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PubMed1988

Permanent cardiac pacing for brady-arrhythmia.

The indications for and technology associated with permanent cardiac pacing have undergone major changes during the past decade. In contrast to the 1970s, most patients now receive cardiac pacing devices subsequent to the diagnosis of sick sinus syndrome. However, since this condition is prevalent in elderly patients, it is crucial that indications for pacing be critically evaluated and pacing recommended only for those with documented symptoms or marked abnormalities indicative of high risk for a subsequent cardiac event. This is particularly important because pacing has not been shown to influence survival in these patients. The technological advances in pacing devices during the last ten years now provide many desirable options. As a result, available pacemakers range from very simple to highly complex; accordingly, they have a wide range of prices. It is more important than ever to carefully select the appropriate pacing device for a given patient, since its cost, longevity, and required follow-up may differ greatly.

Bradycardia↗

PubMed2000

Tilt training: a treatment for malignant and recurrent neurocardiogenic syncope.

The treatment of neurocardiogenic syncope is insufficient in many cases. We hypothesized that the repeated exposure of the cardiovascular system to orthostatic stress could have a therapeutic effect on the regulation of cardiovascular reflex mechanisms. We have started a program of tilt training for heavily symptomatic patients. After hospital admission, patients were tilted daily (60-degree inclination), until syncope, or until a maximum of 45-90 minutes. The patients were instructed to continue a program of daily tilt training at home: two 30-minute sessions of upright standing against a vertical wall. No medication was prescribed. A total of 260 tilt table sessions were performed in 42 patients. The first tilt test was positive after 21 +/- 13 minutes. The syncope was cardioinhibitory in 14 cases, vasodepressor in 19, mixed in 9. At the time of hospital discharge, 41 patients could support 45 minutes of head-up tilting. After a mean follow-up time of 15.1 (SD 7.8) months, 36 patients remained completely free of syncope. Syncope still occurred in one patient and presyncope in four patients. One patient died from an extensive myocardial infarction. The abnormal autonomic reflex activity of neurocardiogenic syncope can be remedied by a program of continued tilt training without the administration of drugs. This new treatment has proven to be effective for the vasodepressor and the cardioinhibitory type of syncope.

Adolescent↗

PubMed1984

The hemi 3 syndrome. Hemihypertrophy, hemihypaesthesia, hemiareflexia and scoliosis.

Three unrelated girls presented with a developmental syndrome of hypertrophy involving half or a quadrant of the body and not involving the face. The appearance was one of inappropriately large size of the affected side rather than contralateral atrophy. On the larger side, there was hypertrophy of muscle and increased power as well as an increase in diameter, but not in length, of long bones. There was areflexia and decreased pain and temperature sensation on that side. The patients also had progressive scoliosis and foot deformities on the enlarged side. One patient had a lumbar myelomeningocoele , and all 3 had a family history of neural tube closure defects. EMG, nerve conduction studies, EEG, skull x-rays, PEG, and cerebral CT scans were normal. Myelography did not demonstrate an enlarged cord, and in particular there was no evidence for syringomyelia. Chromosome studies revealed normal karyotypes. Sex chromatin was female on both sides in one patient. A defect of the dorsal lip of the neural tube or the neural crest is postulated to explain the abnormality. The association with closure defect in one patient and a positive family history of other neural tube defects in all 3 patients suggests that the developmental defect occurs at an early embryonic stage. Recognition of the syndrome is important. It can be distinguished clinically from hemiatrophy of cerebral origin. The neurological abnormalities are static, but the scoliosis is progressive and requires correction. The condition is associated with an increased prevalence of neural tube closure defects in the family, and forms part of a spectrum of genetically and embryologically related CNS malformations with multifactorial inheritance. Probands, parents, siblings and parents' siblings should be counselled that the risks of spina bifida and anencephaly in their offspring are the same as those in relatives of probands with classical neural tube defects, and should be offered prenatal diagnosis.

Adult↗

PubMed1998

[Postural hypotension with non-neurogenic triggers in the elderly].

INTRODUCTION: Orthostatic hypotension (OH) symptoms are the most frequent reason for patients to undergo an autonomic examination. The incidence of these symptoms is greater in two groups: young people with neurally mediated syncope, and adults older than 60 years, all who have normal results in the EKG and EEG test. The baroreflex afferents induce a continuous blood pressure adjustment through the heart rate variation. There are functional changes with aging which reduce this baroreflex sensitivity and therefore the accommodation to the haemodynamic requirements is frequently impaired. A number of no neurogenic triggers can be implicated in the development of OH symptoms such as electrolytic disorders, arrhythmia with low stroke volume and drugs side effects. MATERIAL AND METHODS: To study the influence of these factors in the elderly suffering from OH, we reviewed patients older than 60 years sent to our laboratory during the last three years. In 40 out of 207 cases we found the existence of some of the above mentioned factors as possible trigger of the symptoms, since no autonomic abnormality was present. After arrhythmia correction, in two cases, and the pharmacologic agent suppression, in four more patients, symptoms disappeared and no OH was found in a second tilting test. OH is a frequent problem in the elderly and requires a careful review of the numerous causes that could contribute to develop the symptoms. CONCLUSIONS: To take the proper therapeutic decision, a long term monitoring seems to be necessary for the main parameters: blood pressure and EKG.

Aged↗

PubMed2006

Anhidrosis: a clue to an underlying autonomic disorder.

We describe a patient with Ross syndrome who had the classic triad of segmental anhidrosis, tonic pupils and hyporeflexia. Dilute pilocarpine (0.1%) instillation in both eyes revealed parasympathetic denervation hypersensitivity. There was evidence of cardiac dysautonomia in the form of a decrease in heart rate variability with deep breathing and an abnormal Valsalva response. A thermoregulatory sweat test showed the presence of sweating in the right cheek, neck and upper one-third of the left arm and shoulder only. Sympathetic skin responses were absent in the affected segments of the upper limb. Ross syndrome is a degenerative disorder that progressively involves different fibre populations, starting with autonomic fibres and then involving the unmyelinated and myelinated sensory fibres. A careful clinical examination and simple bedside autonomic tests can confirm the diagnosis.

Adult↗

PubMed1986

Neurological manifestations in xeroderma pigmentosum.

Xeroderma pigmentosum is an unusual neurocutaneous disorder. Recent studies have classified patients with xeroderma pigmentosum into 10 groups by somatic cell hybridization methods. In this report we describe 32 patients with Group A xeroderma pigmentosum, including 1 patient with an atypical case, who were assessed for neurological complications. Of these patients, 17 had microcephaly, 13 short stature, and 21 mental retardation. In patients over 7 years of age, sensorineural deafness and spinocerebellar signs such as nystagmus, dysarthria, tremor, and ataxia were frequently observed; no patients below 7 years of age had such neurological complications. Electroencephalographic studies revealed abnormal slow and low voltage background activity. Two patients had focal abnormal discharges, one of whom developed versive seizures. Cranial computed tomographic scans revealed abnormalities, including ventricular dilatation, cerebral atrophy, cerebellar and brainstem atrophy, and cranial bone thickening. A patient with an atypical case of Group A xeroderma pigmentosum had less skin and neurological involvement, and higher levels of postultraviolet colony-forming ability and host cell reactivation than did a typical Group A case. It is possible that these less severe cytological findings are responsible for the less severe skin lesions and neurological complications noted clinically.

Adolescent↗

PubMed2000

Hemihyperaesthesia and hyperresponsiveness resembling central pain syndrome in a dog with a forebrain oligodendroglioma.

A 4-year-old male Boxer was presented with neurological signs referable to a right forebrain lesion that was confirmed with computed tomography. Whilst characteristic signs of a unilateral forebrain lesion were observed, the dominant and striking finding was a right-sided hemisensory disturbance characterised by hyperaesthesia and hyperresponsiveness. Necropsy revealed a gelatinous mass confined to the right forebrain that was identified histologically as an oligodendroglioma. The lesion was centred on the internal capsule and involved ventral frontal and temporal lobes and the ventrolateral thalamus, including lateral and medial parts of the ventrocaudal nuclear region (ventrobasilar complex) of the thalamus. On clinical and neuroanatomical grounds, the case exhibited features in common with central pain syndrome in human patients with thalamic lesions. These included a somatosensory disorder of hyperaesthesia affecting an entire side of the head and body, behavioural manifestations consistent with spontaneous pain and a lesion involving the ventrobasilar complex. Of interest, the hemisensory abnormality was ipsilateral to the lesion, contrasting with central pain in humans, in which clinical signs are contralateral to analogous lesions. It is suggested that species-specific differences in spinal cord organisation of pain pathways, particularly the greater bilateral projection of nociceptive afferents to thalamic relay nuclei in carnivores, may account for this disparity. Notably, central pain is rare in human patients with brain tumours, even those affecting the thalamus, and this may also be the case in dogs.

Animals↗

PubMed2003

Abnormal habituation of 'nociceptive' blink reflex in migraine--evidence for increased excitability of trigeminal nociception.

We studied the habituation of the 'nociceptive' blink reflex (nBR) in 15 healthy subjects and 17 migraine patients interictally as well as during unilateral migraine headache within six hours of onset and after treatment. In healthy volunteers the mean regression coefficient (MRC) was - 3.9 following right sided and - 4.9 left sided stimulation. This equals an amplitude loss of 19.5% (5 x -3.9) and 24.5% (5 x -4.9), respectively, across five consecutive sweeps. An augmentation of nBR responses was found in migraine patients interictally: MRC = 3.3 following stimulation of the headache side (HA) and MRC = 4.0 of the non-headache side (non-HA). The differences were statistically significant (anova: d.f. = 1, F = 25.8, P < 0.001). During the migraine attack MRCs were negative both before (-5.0, HA and - 4.0, non-HA) and after treatment (-2.6, HA and - 1.9 non-HA) and significantly differed from those outside the migraine attack (anova: d.f. = 2, F = 12.4, P < 0.001). The demonstrated lack of habituation of the nBR responses indicates an abnormal trigeminal nociceptive processing in migraine patients outside the migraine attack.

Adult↗

PubMed1993

[A case of HTLV-I associated myelopathy with flutter-like oscillation].

We reported a 37-year-old woman showing HTLV-I associated myelopathy (HAM) with flutter-like oscillation of the eyes. She had a 15-year history of spastic paraplegia and noticed visual quaking at the age of 37 years. On neurological examination, she showed spasticity in both lower extremities, hyperreflexia in the extremities, neurogenic bladder, and transient, horizontal and pendular ocular oscillation. Anti HTLV-I antibody titers in serum and cerebrospinal fluid were significantly elevated. Brain CT scan revealed no particular abnormalities. In electrooculography, the oscillation consisted of 2 to 4 pendular cycles, having an amplitude of 3 to 10 degrees, and lasting 0.3 to 0.8 second. It occurred immediately after change of fixation at the lateral gaze. This flutter-like oscillation has been thought to result from disorders of cerebellum and its peduncles since the first description by Cogan (1954). Cerebellar signs, although infrequent, have been reported to occur in the patients with HAM. From the present study, flutter-like oscillation should be added as one of cerebellar signs which can occur in patients with HAM.

Adult↗

PubMed2003

A cohort study of health effects of human T-cell lymphotropic virus type I infection in Jamaican children.

OBJECTIVE: Human T-cell lymphotropic virus type I (HTLV-I) infection in childhood is believed to play an important role in risk for adult T-cell leukemia/lymphoma. Although HTLV-I is known to be associated with infective dermatitis in childhood, other HTLV-I-associated morbidity in children has not been well studied. We sought to determine the HTLV-I-associated health effects in Jamaican children. METHODS: We compared incidence rates of several health outcomes in 28 HTLV-I-infected and 280 uninfected children clinically followed from age 6 weeks to a maximum of 10 years. Cox proportional hazards regression analysis was used to analyze these prospectively collected data, adjusting for confounding effects of other variables as necessary. RESULTS: HTLV-I-infected children had significantly higher incidence rates of seborrheic dermatitis (rate ratio [RR] = 4.8, 95% confidence interval [CI] = 1.9-12.5), eczema (RR = 3.1, CI = 1.2-7.9) and persistent hyperreflexia (RR = 3.7, CI = 1.6-8.2). Additionally, HTLV-I infected children had increased rates of severe anemia (RR = 2.5, CI = 0.8-7.9) and abnormal lymphocytes (RR = 2.4, CI = 0.8-7.6) that were of borderline statistical significance. CONCLUSIONS: Our study suggests that HTLV-I-associated skin diseases of childhood may include seborrheic dermatitis and eczema. Additionally, these data suggest that persistent hyperreflexia of the lower limbs may be an early sign of HTLV-I-associated neurologic involvement in children. Expansion and continued clinical observation of this cohort would be valuable.

Anemia↗

PubMed1984

Impairment of cardiopulmonary baroreflexes in Dahl salt-sensitive rats fed low salt.

Abnormalities in neural circulatory control contribute to salt-induced hypertension in Dahl sensitive (DS) rats. This study tested the hypothesis that there is impairment in cardiopulmonary baroreflex function in prehypertensive DS rats. The study was performed in DS and Dahl resistant (DR) rats fed low-salt diet. Arterial baroreceptors were denervated. Sympathetic activity was recorded from the splanchnic nerve during stimulation of cardiopulmonary baroreceptors with volume expansion (iv dextran). Resting mean arterial pressure averaged 93 +/- 6 (SE) in DS vs. 98 +/- 5 mmHg in DR rats. Resting left ventricular end-diastolic pressure (LVEDP) was 13.5 +/- 1.0 in/DS vs. 11.4 +/- 0.9 mmHg in DR rats. Volume expansion with the same amount of dextran caused greater increases in LVEDP in DS (+13 +/- 1 mmHg) than DR (+10 +/- 1 mmHg) but less inhibition of sympathetic activity (-40 +/- 4 vs. -50 +/- 2%) in DS compared with DR rats, respectively. Cardiopulmonary baroreflex gain calculated as percent inhibition of sympathetic activity divided by increases in LVEDP was -3.2 +/- 0.2 in DS vs. -4.9 +/- 0.6%/mmHg in DR rats. Reflex responses to dextran were abolished by vagotomy. Volume expansion also induced increases in mean arterial pressure. These were/greater in DS than DR rats (+43 +/- 4 vs. +28 +/- 5 mmHg, respectively) before vagotomy but were similar in the two groups after vagotomy. The distensibility (delta volume/delta pressure) of the left atrium was similar in DS and DR rats. We conclude that prehypertensive DS rats have impairment of the cardiopulmonary baroreflex.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1987

Enhanced vagal activity and normal arginine vasopressin response in carotid sinus syndrome: implications for a central abnormality in carotid sinus hypersensitivity.

The relation between arginine vasopressin and vagal activity in carotid sinus syndrome was studied in 10 patients and 17 age matched controls using head up tilt as a stimulus. Of the controls, seven had unexplained syncope and 10 were healthy elderly subjects with no previous history of syncope. Subjects were studied supine for 45 min and thereafter during 120 min head up tilt to 40 degrees. Phasic arterial pressure and heart rate were monitored throughout. Serum was sampled at frequent intervals to measure arginine vasopressin, noradrenaline, and adrenaline concentrations. Seventy per cent of carotid sinus patients had vasovagal syncope at (mean(SD)) 25(4) min after tilt compared with 43% of subjects with unexplained syncope and one healthy elderly control. The maximum (mean(SD)) fall in systolic blood pressure and heart rate was 70(20) mmHg and 20(7) beats.min-1 (p less than 0.001 and p less than 0.01 respectively). Arginine vasopressin, noradrenaline, and adrenaline concentrations rose significantly in syncopal subjects (p less than 0.001, p less than 0.01, and p less than 0.05 respectively). Changes in systolic blood pressure, heart rate, and hormone concentrations were similar for patients with carotid sinus syndrome and control subjects. For those who completed the tilt period without the development of symptoms, systolic blood pressure and arginine vasopressin and adrenaline concentrations were unchanged, whereas noradrenaline concentrations and heart rate rose significantly. Vasovagal activity is thus appreciably increased in carotid sinus syndrome. Furthermore, the afferent limb of the carotid sinus reflex appears to be intact in patients with carotid sinus syndrome since the pattern of arginine vasopressin release was not different from controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1994

Acquisition and maintenance of the simplest motor skill: investigation of CNS mechanisms.

The spinal stretch reflex (SSR), or tendon jerk, is the simplest behavior of the vertebrate nervous system. It is mediated primarily by a wholly spinal, two-neuron pathway. Recent studies from several laboratories have shown that primates, human and nonhuman, can gradually increase or decrease the size of the SSR when reward depends on such change. Evidence of this training remains in the spinal cord after all supraspinal influence is removed. Thus, the learning of this simple motor skill changes the spinal cord itself. Comparable spinal plasticity probably plays a role in the acquisition of many complex motor skills. Intracellular physiological and anatomical studies are seeking the location and nature of this spinal cord plasticity. Attention focuses on the most probable sites of change, the group Ia afferent synapse on the alpha motoneuron and the motoneuron itself. Results to date indicate that modifications are present at several places in the spinal cord. Current clinical studies are investigating the use of spinal cord adaptive plasticity as a basis for a new therapeutic approach to spasticity and other forms of abnormal spinal reflex function that result from spinal cord injury, stroke, or other neurological disorders. In the future, understanding of spinal reflex plasticity may lead to development of improved training methods for a variety of motor skills.

Adaptation, Physiological↗

PubMed2004

Plantar nerve AP and skin biopsy in sensory neuropathies with normal routine conduction studies.

OBJECTIVE: To assess the medial plantar nerve action potential (NAP) and skin biopsy in the evaluation of suspected distal sensory neuropathies (SN) with normal routine nerve conduction studies (NCS). METHODS: A total of 110 consecutive patients with suspected distal SN and normal routine NCS underwent medial plantar NAP testing and punch skin biopsy. Patients were clinically stratified as having pure small fiber sensory neuropathy (SFSN), or distal SN with large fiber involvement (SN-LFI). RESULTS: A total of 56 patients were classified as SN-LFI and 54 SFSN. The medial plantar NAP, a measure of large fiber function, was abnormal in 31.8% of patients, more frequently in SN-LFI than SFSN. Distal leg epidermal nerve fiber (ENF) density, a measure of small fibers, was reduced in 47.3% of biopsies, with isolated ENF morphologic changes in 29.1% and normal findings in 23.6%. Biopsy abnormalities were more severe and prevalent in SN-LFI than in SFSN. In patients with a normal medial plantar NAP, distal leg biopsy showed reduced ENF density in 34.7%, and isolated morphologic changes in a further 37% of cases. CONCLUSIONS: The medial plantar nerve action potential and skin biopsy are complementary in evaluation of distal SN with normal routine NCS. Small sensory nerve fibers are affected early in SN, and more severely so when large fiber involvement is apparent clinically.

Action Potentials↗

PubMed1988

Carotid sinus hypersensitivity in patients with unexplained syncope: clinical, electrophysiologic, and long-term follow-up observations.

To assess the incidence and clinical characteristics of carotid sinus hypersensitivity and the relationship to electrophysiologic findings, 76 patients with unexplained syncope underwent carotid sinus massage during electrophysiologic studies for syncope evaluation. Twenty-one patients (28%) were found to have carotid sinus hypersensitivity. Of these 21 patients, 11 (52%) had coronary artery disease, two (10%) had hypertensive heart disease, and eight (38%) had no organic heart disease. During electrophysiologic studies, abnormal sinus node function was found in three patients (14%), abnormal atrioventricular (AV) node function was noted in four (19%), and combined abnormal sinus node and AV node functions were seen in three (14%). Eleven patients (53%) had a normal electrophysiologic study. During carotid sinus massage, sinus arrest alone was observed in 12 patients (57%), and combined sinus arrest and AV nodal block was seen in nine (43%). Thirteen patients were treated with a permanent pacemaker, in whom either carotid sinus massage reproduced the symptom or concomitant sinus node or AV node abnormality, or organic heart disease was present. With a mean follow-up of 42 +/- 19 months, none of these 13 patients had recurrent syncope. However, one of eight patients (13%) who did not receive a pacemaker had recurrence of syncope. Subsequently, this patient has done well after implantation of a pacemaker. These observations suggest that there is a significant incidence of carotid sinus hypersensitivity in patients with unexplained syncope. Permanent pacing appears to be beneficial in selected patients based on clinical and electrophysiologic findings.

Arrhythmia, Sinus↗

PubMedDate not supplied

Syringomyelia and scoliosis: a review of twenty-five pediatric patients.

A retrospective review of 25 patients with scoliosis secondary to syringomyelia was conducted. All patients had large syrinxes, but only 10 patients had abnormal neurologic findings, suggesting that subtle neurologic findings in patients with scoliosis should not be underestimated and supporting liberal use of magnetic resonance imaging (MRI). The results of three approaches to the care of these patients were reviewed: passive observation, bracing, and spinal fusion. Curves progressed > or = 5 degrees in patients aged < 10 years who were observed, but in children aged > 10 years, curves progressed > or = 10 degrees. Curves responded well to bracing, but correction was not maintained out of brace for patients with progressive and/or severe curves. Good corrective was achieved safely during operation.

Adolescent↗

PubMed1997

Pregnancy and delivery in tetraplegic women.

Increase in survival of spinal cord injured (SCI) women, society's acceptance that their lives should be similar to those of non-disabled women and their better general health are increasing the number of SCI women who become pregnant and will be delivered of a child. Vaginal delivery is preferred. Any SCI woman whose level is at T6 or higher is at risk for acute autonomic hyperreflexia as a result of uterine contractions. If induction is with Pitocin/Oxytocin, the risk is even greater. Communication with the woman's obstetrician is essential. The patient should be provided with a packet of information to share with the obstetrician. This should be followed with a phone call from the SCI physician to the obstetrician. Effective management includes epidural anesthesia; vacuum extraction is helpful in the expulsion stage. Episiotomy is usually not needed since the pelvic floor is relaxed. In addition, there is an increased incidence of dehiscence since SCI women should be mobilized early and need to transfer in and out of a wheelchair. Blood pressure needs to be taken during the peak of contraction. This needs to be compared to prenatal blood pressures. If prenatal blood pressure is 80/60 or 90/60 but during contraction is 130/80 with a pounding headache, that indicates autonomic hyperreflexia which is an indication for epidural anesthesia. With improvement of acute care and more effective rehabilitation, pregnancy and delivery in spinal cord injured (SCI) women will occur more frequently. No one has any great experience with this situation and most articles report only a few cases. Even the report by Goller and Paeslack4 dealt with 175 cases from 42 centers in 24 countries. Most women were paraplegic and several who were injured early in their pregnancy had abnormal babies (possibly due to x-rays taken for spinal injury). Our spinal cord injury staff were pleased when we had two tetraplegic patients who were pregnant. It helped confirm our belief that life and its functions continue after paralysis. Staff members were involved in prenatal care, were present during delivery and were involved with postnatal care. Even more important is the fact that rehabilitation from the start was oriented with child care in mind. Occupational therapists used their skills and imagination to develop a program for newborn baby care by the tetraplegic mother.

Adult↗

PubMed1991

Bladder and sphincter behavior in patients with spinal cord lesions.

To ascertain the relationship between the clinical neurological level, and bladder and sphincter behavior, the video-urodynamic studies of 489 patients with spinal cord lesions due to a variety of causes were retrospectively analyzed. Patients were classified based on the clinical neurological level, etiology of the lesion and presence or absence of signs of sacral cord involvement. Urodynamic findings were classified as either detrusor hyperreflexia, detrusor-external sphincter dyssynergia, detrusor areflexia or normal. The results indicate that although there was a general correlation between the neurological level of injury and the expected vesicourethral function, it was neither absolute nor specific. For example, 20 of 117 cervical cord lesions had detrusor areflexia, 42 of 156 lumbar cord lesions had detrusor-external sphincter dyssynergia and 26 of 84 sacral cord had either detrusor hyperreflexia or detrusor-external sphincter dyssynergia. However, if one considers the presence of neurological abnormalities, 84% of the suprasacral cord lesions with detrusor areflexia have sacral cord signs. In contrast, all suprasacral cord lesions with no evidence of sacral cord involvement have either detrusor hyperreflexia or detrusor-external sphincter dyssynergia. The positive predictive value for positive sacral cord signs and detrusor areflexia was 87%. The positive predictive value for negative sacral cord signs and detrusor hyperreflexia/detrusor-external sphincter dyssynergia was 81%. These data suggest that the clinical neurological examination alone is not an adequate barometer to predict neurourological dysfunction and that video-urodynamic evaluation provides a more precise diagnosis for each patient.

Adolescent↗