Issues in the psychoanalytic treatment of single females over thirty.
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Adult↗
Biomedical subjects
J S Lieberman
Publications and source records attributed to J S Lieberman.
Successful rechallenge to etoposide after an acute vasomotor response.
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Drug Administration Schedule↗
Scoliosis associated with Duchenne muscular dystrophy.
This study evaluated the age-related and ambulation-related incidence of scoliosis among boys with Duchenne muscular dystrophy (DMD) and the relationship between wheelchair dependency and scoliosis. Charts of 88 DMD boys were reviewed retrospectively. The relationships between age and both wheelchair dependency and scoliosis were graphed as cumulative distributions with increasing patient age. The relationship between ambulation status and scoliosis was analyzed with a stepwise series of chi square analyses, assuming increasing time intervals between wheelchair dependency and scoliosis. Wheelchair dependency and scoliosis were both age-related phenomena. The relationship between ambulation status and scoliosis became significant only after 3.5 years of wheelchair dependency. It should be noted that 24% of boys with scoliosis developed their curves before cessation of ambulation. While development of scoliosis among DMD boys is clearly an age-related phenomenon, its previously assumed relationship to ambulation requires reevaluation.
Adolescent↗
Relationship of manual muscle testing to objective strength measurements.
This study investigates the relationship between manual muscle test scores (MMT) and quantitative isometric strength measurements (QIS). It also evaluates the implications of that relationship for design of therapeutic trials. Extension and flexion strength at the elbows, hips, and knees of 21 neuromuscular disease patients were tested a total of 26 times utilizing both MMT and QIS testing. Paired data were evaluated with Spearman ranked correlation coefficients, and then QIS was predicted from MMT using Lowess, a consistent form of nonparametric regression. Finally, the implications of the Lowess analysis for designing a therapeutic trial were evaluated. MMT and QIS measurements were significantly correlated in all movements tested. Lowess analysis yielded prediction errors ranging from 16 to 24% of QIS range. Analysis of the sample size needed for a therapeutic trial suggested that a protocol measuring MMT would require more subjects for the same level of statistical significance as a protocol measuring QIS. Since it was not possible to reliably predict QIS values from MMT scores, such conversions are not appropriate for clinical use. This inconsistent relationship between MMT and QIS carries major implications for the design of therapeutic trials. Since therapeutic trials are time consuming, expensive, and most centers do not have large numbers of individuals available, using QIS as an outcome measure is a preferable research design.
Humans↗
Early smooth horizontal eye movement: a favorable prognostic sign in patients with locked-in syndrome.
Locked-in Syndrome (LIS) is a rare clinical entity consisting of quadriplegia, paralysis of lower cranial nerves, mutism, and bilateral paresis of horizontal gaze. The prognosis is usually poor. Most patients who do survive have severe residual impairment or develop chronic LIS. Review of the literature shows that recovery has been reported but not systematically studied, particularly with respect to early prognostic signs. We report a case of LIS with bilateral pontine infarctions shown by MRI. Severe neurological deficits included quadriplegia, paresis of horizontal gaze, facial paralysis, and bulbar palsy. Horizontal eye movements were present and smooth by the second week after onset. The patient had almost full functional motor recovery within four months, after a comprehensive rehabilitation program. Fourteen other reported cases of LIS with full recovery had documented recovery of lateral gaze in the early stage. In addition, smooth horizontal gaze is the most frequent activity in incomplete LIS. Early recovery of horizontal eye movement may indicate that the brain lesion is limited and may be a good prognostic sign in LIS.
Adult↗
Functional ability and equipment use among patients with neuromuscular disease.
The relationship between graded functional ability of a neuromuscular disease patient and his routine daily activities remains unknown. Mobility equipment use may provide an indirect measure of routine daily activities. In this study 110 neuromuscular disease patients were evaluated for both functional grade (Brooke upper extremity and Vignos lower extremity scales) and equipment use. Although several of the Vignos and Brooke categories did not describe significant numbers of these patients, the Vignos scale was able to predict whether or not a given patient would use a wheelchair with 94% accuracy, while the Brooke scale was only 85% accurate. It was not possible to predict manual vs electric wheelchair use based on functional rating scores. Patients with proximal weakness, regardless of diagnosis, displayed a similar pattern of functional outcome and equipment use, which was significantly different from the pattern displayed by groups with distal weakness. As expected, as functional ability diminished, wheelchair use increased; however, wheelchair use began at surprisingly high functional levels. Patients generally preferred a combination of manual and electric wheelchair use.
Activities of Daily Living↗
Upper vs lower extremity functional loss in neuromuscular disease.
Functional rating scales have been developed for evaluation of upper extremity (UE) and lower extremity (LE) function in Duchenne muscular dystrophy (DMD). The Vignos scale is accepted for LE function and the Brooke scale for UE evaluation. The assumption that UE functional loss parallels LE loss has never been tested. Furthermore, there are no studies examining the relationship between UE/LE strength. This study tests the hypothesis that UE/LE strength and functional losses are parallel in DMD and other neuromuscular disorders. Vignos grades, Brooke grades, and manual muscle test scores were determined for 27 patients with DMD, 10 patients with fascioscapulohumeral muscular dystrophy (FSH), 24 patients with myotonic muscular dystrophy (MMD) and 29 patients with other (Becker's dystrophy and limb-girdle syndrome) proximal myopathies (PM). Spearman correlation coefficients were calculated for UE vs LE functional grade and strength scores and subjected to significance analysis. While all correlations were statistically significantly different from zero (p less than 0.01), all were also significantly different from one (p less than 0.01), none representing perfect equivalence. Coefficients of determination indicated that a maximum of 86% (FSH) of variation in UE rank could be explained purely by observing LE rank. While there is a significant relationship between UE/LE strength and functional grade, these measurements were not found to be entirely equivalent. Therefore, while measures of lower body function or strength might be used as a substitute for UE measurements in drug trials in which global effects are expected, the two measurements are not equivalent when evaluating an individual's clinical status.
Child↗
Ultrasound treatment of reflex sympathetic dystrophy.
Three cases of lower extremity reflex sympathetic dystrophy (RSD) responded dramatically to a daily low dose ultrasound (0.5 watts/cm2 X 5 minutes) therapy to the tarsal tunnel and plantar nerve distribution, and the use of a shoe insert. Two of the three cases had been refractory to more standard therapy for RSD, including pharmacologic agents. All three patients preferred a conservative approach to surgical sympathectomy. No complications were observed, and all three cases are now symptom free. Daily ultrasound treatments are time-consuming and costly but are safe and may be useful for patients who refuse surgical sympathectomy. We hypothesize that ultrasound may have affected peripheral sympathetic nerve fibers. However, more indirect effects of ultrasound, such as increased blood flow to the limb, may be part of the action mechanism as well.
Adult↗
Upper extremity functional rating for patients with Duchenne muscular dystrophy.
The corresponding deterioration of upper extremity strength and functional capability with increasing age and the relationship between average strength and function were evaluated in 28 boys with Duchenne muscular dystrophy (DMD). Manual muscle testing (MMT) was used to evaluate strength and the Brooke upper extremity grading scale to rate upper extremity function. Mean MMT was found to relate to age in logarithmic fastion, ie, a 1% increase in age led to a 0.9% decrease in mean MMT. Functional grade (FG) related to age in a more complex fashion, revealing three groups based on age: under age 10, all in FG 1; between ages 10 and 13, concentration in FG 2 through 4; and over age 13, eight of ten in FG 5. Regression analysis did not contribute further useful information. Finally, mean MMT was found to be highly significantly related to FG. The plot of mean MMT against FG revealed an S-shaped curve, with rapidly rising functional grade once an average upper extremity strength of 4w was reached. The Brooke scale appears to be useful in describing upper extremity function among DMD patients, although it may reflect a three-grade rather than a six-grade discrimination capability. In describing the natural history of DMD, this scale demonstrates a pattern of rapid functional deterioration during the early adolescent years. This functional deterioration apparently takes place when the decline in strength reaches a threshold which cannot be compensated by muscle substitutions. At that time function deteriorates along a relatively rapid but undefined course.
Adolescent↗
McArdle's disease heterozygotes. Metabolic adaptation assessed using 31P-nuclear magnetic resonance.
Two daughters of a propositus with documented McArdle's disease were shown by enzyme assay, gel electrophoresis, and immunoblotting to be partially deficient in skeletal muscle phosphorylase and, presumably, heterozygous for the trait. Both exhibited only the adult form of the skeletal muscle isozyme. By 31P-nuclear magnetic resonance, both heterozygotes showed a greater production of acid during fully aerobic exercise than when blood flow was occluded in ischemic exercise. This pattern is in contrast to that of control subjects, where there is significantly greater acid production in ischemic versus aerobic exercise, and distinct from that of phosphorylase-negative patients in which no acid is produced in either circumstance. We suggest that these heterozygotes may have adapted to their diminished phosphorylase by enhancing utilization of plasma glucose. If so, this mechanism could account for the observation that most of the symptoms of McArdle's disease are often manifest only in adulthood. These studies also show that although there are very high concentrations of phosphorylase in skeletal muscle (approximately 2% of the soluble protein), such a high level is essential for normal muscle glycogenolysis.
Adult↗
Intellectual and cognitive function in adults with myotonic muscular dystrophy.
Intellectual and cognitive function was studied in 43 patients (21 men and 22 women) with myotonic muscular dystrophy (MMD). The inheritance distribution was 18 paternal, 10 maternal, and 15 unknown. All patients received the Wechsler Adult Intelligence Scale-Revised, the Wechsler Memory Scale, the Halstead-Reitan Neuropsychological Test Battery, and the Aphasia Screening Test. Significantly lower scores (p less than 0.05) were found on all intellectual and cognitive impairment tests in both men and women of the maternal inheritance MMD subgroup, compared with the paternal inheritance MMD subgroup and with the normative data. Scores for patients with paternally inherited disease did not differ significantly from normative data for verbal, performance, or full scale intelligence quotients and memory quotients. There were no significant differences in intellectual and cognitive test scores between men and women for the group as a whole, for the maternal inheritance subgroup, or for the paternal inheritance subgroup. It is suggested that inheritance pattern is a necessary variable in studies investigating intellectual and cognitive function in MMD. Although the reason for the association of lower intellectual and cognitive function in MMD of maternal inheritance is not known, the data presented here may be useful for genetic counseling.
Adolescent↗
Depression in myotonic muscular dystrophy.
Depression was studied in adults with myotonic muscular dystrophy (MMD). The subject groups consisted of 27 MMD patients, an able-bodied control group of 27 adults (CON), a nonprogressively disabled control group of 17 adult paraplegics (SCI), and a progressively disabled neuromuscular disease control group of 11 adults with limb-girdle syndrome (LGS). The study utilized a test battery consisting of the Minnesota Multiphasic Personality Inventory (MMPI), the Institute of Personality and Ability (IPAT) Depression test, the Suicide Probability Scale (SPS), and the California Psychological Inventory (CPI). The MMD group scored significantly higher on the MMPI Depression scale, the IPAT Depression Test, and the SPS Hopelessness subscale, than the CON or SCI groups. Conversely, the MMD group showed lower levels of positive mental health than these two groups on the MMPI Ego Strength and CPI Well-Being scale scores. The MMD group did not differ significantly from the LGS group in any of the five measures. A definite tendency toward depression was evident among MMD patients. A similar tendency was noted in the small LGS group. The progressive and disabling nature of the disease appears to be the major reason for depression in MMD patients. Our data do not support the concept that depression is an intrinsic characteristic of MMD.
Adult↗
Serotonin-induced contractile and structural changes in fast and slow skeletal muscles in mice.
The effects of serotonin on the contractile properties of the soleus and extensor digitorum longus muscles in mice were studied after chronic intraperitoneal administration with and without a recovery period, and compared to contractile studies of the same muscles performed after acute administration of serotonin. A curarized in vitro preparation of both muscles was used throughout for the contractile studies. In addition, structural changes were studied in the chronic preparations. Chronic serotonin administration produced histologic changes in type 1 and 2A fibers and a reduction in twitch and tetanic tension and the rate of twitch and tetanic tension development limited to the slow (aerobic) soleus muscle. Acute in vitro administration of serotonin altered the contractile properties of both soleus and extensor digitorum longus muscles with the latter being more involved. The effects of chronic serotonin administration appear to be primarily related to ischemia while the effects of acute administration appear to be mediated by direct, nonvascular action on skeletal muscle.
Animals↗
Effect of pentobarbital on contractility of mouse skeletal muscle.
Pentobarbital is a hypnotic drug commonly used as anesthesia for in vivo studies in various animals. A direct effect of pentobarbital on the central nervous system and skeletal neuromuscular junction has been known for at least 30 years. A recent study using single fiber preparations from amphibian muscles indicated a significant acute and direct effect on muscle contractility at drug concentrations within the anesthetic range. The present study using whole muscles from mice demonstrated a similar augmentation of twitch tension and rate of tension development whereas tetanic tension was reduced by this drug at similar concentrations. In addition, most time parameters of contraction were prolonged. It is of interest that the slow (oxidative) muscles were considerably more sensitive to pentobarbital than the fast (primarily anaerobic) muscles. We suggest that pentobarbital should not be used as the anesthetic agent for in vivo studies of other interventions when conclusions are based on changes in muscle contractility.
Animals↗
Psychometric evaluation in myotonic muscular dystrophy.
Systematic psychometric evaluations were performed in 16 patients with myotonic muscular dystrophy (MMD). All patients received the Wechsler Adult Intelligence Scale-Revised and Wechsler Memory Scale-1. In addition, 13 patients received the Reitan-Halstead Neuropsychological Test Battery (R-H), including the Aphasia Screening Test. Despite the high reported incidence of mental retardation in MMD, none of our pilot population showed mental retardation. However, 5 of the 13 patients showed evidence of possible organic mental dysfunction on the R-H. Problems in previous studies which could explain these discrepancies include the following: (1) small sample size, (2) studies limited to young children, and (3) a complete lack of systematic psychometric data in the previous reports. Systematic cooperative studies are suggested to elucidate the degree and type of cognitive involvement in MMD.
Adolescent↗
Fatiguing weakness: an initial symptom in cervical compressive radiculopathy.
Exercise-induced weakness (fatiguing) is described in three patients with cervical compressive radiculopathy. In all three cases, the patients had symptoms only while at work, and in two cases, symptoms occurred in life-threatening situations. All patients had seen several physicians with a functional diagnosis being considered in all cases because of symptoms only at work and the absence of objective findings during examination. Precise history and examination techniques led to the correct diagnosis and treatment after appropriate investigation. The fatiguing weakness in our cases is thought to be a manifestation of early or mild neurologic involvement unmasked by exercise, rather than a manifestation of compression or ischemia.
Adult↗
Vaccine-related poliomyelitis: serum IgM and cerebrospinal fluid antibodies.
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Adult↗
Rehabilitation following brain damage: some neurophysiological mechanisms. Physiological correlates of clinically observed changes in posture and tone following lesions of the central nervous system.
Extensive investigation of the role of the fusimotor system in the production of hypotonic and hypertonic disorders of posture and tone has been undertaken in humans and experimental animals. The data from human studies have usually been from indirect assessment of the fusimotor system, and results are often contradictory. Results are now available from animal studies utilizing direct recording of muscle spindle afferent discharge in a number of models of human disorder. Conditions resulting in hypotonia, e.g. cerebellar ablations, medullary pyramidotomy, VL nucleus, thalamotomy, acute spinal cord transection, and acute motor cortex ablation uniformly result in a depression of muscle spindle primary afferent discharge. Conditions resulting in hypertonia, e.g. chronic spinal cord transection and chronic motor cortex ablation, fail to show heightened muscle spindle afferent discharge, however. Rather the spindle afferent discharge returns to control levels in the models. Recovery from the hypotonic to the hypertonic state is, however, associated with significant recovery of spindle afferent function.
Animals↗