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You can use this variable to permanently enable profiling or to use an alternative debugger (including those with windowed interfaces) See 21 for more information on using the Perl debugger

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a spastic or ataxic paraparesis The tendon re exes are retained and later become hyperactive with extensor plantar re exes; disappearance of the abdominal re exes and varying degrees of deep and super cial sensory loss may be associated It is a useful adage that the patient with MS presents with symptoms in one leg but with signs in both; the patient will complain of weakness, incoordination, or numbness and tingling in one lower limb and prove to have bilateral Babinski signs and other evidence of bilateral corticospinal and posterior column disease Flexion of the neck may induce a tingling, electric-like feeling down the shoulders and back and, less commonly, down the anterior thighs This phenomenon is known as a Lhermitte sign, although it is more a symptom than a sign and was originally described by Babinski in a case of cervical cord trauma Lhermitte s contribution was to draw attention to the frequent occurrence of this phenomenon in MS It is attributable to an increased sensitivity of demyelinated axons to the stretch or pressure on the spinal cord induced by neck exion There are, in addition, several syndromes that are typical of multiple sclerosis and may be the initial manifestations These common modes of onset are: (1) optic neuritis, (2) transverse myelitis, (3) cerebellar ataxia, and (4) various brainstem syndromes (vertigo, facial pain or numbness, dysarthria, diplopia) These are often features of the established disease as well When they represent the initial phases of the illness, they may pose diagnostic questions, since they also certainly occur with numerous diseases other than MS Optic Neuritis (See also page 213) In about 25 percent of all MS patients (and in a larger proportion of children), the initial manifestation is an episode of optic neuritis It will be recalled that the optic nerve is in fact a tract of the brain, and involvement of the optic nerves is therefore consistent with the rule that lesions of MS are con ned to the CNS Characteristically, over a period of several days, there is partial or total loss of vision in one eye Many patients, for a day or two before the visual loss, experience pain within the orbit, worsened by eye movement or palpation of the globe Rarely, the visual loss is steadily progressive for several weeks, mimicking a compressive lesion or intrinsic tumor of the optic nerve (Ormerod and McDonald) Usually a scotoma involving the macular area and blind spot (cecocentral) can be demonstrated, but a wide variety of other eld defects may occur, rarely even hemianopic involvement (sometimes homonymous) In some patients, both optic nerves are involved, either simultaneously or, more commonly, within a few days or weeks of one another, and at least 1 in 8 patients will have repeated attacks Serial examinations may disclose evidence of swelling or edema of the optic nerve head (papillitis) in about half the patients The occurrence of papillitis depends on the proximity of the demyelinating lesion to the nerve head As emphasized in Chap 13, papillitis can be distinguished from the papilledema of increased intracranial pressure by the severe and acute visual loss that accompanies only the former More often, the optic nerve head appears normal or nearly so; this represents a retrobulbar neuritis Subtle manifestations of optic nerve affection such as an afferent pupillary defect (page 206), atrophy of retinal nerve bers or sheathing of retinal veins (page 213) and abnormalities of the visual evoked response (page 30) should be sought in patients who have no visual complaints but are suspected of having MS In cases of substantial visual loss there is a diminished pupillary response to light (afferent pupillary paralysis) and instability of the direct pupillary response,.

This is specific to the Win32 port of Perl (see 22) It specifies the alternative shell that Perl should use internally for executing external commands via system or

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