Sirs,Charcot-Marie-Tooth disease type 2D (CMT2D) and distal spinal muscular atrophy type V (dSMA-V) are autosomal dominant neuropathy/neuronopathy associated with heterozygous mutations in the glycyl-tRNA synthetase (GlyRS) gene (GARS), characterized by weakness and atrophy that primarily affects the upper extremities [1]. We describe clinical findings of a Japanese patient with CMT2D with a novel mutation of the GARS. The gene mutation of this patient has been reported in a recent genetic study on CMT neuropathies in Japan [2].A 10-year-old boy with normal birth and development who could not run fast from his early childhood noticed weakness of the fingers. On admission at the age of 15, he showed atrophy in the bilateral thenar, hypothenar, dorsal interossei and tibialis anterior muscles (Fig. 1a), grade 2 weakness of the tibialis anterior, peroneus longus, and extensor muscles of toes, and grade 4 weakness of the dorsal interossei, thenar, hypothenar, extensor muscles of fingers and tibialis posterior bilaterally, while the gastrocnemius muscles were spared. Pes cavus was not present.Deep tendon reflexes were diminished in all the extremities. There was glove-and stocking-type sensory disturbance for touch, pain and temperature, while the deep sensation was spared. He showed steppage gait. Serum concentrations of creatinine kinase [927 IU/L (normal \ 306)] were elevated. CSF was normal.

Hereditary motor sensory neuropathy type VI (HMSN VI) is hereditary neuropathy accompanied by optic neuropathy. The feasibility of Coenzyme Q10 (CoQ10) as a treatment for subacute visual impairment of HMSN VI was examined.A 37-year-old patient with HMSN VI with a novel mitofusin 2 mutation was treated with high dose of CoQ10 (200 mg/day) for eight months.Visual impairment was partially resolved after CoQ10 therapy. High dose CoQ10 therapy may improve the prognosis of subacute visual impairment in HMSN VI. To confirm the effectiveness of CoQ10 on HMSN VI, further studies are needed.

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A 38-year-old man with pneumococcal meningitis, pneumocephalus, and CSF rhinorrhea was admitted to our hospital. The CSF rhinorrhea had no traumatic or iatrogenic etiology. The patient was treated with meropenem and recovered from meningitis.Axial plain CT showed pneumocephalus (figure 1, A and B), but no skull defects. Three-dimensional CT revealed defects of the posterior walls of the sphenoidal sinus (clivus) (figure 2A), which were not seen on plain CT (figure 1, C-F), although it was reported that clivus defects could be detected by axial plain CT.

Layered perovskite (RNH3)2PbI4 (R = organic cation), which contained the fullerene derivatives, [ethyl-3-tert-butoxycarbonylaminopropyl (1,2-methanofullerene C60)]-61,61-di carboxylate iodide (EAF-I) and di [3-tert-butoxycarbonylaminopropyl (1,2-methanofullerene C60)]- 61,61-dicarboxylate diiodide (DAF-I2) in the organic layers were fabricated and compared with previously reported perovkite compound containing N-methyl-2-(4-aminophenyl)-fulleropyrrolidine iodide (AmPF-I). Because the solvent solubilities of EAF-I and DAF-I2 to N,N-dimethylformamide (DMF) were higher than AmPF-I, the film processability of EAF-I and DAF-I2 was improved compared with AmPF-I. The X-ray diffraction patterns proved the construction of the perovskite structure in (EAF)2PbI4 and (AmPF)2PbI4 cast films. EAF was well-organized and closer-packing than AmPF in the perovskite structure. For the diamine-type fullerene, DAF-I2, it is difficult to form layered structure by spin-coated or cast methods. New sharp X-ray diffraction peaks were observed for DAF-I2 films dipped in PbI2 solution. The intercalations and formation of perovskite structure of (DAF)PbI4 were studied.