False-Positive Serum Botulism Bioassay in Miller-Fisher Syndrome.

We describe a patient with acute progressive weakness and areflexia. Both botulism and Miller-Fisher variant of Guillain-Barré syndrome were initial diagnostic considerations, and she was treated with intravenous immunoglobulin and botulinum antitoxin. A mouse bioassay was positive for botulinum toxin A, although her clinical course, electrodiagnostic studies, and cerebrospinal fluid findings supported Miller-Fisher syndrome.

Contraction induced h reflexes in the diagnosis of cervical radiculopathy.

Purpose: To determine whether Contraction Induced H Reflexes (CIHR) can accurately detect cervical radiculopathy.

Methods: Comparison of CIHR results with Needle Electromyography at academic outpatient Electromyography/Nerve Conduction laboratories. Participants were all patients over 18 with a needle electromyography diagnosis of cervical radiculopathy.

Hereditary motor and sensory neuropathy with proximal predominance (HMSN-P).

Hereditary motor and sensory neuropathy with proximal predominance (HMSN-P) is a rare disorder inherited in an autosomal dominant fashion. Patients present with slowly progressive proximal-predominant weakness, painful muscle cramps, fasciculations, large-fiber sensory loss, and areflexia. Electrodiagnostic (EDX) studies typically reveal abnormalities consistent with a sensorimotor neuronopathy.

Postpartum paraparesis from spinal neurofibroma.

Background Context: One hallmark of neurofibromatosis Type 1 (NF1) is the presence of multiple neurofibromas, which are hormonally responsive and may undergo rapid growth during periods of hormonal surge. Although spinal neurofibromas occur in up to 40% of patients with NF1, they rarely cause neurologic sequelae, especially in the young.

Purpose: To document a unique case of acute postpartum paraparesis in a young woman because of an extradural neurofibroma compressing the conus medullaris and discuss the possible hormonal influences related to rapid growth of this otherwise "benign" nerve sheath tumor.

Increased prevalence of temporary cardiac pacing in people with epilepsy.

Introduction: Even though ictal tachyarrhythmias are more common, ictal brady-asystole is more likely to be fatal, and yet is potentially preventable with pacemaker (PM) implantation. We sought to quantify the degree of association of PM placement in people with and without epilepsy, including neurological and cardiovascular cohorts.

Methods: Retrospective cross-sectional analysis of the National Hospital Discharge database using International Classification of Diseases Clinical Modification (ICD-9-CM) codes.

The mathematical relationship between height and nerve conduction velocity.

Many studies have shown an inverse relationship between axon length (or height) and nerve conduction velocity. A linear relationship was assumed, but there is no physiologic indication the relationship is linear. Furthermore, a linear relationship between height and velocity leads to implausibly low velocities for very long nerves.

Validating an Ataxia Functional Composite Scale in spinocerebellar ataxia.

The Ataxia Functional Composite Scale (AFCS) may provide a sensitive and reproducible assessment of treatment responses in studies of the spinocerebellar ataxias (SCA). We previously assessed the effects of buspirone in a cohort of patients with SCA via the International Cooperative Ataxia Rating Scale (ICARS). At each assessment period, AFCS scores were also obtained.

Treatment of spinocerebellar ataxia with buspirone.

Preliminary data suggest potential benefit of 5-HT receptor agonists in the treatment of ataxias. We studied the effects of buspirone in a cohort of twenty patients with spinocerebellar ataxia (SCA). Twenty patients were treated in this double-blind, placebo controlled, cross-over trial with either buspirone HCl 30 mg twice daily or placebo for 3 months.

Adie's pupils in paraneoplastic ganglionopathy with ANNA-1.

Autonomic disturbances are common in patients with paraneoplastic syndromes associated with type-1 antineuronal nuclear autoantibodies (ANNA-1), although pupillary disturbances are infrequent. The authors describe a patient with ANNA-1 associated paraneoplastic sensory neuronopathy and bilateral Adie's pupils.

A sensitive new median-ulnar technique for diagnosing mild Carpal Tunnel Syndrome.

Carpal Tunnel Syndrome (CTS) is easily the most common focal peripheral nerve compression. The primary diagnostic tool is electrodiagnosis, although 13-27% of patients with symptoms and signs of CTS have normal electrodiagnostic results. The goal of this study was to create a more sensitive and specific latency difference criteria without any additional testing beyond the minimum.

Clinical and magnetic resonance imaging manifestations of neurosarcoidosis.

Objectives: To describe clinical and neuroimaging manifestations of neurosarcoidosis in a cohort of 21 patients.

Patients And Methods: We reviewed records of 21 patients with sarcoidosis and central nervous system (CNS) manifestations referred to Cooper University Hospital, with emphasis on neuroimaging findings and associated clinical and laboratory evidence of sarcoidosis. Nineteen patients were categorized as having "definite," "probable," or "possible" neurosarcoidosis, while 1 had associated CNS vasculitis and another had Hodgkins lymphoma with cauda equina syndrome.

Age and the severity of carpal tunnel syndrome.

Several studies have shown an increased incidence of Carpal Tunnel Syndrome (CTS) with increasing age, as well as a longer Median sensory latency in older CTS patients. In this study, data was analyzed from 19 patients with bilaterally normal UE EMG/NCS and no symptoms of CTS (38 hands), as well as 18 patients with unilateral CTS and 21 with bilateral CTS (60 hands) to determine the effect of age on the severity of nerve conduction abnormalities associated with CTS. Most of these parameters showed increasing severity with age.

Cardiac disease in myasthenia gravis: a literature review.

Patients with myasthenia gravis might develop various cardiac disorders, yet a causal relationship remains unestablished. Because causes of sudden death in this population have not been ascertained, further attention to possible cardiac disease in this population is warranted. We summarize the current literature and describe the possible etiologies and implications of cardiac disease in myasthenics.

Consensus criteria for the diagnosis of multifocal motor neuropathy.

At this time, there are no widely accepted criteria for the diagnosis of multifocal motor neuropathy. Furthermore, there is insufficient empirical data to define clinical and laboratory features that may reliably separate certain lower motor neuron syndromes with overlapping features as distinct. The AAEM therefore developed five criteria through a formal consensus process that are described in this document to act as a guide for diagnosing multifocal motor neuropathy with a high level of confidence (definite multifocal motor neuropathy) or with a moderate level of confidence (probable motor neuropathy).

Stupor from lamotrigine toxicity.

Purpose: To report that acute lamotrigine poisoning may result in severe encephalopathy.

Methods: A 55-year-old woman became stuporous after overdose with lamotrigine (LTG) and valproic acid (VPA) tablets. Evaluation revealed elevated serum LTG levels and no other etiology for encephalopathy.

Clinical Approach to Neuromuscular Weakness in the Critically III Patient.

Neuromuscular disorders developing m critically ill patients often result in increased morbidity and prolonged intensive care hospitalization Detection and assessment of affected patients are frequently hindered by the severe nature of the underlying medical illness. The various syndromes have overlapping clinical and electrodiagnostic features, presenting further obstacles to prompt classification. Nonetheless, an accurate diagnosis can usually be attained through an organized approach to the evaluation and management of critically ill patients with acquired weakness.

Peroneal neuropathy from antithrombotic stockings.

We report an unusual association of peroneal mononeuropathy at the fibular head and the use of antithrombotic stockings. A patient developed clinical evidence of a peroneal nerve deficit temporally related to the use of a tight-fitting antithrombotic stocking. Nerve conduction studies and needle electromyography were performed.

Mononeuropathies associated with liver transplantation.

Mononeuropathies associated with orthotopic liver transplantation were evaluated in a prospective manner. Ten percent of liver transplant recipients were noted to have focal peripheral nerve lesions in the postoperative period. The ulnar nerve was most commonly involved, with intraoperative compression or postoperative trauma as possible mechanisms of injury.

Acute myopathy after liver transplantation.

Acute myopathy is a cause of weakness and additional morbidity in a variety of critically ill patients, including transplant recipients. We report the incidence of and risk factors associated with acute myopathy after orthotopic liver transplantation (OLTx). One hundred consecutive adult patients were prospectively assessed for muscle weakness after OLTx.

Percutaneous needle muscle biopsy in the evaluation of patients with suspected inflammatory myopathy.

Objective: To determine the usefulness of a unique method of percutaneous needle muscle biopsy (NMB) in patients with suspected idiopathic inflammatory myopathy (IIM).

Methods: The yield of percutaneous NMB was studied in 55 patients who were found to have a combination of clinical, laboratory, or electromyographic features of IIM.

Results: A diagnosis of IIM was confirmed histopathologically in 29 patients (53%), other specific myopathies were found in 5 (9%), nonspecific myopathic changes were present in 11 (20%), and a neurogenic process was diagnosed in 3 (5%).