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Biomedical subjects

H Costeff

Publications and source records attributed to H Costeff.

At least 19 recordsLinked to original sources

Prognosis for recovery from prolonged posttraumatic unawareness: logistic analysis.

This study reviews the course and outcome of 130 patients who remained in a state of prolonged unawareness 30 days after severe cranio-cerebral trauma. Prognostic indicators and outcome were fitted by a logistic model. The significant prognostic factors observable in the first week after trauma were found to be ventilatory status, motor reactivity and significant extraneural trauma. The significant prognostic factors after the first month of unawareness were early ventilatory status, early motor reactivity, late epilepsy and hydrocephalus. The estimated probability of recovery of awareness (that is, consciousness) ranged from 0.94 in patients with early decorticate posturing in the absence of both extraneural trauma and ventilatory disturbance to 0.06 in patients with flaccidity, extraneural trauma and ventilatory disturbance in the first week after injury.

Arousal

Covariance analysis of laboratory variance in steady-state serum phenytoin concentrations.

Inpatients (n = 57) on long term prophylaxis with 2 oral phenytoin preparations were followed up via monthly checks of serum drug concentrations. Duplicate serum aliquots were submitted to 2 laboratories, and covariance analysis was used to estimate laboratory error. The laboratory-associated variance of examinations using the 'EMIT' or 'TDX' systems was 7.1 to 10 (mg/L)2, while that of chromatographic assays was 37.1 (mg/L)2. Laboratory errors were distributed in a non-normal fashion. The ratio of the maximum rate of metabolism (Vmax) and the Michaelis-Menten constant (Km) tended to remain constant between individuals. A linear analytical model showed little more residual variance than one based on Michaelis-Menten pharmacokinetics.

Administration, Oral

Growth hormone secretion in Prader-Willi syndrome.

Integrated 12-hour growth hormone secretion studies, peak growth hormone response to clonidine provocation. Somatomedin-C levels, T-4 and TSH levels were studied in six growth-retarded children with the Prader-Willi syndrome, of whom five had a 15 q-karyotype. Only one of the subjects was obese. All showed abnormally low growth hormone secretion. None achieved a nocturnal peak above 10 micrograms/l, none had a mean nocturnal level over 1.8, and none showed a level above 8 micrograms/l after clonidine provocation. These findings contrasted with normal TSH in all and normal T-4 in five. These findings suggest that the poor linear growth in the Prader-Willi syndrome is caused by a true deficiency of growth hormone secretion, and that the low growth hormone levels observed in such cases are not an artifact of obesity.

Child

Long-term follow-up review of 31 children with severe closed head trauma.

Thirty-one children aged 3 to 15 years were followed for 5 to 11 years after suffering severe closed head trauma which caused coma for 1 week or more (median duration of coma 3 weeks). One patient remained in a persistent vegetative state until his death 9 years later. The other 30 recovered consciousness and were discharged. All suffered diminution of their abilities, and 24 of them had major permanent disability. The most common motor disabilities were pure spastic hemiparesis (seven cases), basal ganglia syndromes (four cases), ataxia (three cases), and a combination of hemiparesis and ataxia (five cases). Of the 30 patients, 26 regained independent ambulation, seven were epileptic, and 14 were dysarthric in various degrees. Only 10 had the cognitive ability to profit from the normal educational system, and none had attempted postsecondary education. Social problems were common. The worst outcomes were associated with intracranial bleeding and/or brain contusion seen on computerized tomography (CT) scans at the acute stage; the best were associated with normal CT scans. The degree of residual disability in these children seems no less than that of adults with trauma of similar severity.

Adolescent

Motor abnormalities during sleep in patients with childhood hereditary progressive dystonia, and their unaffected family members.

The structure of sleep and number of body movements (BMS) and periodic leg movements during sleep (PMS), were studied in three unrelated girls suffering from L-DOPA responsive hereditary dystonia with marked diurnal fluctuation and in their 11 healthy, close relatives. All three girls had an increased number of BMS during rapid eye movement (REM) sleep. Five of the six parents and three siblings had abnormal PMS. One pair of parents had BMS similar to those of their affected daughter. The occurrence of BMS and PMS in the families studied may indicate a common mechanism for both. Because familial PMS is quite rare in its pure form, and this type of dystonia is also rarely encountered, the occurrence of BMS and PMS in members of these families may imply a causative relation between these two sleep-related motor phenomena.

Adolescent

A familial syndrome of infantile optic atrophy, movement disorder, and spastic paraplegia.

We describe 19 cases of a familial syndrome consisting of infantile optic atrophy and an early movement disorder in which chorea predominated. About one-half the patients developed spastic paraparesis during the second decade of life. Ataxia and cognitive deficits were common, usually of mild degree. Seventeen of the patients were females. Sixteen had similarly affected siblings, but none had affected parents. All but one belonged to the Iraqi Jewish community in Israel, giving a minimal prevalence rate in this ethnic group of about 1:10,000.

Adolescent

Rehabilitation outcome after anoxic brain damage.

Outcome was analyzed in a group of 31 patients who were comatose for more than 24 hours after cardiopulmonary arrest and were thereafter referred for inpatient rehabilitation. Seventeen regained functional ambulation, 20 regained oral communication, and 13 regained full independence in activities of daily living. Two regained their approximate previous cognitive level, and one regained his previous level of employment. These outcomes are strikingly inferior to those of patients with prolonged coma after craniocerebral trauma who were hospitalized in the same center during the same period. Both age and coma duration were correlated with outcome. The relatively better outcomes were seen in patients who were 25 years or younger at the time of anoxic injury, and whose coma lasted less than 24 hours.

Activities of Daily Living

Late neuropsychologic status after childhood head trauma.

A neurologic and neuropsychologic test battery was administered to a sample of 35 children drawn from all those in a defined geographic area who had been hospitalized for head trauma before age 7 during the years 1970-1976. Examination was performed 3 1/2 to 10 years after injury, at age 6-15. Twelve subjects had been diagnosed at the time of injury as suffering moderate insult and had been referred to the metropolitan neurosurgical center, while twenty-three with only mild injury had been retained for observation in a local pediatric ward. The twelve with more severe insult were significantly inferior to the other subjects on the Block Design and Coding subtests of the revised Wechsler Intelligence Scale for Children. The Koppitz score of the Bender Test, the WISC-R scatter, the Benton Visual Retention Test, the GATB Motor Speed Test and the Bourdon-Wiersma Vigilance Test showed less diagnostic power and failed to distinguish between the group with more severe injury and that with less. A detailed and carefully scored neurologic examination also failed to distinguish between the two groups. The findings suggest that relatively common traumatic injury may be associated with detectable late cognitive deficit, and that some WISC-R subtests may be among the best measures for detecting such deficit.

Brain Injuries

Eye-sighting preference of normal and hemiplegic children and adults.

Monocular eye-sighting preference was examined in 32 children with hemiplegia, 51 adult-onset hemiplegic patients and 57 normal children and adults. Eye preference was compared with ear preference on dichotic listening for 25 of the hemiplegic children. No independent association could be detected between eye preference and ear preference. In both the children and adults with hemiplegia the preferred eye tended to be on the same side as the damaged hemisphere. Among the adults, this tendency was more pronounced with more extensive lesions, as manifested by aphasia and/or hemianopia. These findings are interpreted as indicating that eye-sighting preference is unrelated to unitary hemispheric dominance, and that, unlike dominance for hearing and speech, it is not irreversible after a critical period of development. A simpler explanation than incomplete hemispheric dominance is offered for the weakly positive association between mixed laterality and cerebral dysfunction.

Adult

Blink reflex in stroke: follow-up and correlation with function and CT parameters.

Blink reflex (BR) was examined serially in patients 1, 2 and 3 months after unilateral hemispheric cerebrovascular accident and compared with functional state and CT findings of lesion extent and location. BR R2 components were depressed and correlated with lesion size. Initial walking ability was correlated with latency and amplitude of both direct and consensual R2 elicited by stimulation of the paretic side. No correlation was found between BR and arm function or the final ambulatory ability. A model suggesting a close association between BR projection-facilitating fibers and those mediating facial movements is presented.

Blinking

The risk of having a second retarded child.

We have studied segregation ratios in 282 Israeli families with normal, nonconsanguineous parents and retarded offspring without specific etiologic diagnosis. Severity of retardation and nature of medical history significantly affected recurrence risk, while sex of propositus and sibs and presence or absence of epilepsy, cerebral palsy, microcephaly, and short stature did not. Segregation rations were 0.095 in cases of severe retardation with normal medical history, 0.216 for mild retardation with normal medical history, 0.230 in cases of all retardation with maternal reproductive inefficiency, and 0.110 for all retardation with other reported prenatal, perinatal, or infantile complications. Estimated recurrence risks in most simplex families dropped sharply with each additional normal child. The recurrence risks are higher than some previously published estimates. Different ascertainment criteria may be responsible for this variation. The criteria used here were compatible with a 3.2% population prevalence of mental retardation with a 0.223 segregation ratio in multiplex sibships. It is recommended that future studies of recurrence risks include similar data permitting evaluation of sensitivity of ascertainment criteria.

Birth Order

Fluctuating dystonia responsive to levodopa.

Four cases of hereditary progressive dystonia with diurnal fluctuation were studied. All were sporadic; three of them mimicked spastic diplegia; and the fourth showed some similarity to torsion dystonia. Emotional or cognitive disturbance, or both, was seen in three. The correct diagnosis was suggested by fluctuating signs and symptoms, which worsened towards evening, but this was reached only after many years of handicap, hospital admissions, and invasive diagnostic procedures. Typically there was a prompt, pronounced, and sustained response to moderate doses of levodopa. Sleep recordings were obtained in three patients and showed increased body movements during rapid eye movement sleep. Several close relatives had periods of increased leg movements during sleep. It is suggested that hereditary dystonia responsive to levodopa should be considered as the diagnosis in children with fluctuating signs of motor disability syndromes, simulating torsion dystonia or spastic diplegia. Polysomnographic studies may be helpful in diagnosis and may also detect early or subclinical cases.

Child

Evaluation of Tinel's and Phalen's signs in diagnosis of the carpal tunnel syndrome.

In 80 upper extremities clinically suspected of carpal tunnel syndrome, electromyography (EMG) was performed and the Phalen and Tinel signs were sought. These two signs showed relatively low sensitivity (60-67%) and specificity (59-77%) despite a statistically significant association with the EMG findings. These two signs are not reliable as clinical criteria for carpal tunnel syndrome.

Adult

Survivors of severe traumatic brain injury in childhood. II. Late residual disability.

36 survivors of very severe traumatic childhood brain injury have been followed for a median period of 48 months after injury. One remains in a vegetative state and 35 have been discharged. 34 regained ambulation, and motor function became essentially normal in 14 of them. Ataxia and movement disorders were as common among the residua as was spasticity. Only one child remained aphasic, but 14 showed dysarthria. Six of the 36 showed major dementia and most of the rest showed very significant new impairment of cognitive and social function. This impairment was greater than expected from changes in the I.Q. Motor and speech function showed prolonged recovery while learning ability and social function did not. The overall degree of recovery seems not much better in these children than in adults.

Adolescent