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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology Vol 22 Amsterdam, North-Holland, 1975, chap 22, pp 481 497 KROENKE K, MANGELSDORFF AD: Common symptoms in ambulatory care: Incidence, evaluation, therapy, and outcome Am J Med 86:262, 1989 LEIGH RJ, ZEE DS: The Neurology of Eye Movements, 2nd ed Philadelphia, Davis, 1991 LOCKWOOD AH, SALVI RJ, BURKARD RF: Tinnitus N Engl J Med 347: 904, 2002 MARION MS, CEVETTE MJ: Tinnitus Mayo Clin Proc 66:614, 1991 MATTOX DE, SIMMONS FB: Natural history of sudden sensorineural hearing loss Ann Otol 86:463, 1977 M LLER AR: Pathophysiology of tinnitus Ann Otol Rhinol Laryngol 93: 39, 1984 MORELL RJ, KIM HJ, HOOD LJ, et al: Mutations in the connexin 26 gene (GJB2) among Ashkenazi Jews with nonsyndromic recessive deafness N Engl J Med 339:1500, 1998 NADOL JB JR: Hearing loss N Engl J Med 329:1092, 1993 National Institute on Deafness and Other Communication Disorders: A Report of the Task Force on the National Strategic Research Plan Bethesda, MD, National Institutes of Health, 1989 NEUHAUSER H, LEOPOLD M, VAN BREVERN M, et al: The interpretation of migraine, vertigo and migrainous vertigo Neurology 56:436, 2001 NODAR RH, GRAHAM JT: An investigation of frequency characteristics of tinnitus associated with Meniere s disease Arch Otolaryngol 82:28, ` 1965 PAGE J: Audiologic tests in the differential diagnosis of vertigo Otolaryngol Clin North Am 6:53, 1973 PROCTOR CA, PROCTOR B: Understanding hereditary nerve deafness Arch Otolaryngol 85:23, 1967 RASMUSSEN GI: An efferent cochlear bundle Anat Rec 82:441, 1942
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RUDGE P: Clinical Neuro-otology Edinburgh, Churchill-Livingstone, 1983 SCHUKNECHT HF: Cupulolithiasis Arch Otolaryngol Head Neck Surg 90: 765, 1969 SCHUKNECHT HF, KITAMURA K: Vestibular neuronitis Ann Otol Rhinol Laryngol 90:1, 1981 SEMONT A, FREYSS G, VITTE E: Curing the BPPV with a liberatory maneuver Adv Otorhinolaryngol 42:290, 1988 SISMANIS A, SMOKER WR: Pulsatile tinnitus: Recent advances in diagnosis Laryngoscope 104:681, 1994 STOUFFER JL, TYLER RS: Characterization of tinnitus by tinnitus patients J Speech Hearing Disord 55:439, 1990 STRUPP M, ZINGLER VC, ANBUSOW V, et al: Methylprednisolone, valacyclovir, or the combination for vestibular neuritis N Engl J Med 351: 354, 2004 SUGA S, LINDSAY JR: Histopathological observations of presbyacusis Ann Otol Rhinol Laryngol 85:169, 1976 TANAKA Y, KAMO T, YOSHIDA M, YAMADORI A: So-called cortical deafness Brain 114:2385, 1991 TEKIN M, ARNOS KS, PANDY A: Advances in hereditary deafness Lancet 358:1082, 2001 TOOLE JF, TUCKER H: In uence of head position upon cerebral circulation Arch Neurol 2:616, 1960 TUMARKIN A: The otolithic catastrophe: A new syndrome BMJ 1:175, 1936 VOLLERTSEN RS, MCDONALD TJ, YOUNGE BR, et al: Cogan s syndrome: 18 cases and a review of the literature Mayo Clin Proc 61:344, 1986 VON BREVERN M, SEELIG T, NEUHAUSER H, et al: Benign positional vertigo predominantly affects the night labyrinth J Neurol Neurosurg Psychiatr 75:1487, 2004
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SECTION 4
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Copyright 2005, 2001, 1997, 1993, 1989, 1985, 1981, 1977, by The McGraw-Hill Companies, Inc Click here for terms of use
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In contemporary society, the frequency and importance of epilepsy can hardly be overstated From the epidemiologic studies of Hauser and colleagues, one may extrapolate an incidence of approximately 2 million individuals in the United States who are subject to epilepsy (ie, chronically recurrent cerebral cortical seizures) and predict about 44 new cases per 100,000 population occur each year These gures are exclusive of patients in whom convulsions complicate febrile and other intercurrent illnesses or injuries It has also been estimated that slightly less than 1 percent of persons in the United States will have epilepsy by the age of 20 years (Hauser and Annegers) Over two-thirds of all epileptic seizures begin in childhood (most in the rst year of life), and this is the age period when seizures assume the widest array of forms The incidence increases again slightly after age 60 In the practice of pediatric neurology, epilepsy is one of the most common disorders The chronicity of childhood forms and their persistence in patients of all ages adds to their importance For all these reasons, every physician should know something of the nature of seizure disorders and their treatment It is, however, notable that in striking contrast to the many treatments available for epilepsy, as pointed out by J Engle, 80 to 90 percent of epileptics in the developing world never receive treatment Epilepsy was in the past de ned as an intermittent derangement of the nervous system due to an excessive and disorderly discharge of cerebral nervous tissue on muscles This was the postulate, in 1870, of Hughlings Jackson, the eminent British neurologist, and modern electrophysiology offers no evidence to the contrary The discharge may result in an almost instantaneous loss of consciousness, alteration of perception or impairment of psychic function, convulsive movements, disturbance of sensation, or some combination thereof A terminologic dif culty arises from the diversity of the clinical manifestations The term convulsion, referring as it does to an intense paroxysm of involuntary repetitive muscular contractions, is inappropriate for a disorder that may consist only of an alteration of sensation or consciousness Seizure is preferable as a generic term, since it embraces a diversity of paroxysmal events and also because it lends itself to quali cation The term motor or convulsive seizure is therefore not tautologic, and one may likewise speak of a sensory seizure or psychic seizure The word epilepsy is derived from Greek words meaning to seize upon or a taking hold of Our predecessors referred to it as the falling sickness or the falling evil Although a useful medical term to denote recurrent seizures, the words epilepsy and epileptic still have unpleasant connotations to the laity and should be used advisedly in dealing with patients Viewed in its many clinical contexts, the rst solitary seizure or brief outburst of seizures may occur during the course of many medical illnesses It indicates that the cerebral cortex has been affected by disease, either primarily or secondarily Convulsive seizures by their nature, if repeated every few minutes, as in status epilepticus, may threaten life Equally important, a seizure or a series of seizures may be the manifestation of an ongoing neuro271
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logic disease that demands the employment of special diagnostic and therapeutic measures, as in the case of a brain tumor A more common and less grave circumstance is for a seizure to be but one in an extensive series recurring over a long period of time, with most of the attacks being more or less similar in type In this instance they may be the result of a burned-out lesion that originated in the past and remains as a scar The original disease may have passed unnoticed, or perhaps had occurred in utero, at birth, or in infancy, in parts of the brain inaccessible for examination or too immature to manifest signs It may have affected a very small or silent area in a mature brain The increasingly re ned techniques of magnetic resonance imaging (MRI) are now beginning to reveal small zones of cortical dysplasia and hippocampal sclerosis, both of which tend to be epileptogenic Patients with such long-standing lesions probably make up the majority of those with recurrent seizures but are necessarily classi ed as having idiopathic or cryptogenic epilepsy, because it is often impossible to ascertain the nature of the original disease and the seizures may be the only sign of brain abnormality There are other types of epilepsy for which no pathologic basis has been established and for which there is no apparent underlying cause except perhaps a genetic one These epilepsies have been referred to as primary Included in this category are hereditary forms, such as certain generalized tonic-clonic (grand mal) and absence seizure states Some authors (Lennox and Lennox; Forster) have reserved the term idiopathic for recurrent seizures of these types
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