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Large chromosomal deletions that span LIS1 cause Miller-Dieker syndrome, in which lissencephaly is associated with distinctive facial abnormalities; small defects in the same gene cause only lissencephaly Lissencephaly with cerebellar hypoplasia is caused by mutations in the human reelin gene (RELN), the analogue of the defective gene in reelin mice (which show a reeling gait and abnormal cortical neuronal lamination) Defects in the transcription factor ARX are associated with X-linked lissencephaly, agenesis of the corpus callosum, and hypogonadism Periventricular nodular heterotopia is caused by another gene defect, lamin A gene on the X chromosome Microcephaly In the above-mentioned cerebral dysplasias, the cranium and brain are small, but there is also a primary form of hereditary microcephaly, called microcephaly vera, in which the head is astonishingly reduced in size (circumference less than 45 cm in adult life ie, 5 standard deviations below the mean).

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Diagnosis A syndrome of symmetrical weakness and fatigability of proximal muscles coupled with dry mouth, sphincter distur-.

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In contrast, the face is of normal size, the forehead is narrow and recedes sharply, and the occiput is at Stature is usually moderately reduced Such individuals can be recognized at birth by their anthropoid appearance and later by their lumbering gait, extremely low intelligence, and lack of communicative speech Vision, hearing, and cutaneous sensation are spared In one of our colleagues cases, by laborious effort using operant conditioning, it was possible to teach the patient the shapes of simple gures (His sister s brain, examined by R D Adams, was malformed and weighed only 280 g) In such cases the tendon re exes in the legs are brisk, and the plantar re exes may be extensor Skull lms show that the cranial sutures are present, as are convolutional markings on the inner table.

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This is definitely one area in which we cannot possibly cover all the bells and whistles in detail. As you read through this section, you ll understand why. Each command has a list of additional parameters that can be used to enhance the functionality of the command. In some cases, the list is extremely long, and it would be impractical to attempt to define each parameter. Therefore, I will show you through an example the basic procedural steps to set up a command. As you read through the list of commands, follow these steps to try out some of the other commands, to get comfortable with their functionality. If you use one of the Web items the command wizard is called from the Properties tab option of that particular Web item. When you access the initial screen, you see two tabs: Favorite Commands and All Commands. If you select the Button Group Web item and then access the command wizard, the initial screen is the Favorite Commands tab, shown next. You can assign any number of commands to this Favorite Commands tab by choosing them in the list.

The brain often weighs less than 300 g (normal adult range, 1100 to 1500 g) and shows only a few primary and secondary sulci The cerebral cortex is thick and unlaminated and grossly de cient in neurons In a few reported cases, there has been an associated cerebellar hypoplasia or an infantile muscular atrophy Lesser degrees of microencephaly have been associated with progressive motor neuron disease and degeneration of the substantia nigra (Halperin et al) Evrard and associates have described another rare, special type of microcephaly, which they call radial microbrain Birth is at term, but the infant dies in the rst month of postnatal life The sulcal pattern is normal, and neuronal arrangements in the cerebral cortex are normal as well The defect appears to be in the small number of neurons that are generated, not in their migration.

Biological Disorder is usually tied to: (a) The creation of a particular pattern associated with living things (b) A healthy and disease-free state (c) Breaking of a particular pattern associated with living things (d) Normal body structure and function Ecology is best de ned as: (a) The removal of diseased and damaged plants and animals from the environment (b) Study of the relationships between di erent organisms and their environment (c) The wide occurrence of Biological Disorder within the external environment (d) A complete lack of association between di erent organisms and their environment Natural History essentially approaches Biological Order by: (a) Naming and classifying newly discovered organisms (b) Testing a formal hypothesis (c) Applying the experimental method in a consistent manner (d) Conducting experiments on living organisms and reaching conclusions from the results Homeostasis: (a) Does not usually exist in healthy individuals (b) Represents a control of sameness or relative constancy of the body s internal environment (c) Is basically the same thing as ecological relationships (d) Was extensively studied and de ned by Aristotle

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