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Primary nocturnal enuresis: omega-3 fatty acids may be of therapeutic value.

Primary nocturnal enuresis (PNE), or bed-wetting, is a distressing urinary condition which can persist through childhood and beyond. Altered prostaglandin and nitric oxide production have been observed in children with PNE, and prostaglandin inhibitors are known to be of therapeutic value. Omega-3 fatty acids have the potential to influence the symptoms of PNE by inhibition of prostaglandin and renal nitric oxide production. In addition, children with PNE have an inappropriate startle response and an apparent maturational delay of the central nervous system. Research clearly shows that omega-3 fatty acids play a critical role in the development and function of the central nervous system. It is our contention that inadequate omega-3 intake may play a role in the lack of inhibitory input to the startle and micturition centers in PNE.

Animals↗

The origin of sympathetic outflow in heart failure: the roles of angiotensin II and nitric oxide.

The regulation of sympathetic nerve activity in chronic heart failure (CHF) has been an area of renewed investigation. Understanding the central mechanisms that are responsible for sympatho-excitation in this disease state may help in reducing the deleterious effects of chronic sympatho-excitation. This review will summarize our understanding of abnormal reflex control of the circulation in CHF. The roles of the arterial baroreflex, the chemoreflex, the cardiac sympathetic afferent reflex and the cardiopulmonary reflex are discussed. New experimental techniques that allow genetic manipulation of substances such as nitric oxide synthase in discrete areas of the brain aid in clarifying the role of NO in the modulation of sympathetic tone in the CHF state. Lastly, clinical implications of this work are discussed.

Angiotensin II↗

Sporadic major hyperekplexia in neonates and infants: clinical manifestations and outcome.

The aim of the present study is to report on the syndrome of sporadic major hyperekplexia during the neonatal period and early infancy, diagnosed in 39 patients at an average age of 3.3 months, the most severely affected during the first month of life. The patients mainly presented with marked irritability and recurrent startles in response to handling or even minute sounds, accompanied by rhythmic jerky movements and occasionally breath-holding episodes. Family history was negative for hyperekplexia, although eight parents reported jerky leg movements during sleep. The hallmark of hyperekplexia consisted of a hyper-alert gaze and an exaggerated startle with delayed habituation, also elicited by nose tapping and air blowing on the face accompanied by increasing rigidity. Nine severely affected infants, presenting with relentless startles, marked stiffness, violent rhythmic jerks, and breath-holding episodes were treated with oral low doses of clonazepam and completely recovered. Overall, the debilitating symptoms of hyperekplexia gradually resolved in all 39 infants, and their developmental assessment by 2 years of age was within the normal range. Therefore a prompt diagnosis of hyperekplexia during the neonatal period and early infancy, and then treatment if required with benzodiazepines to alleviate the debilitating symptoms, may prevent life-threatening events and enable better feeding and handling. Establishing the diagnosis of such a relatively benign disorder with a favorable developmental outcome may avoid unjustified extensive investigations or unnecessary treatment, suspecting an ominous progressive neurologic disorder.

Anticonvulsants↗

[Vestibular and oculomotor changes in subjects treated with cisplatin].

Cisplatin is an agent used in the treatment of distinct oncologic diseases. We present the electrooculographic (EOG) findings of 6 patients which were seen at our Department under the diagnosis of chronic toxicity for cisplatin and associated vestibular alterations. Mean of age was 45 years. Three subjects were female (50%). The most frequent pathologic finding was ataxic pursuit tracking (100%). Additionally, spontaneous nystagmus, alterations in positional test, and vestibulo-ocular reflex suppression were also found. These results are discussed and the main literature concerning this matter is reviewed.

Adolescent↗

Effects of passive tilting on capillary filtration in the lower leg in idiopathic dilated cardiomyopathy and after heart transplantation for the same condition.

Abnormal reflex control of the peripheral microvasculature during orthostasis in congestive heart failure (CHF) and after heart transplantation (HT) may cause failure of microvascular homeostasis and peripheral edema. We explored the effect of passive head-up tilt on lower leg capillary filtration measured by strain-gauge plethysmography in 24 patients with CHF, in 20 patients after HT (12 patients with preserved native right atrium, 8 patients without native right atrium), and in 18 controls. We hypothesized that an impaired peripheral microvascular reflex during orthostasis in CHF and HT might allow increased arterial hydrostatic pressure to increase pressure at the capillary level. To identify an impact of changes in arterial hydrostatic pressure, capillary fluid filtration was expressed per mm Hg arterial hydrostatic pressure (capillary filtration coefficient(arterial pressure) [CFC(AP)]) and was measured (1) during elevated venous pressure alone (50 mm Hg venous stasis in supine position), and (2) during elevated hydrostatic pressure at both the venous and arterial side of the vascular tree (head-up tilt with a vertical distance from the right atrium to the strain-gauge of 68 cm of water [50 mmHg]). Elevated venous pressure alone resulted in the highest CFC(AP) in controls (0.79+/-0.28 ml/min x 100 ml mm Hg x 10(-3)+/-SD) versus those with CHF (0.44+/-0.23, p <0.0001) and those after HT (0.54+/-0.22, p <0.01). However, during head-up tilt, CFC(AP) was similar in all 3 groups, because CFC(AP) decreased in controls (to 0.49+/-0.22, p <0.0001), in contrast to unchanged CFC(AP) in those with CHF (0.43+/-0.24) and in those with HT (0.50+/-0.21). HT patients with complete removal of the native right atrium had higher CFC(AP) (0.62+/-0.17) during head-up tilt than patients with preserved native right atrium (0.36+/-0.16, p <0.005). In conclusion, patients with CHF and those after HT have increased capillary filtration to a lesser degree than controls during elevated venous pressure alone. However, during orthostasis this apparent edema-protective mechanism vanishes, probably because of compromised microvascular reflex control. During daily upright activities, this may be one important factor in the edema pathogenesis. The phenomenon is particularly distinct in HT patients without preserved native right atrium.

Adult↗

Relative afferent pupillary defects in optic neuritis.

We measured relative afferent pupillary defects in 105 patients with various stages of optic neuritis. We detected pupillary defects in 96% of acute unilateral cases, 92% of recovered unilateral cases 91.7% of acute cases with evidence of optic neuropathy in the other eye, and 65.8% of recovered bilateral cases. With careful testing, one can find relative afferent pupillary defects in nearly all patients with unilateral optic neuritis and in most patients with bilateral disease. A patient without a pupillary defect after apparent unilateral optic neuritis frequently has evidence of disease in the other eye when visual-evoked potentials are tested.

Evoked Potentials, Visual↗

Effects of methylmercury on the motor and sensory innervation of the rat extensor digitorum longus muscle.

This histochemical study examined the effects of chronic methylmercury (MeHg) intoxication on the motor and sensory innervation of extensor digitorum longus muscles. Light microscopic examination of silver-stained axons in the intramuscular nerve bundles of MeHg-treated rats showed Wallerian-like degeneration and a reduction in the number of nerve fibers. Disrupted axons were predominantly sensory because 22.2% of spindle afferents (Ia) and 90.0% of Golgi tendon organ (Ib) sensory fibers were completely degenerated whereas less than 1% of motor endings were totally destroyed. Partial disruption occurred in the cholinesterase and motor terminals of 13.7% of endplates. Our results demonstrated greater vulnerability of sensory nerves than of motor nerves to MeHg-induced degeneration. Thus, the abnormal reflexes, ataxia, and muscle weakness following MeHg poisoning appear related to reduction of proprioceptive feedback from muscles and tendons in addition to the documented lesions in the central nervous system.

Animals↗

Neonatal ethanol withdrawal: characteristics in clinically normal, nondysmorphic neonates.

Although neonatal withdrawal syndrome is often noted in infants of narcotics addicts, ethanol withdrawal has been reported only among neonates with fetal alcohol syndrome. To examine the possibility that ethanol withdrawal occurs more widely and to identify its characteristics, the behavior of eight neonates born to women who drank a mean of 21 ounces of absolute alcohol per week during gestation was compared with that of two contrast groups: 15 infants whose mothers drank an equivalent amount but stopped in the second trimester, and 29 infants whose mothers never drank. None of the 52 infants had fetal alcohol syndrome, and all were in good health. Neurobehavioral evaluation 3 days postnatally compared the groups for the occurrence of characteristic signs of withdrawal from central nervous system depressants. Whereas there was no difference in the frequency of withdrawal symptoms among infants of mothers who never drank (mean 1.4) or of mothers who stopped drinking (mean 1.8), infants of mothers who continued to drink (mean 4.7) had significantly more tremors, hypertonia, restlessness, excessive mouthing movements, unconsolable crying, and reflex abnormalities. By interfering with state control and interactive behaviors, withdrawal could affect mother-infant bonding as well as the conditions that foster cognitive and social development.

Alcohol Drinking↗

CAG repeat length and disease duration in Machado-Joseph disease: a new clinical classification.

To evaluate the clinical characteristics of Machado-Joseph disease (MJD) with reference to CAG repeat length and disease duration, we analyzed neurologic findings in 108 patients from 84 families. The majority of MJD patients presented with an ataxic gait as the initial symptom. Dysarthria and nystagmus were observed from an early stage. Bulging eyes, muscle atrophy and bradykinesia developed later. Patients with a shorter CAG repeat length or later onset had more frequent involvement of proprioceptive sensory deficit. Incidence of abnormal reflexes, tones, and proprioceptive sensation was not associated with disease duration, but with CAG repeat length. Based on these results, we propose a new clinical classification: type A (juvenile type), with hyperreflexia and dystonia, but without a proprioceptive sensory deficit; type C (adult type), with hyporeflexia and a proprioceptive sensory deficit, but without dystonia; and type B (intermediate type), the remaining patients with a mixed presentation.

Adult↗

Voiding cystourethrography with synchronous measurements of pressures and flow in the diagnosis of subvesical obstruction in men: a radiological view.

Cystourethrography with synchronous urodynamic monitoring was done in 90 men who were diagnosed radiologically as having normal function or bladder outlet obstruction. The separation was based on the caliber of the bladder outlet measured on spot roentgenograms at peak voiding: less than 4 mm. for obstructed and more than 6 mm for normal voiding. The radiological observations are discussed together with the measurements of bladder capacity, residual urine, intravesical pressures and voiding flow rate. The separation between normal men and those with obstruction according to radiologically determined calibers had a strong impact on the distribution of the maximum flow rates. Observations on the onset of micturition suggest that in normal voiding the contracting detrusor meets a simultaneously relaxing bladder neck without interposition of an isometric phase. The patients with subvesical obstruction presented with 2 forms of isometric overload, either as uninhibited contractions or as a variable isometric time lag between the onset of detrusor contraction and opening of the bladder neck. The pre-voiding isometric pressure increase is believed to be an abnormal finding that distinguishes the normal state from obstruction and other disorders. A characteristic decrease in subtracted intravesical pressure at the opening of the bladder neck in patients with uninhibited contractions conceivably indicates that bladder instability is caused by an abnormal reflex originating in the tightened bladder neck. Voiding cystourethrography was an accurate means of diagnosis of subvesical obstruction, especially owing to its ability to quantitate the caliber of a narrowed zone.

Adult↗

The child who has severe neurologic impairment.

The formulation of a diagnosis in a child who has neurologic impairment should not (except in extreme circumstances) be made on the basis of a single finding. For example, the presence of delayed milestones, primitive or exaggerated reflexes, abnormal muscle tone, abnormal posture, abnormal neurologic examination, and temporal course should all be used to make the diagnosis of cerebral palsy. Furthermore, because each condition has a spectrum of disabilities from mild to severe involvement, determination of the child's abilities and impairments should be part of the diagnostic process. Because the child lives in a number of different environments, assessment of the child's functional abilities and evaluation of the environments should be made. Finally, evaluations by specialists in physical therapy, occupational therapy, speech therapy, psychology, special education, and nutrition may be necessary to complete the child's diagnosis. The ability to examine the child over a period of time (i.e., continuity of care) allows the practitioner to provide a more accurate prognosis for future development. It also enables easier diagnosis of the child when he or she is acutely ill. This also allows a more trusting relationship to develop with the parent and enhances the ability to influence the psychosocial conditions that affect outcomes. Finally, all these children should have care coordination (case management) (i.e., someone who can ensure that they receive the care they need, without duplication) across all settings, including home, school, hospitals, and ambulatory facilities.

Child↗

Nursing care of patients with Parkinson's disease.

Nursing interventions for each of the symptoms of Parkinson's disease, muscle rigidity, bradykinesia, tremors at rest and postural reflex abnormalities, are designed to increase the patient's quality of life by minimizing symptoms. Nurses are responsible for planning patient medication schedules to maximize drug effectiveness. Dietary implications include a low-protein regimen for the patient during the day, eliminating foods high in Vitamin B6, high caloric foods, and soft-solid foods offered at frequent feedings. Constipation is addressed by increasing the patient's fiber and fluid intake and by increasing the patient's mobility. Patient mobility is increased when the patient is taught purposeful activities and to concentrate on the way he walks. Communication is facilitated if the patient takes deep breaths before speaking and uses diaphragmatic speech. A telephone receiver which amplifies the patient's voice is also available. Interventions are good only if the patient chooses to implement them; he is the head of the health team planning his care.

Humans↗

Compared study of optokinetic and caloric nystagmus in children with unilateral hyporeflexia and other vestibular disorders.

The authors compare the nystagmus evoked by the caloric test and by two slow and fast optokinetic 'look' stimulations performed in 78 subjects subdivided into two groups and recorded by ENG: group 1 composed of 22 subjects with 'significative' unilateral hyporeflexia and group 2 composed of 56 subjects with important anomalies at the vestibular caloric test. The results can be summarized as follows: 1. the presence of unilateral vestibular hyporeflexia is not exceptional in the child: 22 over 140 cases (15.7%); 2. the comparison between the caloric test and the OKN test in the 22 subjects with significant unilateral hyporeflexia shows: slow and fast TAP homolateral to the side with labyrinthine deficit prevails in ten subjects (45.4%); TAP is inconsistency with respect to the hyporeflexic side (i.e. homolateral in one test and contralateral in the other) in seven cases (31.8%); TAP is contralateral in five cases (22.7%). Within the same group, STAP varies according to cases. 3. In group II, TAP values at the OKN test overlap considerably with respect to the caloric test (18 cases with a total TAP prevailing on the right side, 32.2%; 19 cases with divergent TAP, 33.9%; 19 cases with total TAP prevailing on the left side, 33.9%). 4. The data shown in group 1 with significant vestibular hyporeflexia can be correlated to the time elapsed between the last electronystagmography and that performed soon after disease onset. Since for ENG performed some days after vertigo onset (even though clinical examination is negative) shows a concordance of OKN TAP and the hyporeflexic side (as the mechanisms of central compensation are still being developed) and then when these mechanisms improve with time, an inconsistency of OKN TAP and hyporeflexic side and finally a contralaterality. We might rely on the comparison between OKN TAP and caloric test as a finding of the time distance from the vertigo onset (when unknown) and a rough prognostic sign. The only case of vestibular neuritis by us followed in time seems to confirm our assumption.

Adolescent↗

The significance of Babinski signs in children with head trauma.

A prospective study was undertaken to investigate the significance of Babinski signs in children with head trauma. Thirty-eight children between the ages of 1 and 15 were studied. Twenty-four of the children were admitted to the hospital for observation; 14 were sent home, with 24-hour follow-up in the pediatrics clinic. Of the 24 children admitted, six were believed to have altered CNS status or other focal findings on examination in the emergency department, and 18 were judged by two independent observers to have Babinski signs, either unilateral or bilateral, as their only neurological finding. Twelve hours after admission, all 18 children who had been admitted on the basis of Babinski signs alone had downgoing toes and they continued to have Babinski signs. The isolated presence of Babinski signs in a child with a history of head injury is not indicative of poor neurological outcome. An otherwise asymptomatic child who presents with a history of head injury and a solitary finding of Babinski sign(s) may be observed safely at home rather than being admitted to the hospital for observation.

Adolescent↗

Hyperekplexia-like syndromes without mutations in the GLRA1 gene.

Hyperekplexia (MIM: 149400), or startle disease, is an autosomal dominant neurological disorder characterized by an extreme generalized stiffness immediately after birth, normalizing during the first years of life. Other features of this disorder are excessive startle reactions to unexpected, particularly auditory, stimuli together with a short period of generalized stiffness during which voluntary movements are impossible. Linkage analysis mapped a gene for this disorder to chromosome 5q33-q35. Subsequently, mutations in the GLRA1 gene encoding the alpha 1-subunit of the glycine receptor proved to be causally related to the disease. In the present study, mutation analysis of all exon and flanking intron sequences of this gene was performed in sporadic patients and their parents. Moreover, a branch of the original Dutch hyperekplexia family with a very severely affected individual was screened for an additional mutation in the GLRA1 gene. Except for two polymorphisms, of which one results in an amino acid change, no potentially disease causing mutations were found in the alpha 1-subunit of the glycine receptor. Together with haplotype analysis these results exclude a recessive inheritance or new mutation etiology in these hyperekplexia-like syndrome and emphasize that hyperekplexia-like syndromes can be caused by other genetic factors. The involvement of other genes encoding subunits of the functional glycine receptor complex has not been excluded.

Adult↗

[Electrophysiologic study of Achilles areflexia of aged subjects].

The elderly often presents loss of Achilles tendon reflex. In this electrophysiological study the authors examined 19 patients with loss of the ankle jerk and with no known etiology of peripheral neuropathy. They compared them with 24 subjects who had no areflexia. The study included measurement of the H reflex, of the tendon reflex, of the conduction velocity of Ia fibers and of motor conduction velocity of the tibial nerve. Amplitude of H and tendon response and H/M ratio are diminished in the areflexic group. In 4 cases the H reflex is preserved while the tendon reflex is abolished. The meaning of these impairments is discussed.

Achilles Tendon↗