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Heart failure, cause notwithstanding, is characterized by adaptive (but harmful) increases in sympathetic activity and hormone levels, as well as by abnormal cardiovascular reflexes. Such reflex derangements may contribute to neurohumoral excitation. Consequently, normalization of the reflexes may be an important determinant of the effectiveness of therapy.
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We studied the tendon reflex (T-reflex) in 26 patients with acquired chronic demyelinating polyneuropathy (CDN), including 22 with chronic inflammatory demyelinating polyneuropathy (CIDP). In 7 patients reflexes were brisk or normal on clinical testing. The height adjusted T-reflex was abnormal in 25 (96%) cases, including 6 of 7 patients with brisk or normal reflexes on clinical testing. Mean latency (P < 0.01) and duration (P < 0.05) of the ankle and patellar tendon reflexes were significantly prolonged in the CIDP patients when compared to the controls. Mean latency in the CIDP patients was 152% of normal means. In 7 CIDP patients, the T-reflex latencies were prolonged beyond 150% of normal means. Thus, the T-reflex test is abnormal in a majority of patients with CDN, even in the presence of well-preserved clinical reflexes, and the T-reflex latency is a useful indicator of the presence of a demyelinating peripheral neuropathy in some patients.
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1. This study was undertaken to determine whether, in a group of patients complaining of recurrent syncopal attacks but with no apparent cause, there was evidence of abnormal cardiovascular reflex control. 2. The steady-state responses of blood pressure, heart rate and cardiac output to head-up tilting were determined in 67 patients using entirely 'non-invasive' methods. In some patients we also studied the immediate response of pulse interval to carotid baroreceptor stimulation by neck suction. 3. Two of the patients developed vasovagal attacks during the 20 min test period of head-up tilting. Eighteen others showed postural hypotension, defined as a fall in blood pressure to outside the limits of two SDS from the mean values of age-related control subjects. 4. Patients who showed postural hypotension had a mean fall in cardiac output significantly larger than that in age-related control subjects. Responses in the nonhypotensive patients did not differ significantly from controls. 5. Stimulation of carotid baroreceptors resulted in significantly smaller responses of pulse interval in the patients defined as having postural hypotension compared with the non-hypotensive patients and with the age-related control subjects. 6. In some of the patients who did not show postural hypotension during the standard test, the duration of tilt was prolonged for up to 1 h. Five out of 26 patients developed vasovagal attacks. All the vasovagal patients showed an initial tachycardia and the response of pulse interval to neck suction was significantly larger than in the controls.(ABSTRACT TRUNCATED AT 250 WORDS)
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Spermatid perinuclear RNA-binding protein (SPNR) is a microtubule-associated RNA-binding protein that localizes to the manchette in developing spermatids. The Spnr mRNA is expressed at high levels in testis, ovary, and brain and is present in these tissues in multiple forms. We have generated a gene trap allele of the murine Spnr, named Spnr(+/GT). Spnr(GT/GT) mutants show a high rate of mortality, reduced weight, and an abnormal clutching reflex. In addition to minor anatomical abnormalities in the brain, males exhibit defects in spermatogenesis that include a thin seminiferous epithelium and disorganization of spermatogenesis. Most of the sperm from mutant males display defects in the flagellum and consequently show decreased motility and transport within the oviducts. Furthermore, sperm from mutant males achieve in vitro fertilization less frequently. Our findings suggest that SPNR plays an important role in normal spermatogenesis and sperm function. Thus, the Spnr(GT/GT) mutant male mouse provides a unique model for some human male infertility cases.
Nephropathic cystinosis is a genetic disorder in which the amino acid cystine accumulates in lysosomes, resulting in multiorgan dysfunction. Progressive neuromuscular dysfunction, with bulbar and upper extremity weakness, has been described in adults with this disorder. The purpose of the present study was to determine whether there was evidence of early bulbar involvement, suggested by feeding difficulties or oral motor dysfunction in these patients, and whether the feeding and oral motor problems were associated with other evidence of neurologic dysfunction. Twenty-two children and adolescents with nephropathic cystinosis were studied. Parents completed questionnaires on feeding history and oral motor problems. Eighteen patients were given an oral motor examination, and 14 received a complete neurologic examination. The majority of children had a history of feeding difficulties. Seven children required a gastrostomy tube. Abnormalities on oral motor examination included hypotonia, abnormal gag reflex, and throaty or congested voice. Abnormalities on neurologic examination included hypotonia, muscle weakness, gross and fine motor dysfunction, and ataxia. The results indicate that feeding difficulties and oral motor dysfunction are common in children with cystinosis and appear to correlate with the general degree of neurologic dysfunction. Long-term follow-up is necessary to determine whether the early oral motor problems predict the later development of the progressive myopathy observed in adults with cystinosis.
Electromyographic reaction times (EMG-RTs) of the right knee extension were examined in eight normal subjects and eight patients with paraparesis under three conditions; during passive knee flexion (PFLX), passive extension (PEXT) and no passive movements (STAT). EMG-RTs of the three conditions were not significantly different between the normal and the patient group. A two-way analysis of variance (condition x group) showed that the main effect of condition was significant, but that of group and the interaction were not. Both in the normal and the patient group the shortest EMG-RTs of all was obtained in PEXT, followed by that of STAT and PFLX. Effect of passive movements on EMG-RTs in the patients whose position sense of the knee and muscle strength of the knee extension were within normal range did not vary with the abnormality of tactile sense and/or myotatic reflexes. The results indicated that activities of the pyramidal tract neurons in carrying out a movement were directly influenced by kinesthetic information of the moving limb.
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