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Cu/Zn superoxide dismutase (SOD)-like immunoreactivity (LI) was found within Lewy body-like inclusions (LBIs) in the spinal cords of patients with sporadic amyotrophic lateral sclerosis (ALS) by using an antibody to human Cu/ZnSOD. LBIs were detected in the anterior horn cells in 10 of 20 patients with sporadic ALS. In each of these patients, 7 to 60% of(More)
A topographical immunocytochemical analysis was performed on the substantia nigra from patients with idiopathic Parkinson's disease and striatonigral degeneration. Antibodies to tyrosine hydroxylase, a marker for nigrostriatal dopaminergic neurons, and to calcineurin, a marker for striatonigral projection fibers, were used in this study. There was a marked(More)
This report concerns immunocytochemical and ultrastructural studies on the basophilic inclusions in two cases of sporadic juvenile amyotrophic lateral sclerosis (ALS). The inclusion had a globular, irregular-shaped, or sometimes fragmented appearance. Ultra-structurally, the inclusions consisted mainly of thick filamentous structures associated with(More)
This report concerns a case of sporadic amyotrophic lateral sclerosis (ALS) with dementia and Lewy body-like hyaline inclusions (LBHIs). The patient was a 70-year-old woman who initially showed memory disturbance and later developed bulbar palsy, muscle atrophy and weakness. The total clinical course was 51 months. The postmortem examination revealed(More)
Dystonia is a neurological syndrome characterized by sustained muscle contractions that produce repetitive twisting movements or abnormal postures. X-linked recessive dystonia parkinsonism (XDP; DYT3; Lubag) is an adult-onset disorder that manifests severe and progressive dystonia with a high frequency of generalization. In search for the anatomical basis(More)
A 36-year-old man developed motor neuron signs consisting of weakness and atrophy of the right upper limb, which progressed to involve the other limbs along with development of upper motor neuron signs including pseudobulbar palsy. He died 8.5 years after onset. Bilateral precentral gyri and putamina were grossly atrophic. In addition to severe degeneration(More)
Idiopathic normal pressure hydrocephalus (iNPH) is a treatable cause of dementia, gait disturbance, and urinary incontinence in elderly patients with ventriculomegaly. Its unique morphological feature, called disproportionately enlarged subarachnoid-space hydrocephalus (DESH), may also be a diagnostic feature. Lipocalin-type prostaglandin D synthase(More)
We have previously demonstrated that an antibody to phosphoinositide-specific phospholipase C (PLC) isozyme, PLC-delta, intensely stained neurofibrillary tangles (NFT) in the brain tissue of Alzheimer's disease (AD). This study was performed to determine if abnormal PLC-delta accumulation might be present in the filamentous inclusions of other(More)
A 53-year-old man initially displayed muscle atrophy and weakness and a slight degree of pyramidal signs, which became more prominent with time. Later, gaze palsy, autonomic dysfunction, and bedsore developed. The total clinical course was 63 months. Pathologically, there was loss of upper and lower motor neurons and other regions, as well as degeneration(More)