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remains unclear. Possible factors are the exacerbation of a
hitherto latent Bb infection [Millner et al. Lancet 336:125] by
RA, and/or the failure of the ceftriaxone therapy and/or the immunosupression by corticoids, methotrexate, and cyclosporine
27. HOW TO CLASSIFY SUSPECTED NEUROBORRELIOSIS
Michael M. Millner, R.R. Muellegger, and K.D. Wernecke,
In view of the possible false-positive and false-negative B. burgdorferi (Bb) parameters, the correct diagnosis of neuroborreliosis (NB) in children is known to be difficult. In a former
study a classification of clinically suspected cases of acute NB
into "definite NB" with positive Bb CSF titers (group I), "probable NB" with positive Bb serum titers (group II), and "nonNB" (group III) was developed empirically [Ann Neurol 1990;
28:463]. In order to re-evaluate group II cases and to reconsider
group I and group III cases both univariate and multivariate
statistical analyses were performed using the most relevant clinical (cephalea, stiff neck, paresis 7) = (fl, f2, f3) and lab data
(serum Bb-IgG, CSF protein elevation, CSF cells) = (f4, f5, f6).
Univariate analysis gave the following order of importance with
respect to the classification error (ce): f4 (12% ce), fl (18% ce),
f5 (29% ce), f6 (40% ce), f2 (40% ce), and f3 (60% ce). Multivariate analysis allows one to consider the features not only
separately but in their interactions. Therefore, multivariate discrimination in two groups of 39 (group I) and 61 (group III)
patients, respectively, yielded as the most significant feature f4
indeed (f4 allocated 88% of "probable NB" patients correctly),
but in combination with f5 even 91% of the children are correctly diagnosed. Without access to f4 the combination of fl and IB
nevertheless provides a rate of 82% of the correct classification
(note the separating power of f3 in combination with fl). In this
way patients with "probable NB" (group II) can be uniquely
allocated into one of the two classes "definite NB" or "nonNB." The diagnostic score developed on the basis of these results supports diagnostic/therapeutic decisions in suspected
cases of NB at bedside. Because of the reproducibility the
method may be adapted to other manifestations of lyme borreliosis with uncertain diagnosis as well.
28. N E U R O L O G I C DISORDERS IN SAUDI CHILDREN: A C O M M U N I T Y - B A S E D STUDY
S.M. AI Rajeh, O. Bademosi, H.M. Ismail, A.A. Awada, H.M.
A1 Freihi, A. Dawodu, and S. Assuhaimi, Riyadh and Damman,
We report the findings of a recent community survey of children
up to the age of 15 years in the eastern providence of Saudi
Arabia to provide baseline epidemiologic data on the frequency
of neurologic disorders. A total population survey of Thugbah, a
defined community, was conducted over a 6-month period. All
Saudi children living in the area were initially screened door-todoor by trained interviewers using a pretested questionnaire
(sensitivity 98%, specificity 89%) which included a simple clinical examination of those 7 years of age and older. Subjects with
abnormal responses (i.e., those with potential neurologic disease) were then examined by a neurologist to document the
presence or absence of neurologic disease. The 12,421 children
screened (6,323 males, 6,098 females) constituted 53.5% of the
total community population. Of these, 15% had abnormal responses. The consanguinity rate among the parents was 58%.
The major neurologic disorders with their respective point prevalence ratios per 1,000 population were mental retardation 6.5,
epilepsy 6.4, cerebral palsy 6.3, and microcephaly 3.2. Febrile
convulsion had a prevalence ratio of 1.7/1,000 in children up to
the age of 5 years. It is possible that the high consanguinity rate
in the population played a major role in the pathogenesis of
these disorders. Further studies are required to identify the contribution of other factors.
29. M I G R A I N E AND PUBERTY
Russell D. Snyder, MD and Robert Annett, PhD, Albuquerque,
Adult migraine predominates in women by a factor of about 2 to
1. In childhood a slight predominance in boys has been previously noted with a female predominance beginning about age
12 years and persisting thereafter. This shift is attributed to hormonal changes occurring around puberty in the female. In the
United States the mean age of onset of puberty for girls is 10.5
years and for boys is 12 years. An attempt to verify the pubertal
shift to female predominance was undertaken in a pediatric
headache clinic. The study group consisted of 89 consecutive
children with the diagnosis of migraine or migraine with aura. A
questionnaire, personal interview, and examination by neurology and neuropsychology were used to determine headache type
based upon the classification in Cephalgia [1988;8(suppl 7):996]. No persistent change in sex predominance at clinic presentation was noted at puberty although each sex had a transient
peak in frequency of presentation to clinic 1.5 years after calculated onset of puberty for females and 3 years for males (Fig 1).
When corrected for age of onset of headache rather than age of
presentation (Fig 2), the onset of headache was closely associated with the calculated onset of puberty in the female. These
similar peaks did not occur in children with tension-type headache (n = 43; Fig 3). In this clinic-based study of onset of
migraine in children, both sexes displayed a peak in late childhood before adolescence. Although puberty occurs later in the
male than in the female, the age of onset of headache is slightly
earlier in the male suggesting that puberty per se is not the
primary factor in the development of adolescent migraine. A
post-pubertal shift to female predominance was not demonstrated with the sexes remaining evenly divided after puberty.
The shift in adult migraine to female predominance may occur
later than adolescence and be related to factors other than pubertal hormonal changes.
30. F A M I L I A L DEFICIENCY IN C O M P L E X H O F
R E S P I R A T O R Y CHAIN P R E S E N T I N G AS LEUKODYSTROPHY
Fran~oise Gouti~res, Marie Bourgeois, Jean Aicardi, Pierre
Rustin, and Arnold Munnich, Paris, France
Two sisters born to consanguineous parents presented with a
degenerative neurological disorder beginning at age 10 months,
with marked rigidity, bilateral pyramidal tract signs, and cortical
blindness, clinically suggestive of leukodystrophy. CT and MRI
demonstrated diffuse cerebral white matter abnormalities and
symmetric foci of necrosis in the periventricular gray matter
PEDIATRIC NEUROLOGY Vol. 8 No. 5 351