Peter Siao Tick Chong

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. Methods: The authors reviewed medical records of 10 patients with MMNCB for outcomes in muscle strength (Medical Research Council score), functional disability (Modified Rankin Disability score), CB, and AD. All patients had received IVIg (2g/kg in 5 days for 3 consecutive months), followed by monthly maintenance therapy. Results: Patients were followed for an average of 7.25 years (range, 3.5 to 12 years). There was significant and sustained improvement in muscle strength and functional disability while on IVIg therapy. Furthermore, the authors found significant improvement in CB, decrease in AD, and evidence of reinnervation by the end of the follow-up period. Conclusion: Long-term IV immunoglobulin therapy improves muscle strength and functional disability, decreases the number of conduction blocks and the extent of axonal degeneration, and promotes reinnervation. These findings differ from previous reports of deterioration in neurophysiologic outcome measures. Comparison of the IV immunoglobulin regimen in those reports and this study shows that the authors’ patients were treated with significantly higher IV immunoglobulin maintenance doses. These findings have implications for the long-term treatment of patients with multifocal motor neuropathy with conduction blocks.

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. Transesophageal, percutaneous, and direct open RLN stimulation was performed. Recordings of spontaneous and evoked responses were made with endotracheal tube surface electrodes and bipolar vocal fold needle electrodes. The L-EMG procedures were repeated every 1 to 2 weeks after injury, and intersubject and intertrial differences were evaluated. Results: Low-amplitude motor unit action potentials, polyphasic potentials, fasciculations, and fibrillations were detected in injured animals with bipolar needle recording electrodes with this system of spontaneous L-EMG. The surface recording electrodes did not detect pathologic waveforms. Percutaneous needle stimulation of the RLN is possible at currents slightly higher than those used for direct stimulation. Consistent, discrete, transesophageal stimulation of the RLN could not be reliably performed. Recording evoked responses with needle electrodes generated sharper waveforms, facilitating calculation of latency and wave duration. Evoked L-EMG utilizing surface recording electrodes limited the intertrial and intersubject variability of evoked amplitude. Conclusions: Typical patterns of nerve injury can be detected with this system of intraoperative L-EMG in a canine model. Quantitative measures of amplitude, latency, and wave duration in healthy and injured canine RLNs may be determined with this system.

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. Consequently, the aim of the present study was to assess, through clinical neurophysiology studies, whether different pathophysiologic mechanisms were occurring in the upper and lower extremities. Furthermore, we wanted to investigate whether the presence of conduction block (CB) correlated with axonal degeneration (AD), and to determine the electrophysiological abnormalities that correlate with muscle weakness. METHODS We reviewed medical records of 18 patients with MMN for clinical features (using the Medical Research Council score and Guys Neurology Disability Scale) and neurophysiologic abnormalities (CB, AD prolongation of distal motor and F-wave latencies, and reduction of conduction velocity in the demyelinating range). RESULTS Electrophysiological abnormalities deemed specific of demyelination were non-significantly different in the upper and lower extremities. The presence of axonal degeneration correlated significantly with conduction block (odds ratio 10.4, 95% CI 4.2-25.6), and both parameters correlated with muscle weakness (P<0.01). CONCLUSION Our study suggests that the same pathophysiologic process occurs in the upper and lower extremity nerves. Moreover, one pathophysiologic process may be responsible for the development of CB and AD, and therefore muscle weakness. SIGNIFICANCE The present study has established that both AD and CB occur in MMN, irrespective of extremity, and both correlate with muscle weakness.

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). The latter two types of presentation have not been reported previously. The diagnosis was established by fat pad biopsy in two patients, lymph node biopsy in one, and sural nerve biopsy in one. Two patients were treated with high‐dose melphalan followed by stem cell rescue, and one was treated with oral melphalan and prednisone. All three cases experienced stabilization of neuropathic symptoms. We report these cases in order to raise awareness of the varied clinical presentation of AL neuropathy. Muscle Nerve 28: 696–702, 2003

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. PATIENTS Twenty-five children aged 14 days to 7 years at the time of first LEMG (mean age, 21.4 months) with VFI who underwent flexible fiberoptic laryngeal examination, intraoperative LEMG of the thyroarytenoid muscles, and 12-month follow-up. MAIN OUTCOME MEASURES To compare results of LEMG with flexible fiberoptic laryngeal examination in children with vocal fold paresis and to determine if LEMG can predict vocal fold return. RESULTS In children who had a patent ductus arteriosus ligation, the LEMG data suggest that if there is no activity 6 months after injury, then the nerve is unlikely to regain function. In 3 of 3 children with central causes of VFI, normal LEMG findings predicted return of nerve function 2 to 7 months before vocal fold movement on fiberoptic examination. Finally, in 3 of 3 children with idiopathic VFI, LEMG predicted return within 2 to 14 months of vocal folds with normal findings. CONCLUSIONS Intraoperative LEMG is a safe, easy-to-use method for determining the likelihood of recurrent laryngeal nerve function return in children who have undergone patent ductus arteriosus ligation, in children with centrally correctable lesions, and in children with idiopathic VFI. More work is needed in the area of pediatric LEMG, but it is possible that LEMG data can be used to aid in management strategies and provide families with more information to make better informed decisions regarding their childs care.

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. As the sedation lightened, L-EMG activity was recorded from all electrodes with an intraoperative nerve monitoring system. The neck was opened, and direct recurrent laryngeal nerve (RLN) stimulation was performed. Subsequently, 4 animals underwent crush injury of the left RLN, and 2 animals underwent nerve transection. The L-EMG procedures were repeated every 1 to 2 weeks until left VF motion was observed in the dogs that suffered RLN crush injury. At each time point, the neck was opened and both RLNs were stimulated. Results: Fibrillation potentials were detected in all animals after RLN injury. A change to electrical silence was seen in the animals in the crush injury group that were evaluated during the week preceding VF recovery. Fibrillation potentials and VF immobility persisted in the transection group throughout the complete time course of these experiments. The first appearance of an evoked response coincided with or occurred after the return of left VF motion in the crush injury group. The threshold, latency, and amplitude differed from those of the controls and approached normal values over time. No response was detected in the transected nerves. Conclusions: The disappearance of fibrillations on intraoperative L-EMG was noted in the animals tested the week before the return of VF motion, and the return of motor unit action potentials was seen along with return of VF function. Evoked L-EMG was not helpful in predicting the return of VF mobility, but it may help quantify degrees of RLN injury and predict the speed of recovery.

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. METHODS We reviewed clinical and neurophysiologic data from 38 PDP patients and compared them to 35 patients with motor neuron disease (MND), and 21 healthy controls (HC). RESULTS Mean onset-latency was significantly prolonged in PDP patients. The optimal onset-latency cutoff necessary to distinguish PDP from MND and controls was 17.5 ms for the abductor pollicis brevis (APB) and abductor digiti minimi (ADM), and 7 ms for Biceps and Triceps. Mean reduction in proximal to distal CMAP amplitude to APB and ADM was significantly greater in PDP patients, with an optimal cutoff in proximal to distal CMAP amplitude reduction necessary to distinguish PDP from MND and HC being 45%. CONCLUSIONS CRS is effective in distinguishing PDP from MND and HC based on prolonged onset latency and conduction block criteria. SIGNIFICANCE CRS may increase the diagnostic yield in cases where demyelinating lesions are confined to the proximal peripheral neuraxis.

Acute sensory neuronopathy as the presenting symptom of Sjogren's syndrome

Sensory neuronopathy associated with Sjögrens 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. Nerve conduction studies revealed abnormal sensory but normal motor functions. Lip biopsy showed findings consistent with SS. Sural nerve biopsy showed severe axonal loss. The patient showed modest improvement with immunosuppressive therapies.

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). He responded to plasmapheresis and corticosteroids with simultaneous recovery of his GBS and NS, suggesting a common pathogenesis of the two conditions.

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. Examination revealed mildly high-arched feet with asymmetric weakness of ulnar-innervated muscles (left > right) and asymmetric weakness of peroneal-innervated muscles (right > left). Motor nerve conduction velocities ranged from 18.4 to 24.4 m/s in the upper extremities and from 14.8 to 22.7 in the lower extremities. Left median partial motor conduction block was noted at the forearm segment. Genetic testing demonstrated MPZ mutation with ARG98HIS amino acid change. The patients father is a 68-year-old man who was asymptomatic and who was noticed to have high-arched feet and asymmetric leg muscle atrophy and weakness (right > left). The patients 2-year-old son is “clumsy” with history of neonatal laryngomalacia. He has flat feet, areflexia, and difficulty standing on individual right versus left leg. The patients paternal grandfather had high-arched feet and hearing loss. We conclude that ARG98HIS MPZ mutation may cause hereditary and relatively mild and asymmetric demyelinating sensorimotor polyneuropathy.