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.

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.

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.

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).