Clinical features of LRP4/agrin-antibody-positive myasthenia gravis: A multicenter study.

Introduction: Our aim in this study was to identify the prevalence and clinical characteristics of LRP4/agrin-antibody-positive double-seronegative myasthenia gravis (DNMG).

Methods: DNMG patients at 16 sites in the United States were tested for LRP4 and agrin antibodies, and the clinical data were collected.

Results: Of 181 DNMG patients, 27 (14.

A novel, efficient, randomized selection trial comparing combinations of drug therapy for ALS.

Combining agents with different mechanisms of action may be necessary for meaningful results in treating ALS. The combinations of minocycline-creatine and celecoxib-creatine have additive effects in the murine model. New trial designs are needed to efficiently screen the growing number of potential neuroprotective agents.

Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial.

Background: Minocycline has anti-apoptotic and anti-inflammatory effects in vitro, and extends survival in mouse models of some neurological conditions. Several trials are planned or are in progress to assess whether minocycline slows human neurodegeneration. We aimed to test the efficacy of minocycline as a treatment for amyotrophic lateral sclerosis (ALS).

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

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.

ALS diagnostic criteria, El Escorial, and Philip II of Spain: a historical perspective.

El Escorial, a magnificent palace-monastery in central Spain, was the setting in 1990 for a meeting of ALS experts who developed a consensus document called the El Escorial ALS Diagnostic Criteria. El Escorial was originally conceived by the Spanish Habsburg monarch, Philip II (1527-1598), as an elaborate burial place for his parents, Emperor Charles V and Isabella. It soon became a symbol of the Spanish empire and Philip's Catholic leadership of the Counter-Reformation.

ALS diagnostic criteria of El Escorial Revisited: do they meet the needs of clinicians as well as researchers?

The El Escorial criteria for diagnosis of amyotrophic lateral sclerosis (ALS) have been in use for almost a decade. A revised set of criteria, meant to supersede the original set, was developed at a 1998 World Federation of Neurology (WFN) ALS meeting at Airlie House in Warrenton, Virginia, USA. This revised document, nicknamed El Escorial Revisited, has been published on the WFN-ALS website.

Diagnostic challenges in ALS.

Although the essential requirements for diagnosis of amyotrophic lateral sclerosis (ALS) are clearly defined by the El Escorial criteria, many physicians, including neurologists, still miss the diagnosis. Physician misdiagnosis of ALS relates to lack of knowledge about ALS and skill and to diagnostic difficulty. The differential diagnosis must exclude nonmotor neuron diseases and other adult-onset motor neuron diseases with restricted presentations, e.

The amyotrophic lateral sclerosis (ALS) patient perspective on misdiagnosis and its repercussions.

We studied the rate of initial "misdiagnosis', along with factors than might distinguish such patients, in 64 patients with amyotrophic lateral sclerosis (ALS) who completed a survey of 34 questions. Announcement of the survey was made by electronic newsletter and users group bulletin board directed at ALS patients. The questionnaire was distributed to interested ALS patients via electronic mail (e-mail), and 64 ALS patients (81% from the USA) returned their completed questionnaires via reply e-mail or postal mail.

Follow-up study of risk factors in progressive supranuclear palsy.

The cause of progressive supranuclear palsy (PSP) is not known and has been little studied. The one previous controlled epidemiologic survey, performed at our center in 1986, found small-town experience and greater educational attainment as PSP risks, but, in retrospect, these results may have been produced by ascertainment bias. Since that time, several anecdotal reports have implicated heredity and various environmental exposures in the cause of some cases of PSP.

Percutaneous magnetic coil stimulation of the phrenic nerve roots and trunk.

We describe a technique of percutaneous magnetic coil (MC) stimulation of the phrenic nerve trunk on one side of the neck and phrenic roots over the upper cervical vertebral column in 10 normal subjects and 2 patients. We were able to obtain compound muscle action potentials (CMAPs) from the diaphragm at two sites (xiphoid process and 7th intercostal space) after stimulation of the phrenic nerve trunk and roots. We noted that the onset latencies after phrenic root stimulation remained fixed despite increasing the stimulus intensity from 50% to 100% and on moving the MC vertically or laterally, suggesting that stimulation of the fastest conducting fibers was occurring at a fixed site, most likely at the intervertebral foramina.

Magnetic brain stimulation: safety studies.

We describe short-term and long-term safety studies after low repetition rate magnetic brain stimulation in 10 normal subjects. We obtained quantitative EEG data, psychometric test results, serum prolactin and cortisol levels before and after brain stimulation. EEG and psychometric data were also obtained in 5 of these subjects 16-24 months after the initial experiment.

Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration.

The purpose of this study was to determine the degree of respiratory muscle impairment in patients with newly diagnosed amyotrophic lateral sclerosis (ALS) and the subsequent rate of decline of respiratory function. Thirty-one of 36 patients had respiratory muscle weakness at presentation, although only 7 complained of any respiratory symptoms. Vital capacity (percent predicted) was significantly lower in the symptomatic group (55.

Magnetic coil stimulation of the human lumbosacral vertebral column: site of stimulation and clinical application.

We describe the technique of magnetic coil (MC) stimulation of the lumbosacral roots and the possible site of stimulation in 22 control subjects, and the clinical usefulness of MC stimulation in 5 patients with low back pain. We observed 2 components in the compound muscle action potential of the soleus muscle following MC stimulation over the lumbosacral region. The second component had the physiological properties of the H reflex.

Anterior femoral cutaneous nerve injury following femoral artery reconstructive surgery.

Two cases are presented exhibiting symptoms and signs of bilateral anterior femoral cutaneous nerve injury, clinically sparing femoral nerve branches to the saphenous nerve and quadriceps muscles. This occurred following surgical dissection in the femoral triangles associated with femoral artery reconstructive surgery. Anterior femoral cutaneous nerve injury should be considered when anterior medial thigh pain and numbness occur following aortofemoral bypass graft surgery and other types of femoral artery reconstructive surgery.

Misdiagnosis in patients with amyotrophic lateral sclerosis.

To confirm our impression that a high percentage of patients with amyotrophic lateral sclerosis are initially misdiagnosed, we reviewed records of 33 patients with a definitive diagnosis of amyotrophic lateral sclerosis seen over 10 years. Fourteen patients (43%) were initially misdiagnosed. Mean time to correct diagnosis was significantly greater for the misdiagnosed group (16.

Effect of inspiratory resistance and theophylline on respiratory muscle strength in patients with amyotrophic lateral sclerosis.

The effect of breathing through inspiratory flow resistive loads ranging between 4.5 and 27.0 cm H2O/L/s was assessed in eight patients with amyotrophic lateral sclerosis (ALS) and in eight control subjects.

Short-latency somatosensory evoked potentials in brain-dead patients.

Ten adult brain-dead patients were evaluated for the presence of clearly defined median nerve short-latency somatosensory evoked potentials (SSEPs). All met clinical criteria recommended by the President's Commission report (1981), had positive apnea tests, and had electrocerebral silent EEGs. P13-P14 and N20 were absent in all scalp-scalp channels, although 3 patients showed P13-P14 in scalp-non-cephalic channels.

Apnea testing in brain death.

A standardized protocol was followed in 33 apneic oxygenation tests on 20 patients suspected of being brain dead. Spontaneous respiratory movements developed in just one patient; this patient was the only one who did not show electrocerebral silence on electroencephalography. Significant hypoxemia, hypotension, or cardiac arrhythmias were not encountered despite lung disease in 14 of our 20 patients.

Posterior rhythmic slow activity in EEG after eye closure.

During a 20 month period, EEGs in 7 patients, ages 6-16 years, showed a distinctive posterior rhythmic slow (PRS) activity brought on by eye closure. Following eye opening for 5-10 sec, eye closure on command was followed by a rhythmic high amplitude (100-250 microV) slow wave discharge of 3-4 c/sec lasting for 1.5-3 sec after a latency of 300-500 msec.