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measured by intracellular recordings are initially normal despite the failure of impulse propagation by the sarcolemma One would expect the muscle ber to be hyperpolarized as K moves into it, but it actually becomes depolarized Rudel and associates attribute the latter change to an increased Na conductance The ECG changes also begin at levels of K that are slightly below normal (about 3 meq/L); they consist of prolonged PR, QRS, and QT intervals and attening of T waves Diagnosis at a time when the patient is normal may be facilitated by provocative tests With the patient carefully monitored, including the use of an ECG, the oral administration of 50 to 100 g of glucose or loading with 2 g of NaCl every hour for seven doses, followed by vigorous exercise, brings on an attack, which then can be terminated by 2 to 4 g of oral KCl (the opposite of what pertains in hyperkalemic periodic paralysis) Pathologic Changes The nervous system is entirely normal The muscle bers are uniformly somewhat large, but the most striking change, particularly in the late degenerative phases of the disease, is vacuolization of the sarcoplasm The myo brils are separated by round or oval vacuoles containing clear uid, presumably water, and a few PAS-positive granules There are pathologic changes in myo brils and mitochondria as well and focal increases in muscle glycogen Isolated muscle bers may undergo segmental degeneration Electron microscopic studies have shown that the vacuoles arise as a result of proliferation and degeneration of membranous organelles within the sarcoplasmic reticulum and transverse tubules (A G Engel) Treatment A low-sodium diet (160 meq/day), avoidance of large meals and exposure to cold, and acetazolamide 250 mg three times daily may be helpful in preventing attacks That acetazolamide reduces attacks is somewhat surprising since it is kaluretic, but it may work through the production of acidosis; a few patients have worsened with the drug Patients who are unresponsive to acetazolamide may be treated with the more potent carbonic anhydrase inhibitor, dichlorphenamide, 50 to 150 mg per day or with the potassium-sparing diuretics spironolactone or triamterine (both in doses of 25 to 100 mg/day) but caution must then be exercised with the simultaneous administration of oral potassium supplements The daily administration of 5 to 10 g of KCl orally in an unsweetened aqueous solution prevents attacks in many patients, and apparently this program can be maintained inde nitely If this approach fails, a low-carbohydrate, low-salt, high-K diet combined with a slow-release K preparation may be effective For an acute attack, 025 meq KCl/kg should be given orally or, if this is not tolerated, some other K salt may be tried This dose may be insuf cient, and if there is no improvement in 1 or 2 h, KCl may have to be given intravenously 005 to 01 meq/kg intravenously initially in a bolus at a safe rate, followed by 20 to 40 meq KCl in 5% mannitol, avoiding glucose or NaCl as the carrier solution For the late progressive polymyopathy that follows many severe attacks of periodic paralysis, Dalakas and Engel report successful restoration of strength by the long-term administration of the carbonic anhydrase inhibitor dichlorphenamide Regular exercise (not too strenuous) to keep the patient t is desirable.

Sensory Nerves Fibers that mediate super cial sensation are located in cutaneous sensory or mixed sensorimotor nerves In cutaneous nerves, unmyelinated pain and autonomic bers exceed myelinated bers by a ratio of 3 or 4 : l In contrast, proprioceptive bers are located in deeper, predominantly motor nerves The myelinated bers are of two types, small, lightly myelinated, A- bers for pain and cold, as discussed in Chap 8, and larger, fasterconducting A- bers for touch and pressure The nonmyelinated autonomic bers are efferent (postganglionic) and innervate piloerector muscles, sweat glands, and blood vessels In addition, these nerves contain afferent and efferent spindle and Golgi tendon organ bers and thinner pain afferents The differing conduction velocities of these bers are discussed in Chap 45 A single cutaneous (touch, pain, and temperature) axon con-

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mechanoreceptors that are sensitive to hair movement, skin pressure, or noxious stimuli There are also descending bers in the posterior columns, including bers from cells in the dorsal column nuclei The posterior columns contain a portion of the bers for the sense of touch as well as the bers mediating the senses of touchpressure, vibration, direction of movement and position of joints, and stereoesthesia recognition of surface texture, shape, numbers and gures written on the skin, and two-point discrimination all of which depend on patterns of touch-pressure (see Fig 8-2) The nerve cells of the nuclei gracilis and cuneatus and accessory cuneate nuclei give rise to a secondary afferent path, which crosses the midline in the medulla and ascends as the medial lemniscus to the posterior thalamus However, the ber pathways in the posterior columns are not the sole mediators of proprioception in the spinal cord [see Posterior (Dorsal) Column Syndrome, further on] In addition to these well-de ned posterior column pathways, there are cells in the reticular part of the dorsal column nuclei that receive secondary ascending bers from the dorsal horns of the spinal cord and from ascending bers in the posterolateral columns These dorsal column bers project to brainstem nuclei, cerebellum, and

S1 L5 Figure 9-2 Distribution of the sensory spinal roots on the surface of the body (Reproduced by permission from Sinclair)

cord, terminating in the gracile and cuneate nuclei The central axons of the primary sensory neurons are joined in the posterior columns by other secondary neurons whose cell bodies lie in the posterior horns of the spinal cord (see below) The bers in the posterior columns are displaced medially as new bers from each successively higher root are added, thereby creating somatotopic laminations (see Fig 8-3) Of the long ascending posterior column bers, which are activated by mechanical stimuli of skin and subcutaneous tissues and by movement of joints, only about 25 percent (from the lumbar region) reach the gracile nuclei at the upper cervical cord The rest off collaterals to or terminate in the dorsal horns of the spinal cord, at least in the cat (Davidoff) An estimated 20 percent of ascending bers originate from cells in Rexed layers IV and V of the posterior horns (see Fig 8-1) and convey impulses from low-threshold

4 n

Figure 9-3 Dermatomes of the upper and lower extremities, outlined by the pattern of sensory loss following lesions of single nerve roots (Reproduced by permission from Keegan and Garrett)

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