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Miller Fisher-Guillain-Barré overlap syndrome with enhancing lesions in the spinocerebellar tracts.

The site of lesions in Miller Fisher syndrome, especially those causing ataxia, has been controversial. A 50 year old man with features of Miller Fisher syndrome in whom MRI showed enhancing lesions in the spinocerebellar tracts at the level of the lower medulla is reported. Peripheral involvement of cranial nerves was also indicated by an abnormal blink reflex and by clinical manifestations: complete external ophthalmoplegia, bilateral peripheral facial weakness, convergence disturbance, absence of Bell's phenomenon, oculocephalic, and oculovestibular reflex. Abnormal lesions on MRI disappeared and the blink reflex became normal with clinical improvement. The case is regarded as Miller Fisher-Guillain-Barré overlap syndrome, a postinfectious allergic reaction involving both peripheral nerves in the cranium and neuraxis in the spinocerebellar tract. The lesions in the spinocerebellar tracts are responsible for cerebellar ataxia in this syndrome.

Blinking↗

Hyperreflexia in axonal Guillain-Barré syndrome subsequent to Campylobacter jejuni enteritis.

We describe a patient with the acute motor axonal neuropathy (AMAN) form of Guillain-Barré syndrome (GBS), who showed generalized hyperreflexia. A 24-year-old man developed acute paralysis following Campylobacter jejuni enteritis. He showed exaggerated tendon reflexes with abnormal reflex spread to other segments, and was initially diagnosed as having post-infectious myelitis. Nerve conduction studies showed motor axonal degeneration (the AMAN pattern), and increased soleus H-reflex amplitudes. His serum was positive for IgG antibodies to gangliosides GM1b and GalNAc-GD1a. He was treated with plasmapheresis, resulting in rapid recovery. Hyperreflexia was still present 12 months after onset when muscle strength was completely normal. This case provides further evidence that patients with AMAN can develop increased motor neuron excitability, and possible mechanisms for the hyperreflexia are discussed.

Adult↗

Gadolinium diethylenetriaminepentaacetic acid enhancement in magnetic resonance imaging in relation to symptoms and signs among sciatic patients: a cross-sectional study.

STUDY DESIGN: A cross-sectional descriptive study. OBJECTIVES: Gadolinium enhancement in lumbar magnetic resonance imaging is not used routinely. The current study explored the possible intercorrelations of enhancement patterns with clinical symptoms and signs. SUMMARY OF BACKGROUND DATA: Rim enhancement has been reported to occur in the periphery of disc herniations, and it is thought to represent neovascularization. To the authors' knowledge, the significance of the enhancement in relation to clinical symptoms has not been studied. METHODS: Magnetic resonance imaging of the lumbar spine with intravenous gadolinium diethylenetriaminepentaacetic acid was performed in each patient. Various contrast enhancement parameters and volume of herniation were evaluated, and their correlations with clinical signs and symptoms (straight leg raising, motor defect, Achilles reflex, leg and back pain, disability) were analyzed. RESULTS: The extent of rim enhancement correlated highly significantly with the degree of disc displacement, being most pronounced in the case of sequesters. The duration of sciatic symptoms correlated negatively with enhancement. The clinical symptoms did not correlate significantly with the different enhancement parameters or disc herniation volume. Straight leg raising correlated only slightly with the extent of rim enhancement (P = 0.04) when bulges were excluded. Achilles reflex abnormality correlated significantly with all enhancement parameters for lesions at L5-S1. In the final stepwise logistic regression model, contrast enhancement extent correlated most significantly with abnormal Achilles reflex (P = 0.0002). CONCLUSIONS: Although rim enhancement of disc herniation is thought to represent a beneficial phagocytotic phenomenon, it may also have a harmful effect on the adjacent nerve root.

Achilles Tendon↗

[Babinski and Chaddock signs without apparent pyramidal disfunction].

PURPOSE: The main objective of this study was to verify, in one hundred in-patients from the Serviço de Clínica Médica do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro who did not have a history of clinical symptoms of pyramidal disfunction, the presence of the Babinski and Chaddock signs. As a secondary objective, we looked for a prevalence of one of the signs over the other, and the influence of the head position regarding the obtained responses. METHOD: The patients were examined while supine with their heads in three different positions. RESULTS: Out of the one hundred patients, ten of them (10%) showed hallux extension uni or bilateral. The Babinski sign was positive 18 times (40%), and the Chaddock sign was positive 27 times (60%). CONCLUSION: The Chaddock sign occurred more frequently than the Babinski sign, the abnormal reflex occurred twice as much on the left foot than the right, and apparently there was no interference regarding the head position in relation to the obtained results.

Adult↗

[Electromyographic and neurological aspects of leprosy. Study of 100 cases].

By the neurological, electromyographic, anatomopathological and bacterioscopic examinations of 100 pacients with leprosy the following conclusions have been drawn: There was a high incidence of reflex abnormalities. The proprioceptive reflexes were abnormal in 64.5% and the superficial reflexes in 70.8% of the cases. According to the topography of the segments examined, the deep reflexes showed the following abnormal values: 25.3% in the cephalic segment; 69.1% in the upper limbs and 74.5% in the lower limbs. Of the 2,289 reflexes investigated, abnormalities were found in 773 superficial reflexes (70.8%), while in relation to the proprioceptive reflexes the figures rose to 901 (64.5%). In the early stages of the infirmity, the reflexes were normal in 41.2% of the cases examined, and in all the stages of the evolution of the disease those numbers were always high. Also in the first stage, whose average duration was of 4.9 years, several alternations were noted, although there was a predominance of brisk reflexes (25.1%) over diminshed reflexes (17%), followed by hyperactive (8.6%) and absent reflexes (8%). In the final stage of the infirmity there was a decrease of normal reflexes, in contrast to absent and decreased reflexes, whose rates increased to 32.7% and 30.6% respectively. The disappearance of hyperactive reflexes and the clear decrease of brisk reflexes should also be noted. There was a high percentage of patients (31%) who suffered agression of the eighth nerve, 17 of which (54.8%) were found to suffer from neural deafness. The values related to hearing deficit were rarely in accordance with the histopathological and bacterioscopic positive results. The indexes of abnormalities found out in the examination of the fifth and of the seven nerve (7% and 5% respectively) were much lower in relation to the eighth nerve. These results are in contradiction with the ones stated in the literature. In some of the patients, hyperesthesia of the skin of the plantar region prognosticated the appearance of the infirmity. Among the objective changes, anesthesia of superficial sensibilities combined with deep hyperesthesia and anesthesia of superficial sensibilities with abolition of profound sensibilities were rarely observed. No case was found with exclusive alterations of deep sensibilities. Peripheral motor neuron lesions demonstrable by electromyography were observed in 82 patients (82%)...

Adult↗

Neurological manifestations of aging.

A group of 51 socially active, self-declared neurologically normal subjects ranging from 61 to 84 years of age were studied with a battery of clinical neurological examinations and a semiquantitative vibration perception test. None of the subjects showed a consistent pattern of neurological deficit that could be identified as pathological. On review of systems, the most frequent complaints were difficulty remembering names, numbness and tingling of fingers and toes, and headaches. In the neurological examination, the most commonly observed abnormalities were positive palmomental reflex, snout reflex, abnormalities in pursuit eye movements, convergence insufficiency, and minor mistakes in perception of light touch. There was no apparent trend of worsening of symptoms and signs with advancing age. In the semiquantitative testing of vibration perception, there was a slight trend toward worsening of vibration perception in the upper and lower extremities with advancing age. Motor abnormalities were absent, and there were relatively few subjects who showed reflex abnormalities. This study confirms the previously reported diminution of vibration perception with aging. Caution must be exercised before attributing myotatic reflex and motor abnormalities to the aging process alone.

Aged↗

An abnormal neural reflex plays a role in causing syncope in sinus bradycardia.

OBJECTIVES: This study investigates the role of an abnormal neural reflex in causing syncope in patients with sinus bradycardia. BACKGROUND: Syncope is commonly considered an indication of severity in sinus bradycardia. However, the occurrence of syncope is unpredictable, and the prognosis appears to be similar in patients with and without syncope. METHODS: Head-up tilt testing (60 degrees for 60 min), carotid sinus massage in the supine and standing positions, 24-h Holter ambulatory electrocardiographic (ECG) recording and electrophysiologic study before and after pharmacologic autonomic blockade were performed in 25 patients with sinus bradycardia and syncope (group I, sinus rate < 50 beats/min, age 71 +/- 12 years) and 25 patients with sinus bradycardia and no neurologic symptoms (group II, sinus rate < 50 beats/min, age 67 +/- 16 years). RESULTS: Clinical characteristics and ambulatory ECG monitoring data were similar in the two study groups. A positive response (induction of syncope or presyncope with hypotension and/or bradycardia) was obtained by head-up tilt testing in 15 group I (60%) and in 3 group II (12%) patients (p < 0.001) and by carotid sinus massage in 11 group I (44%) and 6 group II (24%) patients (p = NS). Results of at least one test (head-up tilt testing or carotid sinus massage, or both) were positive in 19 group I (76%) and 9 group II (36%) patients (p < 0.01). Basal and intrinsic corrected sinus node recovery time did not differ significantly between the two groups. An abnormal intrinsic heart rate was present in 66% of group I and 26% of group II patients (p < 0.01). The different percentage of positive findings on head-up tilt testing and carotid sinus massage in the two groups was independent of the presence of intrinsic sinus node dysfunction. CONCLUSIONS: These results indicate that an abnormal neural reflex plays a role in causing syncope in patients with sinus bradycardia. This reflex seems to be unrelated to the severity of sinus node dysfunction, even if the latter could enhance the cardioinhibitory response.

Aged↗

Spectrum of abnormal rectoanal reflex patterns in patients with fecal incontinence.

PURPOSE: Abnormalities of rectoanal inhibitory or excitatory reflex in patients with fecal incontinence are well described. A spectrum of abnormal responses, other than those already described in the literature, has been observed in some patients with fecal incontinence and forms the subject of this report. METHOD: Forty-three patients with idiopathic or traumatic fecal incontinence were studied to evaluate their reflex responses to balloon distention of the rectum, and results were compared with reflex responses of 29 control subjects with no anorectal complaints. RESULTS: Control subjects revealed normal reflex responses consisting of initial excitation followed by inhibition in the proximal anal canal and an excitatory response in the distal anal canal. Patients who were incontinent revealed five different types of reflex patterns. Eleven patients (25.5 percent) with segmental sphincter defects from obstetric injuries exhibited no distal excitation but had normal response in the proximal anal canal (Group 1). Eleven patients (25.5 percent) with idiopathic incontinence exhibited normal proximal response but an inhibitory as opposed to excitatory response in the distal anal canal (Group 2). Three patients (7 percent) with iatrogenic trauma failed to register an excitatory response in the proximal or distal anal canal but revealed a normal inhibitory reflex (Group 3). Nine patients (21 percent) with idiopathic incontinence revealed excitatory response in the entire anal canal but no inhibition (Group 4). Nine patients (21 percent) with idiopathic incontinence had a normal reflex pattern (Group 5). CONCLUSION: Excitatory and inhibitory components of rectoanal reflexes may selectively be abolished in neurogenic or traumatic insults to visceral and somatic anal sphincters, resulting in altered rectoanal reflex patterns.

Adult↗

The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine.

Neurophysiological investigations of a patient suffering from the stiff-man syndrome revealed that exteroceptive reflexes, in particular those elicited from the skin, were excessively enhanced. In contrast, no abnormalities were found within the monosynaptic reflex arc. Clomipramine injection severely aggravated the clinical symptoms whereas diazepam, clonidine, and tizanidine decreased both muscular stiffness and abnormal exteroceptive reflexes. The hypothesis is put forward that the stiff-man syndrome is a disorder of descending brain-stem systems which exert a net inhibitory control on axial and limb girdle muscle tone as well as on exteroceptive reflex transmission. Detection of abnormal exteroceptive reflex activity in conjunction with neuropharmacological testing might help in the diagnosis of this rare disease.

Adult↗

Abnormal cardiovascular reflexes in patients with gastro-oesophageal reflux.

Using five non-invasive tests, abnormalities of cardiovascular reflex function were found in 20 of 50 patients with gastro-oesophageal reflux. Abnormalities of pupil cycle time, an index of non-vagal parasympathetic function, were found in only a few patients, implying that the cardiovascular abnormalities observed were not part of a generalised parasympathetic defect. The findings are consistent with the hypothesis that an abnormality of vagal function may contribute to the pathogenesis of gastro-oesophageal reflux.

Adolescent↗

Abnormal cardiovascular reflexes in juvenile diabetics as preclinical signs of autonomic neuropathy.

The prevalence of abnormalities of cardiovascular reflexes as a marker of autonomic neuropathy was studied in a group of juvenile diabetics with long-standing disease, aged 20-58 years (mean 36). No patient had clinical evidence of autonomic failure. The study was carried out employing the most simple techniques and routine equipment. Resting heart rate, beta-to-beat variation, Valsalva ratio, systolic blood pressure in supine position and immediately after standing were measured. The results were compared with those determined in an age and sex-matched population. Beat-to-beat variation test appeared to be the most sensitive technique in detecting an impaired autonomic function (12/54 = 22.2%). Resting tachycardia was found in 10 patients and postural hypotension in 6. Only 1 patient had an abnormal Valsalva ratio associated with postural hypotension and reduced beta-to-beat variation. In agreement with previous studies our data confirm that autonomic dysfunction is frequent in asymptomatic diabetics. The methods to detect these abnormalities are easy to perform. Therefore they may be routinely used as screening tests in the diabetic population.

Adult↗

Jamaica ginger paralysis. Forty-seven-year follow-up.

In 1930, thousands of Americans were poisoned by an illicit extract of Jamaica ginger ("jake") used to circumvent the Prohibition laws. A neurotoxic organophosphate compound, triorthocresyl phosphate (TOCP), had been used as an adulterant. The earliest reports were of peripheral neuritis, but later it was evident that an upper motor neuron syndrome had supervened. This TOCP poisoning apparently involved various cell groups and tracts in the spinal cord; the lesions was not peripheral at all. We interviewed 11 survivors of the illness residing in eastern Tennessee. Four were carefully examined. The principal findings showed the spasticity and abnormal reflexes of an upper motor neuron syndrome. One patient had mild disease, despite typical findings, and had lived a normal life.

Aged↗

Aspiration risk after acute stroke: comparison of clinical examination and fiberoptic endoscopic evaluation of swallowing.

Aspiration is an important variable related to increased morbidity, mortality, and cost of care for acute stroke patients. This prospective systematic replication study compared a clinical swallowing examination consisting of six clinical identifiers of aspiration risk, i.e., dysphonia, dysarthria, abnormal gag reflex, abnormal volitional cough, cough after swallow, and voice change after swallow, with an instrumental fiberoptic endoscopic evaluation of swallowing (FEES) to determine reliability in identifying aspiration risk following acute stroke. A referred consecutive sample of 49 first-time stroke patients was evaluated within 24 hours poststroke, first with the clinical examination followed immediately by FEES. The endoscopist was blinded to results of clinical testing. The clinical examination correctly identified 19 subjects with aspiration risk, when compared with the criterion standard FEES, but incorrectly identified 3 patients as having no aspiration risk when they did. The clinical examination incorrectly identified 19 subjects with aspiration risk but determined correctly no aspiration risk in 8 patients who did not exhibit aspiration risk on FEES. Clinical examination sensitivity = 86%; specificity = 30%; false negative rate = 14%; false positive rate = 70%; positive predictive value = 50%; and negative predictive value = 73%. It was concluded that the clinical examination, when compared with FEES, underestimated aspiration risk in patients with aspiration risk and overestimated aspiration risk in patients who did not exhibit aspiration risk. Careful consideration of the limitations of clinical testing leads us to believe that a reliable, timely, and cost-effective instrumental swallow evaluation should be available for the majority of patients following acute stroke.

Aged↗

Behavioral characteristics and early temperament of premature infants with intracranial hemorrhage.

Behavioral characteristics of 12 full-term and 44 premature infants with and without intracranial hemorrhage (ICH) were studied. Cranial ultrasonography prospectively documented Grade I-II ICH in 14, Grade III-IV in 19 and no ICH in 11 premature infants. Examination at corrected age of 40 +/- 2 weeks using the Neonatal Behavioral Assessment Scale showed that infants in the ICH groups had lower levels of arousal and more abnormal reflexes than full-terms. Infants with ICH III-IV displayed less optimal motor responses than full-term infants and diminished orientation responses, especially to visual stimuli. Thus, lower level of arousal, immature motoric processes, and poor visual orientation differentiated premature with ICH from full-term infants, although premature infants without these sequelae, did not differ significantly from full-term infants. The above may represent early manifestations of visual-perceptual and motor problems noted in the follow-up of ICH infants. Further, neonatal behavior was found to affect parent ratings of infant temperament (via the Bates Infant Characteristics Questionnaire) at 3 months corrected age, and the relationships between neonatal behavior and parental ratings differed depending upon the infant's gestational age and severity of hemorrhage. We conclude that neonatal behaviors are less optimal in premature infants, and least optimal in premature infants with severe intracranial hemorrhage when compared to fullterm infants.

Arousal↗

Aspiration in patients with acute stroke.

OBJECTIVES: To determine the frequency and clinical predictors of aspiration within 5 days of acute stroke. DESIGN: Case series. SETTING: Tertiary care center. PATIENTS: Consecutive stroke patients (n = 55) with new neurologic deficit evaluated within 5 days of acute stroke. MAIN OUTCOME MEASURES: Comparison of features identified on clinical swallowing and oromotor examinations and occurrence of aspiration (silent or overt) evident on videofluoroscopic swallow study (VSS). RESULTS: Aspiration occurred in 21 of 55 patients (38%). Whereas 7 of 21 patients (33%) aspirated overtly, 14 (67%) aspirated silently on VSS. Chi-square analyses revealed that dysphonia, dysarthria, abnormal gag reflex, abnormal volitional cough, cough after swallow, and voice change after swallow were significantly related to aspiration and were predictors of the subset of patients with silent aspiration. Logistic regression revealed that abnormal volitional cough and cough with swallow, in conjunction, predicted aspiration with 78% accuracy. CONCLUSIONS: Silent aspiration appears to be a significant problem in acute stroke patients because silent aspiration occurred in two thirds of the patients who aspirated. The prediction of patients at risk for aspiration was significantly improved by the presence of concurrent findings of abnormal volitional cough and cough with swallow on clinical examination.

Acute Disease↗

Adaptations of peripheral vasoconstrictor pathways after spinal cord injury.

The consequences of spinal cord injury on the function of sympathetic pathways in the periphery have generally been ignored. We discuss two types of plasticity that follow disruption of sympathetic pathways in rats . The first relates to the partial denervation of sympathetic ganglia that would follow the loss of some preganglionic neurones. Sprouting of residual connections rapidly reinnervates many postganglionic neurones, restoring functional transmission within a few weeks, but other neurones may be permanently decentralized. Some of the new functional connections may generate inappropriate pathways leading to abnormal reflexes . The second type of plasticity concerns the markedly enhanced and prolonged contractile responses to nerve activity in arterial vessels to which ongoing sympathetic activity has been reduced or silenced following spinal cord transection or ganglion decentralization. In a cutaneous artery (the rat tail artery), the mechanisms underlying this arterial hyperreactivity differ from those in the splanchnic arteries (the rat mesenteric artery). In the former, hyperreactivity is mainly postjunctional but independent of changes in alpha1-adrenoceptor sensitivity, whereas the increased responsiveness in the latter vessels can be attributed to a greater responsiveness to alpha1-adrenoceptor activation. There are enough data from humans to suggest that both of these novel findings in experimental animals are likely to apply after spinal cord injury and contribute to autonomic dysreflexia .

Animals↗

Intrauterine exposure to low levels of lead: the status of the neonate.

It has been suggested that lead (Pb) at low exposure levels is a behavioral teratogen. Blood lead (Pb-B) was measured in 185 samples of maternal blood and in 162 samples of cord blood drawn from members of a cohort of mother-infant pairs. Routine newborn assessments, an examination for minor anomalies, the Brazelton Neonatal Behavioral Assessment (NBAS), and part of the Graham/Rosenblith Behavioral Examination (G/R) were administered. Maternal and cord Pb-B correlated 0.80. In regression analyses, Apgar scores, birthweight, length, head circumference, neonatal anomalies, and seven behavioral scales were unrelated to either maternal or cord Pb-B. Three scales--the NBAS Abnormal Reflexes, the G/R Neurological Soft Sign, and the G/R Muscle Tonus Scales--were related minimally to either cord or maternal Pb-B. Because of the contrast in maternal and cord results, despite the high correlation of maternal and cord Pb-B, the data were reanalyzed for 132 cases with paired data. Only the Soft Sign Scale remained significant and that only for cord, but not maternal Pb-B. Regression analysis revealed a suppression with the Soft Sign Scale related to the variance of the cord Pb-B that was not common with maternal Pb-B. The possibility that the fetus under stress tends to accumulate Pb was considered.

Alcohol Drinking↗

Do associated reactions in the upper limb after stroke contribute to contracture formation?

OBJECTIVE: To establish (1) whether associated reactions could contribute to contracture formation and (2) whether the presence of spasticity was essential for their expression, after stroke. SUBJECTS: Subjects were 24 hemiparetics within 13 months of a stroke, unselected for contracture or spasticity. MAIN OUTCOME MEASURES: Associated reactions were identified by the presence of muscle activity in the affected biceps brachii and quantified as the amount of affected elbow flexor torque produced during a moderate contraction of either the contralateral biceps brachii or the contralateral quadriceps muscles. Contracture was measured as loss of elbow joint range of motion and spasticity as the presence of abnormal reflex activity. RESULTS: Associated reactions were present in at least one testing condition in seven subjects. During contractions of the contralateral biceps brachii, the median amount of elbow flexor torque produced was 0.39 (interquartile range, IQR 2.5) Nm while during contractions of the contralateral quadriceps muscle it was 0.19 (IQR 1.6) Nm. Associated reactions were not associated with contracture (p = 0.39) which was present in over half of the subjects. The incidence of associated reactions was about the same as that of spasticity, but the two were not related (p = 0.61). CONCLUSIONS: Even though associated reactions were present in 29% of the subjects during moderate contraction of the contralateral muscles, they were not large, nor were they associated with contracture or spasticity, suggesting that this phenomenon is not usually a major problem for everyday function after stroke.

Adult↗