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At least 1,171 records · Page 65Linked to original sources

Cardiovascular reflexes in the neurological evaluation of impotence.

Testing of the autonomic cardiovascular reflexes (single breath beat-to-beat variation and orthostatic blood pressure change) was performed in 542 patients with erectile dysfunction who were grouped according to aetiological factors and their response to visual sexual stimulation and the papaverine test. Age-related normal values for autonomic tests were determined in the group of 124 patients with psychogenic impotence. Abnormal cardiovascular reflexes were associated with ageing and organic impotence, indicating autonomic dysfunction as a major contribution to impotence. Single breath beat-to-beat variation, as a simple non-invasive test, is useful in the clinical evaluation of impotence, especially in patients with diabetes and a history of alcohol abuse.

Aging↗

Eye movement abnormalities in a family with cerebellar vermian atrophy.

We report the oculographic findings in a family whose members have a dominantly inherited, early onset, non-progressive syndrome which includes spontaneous upbeating nystagmus and mild cerebellar ataxia associated with cerebellar vermian atrophy seen on magnetic resonance scanning. Eye movements recorded with electro-oculography and a magnetic scleral search coil revealed severely impaired horizontal and vertical smooth pursuit, optokinetic nystagmus and visual-vestibular interaction, symmetrical horizontal but asymmetrical vertical vestibulo-ocular reflex, and normal saccades. The midline cerebellum appears to be essential for both horizontal and vertical visual tracking and visual modification of the vestibulo-ocular reflex in man.

Adult↗

Abnormalities on the neurological examination and EEG in young children with pervasive developmental disorders.

This study examined the nature and frequency of neurological and EEG abnormalities in 60 young children (ages 2-6 years) with pervasive developmental disorders. A number of standard neurological functions could not be adequately assessed due to the young age of the children and/or limited comprehension and cooperation. The most common neurological deficits were hyporeflexia, stereotypies, and hypotonia. EEG abnormalities were identified in 32% of the children while only two children were known to have clinical seizures. The frequency of cases with hypotonia or hyporeflexia was more common than in older children with this diagnosis. Results also indicate that EEG abnormalities are common in this young population but clinical seizures are rare, confirming other studies.

Autistic Disorder↗

Pseudotumor cerebri, spinal and radicular pain, and hyporeflexia: a clinical variant of the Guillain-Barré syndrome?

A 5-year-old patient developed pseudotumor cerebri, spinal and radicular pain, and hyporeflexia. Ataxia and multiple cranial nerve involvement also were evident. With the exception of residual hyporeflexia and the abnormalities present in the electrophysiologic studies, the patient's prompt recovery suggested the diagnosis of Guillain-Barré polyradiculoneuritis.

Child, Preschool↗

Sympathetic and parasympathetic baroreflex dysfunction in familial dysautonomia.

OBJECTIVE: To assess the possible abnormalities in the baroreflex modulation of both the heart and the arterial vasculature, in order to better evaluate the role of baroreflex abnormalities in the generation of the cardiovascular symptoms and complications affecting the familial dysautonomia (FD) patient. METHODS: Twenty-one FD patients and 22 controls underwent 3 minutes of passive head-up tilt (HUT) and baroreceptor stimulation by means of sinusoidal neck suction (NS; 0 to -30 mm Hg; 0.1 Hz [LF] and 0.2 Hz [HF]). Respiration was maintained constant during NS at 15 breaths/minute. The authors monitored RR-intervals (RRI), blood pressure (BP) (Colin), and respiration. NS induced changes of RRI and BP were determined by spectral analysis. RESULTS: HUT showed orthostatic hypotension without compensatory tachycardia in FD patients but not in controls. LF-NS increased LF power of RRI and BP and HF-NS increased HF power of RRI in controls, but not in FD patients. CONCLUSIONS: Familial dysautonomia patients have a widespread baroreflex abnormality, involving both the efferent sympathetic arm on the resistance vessels, and the sympathetic and parasympathetic efferent arms on the heart. Therefore, the abnormalities in the control of blood pressure-i.e., supine hypertension, orthostatic hypotension, blood pressure lability-and heart rate-i.e., bradyarrhythmias-are likely due to baroreflex abnormalities.

Adolescent↗

Tremor in ostensibly normal elderly people.

Tremor in ostensibly normal people, aged 70-91, was assessed clinically and electrophysiologically with the goal of estimating the prevalence of abnormal tremor. Fifty men and 50 women, mean age 76.0 +/- 4.7 yrs, were recruited through advertisements for healthy volunteers (n = 50 "biased" control subjects) and from the spouses of patients referred to us for dementia or Parkinson's disease (n = 50 "unbiased" control subjects). All participants were interviewed and examined by the author. Tremor was assessed quantitatively with rating scales, triaxial accelerometry, electromyography, a digitizing tablet, and spectral analysis. Twenty-three people (23%) were judged clinically to have mildly abnormal tremor resembling mild essential tremor. Twelve people with abnormal tremor belonged to the biased group and 11 were in the unbiased group. The clinical diagnosis of abnormal hand tremor correlated well with the presence of motor unit entrainment in the forearm EMG and with writing or drawing tremor that was measurable with a digitizing tablet. Only 10 of 23 people with abnormal tremor were aware of their tremor, and none had been diagnosed previously by a physician. Nine of 77 people (11.7%) with normal tremor had a parent or sibling with possible essential tremor, and five of the 23 people (21.7%) with abnormal tremor had this family history. Mild undiagnosed tremor, resembling essential tremor, is common in this age group.

Aged↗

Severe fetal acidemia: neonatal neurologic features and short-term outcome.

The objectives of this study were to determine if infants delivered with severe acidemia (cord umbilical arterial pH < 7.0) had short-term neurologic effects and whether infants with persistent bradycardia who received cardiopulmonary resuscitation (CPR) in the delivery room would be at greatest risk for subsequently developing neonatal seizures. Forty-seven infants (39 term, 8 preterm) delivered with severe fetal acidemia were studied. The mean (+/- S.D.) for pH, PaCO2, and base deficit for the 47 infants was 6.86 +/- 0.11, 97 +/- 22 mm Hg, and -17 +/- 4, respectively. Labor complications were common and included placental abruption in 8, ruptured uterus in 4, cord prolapse in 3, fetal heart rate decelerations in 12, and other (n = 14). Most infants were delivered via emergency cesarean section (n = 29). Delivery room interventions included oxygen and bag/mask ventilation only (n = 20) and intubation and ventilation (n = 22); 7 of 22 infants received CPR and epinephrine for persistent bradycardia (heart rate < 80 beats/min despite ventilatory support). Five infants required no intervention. Eight infants (17%) had seizures; 6 of these infants received CPR in the delivery room. Short-term outcomes were abnormal in 7 of 8 infants (i.e., death in 5, abnormal neurologic examination at discharge in 2). In 39 infants without seizures, 32 had transient neurologic abnormalities (i.e., irritability, hyperreflexia, proximal hypotonia) which resolved by discharge, and 2 had abnormal and 5 normal examinations.(ABSTRACT TRUNCATED AT 250 WORDS)

Acid-Base Equilibrium↗

A case of brainstem encephalitis with CT scan abnormality mimicking Fisher syndrome.

A 7-year-old boy with brainstem encephalitis is described. He was drowsy in the acute phase. CSF showed pleocytosis without elevated protein. EEG showed diffuse slow wave activity during wakefulness. CT scan disclosed a low density abnormality in the basal ganglia area, which disappeared as the patient recovered. The clinical signs, CT scan abnormality and EEG findings suggest that this case is not a variant of the Guillain-Barré syndrome but is brainstem encephalitis mimicking Fisher syndrome.

Ataxia↗

[Recent outcome of bladder augmentation using deepithelialiezed segment of small intestine lined with urothelium].

OBJECTIVE: To evaluate urodynamically and clinically bladder augmentation using double-deepithelialized segment of the small intestine lined with urothelium to analyse the recent outcome to treatment of hyperreflexia neurogenical bladder. METHODS: 25 patients with neurogenical bladder underwent bladder augmentation using deepithelialized segment of the small intestine lined with urothelium (4 - 14 years old, 16 males and 9 females) from October 1998 to October 2000. 21 patients were followed up. Voiding cystoureterography and urodynamic and clinical evaluation were performed, and serum electrolyte, urea nitrogen and creatinine were tested before and 6 months to 2 years after operation. RESULTS: After operation, bladder volume, maximum urine flow rate and compliance were increased, but residual urine/bladder volume and detrusor pressure decreased. Uninhibitory contraction decreased in 15 patients. In 12 patients associated vesicoureteral reflux, clinical evaluation revealed poor outcomes before operation, but excellent (4 patients), good (9), effective (4), poor outcomes (4) after operation. Seven patients had urinary sensation recovered. Vesicoureteral reflux decreased in 9 patients. There were no electrolyte unbalance and abnormal renal function in all patients. CONCLUSIONS: Bladder augmentation using double-deepithelialized segment of the small intestine lined with urothelium shows a better result currently.

Adolescent↗

["Abnormal" neurologic findings in the "healthy" elderly].

Neuropathological changes and clinical neurological findings of the nervous system of healthy, old people (more than 80 years old) are referenced according to the literature. In addition, the results of a clinical neurological examination of 86 healthy senior citizens (average age: 83.6 years) are demonstrated. The respective findings are summarized in a table according to their frequency. The involution of the human nervous system with age results neuropathologically and clinically-neurologically in damage to the frontal lobe (disinhibition of archaic reflexes), the extrapyramidal system (Parkinsonian walking pattern), the posterior tract of the spine (unsteadiness of walking), and the peripheral nervous system (e.g. loss of the achilles tendon reflex). The Parkinsonian-like walking pattern of the healthy old-aged human is thought to be a disinhibition of an archaic pattern of posture and movement. Brief suggestions for differential diagnosis and therapy are presented.

Aged↗

A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old.

The Wobbler mouse possesses an inherited autosomal recessive form of motoneuron disease. The most characteristic abnormality is the degeneration of motoneurons, mostly in the cervical spinal cord, and in the brain stem cranial motor nuclei. The underlying pathology shows up as symptoms that are only detectable confidently around the time of weaning (age 3 weeks). We now report a new method designed to identify presymptomatic Wobbler mice by behavioral and statistical approaches. We measured body weight, righting reflex (RR) and gender to examine whether these parameters have an impact on the status of the disease before age 3 weeks. Using a total of 341 NFR/wr strain pups, we found a strong association between RR and the Wobbler disease status (p<0.0001) between postnatal days 3 to 7, and achieved greater than 97% correct classification of Wobblers. Therefore the measurement of RR allows the early detection of the affected Wobbler (wr/wr) mice with a minimum of error. This method has been used in our laboratory for immunocytochemical studies that show the early sprouting of immunoreactive serotonin and peptidergic fibers in the cervical spinal ventral horn by postnatal days 7 and 12 respectively. The early detection of Wobbler mice thus facilitates significant new understanding regarding the pathogenesis of motoneuron disease. We can now examine potentially therapeutic approaches which may be more effective than when administered in the symptomatic weanlings (work in progress).

Age Factors↗

A developmental approach to physical disability in childhood.

The reasons for advocating a developmental approach to physical disability in childhood are stated. Relevant characteristics of the developmental periods--antenatal, neonatal, early infancy, late infancy, preschool and school age--are described. Diagnosis, treatment and prognosis are then discussed in this developmental context.

Abnormalities, Multiple↗

Pathophysiological mechanisms of fibromyalgia.

Pain related to fibromyalgia may consist of a complex interaction of nociceptive, neuropathic, dysregulatory central nervous system and psychosomatic mechanisms. Nociceptor pain is based on the excitation of nervous sensors specialized to signal potentially harmful stimuli, i.e., the nociceptors. Metabolic deficiencies in muscle and neurogenic inflammation induced by the release of substance P and other neuropeptides from the peripheral nerve endings may result in chemical sensitization of nociceptors and an ensuing hyperalgesia particularly present in tender points. Neuropathic pain is due to pathological mechanisms within nerve cells and fibers in the peripheral and central nervous system. Pathophysiology may be related to compression (such as in the carpal tunnel syndrome or a vertebral disk herniation) or regeneration of nerves, resulting in ectopic impulse discharges and disturbances of axonal transport. The ensuing neuronal hyperexcitability and trophic changes induced by a disturbed axonal transport system may be major factors of pain in fibromyalgia. Dysregulatory pain denotes pain maintained by dysfunction of efferent control loops. Thus, if spinal motoneuron output results in excessive tension of postural muscle, nociceptors in muscles, tendons and joints might become more excited. Persistent abnormal spinal reflex transmission due to, e.g., peripheral trauma or inappropriate postural habits may result in a vicious circle between muscle hypertension and pain. Similarly, a defective sympathetic control may result in disturbed microcirculation and nociceptor excitation (e.g., in sympathetic algodystrophy). Many symptoms of pain in fibromyalgia (trigger points, pain referral, pain associated with muscle spasm or neurogenic joint immobilization) can be attributed to abnormal control mechanisms in a complex cybernetic system.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗

Role of the brain and sensory pathways in gastrointestinal sensory disorders in humans.

Several features of the irritable bowel syndrome (IBS) suggest involvement of the emotional limbic system in the brain. Abnormalities which upregulate afferent (sensory) signal intensity anywhere in this system, from the gastrointestinal tract to the brain, could induce hypersensitivity, leading to the pain and discomfort that characterise IBS and other functional disorders. Functional gastrointestinal disorders are likely to be heterogeneous given the complexity of the afferent system, and a number of different perturbations are possible. Intestinal hypersensitivity to pain and discomfort and associated reflex alterations in motility might explain the symptoms of functional bowel diseases.

Animals↗

The clinical spectrum of Friedreich's ataxia in German families showing linkage to the FRDA locus on chromosome 9.

The clinical features of Friedreich's ataxia are described and reevaluated in a group of 14 German patients from 9 independent families. In contrast to previous studies, demonstration of linkage to the Friedreich's ataxia locus (FRDA) on chromosome 9p allowed confirmation of the genetic homogeneity of the disease in the patients under study. Marked variability within families was observed for age of onset of the disease (4-24 years) and for age of becoming wheelchair bound (17-37 years). Electrocardiographic changes were present in all and echocardiographic changes in 50% of the patients. Pathological changes of visual evoked potentials were detected in only 50% of the patients while brainstem auditory evoked potentials and somatosensory evoked potentials were always abnormal.

Adolescent↗

Features of the Guillain-Barré syndrome in mice following intraperitoneal injection of patient serum.

Intraperitoneal injection of serum from a patient with the Guillain-Barré syndrome (GBS) produced GBS-like signs in mice: inadequate respiration and weakness in the legs. We studied the clinical, electrophysiological and pathological features of these mice. Three groups of three mice were injected with patient serum from days 6, 10 and 15 after onset of neurological symptoms. GBS-like signs in mice were observed only with serum from day 6 and improved within 48 h. When serum was frozen and thawed more than once no signs were seen. Electrophysiological measurements of the sciatic nerves of injected and control mice were done before and after serum injection. Five days after injection of patient serum of day 6, the mice showed a significant decrease in the ratio between CMAP amplitude from proximal and distal stimulation and increase in H-M interval from proximal stimulation. These electrophysiological changes returned to normal within 12 days. The sciatic nerve showed no morphological abnormalities. Our results indicate that the observed GBS-like signs in mice are caused by peripheral nerve dysfunction.

Adult↗

Neurologic findings in neonates with intrauterine cocaine exposure.

To determine the effects of prenatal cocaine exposure on intrauterine growth and neurologic function, 30 term infants were evaluated prospectively: 14 cocaine-exposed and 16 unexposed. Cocaine-exposed infants had significantly lower mean birth weights, birth lengths, and head circumferences than urine-negative controls. Neurologic abnormalities among cocaine-exposed newborns included hypertonia of any type (86% vs 33%), axial hypertonia (79% vs 19%), plantar extension (46% vs 19%), and coarse tremor (57% vs 12%). The degree of hypertonia was sufficiently severe to warrant a diagnosis of "hypertonic tetraparesis" in 64% of cocaine-exposed and 12% of unexposed infants (P = .007). This diagnosis was highly correlated to small head size (r = 0.48; P = .01). Persistent tonic downward gaze was evident in two neurologically abnormal cocaine-exposed newborns. Gaze abnormalities improved slightly over the next 3 weeks of observation. One such infant re-evaluated at 6 months of age revealed resolution of tonic downward gaze and neurologic findings. We conclude that prenatal cocaine exposure is associated with tone and movement abnormalities in newborn infants.

Cephalometry↗