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mise à jour du
28 novembre 2002
J Pediatr
1990;116; 5; 720-5
 lexique
Primary disorder of vigilance: a novel explanation of inattentiveness, daydreaming, boredom, restlessness, and sleepiness
Weinberg WA, Brumback RA
Department of neurology and pediatrics. University of Texas. Dallas

Chat-logomini

Children and adolescents who are not doing well in school and who are manifesting symptoms of inattentiveness, distractibility, hyperactivity, disorganization, and poor continuous task performance frequently receive the diagnosis of attention deficit-hyperactivity disorder. We present diagnostic criteria and representative cases illustrating a condition that we have designated as primary disorder of vigilance. This disorder can be included in the spectrum of specific conditions having characteristic symptoms that overlap the criteria for attention deficit-hyperactivity disorder.

DEFINITIONS

Vigil ance is steady-state alertness, wakefulness, and arousal-the state of being watchfül, awake, and alert.1 We have noted families in which the major symptoms relate to a basic disturbance in vigilance.2,' Such problems as poor sustained attention, trouble concentrating, and difficulty staying awake (drowsiness or sleepiness) can be the result of disturbed vigilance. Diagnostic criteria for the primary disorder of vigilance, established on the basis of a typical cluster of symptoms seen in affected children and their family members, are presented in Table I. We believe that this condition is a major cause of school failure, results in conflicts between child and parent, and is associated with difficulties in work adjustment and enjoyment of life. If not recognized and properly treated, the primary disorder of vigilance can be severely incapacitating, and the associated accident proneness can even become fatal. Table II prescrits a summary of six representative patients who fulfill the diagnostic criteria listed in Table I, and shows the coincidence of this condition with other common childhood disorders, including depression and epilepsy. Patient 1 is reported in detail.

CASE REPORT

Patient 1, a boy born after a prolonged labor with a birth weight of 3600 g, had a normal early infancy except for mild colic and was described as a "good infant." He sat at 8 months, walked at 17 months, and rode a tricycle at 5 years and a bicycle at 7 years. He began talking at about 3 years of age and was toilet trained at 4 years of age. Despite these delayed developmental milestones, he did well in nursery school and kindergarten. At the age of 7 years 5 months, the Wechsler Intelligence Scale for Children, Revised Edition (WISC-R), showed the following scores: Verbal IQ 128 (subtest scores: Information 13, Comprehension 15, Arithmetic 14, Similarities 12, Vocabulary 15, Digit Span 8), Performance IQ 105 (subtest scores: Picture Completion 11, Picture Arrangement 12, Block Design 12, Object Assembly 13, Coding 6), and Full Scale IQ 117. However, elementary school teachers described him as slow moving, a dawdler, a procrastinator, not competitive, immature, a "daydreamer" with poor attention, easily distracted, lazy, and wanting to avoid work. He was also described as kind, affectionate, compassinate, sensitive to the feelings of others, and a "very good child" who was never a behavior problem. He enjoyed asking questions and telling long stories with great detail.

He was first evaluated at the age of 8 years 9 months, at which time he described himself as restless, needing to move his legs to keep himself from falling asleep. He yawned a lot in school, found school boring, voluntarily avoided doing things in school because such tasks produced boredom and sleepiness, and daydreamed to keep from being bored. He said reading was boring and put him to sleep, and he complained that his thoughts would wander, making it difficult to keep to a task. The mother reported that he would sleep 12 hours at night and still take a 2-hour nap during the day. He was very sleepy in the morning on arising, and his mother had to coax him to get up (otherwise he would sleep beyond 10 AM) and cajole him to dress (often he took 30 minutes to put on his shoes). He was accident prone, having sustained fractures of humerus and the clavicle. He experienced no hypnagogic hallucinations, sleep paralysis, or cataplexy.

The family history was remarkable for the mother's sleeping 12 to 14 hours at night, along with having problems of poor concentration and difficulty staying awake when not busy. She complained of being bored and sleepy when riding in a car, reading a book, or sitting in a lecture hall or church, and indicated that she most enjoyed busy activities. A maternal aunt, maternal first cousin, maternal grandmother, and maternal great-grandmother had similar symptoms.

The patient was treated with methylphenidate (20 mg sustained-release tablet each morning) at the age of 8 years 9 months, with immediate improvement; he described himself as alert, his grades in school improved markedly, and he slept for only 10 hours a night, with no daytime napping. During a 4-month period he read 59 books (and received a trophy from the school for this achievement). He was able to sustain attention to tasks both in school and at play; for example, he built a model of the Hoover Dam out of 25,000 toothpicks, working incessantly on this project for several days. It was noted by the mother and teachers (as well as by the child himself) that as the methylphenidate effect wore off, he would become sleepy and restless, would daydream, would have difficulty concentrating, and would become sleepy after lunch, while riding in a car, and when still (sitting at a table, watching television, or listening to a discussion). His methylphenidate dosage was adjusted during the following 21 months to 40 mg at 7 AM, 40 mg at noon, and 20 mg at 4 Pm. This dosage provided an optimal subjective feeling of alertness, observed wakefulness, and improvement in his school performance and play (he became a competitive Little League football player). Multiple trials of periods without the use of methylphenidate resulted in his becoming sleepy, irritable, easily frustrated, and restless, with deterioration of his school performance and of his interest in play. He reported that he could not concentrate and stated that the methylphenidaie was necessary keep my brain awake!"

At the age of 12 years 3 months, the patient discontinued the methylphenidate regimen for the 2 weeks before formal sleep studies. Without medication, he was sleepy during the day, very restless ("hyperactive"), unable to sustain attention, and irritable, and he had difficulty falling asleep at night. On the Stanford Sleepiness Scale, he endorsed items indicating severe sleepiness and described himself as fighting off sleep, being woozy in the morning, and being only somewhat alert by late afternoon. Polysomnography showed an entirely normal sleep pattern. On the multiple sleep latency tests, with five opportunities every 2 hours to fall alseep, he slept during the first test period at 9 AM (not associated with rapideye-movement sleep) but not during the other opportunities. During the Multiple Vigilance Test, he looked away from the screen much of the time, made a large number of errors, and was impulsive in his responses. After hospital discharge, methylphenidate therapy was restarted, with continued good response and no apparent side effects or loss of effectiveness. In addition, his mother noted a marked improvement in her alertness and ability to concentrate after she began taking methylpheniate, 40 mg at 7 Am and 20 mg at noon.

DISCUSSION

Persons with primary disorder of vigilance are unable to maintain alertness and wakefulness, and this inability results in symptoms indicating a short attention spart, poor concentration, and problems in appearing and being awake. The diagnostic criteria for this disorder provide the minimal symptoms found in affected persons. The most obvious evidence that the patient is having difficulty staymg awake and alert is the frequent fidgeting, yawning, and stretching when sitting still. In various tasks requiring continuous mental performance, the person complains of having trouble concentrating and being bored by the monotony, loses interest, and often does not complete the task. A child with the disorder may resist homework because of difficulty in staying alert during the required continuous mental performance. Thus homework can become a major source of confrontation between child and parent. Affected children may be quick to start new activities but will rapidly tire of thent and quit. They appear disorganized, and imposing excessive structure, sameness, organization, or repetitive activity on them results in poor performance. When participating in highly repetitive activities or activities requiring continuous mental performance or continuous task performance, these children are restless, fidgety, or talkative, or they move and walk about in an apparent attempt to maintain vigilance. If prevented from being active (for example, in church, in school classes, at the dinner table, or strapped in a seat belt while riding in a car), they will stare off, daydream, show minor restlessness, yawn and stretch, and finally may fall asleep.

The primary disorder of vigilance appears to be a lifelong condition that results in varying behaviors at different ages. Difficulties with attention begin to be evident in structured preschool programs. School is characterized by teacher reports of inattentiveness, daydreaming, not completing assigriments, and disruptive behavior characterized by talking, fidgeting, squirming, and moving about. On the playground and after school, these children are busy and active in play. There is increased resistance toward homework as the volume of routine, repetitious material increases. When trying (or forced) to sit at a desk and do homework, the affected child or adolescent will stare off into space, daydream, gel up and move about, and spend an inordinate amount of time attempting to complete a task. Affected persons eventually begin to drop out of structured or repetitive activities and pursue activities and work that are characterized by variety (specifically, activities with random variation in routine, i.e., "orderly randomization"). Most resist reading, attending lectures, watching filmstrips, and writing essays, because these activities promote tedium ("boring," "monotonous," "uninteresting"). When asked directly, they will also indicate that they have trouble with concentration and attention, daydreaming, and feeling sleepy. Affected adults may deny sleepiness but readily admit to boredom in tasks that are repetitious or require continuous mental performance, and they often diligently avoid such activities. The affected adult commonly falls asleep ("catnaps") when still (such as while attending church, watching television or a bail garne, riding in a car, or reading), but the naps are not refreshing. Affected persons are often teased by family members about these naps. Elderly persons may be described as "sleeping the remaining years away."

An intriguing and important characteristic of persons with the primary disorder of vigilance is a kind, caring, compassionate, and affectionate temperament. These children and adolescents never have evidence of conduct disorder and rarely have discipline problems. Affected infants enjoy being field, cuddled, nursed, and loved. Preschool and elementary school children are usually well liked by their teachers and peers. Although middle school and junior high school children may be exasperating to parents and teachers, they remain affectionate, caring, kind, and generous in their compassion for the unfortunate. This "angelic" nature persists into adulthood.

The mechanism of vigilance is unknown and thus the genesis of disturbances of vigilance is speculative. Studies in physiologic psychology have defined vigilance as sustained attention. Available tests used to assess vigilance actually measure sustained attention. No currently available procedures measure pure vigilance, which is the positive ability to stay wakeful, alert, or awake on tasks requiring continuous mental performance. Vigilance is considered to be a major component of the network of sustained or directed attention (or the "attentional matrix"), which also includes affect, memory, motivation, and perception. Vigilance is possibly a right cerebral hemisphere (posteroinferior parietal lobe) function. When the attentional matrix is discussed, words such as "concentration," "perseverance," "alertness," and "vigilance" are used synonymously to describe positive aspects of attention, whereas "comatose," "stuporous,"," sleepy," and "drowsy" are used as a hierarchical schema to denote decreased (negative) levels of attention. We suggest that with the loss of vigilance, there is diminished ability to maintain an alert, awake state. If not allowed to move, fidget, daydream, or be free of tasks requiring continuous mental performance (or repetitiveness), the cerebral alerting necessary to prevent sleep is readily overcome and brainstem-induced sleep ensues.

Children, adolescents, and their parents who manifest the primary disorder of vigilance are free of the rapid eye movement-associated symptoms of hypnagogic hallucinations, sleep paralysis, and cataplexy, and they have none of the narcolepsy findings obtained from polysomnography, multiple sleep latency tests, and tests of the DR2 locus of the human leukocyte antigen. We have seen several families that have both narcolepsy and the primary disorder of vigilance in different individuals, and one young person with both problems. In an evaluation of vigilance, secondary causes of lowered vigilance must also be excluded. Depression metabolic disorders (such as hypothyroidism and hypoglycemia), seizure disorders, and the use of illicit drugs and various medications can all reduce vigilance. In particular, commonly used anticonvulsant agents, antihistamines, and both minor and major tranquilizers often lower vigilance to the point of causing clinical concern. In a recently studied group of 100 consecutive children and adolescents (aged 3 to 17 years) referred to the Children's Medical Center of Dallas Pediatric Behavioral Neurology Program, 47 had evidence of lowered vigilance. Of these, 23 fulfilled the criteria for primary disorder of vigilance; the other 24 had sorne other cause for disturbed vigilance.

The primary disorder of vigilance appears to be a dominantly inherited condition that is lifelong and that worsens with age. Although it is the children and adolescents who may be brought to clinical attention, their affected parents, grandparents, and other relatives usually go unrecogized. An adult having difficulty with continuous mental performance and continuous task performance should also be evaluated for this treatable condition. The excessive somnolence may not be bothersome in and of itself, but the consequences of this disorder can be devastating, including failure in school and in the workplace. Accidents are more likely because of delayed reaction time and falling asleep in dangerous situations (such as driving a car). If the primary disorder of vigilance is recognized and appropriately mariaged, the affected person may be spared much discomfort.

Treatment of this disorder with a stimulant medication, either methylphenidate or pemoline, is often successful when the person is free of depression (Figure). If depression is present, a tricyclic antidepressant agent is necessary (preferably without methylphenidate or pemoline) until depression has lifted. The less sedative antidepressant agents seem most effective (imipramine, desipramine, and protriptyline, in that order). Management must also include the offering of instruction and practice in the context of orderly randomization rather than structured repetition, reiteration, and excessive drill, which lower vigilance (creating boredom, monotony, and the feeling of sleepiness). Short breaks should be built into the scheduling of tasks, the number of items per task should be limited, and scheduled events should change frequently (preferably in randomized fashion).

REFERENCES

  1. American Psychiatric Association. Diagnostic and statistical rnanual of mental disorders. 3rd edition-revised [DSM-IIIR]. Washington, D.C.: American Psychiatric Association, 1987.
  2. Weinberg W, Emslie G. Attention deficit disorder: a form of childhood depression or other disorders of brain. Int Pediatr 1987;2:135-45.
  3. Weinberg RA, Rutman J, Sullivan L, et al. Depression in children referred to an educational diagnostic center: diagnosis and treatment. J PEDIATR 1973;83:1065-72.
  4. Brumback RA, Weinberg WA. Relationship of hyperactivity and depression in children. Percept Mot Skill 1977;45:247-5 1.
  5. Weinberg WA, Brumback RA. Mania in childhood: case studies and literature review. Am J Dis Child 1976; 130:380-5.
  6. Dorland's illustrated medical dictionary. 25th ed. Philadelphia: WB Saunders, 1974:1715.
  7. Weinberg WA, McLean A. A diagnostic approach to developmental specific learning disorders. J Child Neurol 1986;1: 158-72.
  8. Davies DR, Parasuraman R. The psychology of vigilance. NewYork: Academic Press, 1981.
  9. Heilman KM, Watson RT, Valenstein E. Neglect and related disorders. In: Heilman KM, Valenstein E, eds. Clinical neuropsychology. 2nd ed. New York: Oxford University Press, 1985:243-93.
  10. Mesulam M-M. A cortical network for directed attention and unilateral neglect. Ann Neurol 1981;10:309-25.
  11. Mesulam M-M. Attention, confusional states, and neglect. In: Mesulam MM, ed. Principles of behavioral neurology. Philadelphia: FA Davis, 1985:125-68.
  12. Weinberg WA. Epilepsy and interictal behavior disorders in children and adolescents. Int Pediatr 1987;2:196-204.
 Bâillements et troubles de l'attention: un cas clinique
 
Brumback RA. Weinberg's syndrome: a disorder of attention and behavior problems needing further research. Child Neurol. 2000;15(7)478-480.
 
Koch P. Variation of behavioral and physiological variables in children attending kindergarten and primary school. Chronobiol Int. 1987;4(4):525-535.
 
Weinberg WA, Brumback RA Primary disorder of vigilance: a novel explanation of inattentiveness, daydreaming, boredom, restlessness, and sleepiness. J Pediatr. 1990;116(5):720-725. Comment in: J Pediatr. 1991;118(3):489-490.

Table 1. Criteria for primary disorder of vigilance

  • A.Primary disorder of vigilance is a disorder of at least 1 year's duration, with symptoms front all five major symptom categories:
    • 1. Decreasing ability to sustain alertness, wakefulness, arousal, and watchfulness during continuous mental (or other task) performance
      • a.Complaints of tiredness, drowsiness, sleepiness, lethargy
      • b.Yawning, stretching, sleepy-eyed ("glassy-eyed") appearance
      • c.Falling asleep; excessive napping
    • 2. Decreasing attention to present activities
      • a.Daydreaming
      • b.Difficulty focusing attention; loses place in activities and conversation
      • c.Poor performance
      • d.Slow, delayed, or incomplete tasks
      • e.Disorganized
    • 3. Avoidance of structured or repetitive activities
      • a.Loss or interest in, or complaint that, structured activities are dull, boring, monotonous, tedious, uninteresting (or no longer interesting)
      • b.Preference for shifting activities that have random or irregular changes in schedule or activity (orderly randomization)
    • 4. Motor restlessness and behaviors tu improve alertness
      • a.Fidgeting
      • b.Talkativeness
      • c.Moving about
      • d.Busyness
    • 5. Caring, compassionate, affectionate, kind temperament
  • B. A major symptom category is accepted as positive when the symptom or one or more of its behaviors is identified in a semistructured, closed-end interview of patient and primary caretakers.
  • C. This symptom complex must precede the onset of other medical disorders (including depression, narcolepsy, various medications, alcohol and drug abuse, hypothyroidism) that can cause secondary hypovigilance.
  • D. Although symptoms may be identified at any age, the disorder generally becomes more symptornatic with schooling and aging, and can result in increasing maladaptation.