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When treatment for childhood-onset epilepsy is introduced in the transitional period, neurologists must classify the epilepsy syndrome according to the age of onset and etiology. When a patient with refractory epilepsy consults a neurologist during the transition, it is essential to review the antiepileptic medication and reaffirm the treatment goal. In cases where pre-existing disease or comorbidities are present, a neurologist should treat their patients in consultation with general physicians or home-visit doctors.

Kawasaki disease (KD) is a systemic vasculitis in infants. In KD, encephalopathy is rarely (0.1%) associated, however, clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) has previously been reported in some pediatric patients. Here, we report on a 2-year-old girl who had KD complicated with MERS. The patient experienced generalized clonic convulsion and prolonged consciousness disturbance with fever for 2 days. Her head MRI showed a high signal intensity lesion in the splenium of the corpus callosum in diffusion-weighted images, and low apparent diffusion coefficient (ADC) values on day 3. An electroencephalogram showed high voltage slow waves on the occipital and parietal head. On the same day, it was confirmed that the patient showed all the main symptoms of KD. Based on these findings, we diagnosed her with MERS-complicated KD. Even though she was treated with immunoglobulin (total 4 g/kg) and pulsed-dose methylprednisolone, her fever and consciousness disturbance continued, and blood tests showed that inflammation markers remained high. We then treated the patient with infliximab on day 9, and within a few hours of the treatment her fever dropped and all symptoms of KD and consciousness disturbance disappeared. No recurrence of KD or other complications of KD occurred, and she was discharged on day 23. We propose that infliximab is an effective optional treatment for immunoglobulin/glucocorticoid-resistant KD with MERS. To clarify this possibility, further case accumulation is warranted.

We investigated the usefulness of electrodiagnostic (EDX) studies for the early diagnosis of childhood Guillain-Barré syndrome (GBS). We retrospectively reviewed 5 patients (ages, 17-96 months) who fulfilled the diagnostic criteria of GBS. The EDX studies were performed at least twice;they included 1 or more following:motor nerve conduction study (MNCS), F-wave study, electromyography (EMG), and sensory nerve conduction study. The first and second EDX studies were performed at 8 days (range:4-13 days) and 14 days (range: 12-27 days) after the onset of motor symptoms, respectively. Although only 3 of 5 patients showed abnormal findings in the first MNCS, additional EDX studies, namely, F-wave study and EMG confirmed the presence of peripheral neuropathy in all patients. Regarding the classification of GBS subtypes, the results of the first EDX studies lead to the diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP) in 3 patients and the remaining two were diagnosed with AIDP based on results of the second studies. We concluded that serial EDX studies, including F-wave studies, are essential for the early and definite diagnosis of childhood GBS.

We report a case of "HHV-6 encephalopathy with cluster of convulsions during eruptive stage" (HECCES) that showed hyperperfusion in the cerebral hemisphere by single photon emission computed tomography (SPECT) during the acute phase. There are several reports that described brain hypoperfusion of HECCES by SPECT, but all of these descriptions involved the chronic phase. In contrast, our patient showed hyperpefusion on the day of seizure cluster. Since the prognosis for HECCES is sometimes poor, it is important to clarify the etiology and to develop a treatment. The description of this case is significant for these points.