[Case of 12-year-old boy with idiopathic recurrent neuralgic amyotrophy].

Here we report a 12-year-old boy with idiopathic neuralgic amyotrophy who had two episodes of shoulder pain followed by shoulder muscle atrophy and weakness at the age of 7 and 11 years, respectively. These symptoms were self-limited and disappeared within 9 months. During the second episode, electromyograph (EMG) revealed neurogenic changes in the deltoid muscle. Muscle imaging showed the right deltoid muscle atrophy with slightly high intensity areas on T1 and T2 weighted images in MRI. Muscle biopsy from the right deltoid muscle revealed neurogenic changes with denervating and reinnervating processes. Neuralgic amyotrophy is characterized by neuralgic pain followed by weakness and atrophy at a unilateral extremity and is usually self-limited. EMG and imaging studies showed focal neurogenic abnormalities, which were confirmed by muscle biopsy. Neuralgic amyotrophy usually occurs in young adults and it is very rare in children.

[Clinico-etiological study of 116 children with acquired damage to the central nervous system during the last decade in Niigata Prefecture].

One hundred and sixteen children with an acquired damage to the central nervous system (CNS) during the last decade were studied to determine the causes and sequelae and to find strategies for prevention. The etiology was CNS infections and related disorders in 46 cases (39.4%), non-infectious diseases (epileptic, hypoxic etc.) in 16 cases (13.8%), iatrogenic brain injury in 15 cases (12.9%), accident-related brain damage in 31 cases (26.7%), and vascular disorders in 8 cases (6.9%). Since infections and accidents accounted for as much as 66.4%, prevention of their sequelae is important. Of 37 patients (31.9%) with severe motor and intellectual disabilities syndrome (SMIDS), 18 were due to infectious diseases. SMIDS cases accounted for 39.1% of the infectious diseases group, 25% of the non-infectious diseases group, 40% of the iatrogenic group, 25.8% of the accident-related group and 12.5% of the vascular group. The prevalence rate of acquired CNS damage was estimated to be 0.5/10(3)/year and that of SMIDS due to acquired damage to be 0.16/10(3)/year. A monitoring system for patients with an acquired brain damage and for their treatment is important.