Cytoplasmic body myopathy masquerading as motor neuron disease.


:Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without diagnostic features. A diagnosis of possible motor neuron disease was made and the patient was commenced on riluzole. Subsequently, the patient's condition stabilized, prompting reassessment. Repeat EMG demonstrated no features of denervation and was more suggestive of a myopathic process. Review of the original muscle biopsy showed cytoplasmic bodies. The case highlights a further diagnostic possibility in the assessment of patients with "possible" motor neuron disease. The clinical features of CBM are briefly reviewed.


Muscle Nerve


Muscle & nerve


Krishnan AV,Pamphlett R,Burke D,Wills EJ,Kiernan MC




Has Abstract


2004-11-01 00:00:00












  • Calpain 3 cleaves filamin C and regulates its ability to interact with gamma- and delta-sarcoglycans.

    abstract::Calpain 3 (C3) is the only muscle-specific member of the calcium-dependent protease family. Although neither its physiological function nor its in vivo substrates are known, C3 must be an important protein for normal muscle function as mutations in the C3 gene result in limb-girdle muscular dystrophy type 2A. Previous...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Guyon JR,Kudryashova E,Potts A,Dalkilic I,Brosius MA,Thompson TG,Beckmann JS,Kunkel LM,Spencer MJ

    更新日期:2003-10-01 00:00:00

  • Sural nerve biopsy studies in Leigh's subacute necrotizing encephalomyelopathy.

    abstract::Peripheral neuropathy marked by reduced nerve conduction velocities was found in four unrelated children, between the ages of 15 months and 9 years, whose autopsies revealed Leigh's subacute necrotizing encephalomyelopathy. Sural nerve biopsies disclosed primary demyelination and remyelination, as well as loss of myel...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Goebel HH,Bardosi A,Friede RL,Kohlschütter A,Albani M,Siemes H

    更新日期:1986-02-01 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


    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

  • 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


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

    更新日期:2015-06-01 00:00:00

  • Long-term recovery of muscle strength after denervation in the fibular division of the sciatic nerve.

    abstract:INTRODUCTION:The probability and degree of muscle recovery after lesions of long peripheral nerves have not been assessed quantitatively. METHODS:Twelve adults with closed injuries of the fibular division of the sciatic nerve with complete denervation of associated muscles were followed-up for 2-10 years. The onset of...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Stefancic M,Vidmar G,Blagus R

    更新日期:2016-10-01 00:00:00

  • Effects of hemodialysis on intraneural blood flow in end-stage kidney disease.

    abstract:INTRODUCTION:We quantified intraneural blood flow (INBF) in 18 patients with end-stage kidney disease (ESKD) and examined its relationship with nerve size, neuropathy severity, and nerve excitability parameters. METHODS:Sonographic measurements of the median nerve were performed at the same site before and after hemod...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Borire AA,Arnold R,Pussell BA,Kwai NC,Visser LH,Simon NG,Kiernan MC,Krishnan AV

    更新日期:2018-02-01 00:00:00

  • Basal lamina and superfast myosin expression in regenerating cat jaw muscle.

    abstract::We investigated the possible role of extracellular matrix in specifying the expression of superfast myosin during cat jaw muscle regeneration. Equal proportions of muscle tissue from jaw and limb were minced together after killing cellular elements from one source. We allowed the mince to regenerate in the bed of a fa...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Hoh JF,Hughes S

    更新日期:1991-05-01 00:00:00

  • McArdle's disease presenting as recurrent cryptogenic renal failure due to occult seizures.

    abstract::Myophosphorylase deficiency (McArdle's disease) may present with episodic renal failure following exertion. We present a case of adult-onset myophosphorylase deficiency in which recurrent bouts of renal failure could not be associated with any exertional events until a tonic seizure was witnessed after the fourth epis...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Walker AR,Tschetter K,Matsuo F,Flanigan KM

    更新日期:2003-11-01 00:00:00

  • Reference jitter values for concentric needle electrodes in voluntarily activated extensor digitorum communis and orbicularis oculi muscles.

    abstract::The aim of this study was to estimate normal jitter in voluntarily activated extensor digitorum communis (EDC) and orbicularis oculi (OOc) muscles using a disposable concentric needle electrode (CNE). The EDC of 67 normal subjects (22 males and 45 females, mean age 35.5 +/- 10.2 years) and the OOc of 50 normal subject...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Kouyoumdjian JA,Stålberg EV

    更新日期:2008-06-01 00:00:00

  • Clinical, physiological, and histological features in a kindred with the T3271C melas mutation.

    abstract::The majority of patients with MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes) have an A-->G mutation at nucleotide 3243 in mitochondrial transfer (t)RNA. To date there have only been 10 reported cases of MELAS syndrome in patients with a T-->C mutation at position 3271...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Tarnopolsky MA,Maguire J,Myint T,Applegarth D,Robinson BH

    更新日期:1998-01-01 00:00:00

  • Diagnostic usefulness of denervation edema in the multifidus muscles using 3-Tesla magnetic resonance imaging in cervical radiculopathy.

    abstract:BACKGROUND:Diagnosing cervical radiculopathy (CR) can be difficult because of symptomatic overlap with peripheral neuropathies. In this retrospective observational study, we aimed to determine whether short-tau inversion recovery (STIR) magnetic resonance imaging (MRI) sequences are useful for detecting signs of denerv...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Yoshida T,Suwazono S,Sueyoshi T,Izumi Y,Nodera H

    更新日期:2020-12-13 00:00:00

  • Central fatigue assessed by transcranial magnetic stimulation.

    abstract::Central fatigue is a subjective phenomenon which can be examined using transcranial magnetic stimulation (TMS). To assess central fatigue, we compared TMS and peripheral electrical stimulations in patients with central nervous system (CNS) lesions and controls before and after an exhaustive task. The recovery times of...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Liepert J,Kotterba S,Tegenthoff M,Malin JP

    更新日期:1996-11-01 00:00:00

  • Muscle fiber size in healthy children and adults in relation to sex and fiber types.

    abstract:BACKGROUND:In adult males, cross-sectional area (CSA) for type II muscle fibers is generally larger than for type I fibers. In this cross-sectional study the aim was to compare sex-related CSAs of various muscle fiber types during childhood-to-adulthood transition. METHODS:Percutaneous biopsy samples were obtained fro...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Esbjörnsson ME,Dahlström MS,Gierup JW,Jansson EC

    更新日期:2020-12-21 00:00:00

  • Reductions in muscle quality and quantity in chronic inflammatory demyelinating polyneuropathy patients assessed by magnetic resonance imaging.

    abstract:INTRODUCTION:Weakness in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) may be caused by decreases in muscle quantity and quality, but this has not been explored. METHODS:Twelve patients with CIDP (mean age 61 years) and 10 age-matched (mean age 59 years) control subjects were assessed for ankl...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Gilmore KJ,Doherty TJ,Kimpinski K,Rice CL

    更新日期:2018-09-01 00:00:00

  • The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated. METHODS:Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Jenkins TM,Alix JJ,Kandler RH,Shaw PJ,McDermott CJ

    更新日期:2016-09-01 00:00:00

  • Single fiber macro versus concentric trigger macro EMG: a comparison of methods.

    abstract::The values obtained from two different macro EMG methods were compared, and found to be different. Normally, macro electromyography (EMG) is performed with a modified single fiber (SF) needle using the SF potential as a trigger; a new method recommends a modified concentric needle and triggers on the concentric EMG si...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Nix WA,Scherer A

    更新日期:1992-02-01 00:00:00

  • Muscle metabolic alterations assessed by 31-phosphorus magnetic resonance spectroscopy in mild Becker muscular dystrophy.

    abstract::Although the molecular defect causing Becker muscular dystrophy (BMD) has been identified, the biochemical mechanisms that lead to muscle necrosis remain unclear. Exercise-related muscle metabolism in 9 mildly affected BMD patients was assessed by muscle 31-phosphorus magnetic resonance spectroscopy ((31)P MRS) during...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Tosetti M,Linsalata S,Battini R,Volpi L,Cini C,Presciutti O,Muntoni F,Cioni G,Siciliano G

    更新日期:2011-11-01 00:00:00

  • Parenteral branched-chain amino acid treatment and avian dystrophy.

    abstract::Genetically homozygous line 413 dystrophic chickens were given twice-daily intraperitoneal injections of solutions containing branched-chain amino acids (BCCA-leucine, valine, isoleucine) either alone or in combination; and their alpha-ketoacid analogs (alpha-ketoisocaproic and alpha-ketoisovaleric acids). Another tri...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Hudecki MS,Pollina CM,Heffner RR

    更新日期:1982-07-01 00:00:00

  • Time to failure of a sustained contraction is predicted by target torque and initial electromyographic bursts in elbow flexor muscles.

    abstract::The purpose of the study was to identify factors that could predict differences among individuals in the time to failure of a submaximal contraction. Twenty subjects (10 men, 25+/-6 years) supported an inertial load equivalent to 20% of the maximal voluntary contraction (MVC) force with the elbow flexor muscles for as...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Rudroff T,Christou EA,Poston B,Bojsen-Møller J,Enoka RM

    更新日期:2007-05-01 00:00:00

  • On the nature of endogenous antiexcitatory factors in the cerebrospinal fluid of patients with demyelinating neurological disease.

    abstract::The cerebrospinal fluid (CSF) of patients with demyelinating neurological disease, such as Guillain-Barré syndrome or multiple sclerosis, contains factors that inhibit the excitatory Na+ current. Such antiexcitatory factors are occasionally also detectable in CSF from patients with other neurological diseases but were...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Brinkmeier H,Seewald MJ,Wollinsky KH,Rüdel R

    更新日期:1996-01-01 00:00:00

  • Anodal sensory nerve action potentials: From physiological understanding to potential clinical applicability.

    abstract:INTRODUCTION:Low-intensity electrical stimuli of digital nerves may generate a double peak potential (DPp), composed of a cathodal (caAP) and an anodal (anAP) potential in orthodromic recordings. METHODS:We studied the effects on caAP and anAP of stimuli of variable intensity, duration, and frequency. We also applied ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Leote J,Pereira P,Cabib C,Cipullo F,Valls-Sole J

    更新日期:2016-06-01 00:00:00

  • Addressing heterogeneity in amyotrophic lateral sclerosis CLINICAL TRIALS.

    abstract::Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disorder with complex biology and significant clinical heterogeneity. Many preclinical and early phase ALS clinical trials have yielded promising results that could not be replicated in larger phase 3 confirmatory trials. One reason for the lack o...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: Goyal NA,Berry JD,Windebank A,Staff NP,Maragakis NJ,van den Berg LH,Genge A,Miller R,Baloh RH,Kern R,Gothelf Y,Lebovits C,Cudkowicz M

    更新日期:2020-08-01 00:00:00

  • Combined XIL-6R and urocortin-2 treatment restores MDX diaphragm muscle force.

    abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration leading to immobility, respiratory failure, and premature death. As chronic inflammation and stress are implicated in DMD pathology, the efficacy of an anti-inflammatory and anti-stress intervention strategy in ameliorati...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Manning J,Buckley MM,O'Halloran KD,O'Malley D

    更新日期:2017-12-01 00:00:00

  • Pseudometabolic presentation of dystrophinopathy due to a missense mutation.

    abstract::Exercise intolerance with myalgia, muscle stiffness, and recurrent rhabdomyolysis due to mutations in the DMD gene can mimic metabolic myopathies leading to delayed or inaccurate diagnoses. In this retrospective chart review, we report 3 unrelated boys with exertional myalgia, muscle stiffness, myoglobinuria, and norm...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Veerapandiyan A,Shashi V,Jiang YH,Gallentine WB,Schoch K,Smith EC

    更新日期:2010-12-01 00:00:00

  • The utility of various sensory nerve conduction responses in assessing brachial plexopathies.

    abstract::To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Ferrante MA,Wilbourn AJ

    更新日期:1995-08-01 00:00:00

  • Pathophysiology of spastic paresis. I: Paresis and soft tissue changes.

    abstract::Spastic paresis follows chronic disruption of the central execution of volitional command. Motor function in patients with spastic paresis is subjected over time to three fundamental insults, of which the last two are avoidable: (1) the neural insult itself, which causes paresis, i.e., reduced voluntary motor unit rec...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: Gracies JM

    更新日期:2005-05-01 00:00:00

  • Cardiac repolarization abnormalities in multiple sclerosis: spinal cord MRI correlates.

    abstract::Ventricular repolarization dysfunction has recently been reported in multiple sclerosis (MS). We evaluated ventricular repolarization dysfunction in 52 MS patients and looked for a relationship between corrected QT (QTc) abnormalities (i.e., abnormalities of QT intervals corrected for rate) and spinal cord magnetic re...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: de Seze J,Stojkovic T,Gauvrit JY,Saint Michel T,Ayachi M,Pruvo JP,Vermersch P

    更新日期:2000-08-01 00:00:00

  • Eosinophilic fasciitis with subjacent myositis.

    abstract:INTRODUCTION:Eosinophilic fasciitis (EF) is a rare disorder that can present with muscle symptoms that mimic other neuromuscular diseases. METHODS:We report the case of a 43-year-old woman with chronic muscle aches, tightness, and stiffness with hypertrophied, well-defined muscles despite physical inactivity, and thic...

    journal_title:Muscle & nerve



    authors: Whitlock JB,Dimberg EL,Selcen D,Rubin DI

    更新日期:2017-09-01 00:00:00

  • Enrichment of the R77C alpha-sarcoglycan gene mutation in Finnish LGMD2D patients.

    abstract::Limb-girdle muscular dystrophy 2D (LGMD2D) is caused by mutations in the alpha-sarcoglycan gene (SGCA). The most frequently reported mutation, 229CGC>TGC (R77C) in exon 3 of SGCA, results in the substitution of arginine by cysteine. We present here the clinical, immunohistochemical, and genetic data of 11 Finnish pati...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Hackman P,Juvonen V,Sarparanta J,Penttinen M,Aärimaa T,Uusitalo M,Auranen M,Pihko H,Alén R,Junes M,Lönnqvist T,Kalimo H,Udd B

    更新日期:2005-02-01 00:00:00

  • Skeletal muscle expression of clathrin and mannose 6-phosphate receptor in experimental chloroquine-induced myopathy.

    abstract::Previous studies suggest that the muscle fiber lysosome system plays a central role in the increased formation of autophagosomes and autolysosomes that occurs in the context of chloroquine-induced myopathy. The goal of this study was to characterize the contribution of receptor-mediated intracellular transport, partic...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Masuda T,Ueyama H,Nakamura K,Jikumaru M,Toyoshima I,Kumamoto T

    更新日期:2005-04-01 00:00:00