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Biomedical subjects

Louis H Weimer

Publications and source records attributed to Louis H Weimer.

11 recordsLinked to original sources
PubMed2006

Multifocal motor neuropathy with conduction block: slow but not benign.

OBJECTIVE: To describe a patient with multifocal motor neuropathy with conduction block who had annual clinical and physiological examinations for 18 years but declined treatment for personal reasons. DESIGN: Case report. SETTING: Collaboration between 2 academic tertiary care hospitals. Patient One patient with multifocal motor neuropathy with conduction block. RESULTS: At age 44 years, there was weakness and wasting of the left biceps with conduction block in the left musculocutaneous and right ulnar nerves. The left median nerve was inexcitable. The right median, ulnar, and left peroneal nerves developed axonal change (loss of distal compound muscle action potential amplitude) at years 5, 12, and 13. By 2005, new weakness had appeared in 20 muscles (16 in the arms); he could not use a keyboard, button buttons, or write his name. Nerves that initially showed conduction block became inexcitable over the course of the illness. CONCLUSIONS: Multifocal motor neuropathy with conduction block is a disease that may be "only" slowly progressive but is not always benign. Nerves showing conduction block may develop axonal change. Better markers for this disease are needed.

Action Potentials↗
PubMed2006

Immune-mediated autonomic neuropathies.

Improved recognition and availability of noninvasive testing of autonomic disorders has prompted a better understanding of disease mechanisms of some disease forms, especially potentially treatable immune-mediated autonomic neuropathies. Development is acute, subacute, or less commonly chronic. Autonomic involvement is common and an important cause of morbidity and mortality in Guillain-Barré syndrome. Acute autonomic neuropathy can affect parasympathetic, sympathetic, and enteric nerves or neurons and is associated with antibodies to ganglionic nicotinic acetylcholine receptors. These antibodies appear to be causative based on a rabbit immunization model and serum transfer studies from patients and animals. Other important immune autonomic disorders discussed include Lambert-Eaton myasthenic syndrome, some forms of orthostatic intolerance, chronic autonomic neuropathy, and Sjögren syndrome. Paraneoplastic autonomic disorders are clinically indistinguishable and associated with various overlapping antibody associations, including anti-Hu (ANNA-1), ganglionic acetylcholine receptors, CRMP-5, and PCA-2.

Animals↗
PubMed2005

Medication-induced exacerbation of neuropathy in Charcot Marie Tooth disease.

Toxin or medication-induced worsening of preexisting peripheral neuropathy is a generally accepted but not well-studied phenomenon in humans. Drug-induced exacerbation of Charcot Marie Tooth disease (CMT) neuropathy is a common concern; a list of potential drugs to avoid is maintained by the CMT Association but with limited direct evidence or advice on relative risk. An extensive literature search for reported cases of drug effects in CMT patients found the vast majority concerned excessive vincristine toxicity in patients with undiagnosed demyelinating forms of CMT, many after 1 or 2 doses. The CMT North American database was also queried for all drug-related effects. All but one drug cited as worsening neuropathy was present on a compiled inclusive list. These results and other available evidence were used to develop a revised risk stratified list for CMT patients and clinicians to consult prior to discussing risk to benefit ratios and making treatment decisions.

Antineoplastic Agents, Phytogenic↗
PubMed2005

Rituximab treatment of an IgM monoclonal autonomic and sensory neuropathy.

Rituximab, a monoclonal antibody against B-cell membrane marker CD-20, is an effective treatment for immunoglobulin M (IgM) monoclonal anti-myelin-associated glycoprotein (MAG) neuropathies. We report a patient with an autonomic and painful sensory neuropathy associated with an IgM lambda monoclonal gammopathy, responsive to rituximab. Treatment resulted in a decline in total IgM and improvement in the patient's painful neuropathy and dysautonomia. Rituximab may be an effective and tolerable treatment for autonomic and sensory neuropathy associated with IgM monoclonal gammopathy.

Aged↗
PubMed2005

Medication and toxin-induced peripheral neuropathy.

Medication and toxin-induced neuropathies, although uncommon, are important to identify because of potential reversibility. Numerous medications and toxins are implicated with neuropathy, but objective proof is lacking for many. Chemotherapeutic agents, nucleoside analogs, and other medications and toxins have clear causative links with neuropathy, but many agents have only rare temporal associations. Neuropathies are being recognized from new medications approved for use and other medications, which have expanded indications. Some established associations with neuropathy are less widely known while others are overestimated. Peripheral neuropathy from chronic drug exposure is more problematic to determine. Axonal pathology is most common, but some agents cause demyelination and even conduction block, mimicking immune-mediated neuropathies. Multiple and varied toxic mechanisms are implicated. Neuropathy is a dose-limiting complication of some critical treatments and preventative agents are being actively pursued.

Anti-Bacterial Agents↗
PubMed2004

Gene expression profiling in chronic inflammatory demyelinating polyneuropathy.

Gene expression in archived frozen sural nerve biopsies of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) was compared to that in vasculitic nerve biopsies (VAS) and to normal nerve (NN) by DNA microarray technology. Hierarchical clustering analysis demonstrated distinct gene expression patterns distinguishing these disease groups. Of particular interest were: (1) Tachykinin precursor 1, which may be involved in pain mediation; (2) Stearoyl-CoA-desaturase, which may be a marker for remyelination and (3) the Allograft Inflammatory Factor 1 (AIF-1), a modulator of immune response during macrophage activation. Differential gene expression may help distinguish between CIDP, VAS and NN in sural nerve biopsies and identify genes that may be involved in disease pathogenesis.

Adult↗
PubMed2003

Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy.

BACKGROUND: Current electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) are research oriented favoring specificity over sensitivity. Application of such criteria in clinical practice may miss the diagnosis in potentially treatable patients. OBJECTIVES: To analyze the electrophysiologic abnormalities in a cohort of patients with clinically defined CIDP, to compare these data with published electrodiagnostic criteria, and to identify a set of abnormalities that is shared by all patients with CIDP. DESIGN: Retrospective medical record review. SETTING: Academically based neuromuscular clinic. Patients Fifteen patients with clinically diagnosed relapsing sensorimotor CIDP. INTERVENTIONS: Administration of intravenous immunoglobulin or prednisone. MAIN OUTCOME MEASURES: Electrodiagnostic studies. RESULTS: All patients had electrodiagnostic abnormalities in at least 3 nerves with possible partial conduction block or demyelinating range abnormalities in at least 1 nerve. The diagnostic sensitivities of 5 published CIDP criteria were as follows: the Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force (40%), Saperstein et al (47%), Nicolas et al (53%), Hughes et al for the Inflammatory Neuropathy Cause and Treatment Group (60%), and Thaisetthawatkul et al (70%). CONCLUSIONS: Current electrodiagnostic criteria for CIDP are insensitive and may fail to diagnose the condition in a substantial number of patients. More inclusive criteria that allow identification of patients in routine clinical practice are needed.

Action Potentials↗
PubMed2003

Medication-induced peripheral neuropathy.

Although not very common, medication-induced neuropathy is a treatable condition and, therefore, is important to identify. Medications continue to grow in number and expand in usage; consequently, toxic neuropathy continues to be relevant to neurologists. Many agents have toxicities that are tolerated because the treatments are necessary, such as therapies for HIV and malignancy. Additional agents to prevent or ameliorate the toxic neuropathy are being sought and trials are ongoing. Certain patients, however, may be at high risk for peripheral nerve toxicity due to genetic factors or another underlying neuropathy. Newer drug-delivery methods, such as viral transfection, may produce less toxicity in the future. The underlying pathomechanisms remain incompletely elucidated; however, apoptosis is emerging as an important final pathway in some forms of toxic neuropathy. Although most cases demonstrate acute or subacute onset after exposure, recent experiences with statin drugs raise the possibility of occult toxic causes of chronic idiopathic neuropathy.

Animals↗
PubMed2003

Syncope and orthostatic intolerance.

Syncope and orthostatic intolerance remain common and significant clinical problems with many undocumented, misdiagnosed, or cryptogenic cases. Careful clinical assessment and application of advancing laboratory support can further improve diagnosis and treatment. Despite the depth of existing research into these common problems, many underlying mechanisms remain unproven.

Adolescent↗
PubMed2002

Serial studies of carpal tunnel syndrome during and after pregnancy.

Carpal tunnel syndrome (CTS) is a frequent and underdiagnosed complication of pregnancy. Conservative therapies are common initial measures, but data on the course of improvement are limited. We report a case of pregnancy-associated CTS with unusually detailed serial electrophysiologic studies before and after wrist splinting. Physiologic measures reached a nadir and then rapidly improved following conservative therapy, paralleling clinical improvement. Responses took between 6 and 20 months postpartum to approach baseline values.

Action Potentials↗