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nects to several receptors, all of one type, which are irregularly distributed in the skin and account for sensory spots Stimuli from a given spot are conveyed by two or more bers The proprioceptive bers subserve pressure sense and, with endings in articular structures, the sense of position and movement; they enable one to discriminate the form, size, texture, and weight of objects Sensations of tickle, itch, and wetness are believed to arise from combinations of several types of receptors Itch is a distinctive sensation that can be separated on clinical and neurophysiologic grounds from pain Two aspects are recognized: a brief primary localized itch at the site of stimulus or injury, and a subsequent more diffuse sensation that is greatly intensi ed by gentle touch The itch sensation is transmitted by speci c C bers, similarly speci c neurons in the dorsal horns of the cord and directly via the spinothalamic projections to the thalamus, all of which differ from the similar structures utilized by pain There is, however, no specialized peripheral itch receptor, the sensation depending instead on the spinal connections to itch pathways Several forebrain regions are activated by itch; ie, there is no itch center From a neurologic rather than a dermatologic perspective, the main cause of itch is postherpetic neuralgia, but cases of paroxysmal itch of central origin are known to be due to multiple sclerosis The pathophysiology of itching has been discussed by Greaves and Wall and is reviewed further by Yosipovitch and colleagues Spinal Roots All the sensory neurons have their cell bodies in the dorsal root ganglia The peripheral extensions of these cells constitute the sensory nerves; the central projections of these same cells form the posterior (dorsal) roots and enter the spinal cord Each dorsal root contains all the bers from skin, muscles, connective tissue, ligaments, tendons, joints, bones, and viscera that lie within the distribution of a single body segment, or somite This segmental innervation has been amply demonstrated in humans and animals by observing the effects of lesions that involve one or two spinal nerves, such as (1) herpes zoster, which also causes visible vesicles in the corresponding area of skin; (2) the effects of a prolapsed intervertebral disc, which causes hypalgesia in a single root zone; and (3) surgical section of several roots on each side of an intact root (method of remaining sensibility) Maps of the dermatomes derived from these several types of data are shown in Figs 9-2 and 9-3 It should be noted that there is considerable overlap from one dermatomal segment to the other, more so for touch than for pain By contrast, there is little overlap between branches of the trigeminal nerve Also, the maps differ somewhat according to the methods used in constructing them In contrast to most dermatomal charts, those of Keegan and Garrett (based on the injection of local anesthetic into single dorsal root ganglia) show bands of hypalgesia to be continuous longitudinally from the periphery to the spine (Fig 9-3) The distribution of pain bers from deep structures, though not exactly corresponding to that of pain bers from the skin, also follows a segmental pattern Posterior Columns In the dorsal roots, the sensory bers are rst rearranged according to function Large and heavily myelinated bers enter the cord just medial to the dorsal horn and divide into ascending and descending branches The descending bers and some of the ascending ones enter the gray matter of the dorsal horn within a few segments of their entrance and synapse with nerve cells in the posterior horns as well as with large ventral horn cells that subserve segmental re exes Some of the ascending bers run uninterruptedly in the dorsal columns of the same side of the spinal.

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When weaning from the ventilator is anticipated, anticholinesterase agents are reintroduced slowly, and treatment with corticosteroids can be instituted if necessary Oral doses of 60 mg pyridostigmine or 15 mg neostigmine are roughly equivalent to 05 to 1 mg neostigmine intravenously and 15 to 2 mg intramuscularly, as noted in Table 53-1 The management of the critically ill patient with myasthenia has been reviewed in the monograph by Ropper and colleagues Most patients with myasthenic crisis will take several weeks to recover, and a few of our patients have remained ventilatordependent for months In the extensive experience from ColumbiaPresbyterian, half of patients could be safely extubated within 2 weeks and three-quarters by a month (Thomas et al) There were 7 deaths among 53 patients, re ecting the gravity of this syndrome even in the modern era of intensive care Atelectasis, severe anemia, congestive heart failure, and clostridial diarrhea (associated with antibiotic use) portend a prolonged period of generalized weakness and intubation From time to time one encounters a patient in whom respiration and ambulation do not improve for many months after a myasthenic crisis In our experience, these have been middle-aged or older patients, usually women, in whom an element of hyperthyroidism or hypothyroidism may have been operative They become wasted as the proximal limb and axial muscles, including the diaphragm, fail to recover their power, even though the ocular and oropharyngeal muscles improve The role of corticosteroids in producing a concomitant proximal myopathy is a frequent question that can be solved by careful EMG examination The only recourse in cases of long-standing and severe myasthenia is to continue an average dose of corticosteroids and anticholinesterase medications with intermittent trials of immune globulin or plasma exchanges This is also a situation in which high-dose cyclophosphamide followed by granulocyte-stimulating factor as mentioned earlier may result in slow improvement.

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normally, and breast-feeding is not thought to be a problem with regard to the transmission of AChR antibodies The issues of neonatal myasthenia and of reduced intrauterine movements are considered later

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OTHER DISORDERS OF NEUROMUSCULAR TRANSMISSION (Table 53-2)

When a patient walks in the door, he or she should be greeted with eye contact and a smile Nothing conveys a sense of being in the wrong place more than being greeted by the head of a receptionist who is speaking with someone else at the expense of the patient Although this interaction has nothing to do with the patient s actual treatment, this too has a large impact on a patient s experience and this aspect of the visit should be as controlled as the part that involves the actual procedure The patient should be escorted to an examination room in a timely manner (not more than 15 minutes is advised for a cosmetic practice, but in an ideal setting the waiting room time should be limited to 5 minutes) Upon entering the examination room, the patient should be comfortably seated Once comfortable, a conversation should be initiated with the medical assistant, nurse, or cosmetic coordinator about the patient s goals During

Considered here are several disorders of neuromuscular transmission characterized clinically by muscular weakness and fatigability but differing in mechanism from autoimmune myasthenia gravis The Lambert-Eaton myasthenic syndrome, neonatal myasthenia, the congenital myasthenic syndromes, and the myasthenic syndromes induced by drugs and toxins are the main disorders in this group Two additional important members botulism and organophosphate poisoning are described elsewhere in the book (pages 1032 and 1037, respectively)

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