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Dive into the research topics where Raul N. Mandler is active.

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Featured researches published by Raul N. Mandler.


Neurology | 1998

Devic's neuromyelitis optica: A prospective study of seven patients treated with prednisone and azathioprine

Raul N. Mandler; Waseem Ahmed; John E. Dencoff

Seven newly diagnosed patients with Devics neuromyelitis optica were treated with long-term prednisone and azathioprine, and were followed every 2 months for at least 18 months. Their Expanded Disability Status Scale score improved significantly (mean at baseline, 9; mean at 18 months, 3; p < 0.005), and no relapses occurred for more than 18 months. Multicenter controlled studies are needed to prove the efficacy of this therapeutic regimen.


Neurology | 2003

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis

Merit Cudkowicz; Jeremy M. Shefner; David A. Schoenfeld; Robert H. Brown; H. Johnson; Muddasir Qureshi; M. Jacobs; Jeffrey D. Rothstein; Stanley H. Appel; Robert M. Pascuzzi; Terry Heiman-Patterson; Peter D. Donofrio; William S. David; James A. Russell; Rup Tandan; Erik P. Pioro; Kevin J. Felice; Jeffrey Rosenfeld; Raul N. Mandler; George Sachs; Walter G. Bradley; Elizabeth M. Raynor; George D. Baquis; J. M. Belsh; S. Novella; Jill M. Goldstein; J. Hulihan

Objective: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. Methods: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. Results: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). Conclusions: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.


Neurology | 2001

Phase III randomized trial of gabapentin in patients with amyotrophic lateral sclerosis

Robert G. Miller; Dan H. Moore; Deborah F. Gelinas; V. Dronsky; Michelle Mendoza; Richard J. Barohn; Wilson W. Bryan; John Ravits; E. Yuen; Hans E. Neville; Steven P. Ringel; Mark B. Bromberg; Jack H. Petajan; Anthony A. Amato; Carlayne E. Jackson; W. Johnson; Raul N. Mandler; P. Bosch; Benn E. Smith; Michael C. Graves; Mark A. Ross; Eric J. Sorenson; Praful Kelkar; Gareth Parry; Richard K. Olney

Background: Preclinical and clinical studies of gabapentin in patients with ALS led the authors to undertake a phase III randomized clinical trial. Methods: Patients were randomly assigned, in a double-blinded fashion, to receive oral gabapentin 3,600 mg or placebo daily for 9 months. The primary outcome measure was the average rate of decline in isometric arm muscle strength for those with two or more evaluations. Results: Two hundred four patients enrolled, 196 had two or more evaluations, and 128 patients completed the study. The mean rate of decline of the arm muscle strength was not significantly different between the groups. Moreover, there was no beneficial effect upon the rate of decline of other secondary measures (vital capacity, survival, ALS functional rating scale, timed walking) nor was there any symptomatic benefit. In fact, analysis of the combined data from the phase II and III trials revealed a significantly more rapid decline of forced vital capacity in patients treated with gabapentin. Conclusion: These data provide no evidence of a beneficial effect of gabapentin on disease progression or symptoms in patients with ALS.


Cancer | 1992

Castleman's disease in POEMS syndrome with elevated interleukin-6.

Raul N. Mandler; Daniel P. Kerrigan; Joanne Smart; Wietse Kuis; Peter M. Villiger; Martin Lotz

POEMS syndrome is a rare multisystem affliction known for its signs, from which it also takes its acronym name “peripheral neuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, and skin lesions.” Our study chronicles the course of this syndrome in a young woman with Castlemans disease (angiofollicular lymph node hyperplasia). Cerebrospinal fluid (CSF) and serum interleukin‐6 (IL‐6) levels were abnormally elevated at various times during a %month period. The authors conclude that the plasma cell dyscrasia associated with the POEMS syndrome of this patient was Castlemans disease. Elevation of serum IL‐6 levels might contribute to the pathogenesis of the POEMS syndrome. Cancer 1992; 69:2697‐2703.


Acta Neurologica Scandinavica | 2009

Neurologic manifestations of intravascular lymphomatosis.

J. E. Chapin; Larry E. Davis; Mario Kornfeld; Raul N. Mandler

Intravascular lymphomatosis is a rare fatal neoplasm characterized by malignant cells of lymphocytic lineage producing vascular occlusions. The cerebral vasculature is particularly affected. Two patients seen at our institution presented with progressive neurologic deficits including dementia, hemiparesis and myelopathy. Review of an additional 64 reported cases with neurologic involvement indicates that patients developed intermittent fevers, an encephalopathy ranging from acute disorientation to rapidly progressive dementia, and focal signs such as hemiparesis and myelopathy. Common laboratory abnormalities include elevated cerebrospinal fluid protein and a lymphocytic pleocytosis, elevated blood erythrocyte sedimentation rate and serum lactate dehydrogenase. Malignant cells are rarely seen in cerebrospinal fluid, blood or bone marrow. Neuroimaging is usually abnormal with parenchymal lesions seen on cerebral tomography and magnetic resonance imaging along with an occasional meningeal pattern of contrast enhancement. Treatment with corticosteroids, chemotherapy, radiation therapy, or plasmapheresis provided limited benefit. Intravascular lymphomatosis should be considered in the differential diagnosis of unexplained progressive encephalopathy with superimposed focal deficits.


Amyotrophic Lateral Sclerosis | 2005

Promoting excellence in end‐of‐life care in ALS

Hiroshi Mitsumoto; Mark B. Bromberg; Wendy Johnston; Rup Tandan; Ira Byock; Mary Lyon; Robert G. Miller; Stanley H. Appel; Josh Benditt; James L. Bernat; Gian Domenico Borasio; Alan C. Carver; Lora Clawson; Maura Del Bene; Edward J. Kasarskis; Susan B. LeGrand; Raul N. Mandler; Jane McCarthy; Theodore L. Munsat; Daniel Newman; Robert Sufit; Andrea Versenyi

The type and quality of end‐of‐life care varies greatly in ALS; the time to initiate end‐of‐life care is not defined, and decision making is hampered by logistical and financial barriers. There has been no systematic review of these issues in ALS. The goals of this initiative are to: 1) improve end‐of‐life care for patients with ALS and families based on what limited evidence is available; 2) increase awareness, interest, and debate on the end‐of‐life care in ALS; and 3) identify areas needed for new prospective clinical research. The ALS Peer Workgroup reviewed the literature and 1) identified the current state of knowledge, 2) analysed the gaps in care, and 3) provided recommendations for standard of care and future research. It was shown that areas of investigation are needed on the incorporation of an interdisciplinary approach to care in ALS that includes: psychosocial evaluation and spiritual care; the use of validated instruments to assess patient and caregiver quality of life; and the establishment of proactive caregiver programs. Several public policy changes that will improve coverage for medical care, hospice, and caregiver costs are also reviewed. More clinical evidence is needed on how to provide optimal end‐of‐life care specifically in ALS.


Amyotrophic Lateral Sclerosis | 2001

The ALS Patient Care Database: insights into end-of-life care in ALS.

Raul N. Mandler; Frederick A. Anderson; R. G. Miller; L. Clawson; Merit Cudkowicz; Ml Del Bene; Walter G. Bradley; Linda I. Boynton de Sepulveda; Benjamin Rix Brooks; Neil R. Cashman; Michael Graves; Yadollah Harati; Terry Heiman-Patterson; Mary Lyon; Hiroshi Mitsumoto; Dan H. Moore; Steven P. Ringel; Jeffrey Rosenfeld; Mark A. Ross; Michael J. Strong; Robert Sufit

OBJECTIVE: To study clinical practices and patient outcomes near the end of life in amyotrophic lateral sclerosis (ALS). BACKGROUND: Patients, families, and healthcare providers face several dilemmas in selecting and delivering care near the end of life in ALS. Published data on clinical practices and their benefits during end-of-life care for ALS patients consist of anecdotal reports based on small case series or individual case reports. METHODS: Data were obtained from 1014 American and Canadian patients with ALS who died while participating in a large observational registry (the ALS Patient Care Database) during the past four years. Following death, a caregiver or family member provided data for each patient using a standard questionnaire. Data were principally generated through American and Canadian ALS multidisciplinary centers of excellence. RESULTS: Most patients died peacefully (90.7%) and 62.4% died in a hospice-supported environment. Advance directives were in place for 88.9% of patients and were followed in 96.8%. Among the 67 patients who exhibited distress in the dying process, symptoms included breathing difficulties (82.1%), fear/anxiety (55.2%), pain (23.9%), insomnia (14.9%), and choking (14.93%). Oxygen was given to 52.6% of patients, and pain medications were given to 74%. CONCLUSION: These data suggest that palliative care at the end of life was relatively well managed for most patients with ALS who participated in this study; nevertheless, several opportunities for improvement were identified.


Journal of Neuroscience Methods | 1995

Quantitative measurement of muscle strength in the mouse

Jeffrey P. Smith; Pamela S. Hicks; Leroy Ortiz; Michael J. Martinez; Raul N. Mandler

We have designed a special dynamometer for measuring mouse forelimb muscle strength and endurance. The device exploits a mouses tendency to grasp a horizontal metal bar while suspended by its tail. A threshold value for the magnitude and duration of force that the mouse can exert is obtained by first allowing the animal to grasp the bar and then applying a steadily increasing downward force to the opposite end of a cable to which the mouse attaches. The bar is attached to a force transducer and pen recorder to produce a permanent record of the force produced by the mouse. Test results show that this dynamometer provides quantitative measurements of muscle strength and endurance in the mouse. Comparisons between experimental groups of normal and wobbler mice, a model for lower motor neuron disease, show that both the force exerted by the animals (muscle strength), and the duration of the pull (endurance), can be quantified and statistically analyzed. This technique can be used as an assay for quantitating the effects of in vivo drug treatments on murine neuromuscular disorders.


Amyotrophic Lateral Sclerosis | 2006

Factors correlated with NPPV use in ALS.

Carlayne E. Jackson; Steven Lovitt; Neelam Gowda; Frederick A. Anderson; Robert G. Miller; Walter G. Bradley; Mark B. Bromberg; Benjamin Rix Brooks; Neil R. Cashman; Lora Clawson; Merit Cudkowicz; Dallas Forshew; Michael C. Graves; Terry Heiman-Patterson; Mary Lyon; Hiroshi Mitsumoto; Dan H. Moore; Steven P. Ringel; Jeffrey Rosenfeld; Mark A. Ross; Michael J. Strong; Robert Sufit; Valerie Cwik; Linda I. Boynton de Sepulveda; Yadollah Harati; Raul N. Mandler; Eric Pioro

In spite of emerging evidence of therapeutic benefit from non‐invasive positive pressure ventilation (NPPV), only a minority of ALS patients use this therapy. We examined factors which correlate with use of NPPV in ALS patients. Data were analyzed from the ALS CARE Database on the use of NPPV in patients with FVC less than 50% of predicted and probable or definite ALS based on modified El Escorial criteria. Of the 403 eligible patients, 146 (36%) used NPPV. NPPV compliance was strongly correlated with symptoms of dyspnea and orthopnea as well as with the use of other therapies including PEG tubes, augmentative speech devices, and riluzole. Male gender and household income >


Amyotrophic Lateral Sclerosis | 2004

Western ALS study group

Robert G. Miller; Dan H. Moore; Carlayne E. Jackson; Deborah F. Gelinas; Richard J. Barohn; April L. McVey; Art Dick; David Saperstein; Jeffrey Rosenfeld; Mark B. Bromberg; Jack H. Petajan; John Ravits; Edward J. Kasarskis; Hans E. Neville; Steven P. Ringel; Dianna Quan; Raul N. Mandler; E. P. Bosch; Benn E. Smith; Mark A. Ross; Michael C. Graves; Angela Genge; Catherine Lomen-Hoerth; Richard K. Olney; Alan Pestronk; Paul H. Gordon; David Walk; John W. Day; Gareth Parry; Jau Shin Lou

80,000 were also associated with higher NPPV use. There was no correlation between age, race, type of insurance, forced vital capacity, duration of symptoms, ALSFRS‐R, caregiver burden or quality of life with the use of NPPV. These data suggest that the factors which are most closely associated with NPPV utilization are symptomatic orthopnea and dyspnea. The findings may be useful in designing prospective studies to examine the factors which might explain the underutilization of NPPV and the optimal use of this treatment.

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Robert G. Miller

California Pacific Medical Center

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Dan H. Moore

California Pacific Medical Center

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Steven P. Ringel

George Washington University

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Carlayne E. Jackson

University of Texas Health Science Center at San Antonio

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Joanne Smart

University of New Mexico

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