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Corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) have some clinical and pathological features in common. Each, however, has been shown to have specific astrocytic inclusions. CBD is characterized by astrocytic plaques, and PSP is characterized by tuft-shaped astrocytes. To clarify differences between these inclusions, we investigated intracerebral distribution of astrocytic plaques and tuft-shaped astrocytes in autopsied brains of patients with either CBD or PSP. Specimens from ten patients with CBD and five patients with PSP were stained by the Gallyas-Braak method. Densities of the astrocytic plaques and tuft-shaped astrocytes were determined for 11 cerebral cortical regions, 6 subcortical nuclei, 5 brain stem regions, the cerebellar cortex and the dentate nucleus. Astrocytic plaques were most abundant in the prefrontal and premotor areas in the cerebral cortex of CBD brains, whereas tuft-shaped astrocytes were most prominent in the precentral gyrus and premotor area of PSP brains. Many astrocytic plaques were observed in the caudate nucleus of CBD brains, whereas tuft-shaped astrocytes were abundant in both the caudate and putamen and moderate in number in the globus pallidus, subthalamic nucleus and thalamus in PSP brains. Very slight accumulation of astrocytic plaques was seen in the brain stem of CBD brains, whereas numerous tuft-shaped astrocytes were found in the red nucleus and superior colliculus of PSP brains. Distribution of the astrocytic plaques and tuft-shaped astrocytes differed greatly. Thus, CBD and PSP can be considered different entities.

Vertebral deformities in red sea bream, Pagrus major, remain serious obstacles to the improvement of seedling quality for its aquaculture. However, the causalities of the deformities remain unclear and prevention methods have not yet been established. In this paper, oxygen deficiency during somitogenesis was demonstrated to cause centrum defects (formerly called fused vertebrae in many cases), which are the major vertebral deformity in cultured red sea bream. An induction experiment of centrum defects was conducted by placing fertilized red sea bream eggs under low dissolved oxygen conditions (10.3–16.6%). The low oxygen treatment was carried out for five different developmental stages of embryo: two‐cell stage to blastula stage; gastrula stage; three to 10 somites stage; 11–17 somites stage and 18–24 somites stage. Oxygen deficiency during somitogenesis induced a high incidence of centrum defects. In contrast, it hardly induced centrum defects during the other stages. The dissolved oxygen concentration in the rearing water should be carefully regulated for fertilized eggs, especially during somitogenesis to reduce the incidence of vertebral deformities in the red sea bream.

We describe two sisters with a mild‐onset variant of Canavan's disease who presented at age 50 and 19 months with developmental delay but without macrocephaly, hypotonia, spasticity, or seizures. Remarkably, both patients had age‐appropriate head control, gross motor development, and muscle tone. There were very mild deficits in fine motor skills, coordination, and gait. Both sisters had a history of strabismus, but otherwise vision was normal. The older child showed evidence of mild cognitive and social impairment, whereas language and behavior were normal for age in the infant. Both patients were found to be compound heterozygotes for C914A (A305E) and G212A (R71H) mutations in ASPA. Like all other known ASPA mutations, this previously unknown G212A mutation appears to have low absolute enzyme activity. Nevertheless, it is associated in these patients with an extremely benign phenotype that is highly atypical of Canavan's disease. Biochemical and clinical data were evaluated using a generalized linear mixed model generated from 25 other subjects with Canavan's disease. There were statistically significant differences in brain chemistry and clinical evaluations, supporting a distinct variant of Canavan's disease. Future studies of ASPA enzyme structure and gene regulation in these subjects could lead to a better understanding of Canavan's pathophysiology and improvements in ASPA gene therapy Ann Neurol 2006;59:428–431