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This obscure and oddly named disease is included in this chapter because an abnormality of voltage-gated potassium channels has been discovered in some patients It is characterized by continuous muscle ber activity referred to as neuromyotonia, hyperhydrosis, severe weight loss, insomnia, and hallucinations Most cases so far described have ended fatally in a matter of months, but plasma exchange has reversed the syndrome in the case described by Ligouri and colleagues Some cases have an associated thymoma The condition is also included in the discussion of the other forms of continuous muscle ber activity in the following chapter In addition to the various seizure disorders that are attributable to disorders of ion channels, also mentioned here for completeness are the Lambert-Eaton myasthenic syndrome that results from an autoimmune attack in calcium channels (it is discussed in the previous chapter), a rare paraneoplastic encephalitis that has been attributed to antibodies against potassium channels (Thieben et al), and spinocerebellar ataxia type 6, which is due to an inherited abnormality of the -1A voltage-dependent calcium channel (see Chap 5)

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This state, in which hyperthermia occurs as an idiosyncratic reaction to neuroleptic drugs, is also accompanied by widespread myonecrosis It shares some features with malignant hyperthermia but is a distinct entity as discussed in Chap 43

The discovery of several types of seizure disorder based on inherited defects of the potassium channel has elicited considerable

ANDERSEN ED, KRASILNIKOFF PA, OVERVAD H: Intermittent muscular weakness, extrasystoles, and multiple developmental anomalies Acta Paediatr Scand 60:559, 1971 ATSUMI T, ISHIKAWA S, MIYATAKE T, YOSHIDA M: Myopathy and primary aldosteronism: Electron microscopic study Neurology 29:1348, 1979 BECKER PE: Genetic approaches to the nosology of muscle disease: Myotonias and similar diseases: part 7 Muscle, in Bergsma D (ed): The Clinical Delineation of Birth Defects Baltimore, Williams & Wilkins, 1971 BRYANT SH: Cable properties of external intercostal muscle bers from myotonic and nonmyotonic goats J Physiol 204:539, 1969

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Which type of memory is responsible for Maintaining a device s basic operating instructions Anticipating processor requests and making the proper data available Providing temporary storage for application files Solution ROM Cache RAM

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CANNON SC: An expanding view for the molecular basis of familial periodic paralysis Neuromusc Disord 12:533, 2002 CANNON SC, BROWN RH JR, COREY DP: Theoretical reconstruction of myotonia and paralysis caused by incomplete inactivation of sodium channels Biophys J 65:270, 1993 CONN JW, KNOPF RF, NESBIT RM: Clinical characteristics of primary aldosteronism from an analysis of 145 cases Am J Surg 107:159, 1964 CONN JW, ROVNER DR, COHEN EL: Licorice-induced pseudoaldosteronsim: Hypertension, hypokalemia, aldosteronopenia and suppressed plasma renin activity JAMA 205:492, 1968 DALAKAS MC, ENGEL WK: Treatment of permanent muscle weakness in familial hypokalemic periodic paralysis Muscle Nerve 6:182, 1983 DEJONG JGY: Myotonia levior, in Kuhn E (ed): Progressive Muskeldystrophie-Myotonie-Myasthenie Heidelberg, Springer, 1966, pp 255 259 DENBOROUGH M: Malignant hyperthermia Lancet 352:1131,1998 DENBOROUGH MA, LOVELL RRH: Anaesthetic deaths in a family Lancet 2:45, 1960 DENNY-BROWN D, FOLEY JM: Evidence of a chemical mediator in myotonia Trans Assoc Am Physicians 62:187, 1949 DENNY-BROWN D, NEVIN S: The phenomenon of myotonia Brain 64:1, 1941 ENGEL AG: Evolution and content of vacuoles in primary hypokalemic periodic paralysis Mayo Clin Proc 45:774, 1970 FONTAINE B, KHURANA TS, HOFFMAN EP, et al: Evidence that the genetic defect in hyperkalemic periodic paralysis is in the adult muscle sodium channel -subunit gene Science 250, 1000, 1990 FONTAINE B, VALE SANTOS JM, JURKAT-ROTT JK, et al: Mapping of hypokalemic periodic paralysis (hypo PP) to chromosome 1q31-q32 by a genome-wide search in three European families Nature Genet 6:267, 1994 FRANK JP, HARATI Y, BUTLER JJ, et al: Central core disease and malignant hyperthermia syndrome Ann Neurol 7:11, 1980 GAMSTORP I: Adynamia episodica hereditaria Acta Paediatr Scand (suppl 108) 45:1 126, 1956 HAASS A, RICKER K, RUDEL R, et al: Clinical study of paramyotonia congenita with and without myotonia in a warm environment Muscle Nerve 4:388, 1981 HANNA MG, WOOD NW, KULLMANN DM: Ion channels and neurological disease: DNA based diagnosis is now possible, and ion channels may be important in common paroxysmal disorders J Neurol Neurosurg Psychiatry 65:427, 1998 HARRIMAN DGF, SUMMER DW, ELLIS FR: Malignant hyperthermia myopathy Q J Med 42:639, 1973 ISAACS H, BARLOW MB: Malignant hyperpyrexia J Neurol Neurosurg Psychiatry 36:228, 1973 KOCH MC, STEINMEYER K, LORENZ C, et al: The skeletal muscle chloride channel in dominant and recessive human myotonia Nature 354:304, 1991 LAYZER RB: Neuromuscular Manifestations of Systemic Disease Philadelphia, Davis, 1985 LEHMANN-HORN F, RUDEL R, JURKAT-ROTT K: Nondystrophic myotonias and periodic paralyses, in Engel AG, Franzini-Armstrong C (eds): Myology, 3rd ed New York, McGraw-Hill, 2004, pp 1257 1300 LEHMANN-HORN F, MAILANDER V, HEINE R, GEORGE AL: Myotonia levior is a chloride channel disorder Hum Mol Genet 4:1397, 1995 LEHMANN-HORN F, RUDEL R, RICKER K: Membrane defects in paramyotonia congenita (Eulenberg) Muscle Nerve 10:633, 1987 LEWI Z, BAR-KHAYIM Y: Food poisoning from barium carbonate Lancet 2:342, 1964 LIGOURI R, VINCENT A, CLOVER L, et al: Morvan s syndrome: Peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels Brain 124:2417, 2001 LIPICKY RJ, BRYANT SH: Ion content, potassium ux, and cable properties of myotonic, human, external intercostal muscle Trans Am Neurol Assoc 96:34, 1971 LIPICKY RJ, BRYANT SH: Sodium, potassium, and chloride uxes in in-.

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ROM is nonvolatile, so it does not lose its contents when the computer s power is turned off This makes ROM ideal for storing a device s most basic operation and communication instructions A number of computer components include ROM chips, which contain device-specific basic instructions Information on these ROM chips is said to be hardwired or hard coded because it cannot be changed These types of devices are termed firmware, to indicate that they are a mixture of hardware and software

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