Objective: To investigate the association between mineralizing angiopathy (MA) and basal ganglia infarction after minor head trauma and to describe the clinical and imaging features of infarction. Methods: Children who presented to our center with cerebral infarction between April 1980 and November 2016 were retrospectively reviewed, and those with post-traumatic infarction in the basal ganglia were evaluated. Results: Among 119 children who suffered from arterial ischemic strokes, five presented with basal ganglia infarctions after minor head trauma. All of them were males, with a median age of 33 months (from 10 months to 11 years). Four children experienced left hemiparesis, and one child showed no neurological symptoms. The onset of neurological symptoms occurred between 15 min and 3 h after minor head trauma. Paralysis improved in all children. On brain imaging, four children showed infarctions on the right side and one showed infarction on the left side within the basal ganglia. On computed tomography (CT), four children exhibited high densities within the basal ganglia, indicating MA of perforators ; among these, three children exhibited bilateral lenticulostriate artery mineralization. An 11-year-old boy had an old infarction on the contralateral basal ganglia and had probably suffered a perforator infarction previously. Conclusions: Most patients experiencing basal ganglia infarction after minor head trauma suffer from lenticulostriate artery mineralization. Considering that such patients reported no other cause of infarction, MA may be associated with the pathogenesis of basal ganglia infarction after minor head trauma. Cerebral infarction after minor head trauma has been frequently reported in infants. This study included patients older than those included in previous studies. Our findings indicate the possible onset of basal ganglia infarction during the juvenile period and the possibility of bilateral lenticulostriate artery mineralization leading to a possible risk of infarction relapse.

  Objective: The purpose of this study was to elucidate the efficacy of topiramate (TPM) on drug-resistant migraine in pediatric patients. Methods: The subjects were five patients with pediatric migraine for whom preceding prophylactic medicines showed little effect. We retrospectively analyzed clinical backgrounds ; headache characteristics ; concomitant medication ; and duration, efficacy, and adverse effects of TPM administration. Results: Four patients were diagnosed with chronic daily headache at TPM initiation. The number of days with headache reduced during TPM dosage titration in all patients, and the frequency of severe headache in two. However, two patients with psychosocial problems or autism spectrum trait relapsed within 1-2 months after initiation. Although two patients had sleepiness and one had weight loss, no patient discontinued the medication because of adverse events. Conclusions: Topiramate was effective and well-tolerated in this study. Topiramate could be proposed as a prophylaxis treatment option for drug-resistant migraine in pediatric patients. However, its effects may be limited on the chronic daily headache with backgrounds of serious psychosocial/developmental problems.

  Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been implicated in various demyelinating diseases. Recently, acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ON) with positive serum anti-MOG antibodies has been reported as a new phenotype of demyelinating diseases. An 8-year-old boy was admitted because of fever, headache and urinary retention. The symptoms and magnetic resonance imaging results led to a diagnosis of ADEM. The treatment consisted of intravenous methylprednisolone (IVMP) followed by a tapered dose of oral prednisolone (PSL), resulting in immediate improvement of his symptoms. However, the ADEM relapsed twice. Ocular pain and visual disturbance in the right eye developed with the third relapse. He was diagnosed with ON and treated with IVMP followed by PSL and various immunosuppressant, and gradually recovered. However, the ON relapsed four times. IVMP was ineffective for the fourth relapse, but his visual disturbance improved with plasma exchange (PE). Clinical remission was achieved within 5 months after the last relapse by the administration of PSL and rituximab. The serum analysis was positive for anti-MOG antibodies. He was diagnosed with ADEM followed by ON based on the clinical course. It is possible that IVMP become ineffective for recurrent ADEM followed by ON. When IVMP is ineffective, PE should be considered.

  A 2-year-old boy with normal development experienced tonic clonic seizures during an episode of influenza. Thereafter, he was unable to walk and his ability to speak gradually declined. He showed no severe consciousness impairment. Both his anti-glutamate receptor (anti-GluR) antibody and serum anti-voltage-gated potassium channel (anti-VGKC) complex antibody levels were elevated. Immunoglobulin and corticosteroid therapy were effective, and led to the improvement of his motor function and speech. The pathogenesis underlying the symptoms was considered to have been mediated by an autoimmune mechanism.

  We report the case of a 12 year-old girl with anti-muscle-specific kinase (MuSK) positive myasthenia gravis (MG). She exhibited ptosis, dysphagia, and facial muscle weakness and was tested positive for MuSK autoantibodies. She was initially treated with methylprednisolone pulse therapy ; however, her symptoms persisted. She then underwent five sessions of plasma exchange, which was very effective. Subsequently, she was prescribed oral tacrolimus and has maintained complete stable remission for 2 years. Aggressive treatments including the early induction of plasma exchange and immunosuppression therapy may be effective for childhood-onset anti-MuSK positive MG.