K Reiners

University of Wuerzburg, Würzburg, Bavaria, Germany

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Publications (160)709.75 Total impact

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    Journal of neurology, neurosurgery, and psychiatry 12/2013; · 4.87 Impact Factor
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    ABSTRACT: Fibromyalgia syndrome is a clinically well-characterized chronic pain condition of high socio-economic impact. Although the pathophysiology is still unclear, there is increasing evidence for nervous system dysfunction in patients with fibromyalgia syndrome. In this case-control study we investigated function and morphology of small nerve fibres in 25 patients with fibromyalgia syndrome. Patients underwent comprehensive neurological and neurophysiological assessment. We examined small fibre function by quantitative sensory testing and pain-related evoked potentials, and quantified intraepidermal nerve fibre density and regenerating intraepidermal nerve fibres in skin punch biopsies of the lower leg and upper thigh. The results were compared with data from 10 patients with monopolar depression without pain and with healthy control subjects matched for age and gender. Neurological and standard neurophysiological examination was normal in all patients, excluding large fibre polyneuropathy. Patients with fibromyalgia syndrome had increased scores in neuropathic pain questionnaires compared with patients with depression and with control subjects (P < 0.001 each). Compared with control subjects, patients with fibromyalgia syndrome but not patients with depression had impaired small fibre function with increased cold and warm detection thresholds in quantitative sensory testing (P < 0.001). Investigation of pain-related evoked potentials revealed increased N1 latencies upon stimulation at the feet (P < 0.001) and reduced amplitudes of pain-related evoked potentials upon stimulation of face, hands and feet (P < 0.001) in patients with fibromyalgia syndrome compared to patients with depression and to control subjects, indicating abnormalities of small fibres or their central afferents. In skin biopsies total (P < 0.001) and regenerating intraepidermal nerve fibres (P < 0.01) at the lower leg and upper thigh were reduced in patients with fibromyalgia syndrome compared with control subjects. Accordingly, a reduction in dermal unmyelinated nerve fibre bundles was found in skin samples of patients with fibromyalgia syndrome compared with patients with depression and with healthy control subjects, whereas myelinated nerve fibres were spared. All three methods used support the concept of impaired small fibre function in patients with fibromyalgia syndrome, pointing towards a neuropathic nature of pain in fibromyalgia syndrome.
    Brain 03/2013; · 10.23 Impact Factor
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    ABSTRACT: BACKGROUND: Dysferlinopathies are autosomal recessive disorders caused by mutations in the dysferlin (DYSF) gene encoding the dysferlin protein. DYSF mutations lead to a wide range of muscular phenotypes, with the most prominent being Miyoshi myopathy (MM) and limb girdle muscular dystrophy type 2B (LGMD2B). METHODS: We assessed the one-year-natural course of dysferlinopathy, and the safety and efficacy of deflazacort treatment in a double-blind, placebo-controlled cross-over trial. After one year of natural course without intervention, 25 patients with genetically defined dysferlinopathy were randomized to receive deflazacort and placebo for six months each (1 mg/kg/day in month one, 1 mg/kg every 2nd day during months two to six) in one of two treatment sequences. RESULTS: During one year of natural course, muscle strength declined about 2% as measured by CIDD (Clinical Investigation of Duchenne Dystrophy) score, and 76 Newton as measured by hand-held dynamometry. Deflazacort did not improve muscle strength. In contrast, there is a trend of worsening muscle strength under deflazacort treatment, which recovers after discontinuation of the study drug. During deflazacort treatment, patients showed a broad spectrum of steroid side effects. CONCLUSION: Deflazacort is not an effective therapy for dysferlinopathies, and off-label use is not warranted. This is an important finding, since steroid treatment should not be administered in patients with dysferlinopathy, who may be often misdiagnosed as polymyositis.Trial registration: This clinical trial was registered at www.ClincalTrials.gov, identifier: NCT00527228, and was always freely accessible to the public.
    Orphanet Journal of Rare Diseases 02/2013; 8(1):26. · 4.32 Impact Factor
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    ABSTRACT: Introduction: Persistently elevated serum creatine kinase (CK) is frequently associated with predisposition to malignant hyperthermia (MH). We investigated whether a minimally invasive metabolic test is suitable to diagnose MH susceptibility among patients with hyperCKemia. Methods: Thirty-nine participants were included: 10 were MH susceptible (MHS); 21 MH were non-susceptible (MHN); and 8 had MHN with persistent hyperCKemia >500 U/L. Microdialysis probes were inserted into the vastus lateralis muscle, and halothane or caffeine was injected via an attached microtubing catheter. Lactate concentrations in dialysis samples were measured spectrophotometrically. Results: Baseline lactate did not differ between the groups. After local application of halothane or caffeine, a significant lactate increase was detected only in the MHS group. Conclusions: Test results were not influenced by hyperCKemia. To avoid risks and complications of a surgical muscle biopsy this microdialysis test might be a useful screening tool for MH susceptibility among patients with persistent CK elevation. Muscle Nerve, 2013.
    Muscle & Nerve 08/2012; · 2.31 Impact Factor
  • C Wessig, M Bendszus, K Reiners, M Pham
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    ABSTRACT: The diagnostic work-up of peripheral neuropathies largely depends on neurophysiological investigations. Recently, progress in magnetic resonance imaging (MRI) has lead to new perspectives in the diagnostics of disorders of the peripheral nervous system (PNS). Experimental data show how MR neurography visualises axonal and demyelinating lesions of the PNS. In clinical use, difficult cases of focal nerve compression, traumatic or inflammatory lesions can be solved by the combination of MR neurography and neurophysiology. In particular, the localisation of nerve lesions can be improved by MR techniques. Furthermore, MR neurography enables new insights in the pathophysiology of neuropathies which will be shown for diabetic polyneuropathy.
    Handchirurgie · Mikrochirurgie · Plastische Chirurgie 06/2012; 44(3):155-62. · 0.86 Impact Factor
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    ABSTRACT: Pain-related evoked potentials (PREPs) represent a novel method for the evaluation of peripheral and central nociceptive pathways, e.g. in the diagnosis of small fiber neuropathy (SFN) or after therapeutic interventions for headache. Compared to contact heat-evoked and laser-evoked potentials, recording of PREPs is less stressful for the subjects and technically less demanding. The clinical usefulness of PREPs has been described for SFN associated with diabetes, HIV and hepatitis C infections as well as in headache and facial pain disorders. They have also been evaluated after interventional methods, such as direct current stimulation (tDCS). The article reviews and discusses the advantages and pitfalls of this technique in the context of recent clinical studies as compared to other paradigms of peripheral electrical stimulation and delineates perspectives and possible indications.
    Der Schmerz 12/2011; 26(1):8-15. · 1.02 Impact Factor
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    ABSTRACT: Fabry disease (FD) is an X-linked lysosomal storage disorder which may lead to impaired peripheral nerve function, mostly affecting small nerve fibers, and to neuropathic pain. Characteristics of the neuropathy associated with FD and the covariates for its development and temporal course have not been described in a large cohort. We studied small fiber function and morphology in 120 Fabry patients at baseline and in subgroups of these until 4-year follow-up. Baseline neurological (89/120) and electrophysiological (106/120) examination was mostly normal. Quantitative sensory testing revealed impaired cold detection thresholds in 84% of men and 39% of women. Lower leg intraepidermal nerve fiber density (IENFD) was reduced to 46% in Fabry patients compared to controls and to 12.5% in men with impaired renal function. Patients with abnormal IENFD more often had pain. Group means for IENFD did not improve under enzyme replacement therapy (ERT), but IENFD in the back increased under ERT in 4/15 patients with good renal function and clinical improvement. Cutaneous cytokine gene expression did not differ from controls. We conclude that ERT may improve proximal skin innervation in patients with good renal function, but does not protect small fiber function in men with impaired renal function.
    Journal of the Peripheral Nervous System 12/2011; 16(4):304-14. · 2.57 Impact Factor
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    ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease with variable involvement of other systems. A pathogenetic role of immune-mediated mechanisms has been suggested. We retrospectively analyzed sural nerve pathology and the clinical course in 18 patients with ALS. These patients had undergone sural nerve biopsy because of clinical or neurophysiological signs indicating sensory involvement (ALS+). Eleven of the 18 ALS+ patients had inflammatory cell infiltrates (ALSvasc) resembling infiltrates seen in patients with vasculitic neuropathy. Data were compared with the 7 patients without vasculitic infiltrates (ALSnonvasc) and with those of 16 patients with isolated peripheral nerve vasculitis (NPvasc). Biopsy specimens were processed with standard histological stains and with immunohistochemistry for a panel of inflammatory markers, with the hypothesis that the composition of infiltrates should differ between ALSvasc and NPvasc. Immunoreactive cells were quantified in a blinded manner. Unlike patients with NPvasc, those with ALSvasc had only minor neurophysiological abnormalities in the sural nerve and, except for the infiltrates, almost normal nerve morphology on semithin sections. The difference in epineurial T cell count was significant between ALSvasc and ALSnonvasc (p=0.031). Surprisingly, the cellular composition of epineurial infiltrates in sural nerve biopsies was indistinguishable between ALSvasc and NPvasc despite a significant difference in fiber pathology (p<0.0001). Standard immunosuppressive treatment did not prevent clinical progression of the motor neuron disease in any of the patients with ALSvasc. ALSvasc appears as a neuropathological subtype in ALS+ suggesting immune-mediated disease components but without response to standard immunosuppressive treatment. KeywordsAmyotrophic lateral sclerosis (ALS)–Sural nerve biopsy–Nonsystemic vasculitic neuropathy
    Acta Neuropathologica 09/2011; 122(3):343-352. · 9.73 Impact Factor
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    ABSTRACT: To obtain information on functional integrity of the facial nerve by transcranial electrical motor evoked potentials independent of nerve visualization and to improve prediction of postoperative function. In a prospective clinical study, 68 patients with cerebello-pontine angle tumors and 5 patients with trigeminal neuralgia were investigated by facial motor evoked potentials (FMEP) elicited by multi-pulse transcranial electrical motor cortex stimulation. For recording the same electrode set-up was used as for continuous EMG monitoring of the orbicularis oculi and oris muscles. Pre-surgical FMEP amplitudes and latencies were correlated with tumor extensions. End to start amplitude ratios were compared to early and long-term facial nerve function by House-Brackmann-Grading (HB) documented by pre- and post-operative photo and video documentation. Reliable FMEP were obtained in 57 patients. FMEP responses at the start of surgery correlated with the degree of tumor extension. Largest FMEP amplitudes and shortest latencies were found in patients with trigeminal neuralgia. FMEP quality was reduced with increasing tumor extension (P<0.05). The ratio of end-operative to start-operative FMEP-amplitude showed a positive correlation with early and late facial nerve function. Correlation was especially close with early function: an amplitude preservation rate of 86% led to HB°1 or HB°2, of 67% to HB°3, at 33% to HB°4 and at 15% or lower to HB°5 or HB°6. Initial FMEP amplitudes correlate with the presumed pre-operative nerve affection by space occupying tumors, a phenomenon reported here for the first time. Intact FMEP are highly reliable for preserved nerve continuity and hereby are of special help to the neurosurgeon for those surgical phases where the facial nerve is not visible and still covered by tumor and where conventional EMG monitoring is of very limited use. The end-to-start amplitude ratio of the FMEP is closely related to early and late clinical function. Amplitude reduction by 30% or more should result in a change of microsurgical action to enable fast recovery. As an adjunct to intraoperative EMG, FMEP are superior in two respects, first in identifying pre-surgical latent nerve lesions and second in monitoring nerve integrity without direct nerve visualization. FMEP are highly reliable in predicting early and late postoperative function.
    Clinical neurology and neurosurgery 07/2011; 113(10):872-9. · 1.30 Impact Factor
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    Daniel Zeller, Karlheinz Reiners, Guido Stoll
    Movement Disorders 02/2011; 26(2):357-8. · 5.63 Impact Factor
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    ABSTRACT: We report on magnetic resonance neurography (MRN) as a supplementary diagnostic tool in sciatic nerve injection injury. The object of the study was to test if T2-weighted (w) contrast within the sciatic nerve serves as an objective criterion for sciatic injection injury. Three patients presented with acute sensory and/or motor complaints in the distribution of the sciatic nerve after dorsogluteal injection and underwent MRN covering gluteal, thigh and knee levels. Native and contrast-enhanced T1-w images were employed to identify the tibial and peroneal division of the sciatic nerve while T2-w images with fat suppression allowed visualization of the site and extent of the nerve lesion. MRN in the two patients with clinically severe sensory and motor impairment correctly depicted sciatic injury: continuity of the T2-w lesion within the nerve at the lesion site and distal to it corresponded well to severe injury confirmed by NCS/EMG as axonotmetic or neurotmetic. Topography of the T2-w lesion on cross-section corresponded to predominant peroneal involvement; moreover, associated denervation patterns of distal target muscles were revealed. One of these patients completely recovered with concomitant complete regression of MRN abnormalities on follow-up. The third patient experienced transient sensory and mild motor impairment with complete recovery after 2 weeks. In this patient, T2-w signal within the nerve and distal target muscles remained normal indicating only mild, non-axonal nerve affliction. Our case series shows that MRN can be very useful in precisely determining the site of sciatic injection injury and may provide diagnostic criteria for the assessment of lesion severity and recovery.
    Journal of Neurology 01/2011; 258(6):1120-5. · 3.58 Impact Factor
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    ABSTRACT: Pain-related evoked potentials (PREPs) represent a novel method for the evaluation of peripheral and central nociceptive pathways, e.g. in the diagnosis of small fiber neuropathy (SFN) or after therapeutic interventions for headache. Compared to contact heat-evoked and laser-evoked potentials, recording of PREPs is less stressful for the subjects and technically less demanding. The clinical usefulness of PREPs has been described for SFN associated with diabetes, HIV and hepatitis C infections as well as in headache and facial pain disorders. They have also been evaluated after interventional methods, such as direct current stimulation (tDCS). The article reviews and discusses the advantages and pitfalls of this technique in the context of recent clinical studies as compared to other paradigms of peripheral electrical stimulation and delineates perspectives and possible indications.
    Der Schmerz 01/2011; · 1.02 Impact Factor
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    ABSTRACT: In focal hand dystonia, long-term potentiation (LTP) and depression (LTD)-like neuronal plasticity, as assessed by paired associative stimulation (PAS) targeting the hand-associated motor cortex, is enhanced and the topographic organization of plasticity is lost. However, if any of these abnormalities alone is sufficient to cause focal dystonia (FD) remains unknown. Ten patients with cervical dystonia (CD), 9 with blepharospasm (BS) and 16 age- and sex-matched controls were examined. PAS was performed by combining repetitively electric stimulation of the median nerve with subsequent transcranial magnetic stimulation of the contralateral motor cortex at 21.5ms (PAS21.5) and 10ms (PAS10). Corticospinal excitability was indexed by the magnitude of motor evoked potentials (MEPs) recorded from abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. In controls, MEP size of the homotopically conditioned APB increased after PAS21.5 whereas the MEP size of the heterotopically conditioned ADM remained stable. PAS10 led to a decrease of MEP size of the APB and to an increase of the heterotopic ADM. In contrast, after PAS21.5 and PAS10 in CD and BS MEP size increased and decreased, respectively, in both muscles. The magnitude of excitability changes, however, did not differ between dystonic patients and healthy controls. In FD the topographic organization of PAS21.5 and PAS10-induced plasticity is deranged in cortical areas not involved in the control of the dystonic body part. Somatotopical disorganization of plasticity may represent an endophenotypic trait in FD but may not be sufficient to generate a dystonic phenotype. Development of a dystonic phenotype may require that the gain of plasticity is additionally enhanced. This article is part of a Special Issue entitled "Advances in dystonia".
    Neurobiology of Disease 11/2010; 42(2):171-6. · 5.62 Impact Factor
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    ABSTRACT: Symptomatic treatment of amyotrophic lateral sclerosis (ALS) is relevant in preventing complications and improving quality of life as long as curative therapies are still out of sight. About one third of ALS patients show disabling problems associated with dysarthria, dysphagia, sialorrhea, and a pseudobulbar affective disorder already in the early stages of ALS. A multidisciplinary approach is the cornerstone of symptomatic treatment of bulbar and pseudobulbar ALS features. Except for riluzole randomized controlled trials are lacking. Here, we review the current views with regard to epidemiology, pathophysiology, diagnosis, and practical aspects of treating bulbar and pseudobulbar symptoms.
    Der Nervenarzt 10/2010; 81(10):1218-25. · 0.80 Impact Factor
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    ABSTRACT: We report on a 51-year-old woman with episodic ataxia type 2 (EA2) and a novel CaV2.1 C-terminal single amino acid substitution (R2090Q). She had a 4-year history of acute episodes with ataxia, hemihypesthesia and hemicrania. Furthermore, fluctuating neuromuscular transmission abnormalities rarely described in patients with EA2 were clinically and electrophysiologically documented in this patient. Upon initiation of acetazolamide treatment she experienced a dose-dependent severe increase of attack frequency and severity along with a shorter attack duration, while she responded well to subsequent therapy with 4-aminopyridine.
    Journal of the neurological sciences 09/2010; 296(1-2):104-6. · 2.32 Impact Factor
  • European Journal of Neurology 09/2010; 17:37. · 4.16 Impact Factor
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    ABSTRACT: Die Symptome der amyotrophen Lateralsklerose (ALS) werden überwiegend verursacht durch die erkrankungstypische Degeneration des 1. und 2. motorischen Neurons. Weitere Systeme des zentralen Nervensystems (ZNS) sind ebenfalls betroffen, wie das autonome, sensorische und sensible System. Bei der ALS existiert bislang kein kurativer Therapieansatz. Daher kommt der symptomatischen Therapie für die Verbesserung der Lebensqualität, zur Prävention von Komplikationen und damit auch zur Prognoseverbesserung eine besondere Bedeutung zu. Etwa ein Drittel der ALS-Patienten klagen schon zu Beginn der Erkrankung über Veränderung des Sprech- und Schluckvermögens, verbunden mit Sialorrhö. Gelegentlich treten pathologisches Lachen und Weinen auf, die ohne entsprechende innere Regung ablaufen und als pseudobulbäre Symptome gelten. Die pathophysiologischen Zusammenhänge, die zu dieser pseudobulbären Affektstörung führen, sind bislang nicht ausreichend geklärt. In der Regel wird heute für die bulbären und pseudobulbären Symptome eine interdisziplinäre Betreuung angeraten. Für die bulbären ALS-Symptome kann eine leichte Verlangsamung der Progredienz durch Riluzol erreicht werden. Die Übersicht fasst die wesentlichen Erkenntnisse zur Epidemiologie und Pathophysiologie der die Lebensqualität und Prognose von ALS-Patienten stark beeinträchtigenden bulbären und der pseudobulbären Symptome zusammen. Die dargestellten symptomatischen Behandlungsmöglichkeiten berücksichtigen speziell pragmatische Aspekte in der ambulanten Therapie.
    Der Nervenarzt 01/2010; 81(10). · 0.80 Impact Factor

Publication Stats

3k Citations
709.75 Total Impact Points

Institutions

  • 1991–2013
    • University of Wuerzburg
      • Department of Neurology
      Würzburg, Bavaria, Germany
  • 2011
    • University of Ferrara
      Ferrare, Emilia-Romagna, Italy
  • 2005
    • University of Rostock
      • Klinik und Poliklinik für Neurologie
      Rostock, Mecklenburg-Vorpommern, Germany
    • Evangelic Hospital Bielefeld
      Bielefeld, North Rhine-Westphalia, Germany
  • 2003
    • Universität Regensburg
      Ratisbon, Bavaria, Germany
  • 1996–1997
    • Martin Luther University of Halle-Wittenberg
      Halle-on-the-Saale, Saxony-Anhalt, Germany
    • Ludwig-Maximilians-University of Munich
      München, Bavaria, Germany
  • 1982–1994
    • Heinrich-Heine-Universität Düsseldorf
      • • Neurologische Klinik
      • • Department of Urology
      Düsseldorf, North Rhine-Westphalia, Germany