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Asperger's syndrome.

This study presents a 14-year-old Saudi child with poor school achievement, diagnosed by psychiatrists as mentally retardated with a significant social deficit. No neurological disorder was evident. She has high to very high intellectual abilities on testing her intelligence quotient. Her verbal learning ability was also very high. Her social skills and capacity were impaired. No evidence of poor language was detected. The patient is diagnosed as suffering from Asperger's syndrome, and not mentally retarded. She has the criteria for Asperger's syndrome according to Diagnostic and Statistical Manual, 4th edition, including the significant social deficit with normal intellectual functioning. The utility of such a diagnosis is briefly discussed.

Adolescent↗

[How to examine a patient with higher cerebral dysfunction].

The mysterious maneuvers of the neuropsychological examination actually conceal the very intellectual process by which diagnosis is obtained. Five features that are usually sufficient to diagnose aphasia are 1) spontaneous speech, 2) speech comprehension, 3) repetition, 4) reading aloud with comprehension, and 5) writing. In spontaneous speech, fluent aphasics have normal rhythm, melody, and well-articulated sentence, but the speech is filled with paraphasias. The speech of non-fluent aphasics is slow, labored, and poorly articulated. When patients with spatial neglect are asked to perform a variety of tasks in space, they neglect the hemispace contralateral to their lesion. For example, when asked to draw a picture of a flower, they may draw only a half of the flower. The patients may fail to cross out lines distributed over a page. Amnesic patients, despite normal scores on psychometric tests of intelligence and normal performances on immediate memory, show significant impairments in new learning (anterograde amnesia) and remote memory (retrograde amnesia). In this article, The author also explained about two topics briefly of language functions (tactile reading) and hemispatial neglect (reference frames).

Amnesia↗

Neonatal hypoxic risk in preterm birth infants: the influence of sex and severity of respiratory distress on cognitive recovery.

The authors examined the differential effects of neonatal respiratory distress syndrome (RDS)--a condition associated with elevated risk for neonatal hypoxia-ischemia--on the cognitive development of the two sexes. The authors also attempted to establish whether the severity of respiratory distress is linked to cognitive outcome. Findings revealed an appreciable female advantage in cognitive recovery from RDS, yet the association between severity of RDS and outcome did not reach conventional statistical significance level. The sex effect on intelligence test performance was significantly greater for nonverbal subtests than for verbal ones. These results augment earlier findings of sex differences in cognitive recovery from perinatal intracranial hemorrhage. The current investigation also extends these results to a population of children at neonatal hypoxic risk for whom early brain injury was excluded on the basis of neonatal cranial ultrasound findings.

Cognition Disorders↗

Central nervous system involvement in nocturnal enuresis: evidence of general neuromotor delay and specific brainstem dysfunction.

PURPOSE: To assess the involvement of central nervous system factors in the etiology of childhood nocturnal enuresis, an ongoing study of the fine neurology and neurophysiology has been instituted. MATERIALS AND METHODS: A total of 22 patients 8 to 14 years old were subcategorized sporadic and familial groups. Patients underwent a number of examinations, including a complete family pedigree; blood samples for linkage analyses; pediatric examination; urinalysis; uroflowmetry with pelvic floor electromyography; 24-hour flow charts; parental micturition questionnaire; child behavior checklist; intelligence test; standardized child psychiatric interview; acoustic, visual and event related late evoked potentials; Zurich Neuromotor Test Battery and the startle-blink paradigm. In this preliminary report only the results of the Zurich Neuromotor Test Battery and startle-blink paradigm are reported. RESULTS: In the Zurich Neuromotor Test Battery patients with enuresis had a longer timed performance but did not have more associated movements than the controls. In the startle-blink modulation paradigm inhibition of the electromyograph amplitudes was greatest following prestimulation with an interval of 120 milliseconds. This inhibition is known to be reduced among patients with enuresis compared to nonwetting children. The results indicated a general developmental (neuromotor) delay, in addition to specific dysfunction of the brain stem in patients with enuresis. The pre-pulse inhibition, as well as inhibition of bladder emptying at sleep, are regulated by brainstem centers in close anatomical proximity to the pontine micturition center and by analogous mechanisms. CONCLUSIONS: Reduced pre-pulse inhibition may represent a genetically transmitted trait indicative of nocturnal enuresis.

Adolescent↗

[A case of early onset cerebellar ataxia with hearing loss, mental disturbance and primary hypogonadism].

A 14-year-old girl, whose birth and developmental history were normal till the age of 7, was admitted to our hospital because of slowly progressive difficulties in walking, speaking and hearing. She also complained of absence of menstruation. She showed poor school records since the age of 7. On neurological examination, she showed limb and truncal ataxia. There was no nystagmus but slurred speech was found. Muscular power was good and her sensory system was normal. Tendon reflexes were equally present, and plantar reflexes were flexor. Bilateral moderate nerve deafness was also present. Mental deficiency was diagnosed on an intelligence test. Brain CT and MRI showed cerebellar atrophy. Gynecological examination revealed scanty pubic hair and small uterus. Karyotype was 46XX. Endocrinological studies demonstrated high level of FSH, low level of E2, and the normal response to pituitary stimulation with LHRH, indicating the existence of primary hypogonadism. Although the etiology of this multisystem disorder is unknown, it is possible that both nervous and endocrine disorders were genetically determined.

Adolescent↗

"MARKINGS" OF A CLASS: THE STATISTICAL TRAIL LEFT BY THE FIRST GRADUATING CLASS IN MEDICINE, UNIVERSITY OF BRITISH COLUMBIA.

In 1954 the first class in medicine graduated from the University of British Columbia. This class of 57 men and three women left a statistical trail behind them which began before they entered medical school, and which now has extended 10 years into their professional postgraduate careers. This first class was made up largely of British Columbians of older age than subsequent classes. The overall achievement and aptitude of the class was high, as measured by premedical grades, intelligence tests and Medical College Admission Test scores. Interest tests at the time of admission indicated that the members of the class had major interest levels in the fields of science and social service or humanitarianism. The subsequent medical school performance of the class was exceptional. Of the class, 63.4% interned in teaching hospitals. By 1964 only 53.4% of the graduates were engaged in general practice. Most of the graduates are now practising in British Columbia.

Achievement↗

Congenital myotonic dystrophy: report of one case.

We report a laboratory-confirmed pediatric case of congenital myotonic dystrophy (CMD) in Taiwan. A 5-year-old girl presented in our neurological clinic with the chief complaints of developmental delay and slowness in learning. Clinical examination, intelligence tests, and developmental examinations revealed myopathic facial weakness, passivity and delayed cognitive development. Past history review showed neonatal hypotonia, feeding difficulty with cyanosis, and poor activity. Neonatal studies only revealed subgaleal hematoma and subarachnoid hemorrhage with mild degree right side intraventricular hemorrhage. Current neurological examination disclosed percussion myotonia and myotonia as poor muscle relaxation after forceful handgrip. CMD was confirmed by electromyography study and DNA analysis. The patient's mother, though minimally affected, had similar laboratory results. CMD affects children in both motor and cognitive function; the diagnosis should be entertained in developmentally delayed children with subtle neurological signs, even without strong family history.

Child, Preschool↗

[A preliminary study on speech therapy for adult cleft palate patients].

OBJECTIVE: To study speech feature and treatment of adult with misarticulation by review 20 patients' clinical information. METHODS: 20 adult patients (10 male, 10 female, age ranging from 20 to 38 years, mean age 20.35 years) with misarticulation were available for this study. All received specialized examination, Chinese speech intelligibility test and blowing test before speech treatment, 17 cases underwent posterior pharyngeal flap operation before speech treatment. Speech treatment included audio discrimination, behaviour training, lip phone training, aspirates and fricatives training, tongue lolling out, comprehensive training, et al. RESULTS: All cases were restored normal speech approximately after speech treatment, consonants loss and hypernasality of some patients totally disappeared. But there were some complications in speech treatment of 4 cases. CONCLUSION: The outcome of appropriate adult speech treatment was satisfactory, though adult speech treatment was more difficult than child's.

Adult↗

[Diagnosis of velopharyngeal incompetence after primary cleft palate repair].

Fifty four patients who had had primary cleft palate surgery and whose speech remained defective were examined by speech intelligibility test, lateral videofluoroscopy and flexible nasopharyngoscopy. We found that there are obvious individual differences in the velopharyngeal closure pattern of the patients with speech deficiency following primary surgery. The velopharyngeal closure patterns can be grouped into five categories according to the manner of the velum and lateral pharyngeal wall movements. The criteria of the classification and its significance are discussed in this paper.

Adolescent↗