Beneficial effects of Rituximab in patients with anti-MAG (myelin-associated glycoprotein) neuropathy: case reports.

Anti-myelin-associated glycoprotein (MAG) neuropathy is a primary demyelinating sensorimotor polyneuropathy that can be very debilitating and is known to be resistant to treatment. There are only a few conflicting reports on the effect of Rituximab in anti-MAG neuropathy. We present three patients who improved remarkably with Rituximab infusions.

Phosphoglycerate mutase deficiency with tubular aggregates in a patient from Panama.

Introduction: Phosphoglycerate mutase deficiency (PGAM) is a rare metabolic myopathy that results in terminal block in glycogenolysis. Clinically, patients with PGAM deficiency are asymptomatic, except when they engage in brief, strenuous efforts, which may trigger myalgias, cramps, muscle necrosis, and myoglobinuria. An unusual pathologic feature of PGAM deficiency is the association with tubular aggregates.

Intraoperative laryngeal electromyography in children with vocal fold immobility: results of a multicenter longitudinal study.

Objectives: To determine whether laryngeal electromyography (LEMG) can predict recurrent laryngeal nerve function return in children and whether LEMG can aid in the management of vocal fold immobility (VFI).

Design: Prospective case series.

Setting: Tertiary pediatric aerodigestive centers.

Asymmetric phenotype associated with rare myelin protein zero mutation.

Myelin protein zero (MPZ) mutations cause demyelinating neuropathies that range from severe neonatal to milder adult forms. We report a 36-year-old man who developed weakness of his left little finger adduction 3 years earlier. The weakness progressed to his other limbs.

Spontaneous and evoked laryngeal electromyography of the thyroarytenoid muscles: a canine model for intraoperative recurrent laryngeal nerve monitoring.

Objectives: We sought to determine the feasibility of performing spontaneous and evoked intraoperative laryngeal electromyography (L-EMG) using nerve monitoring equipment and to compare recording electrode configurations and methods of recurrent laryngeal nerve (RLN) stimulation in dogs.

Methods: Four beagles underwent crush injury of the left RLN, and 2 beagles underwent left RLN transection. Serial spontaneous and evoked L-EMG was recorded with the NIM-Response nerve monitoring system under sedation.

Patient with amyloidoma of the ulnar nerve and salivary glands.

Amyloidomas of the peripheral nervous system have been reported rarely. We describe a patient with a history of localized amyloidosis of the salivary glands who presented a few years later with paresthesias of her left medial arm, forearm, and fifth digit. A mass affecting the left ulnar nerve was confirmed by MRI studies.

Serial electromyography of the thyroarytenoid muscles using the NIM-response system in a canine model of vocal fold paralysis.

Objectives: We sought to determine whether serial intraoperative laryngeal electromyography (L-EMG) or evoked L-EMG predicts vocal fold (VF) recovery following iatrogenic injury.

Methods: Six beagles were sedated, and bipolar needle electrodes were inserted into each thyroarytenoid (TA) muscle. Endotracheal tube surface electrodes were also placed.

Relapsing Guillain Barré Syndrome and nephrotic syndrome secondary to focal segmental glomerulosclerosis.

A 49-year-old man developed simultaneously a Guillain Barré Syndrome (GBS) and a nephrotic syndrome (NS). The patient relapsed twice, despite treatment with intravenous immunoglobulins (IVIg) after a full or partial recovery, and became resistant to IVIg. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS).

Intraoperative laryngeal electromyography in children with vocal fold immobility: a simplified technique.

Objectives: The primary objective of this study was to determine whether a simplified technique for intraoperative laryngeal electromyography was feasible using standard nerve integrity monitoring electrodes and audiovisual digital recording equipment. Our secondary objective was to determine if laryngeal electromyography data provided any additional information that significantly influenced patient management.

Methods: Between February 2006 and February 2007, 10 children referred to our institution with vocal fold immobility underwent intraoperative laryngeal electromyography of the thyroarytenoid muscles.

Myotonic dystrophy type 1 presenting with ventilatory failure.

Objective: To report a series of patients with adult onset myotonic dystrophy type 1 (DM1) in whom the presenting symptom was ventilatory failure.

Background: Ventilatory failure is a common complication of DM1 and may be a presenting symptom in the setting of anesthesia or surgery, but it is not known to be a heralding manifestation.

Method: Case series.

Multifocal motor neuropathy with conduction block: Distribution of demyelination and axonal degeneration.

Objective: Multifocal motor neuropathy with conduction block (MMN) is an immune-mediated neuropathy, characterized by progressive muscle weakness. Although demyelination is regarded as the underlying pathophysiologic mechanism of MMN, recently, it was reported that different pathophysiologic mechanisms were responsible for disease in the upper and lower limbs. Specifically, demyelination in the upper limbs and axonal loss in the lower limbs.

Acute sensory neuronopathy as the presenting symptom of Sjögren's syndrome.

Sensory neuronopathy associated with Sjögren's syndrome (SS) usually has a subacute or chronic onset. We report the case of a 37-year-old woman who presented with an unusual hyperacute form of SS ganglionopathy. She initially developed paresthesias of her fingertips and rapidly became severely ataxic.

Cervical nerve root stimulation. Part II: findings in primary demyelinating neuropathies and motor neuron disease.

Objective: Cervical nerve root stimulation (CRS) allows the assessment of conduction in the proximal segments of motor fibers destined to the upper extremities, which are not evaluated by routine nerve conduction studies (NCS). Since many primary demyelinating polyneuropathies (PDP) are multifocal lesions may be confined to the proximal nerve segments. CRS may therefore increase the yield of neurophysiologic studies in diagnosing PDP.

Cervical nerve root stimulation. Part I: technical aspects and normal data.

Objective: Cervical nerve root stimulation (CRS) is a technique of assessing the proximal segments of motor axons destined to upper extremity muscles. Few studies report normal values. The objective was to determine CMAP onset-latencies and CMAP amplitude, area, and duration changes in healthy controls for the abductor pollicis brevis (APB), abductor digiti minimi (ADM), biceps, and riceps muscles.

Multifocal motor neuropathy: decrease in conduction blocks and reinnervation with long-term IVIg.

Background: Multifocal motor neuropathy with conduction blocks (MMNCB) is an immune-mediated motor neuropathy. Previous long-term IV immunoglobulin (IVIg) treatment studies have documented improvement in muscle strength and functional disability but revealed a concomitant increase in acute axonal degeneration (AD) and conduction block (CB).

Objective: To determine the long-term effects of IVIg therapy on clinical and neurophysiologic outcome measures in MMNCB patients responsive to IVIg.

Clinical utility of electrodiagnostic consultation in suspected polyneuropathy.

Although paresthesias of the distal lower limbs are characteristic features of polyneuropathy, they may also herald the presence of a focal neuropathy, polyradiculopathy, or myelopathy. Electromyography and nerve conduction studies (EMG/NCS) are widely used in the evaluation of such symptoms, but their utility has not been subjected to vigorous scrutiny. We investigated the clinical impact of the electrodiagnostic consultation in assessing suspected polyneuropathy.

Neurophysiologic findings in early acute inflammatory demyelinating polyradiculoneuropathy.

Background: Patients with early acute inflammatory demyelinating polyradiculoneuropathy (AIDP) may not meet the current neurophysiologic criteria.

Objective: To document neurophysiologic findings in early AIDP.

Methods: Clinical and neurophysiologic data from 38 AIDP patients, assessed within 10 days of symptom onset were reviewed.

Technology literature review: quantitative sensory testing.

The development of the personal computer has simplified the process of quantitating sensory thresholds using various testing algorithms. We reviewed the technical aspects and reproducibility of different methods to determine threshold for light touch-pressure, vibration, thermal, and pain stimuli. Clinical uses and limitations of quantitative sensory testing (QST) were also reviewed.

Atypical presentations of primary amyloid neuropathy.

Primary amyloidosis (AL) may be complicated by peripheral neuropathy in 15-35% of cases. We report on four patients with atypical neurological presentations of AL neuropathy, whose diagnoses were delayed due to varied clinical presentations. The clinical presentation included painful sensory neuropathy (two patients), mononeuropathy multiplex (one patient), and primary demyelinating polyneuropathy (one patient).