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Cognitive impairments in patients with congenital nonprogressive cerebellar ataxia.

OBJECTIVE: To report neuropsychologic functions and developmental problems of patients with congenital nonprogressive cerebellar ataxia. BACKGROUND: Growing interest in cerebellar function has prompted closer attention to cognitive impairments in patients with cerebellar damage. METHODS: The authors studied 11 patients with nonprogressive congenital ataxia (NPCA) with Wechsler's intelligence testing, with additional tests of attention, memory, language, visual perception, and frontal functions. RESULTS: Seven of the 11 patients had an IQ of 60 to 92, with marked nonverbal deficits and subnormal to normal verbal performance (group A). Four patients had an IQ of 30 to 49 without pronounced profile asymmetry (group B). Four of the 7 group A patients had decreased alertness and sustained attention, but all had normal selective attention. Tests of frontal functions and memory yielded higher verbal scores than nonverbal scores. There was no deficit on the Aachener Naming Test (similar to the Boston Naming Test), because there were marked difficulties in the majority with visuoconstructive tasks and visual perception. Group B was significantly abnormal in almost all subtests, having a less prominent but similar profile. CONCLUSION: Patients with NPCA have significant cognitive deficits with an asymmetric profile and better verbal than nonverbal performance. Effects on nonverbal performance of longstanding deficits in visuospatial input during learning, the influence of impaired procedural learning, and asymmetric plasticity of the cerebral hemispheres may contribute to this uneven neuropsychological profile.

Adolescent↗

Sixty children with congenital hypothyroidism detected by neonatal thyroid: mental development at 1, 4, and 7 years: a longitudinal study.

A prospective study of mental development has been carried out in 60 unselected children with congenital hypothyroidism (C.H.) detected between 1976 and February 1985. Treatment with 1-thyroxin was initiated at the age of 10.4 +/- 2.5 days and supervised by T4 and TSH monitoring in 3-6 monthly intervals. Although clinical signs were mild or missing, skeletal maturation was prenatal in more than half. Developmental testing was carried out at 1, 4 and 7 years by the Brunet-Lezine (n = 60), the non-verbal Snijders-Oomen (n = 40), and the german version of the Wechsler intelligence test (n = 20), respectively. The global DQ/IQ in all but a few children at each age group are within the 3rd and 97th percentile of the DQ/IQ distribution in a control group tested during the same period of time. The mean values of all global and subscores, however, are lower than that one in the control group, differences being highly significant at 1 year. In 21 of our 60 children, associated findings and/or risk factors were present. The DQ/IQ in children without risk factors are identical to that of the control group. There are no significant DQ/IQ differences neither between athyroidism and ectopic thyroid glands, nor between prenatal bone age and normal skeletal maturation at diagnosis. We assume that the favourable results in our children with C.H. may be ascribed to the early onset of thyroid hormone replacement and the strictly controlled compliance.

Aging↗

Assessing cognitive estimation.

The Cognitive Estimation Test (CET) was devised in an attempt to quantify the tendency observed in patients with frontal lobe lesions to produce bizarre estimations in response to fairly simple questions, despite performing normally on standard intelligence tests. Several studies have been published where CET performance in patient groups has been examined. However, there is a paucity of adequate normative data. In the present study, representative normative data are provided from 150 healthy controls. CET performance was found to be moderately related to general intellectual ability, with females performing more poorly than males. The scale was examined psychometrically, and was found to be factorially impure, with poor internal reliability but adequate inter-rater reliability. Further revision of the CET is required in order to render it a psychometrically acceptable instrument.

Adolescent↗

Villchur revisited: another look at automatic gain control simulation of recruiting hearing loss.

An algorithm to simulate the effects of sensorineural hearing impairment on speech reception was investigated. Like that described by Villchur [J. Acoust. Soc. Am. 62, 665-674 (1977)], this simulation employs automatic gain control in independent frequency bands to reproduce the elevated audibility thresholds and loudness recruitment that are characteristic of this type of loss. In the present implementation, band gains are controlled in an effort to simulate loudness recruitment directly, using recruitment functions that depend only on the magnitude of hearing loss in the band. In a preliminary evaluation, two normal-hearing subjects listened to the simulation matched to hearing losses studied previously [Zurek and Delhorne, J. Acoust. Soc. Am. 82, 1548-1559 (1987)] with noise-masking simulations. This evaluation indicated that the present automatic gain control simulation yielded scores roughly similar to those of both the hearing-impaired listeners and the masked-normal listeners. In the more-detailed evaluation, the performance of three listeners with severe sensorineural hearing loss on several speech intelligibility tests was compared to that of normal-hearing subjects listening to the output of the simulation. These tests included consonant-vowel syllable identification and sentence keyword identification for several combinations of speech-to-noise ratio, frequency-gain characteristic, and overall level. Generally, the simulation algorithm reproduced speech intelligibility well, though there was a clear trend for the simulation to result in better intelligibility than observed for impaired listeners when high-frequency emphasis placed more of the speech spectrum above threshold at higher frequencies. Also, the hearing-impaired listener with the greatest loss showed the largest discrepancies with the simulation. Overall, however, the simulation provides a very good approximation to speech reception by hearing-impaired listeners. The results of this study, together with previous studies of noise-making simulation, suggest that threshold elevation and recruitment, which are necessary features of a simulation of cochlear hearing loss, can also be largely sufficient for simulating the speech-reception performance of listeners with moderate to severe hearing impairments.

Adult↗

[Treatment with hyperbaric oxygen. Illustrated by the treatment of a patient with retinitis pigmentosa].

A 26 year old man, who was physically fit and occupationally active but had retinitis pigmentosa and a slight hearing impairment, received hyperbaric oxygen treatment for 97 minutes at a pressure of 240 kPA for five days a week over a period of four weeks. After cessation of the treatment his lateral vision was found to be improved. The patient was examined both prior to and after the hyperbaric oxygen treatment to assess possible adverse effects. The examination consisted of a comprehensive neuropsychological test battery, audiometry and spirometry. The patient showed a reversible reduction in gas diffusion for CO (TLCO). No adverse effects on the central nervous system were found. He achieved higher scores in an intelligence test when retested. The gain was greater than usually found as a practice effect. The article discusses possible indications for hyperbaric oxygen treatment.

Adult↗

[Influence of iron status on attention and intellectual performance of a population of Spanish adolescents].

Dietetic, hematologic and biochemical data were used to asses the iron status of a group of 64 adolescents (37 males and 27 females), aged 15 to 18 (mean age 15.94 +/- 0.76 years), who study in a High School in the comunidad Autónoma de Madrid. All were asked to keep a dietary record during 5 days, one of which had to be a sunday. Iron intake was estimated using the Food Composition Tables of the Instituto de Nutrición (1990). The hematologic survey determined hemoglobin hematocrit mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), serum iron and serum ferritin. These data were correlated with the scores obtained in the attention and school capability test (AT), that gave information about the verbal (V), reasoning (R) and calculus (C) capabilities. There is a positive correlation between MCV (r = 0.2705), MCH (r = 0.3370) ferritin (r = 0.3383) and attention. MCV (r = 0.2995), MCH (r = 0.3998), MCHC (r = 0.3134) and ferritin (r = 0.3970) were also correlated with the speed capability shown on the attention test and the hemoglobin level was correlated with the calculus capability (r = 0.2905). The students who obtained higher scores in the school capability test had also better blood parameter values. This was statistically significant for serum ferritin in males students and for hemoglobin and MCHC in female students. 19.6% of the adolescents had ferritin levels lower than 12 ng/ml. Their intelligence test scores were lower to those who had serum ferritin > or = 12 ng/ml.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[BREV: a new clinical scale for the evaluation of cognitive function in school-age and preschool-age children].

BREV (Batterie Rapide d'Evaluation des fonctions cognitives) is a new evaluation test for the screening of cognitive disorders in 4-9-year-old children, based on a neuropsychological process. It is made up of 17 subtests which have been carefully standardized. It is not an intelligence test but a tool for children' health professionals to use as a rapid neuropsychological screening test. It is particularly recommended for any child with a school learning disorder or neurological history with a high risk of cognitive disturbances such as epilepsy. It may also be used as a systematic screening test.

Child↗

Development and testing of an intraoral electrolarynx for laryngectomy patients.

A wireless intraoral electrolarynx for laryngectomy patients has been developed. The instrument consists of an earphone receiver and a pickup coil housed in the patient's denture. A separate transmitter with hand switch, worn under the patient's clothing, activates the receiver. Intelligibility testing revealed that this device was comparable to the Verbalizer Electrolarynx, a transoral instrument similar to the Cooper-Rand Electrolarynx. If the long-term endurance study now in progress proves that the device can meet the communicative needs of the laryngectomee, and if the favorable results obtained in this study can be duplicated with subsequent instruments, this electrolarynx may be a viable alternative to others now available.

Humans↗

Retest reliability of the Wechsler Preschool and Primary Scale of Intelligence restandardized in Iran.

Retest reliability of the Wechsler Preschool and Primary Scale of Intelligence test scores was performed in Iran using 30 subjects in a time interval of 5 to 6 wk. The results of this analysis suggest that this restandardization of the WPPSI provides a consistent measure of intelligence for application in Iran. Reliability coefficients of .83, .90, and .88 were found for Verbal IQ, Performance IQ, and Full Scale IQ, respectively.

Child↗

Studies of psychophysiological and temporal conditions of work.

The main areas studied until the 70s were fatigue and rest. Many tests for fatigue were examined, and studies failed to find their practical use. Fatigue grows according to the positive acceleration curve while recovery grows according to the negative acceleration curve. Different mechanisms are involved in intelligence test performance when subjects are fatigued and when rested. Contrary to the pharmacological, psychological stimulants increase performance without adverse effects on the organism. The differences in absenteeism between male and female workers are related to family duties. After the 70s, research was focused on shiftwork. Shiftwork is associated with imposed change in a worker's normal activity pattern. It brings about fatigue, negative moods, and impaired health, sleep, safety, and working capacity. The shiftwork tolerance is connected with introversion, neuroticism, morningness, control of behavioural arousal, and parameters of circadian rhythms. The most important predictors of shiftwork tolerance are dimensions of control of behavioural arousal and morningness, while the most important criterion is sleep quality.

Central Nervous System Stimulants↗

Late effects of treatment on the intelligence of children with posterior fossa tumors.

This retrospective pilot study was undertaken to evaluate the late effects of treatment on intelligence in a population of children with posterior fossa tumors. Ten children with posterior fossa tumors treated with radiation and chemotherapy received intellectual evaluations at least one year following diagnosis. Six children had medulloblastomas, one child had a fourth ventricular ependymoma, two children had brainstem gliomas, and one child had a recurrent cerebellar astrocytoma. Children with supratentorial tumors were specifically excluded from the study in order to eliminate the possible influence of the tumor on intellectual functioning. Four children had had intelligence testing in school prior to treatment of their tumor. In each case results following treatment revealed a deterioration of full scale IQ of at least 25 points. Six children did not have prior testing; of these, two had IQ's less than 20. Overall, 50% of the patients had IQ's of less than 80 and 20% had IQ's of greater than 100. Furthermore, four children with normal intelligence (IQ greater than 80) have learning problems requiring special classes. Thus, of the ten children evaluated, all have either dementia, learning disabilities, or evidence of intellectual retardation. This study suggests that aggressive treatment of children with brain tumors may improve survivals but may be associated with significant long-term disabilities.

Adolescent↗

[A simple audiometry test of speech intelligibility in background noise].

We propose a new audiometric test to assess French speakers' speech discrimination in noise. This soundfield test uses a standard cocktail-party background noise superimposed on Fournier's disyllabic word lists. The background noise is CD-recorded at constant 55 dB SPL. The discrimination score is measured at a signal-to-noise ratio of -5 dB, 0, and dB. In comparison with other audiometric tests in noise, our test requires only 5 minutes and is simple for both patient and audiologist. All patients are tested using the same standard condition, one that approaches daily life and uses a noise of moderate intensity. The result is reproducible and easy to calculate and graph. We propose this test for diagnosing in-noise speech intelligibility disorders and for assisting the process of conventional or implantable hearing-aid fitting.

Audiometry↗

Speaking clearly for the hard of hearing. III: An attempt to determine the contribution of speaking rate to differences in intelligibility between clear and conversational speech.

Previous studies (Picheny, Durlach, & Braida, 1985, 1986) have demonstrated that substantial intelligibility differences exist for hearing-impaired listeners for speech spoken clearly compared to speech spoken conversationally. This paper presents the results of a probe experiment intended to determine the contribution of speaking rate to the intelligibility differences. Clear sentences were processed to have the durational properties of conversational speech, and conversational sentences were processed to have the durational properties of clear speech. Intelligibility testing with hearing-impaired listeners revealed both sets of materials to be degraded after processing. However, the degradation could not be attributable to processing artifacts because reprocessing the materials to restore their original durations produced intelligibility scores close to those observed for the unprocessed materials. We conclude that the simple processing to alter the relative durations of the speech materials was not adequate to assess the contribution of speaking rate to the intelligibility differences; further studies are proposed to address this question.

Hearing Loss, Sensorineural↗

Test-retest variability in children with epilepsy--a comparison of WISC-R profiles.

Reports of test-retest variability appear recurrently in relation to epilepsy. To date, the amount of information available on the stability of cognitive profiles over a number of years is limited. In this study, 45 children were assessed, using the Wechsler Intelligence Test for Children - Revised (WISC-R). The children were retested after a period of at least 2 years. A group of 20 children was tested 3 times. The average follow-up period was 4.2 years. Although most subjects in our sample had a high seizure frequency and continued to have their seizures during the period of follow-up, a marked stability of cognitive profiles is found over a period of time. These profiles show low scores for the subtests Information, Coding, Digit Span, Vocabulary and resemble profiles reported in other studies on subjects with intractable epilepsies.

Anticonvulsants↗

Sequential MR imaging and proton MR spectroscopy in patients who underwent recent detoxification for chronic alcoholism: correlation with clinical and neuropsychological data.

BACKGROUND AND PURPOSE: Chronic alcohol abuse may cause neuropsychological disorders and result in brain atrophy. The purpose of this study was to evaluate the metabolic, morphologic, and functional cerebral changes in the early stage of abstinence from chronic alcoholism. METHODS: Seventeen alcohol-dependent patients underwent MR imaging and MR spectroscopy on days 1 through 3 and days 36 through 39 of abstinence. In addition, psychological performance measures testing intelligence, concentration, attention, and memory were applied. Neuropsychological data were correlated with spectroscopic and volumetric results by using a Pearson's product moment correlation. The same measurements were also performed in 12 healthy, age-matched control subjects. Peak integral values for N-acetylaspartate (NAA) and choline (Cho) were referred to the peak integral value of creatine (Cr) as the internal reference. RESULTS: NAA/Cr was decreased in the patients in both the frontal lobes and cerebellum immediately after cessation of drinking (days 1 through 3). After 36 to 39 days of abstinence, NAA/Cr had significantly increased in the patients and corresponded to performance on psychological tests. The Cho/Cr ratio was decreased in the cerebellum during early abstinence but was recovered on days 36 through 39. The patients had enlarged CSF spaces 1 to 3 days after detoxification, which decreased during sobriety. The extent of brain atrophy did not correspond to performance on psychological performance tests. CONCLUSION: Regression of brain atrophy and metabolic recovery occurs at an early stage after abstinence from chronic alcohol abuse. MR spectroscopy findings return to normal metabolic levels within weeks after detoxification. The recovery of NAA/Cr is associated with improved performance on neuropsychological tests.

Adult↗

Intelligence and EEG current density using low-resolution electromagnetic tomography (LORETA).

The purpose of this study was to compare EEG current source densities in high IQ subjects vs. low IQ subjects. Resting eyes closed EEG was recorded from 19 scalp locations with a linked ears reference from 442 subjects ages 5 to 52 years. The Wechsler Intelligence Test was administered and subjects were divided into low IQ (< or =90), middle IQ (>90 to <120) and high IQ (> or =120) groups. Low-resolution electromagnetic tomographic current densities (LORETA) from 2,394 cortical gray matter voxels were computed from 1-30 Hz based on each subject's EEG. Differences in current densities using t tests, multivariate analyses of covariance, and regression analyses were used to evaluate the relationships between IQ and current density in Brodmann area groupings of cortical gray matter voxels. Frontal, temporal, parietal, and occipital regions of interest (ROIs) consistently exhibited a direct relationship between LORETA current density and IQ. Maximal t test differences were present at 4 Hz, 9 Hz, 13 Hz, 18 Hz, and 30 Hz with different anatomical regions showing different maxima. Linear regression fits from low to high IQ groups were statistically significant (P < 0.0001). Intelligence is directly related to a general level of arousal and to the synchrony of neural populations driven by thalamo-cortical resonances. A traveling frame model of sequential microstates is hypothesized to explain the results.

Adolescent↗