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The cause of this peculiar condition is not known The few adequate pathologic studies have shown a lack of nerve cells in the motor nuclei of the brainstem, changes that also characterize the Fazio-Londe type of muscular dystrophy (page 947) Rarely, there may be an aplasia of facial muscles The Mobius syndrome is also referred to on page 1246 in relation to restricted palsies of myopathic and nuclear origin Partial paralysis of facial muscles that dates from birth and cannot be attributed to obstetric trauma is not infrequent In a common type, the lower lip on one side remains immobile when the child smiles or cries; the lip on the unaffected side is drawn downward and outward, resulting in a prominent asymmetry of the lower face Often it is not appreciated that the side that droops during crying is the normal side (Hoefnagel and Penry).

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In one of our familial cases, a dystonia of the foot appeared during adolescence and later disappeared; we have also observed this time-limited occurrence to happen occasionally in spasmodic torticollis, which is a common form of restricted or segmental dystonia, described further on In a survey of idiopathic torsion dystonia of local and generalized type in the United Kingdom by Fletcher and associates, the investigators concluded that approximately 85 percent of these cases were due to an autosomal dominant gene of low penetrance and that a small proportion represented new mutations The risk of the disease in a rst-degree relative of a familial case is about 25 percent Molecular genetic studies, though still incomplete, hold the.

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Congenital Lack of Lateral Gaze (Oculomotor Apraxia of Cogan) (See page 228) Children with this congenital defect are unable to turn their eyes to either side volitionally or on command Attempting to look to the right, the child turns the head to the right (there is no associated apraxia of head turning, as in acquired apraxia), but the eyes lag and turn to the left As a result, the patient has to overshoot the mark with the head in order to attain ocular xation Once the eyes xate, the head returns to the primary position To compensate for the de ciency of eye movements, the patient develops jerky thrusting movements of the head, which characterize all attempts at voluntary gaze Caloric stimulation of the labyrinth causes tonic movement of the eyes but not nystagmus, as in the normal person Also, optokinetic nystagmus cannot be induced Vertical eye movements are normal.

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A similar ocular condition may occur in conjunction with ataxiatelangiectasia and Gaucher disease Children with oculomotor apraxia are slow to walk; Ford observed one such child whose sibling had an absence of the vermis of the cerebellum Aside from.

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04/12/10

Diagnostic logging is another form of logging you can enable on Exchange servers. This feature is used primarily to troubleshoot problems with Exchange services and generates entries in the Windows 2000 Event logs. Table 24.2 lists the different Exchange services on which diagnostic logging can be enabled, together with their Event Source names, which identify these events in the Event logs.

the variance predicted by P . Analysis of residual sequences is one of the best methods to detect model de ciencies. In doing so, the analyst should keep in mind that Hk Pk Hk + Rk is predicting the variance of the residual rk over an ensemble of experiments. Therefore, for example, the residual sequence of Figure 6.7 does indicate a model de ciency, but by itself does not indicate that the residual is biased. If the experiment was repeated N times, with the residual sequence from the i-th trial run denoted by rki , and all residual sequences tended to drift in the same direction, then the analyst might reconsider the system model to nd the cause of and model the bias. AlterN natively, if the average of the residual sequences rk = i=1 rki is essentially zero mean, but the ensemble of residual sequences is not well-modeled by the P , then the analyst must consider possible alternative explanations. To illustrate the nature of a residual sequence for a lter designed with a correct model, Figure 6.8 shows the residual for the lter designed with Qh = 0.5. The residual is essentially zero mean, has no noticeable trends, and has steady-state variance that closely matches the P .

Whatever you do, if you have two or more storage groups, never place their transaction log files on the same physical drive or performance will be terrible! Writing transaction logs is a very disk-intensive process and needs its own separate physical drive (with its own controller) for each set of logs. From this setup, with its liberal use of fault-tolerant disk technologies (disk mirroring and hardware RAID), we can see that our Exchange data is well protected against operating-system crashes and data loss. If

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