MP, 5 AEDs; at investigation: VPA, NZP, LTG, CBZ, KD, cyclophosphamide monthly 8 times | At 4 years, nearly recovered, autoantibody remains positive in CSF |
Case 4 5 years and 7 months Male 105 days |
Febrile-infection-related epilepsy syndrome | Unknown etiology, Mycoplasma pneumoniae infection? | Lasting 87.5 min, asymmetrical spasm | Convulsive and non-convulsive SE, multi-focal, primarily frontal in origin | Brain atrophy including hippocampi improved during the last 3 months | IVIG, PE, KD, propofol, ketamine, chloralhydrate, diazepam, 7 AEDs, with 5 AEDs when discharged. | At 4 years, walking, speaking, but memory was impaired |
Case 5 2 years Male 29 days |
Allergenic demyelinating encephalitis | Vaccination of measles-mumps-rubella triad vaccine | Lasting 2 months, each cluster lasting more than 30 min, burst suppression and hypsarrhythmia | Focal seizures, left frontal and temporal, bilateral occipital epileptiform discharges | Atrophy of thalamus, occipital region and left hippocampus improved? | IVIG, MP, 3 AEDs, ACTH | At 4 years, nearly recovered as before, learning and language slightly impaired |
Case 6 3 years and 4 months Female 141 days |
Acute encephalopathy | Severe allergy, post-cardiopulmonary resuscitation, iodine allergy | Lasting 30–60 min daily for 50 days. EEG hypsarrhythmia | Focal seizures generalized at early stage of disease | Caudate nucleus and thalamus abnormal signal on Diffusion-Weighted Images | TPM, ACTH, prednisone | At 4 years 2 months, left arm movement and speech not fully recovered, otherwise normal |
Case 7 11 months Female 15 days |
Intractable epilepsy, focal seizures, developmental delay | Compound heterozygous mutation in the TBCD gene | 3 times continuous spasms, each lasing 1.5 weeks | Low voltage on EEG, non-Convulsive status epilepticus, convulsive status epilepticus | At 20 months: significant atrophy, 3 months later atrophy aggravated | IVIG, MP, KD, 3 AEDs, another 3 AEDs used | At 5.5 years, no language, rolling and walking, severe development delay |
Case 8 5 months Male 13 days |
Intractable epilepsy, infantile spasms, Ohtahara Syndrome transition Severe asphyxia | Ohtahara Syndrome, genetic mutation? | Lasing 2 h, burst suppression and hypsarrhythmia | Focal seizures, tonic seizures frequently, Multifocal spikes | Severe brain atrophy | KD, prednisone, 4 AEDs, ACTH | At 4 years 3 months: microcephaly, no head control or language, cannot recognize parents, seizures decreased |
Case 9 11 months Female 17 days |
Infantile spasms, psychomotor retardation, epileptic encephalopathy | Unknown | Lasing 3 months and 5 days, EEG, burst suppression | Focal seizures, refractory epilepsy | Abnormal signal at white matter, thalamus atrophy | TPM, KD, VPA, LEV, ACTH | At 6 years: developmentally delayed, could not sit, still daily seizures |
Case 10 1 month and 20 days Male 15 days |
Ohtahara Syndrome Asphyxia | Genetic, a de novo missense mutation in the KCNQ2 gene | Lasting 108 min, spasms in cluster or singly | Tonic seizures, focal seizures | Un remarkable | ACTH, 3 AEDs | At 3 years and 4 months: spasms, no visual fixation, deceased at age of 8 months due to severe infection. |
Case 11 4 months Male 15 days |
Pachygyria malformation, Intractable epilepsy | Genetic, 489 Kb deletion at 17p13.3 | Lasting 30 min, in cluster or single, hypsarrhythmia | Tonic seizures, focal seizures | Pachygyria | 3 AEDs, ACTH, sirolimus, KD | At 5 years: severely delayed development, no head control, language or visual following, seizures 3.5 times daily |
IVIG, intravenous immunoglobulin; PE, plasma exchange; MP, methylprednisolone; ACTH, adrenocortical tropical hormone; AEDs, antiepileptic drugs; TPM, topiramate; LTG, lamotrigine; LEV, levoparacetam; NZP, nitrozepam; CBZ, carbamazepine; KD, ketogenic diet.