The Neuronal Ceroid Lipofuscinoses (NCL) are the most common autosomal recessive neurodegenerative disorders in childhood. Ten different subtypes are classified based on age at onset, clinical features, biochemical aspects and genetic background. NCL6 (vLINCL gene on chromosome 15 q21-q23) has been associated with the Late-Infantile variant form and presents with epileptic seizures, myoclonus, speech delay, progressive mental and motor deterioration, and visual failure. We report a case of a boy of 13 years who present at the age of 5 with speech delay and mild coordination disorder. In the following few years he developed parkinsonism, ataxia, neuromotor regression, dysarthria with severe palylalia and generalized epilepsy. The mental functioning was relatively preserved. Brain MRI was initially normal and the possibility of a Dopa-responsive disorder was considered. However, CSF examination showed normal neurotransmitters and pterins. Afterwards a progressive cerebral and cerebellar atrophy became evident associated with a severe decline of white and gray matter NAA on H-MRS. In contrast there was no evidence of retinopathy with loss of vision. Genetic analysis of the vLINCL 6 gene revealed that the patient was homozygous for the F234L mutation. This case suggests that NCL6 should be considered in the differential diagnosis of early onset parkinsonism. We describe a 4 year old female patient born healthy and naturally, after normal pregnancy course. Neurophysiological development and anthropometric parameters were reported as adequate until 2=years old, when she showed episodes of acute vomiting, with concomitant language and neurologic disturbance and macrocephaly. The girl was diagnosed with a phonological disorder with specific language impairment. The following investigations were done when she came to our department, at 4 years of age: aminoacids, screening for congenital disorders of glycosylation (CDG), galactocerebrosidase, arylsulfatase A, VLCFA, N-acetylaspartate, karyotype, telomeres, urinary aminoacids, oligosaccharides, mucopolysaccharides and organic acids, electroencephalogram, were all normal. MRI showed frontal lobe white matter abnormalities. Mutation analysis for the GFAP gene was positive. Novel DNA mutations in exon 4: c.715 C>T (p.R239C) of the glial fibrillary acidic protein gene (GFAP), underlie the infantile form of Alexander disease, a rare autosomal recessive disorder (1:100.000) of the central nervous system of unknown etiology, characterized by the accumulation of Rosenthal fibers. Some authors have reported the p. R239C mutation in 4 patients with Alexander disease, 3 of them died before 4 years old and 1 before 6. So far this mutation was reported in patient with hereditary spastic paraplegia. Takahashi, T P-193 Takakura, H P-398 Takayanagi Tedesco, L P-392  Teerlink, T P-114, P-110  Teertstra, TK P-042  Tehrani, FT P-033  Tel, F A-007  Teles, EL P-  Assessment of Virally Vectored Autoimmunity as a Biocontrol Strategy for Cane Toads BACKGROUND: The cane toad, Bufo (Chaunus) marinus, is one of the most notorious vertebrate pests introduced into Australia over the last 200 years and, so far, efforts to identify a naturally occurring B. marinus-specific pathogen for use as a biological control agent have been unsuccessful. We explored an alternative approach that entailed genetically modifying a pathogen with broad host specificity so that it no longer caused disease, but carried a gene to disrupt the cane toad life cycle in a species specific manner. METHODOLOGY/PRINCIPAL FINDINGS: The adult beta globin gene was selected as the model gene for proof of concept of autoimmunity as a biocontrol method for cane toads. A previous report showed injection of bullfrog tadpoles with adult beta globin resulted in an alteration in the form of beta globin expressed in metamorphs as well as reduced survival. In B. marinus we established for the first time that the switch from tadpole to adult globin exists. The effect of injecting B. marinus tadpoles with purified recombinant adult globin protein was then assessed using behavioural (swim speed in tadpoles and jump length in metamorphs), developmental (time to metamorphosis, weight and length at various developmental stages, protein profile of adult globin) and genetic (adult globin mRNA levels) measures. However, we were unable to detect any differences between treated and control animals. Further, globin delivery using Bohle iridovirus, an Australian ranavirus isolate belonging to the Iridovirus family, did not reduce the survival of metamorphs or alter the form of beta globin expressed in metamorphs. CONCLUSIONS/SIGNIFICANCE: While we were able to show for the first time that the switch from tadpole to adult globin does occur in B. marinus, we were not able to induce autoimmunity and disrupt metamorphosis. The short development time of B. marinus tadpoles may preclude this approach. The spread of the cane toad, Bufo (Chaunus) marinus, into the Australian environment following the initial introduction at Gordonvale near Cairns, Queensland in 1935 has been spectacularly successful. Cane toads are now present throughout most of tropical northern Australia and their range is continuing to expand into world heritage areas. Predicted warming due to climate change could extend the range of the cane toad south into what are currently temperate regions [1] . The cane toad is a highly toxic and hardy introduced species and presents wide ranging ecological and social impacts within the Australian landscape. Native species such as quolls, goannas and native frogs are particularly susceptible to the cane toad toxin and many populations have been severely impacted by the arrival of cane toads [2, 3, 4] .