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Biomedical subjects

E D Louis

Publications and source records attributed to E D Louis.

At least 19 recordsLinked to original sources
PubMed1999

Dystonia in Huntington's disease: prevalence and clinical characteristics.

BACKGROUND: The prevalence and clinical characteristics of dystonia in Huntington's disease (HD) have not been formally assessed. OBJECTIVES: To study (1) the prevalence of dystonia in HD in a clinic population, (2) the clinical features of dystonia, and (3) clinical correlates of dystonia (for example, age, disease duration). METHODS: Patients with HD attending the HD Center at the New York State Psychiatric Center were administered the Unified HD Rating Scale and underwent a standardized 5.5-minute videotaped examination. Two neurologists reviewed the videotaped examination and rated the severity and constancy of dystonia, calculating a total dystonia score for each patient. RESULTS: Prevalence of dystonia of any severity was 95.2%. Twenty-four of 42 (57.1%) had dystonia in at least one body region that was moderate and present more than half of the time, and seven of 42 (16.7%) had dystonia that was severe and constant. The most prevalent types of dystonia were internal shoulder rotation (64.3%), sustained fist clenching (47.1%), excessive knee flexion (42.9%), and foot inversion (42.9%). In 37 of 42 (88.1%) patients, there were more than two types of dystonia, and in the average patient, three to four types of dystonia. The mean severity was between 1 (mild) and 2 (moderate), and the mean constancy was between 2 (present less than half of the time) and 3 (present more than half of the time). Multivariate linear regression revealed that disease duration (p = 0.0005) and taking an antidopaminergic agent (p = 0.03) were positively associated with the total dystonia score. CONCLUSIONS: The majority of patients in this HD clinic exhibited some dystonia. The dystonia was present in several body regions and manifested by a variety of movements and postures not typical of idiopathic torsion dystonia. The dystonia was not bothersome to most patients, and its severity was a function of disease duration and use of an antidopaminergic agent.

Adult↗
PubMed1998

Is essential tremor symmetric? Observational data from a community-based study of essential tremor.

BACKGROUND: Essential tremor (ET) has been variably portrayed in the literature both as a symmetric arm tremor and as an asymmetric arm tremor. Few quantitative clinical or neurophysiological data specifically address the issue of tremor asymmetry in ET. OBJECTIVES: To examine a community-dwelling cohort of subjects with ET to (1) estimate the prevalence of tremor asymmetry and (2) quantify the magnitude of tremor asymmetry. METHODS: Fifty-four subjects with ET, identified in a community-based study of ET in New York City, underwent a Tremor Interview and a videotaped Tremor Examination. The examination included 6 tasks: sustained arm extension, pouring water, drinking water, using a spoon, finger-to-nose movements, and drawing spirals with each arm. Two neurologists rated the severity of tremor using a 0 to 3 clinical rating scale and a total tremor score was calculated (range, 0-36). Fourteen (25%) of 54 subjects also underwent quantitative computerized tremor analysis. RESULTS: The prevalence of asymmetry depended on the definition of asymmetry; small to moderate differences between sides were common. The mean side-to-side difference in clinical ratings for each of the 6 tasks was 0.54 of 3 points, which represented a 1.32-fold difference between sides. Clinical rating scores were higher in the nondominant arm in 39 subjects (72%), higher in the dominant arm in 9 (17%), and equal in 6 (11%). The 2 left-handed subjects had higher clinical ratings on the right. During quantitative computerized tremor analysis, there was a 1.71-fold mean difference between tremor amplitudes in the dominant and nondominant sides, and in 12 subjects (86%), the maximum tremor amplitude was in the nondominant arm. CONCLUSIONS: Small to moderate differences between sides were common in ET. In most community-dwelling subjects, tremor amplitude was greatest in the nondominant arm. In contrast, clinic-based studies have reported greater tremor in the dominant arm; those with ET who seek medical attention are more likely to exhibit severe tremor in their dominant arms. This study documents that mild asymmetry is a fundamental property of ET and that tremor is more severe in the nondominant arm.

Aged↗
PubMed1998

How normal is 'normal'? Mild tremor in a multiethnic cohort of normal subjects.

BACKGROUND: While many "normal" subjects exhibit mild clinically detectable tremor, the extent to which this tremor is present has received little attention. OBJECTIVE: To characterize the prevalence and clinical characteristics of mild, clinically detectable tremor in a multiethnic cohort of normal subjects. METHODS: Normal control subjects (n=36) and their relatives (n=67) were enrolled in a community-based case-control study of the familial aggregation of essential tremor. Subjects underwent a tremor interview and videotaped tremor examination. Two neurologists independently rated the severity of tremor during different postures and tasks, and a total tremor score (maximum score, 36) was calculated for each subject. Eight subjects were randomly selected to undergo quantitative computerized tremor analysis. RESULTS: In 103 normal subjects (36 control subjects and 67 relatives of control subjects) the mean total tremor score was 4.8 (range, 0-12.5). Ninety-nine (96%) of 103 subjects had tremor, as defined by a total tremor score of 0.5 or higher. For most tasks, the mean tremor score was greater in the nondominant than in the dominant arm. The total tremor score correlated highly with age (r=0.28; P=.004). There were no sex or ethnic differences in the mean total tremor scores. On tremor analysis, the amplitude and frequency of the tremor differed from that in a group of similarly studied subjects with essential tremor. CONCLUSIONS: Normal subjects almost uniformly have a clinically detectable tremor that is mild and age dependent. Characterization of this tremor helps to establish standards for normal tremor. These standards are crucial for accurate diagnostic classification in population-based studies of essential tremor.

Adolescent↗
PubMed1998

Diagnostic criteria for essential tremor: a population perspective.

BACKGROUND: Prevalence estimates vary 2750-fold among the 20 studies of essential tremor (ET). It is not clear how the choice of diagnostic criteria affects research results. OBJECTIVE: To determine the impact of alternative sets of diagnostic criteria on the diagnosis of ET. METHODS: As part of the Washington Heights-Inwood Genetic Study of ET (WHIGET), a population-based study of ET, 285 subjects who include 36 case subjects with probable or definite ET, 34 case subjects with possible ET, and 215 normal subjects were interviewed and examined. All diagnoses in WHIGET were assigned by 2 neurologists. Ten of the 20 published prevalence studies of ET provided diagnostic criteria for ET. Criteria differed in terms of requirements for the distribution, duration, and severity of tremor. These 10 sets of criteria were then each separately applied to the subjects in the WHIGET cohort to determine their impact on the diagnosis of ET. RESULTS: Depending on which diagnostic criteria were applied to the WHIGET cohort, the proportion of WHIGET case subjects with definite or probable ET who would have been diagnosed as having ET was as low as 14% and the proportion of WHIGET normal subjects who would have been diagnosed as having ET was as high as 51%. Diagnostic criteria that included a positive family history of ET or a lengthy duration of tremor would have classified many WHIGET case subjects with ET as normal, whereas criteria that did not specify a minimal tremor severity would have classified many WHIGET normal subjects as having ET. CONCLUSIONS: Alternative sets of diagnostic criteria for ET greatly impact on the diagnosis of ET. For population-based studies, information on tremor type and severity rather than family history should be included in diagnostic criteria.

Adolescent↗
PubMed1998

How common is the most common adult movement disorder? estimates of the prevalence of essential tremor throughout the world.

Essential tremor (ET) is the most common adult movement disorder, as much as 20 times more prevalent than Parkinson's disease. Estimates of the crude prevalence of ET range widely from 0.08 to 220 cases per 1000 persons, a 2750-fold difference. There has been no formal attempt to synthesize these disparate results. Our purpose is to provide an overview of existing studies, to examine methodologic issues that may account for this tremendous variability in results, and to provide a more precise estimate of the prevalence of ET. Nineteen studies of the prevalence of ET were reviewed. Factors that contribute to the broad range of prevalence estimates include (a) differences in study design that influence validity and (b) differences in characteristics of study populations that influence comparability of studies. If we limit our examination to studies that (a) provided diagnostic criteria for ET, (b) defined ET as an action tremor, and (c) used community-based rather than service-based designs, then five studies remain, and the prevalence of ET is 4.1 to 39.2 cases per 1000, a 9.6-fold difference. Four of these five provided age-stratified data. Among these four, the prevalence of ET in those over the age of 60 years was 13.0 to 50.5 cases per 1000, a 3.9-fold difference.

Adult↗
PubMed1998

Reliability between two observers using a protocol for diagnosing essential tremor.

Protocols with demonstrated reliability have been established for the diagnosis of numerous movement disorders. whereas in the essential tremor (ET) literature, there is no discussion about the reliability of diagnostic protocols. Lack of knowledge of the reliability of diagnostic protocols in ET limits the use of these protocols because reliability is an essential requirement for scientific quality in data management. The objective of this study was to determine the reliability of a protocol for diagnosing ET. The protocol consists of a Tremor Interview, a videotaped Tremor Examination, and a diagnostic algorithm. Eighty-three subjects with ET, identified in a community-based health study in Washington Heights-Inwood, New York, were matched with 83 control subjects from the same community. These subjects and their relatives are being recruited to participate in the Washington Heights-Inwood Genetic Study of ET. Two hundred twenty-six subjects have been evaluated to date (35 ET cases, 40 controls, 151 relatives). All 226 underwent an 84-item Tremor Interview and 26-item videotaped Tremor Examination. Diagnoses (normal, possible ET, probable ET, definite ET) were independently assigned by two blinded neurologists specializing in movement disorders. The kappa statistic, k, was used to determine diagnostic agreement between these two neurologists. The concordance rate between two raters using diagnostic categories definite ET, probable ET. possible ET, and normal was 80%; kw = 0.84 (near perfect to perfect agreement). The concordance rate between two raters using two diagnostic categories (definite ET and normal) was 100%; k = 1.00 (perfect agreement). There was high correlation between the two raters' total tremor scores (r = 0.89, p < 0.00001). This diagnostic protocol is highly reliable. Research in ET would greatly benefit from diagnostic protocols with demonstrated reliability.

Adolescent↗
PubMed1998

Clinical characteristics of essential tremor: data from a community-based study.

BACKGROUND: 99.5% of individuals with essential tremor (ET) who live in the community have mild tremor and do not attend clinics. Clinic-based studies of ET have not allowed investigators to characterize the full clinical spectrum of this disorder. In community-based studies of ET, the primary focus has been the prevalence rather than the clinical characteristics of ET. OBJECTIVE: To describe the clinical characteristics of ET as seen in a community-based study. METHODS: 73 subjects with ET, identified in a community-based study of ET in Washington Heights-Inwood, New York, underwent a standardized 84-item physician-administered tremor interview and a 26-item videotaped tremor examination which included 12 bedside tests for ET. Two neurologists who specialized in movement disorders and who demonstrated excellent interrater agreement rated the severity of tremor using a 0 to +3 clinical rating scale and assigned a total tremor score (range, 0-36) and a diagnosis of ET. RESULTS: Diagnoses in the 73 cases were: definite ET (18, 24.7%), probable ET (32, 43.8%), and possible ET (23, 31.5%). The mean total tremor score was 17.8 of 36. Thirty-six of 73 (49.3%) were asymptomatic, answering "no" to the question "do you often have shaking or tremor that you can't control?" Sixty-seven of 73 (91.8%) had not been prescribed medication for tremor. On average, subjects received tremor ratings of > or =+2 on only 5.4 of the 12 bedside tests for ET. Kinetic tremor was rated as more severe than postural tremor in 72 (98.6%) of 73 cases. CONCLUSIONS: We present the clinical findings of a group of largely untreated, unselected cases of ET that would not otherwise have come to neurologic attention. The tremor was mild, often asymptomatic, and not uniformly present throughout the examination. It was rarely treated. The kinetic component of the tremor was more severe than the postural component. These clinical data further our understanding of the clinical spectrum of ET.

Adolescent↗
PubMed1998

Outcome of selective ramisectomy for botulinum toxin resistant torticollis.

OBJECTIVE: To investigate the long term outcome of selective ramisectomy denervation in patients with botulinum toxin resistant spasmodic torticollis. BACKGROUND: The published surgical series of ramisectomy treatment for torticollis do not provide systematic information on patients who develop resistance to the current standard of treatment-botulium toxin injections. Moreover, there is little information on surgical outcome using rating scale measurements of torticollis, or assessments of functional and occupational capacity. METHODS: Using a structured interview format and videotape assessments of severity of dystonia in a retrospective fashion, detailed follow up information was obtained on 16 patients who underwent open label selective denervation for severe, disabling torticollis, refractory to injections of botulinum toxin. RESULTS: Of 16 patients with disabling torticollis followed up postoperatively for a mean of 5 years, six (37.5%) had a moderate or complete return of normal neck function, as determined using functional capacity scales, whereas 10 had only minimal relief of dystonia or gain in function. Six of the 16 patients (37.5%) underwent a second peripheral denervation operation, and one required a third. Of 11 patients working outside the home before surgery, nine were disabled by dystonia, and only one continued to work after surgery. Dystonia rating scale scores of videotaped examinations using a modification of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) improved in 12 of 14 patients (85.7%) who underwent selective ramisectomy. When patients with primary botulinum toxin resistance were excluded, the magnitude of benefit for this subgroup was 31.9% of the baseline dystonia score (p<0.0002), comparable with the degree of improvement in a group of control patients receiving botulinum toxin treatment for torticollis. CONCLUSION: About one third of patients with torticollis resistant to injections of botulinum toxin may derive modest long term functional improvement from selective denervation, with a reduction in dystonia by about 30%, but remain unable to work.

Adult↗
PubMed1998

Does a screening questionnaire for essential tremor agree with the physician's examination?

In research studies of essential tremor (ET), monetary and geographic factors often necessitate diagnosis by interview rather than by examination. Few attempts have been made to determine the validity of a screening instrument for ET. A total of 242 subjects (33 definite or probable ET, 54 possible ET, and 155 normal) were part of a community-based family study of ET in northern Manhattan. Subjects underwent a tremor interview and videotaped tremor examination. The interview included 12 screening questions for ET. Two neurologists rated the severity of tremor on videotape and assigned diagnoses. The subjects' responses to the 12 screening questions were compared with the neurologic examination and the neurologists' diagnoses. There was an association between the number of affirmative responses on the 12-item screen and the severity of tremor during the videotaped tremor examination (r = 0.66, p < 0.01). Including those with probable and definite ET and defining a positive screen result as an affirmative response to at least 1 of 12 screening questions, the sensitivity of the screening questionnaire was 73%, the specificity was 96%, and the positive predictive value was 80%. The responses to the questionnaire closely correlated with the severity of tremor on neurologic examination. The questionnaire had moderate validity for subjects with probable or definite ET; subjects with mild ET often screened negative. Hence, in population-based studies and family studies of ET, it is important to examine all subjects for the presence of mild asymptomatic ET.

Adolescent↗
PubMed1997

Prevalence of asymptomatic tremor in relatives of patients with essential tremor.

BACKGROUND: The extent to which essential tremor (ET) clusters within families (ie, the familial aggregation of ET) is not precisely known. In part, this is because studies assign disease status in relatives of patients with ET based solely on interviews without conducting physical examinations. This may lead to underascertainment of affected relatives with mild asymptomatic ET. OBJECTIVE: To determine the prevalence of asymptomatic ET among relatives of patients with ET. METHODS: Interview and examination of 25 patients with ET and 58 of their relatives. The interview included 12 questions that screened the patients for ET. Two neurologists who specialize in movement disorders reviewed the videotaped examinations. Based on standardized criteria, diagnoses included ET (definite, probable, or possible) on normal. RESULTS: Of the 8 relatives who received diagnoses of ET, 5 (62.5%) had asymptomatic ET. Hence, 5 (8.6%) of the 58 relatives (95% confidence interval 1.4%-15.8%) had asymptomatic ET. In those with asymptomatic ET, there was a preponderance of young individuals with mild tremor in the nondominant hand. CONCLUSIONS: The prevalence of asymptomatic ET in relatives of patients with ET was similar to that of symptomatic ET. Family studies that do not perform both an interview and a physical examination will underascertain the number of affected relatives. Therefore, future family studies should evaluate relatives of patients with ET with an interview supplemented by a physical examination.

Aged↗
PubMed1997

Mortality from Parkinson disease.

BACKGROUND: Levodopa therapy for Parkinson disease (PD) has improved quality of life, but mortality rates remain high. Although the presence of dementia and severity of extrapyramidal signs (EPSs) influence morbidity in PD, it is not known whether these manifestations contribute to mortality. METHODS: Patients with PD were compared with nondemented and demented elderly subjects. Each underwent annual neurological and neuropsychological examinations. Dementia was diagnosed by Diagnostic and Statistical Manual of Mental Disorders, Third Edition, Revised criteria, and EPSs were rated with the Unified Parkinson's Disease Rating Scale. Survival rates were compared using Kaplan-Meier analysis and Cox proportional hazards models. RESULTS: The risk of mortality, when compared with nondemented elderly subjects, was highest among those with both PD and dementia (rate ratio, 4.9; 95% confidence interval, 3.4-7.1), but also was elevated in patients with PD only (rate ratio, 2.7; 95% confidence interval, 1.7-4.4). Dementia in the absence of PD also was associated with an increased risk of mortality (rate ratio, 1.6; 95% confidence interval, 1.1-2.3). A high baseline total EPS score was associated with significantly earlier mortality. CONCLUSIONS: Compared with nondemented elderly people in the same community, patients with PD have a 2- to 5-fold increased risk of mortality. The risk is strongly related to the presence of severe EPSs, especially bradykinesia. Despite the introduction of levodopa and other advances in the treatment of PD, these factors greatly increase mortality.

Aged↗
PubMed1997

Polymerase chain reaction-based detection of Tropheryma whippelii in central nervous system Whipple's disease.

Whipple's disease of the central nervous system (CNS) may be associated with normal intestinal histology as a result of minimal or patchy involvement. The diagnosis is difficult and is frequently made post mortem. We studied 6 patients with clinically suspected CNS Whipple's disease; 2 had oculomasticatury myorhythmia (OMM) fitting criteria for a diagnosis of definite CNS Whipple's disease. One of the 2 had duodenal histology highly suggestive of Whipple's disease the other 5 patients had normal duodenal histology. DNA was extracted from paraffin-embedded duodenal tissues in all patients and frozen pontine tissue in 1. Two primer pairs (W3F-W4R, W3F-W2R) were used in separate polymerase chain reactions (PCRs) to amplify fragments of Tropberyma whippelii 16S rDNA from these tissue samples. PCR amplicons were detected only in the duodenal tissues from the 2 patients with OMM. The sequences of these amplicons were identical to the corresponding region of the previously published Tropheryma whippelii 16S rDNA sequence. PCR-based assays of intestinal or brain tissue may be of value for confirming, and possibly refuting, a clinical diagnosis of CNS Whipple's disease in a patient with any combination of dementia, supranuclear gaze palsy, hypothalamic manifestations, myoclonus, seizures, ataxia, or OMM, especially when tissue histology is unrevealing.

Actinobacteria↗
PubMed1997

Agreement among movement disorder specialists on the clinical diagnosis of essential tremor.

Even though essential tremor (ET) is the most prevalent movement disorder, there has been little agreement in the neurologic literature regarding diagnostic criteria for ET. The authors attempted to determine the extent and source of agreement and disagreement among neurologists regarding diagnostic criteria for clinically definite ET. The authors designed and mailed a semistructured questionnaire to 160 neurologists who specialize in movement disorders in 24 countries. The questionnaire included three sections: a list of inclusion criteria, a list of exclusion criteria, and a list of potential clinical scenarios (for example, isolated site-specific tremor and primary orthostatic tremor). The questionnaire was completed by 98 (61%) of 160 targeted neurologists. There was greater consensus regarding features considered unnecessary inclusion criteria for clinically definite ET (extent of disability, disease duration, and positive family history) than for those considered necessary inclusion criteria (postural versus action tremor). With regard to exclusion criteria, there was some consensus in terms of the presence of Parkinson's disease, dystonia, history of hyperthyroidism or concurrent use of tremor-inducing medications, and cerebellar signs. The majority of neurologists would diagnose ET in the setting of isolated head or voice tremor. There are areas of both consensus and divergence among neurologists with regard to diagnostic criteria for ET. The choice of diagnostic criteria may vary depending on the intended use of the criteria (that is, clinical versus genetic studies). Hopefully, this study will foster further discussion to achieve a more general consensus.

Humans↗
PubMed1997

The shaking palsy, the first forty-five years: a journey through the British literature.

The authors examined the British medical literature published in the 45-year-period following Parkinson's treatise on the shaking palsy to determine the number and type of references to the shaking palsy or paralysis agitans during this particular period. Several sources suggest that Parkinson's 1817 treatise on the shaking palsy received little immediate attention in his native country, England, and that not until 1861, in France, did Charcot began to elucidate the clinical features of this entity, separating it from other neurologic disorders (for example, multiple sclerosis). A review of the British medical literature from the 45-year-period 1817-1861 revealed a number of references to paralysis agitans, including those by Cooke (1820), Good (1824 and 1829), Elliotson (1827, 1829, 1830, 1831, and 1833), Gowry (1831), anonymous (1832), Todd (1833), Watson (1836), Gibson (1839), Hall (1838 and 1841), Thompson (1842), Graves (1843), Birkett (1853), Paget (1855), and Reynolds (1855). Many of these did not report new or personally observed cases, did not separate Parkinson's disease from other disease entities characterized by both "shaking" and "palsy" (for example, tonic-clonic seizures), or misattributed motor signs to dysfunction of the pyramidal system rather than an extrapyramidal system (that is, attributing bradykinesia or rigidity to weakness). Although there were several references to "shaking palsy" in the early- to mid-19th-century British medical literature, there were few original case reports of Parkinson's disease. This may have contributed to the fact that during this period little was added to the original observations made by Parkinson in 1817. In particular, the separation of bradykinesia and weakness did not become apparent until later work by the French.

Cognition Disorders↗
PubMed1997

The Washington Heights-Inwood Genetic Study of Essential Tremor: methodologic issues in essential-tremor research.

Essential tremor (ET) is the most prevalent movement disorder. It is unknown to what extent ET clusters within families, and the role of genetic susceptibility in etiology of ET has not been adequately investigated at the population level. The problem is largely methodological, with few well-designed studies. The Washington Height-Inwood Genetic Study of ET, begun in 1955, is designed to investigate the genetics of ET using a methodology that has not been applied to ET research to date. Part of the design includes a new set of clinical and electrophysiological diagnostic criteria for ET; the present paper describes this novel study design.

Case-Control Studies↗
PubMed1997

Comparison of extrapyramidal features in 31 pathologically confirmed cases of diffuse Lewy body disease and 34 pathologically confirmed cases of Parkinson's disease.

OBJECTIVE: To compare the extrapyramidal features of pathologically confirmed cases of diffuse Lewy body disease (DLBD) and Parkinson's disease (PD). BACKGROUND: The proportion of pathologically confirmed cases of DLBD diagnosed clinically as PD is as high as 88%. Few papers focus specifically on the extrapyramidal features of DLBD. Further characterization of these features might facilitate antemortem diagnosis, in particular, distinguishing DLBD from PD. METHODS: Review of prospective and retrospective clinical data on a large series of pathologically diagnosed cases of DLBD (N = 31) and PD (N = 34) seen between 1984 and 1995 at Columbia-Presbyterian Medical Center or the University of Rochester. RESULTS: Those with DLBD had an older mean age of onset (67.9 years) than PD (62.0 years) (z = 6.5, p < 0.0001). Rest tremor was more common in PD (85.0%) than DLBD (55.0%) (chi 2 = 4.3, p = 0.038). Myoclonus was more common in DLBD (18.5%) than PD (0%) (Fisher's p = 0.021). There were no differences in rigidity, bradykinesia, dystonia, or gaze palsies. Clinical response to levodopa may have been more common in PD (100%) than DLBD (70.0%) (Fisher's p = 0.059). The occurrence of any one of four clinical features (myoclonus, absence of rest tremor, no response to levodopa, or no perceived need to treat with levodopa) was 10 times more likely in DLBD than PD (odds ratio = 10.29, 95% confidence interval = 2.58-41.11). CONCLUSIONS: We demonstrated that several clinical features distinguish DLBD from PD. These features, in combination with reported differences in cognitive and psychiatric manifestations, may be used for diagnostic purposes in distinguishing DLBD from PD in prospective longitudinal cohort studies of DLBD.

Aged↗
PubMed1997

Lymphoproliferative disorders and motor neuron disease: an update.

We studied 26 patients with both motor neuron disease and lymphoproliferative disease (LPD). Twenty-three patients had definite or probable upper motor neuron signs; none had electrophysiologic evidence of motor neuropathy. LPD syndromes comprised Waldenström's macroglobulinemia, multiple myeloma, chronic lymphocytic leukemia, follicular cell lymphoma, and Hodgkin's disease. In all but one patient, the cause of disability or death was neurologic. LPD was confined to bone marrow in 14 patients; eight of 14 had monoclonal paraproteinemia. One patient had LPD discovered at autopsy. Treatment of LPD in 20 patients resulted in neurologic improvement in 1 patient and arrest in another; both had progressive spinal muscular atrophy. Eleven patients were worse and 13 died. At least 30 cases have been reported from other centers, bringing the total to 56. Among the unusual reported concomitants were POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma, and skin changes) syndrome of myeloma and angiotropic lymphoma.

Adult↗