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[Tendon areflexia in congenital myopathies accompanied by atrophy of type I fibers. Electrophysiologic study].

A predominance and/or an atrophy of type I fibers and a loss of deep tendon reflexes are often observed in different types of congenital myopathy. Various data indicate that both findings can be linked: dysfunction of the myotatic reflex can induce predominant involvement of type I fibers. In order to specify the mechanism of the loss of tendon reflex, an investigation of the Hoffmann reflex (H reflex) was performed in one case of centronuclear myopathy and in one case of congenital type fiber disproportion with type I hypertrophy. The Achilles tendon reflex was absent but the H reflex showed normal recruitment amplitude and latency. The Jendrassik maneuver reinforced the H reflex. These results indicate the involvement of muscle spindles or impairment of the fusimotor system. Nuclear bag intrafusal fibers have common characteristics with type I extrafusal fibers. Both types of fibers could be involved simultaneously in congenital myopathies, thus explaining the loss of tendon reflex.

Child↗

H-reflex study in normal children and patients with cerebral palsy.

The H-reflex was studied in 53 children with cerebral palsy, and the results were compared with those obtained for 56 normal control subjects. Pairs of identical stimuli were delivered and the time course of recovery of the amplitude of the H-reflex was determined. Recovery of the H-reflex was increased in the normal control group aged 0 to 12 mos compared to that in the normal control group aged 1 to 9 yrs, especially at interstimulus intervals from 100 to 800 msec. In children with spasticity, marked recoveries of the H-reflex were observed in both age groups, 0 to 12 mos and 1 to 9 yrs, compared to those in normal controls. In the cases of athetosis, recovery of the H-reflex was also marked. In children with ataxia, it was not pronounced and similar to that in normal controls. From these findings, an H-reflex study was considered to be useful for evaluation of central nervous system function in childhood.

Ataxia↗

[Evaluation of ischemic repercussions of intracranial hypertension].

The main risk involved in severe intracranial hypertension is, the occurrence of cerebral ischaemia, either locally during herniation or globally as a consequence of reduced cerebral perfusion pressure (CPP). Neurological features of ischaemia occur at a late stage. A continuous monitoring of brain function with EEG or evoked potential techniques, while largely used in the operating room have not been so far fully evaluated in the intensive care setting. Therefore, ischaemic criteria based on the registration of haemodynamic or metabolic data are gaining importance in management of increased intracranial pressure (ICP). Transcranial Doppler of middle cerebral arteries allows at any time the detection of a decrease in brain perfusion. An increased pulsatility index has been repeatedly demonstrated to correlate with decreased CPP. From these reports, the lower limit of autoregulation in brain injured patients appears to be much higher (70 mmHg) than previously estimated (40 mmHg). However, therapies with a cerebral vasoconstrictor impact and associated vasospasm are to be considered for a correct interpretation of Doppler data. Moreover, as a reduced cerebral blood flow is not necessarily insufficient to meet metabolic requirements, a routine insight in cerebral oxygenation and lactate production must be available. Continuous monitoring of jugular blood oxyhaemoglobin saturation (SjO2) measures the reserve of oxygen extraction and a decrease in SjO2 below 50% is considered as to indicate an impending cerebral ischaemia. Indeed, critically reduced CPP under a 70 mmHg limit is reflected by venous desaturation episodes. Increased cerebral lactate production, routinely appraisable by serial measurements of [(a-v) lactate], may afford confirmation of an existing ischaemia. ICP and CPP monitoring remains the basis for intensive care surveillance during the phase of intracranial hypertension, with alarming settled at admitted critical values (ICP = 30 mmHg; CPP = 70 mmHg). As ischaemic threshold for cerebral blood flow may be different in patients and in normal experimental animals, the reliability of these critical values of ICP and CPP is uncertain. Therefore, transcranial Doppler, jugular metabolic monitoring and, as recently available, cortical tissue PO2 monitoring are mandatory for early detection and assessment of ischaemia.

Acidosis, Lactic↗

Influence of prenatal cocaine exposure on full-term infant neurobehavioral functioning.

This study investigated infant neurobehavioral functioning during the newborn period in 334 full-term, African American neonates (187 cocaine exposed, 147 non-cocaine exposed) enrolled prospectively at birth, with documentation of drug exposure status through maternal interview and urine and meconium toxicology assays. Infants were assessed using the Brazelton Neonatal Behavioral Assessment Scale (BNBAS) during the newborn period (0-6 postnatal days). Findings from multivariate profile analyses support a consistent, modest effect of prenatal cocaine exposure on neurobehavioral functioning in full-term neonates. All of the BNBAS cluster scores, with the exception of abnormal reflexes, were similarly affected, sharing a common slope (D=-0.14; 95% CI=-0.27, -0.003; P=.046) representing a -0.14 point difference between cocaine-exposed and non-cocaine-exposed infants after controlling for prenatal exposure to alcohol, tobacco, and marijuana (ATM); maternal age, education, employment, primigravida status, and prenatal care visits; and infant sex and postnatal age in days. Fetal growth was also related to neurobehavioral functioning and, in part, mediated the relationship between cocaine exposure and the BNBAS cluster scores. Cocaine exposure during each trimester similarly influenced infant neurobehavioral profiles, with cocaine-associated deficits most pronounced in infants with exposure in all three trimesters. Results from qualitative and quantitative urine and meconium bioassay indicators further substantiated these results. Findings, while significant, represent modest effect sizes in full-term infants.

Black or African American↗

The effect of prenatal cocaine exposure on neurobehavioral outcome: a meta-analysis.

A meta-analysis was performed of the research published from 1985 to 1998 examining the effect of in utero exposure to cocaine on infant neurobehavioral outcome. The initial search for articles to include in the meta-analysis identified 18 studies with potentially meta-analytic variables. Of the studies originally retrieved, 13 failed to meet all of the inclusion criteria and were excluded from the meta-analysis. A total of 14 meta-analyses were performed comparing cocaine-exposed infants to nonexposed infants on NBAS cluster scales at birth and at 3-4 weeks of age. While the meta-analytic combination of studies produced a large enough sample size to drive statistical significance in a small majority of the tests of difference between the cocaine-exposed and nonexposed infants both at birth and soon after, the magnitude of all effects was small. The largest reliable differences appeared for the motor performance and abnormal reflexes clusters. Both also demonstrated a slight trend for increasing standard differences from birth to measures obtained at 3-4 weeks. The orientation and autonomic regulation clusters produced small, significant effects at both time periods, but the trend was for reduced effect sizes over time. All other effects appear truly negligible.

Cocaine-Related Disorders↗

Startle disease--hyperekplexia (two sibling cases).

Two sisters aged 12 and 13 applied to our hospital with complaints of jerking tonic contraction triggered by sudden noise. Diffuse bilaterally polyspike-wave discharges appeared on waking electroencephalography and during that time a startle-induced tonic contraction was seen. Their neurological examinations and neuro-radiological imaging were normal except for hyperreflexia. We presented these cases because of being rare.

Acoustic Stimulation↗

Hypertonia, hyperreflexia, and excessive startle response in a neonate.

Following an uneventful gestation, a newborn girl presented with hypertonia, hyperreflexia, tremor, and excessive startle response. Nose tap elicited a dramatic head recoil. Her mother had similar symptoms beginning as a child that improved but persisted into adulthood. In addition, several members of mother's family died unexpectedly in infancy. Hypertonia in the newborn period indicates central nervous system dysfunction of several possible causes, most of which are associated with severe cognitive deficits and limited neurological development.

Anticonvulsants↗

Neurobehavioural profile of low-birthweight infants with cystic periventricular leukomalacia.

Twenty-three low-birthweight infants (17 males, six females) diagnosed with cystic periventricular leukomalacia (PVL; median gestational age 30 wks, postmenstrual age range 25 to 36 wks; median birthweight 1365 g, range 680 to 2010 g) were evaluated and compared with 209 comparison infants (117 males, 92 females; median gestational age 33 wks, postmenstrual age range 25 to 39 wks; birthweight 1771 g, range 670 to 2460 g). There were three assessment times: 36 to 38 weeks (preterm), 40 to 42 weeks (term), and 44 to 46 weeks (post-term); the Neonatal Behavioral Assessment Scale (NBAS) was used for assessment. Outcome at 2 years was assessed on the basis of a neurological examination, magnetic resonance imaging, computerized tomography, electroencephalography, and the Mental and Psychomotor Development Indices of the Bayley Scales of Infant Development. All infants in the PVL group were found to have evidence of cerebral palsy, whereas all infants in the comparison group were normally developing. Infants with PVL performed more poorly on all elements of the NBAS examination at all three assessment times compared with the comparison group. They demonstrated poorer motor control, less responsiveness to environmental stimuli, less regulatory capacity, and more abnormal reflexes compared with the comparison group. These results suggest that LBW infants with PVL show dysfunction and/or disorganization in their neurobehavioural systems in the neonatal period. Assessment of neonatal neurobehavioural characteristics using the NBAS may assist clinicians in identifying LBW infants with PVL, and in formulating plans for the developmental care of these infants.

Age Factors↗

Pupillary areactivity in hydrocephalus of recent onset.

A patient who presented with bilateral loss of all pupillary reactions and normal ocular motor function is reported. Investigation revealed the presence of massive hydrocephalus. The syndrome developed shortly after transsphenoidal surgery for a suprasellar craniopharyngioma. Pupillary function returned to normal following the insertion of a ventriculo-peritoneal shunt. It is suggested that the syndrome was due to compression of the visceral oculomotor nuclei by a dilated sylvian aqueduct.

Adult↗

The crossed adductor reflex in humans: an EMG study.

Crossed adductor reflexes were recorded electromyographically in ten patients with bilateral lesions of the upper motor neuron. Three of these had a femoral neuropathy. Following blows directed to either the tendon of the adductor muscle or to the medial epicondyle of the femur, large amplitude responses of short latency and large amplitude responses of long latency were recorded from the contralateral adductor. The responses of short latency which were recorded from the ipsilateral and contralateral adductor muscles ranged from 18 to 27 ms. The contralateral responses of long latency ranged from 60 to 67 ms. On tapping the patella or patellar tendon, large amplitude responses of short latency were recorded from the contralateral adductor muscles while the ipsilateral adductor responses were absent. These responses resulted from stretch of the ipsilateral and contralateral adductor muscles. The contralateral responses were mediated across from the midline by an extraspinal mechanical pathway.

Adult↗

[Physiotherapy in spinal stenosis].

Spinal stenosis mainly is a disease of the elderly patient. Mostly the lumbar spine is affected. The assessment is based on the typical constellation of symptoms (neurogenic claudication, subjective weakness) and physical findings (abnormal reflex status, loss of strength, sensory deficits). It further is supported by the radiographic proof of stenosis of the spinal canal, the lateral recess and the intervertebral foramina. The targets of physical therapy are the relief of pain and an improvement concerning the activities of daily living, which are especially impaired by reduced walking distance and difficulties in climbing stairs as well. That can be achieved by physiotherapy treatment of myofascial disorders oral medication/local injection.

Age Factors↗

The polystyrene vacuum wheelchair cushion.

A polystyrene bead vacuum wheelchair cushion has been tried with several patients with abnormal postural reflex activity. Excess spasticity can be reduced by this means and sitting comfort enhanced.

Humans↗

The clinical neuropathology of spinal cord injury. A guide to the future.

The neuropathology of spinal cord injury (SCI) is reviewed in the light of clinical problems and as a guide to future research. The pathology of SCI in the acute stage suggests that the spinal cord may be partly preserved even in the most severe injuries. This finding emphasises the need for great care in roadside management. In the acute phase there are irreversible changes and possibly reversible changes which have not been adequately identified. Even a small percentage of nerve fibres escaping the initial injury would be of great benefit to the patient. In the subacute stage when transynaptic degeneration is proceeding there may also be associated functional changes leading to abnormal reflex activity. It is possible through an improved understanding of the neuropathology and neurophysiology of the isolated or partly isolated segments of the cord that new reflex connections may be stimulated to develop by artificial means. In the chronic stage there are well recognised complications such as osteoarthrosis with spinal stenosis, post-traumatic syringomyelia and traumatic nerve root neuroma formation, which may lead to clinical deterioration and which may be amenable to treatment. In a more theoretical sense it is possible that improved understanding of CNS plasticity and transplant neurobiology using recombinant DNA technology, grafting and 're-education' of the regenerated tissues may be rewarding in the longterm future. Although this outcome is entirely hypothetical at this stage basic research deserves great emphasis.

Forecasting↗

A clinical evaluation of head impulse testing.

The head impulse test is a simple clinical test comprising high acceleration head rotation. In the presence of a severe unilateral vestibular weakness the normal vestibulo-ocular reflex is replaced by a misalignment of the eye followed by a series of corrective saccades which are evident to the examiner. Previous reports have shown the high sensitivity of the head impulse test in detecting complete unilateral weakness, but indicate poor sensitivity for mild weaknesses. This prospective, blinded study examined the head impulse test in a general clinical population of balance disorder patients to examine the sensitivity and specificity of the test, and to determine the degree of vestibular weakness that is required before the test becomes positive. One hundred and fifty patients were examined and the head impulse test results were compared to results from bithermal caloric testing. Results show that the overall sensitivity of the head impulse test is 34% with a specificity of 100%. The test does not detect mild or moderate vestibular weaknesses but is very sensitive to the presence of a severe paresis (87.5%). Head impulse testing will not replace caloric testing but is a very useful adjunct to it.

Acceleration↗

Effects of dorsolateral spinal lesions on stretch reflex threshold and stiffness in awake cats.

Measurements of threshold angle and incremental dynamic stiffness (IDS) were derived from triceps surae stretch reflexes, elicited by ramp and hold flexion at the ankle joint of four cats that were tested while awake. Stretch reflexes were assessed from trials that began from different ankle joint start positions or were matched using a post-hoc analysis for initial background force during testing sessions before and following unilateral lesions of the dorsolateral funiculus at levels ranging from T13 to L3. Unilateral lesions of the dorsolateral funiculus (DLF) produced significant ipsilateral decreases in stretch reflex threshold and increases in reflex gain, measured as incremental dynamic stiffness (IDS). ANCOVA testing indicated that the reduction in threshold, but not the increase in IDS, was dependent upon the level of background force. Reflex testing from different start angles demonstrated that DLF lesions diminished the correlation between threshold and IDS. Intravenous infusion of ketamine dose-dependently reduced IDS, compared with testing in the unanaesthetized state. Postoperative reflex testing during infusion of ketamine at 22.2 mg/kg per h, when electromyographic responses were reduced to 24% of control levels, abolished differences in IDS between the ipsilateral and contralateral hindlimbs. These and related observations suggest that the postoperative increase in IDS in awake animals was not due to an increase in passive stiffness.

Anesthetics, Dissociative↗

Saphenous neuralgia after arthroscopically assisted anterior cruciate ligament reconstruction with a semitendinosus and gracilis tendon graft.

A case report of saphenous neuralgia following arthroscopically assisted anterior cruciate ligament reconstruction with hamstring tendons is presented. The patient complained of paresthesia in the anteromedial region of the lower leg and tenderness at the medial side of the knee without motor or reflex abnormalities. Because saphenous neuralgia can mimic disorders of the knee, peripheral vascular disease, and lumbar nerve root compression, diagnosis can be confirmed by anesthetic blockade. The patient underwent saphenous neurolysis. Six months after surgery, the patient had normal cutaneous sensation at the medial aspect of the lower leg and ankle and she no longer complained of any painful dysesthesia. To minimize the risk of damaging the saphenous nerve when harvesting hamstring tendons, the knee should be flexed and the hip external rotated.

Adult↗