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tercostal muscle from normal goats and goats with hereditary myotonia J Gen Physiol 50:89, 1966 MCFADZEAN AJS, YEUNG R: Periodic paralysis complicating thyrotoxicosis in Chinese BMJ 1:451, 1967 MEYERS KR, GILDEN DH, RINALDI CF, HANSEN JL: Periodic muscle weakness, normokalemia, and tubular aggregates Neurology 22:269, 1972 NISSEN K: Beitrage zur Kenntnis der Thomsen schen Krankheit (Myotonia congenita), mit besonderer Berucksichtigung des hereditaren Momentes und seinen Beziehungen zu den Mendelschen Vererbungsregeln Z Klin Med 97:58, 1923 OKINAKA S, SHIZUME K, IINOS S, et al: The association of periodic paralysis and hyperthyroidism in Japan J Clin Endocrinol Metab 17:1454, 1957 PLASTER NM, TAWIL R, TRISTANI-FIROUZI M, et al: Mutations in Kir21 cause the developmental and episodic electrical phenotypes of Andersen s syndrome Cell 105:511, 2001 POSKANZER DC, KERR DNS: A third type of periodic paralysis with normokalemia and favorable response to NaC1 Am J Med 31:328, 1961 PTACEK LJ, FU YH: Channelopathies: Episodic disorders of the nervous system Epilepsia 42(suppl 5):35, 2001 PTACEK LJ, TAWIL R, GRIGGS RC, et al: Dihydropyridine receptor mutations cause hypokalemic periodic paralysis Cell 77:963, 1994 PTACEK LJ, TAWIL R, GRIGGS RC, et al: Sodium channel mutations in acetazolamide-responsive myotonia congenita, paramyotonia congenita, and hyperkalemic periodic paralysis Neurology 44:1500, 1994 QUANE KA, HEALY JMS, KEATING KE, et al: Mutations in the ryanodine receptor gene in central core disease and malignant hyperthermia Nature Genet 5:51, 1993 RICKER K, MOXLEY RT, HEINE R, LEHMANN-HORN F: Myotonia uctuans: A third type of muscle sodium channel disease Arch Neurol 51: 1095, 1994 ROSENFELD J, SLOAN-BROWN K, GEORGE AL: A novel muscle sodium channel mutation causes painful congenital myotonia Ann Neurol 42: 811, 1997 RUDEL R, LEHMANN-HORN F, RICKER K, et al: Hypokalemic periodic paralysis: In vitro investigation of muscle ber membrane parameters Muscle Nerve 7:110, 1984 SANSONE V, GRIGGS RC, MEOLA G, et al: Andersen s syndrome: A distinct periodic paralysis Ann Neurol 42:305, 1997 SCHROEDER JM, ADAMS RD: The ultrastructural morphology of the muscle ber in myotonic dystrophy Acta Neuropathol 10:218, 1968 SPAANS F, THEUNISSEN P, REEKERS AD, et al: Schwartz-Jampel syndrome: 1 Clinical, electromyographic, and histologic studies Muscle Nerve 13:516, 1990 STEVENS JR: Familial periodic paralysis, myotonia, progressive amyotrophy, and pes cavus in members of a single family Arch Neurol Psychiatry 72:726, 1954 STREIB EW: Paramyotonia congenita: Successful treatment with tocainide: Clinical and electrophysiologic ndings in seven patients Muscle Nerve 10:155, 1987 STREIB EW: Successful treatment with tocainide of recessive generalized congenital myotonia Ann Neurol 19:501, 1986 SUBRAMONY SH, WEE AS, MISHRA SK: Lack of cold sensitivity in hyperkalemic periodic paralysis Muscle Nerve 9:700, 1986 SUN SF, STREIB EW: Autosomal recessive generalized myotonia Muscle Nerve 6:143, 1983 TALBOTT JH: Periodic paralysis: A clinical syndrome Medicine 20:85, 1941 TAWIL R, MCDERMOTT MP, BROWN RH Jr, et al: Randomized trials of dichlorphenamide in the periodic paralyses Working Group on Periodic Paralysis Ann Neurol 47(1):46, 2000 THIEBEN MJ, LENNON VA, ASKANIT AJ, et al: Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody Neurology 62:1177, 2004.

THOMASEN E: Myotonia, Thomsen s Disease, Paramyotonia, Dystrophia Myotonica Aarhus, Denmark, Universitetsforlaget i Aarhus, 1948 THOMSEN J: Tonische Krampfe in willkurlich beweglichen Muskeln in Folge von ererbter psychischer disposition (Ataxia muscularis ) Arch Psychiatr Nervenkr 6:706, 1876 TRUDELL RG, KAISER KK, GRIGGS RC: Acetazolamide-responsive myotonia congenita Neurology 37:488, 1987

TYLER FH, STEPHENS FE, GUNN FD, PERKOFF GT: Studies on disorders of muscle: VII Clinical manifestations and inheritance of a type of periodic paralysis without hypopotassemia J Clin Invest 30:492, 1951 YAZAKI K, KURIBAYASHI T, YAMAMURA Y, et al: Hypokalemic myopathy associated with a 17 -hydroxylase de ciency: A case report Neurology 32:94, 1982

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Quite apart from spasticity and rigidity, which are due to a disinhibition of spinal motor mechanisms, there are forms of muscular stiffness and spasm that can be traced to abnormalities of the lower motor neuron and its spinal inhibitory mechanisms or to the sarcolemma of the muscle ber and its intrinsic conducting apparatus Thus, muscles may go into spasm because of an unstable depolarization of motor axons, which sends volleys of impulses across neuromuscular junctions as occurs in myokymia, hypocalcemic tetany, and pseudohypoparathyroidism In other states, discussed in the preceding chapter, the innervation of muscle may be normal but contraction persists despite attempts at relaxation (myotonia) Or, after one or a series of contractions, the muscle may be slow in decontracting, as occurs in paradoxical myotonia and hypothyroidism; in the contracture of McArdle phosphorylase de ciency and phosphofructokinase de ciency, muscle, once contracted, may lack the energy to relax Each of these conditions evokes the complaint of cramp or spasm, which is variably painful and interferes with free and effective voluntary activity Each condition has its own identifying clinical and electromyographic (EMG) characteristics, and most of them respond favorably to therapy A premium is therefore attached to the clinical differentiation of these phenomena

The function of all storage devices is to hold, or store, information, even when the computer s power is turned off Unlike information in RAM, files that are kept on a storage device remain there unless they are manually removed or altered by the user or the computer s operating system A great variety of storage devices are available, including floppy drives, hard drives, and Zip drives However, the A+ tests focus only on the standard storage devices explained in the following sections

The notification events occurring in the Ops Center will be forwarded by the Ops Center Management Console to the Oracle Enterprise Manager through the web services interface Once the notifications are propagated to Oracle Enterprise Manager, they are treated as native Oracle Enterprise Manager alerts, and will utilize the built-in notifications framework and the policies that are set up in Enterprise Manager for notifying administrators of critical events occurring in the system The key features of the Ops Center Connector are

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