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Most current speech recognition systems use hidden Markov models (HMMs) to deal with the temporal variability of speech and Gaussian mixture models (GMMs) to determine how well each state of each HMM fits a frame or a short window of frames of coefficients that represents the acoustic input. An alternative way to evaluate the fit is to use a feed-forward(More)
This paper reviews hierarchical observation models, used in functional neuroimaging, in a Bayesian light. It emphasizes the common ground shared by classical and Bayesian methods to show that conventional analyses of neuroimaging data can be usefully extended within an empirical Bayesian framework. In particular we formulate the procedures used in(More)
The ratio of acoustic myography (AMG) amplitude to surface electromyography (EMG) amplitude is proposed as a measure of mechanical output compared with electrical activity of the contractile system. AMG to EMG ratios were measured from 16 children with muscle disease diagnosed by clinical criteria, EMG, and/or muscle biopsy. These were compared with the(More)
The clinical, pathological, and biochemical findings in a young woman with a new variant of metachromatic leukodystrophy (MLD) are reported. The patient showed slow early development and deteriorated further during her first two decades. Nerve conductions were slow, and a sural nerve biopsy showed features of a sulfatide lipidosis. Urinary sulfatide(More)
Juvenile cerebellar astrocytoma characteristically has a very benign course and good prognosis. A case is reported of juvenile cerebellar astrocytoma with massive craniospinal leptomeningeal spread prior to surgical intervention. The patient died 8 months after the onset of symptoms and only 5 weeks after presentation to the hospital. At postmortem(More)
We present a connectionist method for representing images that explicitly addresses their hierarchical nature. It blends data from neu-roscience about whole-object viewpoint sensitive cells in inferotem-poral cortex 7 and attentional basis-eld modulation in V4 3 with ideas about hierarchical descriptions based on microfeatures. 4, 10 The resulting model(More)
Hereditary trembling chin is an autosomal dominant condition characterized by recurrent bouts of tremor involving the chin. These episodes are precipitated by emotional upset. There has been considerable debate about the gravity of this condition. This may be a benign movement disorder; however, the rhythmic trembling of mentalis at rest or during times of(More)
This first child of non-Jewish parents had nystagmus at 4 months of age, bilateral cherry-red macular spots at 7 months of age, and hyperacusis at 8 months of age; the patient has deteriorated progressively following a clinical course typical of Tay-Sachs disease B variant. Total beta-N-acetylhexosaminidase assayed with 4-methylumbelliferyl-beta-glucosamine(More)