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Biomedical subjects

C Michel Harper

Publications and source records attributed to C Michel Harper.

15 recordsLinked to original sources
PubMed2005

Pulmonary function tests and blood gases in worsening myasthenia gravis.

The aim of this study was to determine whether pulmonary function tests have a role in predicting the need for ventilation support in myasthenia gravis. Medical records were reviewed for 42 patients with severe myasthenia gravis who required 55 admissions to the intensive care unit. Patients with a vital capacity of more than 20 ml/kg, a maximal expiratory pressure more than 40 cm H(2)O, or a maximal inspiratory pressure more negative than -40 cm H(2)O are unlikely to require mechanical ventilation. A decline of 30% or more in maximal inspiratory pressure predicted a group at higher risk of requiring mechanical or noninvasive ventilation. Hypercapnia was frequent and was more common in patients who required mechanical ventilation. Worsening of these pulmonary function and blood gas values in patients with myasthenia gravis may guide decisions about intubation and ventilatory support.

Blood Gas Analysis↗
PubMed2005

Preoperative and intraoperative electrophysiologic assessment of brachial plexus injuries.

Preoperative and intraoperative electrophysiologic studies complement one another and complement information obtained from clinical and imaging studies in brachial plexus injury. The information provided by electrodiagnostic studies helps to better define the number, location, and severity of plexus lesions. In addition, establishing the presence or absence of nerve root continuity and early regeneration through postganglionic lesions helps to direct surgical therapy.

Brachial Plexus↗
PubMed2005

Monotonicity of nerve tests in diabetes: subclinical nerve dysfunction precedes diagnosis of polyneuropathy.

OBJECTIVE: The objective of this study was to test whether monotone worsening of nerve function, attributable to diabetes, can be demonstrated before criteria for diabetic sensorimotor polyneuropathy (DSPN) have been met. Which nerve tests are best? RESEARCH DESIGN AND METHODS: From a prevalence cohort of 504 individuals in the Rochester Diabetic Neuropathy study (RDNS), we identified 238 individuals (group 1) who at first examination were without polyneuropathy (DSPN) by a sum score of the normal deviates (from percentiles) of five attributes of nerve conduction of the legs (i.e., their five nerve conduction normal deviate values were <97.5th percentile) and were followed longitudinally two or more times. Of these 238, 90 (group 2) were followed six or more times at yearly or bi-yearly intervals. We compared different nerve tests for the ones most sensitive and reliable in showing latent nerve dysfunction and monotone (the extent to which a variable measured repeatedly over time reveals a significant trend of worsening or improvement). RESULTS: In group 1 patients, the mean sum score of five attributes of nerve conduction (sigma 5 NC nds) at baseline was 1.08 and at the last examination (only patients with Sigma 5 NC nds <97.5th percentile) was 3.63, markedly higher than that in healthy subjects (only of individuals with Sigma 5 NC nds <97.5th percentile) (-0.12), indicating a subtle latent shift of nerve conduction tests toward abnormality. Serial evaluations of many individual and especially sum scores of nerve conduction tests in group 2 patients showed statistically significant worsening with time, even when nerve conduction tests were still well within normal limits. Neurologic signs also worsened but barely to significant levels; however, symptoms and quantitative sensation tests did not. Considering the composite score sigma 5 NC nds, 42 (of 90 group 2 patients) showed significant worsening, 22 were still without DSPN by nerve conduction test criteria, and some were even below the 50th percentile at the last evaluation. CONCLUSIONS: Subtle and latent functional worsening of nerve conduction can be demonstrated even before nerve conduction test criteria for DSPN have been met. For demonstrating monotone worsening, the order (from best to worst) of tests was: some composite scores of nerve conduction and individual attributes of nerve conduction. We did not show monotone worsening of symptoms or of quantitative sensation test results. In multivariate analysis of risk factors and their association with worsening sigma 5 NC nds, 24-h microalbuminuria (a marker of microvessel disease) was found to be a significant covariate, an indication that the asymptomatic alterations of nerve conduction are meaningful.

Adult↗
PubMed2004

Intraoperative cranial nerve monitoring.

The purpose of intraoperative monitoring is to preserve function and prevent injury to the nervous system at a time when clinical examination is not possible. Cranial nerves are delicate structures and are susceptible to damage by mechanical trauma or ischemia during intracranial and extracranial surgery. A number of reliable electrodiagnostic techniques, including nerve conduction studies, electromyography, and the recording of evoked potentials have been adapted to the study of cranial nerve function during surgery. A growing body of evidence supports the utility of intraoperative monitoring of cranial nerve nerves during selected surgical procedures.

Action Potentials↗
PubMed2004

Trigeminal nerve repetitive stimulation in myasthenia gravis.

The aim of this study was to evaluate the utility of repetitive nerve stimulation (RNS) of the trigeminal nerve in assessing patients with myasthenia gravis (MG). In 26 normal controls and 21 patients with myasthenia gravis (MG), 2-Hz repetitive stimulation of the trigeminal nerve was performed using a monopolar needle for percutaneous nerve stimulation and recording over the surface of the masseter. In the MG patients, repetitive stimulation of the ulnar, spinal accessory, and facial nerves was also performed. The mean percent decrement in the compound muscle action potential (CMAP) amplitude among the different nerves at rest were: ulnar, 4.3%; spinal accessory, 10.1%; facial, 14%; and trigeminal, 17.3%. The facial nerve demonstrated abnormal decrement in 57% of all patients, compared with the spinal accessory (48%), trigeminal (43%), and ulnar (20%) nerves. All patients tolerated trigeminal RNS better than or as well as facial RNS. The study demonstrates that trigeminal RNS is a safe, reliable, efficient, and well-tolerated technique that provides another cranial nerve-muscle combination that can be used to supplement repetitive stimulation of other limb or cranial nerves in the evaluation of patients with bulbar or generalized MG.

Accessory Nerve↗
PubMed2004

Intraoperative electromyographic monitoring of the recurrent laryngeal nerve in reoperative thyroid and parathyroid surgery.

BACKGROUND: Injury to the recurrent laryngeal nerve (RLN) is a rare complication of initial thyroid and parathyroid surgery, but the prevalence is much higher in the reoperative setting. The use of continuous, intraoperative electromyographic monitoring of the RLN has been suggested to improve the safety of cervical explorations. METHODS: Outcomes of a group of reoperative thyroid and parathyroid cases that used EMG monitoring with endoscopically applied hook-wire electrodes were compared with a group of cervical reoperations without monitoring. Office laryngoscopy (indirect or fiberoptic) was used to evaluate and follow suspected RLN complications. RESULTS: Electromyography was used in 52 cervical reexploration procedures. Patients averaged 1.8 previous explorations (range, 1-7 explorations) and underwent procedures for parathyroid (31%) and/or thyroid (77%) disease (overall, 72% malignant). The non-monitored group had 59 patients with similar characteristics. Only 1 permanent nerve complication in each group was unintended (electromyography, 1.9%; non-electromyography, 1.7%). Seven false-negative and 2 false-positive electromyographic findings occurred. No complications resulted from placement of the electromyography electrodes. CONCLUSIONS: Intraoperative electromyographic monitoring of the RLN in reoperative neck surgery can be performed safely but did not decrease RLN complications in this study. Experience and routine nerve exposure remain crucial to the minimization of RLN complications.

Adolescent↗
PubMed2004

Congenital myasthenic syndromes.

Congenital myasthenic syndromes are genetic disorders of neuromuscular transmission that should be considered in the differential diagnosis of seronegative myasthenia gravis and other neuromuscular disorders. They are present at birth but may not manifest until childhood or adult life. A classification system of congenital myasthenic syndromes based on molecular genetics is under evolution. Clinical and neurophysiological correlations with molecular studies have defined diagnostic criteria that assist the clinician in identifying specific clinical myasthenic syndromes. Some types of congenital myasthenia (e.g., slow-channel and fast-channel syndrome, acetylcholinesterase deficiency, and choline acetyltransferase deficiency) can be identified by clinical features, response to cholinesterase inhibitors, and standard electrodiagnostic studies. The molecular genetics, pathogenesis, clinical features, differential diagnosis, natural history, and treatment of well-characterized congenital myasthenic syndromes are discussed.

Acetylcholinesterase↗
PubMed2003

Treatment of slow-channel congenital myasthenic syndrome with fluoxetine.

The authors found that fluoxetine significantly shortens at 5 microM/L and nearly normalizes at 10 microM/L the prolonged opening bursts of slow-channel congenital myasthenic syndrome (SCCMS) acetylcholine receptors (AChR) expressed in fibroblasts. Prompted by this observation, they treated two SCCMS patients allergic to quinidine with up to 80 to 120 mg of fluoxetine per day over 3 years (serum fluoxetine + norfluoxetine levels 8 to 11 microM/L). Both patients showed marked subjective and objective improvement by quantitative muscle strength testing and electromyography.

Adult↗
PubMed2003

Individual attributes versus composite scores of nerve conduction abnormality: sensitivity, reproducibility, and concordance with impairment.

Composite scores may be more sensitive and reproducible than single attributes of nerve conduction for detection of peripheral neuropathy, but this requires validation in large patient cohorts. Also, the concordance of individual attributes versus composite scores with clinical measures of severity has not been tested. Here, we study these issues in prospectively studied cohorts: diabetic patients from Rochester, Minnesota (RDNS; n = 396); chronic inflammatory demyelinating polyneuropathy (CIDP) patients (n = 55); and multifocal motor neuropathy (MMN) patients (n = 18). With specificity fixed at the 97.5 percentile, we found that, in generalized polyneuropathies (diabetic and CIDP), composite scores (especially ones including conduction velocity, distal latencies, and F-waves) of individual or multiple nerves tended to be more sensitive than individual attributes. By contrast, for multiple mononeuropathies, some individual attributes or composite scores of individual nerves were more sensitive than composite scores. In diabetic polyneuropathy, composite scores tended to be more reproducible than individual attributes of nerve conduction. Highly significant correlations were found between individual attributes or composite scores and neurologic impairment in diabetic polyneuropathy and in CIDP; in general, correlation coefficients were higher for composite scores. These correlations were higher for amplitudes than for conduction velocities or distal latencies. We conclude that, with the availability of microprocessors and normative databases, electromyographers may increasingly seek to express nerve conduction abnormality also as composite scores of individual or several nerves.

Diabetic Neuropathies↗
PubMed2003

Needle EMG of abductor hallucis and peroneus tertius in normal subjects.

Normal values for standard concentric-needle electromyography (EMG) of the abductor hallucis (AH) and peroneus tertius (PT) muscles have not been established to date, yet both are potentially useful muscles in the diagnosis of length-dependent peripheral nerve disorders. Forty-three normal asymptomatic subjects stratified by age underwent a standard set of nerve conduction studies and concentric-needle examination to exclude asymptomatic disease and develop normal values for the AH and the PT for various EMG parameters. Fibrillation potentials were observed in the AH in 21% of normal subjects (10% < 60 years old, 30% > 60 years old); 19% of subjects were unable to voluntarily activate the AH. All subjects could activate the PT regardless of age. No fibrillation potentials were observed in subjects < 60 years of age, whereas 9% of those older than 60 years had mild fibrillation potentials in the PT. The PT is a useful muscle in the evaluation of length-dependent peripheral neuropathy and other peripheral nerve disorders of the lower extremities, on the basis of ease of motor unit potential activation and analysis, and relative infrequency of fibrillation potentials in normal subjects.

Action Potentials↗
PubMed2002

Rapsyn mutations in humans cause endplate acetylcholine-receptor deficiency and myasthenic syndrome.

Congenital myasthenic syndromes (CMSs) stem from genetic defects in endplate (EP)-specific presynaptic, synaptic, and postsynaptic proteins. The postsynaptic CMSs identified to date stem from a deficiency or kinetic abnormality of the acetylcholine receptor (AChR). All CMSs with a kinetic abnormality of AChR, as well as many CMSs with a deficiency of AChR, have been traced to mutations in AChR-subunit genes. However, in a subset of patients with EP AChR deficiency, the genetic defect has remained elusive. Rapsyn, a 43-kDa postsynaptic protein, plays an essential role in the clustering of AChR at the EP. Seven tetratricopeptide repeats (TPRs) of rapsyn subserve self-association, a coiled-coil domain binds to AChR, and a RING-H2 domain associates with beta-dystroglycan and links rapsyn to the subsynaptic cytoskeleton. Rapsyn self-association precedes recruitment of AChR to rapsyn clusters. In four patients with EP AChR deficiency but with no mutations in AChR subunits, we identify three recessive rapsyn mutations: one patient carries L14P in TPR1 and N88K in TPR3; two are homozygous for N88K; and one carries N88K and 553ins5, which frameshifts in TPR5. EP studies in each case show decreased staining for rapsyn and AChR, as well as impaired postsynaptic morphological development. Expression studies in HEK cells indicate that none of the mutations hinders rapsyn self-association but that all three diminish coclustering of AChR with rapsyn.

Adult↗
PubMed2002

The natural history of long thoracic and spinal accessory neuropathies.

A cohort of 106 patients with electrodiagnostically confirmed long thoracic neuropathy (50 patients) or spinal accessory neuropathy (56 patients) seen at the Mayo Clinic over a 22-year period were retrospectively studied to better understand the natural history of these disorders and to determine the role of electrodiagnostic testing in predicting prognosis. Mean follow-up was 48 and 50 months, respectively. Good functional recovery was generally observed regardless of the results of electrodiagnostic studies, but improvement in the amplitude of the spinal accessory compound muscle action potential on serial nerve conduction studies tended to predict a good outcome. No electrodiagnostic findings correlated with poor outcome. Traumatic neuropathies generally did worse than neuropathies of other causes. In spinal accessory neuropathies, involvement of the dominant limb, scapular winging, and impaired arm elevation were associated with a poor outcome. Our data suggest that, contrary to other focal neuropathies, the electrodiagnostic findings do not predict functional outcome in these neuropathies.

Accessory Nerve↗
PubMed2002

Intraoperative electromyographic monitoring during periacetabular osteotomy.

Periacetabular osteotomy has become the procedure of choice in many centers for the treatment of symptomatic hip dysplasia. Intraoperative real-time nerve monitoring has been advocated during acetabular fracture repair and complex total hip arthroplasties to prevent iatrogenic sciatic nerve injury. To the authors' knowledge there is no information concerning the use of intraoperative electromyographic monitoring during periacetabular osteotomy. The purpose of the current study was to investigate the use of intraoperative continuous electromyographic monitoring during periacetabular osteotomy in a relatively large consecutive series of patients as a mechanism to prevent nerve injury during surgery and as a prognostic indicator of neurologic function after periacetabular osteotomy. From September 1992 to July 1999, 140 consecutive periacetabular osteotomies were done in 127 patients at the authors' institution. There were 96 females and 31 males, with an average age of 32 years at the time of surgery. All patients had intraoperative electromyographic monitoring of femoral and sciatic innervated muscles. All patients were followed up for a minimum of 1 year, until complete resolution of neurologic deficits, or both. Thirty-six patients (26%) had abnormal electromyographic activity recorded during surgery. Seven patients (5%) had peroneal nerve deficits postoperatively including extensor hallucis longus and tibialis anterior weakness with loss of sensation in the first web space. Abnormal electromyographic activity was observed intraoperatively in five of the seven patients with postoperative deficits. Six of the seven injuries resolved completely. One patient with intraoperative electromyographic activity (0.7%) had a postoperative foot drop that persisted for greater than 1 year. There were no femoral, tibial, or obturator nerve deficits observed. Electromyographic monitoring appears to provide prediction of postoperative neurologic deficit.

Acetabulum↗
PubMed2002

Reversible myelopathy in a 34-year-old man with vitamin B12 deficiency.

Vitamin B12 deficiency is common, with most patients lacking classic features of advanced severe deficiency. Early diagnosis and treatment prevent severe anemia and irreversible damage to the nervous system. We describe a 34-year-old man with pernicious anemia who presented with clinical and radiologic features of early myelopathy and borderline low serum levels of vitamin B12. Prompt diagnosis based on the measurement of serum methylmalonic acid and treatment with cyanocobalamin injections led to rapid resolution of clinical manifestations and magnetic resonance imaging abnormalities. We review the literature of magnetic resonance imaging in vitamin B12 deficiency myelopathy and discuss the issues relating to diagnosis and early treatment of this potentially reversible condition.

Adult↗