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A 38-year-old Japanese man presented with a 2-year history of cerebellar ataxia, fever, and headaches. MRI revealed migration of the lesion (figure, A-C). CSF cytology showed eosinophilic meningitis (figure, D); serum and CSF ELISA were positive for Spirometra erinaceieuropaei.Human sparganosis is a rare parasitic infection by the larval cestode of Spirometra that results from ingesting the plerocercoid harbored in frogs, snakes, and chickens. Reported worldwide, sparganosis is most prevalent in Southeast and Eastern Asia. The diagnosis is suggested by a wandering lesion, especially in endemic areas; the tunnel sign on postcontrast MRI is characteristic.1 The preferred treatment is the surgical removal of live worm.

Dickson DW. The relationship between histopathological features of progressive supranuclear palsy and disease duration. Parkinsonism Relat Disord 2006;12:109 -112. 7. Demer JL. Pivotal role of orbital connective tissues in binocular alignment and strabismus: the Friedenwald lecture. Invest Ophthalmol Vis Sci 2004;45:729 -738. NOVEL COMPOUND HETEROZYGOUS ALS2 MUTATIONS CAUSE JUVENILE AMYOTROPHIC LATERAL SCLEROSIS IN JAPANHomozygous mutations in ALS2 on chromosome 2q33 are responsible for autosomal recessive, early-onset forms of upper motor neuron diseases such as infantile ascending hereditary spastic paraplegia (IAHSP) and juvenile primary lateral sclerosis (JPLS). 1 These mutations are rarely associated with lower motor neuron involvement in juvenile amyotrophic lateral sclerosis (JALS). 2 Seventeen mutations from 16 families have been reported, but the phenotype-genotype correlation remains unclear. 3-5 We searched for ALS2 mutations in 2 Japanese siblings presenting with infantile-onset ascending spastic paraplegia, pseudobulbar palsy, and amyotrophy of the extremities.Case reports. The older sibling was a 32-year-old Japanese man who started walking on tiptoes at 13 months of age and had never run. He developed dysarthria at 11 years of age and lost the ability to speak at 14. He displayed spasticity predominantly in the lower limbs, bilateral extensor plantar responses, and complete paralysis of the tongue without atrophy or fasciculation at 32 years of age. He also had discrete signs of lower motor neuron involvement manifesting as muscular atrophy of the distal upper and lower limbs. However, he was ambulatory with support and there was no evi-dence of cognitive dysfunction. Brain and spinal MRI showed no abnormalities. The peripheral nerve conduction velocities and latencies were within normal limits, but electromyography showed chronic denervation in the tongue and leg muscles. A 23-year-old brother showed progressive symptoms similar to his older brother; however, his symptoms were relatively mild. He started walking on tiptoes at 3 years of age, but he was able to participate in gymnastics in elementary school. He experienced slight muscular atrophy in the extremities. His speech was unintelligible, but his cognitive function was normal. Their nonconsanguineous parents had no neuromuscular disorders.An analysis of the ALS2. After obtaining informed consent, genomic DNA and RNA were extracted from the patients' blood. We directly sequenced all of the 34 exons and flanking intronic regions of the ALS2 and cDNA was synthesized by reversed transcription methods. Novel compound heterozygous mutations were detected in the ALS2 in each patient. These mutations comprised a 1-bp deletion at position 3565 leading to the creation of a premature stop codon and a transition of guanine to cytosine 5 nucleotides downstream from the normal exon 22 5Ј splice site