Prof. Dr. Stephan Zierz

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Seit dem Jahr 1994 Direktor der Klinik und Poliklinik für Neurologie.

Die Klinik deckt das gesamte Spektrum der neurologischen Krankheitsbilder ab. Die Betreuung der Patienten erfolgt auf zwei neurologischen Allgemeinstation, einer Stroke unit, einer Neurologischen Intensiv-Station. In den Ambulanzen wird eine Vielzahl von speziellen neurologischen Krankeitsbildern behandelt.

Klinische und wissenschaftliche Schwerpunkte der Klinik umfassen neuromuskuläre Erkrankungen, Kopfschmerzen und zerebrovaskuläre Erkrankungen.

Seit mehr als 15 Jahren besteht eine Kooperation mit der Neurologie der Mongolei mit jährlichen Fortbildungsveranstaltungen in Ulaan Bataar,  Hospitationen mongolischer Ärzte in Halle.

1973-1980: Medizinstudium an der Universität Gießen und Bonn

1980-1981: Assistenzarzt an der Pfalzklinik Landeck, Klingenmünster, Psychiatrisches Landeskrankenhaus

1981-1983: Wissenschaftlicher Assistent am Physiologisch-Chemischen Institut der Universität Göttingen

1984-1985: Research Fellow am Neuromuscular Research Center, Department of Neurology, Mayo Clinic Rochester, Minnesota

1986-1987: Assistenzarzt an der Neurologischen Universitätsklinik Bonn

1988: Assistenzarzt an der Epileptologischen Universitätsklinik Bonn

1989-1994: Oberarzt an der Neurologischen Universitätsklinik Bonn

19.12.1990: Habilitation für das Fach Neurologie "Ophthalmoplegia-plus und Kearns-Sayre-Syndrom: Klinik, Biochemie und Therapie­versuch mit Coenzym Q"

9.9.1993: Berufung auf die C4-Professur für Neurologie der Medizinischen Fakultät der Martin-Luther-Universität Halle-Wittenberg

Seit 1.9.1994 Professor (C4) für Neurologie und Direktor der Neurologischen Klinik der Martin-Luther-Universität Halle-Wittenberg

 

Akademische Funktionen

Dekan der Medizinischen Fakultät der Martin-Luther-Universität Halle-Wittenberg (in 2 Wahlperioden von 1996-2000)

Mitglied des Akademischen Senats der Martin-Luther-Universität Halle-Wittenberg (in 2 Wahlperioden von 1998-2003)

Gewählter Fachgutachter der Deutschen Forschungsgemeinschaft (DFG) (seit 2000)

  • Deutsche Gesellschaft für Neurologie e.V. (DGN)    
  • Deutsche Gesellschaft für  Muskelerkrankte e.V. (DGM)    
  • European Academy of Neurology (EAN, seit 2017 Fellow of EAN)

1. Adolph Seeligmüller (1837-1912).

Arendt C, Zierz S.

J Neurol. 2020 Oct 21. doi: 10.1007/s00415-020-10268-w. Online ahead of print.

PMID: 33084941 No abstract available.

2. Effect of Recanalization on Cerebral Edema, Long-Term Outcome, and Quality of Life in Patients with Large Hemispheric Infarctions.

Wartenberg KE, Henkner J, Brandt S, Zierz S, Müller TJ.

J Stroke Cerebrovasc Dis. 2020 Oct 6;29(12):105358. doi: 10.1016/j.jstrokecerebrovasdis.2020.105358. Online ahead of print.

PMID: 33035882

3. Palmitate but Not Oleate Exerts a Negative Effect on Oxygen Utilization in Myoblasts of Patients with the m.3243A>G Mutation: A Pilot Study.

Motlagh Scholle L, Schieffers H, Al-Robaiy S, Thaele A, Lehmann Urban D, Zierz S.

Life (Basel). 2020 Sep 16;10(9):204. doi: 10.3390/life10090204.

PMID: 32947855 Free PMC article.

4. Cellular Stress in the Pathogenesis of Muscular Disorders-From Cause to Consequence.

Mensch A, Zierz S.

Int J Mol Sci. 2020 Aug 13;21(16):5830. doi: 10.3390/ijms21165830.

PMID: 32823799 Free PMC article. Review.

5. The Effect of Resveratrol on Mitochondrial Function in Myoblasts of Patients with the Common m.3243A>G Mutation.

Motlagh Scholle L, Schieffers H, Al-Robaiy S, Thaele A, Dehghani F, Lehmann Urban D, Zierz S.

Biomolecules. 2020 Jul 24;10(8):1103. doi: 10.3390/biom10081103.

PMID: 32722320 Free PMC article.

6. Whole-body muscle MRI of patients with MATR3-associated distal myopathy reveals a distinct pattern of muscular involvement and highlights the value of whole-body examination.

Mensch A, Kraya T, Koester F, Müller T, Stoevesandt D, Zierz S.

J Neurol. 2020 Aug;267(8):2408-2420. doi: 10.1007/s00415-020-09862-9. Epub 2020 May 2.

PMID: 32361838 Free PMC article.

7. Exon skipping in Duchenne Muscle dystrophy due to a silent p.Ser443= mutation in the DMD gene.

Raj Joshi P, Sarangerel J, Munkhbayar R, Gläser D, Zierz S.

J Clin Neurosci. 2020 Jun;76:229-232. doi: 10.1016/j.jocn.2020.04.065. Epub 2020 Apr 19.

PMID: 32317190

8. Muscle Carnitine Palmitoyltransferase II (CPT II) Deficiency: A Conceptual Approach.

Joshi PR, Zierz S.

Molecules. 2020 Apr 13;25(8):1784. doi: 10.3390/molecules25081784.

PMID: 32295037 Free PMC article. Review.

9. Heteroplasmy and Copy Number in the Common m.3243A>G Mutation-A Post-Mortem Genotype-Phenotype Analysis.

Scholle LM, Zierz S, Mawrin C, Wickenhauser C, Urban DL.

Genes (Basel). 2020 Feb 18;11(2):212. doi: 10.3390/genes11020212.

PMID: 32085658 Free PMC article.

10. McArdle Disease: Clinical, Biochemical, Histological and Molecular Genetic Analysis of 60 Patients.

Joshi PR, Deschauer M, Zierz S.

Biomedicines. 2020 Feb 15;8(2):33. doi: 10.3390/biomedicines8020033.

PMID: 32075227 Free PMC article.

11. Basilar artery thrombosis during sexual intercourse.

Posa A, Mueller T, Ungurs O, Kornhuber M, Zierz S.

J Clin Neurosci. 2020 Apr;74:238-240. doi: 10.1016/j.jocn.2020.01.071. Epub 2020 Jan 22.

PMID: 31982277

12. Response: Late-onset Pompe disease manifests in the brain.

Schneider I, Hensel O, Zierz S.

Mol Genet Metab Rep. 2019 Oct 16;21:100516. doi: 10.1016/j.ymgmr.2019.100516. eCollection 2019 Dec.

PMID: 31660291 Free PMC article. No abstract available.

13. Prevalence and characteristics of headache attributed to ingestion or inhalation of a cold stimulus (HICS): A cross-sectional study.

Kraya T, Schulz-Ehlbeck M, Burow P, Watzke S, Zierz S.

Cephalalgia. 2020 Mar;40(3):299-306. doi: 10.1177/0333102419884938. Epub 2019 Oct 24.

PMID: 31645112

14. Reply: Adult-onset distal spinal muscular atrophy: a new phenotype associated with KIF5A mutations.

Brenner D, Rosenbohm A, Yilmaz R, Müller K, Grehl T, Petri S, Meyer T, Grosskreutz J, Weydt P, Ruf W, Neuwirth C, Weber M, Pinto S, Claeys KG, Schrank B, Jordan B, Knehr A, Günther K, Hübers A, Zeller D, Kubisch C, Jablonka S, Sendtner M, Klopstock T, de Carvalho M, Sperfeld A, Borck G, Volk AE, Dorst J, Weis J, Otto M, Schuster J, Del Tredici K, Braak H, Danzer KM, Freischmidt A, Meitinger T, Ludolph AC, Andersen PM, Weishaupt JH; German ALS network MND-NET.

Brain. 2019 Dec 1;142(12):e67. doi: 10.1093/brain/awz306.

PMID: 31612906 No abstract available.

15. Recurrent Myalgia since Early Infancy-Misleading Clinical Course in a Child with Carnitine Palmitoyltransferase-II Deficiency.

Arélin M, Zierz S, Ceglarek U, Heinemann M, Beblo S, Merkenschlager A.

Neuropediatrics. 2020 Feb;51(1):53-56. doi: 10.1055/s-0039-1694977. Epub 2019 Sep 21.

PMID: 31541997

16. Increased Blood Flow Velocity in Middle Cerebral Artery and Headache Upon Ingestion of Ice Water.

Hensel O, Burow P, Mages S, Wienke A, Kraya T, Zierz S.

Front Neurol. 2019 Jun 28;10:677. doi: 10.3389/fneur.2019.00677. eCollection 2019.

PMID: 31316454 Free PMC article.

17. White matter lesions in treated late onset Pompe disease are not different to matched controls.

Schneider I, Hensel O, Zierz S.

Mol Genet Metab. 2019 Jun;127(2):128-131. doi: 10.1016/j.ymgme.2019.05.007. Epub 2019 May 14.

PMID: 31153821

18. Understanding mitochondrial DNA maintenance disorders at the single muscle fibre level.

Lehmann D, Tuppen HAL, Campbell GE, Alston CL, Lawless C, Rosa HS, Rocha MC, Reeve AK, Nicholls TJ, Deschauer M, Zierz S, Taylor RW, Turnbull DM, Vincent AE.

Nucleic Acids Res. 2019 Aug 22;47(14):7430-7443. doi: 10.1093/nar/gkz472.

PMID: 31147703 Free PMC article.

19. Myositis in Lewis rats induced by the superantigen Staphylococcal enterotoxin A.

Emmer A, Abobarin-Adeagbo A, Posa A, Jordan B, Delank KS, Staege MS, Surov A, Zierz S, Kornhuber ME.

Mol Biol Rep. 2019 Aug;46(4):4085-4094. doi: 10.1007/s11033-019-04858-9. Epub 2019 May 13.

PMID: 31087247

20. Successful use of an immune checkpoint inhibitor in a patient with myasthenia gravis in remission.

Jordan B, Zierz S, Weber T, Jordan K.

Muscle Nerve. 2019 Jul;60(1):E7-E8. doi: 10.1002/mus.26495. Epub 2019 May 7.

PMID: 31018243 No abstract available.

21. Normal FGF-21-Serum Levels in Patients with Carnitine Palmitoyltransferase II (CPT II) Deficiency.

Motlagh Scholle L, Lehmann D, Joshi PR, Zierz S.

Int J Mol Sci. 2019 Mar 20;20(6):1400. doi: 10.3390/ijms20061400.

PMID: 30897730 Free PMC article.

22. Lack of activation of the S113L variant of carnitine palmitoyltransfersase II by cardiolipin.

Motlagh Scholle L, Thaele A, Beckers M, Meinhardt B, Zierz S.

J Bioenerg Biomembr. 2018 Dec;50(6):461-466. doi: 10.1007/s10863-018-9781-9. Epub 2019 Jan 3.

PMID: 30604089

23. Phenotype of carnitine palmitoyltransferase II (CPT II) deficiency: A questionnaire-based survey.

Joshi PR, Deschauer M, Zierz S.

J Clin Neurosci. 2019 Jan;59:32-36. doi: 10.1016/j.jocn.2018.11.023. Epub 2018 Nov 16.

PMID: 30455135

24. The p.S85C-mutation in MATR3 impairs stress granule formation in Matrin-3 myopathy.

Mensch A, Meinhardt B, Bley N, Hüttelmaier S, Schneider I, Stoltenburg-Didinger G, Kraya T, Müller T, Zierz S.

Exp Neurol. 2018 Aug;306:222-231. doi: 10.1016/j.expneurol.2018.05.012. Epub 2018 May 12.

PMID: 29763601 No abstract available.

25. Treatment of adult idiopathic inflammatory myopathies with conventional immunosuppressive drugs : Results of a retrospective study.

Keyßer G, Zierz S, Kornhuber M.

Z Rheumatol. 2019 Mar;78(2):183-189. doi: 10.1007/s00393-018-0471-0.

PMID: 29713867 English.

26. Decreased outlet angle of the superior cerebellar artery as indicator for dolichoectasia in late onset Pompe disease.

Hensel O, Schneider I, Wieprecht M, Kraya T, Zierz S.

Orphanet J Rare Dis. 2018 Apr 13;13(1):57. doi: 10.1186/s13023-018-0794-6.

PMID: 29653542 Free PMC article.

27. Comprehensive analysis of the mutation spectrum in 301 German ALS families.

Müller K, Brenner D, Weydt P, Meyer T, Grehl T, Petri S, Grosskreutz J, Schuster J, Volk AE, Borck G, Kubisch C, Klopstock T, Zeller D, Jablonka S, Sendtner M, Klebe S, Knehr A, Günther K, Weis J, Claeys KG, Schrank B, Sperfeld AD, Hübers A, Otto M, Dorst J, Meitinger T, Strom TM, Andersen PM, Ludolph AC, Weishaupt JH; German ALS network MND-NET.

J Neurol Neurosurg Psychiatry. 2018 Aug;89(8):817-827. doi: 10.1136/jnnp-2017-317611. Epub 2018 Apr 12.

PMID: 29650794

28. High-altitude cerebral edema or acute demyelinating encephalomyelitis in the Himalayas.

Hensel O, Niroula P, Paudel R, Sherpa T, Kraya T, Presek P, Zierz S.

Neurol Clin Pract. 2018 Feb;8(1):77-78. doi: 10.1212/CPJ.0000000000000416.

PMID: 29517058 Free PMC article. No abstract available.

29. TIA1 variant drives myodegeneration in multisystem proteinopathy with SQSTM1 mutations.

Lee Y, Jonson PH, Sarparanta J, Palmio J, Sarkar M, Vihola A, Evilä A, Suominen T, Penttilä S, Savarese M, Johari M, Minot MC, Hilton-Jones D, Maddison P, Chinnery P, Reimann J, Kornblum C, Kraya T, Zierz S, Sue C, Goebel H, Azfer A, Ralston SH, Hackman P, Bucelli RC, Taylor JP, Weihl CC, Udd B.

J Clin Invest. 2018 Mar 1;128(3):1164-1177. doi: 10.1172/JCI97103. Epub 2018 Feb 19.

PMID: 29457785 Free PMC article.

30. Frequency of Headache in Mitochondrial Disorders: A Response.

Kraya T, Zierz S.

Headache. 2018 Feb;58(2):315-316. doi: 10.1111/head.13253.

PMID: 29411368 No abstract available.

31. Hot-spot KIF5A mutations cause familial ALS.

Brenner D, Yilmaz R, Müller K, Grehl T, Petri S, Meyer T, Grosskreutz J, Weydt P, Ruf W, Neuwirth C, Weber M, Pinto S, Claeys KG, Schrank B, Jordan B, Knehr A, Günther K, Hübers A, Zeller D, Kubisch C, Jablonka S, Sendtner M, Klopstock T, de Carvalho M, Sperfeld A, Borck G, Volk AE, Dorst J, Weis J, Otto M, Schuster J, Del Tredici K, Braak H, Danzer KM, Freischmidt A, Meitinger T, Strom TM, Ludolph AC, Andersen PM, Weishaupt JH; German ALS network MND-NET.

Brain. 2018 Mar 1;141(3):688-697. doi: 10.1093/brain/awx370.

PMID: 29342275 Free PMC article.

32. FGF-21 as a Potential Biomarker for Mitochondrial Diseases.

Scholle LM, Lehmann D, Deschauer M, Kraya T, Zierz S.

Curr Med Chem. 2018;25(18):2070-2081. doi: 10.2174/0929867325666180111094336.

PMID: 29332568 Review.

33. Foodborne botulism due to ingestion of home-canned green beans: two case reports.

Hellmich D, Wartenberg KE, Zierz S, Mueller TJ.

J Med Case Rep. 2018 Jan 4;12(1):1. doi: 10.1186/s13256-017-1523-9.

PMID: 29301587 Free PMC article.

34. Cognitive impairment, clinical severity and MRI changes in MELAS syndrome.

Kraya T, Neumann L, Paelecke-Habermann Y, Deschauer M, Stoevesandt D, Zierz S, Watzke S.

Mitochondrion. 2019 Jan;44:53-57. doi: 10.1016/j.mito.2017.12.012. Epub 2017 Dec 29.

PMID: 29289801

35. Polyglucosan myopathy and functional characterization of a novel GYG1 mutation.

Hedberg-Oldfors C, Mensch A, Visuttijai K, Stoltenburg G, Stoevesandt D, Kraya T, Oldfors A, Zierz S.

Acta Neurol Scand. 2018 Mar;137(3):308-315. doi: 10.1111/ane.12865. Epub 2017 Nov 15.

PMID: 29143313

36. Prevalence of Headache in Patients With Mitochondrial Disease: A Cross-Sectional Study.

Kraya T, Deschauer M, Joshi PR, Zierz S, Gaul C.

Headache. 2018 Jan;58(1):45-52. doi: 10.1111/head.13219. Epub 2017 Nov 15.

PMID: 29139113

37. "Thoracic migraine" as a new manifestation of migraine: Case report.

Kraya T, Mages S, Zierz S.

Cephalalgia. 2018 Jun;38(7):1399-1401. doi: 10.1177/0333102417733960. Epub 2017 Sep 24.

PMID: 28944683

38. "Patients with amyotrophic lateral sclerosis (ALS) are usually nice persons"-How physicians experienced in ALS see the personality characteristics of their patients.

Mehl T, Jordan B, Zierz S.

Brain Behav. 2016 Nov 10;7(1):e00599. doi: 10.1002/brb3.599. eCollection 2017 Jan.

PMID: 28127517 Free PMC article.

39. Muscle Carnitine Palmitoyltransferase II Deficiency: A Review of Enzymatic Controversy and Clinical Features.

Lehmann D, Motlagh L, Robaa D, Zierz S.

Int J Mol Sci. 2017 Jan 3;18(1):82. doi: 10.3390/ijms18010082.

PMID: 28054946 Free PMC article. Review.

40. Cognitive fatigue in patients with myasthenia gravis.

Jordan B, Schweden TLK, Mehl T, Menge U, Zierz S.

Muscle Nerve. 2017 Sep;56(3):449-457. doi: 10.1002/mus.25540. Epub 2017 Mar 23.

PMID: 28033668

41. The concept and diagnostic criteria of primary lateral sclerosis.

Wais V, Rosenbohm A, Petri S, Kollewe K, Hermann A, Storch A, Hanisch F, Zierz S, Nagel G, Kassubek J, Weydt P, Brettschneider J, Weishaupt JH, Ludolph AC, Dorst J.

Acta Neurol Scand. 2017 Sep;136(3):204-211. doi: 10.1111/ane.12713. Epub 2016 Nov 15.

PMID: 27858953

42. Peripheral neuropathy in patients with CPEO associated with single and multiple mtDNA deletions.

Lehmann D, Kornhuber ME, Clajus C, Alston CL, Wienke A, Deschauer M, Taylor RW, Zierz S.

Neurol Genet. 2016 Oct 19;2(6):e113. doi: 10.1212/NXG.0000000000000113. eCollection 2016 Dec.

PMID: 27822509 Free PMC article.

43. Normal activities of AMP-deaminase and adenylate kinase in patients with McArdle disease.

Joshi PR, Apitz T, Zierz S.

Neurol Res. 2016 Dec;38(12):1052-1055. doi: 10.1080/01616412.2016.1243638. Epub 2016 Oct 20.

PMID: 27760513

44. Assessment of physical fatigability and fatigue perception in myasthenia gravis.

Jordan B, Mehl T, Schweden TLK, Menge U, Zierz S.

Muscle Nerve. 2017 May;55(5):657-663. doi: 10.1002/mus.25386. Epub 2017 Jan 6.

PMID: 27543741

45. MTHFR and ACE Polymorphisms Do Not Increase Susceptibility to Migraine Neither Alone Nor in Combination.

Essmeister R, Kress HG, Zierz S, Griffith L, Lea R, Wieser T.

Headache. 2016 Sep;56(8):1267-73. doi: 10.1111/head.12893. Epub 2016 Aug 2.

PMID: 27483173

46. Experimental provocation of 'ice-cream headache' by ice cubes and ice water.

Mages S, Hensel O, Zierz AM, Kraya T, Zierz S.

Cephalalgia. 2017 Apr;37(5):464-469. doi: 10.1177/0333102416650704. Epub 2016 May 19.

PMID: 27206961

47. Prevalence of Pompe disease in 3,076 patients with hyperCKemia and limb-girdle muscular weakness.

Lukacs Z, Nieves Cobos P, Wenninger S, Willis TA, Guglieri M, Roberts M, Quinlivan R, Hilton-Jones D, Evangelista T, Zierz S, Schlotter-Weigel B, Walter MC, Reilich P, Klopstock T, Deschauer M, Straub V, Müller-Felber W, Schoser B.

Neurology. 2016 Jul 19;87(3):295-8. doi: 10.1212/WNL.0000000000002758. Epub 2016 May 11.

PMID: 27170567 Free PMC article.

48. Stabilization of the thermolabile variant S113L of carnitine palmitoyltransferase II.

Motlagh L, Golbik R, Sippl W, Zierz S.

Neurol Genet. 2016 Feb 25;2(2):e53. doi: 10.1212/NXG.0000000000000053. eCollection 2016 Apr.

PMID: 27123472 Free PMC article.

49. Ice cream headache in students and family history of headache: a cross-sectional epidemiological study.

Zierz AM, Mehl T, Kraya T, Wienke A, Zierz S.

J Neurol. 2016 Jun;263(6):1106-10. doi: 10.1007/s00415-016-8098-z. Epub 2016 Apr 2.

PMID: 27039390

50. Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.

Vercruysse P, Sinniger J, El Oussini H, Scekic-Zahirovic J, Dieterlé S, Dengler R, Meyer T, Zierz S, Kassubek J, Fischer W, Dreyhaupt J, Grehl T, Hermann A, Grosskreutz J, Witting A, Van Den Bosch L, Spreux-Varoquaux O; GERP ALS Study Group, Ludolph AC, Dupuis L.

Brain. 2016 Apr;139(Pt 4):1106-22. doi: 10.1093/brain/aww004. Epub 2016 Mar 16.

PMID: 26984187 Clinical Trial.

51. Utility of a next-generation sequencing-based gene panel investigation in German patients with genetically unclassified limb-girdle muscular dystrophy.

Kuhn M, Gläser D, Joshi PR, Zierz S, Wenninger S, Schoser B, Deschauer M.

J Neurol. 2016 Apr;263(4):743-50. doi: 10.1007/s00415-016-8036-0. Epub 2016 Feb 17.

PMID: 26886200

52. Thymic pathologies in myasthenia gravis: a preoperative assessment of CAT scan and nuclear based imaging.

Jordan B, Kellner J, Jordan K, Bähre M, Behrmann C, Zierz S.

J Neurol. 2016 Apr;263(4):641-8. doi: 10.1007/s00415-016-8023-5. Epub 2016 Jan 25.

PMID: 26810725

53. Clinical features and differential diagnosis of flail arm syndrome.

Hübers A, Hildebrandt V, Petri S, Kollewe K, Hermann A, Storch A, Hanisch F, Zierz S, Rosenbohm A, Ludolph AC, Dorst J.

J Neurol. 2016 Feb;263(2):390-395. doi: 10.1007/s00415-015-7993-z. Epub 2015 Dec 24.

PMID: 26705123

54. Switch to double positive late onset MuSK myasthenia gravis following thymomectomy in paraneoplastic AChR antibody positive myasthenia gravis.

Jordan B, Schilling S, Zierz S.

J Neurol. 2016 Jan;263(1):174-6. doi: 10.1007/s00415-015-7982-2. Epub 2015 Dec 8.

PMID: 26645394 No abstract available.

55. Malony-CoA inhibits the S113L variant of carnitine-palmitoyltransferase II.

Motlagh L, Golbik R, Sippl W, Zierz S.

Biochim Biophys Acta. 2016 Jan;1861(1):34-40. doi: 10.1016/j.bbalip.2015.10.005. Epub 2015 Oct 23.

PMID: 26477380

56. HMGB1 and RAGE in skeletal muscle inflammation: Implications for protein accumulation in inclusion body myositis.

Muth IE, Zschüntzsch J, Kleinschnitz K, Wrede A, Gerhardt E, Balcarek P, Schreiber-Katz O, Zierz S, Dalakas MC, Voll RE, Schmidt J.

Exp Neurol. 2015 Sep;271:189-97. doi: 10.1016/j.expneurol.2015.05.023. Epub 2015 Jun 3.

PMID: 26048613

57. Pathogenic mitochondrial mt-tRNA(Ala) variants are uniquely associated with isolated myopathy.

Lehmann D, Schubert K, Joshi PR, Hardy SA, Tuppen HA, Baty K, Blakely EL, Bamberg C, Zierz S, Deschauer M, Taylor RW.

Eur J Hum Genet. 2015 Dec;23(12):1735-8. doi: 10.1038/ejhg.2015.73. Epub 2015 Apr 15.

PMID: 25873012 Free PMC article.

58. A novel m.7539C>T point mutation in the mt-tRNA(Asp) gene associated with multisystemic mitochondrial disease.

Lehmann D, Schubert K, Joshi PR, Baty K, Blakely EL, Zierz S, Taylor RW, Deschauer M.

Neuromuscul Disord. 2015 Jan;25(1):81-4. doi: 10.1016/j.nmd.2014.09.008. Epub 2014 Sep 28.

PMID: 25447692 Free PMC article.

59. Frequencies of myohistological mitochondrial changes in patients with mitochondrial DNA deletions and the common m.3243A>G point mutation.

Zierz CM, Joshi PR, Zierz S.

Neuropathology. 2015 Apr;35(2):130-6. doi: 10.1111/neup.12173. Epub 2014 Nov 6.

PMID: 25378026

60. Histological characterization and biochemical analysis of paraspinal muscles in neuromuscularly healthy subjects.

Zimmermann C, Kalepu R, Ponfick M, Reichel H, Cakir B, Zierz S, Gdynia HJ, Kassubek J, Ludolph AC, Rosenbohm A.

Muscle Nerve. 2015 Jul;52(1):45-54. doi: 10.1002/mus.24490. Epub 2015 May 28.

PMID: 25307884

61. Phenotype of matrin-3-related distal myopathy in 16 German patients.

Müller TJ, Kraya T, Stoltenburg-Didinger G, Hanisch F, Kornhuber M, Stoevesandt D, Senderek J, Weis J, Baum P, Deschauer M, Zierz S.

Ann Neurol. 2014 Nov;76(5):669-80. doi: 10.1002/ana.24255. Epub 2014 Sep 16.

PMID: 25154462

62. SANDO syndrome in a cohort of 107 patients with CPEO and mitochondrial DNA deletions.

Hanisch F, Kornhuber M, Alston CL, Taylor RW, Deschauer M, Zierz S.

J Neurol Neurosurg Psychiatry. 2015 Jun;86(6):630-4. doi: 10.1136/jnnp-2013-306748. Epub 2014 Aug 20.

PMID: 25143630

63. Electrical human motor nerve stimulation by Johann Christian Reil in 1792.

Kornhuber ME, Zierz S.

Muscle Nerve. 2014 Jun;49(6):931-2. doi: 10.1002/mus.24200. Epub 2014 Apr 8.

PMID: 24715478 No abstract available.

64. Normal protein content but abnormally inhibited enzyme activity in muscle carnitine palmitoyltransferase II deficiency.

Lehmann D, Zierz S.

J Neurol Sci. 2014 Apr 15;339(1-2):183-8. doi: 10.1016/j.jns.2014.02.011. Epub 2014 Feb 20.

PMID: 24602495

65. Carnitine palmitoyltransferase II (CPT II) deficiency: genotype-phenotype analysis of 50 patients.

Joshi PR, Deschauer M, Zierz S.

J Neurol Sci. 2014 Mar 15;338(1-2):107-11. doi: 10.1016/j.jns.2013.12.026. Epub 2013 Dec 23.

PMID: 24398345

66. The significance of pathological spontaneous activity in various myopathies.

Hanisch F, Kronenberger C, Zierz S, Kornhuber M.

Clin Neurophysiol. 2014 Jul;125(7):1485-90. doi: 10.1016/j.clinph.2013.11.021. Epub 2013 Dec 2.

PMID: 24370491

67. Sialylation and muscle performance: sialic acid is a marker of muscle ageing.

Hanisch F, Weidemann W, Großmann M, Joshi PR, Holzhausen HJ, Stoltenburg G, Weis J, Zierz S, Horstkorte R.

PLoS One. 2013 Dec 11;8(12):e80520. doi: 10.1371/journal.pone.0080520. eCollection 2013.

PMID: 24349002 Free PMC article.

68. Distal myopathies: from clinical classification to molecular understanding.

Kraya T, Zierz S.

J Neural Transm (Vienna). 2013 Sep;120 Suppl 1:S3-7. doi: 10.1007/s00702-013-1058-1. Epub 2013 Jul 11.

PMID: 23842731 Review.

69. [Treatability of sporadic late onset nemaline myopathy].

Hanisch F, Schneider I, Müller T, Romeike BF, Stoltenburg G, Holzhausen HJ, Zierz S.

Nervenarzt. 2013 Aug;84(8):955-61. doi: 10.1007/s00115-013-3825-5.

PMID: 23836301 Review. German.

70. Diagnostic impact of myotonic discharges in myofibrillar myopathies.

Hanisch F, Kraya T, Kornhuber M, Zierz S.

Muscle Nerve. 2013 Jun;47(6):845-8. doi: 10.1002/mus.23716. Epub 2013 Apr 21.

PMID: 23605961

71. [Myofibrillary myopathy due to the ZASP mutation Ala147Thr : two cases with exclusively distal leg involvement].

Kraya T, Kress W, Stoevesant D, Deschauer M, Zierz S.

Nervenarzt. 2013 Feb;84(2):209-13. doi: 10.1007/s00115-012-3689-0.

PMID: 23263837 German.

72. Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.

Joshi PR, Deschauer M, Zierz S.

Wien Klin Wochenschr. 2012 Dec;124(23-24):851-4. doi: 10.1007/s00508-012-0296-9. Epub 2012 Nov 27.

PMID: 23184072

73. Respiratory function in late-onset Pompe disease patients receiving long-term enzyme replacement therapy for more than 48 months.

Schneider I, Hanisch F, Müller T, Schmidt B, Zierz S.

Wien Med Wochenschr. 2013 Jan;163(1-2):40-4. doi: 10.1007/s10354-012-0153-5. Epub 2012 Nov 19.

PMID: 23160972

74. C19orf12 mutations in neurodegeneration with brain iron accumulation mimicking juvenile amyotrophic lateral sclerosis.

Deschauer M, Gaul C, Behrmann C, Prokisch H, Zierz S, Haack TB.

J Neurol. 2012 Nov;259(11):2434-9. doi: 10.1007/s00415-012-6521-7. Epub 2012 May 15.

PMID: 22584950

75. Unusual manifestations in two cases of necrotizing myopathy associated with SRP-antibodies.

Hanisch F, Müller T, Stoltenburg G, Zierz S.

Clin Neurol Neurosurg. 2012 Sep;114(7):1104-6. doi: 10.1016/j.clineuro.2011.12.055. Epub 2012 Feb 3.

PMID: 22306424

76. [Muscular dystrophy due to mutations in anoctamin 5: clinical and molecular genetic findings].

Deschauer M, Joshi PR, Gläser D, Hanisch F, Stoltenburg G, Zierz S.

Nervenarzt. 2011 Dec;82(12):1596-603. doi: 10.1007/s00115-011-3325-4.

PMID: 21739273 German.

77. [Facioscapulohumeral muscular dystrophy. Clinical picture, atypical forms, diagnostics, genetics].

Jordan B, Müller-Reible C, Zierz S.

Nervenarzt. 2011 Jun;82(6):712-22. doi: 10.1007/s00115-010-2968-x.

PMID: 21567298 German.

78. Phenotype variability and histopathological findings in centronuclear myopathy due to DNM2 mutations.

Hanisch F, Müller T, Dietz A, Bitoun M, Kress W, Weis J, Stoltenburg G, Zierz S.

J Neurol. 2011 Jun;258(6):1085-90. doi: 10.1007/s00415-010-5889-5. Epub 2011 Jan 9.

PMID: 21221624

79. Camptocormia phenotype of FSHD: a clinical and MRI study on six patients.

Jordan B, Eger K, Koesling S, Zierz S.

J Neurol. 2011 May;258(5):866-73. doi: 10.1007/s00415-010-5858-z. Epub 2010 Dec 17.

PMID: 21165637

80. Beevor's sign in facioscapulohumeral muscular dystrophy: an old sign with new implications.

Eger K, Jordan B, Habermann S, Zierz S.

J Neurol. 2010 Mar;257(3):436-8. doi: 10.1007/s00415-009-5342-9. Epub 2009 Oct 17.

PMID: 19838767

81. Gasperini syndrome as clinical manifestation of pontine demyelination.

Krasnianski M, Müller T, Zierz S, Winterholler M.

Eur J Med Res. 2009 Sep 1;14(9):413-4. doi: 10.1186/2047-783x-14-9-413.

PMID: 19748860 Free PMC article.

82. Mycophenolate mofetil as second line immunosuppressant in Myasthenia gravis--a long-term prospective open-label study.

Hanisch F, Wendt M, Zierz S.

Eur J Med Res. 2009 Aug 12;14(8):364-6. doi: 10.1186/2047-783x-14-8-364.

PMID: 19666397 Free PMC article.

83. Use of complementary and alternative medicine in patients suffering from primary headache disorders.

Gaul C, Eismann R, Schmidt T, May A, Leinisch E, Wieser T, Evers S, Henkel K, Franz G, Zierz S.

Cephalalgia. 2009 Oct;29(10):1069-78. doi: 10.1111/j.1468-2982.2009.01841.x. Epub 2009 Apr 2.

PMID: 19366356 Clinical Trial.

84. [Polymyositis associated with thymoma].

Jordan B, Eger K, Zierz S.

Nervenarzt. 2009 Jun;80(6):708-11. doi: 10.1007/s00115-009-2689-1.

PMID: 19347264 German.

85. Efalizumab-induced isolated cerebral lupus-like syndrome.

Wendt M, Wohlrab J, Zierz S, Deschauer M.

Neurology. 2009 Jan 6;72(1):96-7. doi: 10.1212/01.wnl.0000338627.07348.d8.

PMID: 19122039 No abstract available.

86. Orthognathic surgery in a case of infantile facioscapulohumeral muscular dystrophy with macroglossia.

Kriwalsky MS, Deschauer M, Eckert AW, Schubert J, Zierz S.

Oral Maxillofac Surg. 2008 Dec;12(4):195-8. doi: 10.1007/s10006-008-0116-6.

PMID: 18682994

87. Triple A syndrome mimicking ALS.

Strauss M, Koehler K, Krumbholz M, Huebner A, Zierz S, Deschauer M.

Amyotroph Lateral Scler. 2008 Oct;9(5):315-7. doi: 10.1080/17482960802259016.

PMID: 18615337

88. Molecular diagnosis of German patients with late-onset glycogen storage disease type II.

Joshi PR, Gläser D, Schmidt S, Vorgerd M, Winterholler M, Eger K, Zierz S, Deschauer M.

J Inherit Metab Dis. 2008 Dec;31 Suppl 2:S261-5. doi: 10.1007/s10545-008-0820-2. Epub 2008 Jul 10.

PMID: 18607768

89. Bruns syndrome caused by intraventricular tumor.

Krasnianski M, Müller T, Stock K, Zierz S.

Eur J Med Res. 2008 Apr 30;13(4):179-81.

PMID: 18504174

90. AMP deaminase deficiency in skeletal muscle is unlikely to be of clinical relevance.

Hanisch F, Joshi P, Zierz S.

J Neurol. 2008 Mar;255(3):318-22. doi: 10.1007/s00415-008-0530-6. Epub 2008 Mar 14.

PMID: 18338202

91. [Neuromyelitis optica: a separate disease entity associated with aquaporin-4-antibodies].

Jordan B, Eger K, Kornhuber ME, Zierz S.

Fortschr Neurol Psychiatr. 2008 Jan;76(1):21-7. doi: 10.1055/s-2007-993032.

PMID: 18189220 Review. German.

92. Bruns syndrome caused by intraventricular tumor.

Krasnianski M, Müller T, Stock K, Zierz S.

Eur J Med Res. 2007 Dec 14;12(12):582-4.

PMID: 18024268

93. Frequency of calpain-3 c.550delA mutation in limb girdle muscular dystrophy type 2 and isolated hyperCKemia in German patients.

Hanisch F, Müller CR, Grimm D, Xue L, Traufeller K, Merkenschlager A, Zierz S, Deschauer M.

Clin Neuropathol. 2007 Jul-Aug;26(4):157-63. doi: 10.5414/npp26157.

PMID: 17702496

94. MELAS associated with mutations in the POLG1 gene.

Deschauer M, Tennant S, Rokicka A, He L, Kraya T, Turnbull DM, Zierz S, Taylor RW.

Neurology. 2007 May 15;68(20):1741-2. doi: 10.1212/01.wnl.0000261929.92478.3e.

PMID: 17502560 No abstract available.

95. Analysis of spectrum and frequencies of mutations in McArdle disease. Identification of 13 novel mutations.

Deschauer M, Morgenroth A, Joshi PR, Gläser D, Chinnery PF, Aasly J, Schreiber H, Knape M, Zierz S, Vorgerd M.

J Neurol. 2007 Jun;254(6):797-802. doi: 10.1007/s00415-006-0447-x. Epub 2007 Apr 3.

PMID: 17404776

96. Subtle cognitive dysfunction in adult onset myotonic dystrophy type 1 (DM1) and type 2 (DM2).

Gaul C, Schmidt T, Windisch G, Wieser T, Müller T, Vielhaber S, Zierz S, Leplow B.

Neurology. 2006 Jul 25;67(2):350-2. doi: 10.1212/01.wnl.0000225180.27833.c1.

PMID: 16864839 Clinical Trial.

97. [Isolated pareses of the terminal branches of the facial nerve: clinical findings, etiology and differential diagnosis].

Kissig B, Zierz S.

Fortschr Neurol Psychiatr. 2006 Jul;74(7):371-6. doi: 10.1055/s-2005-915579.

PMID: 16804805 Review. German.

98. Cardiac involvement in limb-girdle muscular dystrophy 2I : conventional cardiac diagnostic and cardiovascular magnetic resonance.

Gaul C, Deschauer M, Tempelmann C, Vielhaber S, Klein HU, Heinze HJ, Zierz S, Grothues F.

J Neurol. 2006 Oct;253(10):1317-22. doi: 10.1007/s00415-006-0213-0. Epub 2006 Jun 19.

PMID: 16786213

99. Between Wallenberg syndrome and hemimedullary lesion: Cestan-Chenais and Babinski-Nageotte syndromes in medullary infarctions.

Krasnianski M, Müller T, Stock K, Zierz S.

J Neurol. 2006 Nov;253(11):1442-6. doi: 10.1007/s00415-006-0231-3. Epub 2006 Jun 14.

PMID: 16775654

100. Novel mitochondrial transfer RNA(Phe) gene mutation associated with late-onset neuromuscular disease.

Deschauer M, Swalwell H, Strauss M, Zierz S, Taylor RW.

Arch Neurol. 2006 Jun;63(6):902-5. doi: 10.1001/archneur.63.6.902.

PMID: 16769874

101. Isolated bilateral blindness as the sole manifestation of transient ischaemic attacks.

Krasnianski M, Bau V, Neudecker S, Lindner A, Zierz S.

Acta Ophthalmol Scand. 2006 Jun;84(3):415-8. doi: 10.1111/j.1600-0420.2006.00635.x.

PMID: 16704710

102. Lactate production upon short-term non-ischemic forearm exercise in mitochondrial disorders and other myopathies.

Hanisch F, Eger K, Bork S, Lehnich H, Deschauer M, Zierz S.

J Neurol. 2006 Jun;253(6):735-40. doi: 10.1007/s00415-006-0101-7. Epub 2006 Apr 20.

PMID: 16619130 Clinical Trial.

103. Genetic heterogeneity in 30 German patients with oculopharyngeal muscular dystrophy.

Müller T, Deschauer M, Kolbe-Fehr F, Zierz S.

J Neurol. 2006 Jul;253(7):892-5. doi: 10.1007/s00415-006-0126-y. Epub 2006 Apr 20.

PMID: 16619122

104. Lactate increase and oxygen desaturation in mitochondrial disorders--evaluation of two diagnostic screening protocols.

Hanisch F, Müller T, Muser A, Deschauer M, Zierz S.

J Neurol. 2006 Apr;253(4):417-23. doi: 10.1007/s00415-006-0987-0. Epub 2006 Apr 20.

PMID: 16619117

105. Molecular and biochemical investigations in fumarase deficiency.

Deschauer M, Gizatullina Z, Schulze A, Pritsch M, Knöppel C, Knape M, Zierz S, Gellerich FN.

Mol Genet Metab. 2006 Jun;88(2):146-52. doi: 10.1016/j.ymgme.2006.01.007. Epub 2006 Feb 28.

PMID: 16510303

106. Ecto- and cytosolic 5'-nucleotidases in normal and AMP deaminase-deficient human skeletal muscle.

Hanisch F, Hellsten Y, Zierz S.

Biol Chem. 2006 Jan;387(1):53-8. doi: 10.1515/BC.2006.008.

PMID: 16497164

107. [Ocular involvement in MuSK antibody-positive myasthenia gravis].

Bau V, Hanisch F, Hain B, Zierz S.

Klin Monbl Augenheilkd. 2006 Jan;223(1):81-3. doi: 10.1055/s-2005-858629.

PMID: 16418940 German.

108. Successful treatment of MuSK antibody-positive myasthenia gravis with rituximab.

Hain B, Jordan K, Deschauer M, Zierz S.

Muscle Nerve. 2006 Apr;33(4):575-80. doi: 10.1002/mus.20479.

PMID: 16323216

109. Update on chronic progressive external ophthalmoplegia.

Bau V, Zierz S.

Strabismus. 2005 Sep;13(3):133-42. doi: 10.1080/09273970500216432.

PMID: 16251143 Review.

110. Only subtle cognitive deficits in non-bulbar amyotrophic lateral sclerosis patients.

Röttig D, Leplow B, Eger K, Ludolph AC, Graf M, Zierz S.

J Neurol. 2006 Mar;253(3):333-9. doi: 10.1007/s00415-005-0992-8. Epub 2005 Oct 10.

PMID: 16208524

111. [Hypokalemic thyrotoxic periodic paralysis (HTPP). Rare differential diagnosis in case of acute tetraparesis in Europe].

Gaul C, Leonhardt G, Spens A, Schneyer U, Zierz S.

Med Klin (Munich). 2005 Sep 15;100(9):583-6. doi: 10.1007/s00063-005-1080-7.

PMID: 16170648 German.

112. Late-onset mitochondrial myopathy with dystrophic changes due to a G7497A mutation in the mitochondrial tRNA(Ser(UCN)) gene.

Müller T, Deschauer M, Neudecker S, Zierz S.

Acta Neuropathol. 2005 Oct;110(4):426-30. doi: 10.1007/s00401-005-1063-z. Epub 2005 Aug 25.

PMID: 16133542

113. Congenital fibrosis of extraocular muscles type 1 with progression of ophthalmoplegia.

Hanisch F, Bau V, Zierz S.

Eur J Med Res. 2005 Aug 17;10(8):366-8.

PMID: 16131480

114. The problem of interlab variation in methods for mitochondrial disease diagnosis: enzymatic measurement of respiratory chain complexes.

Gellerich FN, Mayr JA, Reuter S, Sperl W, Zierz S.

Mitochondrion. 2004 Sep;4(5-6):427-39. doi: 10.1016/j.mito.2004.07.007. Epub 2004 Oct 18.

PMID: 16120404

115. Cyclic vertical deviation after ocular myositis and treatment by recession of the inferior rectus muscle.

Bau V, Sievert M, Roggenkämper P, Zierz S.

Graefes Arch Clin Exp Ophthalmol. 2005 Oct;243(10):1062-5. doi: 10.1007/s00417-005-1173-2. Epub 2005 Oct 20.

PMID: 15906070

116. Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies.

Krasnianski A, Deschauer M, Neudecker S, Gellerich FN, Müller T, Schoser BG, Krasnianski M, Zierz S.

Brain. 2005 Aug;128(Pt 8):1870-6. doi: 10.1093/brain/awh540. Epub 2005 May 18.

PMID: 15901649

117. Dilated cardiomyopathy may be an early sign of the C826A Fukutin-related protein mutation.

Müller T, Krasnianski M, Witthaut R, Deschauer M, Zierz S.

Neuromuscul Disord. 2005 May;15(5):372-6. doi: 10.1016/j.nmd.2005.02.004.

PMID: 15833432

118. A novel ANT1 gene mutation with probable germline mosaicism in autosomal dominant progressive external ophthalmoplegia.

Deschauer M, Hudson G, Müller T, Taylor RW, Chinnery PF, Zierz S.

Neuromuscul Disord. 2005 Apr;15(4):311-5. doi: 10.1016/j.nmd.2004.12.004. Epub 2005 Jan 28.

PMID: 15792871

119. [Differential influence of immune therapy on relapses and progression in multiple sclerosis: interpretation and therapeutic consequences].

Kornhuber ME, Presek P, Zierz S.

Fortschr Neurol Psychiatr. 2005 Mar;73(3):143-9. doi: 10.1055/s-2004-830102.

PMID: 15747223 Review. German.

120. False-positive diagnosis of a single, large-scale mitochondrial DNA deletion by Southern blot analysis: the role of neutral polymorphisms.

Deschauer M, Krasnianski A, Zierz S, Taylor RW.

Genet Test. 2004 Winter;8(4):395-9. doi: 10.1089/gte.2004.8.395.

PMID: 15684869

121. Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects.

Deschauer M, Wieser T, Zierz S.

Arch Neurol. 2005 Jan;62(1):37-41. doi: 10.1001/archneur.62.1.37.

PMID: 15642848

122. Effects of extramitochondrial ADP on permeability transition of mouse liver mitochondria.

Gizatullina ZZ, Chen Y, Zierz S, Gellerich FN.

Biochim Biophys Acta. 2005 Jan 7;1706(1-2):98-104. doi: 10.1016/j.bbabio.2004.09.013.

PMID: 15620369

123. Mitochondrial function in turkey skeletal muscle--impact on meat quality.

Opalka JR, Wicke M, Gellerich FN, Schmidt R, Rosner F, Zierz S, von Lengerken G.

Br Poult Sci. 2004 Jun;45(3):367-79. doi: 10.1080/00071660410001730860.

PMID: 15327123

124. [Classical crossed pontine syndromes].

Krasnianski M, Neudecker S, Zierz S.

Fortschr Neurol Psychiatr. 2004 Aug;72(8):460-8. doi: 10.1055/s-2004-818392.

PMID: 15305240 German.

125. Alfred Hauptmann, Siegfried Thannhauser, and an endangered muscular disorder.

Krasnianski M, Ehrt U, Neudecker S, Zierz S.

Arch Neurol. 2004 Jul;61(7):1139-41. doi: 10.1001/archneur.61.7.1139.

PMID: 15262752 No abstract available.

126. Rimmed vacuoles in facioscapulohumeral muscular dystrophy: a unique ultrastructural feature.

Neudecker S, Krasnianski M, Bahn E, Zierz S.

Acta Neuropathol. 2004 Sep;108(3):257-9. doi: 10.1007/s00401-004-0894-3. Epub 2004 Jun 24.

PMID: 15221332

127. [The clinical spectrum of limb-girdle muscular dystrophies type 2I in cases of a mutation in the "fukutin-related- protein"-gene].

Krasnianski M, Neudecker S, Deschauer M, Zierz S.

Nervenarzt. 2004 Aug;75(8):770-5. doi: 10.1007/s00115-004-1726-3.

PMID: 15221067 German.

128. [Congenital fibrosis of extraocular muscles (CFEOM) and other phenotypes of congenital cranial dysinnervation syndromes (CCDD)].

Hanisch F, Bau V, Zierz S.

Nervenarzt. 2005 Apr;76(4):395-402. doi: 10.1007/s00115-004-1742-3.

PMID: 15221064 Review. German.

129. External ophthalmoplegia due to ocular myositis in a patient with ophthalmic herpes zoster.

Krasnianski M, Sievert M, Bau V, Zierz S.

Neuromuscul Disord. 2004 Jul;14(7):438-41. doi: 10.1016/j.nmd.2004.03.007.

PMID: 15210167

130. Persistent ocular motor disturbances in migraine without aura.

Wieser T, Wolff R, Hoffmann KP, Schulte-Mattler W, Zierz S.

Neurol Sci. 2004 Apr;25(1):8-12. doi: 10.1007/s10072-004-0218-6.

PMID: 15060809

131. Energetic depression caused by mitochondrial dysfunction.

Gellerich FN, Trumbeckaite S, Müller T, Deschauer M, Chen Y, Gizatullina Z, Zierz S.

Mol Cell Biochem. 2004 Jan-Feb;256-257(1-2):391-405. doi: 10.1023/b:mcbi.0000009885.34498.e6.

PMID: 14977198 Review.

132. Different sensitivities of CPT I and CPT II for inhibition by l-aminocarnitine in human skeletal muscle.

Traufeller K, Gellerich FN, Zierz S.

Biochim Biophys Acta. 2004 Feb 15;1608(2-3):149-54. doi: 10.1016/j.bbabio.2003.11.005.

PMID: 14871492

133. [Avellis' syndrome in brainstem infarctions].

Krasnianski M, Neudecker S, Schlüter A, Zierz S.

Fortschr Neurol Psychiatr. 2003 Dec;71(12):650-3. doi: 10.1055/s-2003-45345.

PMID: 14661158 German.

134. [The Schmidt and Vernet classical syndrome. Alternating brain stem syndromes that do not exist?].

Krasnianski M, Neudecker S, Zierz S.

Nervenarzt. 2003 Dec;74(12):1150-4. doi: 10.1007/s00115-003-1610-6.

PMID: 14647918 Review. German.

135. Only transient increase of serum CoQ subset 10 during long-term CoQ10 therapy in mitochondrial ophthalmoplegia.

Hanisch F, Zierz S.

Eur J Med Res. 2003 Nov 12;8(11):485-91.

PMID: 14644702 Clinical Trial.

136. Atypical phenotypes in patients with facioscapulohumeral muscular dystrophy 4q35 deletion.

Krasnianski M, Eger K, Neudecker S, Jakubiczka S, Zierz S.

Arch Neurol. 2003 Oct;60(10):1421-5. doi: 10.1001/archneur.60.10.1421.

PMID: 14568813 Clinical Trial.

137. Typical facioscapulohumeral dystrophy phenotype in patients without FSHD 4q35 deletion.

Krasnianski M, Neudecker S, Eger K, Jakubiczka S, Zierz S.

J Neurol. 2003 Sep;250(9):1084-7. doi: 10.1007/s00415-003-0158-5.

PMID: 14504970

138. Babinski-Nageotte's syndrome and Hemimedullary (Reinhold's) syndrome are clinically and morphologically distinct conditions.

Krasnianski M, Neudecker S, Schluter A, Zierz S.

J Neurol. 2003 Aug;250(8):938-42. doi: 10.1007/s00415-003-1118-9.

PMID: 12928912

139. [Classical crossed syndromes of the medulla oblongata. A historical and topodiagnostic discussion].

Krasnianski M, Winterholler M, Neudecker S, Zierz S.

Fortschr Neurol Psychiatr. 2003 Aug;71(8):397-405. doi: 10.1055/s-2003-41192.

PMID: 12910445 Review. German.

140. A splice junction mutation in muscle carnitine palmitoyltransferase II deficiency.

Deschauer M, Chrzanowska-Lightowlers ZM, Biekmann E, Pourfarzam M, Taylor RW, Turnbull DM, Zierz S.

Mol Genet Metab. 2003 Jun;79(2):124-8. doi: 10.1016/s1096-7192(03)00067-2.

PMID: 12809643

141. [Age dependence of Doppler parameters in the basal cerebral arteries evaluated by transcranial color-coded duplex sonography. Reference data from 290 volunteers].

Shambal S, Grehl H, Zierz S, Lindner A.

Fortschr Neurol Psychiatr. 2003 May;71(5):271-7. doi: 10.1055/s-2003-39064.

PMID: 12740759 Clinical Trial. German.

142. Carnitine palmitoyltransferase II deficiency: molecular and biochemical analysis of 32 patients.

Wieser T, Deschauer M, Olek K, Hermann T, Zierz S.

Neurology. 2003 Apr 22;60(8):1351-3. doi: 10.1212/01.wnl.0000055901.58642.48.

PMID: 12707442

143. Absence of known familial hemiplegic migraine (FHM) mutations in the CACNA1A gene in patients with common migraine: implications for genetic testing.

Wieser T, Mueller C, Evers S, Zierz S, Deufel T.

Clin Chem Lab Med. 2003 Mar;41(3):272-5. doi: 10.1515/CCLM.2003.042.

PMID: 12705332

144. Calpain inhibitor (BSF 409425) diminishes ischemia/reperfusion-induced damage of rabbit heart mitochondria.

Trumbeckaite S, Neuhof C, Zierz S, Gellerich FN.

Biochem Pharmacol. 2003 Mar 1;65(5):911-6. doi: 10.1016/s0006-2952(02)01610-6.

PMID: 12628497

145. [Facioscapulohumeral muscular dystrophy. The spectrum of clinical manifestations and molecular genetic changes].

Krasnianski M, Neudecker S, Eger K, Schulte-Mattler W, Zierz S.

Nervenarzt. 2003 Feb;74(2):151-8. doi: 10.1007/s00115-002-1455-4.

PMID: 12596016 German.

146. Energetic depression caused by mitochondrial dysfunction.

Gellerich FN, Trumbeckaite S, Chen Y, Deschauer M, M ller T, Zierz S.

Eur Cytokine Netw. 2002 Oct-Dec;13(4):395-7.

PMID: 12517722 Review.

147. Two novel mutations in the myophosphorylase gene in a patient with McArdle disease.

Deschauer M, Hertel K, Zierz S.

Muscle Nerve. 2003 Jan;27(1):105-7. doi: 10.1002/mus.10261.

PMID: 12508303

148. Mitochondrial dysfunction in sepsis: evidence from bacteraemic baboons and endotoxaemic rabbits.

Gellerich FN, Trumbeckaite S, Opalka JR, Gellerich JF, Chen Y, Neuhof C, Redl H, Werdan K, Zierz S.

Biosci Rep. 2002 Feb;22(1):99-113. doi: 10.1023/a:1016017224003.

PMID: 12418553 Review.

149. [Hauptmann-Thannhauser muscular dystrophy and differential diagnosis of myopathies associated with contractures].

Hanisch F, Neudecker S, Wehnert M, Zierz S.

Nervenarzt. 2002 Oct;73(10):1004-11. doi: 10.1007/s00115-002-1388-y.

PMID: 12376891 German.

150. Mitochondrial respiratory rates and activities of respiratory chain complexes correlate linearly with heteroplasmy of deleted mtDNA without threshold and independently of deletion size.

Gellerich FN, Deschauer M, Chen Y, Müller T, Neudecker S, Zierz S.

Biochim Biophys Acta. 2002 Oct 3;1556(1):41-52. doi: 10.1016/s0005-2728(02)00305-5.

PMID: 12351217

151. The quantitation of ADP diffusion gradients across the outer membrane of heart mitochondria in the presence of macromolecules.

Gellerich FN, Laterveer FD, Zierz S, Nicolay K.

Biochim Biophys Acta. 2002 Apr 22;1554(1-2):48-56. doi: 10.1016/s0005-2728(02)00212-8.

PMID: 12034470

152. Effect of the new matrix metalloproteinase inhibitor RO-28-2653 on mitochondrial function.

Opalka JR, Gellerich FN, Kling L, Müller-Beckmann B, Zierz S.

Biochem Pharmacol. 2002 Feb 15;63(4):725-32. doi: 10.1016/s0006-2952(01)00867-x.

PMID: 11992641

153. Brainstem infarctions with normal MRI.

Krasnianski M, Lindner A, Zierz S.

Eur J Med Res. 2002 Mar 28;7(3):125-7.

PMID: 11953283

154. A novel nonsense mutation (515del4) in muscle carnitine palmitoyltransferase II deficiency.

Deschauer M, Wieser T, Schröder R, Zierz S.

Mol Genet Metab. 2002 Feb;75(2):181-5. doi: 10.1006/mgme.2001.3281.

PMID: 11855939

155. A novel nonsense mutation (R269X) in the myophosphorylase gene in a patient with McArdle disease.

Deschauer M, Opalka JR, Lindner A, Zierz S.

Mol Genet Metab. 2001 Dec;74(4):489-91. doi: 10.1006/mgme.2001.3252.

PMID: 11749054

156. Age and sex dependency of carnitine concentration in human serum and skeletal muscle.

Opalka JR, Gellerich FN, Zierz S.

Clin Chem. 2001 Dec;47(12):2150-3.

PMID: 11719481 No abstract available.

157. Hearing impairment is common in various phenotypes of the mitochondrial DNA A3243G mutation.

Deschauer M, Müller T, Wieser T, Schulte-Mattler W, Kornhuber M, Zierz S.

Arch Neurol. 2001 Nov;58(11):1885-8. doi: 10.1001/archneur.58.11.1885.

PMID: 11708999

158. [Progressive limb-girdle muscular dystrophy].

Eger K, Zierz S.

Dtsch Med Wochenschr. 2001 Jun 1;126(22):655-8. doi: 10.1055/s-2001-14484.

PMID: 11450625 Review. German. No abstract available.

159. Doppler microembolic signals in patients with two different types of bileaflet valves.

Georgiadis D, Braun S, Uhlmann F, Bernacca GM, Schulte-Mattler WJ, Zierz S, Zerkowski HR.

J Thorac Cardiovasc Surg. 2001 Jun;121(6):1101-6. doi: 10.1067/mtc.2001.113176.

PMID: 11385377

160. Length dependence of variables associated with temporal dispersion in human motor nerves.

Schulte-Mattler WJ, Müller T, Georgiadis D, Kornhuber ME, Zierz S.

Muscle Nerve. 2001 Apr;24(4):527-33. doi: 10.1002/mus.1036.

PMID: 11268025 Clinical Trial.

161. [Familial mitochondrial chronic progressive external ophthalmoplegia. Five families with differing genetics].

Deschauer M, Müller T, Dreha S, Zierz S.

Nervenarzt. 2001 Feb;72(2):122-9. doi: 10.1007/s001150050724.

PMID: 11256146 German.

162. Different sensitivity of rabbit heart and skeletal muscle to endotoxin-induced impairment of mitochondrial function.

Trumbeckaite S, Opalka JR, Neuhof C, Zierz S, Gellerich FN.

Eur J Biochem. 2001 Mar;268(5):1422-9. doi: 10.1046/j.1432-1327.2001.02012.x.

PMID: 11231295

163. Images in clinical medicine. Retroperitoneal hemorrhage.

Lindner A, Zierz S.

N Engl J Med. 2001 Feb 1;344(5):348. doi: 10.1056/NEJM200102013440505.

PMID: 11172166 No abstract available.

164. Discharge patterns of spontaneous activity and motor units on concentric needle electromyography.

Schulte-Mattler WJ, Georgiadis D, Zierz S.

Muscle Nerve. 2001 Jan;24(1):123-6. doi: 10.1002/1097-4598(200101)24:1<123::aid-mus18>3.0.co;2-v.

PMID: 11150976 Clinical Trial.

165. GCG repeats and phenotype in oculopharyngeal muscular dystrophy.

Müller T, Schröder R, Zierz S.

Muscle Nerve. 2001 Jan;24(1):120-2. doi: 10.1002/1097-4598(200101)24:1<120::aid-mus17>3.0.co;2-0.

PMID: 11150975

166. Automated identification of Doppler microembolic signals: comparison of two techniques.

Georgiadis D, Uhlmann F, Astler M, Cencetti S, Zierz S.

Neurol Res. 2000 Oct;22(7):738-40. doi: 10.1080/01616412.2000.11740749.

PMID: 11091982

167. Higher proportion of mitochondrial A3243G mutation in blood than in skeletal muscle in a patient with cardiomyopathy and hearing loss.

Deschauer M, Neudecker S, Müller T, Gellerich FN, Zierz S.

Mol Genet Metab. 2000 Jul;70(3):235-7. doi: 10.1006/mgme.2000.3002.

PMID: 10924279

168. [Bilateral inferior hemianopsia as an early symptom of Heidenhain type Creutzfeldt-Jakob disease].

Deschauer M, Stephan M, Stuhlträger U, Holzhausen HJ, Duncker G, Zierz S.

Klin Monbl Augenheilkd. 2000 Apr;216(4):227-31. doi: 10.1055/s-2000-10549.

PMID: 10820709 German.

169. Function of the mitochondrial outer membrane as a diffusion barrier in health and diseases.

Gellerich FN, Trumbeckaite S, Opalka JR, Seppet E, Rasmussen HN, Neuhoff C, Zierz S.

Biochem Soc Trans. 2000 Feb;28(2):164-9. doi: 10.1042/bst0280164.

PMID: 10816120 Review.

170. Differentiation between true microembolic signals and artefacts using an arbitrary sample volume.

Georgiadis D, Uhlmann F, Lindner A, Zierz S.

Ultrasound Med Biol. 2000 Mar;26(3):493-6. doi: 10.1016/s0301-5629(99)00158-1.

PMID: 10773382

171. A family with PROMM not linked to the recently mapped PROMM locus DM2.

Wieser T, Bönsch D, Eger K, Schulte-Mattler W, Zierz S.

Neuromuscul Disord. 2000 Feb;10(2):141-3. doi: 10.1016/s0960-8966(99)00081-4.

PMID: 10714590

172. Genetics of carnitine palmitoyltransferase II deficiencies.

Wieser T, Deschauer M, Zierz S.

Adv Exp Med Biol. 1999;466:339-45. doi: 10.1007/0-306-46818-2_39.

PMID: 10709661 Review. No abstract available.

173. Kinetic investigation of carnitine palmitoyltransferases in homogenates of human skeletal muscle using L-amino-carnitine and malonyl-CoA.

Hertel K, Gellerich FN, Hein W, Zierz S.

Adv Exp Med Biol. 1999;466:87-93. doi: 10.1007/0-306-46818-2_9.

PMID: 10709631 No abstract available.

174. Relation between maximum discharge rates on electromyography and motor unit number estimates.

Schulte-Mattler WJ, Georgiadis D, Tietze K, Zierz S.

Muscle Nerve. 2000 Feb;23(2):231-8. doi: 10.1002/(sici)1097-4598(200002)23:2<231::aid-mus14>3.0.co;2-4.

PMID: 10639616 Clinical Trial.

175. Predictive value of S-100beta and neuron-specific enolase serum levels for adverse neurologic outcome after cardiac surgery.

Georgiadis D, Berger A, Kowatschev E, Lautenschläger C, Börner A, Lindner A, Schulte-Mattler W, Zerkowski HR, Zierz S, Deufel T.

J Thorac Cardiovasc Surg. 2000 Jan;119(1):138-47. doi: 10.1016/s0022-5223(00)70229-7.

PMID: 10612773

176. Transcranial magnetic stimulation compared with upper motor neuron signs in patients with amyotrophic lateral sclerosis.

Schulte-Mattler WJ, Müller T, Zierz S.

J Neurol Sci. 1999 Nov 15;170(1):51-6. doi: 10.1016/s0022-510x(99)00201-4.

PMID: 10540036

177. Mitochondrial 3243 A-->G mutation (MELAS mutation) associated with painful muscle stiffness.

Deschauer M, Wieser T, Neudecker S, Lindner A, Zierz S.

Neuromuscul Disord. 1999 Jul;9(5):305-7. doi: 10.1016/s0960-8966(99)00019-x.

PMID: 10407850

178. Venous microemboli in patients with artificial heart valves.

Georgiadis D, Baumgartner RW, Uhlmann F, Lindner A, Zerkowski HR, Zierz S.

Cerebrovasc Dis. 1999 Jul-Aug;9(4):238-41. doi: 10.1159/000015962.

PMID: 10393412

179. Assessment of temporal dispersion in motor nerves with normal conduction velocity.

Schulte-Mattler WJ, Jakob M, Zierz S.

Clin Neurophysiol. 1999 Apr;110(4):740-7. doi: 10.1016/s1388-2457(98)00068-6.

PMID: 10378747

180. Impaired energy metabolism in hearts of septic baboons: diminished activities of Complex I and Complex II of the mitochondrial respiratory chain.

Gellerich FN, Trumbeckaite S, Hertel K, Zierz S, Müller-Werdan U, Werdan K, Redl H, Schlag G.

Shock. 1999 May;11(5):336-41.

PMID: 10353539

181. Treatment of tension-type headache with botulinum toxin: a pilot study.

Schulte-Mattler WJ, Wieser T, Zierz S.

Eur J Med Res. 1999 May 26;4(5):183-6.

PMID: 10336407

182. Focal sensory nerve abnormalities in patients with amyotrophic lateral sclerosis.

Schulte-Mattler WJ, Jakob M, Zierz S.

J Neurol Sci. 1999 Jan 15;162(2):189-93. doi: 10.1016/s0022-510x(98)00321-9.

PMID: 10202986

183. [The diagnosis of encephalopathy, neuropathy and myopathy in the critical patient].

Lindner A, Hertel K, Zierz S.

Dtsch Med Wochenschr. 1999 Mar 5;124(9):257-60. doi: 10.1055/s-2007-1024281.

PMID: 10102012 Review. German. No abstract available.

184. Dextran strongly increases the Michaelis constants of oxidative phosphorylation and of mitochondrial creatine kinase in heart mitochondria.

Gellerich FN, Laterveer FD, Korzeniewski B, Zierz S, Nicolay K.

Eur J Biochem. 1998 May 15;254(1):172-80. doi: 10.1046/j.1432-1327.1998.2540172.x.

PMID: 9652411

185. [Acute encephalopathy, polyneuropathy and myopathy in the critically ill patient].

Lindner A, Kappen K, Zierz S.

Internist (Berl). 1998 May;39(5):485-92. doi: 10.1007/s001080050200.

PMID: 9647983 Review. German. No abstract available.

186. The forgotten condyle: Delayed hypoglossal nerve palsy caused by fracture of the occipital condyle.

Demisch S, Lindner A, Beck R, Zierz S.

Clin Neurol Neurosurg. 1998 Mar;100(1):44-5. doi: 10.1016/s0303-8467(97)00111-x.

PMID: 9637204

187. Influence of transducer frequency on Doppler microemboli signals in an in vivo model.

Georgiadis D, Wenzel A, Zerkowski HR, Zierz S, Lindner A.

Neurol Res. 1998 Apr;20(3):198-200. doi: 10.1080/01616412.1998.11740506.

PMID: 9583579

188. Intracranial microembolic signals in patients with artificial heart valves: drowning in numbers.

Georgiadis D, Lindner A, Zierz S.

Eur J Med Res. 1998 Feb 21;3(1-2):99-102.

PMID: 9512976 Review.

189. Automated intraoperative detection of Doppler microembolic signals using the bigate approach.

Georgiadis D, Wenzel A, Zerkowski HR, Zierz S, Lindner A.

Stroke. 1998 Jan;29(1):137-9. doi: 10.1161/01.str.29.1.137.

PMID: 9445342

190. [Proximal myotonic myopathy (PROMM). Clinical variability within a family].

Eger K, Schulte-Mattler WJ, Zierz S.

Nervenarzt. 1997 Oct;68(10):839-44. doi: 10.1007/s001150050203.

PMID: 9441258 German.

191. Influence of oxygen ventilation on Doppler microemboli signals in patients with artificial heart valves.

Georgiadis D, Wenzel A, Lehmann D, Lindner A, Zerkowski HR, Zierz S, Spencer MP.

Stroke. 1997 Nov;28(11):2189-94. doi: 10.1161/01.str.28.11.2189.

PMID: 9368563

192. Time course of high-intensity transient signals in patients undergoing elective heart valve replacement: a prospective study.

Lindner A, Georgiadis D, Lühmann A, Stephan M, Preiss M, Zerkowski HR, Zierz S.

J Heart Valve Dis. 1997 Sep;6(5):527-30.

PMID: 9330175

193. Identification of Doppler microembolic signals with a bigate probe in patients with prosthetic heart valves.

Lindner A, Georgiadis D, Fischer G, Zerkowski HR, Zierz S.

Eur J Med Res. 1997 Jul 28;2(7):299-301.

PMID: 9233904

194. Doppler microembolic signals in children with prosthetic cardiac valves.

Georgiadis D, Preiss M, Lindner A, Gybels Y, Zierz S, Zerkowski HR.

Stroke. 1997 Jul;28(7):1328-9. doi: 10.1161/01.str.28.7.1328.

PMID: 9227678

195. [Differential sciatica pain diagnosis from the neurologic viewpoint].

Lindner A, Zierz S.

Med Klin (Munich). 1997 Jun 15;92(6):335-43. doi: 10.1007/BF03044774.

PMID: 9297065 Review. German.

196. [Multiple, disseminated hypodensities in cranial CT. Multiple disseminated ischemia in pneumococcus-induced cerebral vasculitis].

Deschauer M, Lindner A, Behrmann C, Zierz S.

Radiologe. 1997 Jun;37(6):481-2. doi: 10.1007/s001170050242.

PMID: 9340678 German. No abstract available.

197. [Postpartum obturator nerve syndrome: case report and review of the nerve compression syndrome during pregnancy and delivery].

Lindner A, Schulte-Mattler W, Zierz S.

Zentralbl Gynakol. 1997;119(3):93-9.

PMID: 9173773 Review. German.

198. Autonomic neuropathy in a patient with adrenomyeloneuropathy.

Schulte-Mattler WJ, Lindner A, Zierz S.

Eur J Med Res. 1996 Nov 25;1(12):559-61.

PMID: 9438162

199. [Delusion of mercury poisoning in multiple sclerosis].

Brieger P, Schulte-Mattler W, Zierz S.

Nervenarzt. 1996 Sep;67(9):785-8. doi: 10.1007/s001150050054.

PMID: 8992377 German.