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dergo slight atrophy, in a few cases, the atrophy of hand muscles is severe Interestingly, in such cases, the spondylotic compression, as judged by MRI or CT myelography, may be con ned to the high cervical cord, well above the levels of the motor neurons that innervate these muscles In patients with sensory loss, pain and thermal sensation often appear to be affected more than tactile sense An unexpected Babinski sign has already been mentioned and a few fasciculations may be seen, especially in proximal muscles As the myelopathy progresses, sometimes intermittently, both legs become weaker and more spastic Sphincteric control may then be altered; slight hesitancy or precipitancy of micturition are the usual complaints; frank incontinence is infrequent In the more advanced form of this condition, walking requires the aid of a cane or canes or a walker; in some cases all locomotion ultimately becomes impossible, especially in the elderly patient Abrupt worsening, even paraplegia or quadriplegia, may follow forceful exion or extension injuries of the neck, as indicated later Pathologic Changes The fundamental lesion is generated initially by a fraying of the annulus brosus, with extrusion of disc material into the spinal canal The disc becomes covered with brous tissue or partly calci ed, thereby forming a transverse osteophytic spondylitic bar or there may be simply central bulging of the annulus without extrusion of nuclear material The latter changes, unlike ruptured discs that occur mainly at the C5-6 or C67 interspace, often involve higher interspaces and almost invariably occur at several adjacent levels The dura mater may be thickened and adherent to the posterior longitudinal ligament at affected levels The underlying pia-arachnoid is also thickened and the adjacent ligamentous hypertrophy contributes to compression of the cord or the nerve roots This series of pathologic changes is often ascribed to hypertrophic osteoarthritis However, in lesser degree, the osteophyte formation and ridging are so frequently observed in patients who have no other signs of arthritic disease that this explanation is surely not totally correct Subclinical trauma is far more likely to be the cause of spondylotic bar formation, in the authors opinion When a cervical nerve root is compressed by lateral osteophytic overgrowth, the dural sleeve is thickened and occluded and the root bers are damaged Usually the fth, sixth, or seventh cervical roots are affected in this way, both the anterior and posterior or only the anterior, on one or both sides A small neuroma may rarely appear proximal to the site of anterior root compression The dura is ridged and the underlying spinal cord is attened The root lesions may lead to secondary wedge-shaped areas of degeneration in the lateral parts of the posterior columns at higher levels The most marked changes in the spinal cord are at the level(s) of compression There may be zones of demyelination or focal necrosis at the points of attachment of the dentate ligaments (which tether the spinal cord to the dura) and areas of rarefaction in the posterior and lateral columns as well as loss of nerve cells Ventral gray matter lesions, often asymmetrical, are attributed by Hughes to ischemia Pathogenesis The particular vulnerability of the cervical spine to degenerative change has no ready explanation Most likely it is related in some way to the high degree of mobility of the lower cervical vertebrae, which is accentuated by their location next to the relatively immobile thoracic spine The mechanism of spinal cord injury would seem to be one of simple compression and ischemia as mentioned When the spinal.

(See Table 50-1) This is another X-linked muscular dystrophy, characterized by the special feature of muscle contractures, but relatively benign in comparison with the Duchenne type It was described originally by Emery and Dreifuss and subsequently by Hopkins and by Merlini and their colleagues The primary gene defect is a de ciency of the protein emerin, encoded by a gene on the X chromosome Emerin is a constituent of the nuclear membrane (Fig 50-2) The age of onset varies from childhood to late adolescence or adulthood Weakness affects rst the upper arm and pectoral girdle musculature and later the pelvic girdle and the distal muscles in the lower extremities A consistent and distinguishing feature of the disease is the early appearance of contractures in the exors of the elbow, extensors of the neck, and posterior calf muscles Facial

Facioscapulohumeral (FSH) Muscular Dystrophy (Landouzy-Dejerine Muscular Dystrophy) (See Table 50-2)

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674 Test: Part four 28 At low volume levels in an audio amplifier system, the dynamic range is limited primarily by the: A Input impedance B Efficiency C Input power D Noise level E Frequency 29 In a wireless system, noise always: A Improves the bandwidth B Increases data transfer rate C Reduces the transmitted signal power D Degrades performance E Makes communication or data transfer impossible 30 The noise level in a wireless receiver can be reduced using: A A more powerful final amplifier B A grounded-grid configuration C Cryotechnology D The Global Positioning System E A large antenna 31 Image resolution is an important specification in: A Oscillators B Computer monitors C Receiver sensitivity D Rectifier diodes E Superheterodyne receivers 32 According to Asimov s laws, a robot must obey all orders from humans: A No matter what B As long as nobody gets hurt or killed as a result C As long as the power is on D Unless its controller is infected with a computer virus E As long as it does not cause the robot controller to crash 33 The use of a cell phone is prohibited in a commercial aircraft in flight because: A It can annoy other passengers B It can interfere with other people s cell phones C The interior of the aircraft produces a dangerous RF resonant cavity D It won t work anyhow

This is a slowly progressive dystrophy involving primarily the musculature of the face and shoulders, often with long periods of nearly complete arrest The pattern of inheritance is usually autosomal dominant While less common than the Duchenne and myotonic dystrophies, this disease is not rare (an estimated yearly incidence rate of 5:100,000) and we see one or more new cases yearly The age of onset is usually between 6 and 20 years but cases beginning in early adult life are occasionally encountered As a rule, the rst manifestations are dif culty in raising the arms above the head and winging of the scapulae, although in many cases bifacial weakness may have initially attracted attention, even in early childhood There is involvement especially of the orbicularis oculi, the zygomaticus, and the orbicularis oris, whereas the masseters as well as the temporalis, extraocular, pharyngeal, and respiratory muscles

Table 50-2 Selected muscular dystrophies*

Myotonic dystrophy Proximal myotonic myopathy Facioscapulohumeral dystrophy Oculopharyngeal dystrophy Bethlem myopathy

Expanded intronic CTG repeat in myotonin kinase Expanded intronic CCTG repeat in zinc nger protein Multigene dysregulation at 4q telomore Exonic GCG expansion (alanine) in poly-A binding protein Collagen VI, subunits 1-3

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