Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy.

Abstract:

:Chronic inflammatory demyelinating polyneuropathy (CIDP) presents in rare instances with focal or multifocal upper limb involvement. We reviewed the clinical and electromyographic (EMG) characteristics of 10 such patients (UL-CIDP) and compared them with patients with typical generalized CIDP (G-CIDP) and multifocal motor neuropathy (MMN). There were six men and four women, with a mean age of 54 years. Symptoms began in one arm or hand in six patients and in both arms or hands in four and included numbness (n = 10), paresthesias (n = 9), weakness (n = 8), and pain (n = 6). Findings were initially restricted to the ulnar nerve distribution in three patients, and median and axillary nerve in one patient each, and involved multiple nerves in five. Conduction block was detected in the forearm segment of 68% of the median and ulnar motor nerves tested; in contrast to multifocal motor neuropathy, 73% of the sensory nerves tested were abnormal, and none had anti-GM1 antibodies. Aside from a regional onset, there were no clinical or electrophysiological features that distinguished patients with UL-CIDP from those with G-CIDP. However, the magnitude of recovery following treatment was greater in patients with G-CIDP. We conclude that a multifocal variant of CIDP begins with upper extremity sensorimotor symptoms, simulates isolated or multiple mononeuropathies, can be distinguished from MMN, and may have a less favorable response to treatment.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Gorson KC,Ropper AH,Weinberg DH

doi

10.1002/(sici)1097-4598(199906)22:6<758::aid-mus13

subject

Has Abstract

pub_date

1999-06-01 00:00:00

pages

758-65

issue

6

eissn

0148-639X

issn

1097-4598

pii

10.1002/(SICI)1097-4598(199906)22:6<758::AID-MUS13

journal_volume

22

pub_type

临床试验,杂志文章
  • Ischemic injury and repair process after transection in hypothyroid rat muscles.

    abstract::Hindlimb ischemia for 4 h, followed by reperfusion, resulted in necrosis of most soleus muscle in euthyroid rats, whereas only slight damage occurred in hypothyroid rats. Muscle repair after transection of the tibialis anterior muscle of hypothyroid rats showed delayed debris removal, initial retardation of myotube fo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10364

    authors: Ozawa J,Kawamata S,Kurosaki T,Iwamizu Y,Matsuura N,Abiko S,Kai S

    更新日期:2003-05-01 00:00:00

  • Nerve conduction abnormalities in aging mice deficient for myelin-associated glycoprotein.

    abstract::Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of m...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1159

    authors: Weiss MD,Luciano CA,Quarles RH

    更新日期:2001-10-01 00:00:00

  • Analysis of crosstalk in the mechanomyographic signals generated by forearm muscles during different wrist postures.

    abstract:INTRODUCTION:In this study, we analyzed the crosstalk in mechanomyographic (MMG) signals generated by the extensor digitorum (ED), extensor carpi ulnaris (ECU), and flexor carpi ulnaris (FCU) muscles of the forearm during wrist flexion (WF) and extension (WE) and radial (RD) and ulnar (UD) deviations. METHODS:Twenty r...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24454

    authors: Islam A,Sundaraj K,Ahmad RB,Sundaraj S,Ahamed NU,Ali MA

    更新日期:2015-06-01 00:00:00

  • Pulmonary function and electromyographic study of respiratory muscles in myotonic dystrophy.

    abstract::Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory resistive loads. Present...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880080708

    authors: Jammes Y,Pouget J,Grimaud C,Serratrice G

    更新日期:1985-09-01 00:00:00

  • The 6-minute walk test and other endpoints in Duchenne muscular dystrophy: longitudinal natural history observations over 48 weeks from a multicenter study.

    abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints. ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1002/mus.23902

    authors: McDonald CM,Henricson EK,Abresch RT,Florence JM,Eagle M,Gappmaier E,Glanzman AM,PTC124-GD-007-DMD Study Group.,Spiegel R,Barth J,Elfring G,Reha A,Peltz S

    更新日期:2013-09-01 00:00:00

  • Estimation of conduction velocity of A delta fibers in humans.

    abstract::Conduction velocity of A delta fibers of the human peripheral nerves was measured by using pain-related somatosensory evoked potentials following CO2 laser stimulation. It was found to be approximately 9 m/s in the forearm as well as in the lower leg. Because conventional conduction study using electric stimulation re...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880141209

    authors: Kakigi R,Endo C,Neshige R,Kuroda Y,Shibasaki H

    更新日期:1991-12-01 00:00:00

  • Sparing of the flexor carpi ulnaris in ulnar neuropathy at the elbow.

    abstract::A common misconception attributes sparing of the flexor carpi ulnaris (FCU) in ulnar neuropathy at the elbow (UNE) to its innervating branch arising "at or above the elbow." We examined the relationship of FCU branches to the medial epicondyle (ME) and humeroulnar aponeurotic arcade (HUA) in 30 cadaver elbows. In only...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880121203

    authors: Campbell WW,Pridgeon RM,Riaz G,Astruc J,Leahy M,Crostic EG

    更新日期:1989-12-01 00:00:00

  • Conduction block in neuralgic amyotrophy.

    abstract::We describe two cases of neuralgic amyotrophy with electrophysiological evidence of conduction block across the lower trunk of the brachial plexus. Low-output impedance stimulation of the cervical spinal roots in combination with collision was used to accurately demonstrate the conduction block. Complete electrophysio...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1041

    authors: Watson BV,Nicolle MW,Brown JD

    更新日期:2001-04-01 00:00:00

  • Beta-oxidation enzymes in normal human muscle and in muscle from a patient with an unusual form of myopathic carnitine deficiency.

    abstract::In a reported patient with myopathic carnitine deficiency, addition of exogenous carnitine to muscle homogenates failed to correct palmitate oxidation, and oral carnitine was of no clinical benefit. In a muscle biopsy from this patient, we found that, in contrast to the marked deficiency of free carnitine (3% of norma...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880080809

    authors: Trevisan CP,Reichmann H,DeVivo DC,DiMauro S

    更新日期:1985-10-01 00:00:00

  • Effect of static stretch training on neural and mechanical properties of the human plantar-flexor muscles.

    abstract::To determine the contributions of neural and mechanical mechanisms to the limits in the range of motion (ROM) about a joint, we studied the effects of 30 sessions of static stretch training on the characteristics of the plantar-flexor muscles in 12 subjects. Changes in the maximal ankle dorsiflexion and the torque pro...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10549

    authors: Guissard N,Duchateau J

    更新日期:2004-02-01 00:00:00

  • Central nervous system involvement in multifocal demyelinating neuropathy with persistent conduction block.

    abstract::We report the case of a 27-year-old man treated for bilateral optic neuritis 5 and 3 years before who within a few months developed sensorimotor disorders of the arms and legs characterized by asymmetric distribution and distal prominence. In addition to sensorimotor defects, which were particularly marked in the left...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880171106

    authors: Péréon Y,Jardel J,Guillon B,Guihéneuc P

    更新日期:1994-11-01 00:00:00

  • Interspike interval analysis in a patient with peripheral nerve hyperexcitability and potassium channel antibodies.

    abstract::Neuromyotonia or Isaacs' syndrome is a rare peripheral nerve hyperexcitability disorder caused by antibodies against potassium channels of myelinated axons. We present the high-density surface electromyographic (EMG) recordings of a patient with fasciculations and cramps due to neuromyotonia. To characterize the time ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20849

    authors: Kleine BU,Stegeman DF,Drost G,Zwarts MJ

    更新日期:2008-02-01 00:00:00

  • Peripheral nerve ultrasound in cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS).

    abstract:INTRODUCTION:We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS:The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compa...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25476

    authors: Pelosi L,Leadbetter R,Mulroy E,Chancellor AM,Mossman S,Roxburgh R

    更新日期:2017-07-01 00:00:00

  • Update on immune-mediated therapies for myasthenia gravis.

    abstract::With the exception of thymectomy, immune modulatory treatment strategies and clinical trials in myasthenia gravis over the past 50 y were mainly borrowed from experience in other nonneurologic autoimmune disorders. The current experimental therapy paradigm has significantly changed such that treatments directed agains...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审

    doi:10.1002/mus.26919

    authors: Habib AA,Ahmadi Jazi G,Mozaffar T

    更新日期:2020-11-01 00:00:00

  • Neuromyotonia, peripheral neuropathy and myasthenia gravis.

    abstract::A patient with neuromyotonia, peripheral neuropathy and myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed continuous muscle discharges of motor unit action potentials (MUAPs) in duplets and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies revealed mild axonal and demyel...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(SICI)1097-4598(199604)19:4<505::AID-MUS10

    authors: Martinelli P,Patuelli A,Minardi C,Cau A,Riviera AM,Dal Pozzo F

    更新日期:1996-04-01 00:00:00

  • Macroelectromyography in reinnervation.

    abstract::Macroelectromyography is used to study the electrical activity of the entire motor unit. The amplitude of the motor unit action potential (MUP), recorded from a bared cannula, is dependent on the number and size of muscle fibers in the motor unit. In most situations of reinnervation the local fiber density (FD) as rec...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:

    authors: Stålberg E

    更新日期:1982-01-01 00:00:00

  • Novel mutations in the gene encoding very long-chain acyl-CoA dehydrogenase identified in patients with partial carnitine palmitoyltransferase II deficiency.

    abstract:INTRODUCTION:Twenty-six patients with clinical symptoms of adult onset carnitine palmitoyltransferase II (CPTII) deficiency were examined. All patients had skeletal muscle CPTII enzyme activity levels indicative of heterozygosity for CPT2 mutations, however sequence analysis identified no pathogenic mutations within th...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23498

    authors: Isackson PJ,Sutton KA,Hostetler KY,Vladutiu GD

    更新日期:2013-02-01 00:00:00

  • Two patients with GMPPB mutation: The overlapping phenotypes of limb-girdle myasthenic syndrome and limb-girdle muscular dystrophy dystroglycanopathy.

    abstract:INTRODUCTION:Mutations in the guanosine diphosphate-mannose pyrophosphorylase-B gene (GMPPB) have been identified in congenital muscular dystrophies, limb-girdle muscular dystrophy (LGMD2T), and congenital myasthenic syndromes (CMSs); overall, 41 patients have been described. METHODS:Two patients presented with a myas...

    journal_title:Muscle & nerve

    pub_type:

    doi:10.1002/mus.25485

    authors: Montagnese F,Klupp E,Karampinos DC,Biskup S,Gläser D,Kirschke JS,Schoser B

    更新日期:2017-08-01 00:00:00

  • Thenar hypoplasia with associated radiologic abnormalities.

    abstract::We report on five patients who presented with unilateral or bilateral hypoplasia of the thenar eminence. X rays of the affected limbs showed varying degrees of malformation and underdevelopment of the thumb phalanges and first metacarpal bone, and of the carpal bones at the base of the thumb, indicating a developmenta...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880020604

    authors: Cavanagh NP,Yates DA,Sutcliffe J

    更新日期:1979-11-01 00:00:00

  • Estimation of the number and size of human flexor digiti minimi muscle motor units using histological methods.

    abstract::Motor unit (MU) number and size estimates were obtained from the human flexor digiti minimi muscle using histological methods. Ten adult fresh cadavers (33-74 years old) were used. The number of MUs was 130 +/- 15 and the MU size was 108 +/- 10. These values are similar to those reported for other hypothenar muscles, ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199801)21:1<112::aid-mus14

    authors: Santo Neto H,de Carvalho VC,Marques MJ

    更新日期:1998-01-01 00:00:00

  • In vivo bioluminescent imaging of Schwann cells in a poly(DL-lactide-epsilon-caprolactone) nerve guide.

    abstract::Nerve guides seeded with Schwann cells (SCs) promote axonal regeneration in peripheral nerve lesions. We examined the applicability of bioluminescent imaging (BLI) for monitoring the fate of SCs in nerve guides after implantation. Rat SCs were transfected with the firefly luciferase (Fluc) gene and subsequently seeded...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21372

    authors: Ma MS,Van Dam G,Meek M,Boddeke E,Copray S

    更新日期:2009-11-01 00:00:00

  • Sodium withdrawal contractures in developing and regenerating rat extensor digitorum longus muscles.

    abstract::During postnatal development of extensor digitorum longus (EDL) muscle, sodium withdrawal contractures were observed during the first 6 days after birth, and not after this time. In regenerating EDL muscles, zero-Na contractures were demonstrated: (1) 7 days after bupivacaine injection, but not 14 or 90 days after thi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199811)21:11<1530::aid-mus

    authors: Louboutin JP,Noireaud J

    更新日期:1998-11-01 00:00:00

  • The effect of fatigue on knee position sense is not dependent upon the muscle group fatigued.

    abstract:INTRODUCTION:Muscle fatigue could have a greater impact on position sense when antagonists of the movement are fatigued. Hence, this study aimed to compare the effects of antagonist and agonist exercise-induced muscle fatigue on knee joint position sense. METHODS:This within-subjects repeated-measures study included 4...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22018

    authors: Ribeiro F,Venâncio J,Quintas P,Oliveira J

    更新日期:2011-08-01 00:00:00

  • Patterns of clinical and electrodiagnostic abnormalities in early amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread. METHODS:Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the cl...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24244

    authors: Simon NG,Lomen-Hoerth C,Kiernan MC

    更新日期:2014-12-01 00:00:00

  • Estimating electromyographic and heart rate fatigue thresholds from a single treadmill test.

    abstract:INTRODUCTION:The purposes of this study were to (1) develop a fatigue threshold based on electromyography (EMG) and heart rate (HR) responses for treadmill running from a single incremental test; and (2) propose a new fatigue threshold called the RV(EMGFT) and RV(HRFT). METHODS:Eleven men performed incremental treadmi...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章

    doi:10.1002/mus.23345

    authors: Guffey DR,Gervasi BJ,Maes AA,Malek MH

    更新日期:2012-10-01 00:00:00

  • Coexistence of two chronic neuropathies in a young child: Charcot-Marie-Tooth disease type 1A and chronic inflammatory demyelinating polyneuropathy.

    abstract::We report an 18-month-old Charcot-Marie-Tooth type 1A (CMT1A) patient who developed a rapid-onset neuropathy, with proximal and distal weakness, and non-uniform nerve conduction studies. The neuropathy responded well to immunomodulation, confirming the coexistence of an inherited and an inflammatory neuropathy. Unexpe...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21753

    authors: Marques W Jr,Funayama CA,Secchin JB,Lourenço CM,Gouvêa SP,Marques VD,Bastos PG,Barreira AA

    更新日期:2010-10-01 00:00:00

  • Hypothyroid myopathy with a strikingly elevated serum creatine kinase level.

    abstract::Although serum creatine kinase (CK) levels are frequently modestly elevated in patients with hypothyroid myopathy, elevations in serum CK to the levels usually seen in inflammatory myopathies or dystrophies are rare. We report a patient with progressive proximal weakness and a serum CK level of over 29,000 IU/L, in wh...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10128

    authors: Scott KR,Simmons Z,Boyer PJ

    更新日期:2002-07-01 00:00:00

  • Effects of hyperbaric exposure with high oxygen concentration on glucose and insulin levels and skeletal muscle-fiber properties in diabetic rats.

    abstract::The effects of hyperbaric exposure with high oxygen concentration on glucose and insulin levels and skeletal muscle-fiber properties were investigated in type 2 diabetic Goto-Kakizaki rats. Five-week-old rats were exposed to a hyperbaric environment (1.25 atmospheric pressure) with a high oxygen concentration (36%) fo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20692

    authors: Yasuda K,Adachi T,Gu N,Matsumoto A,Matsunaga T,Tsujimoto G,Tsuda K,Ishihara A

    更新日期:2007-03-01 00:00:00

  • Peripheral axon caliber and conduction velocity are decreased after burn injury in mice.

    abstract::Peripheral neuropathies are reported to arise as a result of the systemic inflammatory response produced by a full-thickness cutaneous burn injury. This study was designed to characterize the magnitude and time course of functional and morphological changes in peripheral axons that arise after a full-thickness dermal ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20306

    authors: Higashimori H,Whetzel TP,Mahmood T,Carlsen RC

    更新日期:2005-05-01 00:00:00

  • Ethical review of research involving human subjects: when and why is IRB review necessary?

    abstract::Requirements for ethical review of research involving human subjects are based on widely accepted international standards that are implemented by various national regulations and institutional policies. In the United States, most human research is reviewed by institutional review boards (IRBs) applying federal standar...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审

    doi:10.1002/mus.10398

    authors: Wagner RM

    更新日期:2003-07-01 00:00:00