J Finsterer

Summary

Country: Germany

Publications

  1. doi Unclassified cardiomyopathies in neuromuscular disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180, Vienna, Austria
    Wien Med Wochenschr 163:505-13. 2013
  2. doi Hirayama disease in Austria
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria Electronic address
    Joint Bone Spine 80:503-7. 2013
  3. doi Parasitoses of the human central nervous system
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Helminthol 87:257-70. 2013
  4. doi Mitochondrial epilepsy in pediatric and adult patients
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Scand 128:141-52. 2013
  5. doi Dementia from the ABCD1 mutation c.1415-1416delAG in a female carrier
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Gene 530:155-7. 2013
  6. doi Influence of mitochondrion-toxic agents on the cardiovascular system
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Regul Toxicol Pharmacol 67:434-45. 2013
  7. ncbi Repurposed drugs in metabolic disorders
    Josef Finsterer
    Postfach 20, 1180 Vienna, Austria
    Curr Top Med Chem 13:2386-94. 2013
  8. doi Cardiomyopathy in neurological disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Cardiovasc Pathol 22:389-400. 2013
  9. doi Sudden death possibly related to lenalidomide given for cardiac and muscle AL amyloidosis secondary to light chain deposition disease
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Oncol Pharm Pract 19:170-4. 2013
  10. ncbi Brachydactylia as a phenotypic feature of mitochondrial disorder
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Med Iran 50:831-5. 2012

Detail Information

Publications153 found, 100 shown here

  1. doi Unclassified cardiomyopathies in neuromuscular disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180, Vienna, Austria
    Wien Med Wochenschr 163:505-13. 2013
    ..Unclassified CMPs are frequently associated with noncardiac disease, including neuromuscular disorders (NMDs). This review aims at summarizing and discussing recent findings concerning the association of NMDs with unclassified CMPs...
  2. doi Hirayama disease in Austria
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria Electronic address
    Joint Bone Spine 80:503-7. 2013
    ..The study aimed at systematically collecting cases of HD in Austria and at describing and discussing their presentation on clinical and instrumental investigations and at comparing them with cases reported from other countries...
  3. doi Parasitoses of the human central nervous system
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Helminthol 87:257-70. 2013
    ..g. Angiostrongylus costaricensis) or whether they remain undetected or asymptomatic, like 25% of neurocysticerciasis cases. ..
  4. doi Mitochondrial epilepsy in pediatric and adult patients
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Scand 128:141-52. 2013
    ..To optimize management of mitochondrial epilepsy, it is essential to differentiate between early and late-onset forms. ..
  5. doi Dementia from the ABCD1 mutation c.1415-1416delAG in a female carrier
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Gene 530:155-7. 2013
    ..We report a unique female X-ALD carrier presenting with severe, progressive dementia, paraspasticity, sphincteric dysfunction, and multisystem disease...
  6. doi Influence of mitochondrion-toxic agents on the cardiovascular system
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Regul Toxicol Pharmacol 67:434-45. 2013
    ..Particularly coenzyme-Q, riboflavin, vitamin-E, vitamin-C, L-carnitine, vitamin-D, thiamin, folic acid, omega-3 fatty acids, and D-ribose may alleviate mitochondrial cardiotoxic effects...
  7. ncbi Repurposed drugs in metabolic disorders
    Josef Finsterer
    Postfach 20, 1180 Vienna, Austria
    Curr Top Med Chem 13:2386-94. 2013
    ..Future investigations will need to identify which candidate drugs may leave the pipeline status to acquire approval for new indications. ..
  8. doi Cardiomyopathy in neurological disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria Electronic address
    Cardiovasc Pathol 22:389-400. 2013
    ..Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. ..
  9. doi Sudden death possibly related to lenalidomide given for cardiac and muscle AL amyloidosis secondary to light chain deposition disease
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Oncol Pharm Pract 19:170-4. 2013
    ..Restrictive cardiomyopathy due to AL amyloidosis has not been reported as the cause of sudden death. The risk of sudden death in AL amyloidosis may be further increased by potentially cardiotoxic medication, as in the following case...
  10. ncbi Brachydactylia as a phenotypic feature of mitochondrial disorder
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Med Iran 50:831-5. 2012
    ..The family history was positive for diabetes but negative for brachydactylia or other features of a MID. MIDs may be associated with brachydactylia. Skeletal deformities may be a phenotypic manifestation of MIDs...
  11. doi Familal left ventricular hypertrabeculation (noncompaction) is myopathic
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Danube University Krems, Austria
    Int J Cardiol 164:312-7. 2013
    ..In most cases LVHT is associated with neuromuscular disorders (NMDs) or other rare non-neuromuscular genetic syndromes. Occasionally, LVHT occurs familiarly...
  12. ncbi Alive with almost no Brain: Severe Post-Hemorrhagic Internal Hydrocephalus
    J Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, Vienna, Vienna 1180, Austria
    Med J Malaysia 68:86-7. 2013
    ..Severe post-hemorrhaghic internal hydrocephalus with almost complete atrophy of the cerebral parenchyma, as in the following case, is rare...
  13. doi Presentation of adult mitochondrial epilepsy
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Seizure 22:119-23. 2013
    ..We were interested in the types of epilepsy, the prevalence of mitochondrial epilepsy, the type and effectiveness of treatment, and in the outcome of adult MID patients with epilepsy...
  14. doi Thrombolysis of ischemic stroke from noncompaction in metabolic myopathy
    Josef Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neurologist 18:296-7. 2012
    ..Ischemic stroke has not been reported together with left ventricular hypertrabeculation/noncompaction (LVHT) and metabolic myopathy...
  15. ncbi Beneficial effect of ivabradine in dilated cardiomyopathy from Becker muscular dystrophy
    J Finsterer
    Danube University Krems, Krems, Osterreich
    Herz 37:702-5. 2012
    ..The I(f) blocker ivabradine reduces heart rate and improves systolic function without causing arterial hypotension. Ivabradine has not been reported to improve cardiac involvement in Becker muscular dystrophy (BMD)...
  16. doi Leukoencephalopathies in mitochondrial disorders: clinical and MRI findings
    Josef Finsterer
    Danube University Krems, Krems, Austria
    J Neuroimaging 22:e1-11. 2012
    ..Because the presence of LEM has an impact on the prognosis of an MID, all MID patients should undergo cerebral imaging even in the absence of clinical CNS manifestations...
  17. doi Arrhythmia-related workup in hereditary myopathies
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Electrocardiol 45:376-84. 2012
    ..Arrhythmias determine life expectancy in patients with hereditary myopathies...
  18. doi Cognitive dysfunction in mitochondrial disorders
    J Finsterer
    Danube University Krems, Krems and Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Scand 126:1-11. 2012
    ..Cognitive impairment may be a CNS manifestation of syndromic as well as non-syndromic MIDs. Correct diagnosis of mitochondrial cognitive impairment is a prerequisite for the optimal management of these patients...
  19. doi Hereditary spastic paraplegias with autosomal dominant, recessive, X-linked, or maternal trait of inheritance
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Wien, Austria
    J Neurol Sci 318:1-18. 2012
    ..Among the XL-SPGs, 3 causative genes have been identified (L1CAM (SPG1), PLP1 (SPG2), and SLC16A2 (SPG22)). The diagnosis of SPGs is based on clinical, instrumental and genetic investigations. Treatment is exclusively symptomatic...
  20. doi Epilepsy in mitochondrial disorders
    Josef Finsterer
    Danube University Krems, Krems, Austria
    Seizure 21:316-21. 2012
    ..Aim of the review was to describe epilepsy in syndromic and non-syndromic mitochondrial disorders with epilepsy as a dominant or collateral feature of the phenotype...
  21. doi Mimicry between mitochondrial disorder and multiple sclerosis
    Josef Finsterer
    Danube University Krems, Austria
    Metab Brain Dis 27:217-20. 2012
    ..It is concluded that even if CSF investigations or imaging studies suggest MS, differentials such as MIDs need to be excluded before prescribing medication possibly toxic to a MID. An "inflammatory CSF" may also occur in MIDs...
  22. doi Dilative arteriopathy in metabolic myopathies, particularly Pompe's disease
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria
    Acta Neurol Belg 112:15-8. 2012
    ..Early recognition of ectasias and aneurysms and appropriate interventions by enzyme replacement therapy, stenting, coiling or surgery may prevent mild or severe cerebrovascular complications or even death in some of these patients...
  23. doi Inherited mitochondrial disorders
    Josef Finsterer
    Danube University Krems, Vienna, Austria Europe
    Adv Exp Med Biol 942:187-213. 2012
    ..Genetic testing should be guided by the phenotype, the biopsy findings, and the biochemical results...
  24. ncbi Dopamine-deficiency-enhanced hyperthermia and rhabdomyolysis during a heat wave in a metachromatic leucodystrophy heterozygote with metabolic myopathy
    Josef Finsterer
    Acta Neurol Belg 111:321-4. 2011
    ..Hyper-CK-emia returned to similar levels as before rhabdomyolysis. Reduction of dopamine agonists during a heat wave may induce a dopamine deficiency syndrome with hyperthermia, rhabdomyolysis and thrombocytopenia...
  25. pmc Cardiac involvement in Becker muscular dystrophy
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Can J Cardiol 24:786-92. 2008
    ..Because CI in BMD is progressive and adequate therapy is available, cardiac investigations need to be regularly repeated. If CI in BMD is recognized early, appropriate therapy may be applied early, resulting in a more favourable outcome...
  26. ncbi Asymptomatic, progressive electrocardiographic abnormalities over twelve years in myotonic dystrophy 1
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna Austria
    Acta Cardiol 62:513-5. 2007
  27. ncbi Bulbar and spinal muscular atrophy (Kennedy's disease): a review
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur J Neurol 16:556-61. 2009
    ..The course is slowly progressive, the ability to walk lost only late in life, only few patients require ventilatory support, and life expectancy only slightly reduced...
  28. doi Heterozygous promotor haplotype LXA/LYB in MBL-deficiency associated with myopathy and left ventricular hypertrabeculation/noncompaction
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Ir J Med Sci 180:909-11. 2011
    ..To report the genetic background of mannose-binding lectin (MBL)-deficiency in a patient with recurrent infections, cardiac disease, and myopathy...
  29. ncbi Refsum disease due to the splice-site mutation c.135-2A>G before exon 3 of the PHYH gene, diagnosed eight years after detection of retinitis pigmentosa
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Neurol Sci 266:182-6. 2008
    ..If Refsum disease (RD) is not considered as a differential at onset of the initial manifestations the diagnosis of RD remains unrecognized for a long time as in the following case...
  30. doi Recurrent posterior reversible encephalopathy syndrome in mitochondrial disorder
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Blood Press 18:126-9. 2009
    ..Recurrent posterior reversible encephalopathy syndrome (PRES) has not been reported in association with multi-system mitochondrial disorder (MID)...
  31. doi [Cerebral CT and MRI in mitchondrial disorders]
    J Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180, Wien, Osterreich
    Nervenarzt 80:700-7. 2009
    ....
  32. doi Laminar cortical necrosis in mitochondrial disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Clin Neurol Neurosurg 111:655-8. 2009
    ....
  33. doi Treatment of mitochondrial disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur J Paediatr Neurol 14:29-44. 2010
    ..Despite limited possibilities, symptomatic treatment should be offered to MID patients, since it can have a significant impact on the course and outcome...
  34. doi Malignant hyperthermia susceptibility in a patient with mitochondrial disorder
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria
    Metab Brain Dis 24:501-6. 2009
    ..Though previous general anesthesias had been carried out without major complications, it cannot be ruled out that MH-like manifestations may develop during future anesthesias if trigger agents are not avoided...
  35. doi Disappearance of episodic weakness during pregnancy in hyperkalemic periodic paralysis from the SCNA4 mutation T704M
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe
    Neurologist 15:289-90. 2009
    ..Disappearance of episodic weakness during pregnancy and an increase in myotonia after delivery have not been reported in hyperkalemic periodic paralysis (hyperPP)...
  36. doi Management of mitochondrial stroke-like-episodes
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna and Danube University, Krems, Austria
    Eur J Neurol 16:1178-84. 2009
    ..The most effective strategy for the treatment of SLEs appears to be the application of L-arginine, coenzyme-Q, steroids, edaravone, creatine-monohydrate, or dichloracetate...
  37. doi Drugs interfering with mitochondrial disorders
    Josef Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Drug Chem Toxicol 33:138-51. 2010
    ..This review aims to give an overview about those agents that interfere with mitochondrial functions...
  38. doi Prevalence of Barth syndrome in adult left ventricular hypertrabeculation / noncompaction
    Josef Finsterer
    Hospital Rudolfstiftung, Vienna, Austria
    Scand Cardiovasc J 42:157-60. 2008
    ..Aim of the present investigation was to determine the prevalence of these characteristics in a large cohort of adult LVHT patients...
  39. ncbi Primary periodic paralyses
    J Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Scand 117:145-58. 2008
    ..To review the current knowledge about primary periodic paralyses (PPs)...
  40. doi Multi-system mitochondrial disorder with recurrent steroid-responsive eosinophilia
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria
    Rheumatol Int 30:135-9. 2009
    ..Mitochondrial disease may go along with marked eosinophilia mimicking Churg–Strauss syndrome. Steroids may be useful to resolve episodic eosinophilia but may be ineffective for other features of mitochondrial disorders...
  41. ncbi Significance of CK-elevation in noncompaction with regard to cardiac and neuromuscular disease
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Juchgasse 25, 1130 Vienna, Europe, Austria
    Int J Cardiol 130:174-9. 2008
    ....
  42. doi Recovery from acute paraplegia due to spontaneous spinal, epidural hematoma under minimal-dose acetyl-salicylic acid
    Josef Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neurol Sci 29:271-3. 2008
    ..Spontaneous spinal epidural hematoma (SEH) has not been reported under anti-thrombotic therapy with acetyl-salicylic acid (ASA) in a dosage of 50 mg/d...
  43. doi [MELAS syndrome as a differential diagnosis of ischemic stroke]
    J Finsterer
    Krankenanstalt Rudolfstiftung, Wien, Osterreich
    Fortschr Neurol Psychiatr 77:25-31. 2009
    ..The diagnosis is confirmed by demonstration of a biochemical respiratory chain defect or one of the disease-causing mutations, of which 80 % affect the mitochondrial tRNALeu gene...
  44. doi Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria, Europe
    Pediatr Cardiol 30:659-81. 2009
    ..This study aimed to review the current knowledge about the genetic or pathogenetic background of LVHT...
  45. ncbi Strategies for primary and secondary stroke prevention in atrial fibrillation
    J Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neth J Med 66:327-33. 2008
    ....
  46. doi Leigh and Leigh-like syndrome in children and adults
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Pediatr Neurol 39:223-35. 2008
    ..Associated mutations affect genes of the mitochondrial or nuclear genome. Leigh syndrome and Leigh-like syndrome are the mitochondrial disorders with the largest genetic heterogeneity...
  47. doi Non-manifesting Refsum heterozygotes carrying the c.135-2A>G PAHX gene transition
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neurol Sci 29:173-5. 2008
    ..In the absence of elevated phytanic acid concentrations, clinical neurologic abnormalities in heterozygous relatives of Refsum patients are not attributable to heterozygosity for PAHX mutations...
  48. doi Mitochondrial disorders, cognitive impairment and dementia
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria address
    J Neurol Sci 283:143-8. 2009
    ..Overall, mitochondrial dementia is an important differential of dementias and should be considered in patients with multi-system disease...
  49. doi Asymptomatic, nonsustained ventricular tachycardia in myotonic dystrophy type 1 detected with a loop recorder
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur Neurol 60:109-11. 2008
  50. pmc Management of peripheral facial nerve palsy
    Josef Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Postfach 20, 1180, Vienna, Austria
    Eur Arch Otorhinolaryngol 265:743-52. 2008
    ..Prognosis of Bell's palsy is fair with complete recovery in about 80% of the cases, 15% experience some kind of permanent nerve damage and 5% remain with severe sequelae...
  51. ncbi Is atherosclerosis a mitochondrial disorder?
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Vasa 36:229-40. 2007
    ..Overall, atherosclerosis appears to be the endpoint of various different pathogenetic mechanisms, of which oxidative stress and disturbed mitochondrial metabolism and function are key factors...
  52. doi Acquired left ventricular noncompaction as a cardiac manifestation of neuromuscular disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Scand Cardiovasc J 42:25-30. 2008
    ....
  53. doi Primary myopathies and the heart
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Scand Cardiovasc J 42:9-24. 2008
    ..Patients with myopathy should be cardiologically investigated as soon as their neurological diagnosis is established, since supportive cardiac therapy is available, which markedly influences prognosis and outcome of CI in these patients...
  54. ncbi Progressive mitral valve thickening and progressive muscle cramps as manifestations of glycogenosis VII (Tarui's Disease)
    Josef Finsterer
    Second Medical Department, and Krankenanstalt Rudolfstiftung, Vienna, Austria
    Cardiology 110:238-40. 2008
    ..Valve thickening may consecutively lead to valve insufficiency, enlargement of the atrium and atrial fibrillation. Progression of neurological manifestations may manifest as worsening muscle cramps...
  55. doi [Mitochondrial myopathies]
    J Finsterer
    Krankenanstalt Rudolfstiftung, Wien Osterreich
    Fortschr Neurol Psychiatr 77:631-8. 2009
    ..General anesthesia needs to be performed in the same way as in patients with susceptibility for malignant hyperthermia...
  56. doi Stroke in myopathies
    Josef Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Cerebrovasc Dis 29:6-13. 2010
    ..Only few data are available about the risk of myopathy patients experiencing a cerebral stroke...
  57. ncbi EFNS guidelines on the molecular diagnosis of mitochondrial disorders
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Danube University Krems, Krems, Austria
    Eur J Neurol 16:1255-64. 2009
    ....
  58. doi Recovery of systolic dysfunction in duchenne muscular dystrophy due to the point mutation c.4213C>T
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Cardiology 117:265-7. 2010
    ....
  59. doi Parkinson's syndrome and Parkinson's disease in mitochondrial disorders
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Danube University, Krems, Austria
    Mov Disord 26:784-91. 2011
    ....
  60. doi Inherited mitochondrial neuropathies
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Neurol Sci 304:9-16. 2011
    ..Involvement of other organs may require specific treatment. Mitochondrial neuropathies should be included in the differential diagnosis of hereditary neuropathies...
  61. doi Neurological manifestations of atrio-esophageal fistulas from left atrial ablation
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur J Neurol 18:1212-9. 2011
    ..Diagnostic work-up must avoid measures, which enlarge the fistula. Treatment is surgical exclusively...
  62. ncbi Is resection of a thymoma WHO A indicated in the absence of myasthenia gravis?
    J Finsterer
    Krankenanstalt Rudolfstiftung, Medical University, Vienna, Austria
    Clin Ter 162:37-9. 2011
    ..In conclusion a thymoma WHO A requires complete surgical resection, irrespective if the thymoma is accompanied by MG or not...
  63. doi Restrictive cardiomyopathy as a cardiac manifestation of myofibrillar myopathy
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Heart Lung 40:e123-7. 2011
    ..Restrictive cardiomyopathy (RCM) has been repeatedly reported as a cardiac manifestation of certain neuromuscular disorders, but only in single patients with myofibrillar myopathy (MFMP)...
  64. doi Deterioration of anti-Yo-associated paraneoplastic cerebellar degeneration
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Neurol Sci 308:139-41. 2011
    ..Usually, the course of paraneoplastic cerebellar degeneration(PCD) is stable or progresses only slowly. Sudden marked progression after several years, as in the following case, has not been reported...
  65. doi Motor neuron, nerve, and neuromuscular junction disease
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Curr Opin Neurol 24:469-74. 2011
    ..The aim is to review the most relevant findings published during the last year concerning clinical, genetic, pathogenic, and therapeutic advances in motor neuron disease, neuropathies, and neuromuscular junction disorders...
  66. ncbi Brachydactylia associated with mitochondrial disorder in an octogenarian
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria
    Kobe J Med Sci 56:E239-41. 2011
    ..Whether the relation between brachydactylia and mitochondrial disease was causal or coincidental remains speculative...
  67. doi Mitochondrial toxicity of antiepileptic drugs and their tolerability in mitochondrial disorders
    Josef Finsterer
    Danube University Krems, Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Krems Austria, Europe
    Expert Opin Drug Metab Toxicol 8:71-9. 2012
    ..The problem with this approach is that some antiepileptic drugs (AEDs) are mitochondrial toxic and care is, therefore, needed when administering these AEDs to patients with MIDs...
  68. ncbi Neuromuscular disorders associated with apical hypertrophic cardiomyopathy
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Cardiol 64:85-9. 2009
    ..Cardiomyopathy is a frequent manifestation of neuromuscular disorders...
  69. ncbi Management of cryptogenic stroke
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Belg 110:135-47. 2010
    ..If the cause of CS can be identified, appropriate treatment is indicated. A PFO requires antiplatelet medication, OAC if there are other indications for OAC, and closure in case of recurrent CS under OAC...
  70. doi [Neurological complications of atrioesophageal fistulas: postprandial insults, epilepsy and meningitis]
    J Finsterer
    Krankenanstalt Rudolfstiftung, Wien, Osterreich
    Nervenarzt 82:198-201. 2011
    ..AEFs result in an overflow of esophageal contents into the left atrium with subsequent systemic embolization...
  71. doi Causes of camptocormia
    Josef Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Disabil Rehabil 33:1702-3. 2011
    ..To identify and highlight the variable causes of camptocormia...
  72. doi [Diagnosis of myopathies]
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Fortschr Neurol Psychiatr 78:382-93. 2010
    ..For diagnostic, therapeutic and prognostic implications, diagnostic work-up should be carried out as soon as possible if myopathy is suspected...
  73. ncbi Mitochondrial disorder mimicking ocular myasthenia
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe
    Acta Neurol Belg 110:110-2. 2010
    ..Ocular myasthenia (OM) and mitochondrial disorder (MID) may be easily mixed up, if the MID presents with similar manifestations as OM and if MID manifestations progress only slowly...
  74. doi Left ventricular non-compaction and its cardiac and neurologic implications
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Danube University Krems, Krems, Postfach 20, 1180, Vienna, Austria
    Heart Fail Rev 15:589-603. 2010
    ..Though initially assessed as poor, the prognosis of LVHT has meanwhile improved, most likely due to the increased awareness for the abnormality and the timely administration of adequate therapy...
  75. doi Cardiac and cerebral air embolism from endoscopic retrograde cholangio-pancreatography
    Josef Finsterer
    Second Medical Department, Institute of Radiology, Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe
    Eur J Gastroenterol Hepatol 22:1157-62. 2010
    ....
  76. ncbi Neuromuscular disorders in left ventricular hypertrabeculation/noncompaction
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Curr Pharm Des 16:2895-904. 2010
    ....
  77. doi Presentation, etiology, diagnosis, and management of camptocormia
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur Neurol 64:1-8. 2010
    ..The outcome is generally fair. Some patients profit from therapy whereas others do not respond to treatment and become progressively immobile...
  78. doi Neurotoxocariasis associated with lower motor neuron disease. Report of one case
    Josef Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Rev Med Chil 138:483-6. 2010
    ..Whether lower motor neuron disease was causally related to neurotoxocariasis or due to a general metabolic defect, remains speculative...
  79. doi Cardiac findings in congenital muscular dystrophies
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Danube University, Krems, Vienna, Austria
    Pediatrics 126:538-45. 2010
    ..Patients with CMD with CI require regular cardiologic surveillance so that severe, treatable cardiac disease is not overlooked...
  80. doi Perspectives of Kennedy's disease
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    J Neurol Sci 298:1-10. 2010
    ..The course is slowly progressive and the ability to walk lost only late in life. Only few patients require ventilatory support and life expectancy is only slightly compromised...
  81. doi Regression of stroke-like lesions in MELAS-syndrome after seizure control
    Josef Finsterer
    Krankenanstalt Rudolfstiftung, Institute of Radiology Urania, Vienna, Austria
    Epileptic Disord 12:330-4. 2010
    ..This case indicates that adequate seizure control in a patient with MELAS syndrome may prevent the recurrence of stroke-like episodes and may result in the disappearance of stroke-like lesions on MRI...
  82. pmc Myotoxicity of telbivudine in pre-existing muscle damage
    Josef Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Virol J 7:323. 2010
    ..It is unknown if telbivudine causes muscle damage only in patients with pre-existing muscle pathology...
  83. ncbi Nail-patella syndrome associated with respiratory chain disorder
    J Finsterer
    Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, Krankenanstalt Rudolfstiftung, A 1180 Vienna, Austria
    Eur Neurol 46:92-5. 2001
    ..In conclusion, this case demonstrates that NPS may be randomly associated with RCD. NPS patients should undergo detailed cardiological and neurological investigations, in order not to overlook a double trouble partially mimicking NPS...
  84. ncbi Isolated left ventricular abnormal trabeculation is a cardiac manifestation of neuromuscular disorders
    C Stollberger
    Krankenanstalt Rudolfstiftung, Wien, Osterreich
    Cardiology 94:72-6. 2000
    ..The aims of the study were to assess by echocardiography the prevalence of ILVAT, look for cardiac findings in ILVAT and find out if ILVAT is associated with neuromuscular disorders...
  85. ncbi Progression of cardiac involvement in patients with myotonic dystrophy, Becker's muscular dystrophy and mitochondrial myopathy during a 2-year follow-up
    C Stollberger
    Zweite Medizinische Abteilung, Krankenanstalt Rudolfstiftung, Wien, Osterreich
    Cardiology 90:173-9. 1998
    ..CI and neurologic impairment were not related (p = 0.54). Cardiac investigations should be repeated at yearly intervals irrespective of whether there is neurologic deterioration or not...
  86. ncbi Isolated left ventricular abnormal trabeculation: follow-up and association with neuromuscular disorders
    C Stollberger
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Can J Cardiol 17:163-8. 2001
    ....
  87. ncbi Parkinson syndrome as a manifestation of mitochondriopathy
    J Finsterer
    Ludwig Boltzmann Institute for Epilepsy and Neuromuscular Disorders, Postfach 348, 1180 Vienna, Austria
    Acta Neurol Scand 105:384-9. 2002
    ..Although there is growing evidence for a relation between Parkinson syndrome (PS) and mitochondriopathy (MCP), little is known about the frequency of PS in MCP...
  88. doi Treatment of central nervous system manifestations in mitochondrial disorders
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Danube University Krems, Austria, Europe
    Eur J Neurol 18:28-38. 2011
    ..Effective treatment acting on the pathogenic cascade may increase the quality of life and outcome in patients with MID and may prevent a therapeutic nihilism occasionally upcoming with MIDs...
  89. ncbi Complex mitochondriopathy associated with 4 mtDNA transitions
    J Finsterer
    Ludwig Boltzmann Institute for Epilepsy and Neuromuscular Disorders, Vienna, Austria
    Eur Neurol 44:37-41. 2000
    ..Three of the patient's transitions were also detected in blood leukocytes of the patient's maternal grandmother, mother and brother. Mutant mtDNA was heteroplasmic at >75% in the patient's skeletal muscle...
  90. ncbi Systemic and non-systemic vasculitis affecting the peripheral nerves
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe
    Acta Neurol Belg 109:100-13. 2009
    ..In single cases immunoglobulins, immunoadsorbtion, or plasma exchange have been successfully applied. In case of virus-associated vasculitis interferon-alpha plus lamivudine or ribaverin may be beneficial...
  91. ncbi Respiratory chain complex-I defect mimicking myasthenia
    J Finsterer
    Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, Vienna, Austria
    Metab Brain Dis 17:41-6. 2002
    ..It is concluded that respiratory chain disorders due to a complex-I defect may mimic ocular myasthenia clinically, electrophysiologically, and even therapeutically...
  92. ncbi Cardiac and noncardiac, particularly neuromuscular, disease with troponin-T positivity
    J Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neth J Med 65:289-95. 2007
    ..The present study aimed to retrospectively evaluate the frequency of primary cardiac and secondary cardiac causes of troponin-T positivity, in particular how often troponin-T positivity is associated with neuromuscular disorders...
  93. ncbi Concentric-needle versus macro EMG. II. Detection of neuromuscular disorders
    J Finsterer
    Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, Vienna, Austria
    Clin Neurophysiol 112:853-60. 2001
    ..Little is known about the relation and sensitivity of macro-EMG (MA-EMG) compared with concentric-needle EMG (CN-EMG) in the detection of neuromuscular disorders...
  94. ncbi Skeletal muscle involvement in congenital long QT syndrome
    J Finsterer
    Schindlergasse 9 10, 1180 Vienna, Austria
    Neurol Sci 25:238-40. 2004
    ..LQTS, due to a KVLQT1 mutation, may manifest not only in the heart, but subclinically also in the skeletal muscle...
  95. ncbi Phenotype variability in 130 adult patients with respiratory chain disorders
    J Finsterer
    Neurological Department, , Ludwig Boltzmann Institute for Epilepsy and Neuronmuscular Disorders, Vienna, Austria
    J Inherit Metab Dis 24:560-76. 2001
    ..Systems most often involved in RCDs were the PNS, CNS, endocrine system and heart. The diagnosis of RCD requires awareness of the great phenotypic heterogeneity and an individualized, integral, multidisciplinary approach...
  96. ncbi Central nervous system manifestations of mitochondrial disorders
    J Finsterer
    Krankenanstalt Rudolfstiftung, Vienna, Austria
    Acta Neurol Scand 114:217-38. 2006
    ..Valproate, carbamazepine, corticosteroids, acetyl salicylic acid, local and volatile anesthetics should be applied with caution. Avoiding certain drugs is often more beneficial than application of established, apparently indicated drugs...
  97. ncbi Diagnostic yield of the lactate stress test in respiratory chain disorders under absolute and relative workload
    J Finsterer
    Ludwig Boltzmann Institute for Research in Neuromuscular Disorders, Postfach 348, 1180 Vienna, Austria
    J Neurosci Methods 108:65-70. 2001
    ..In conclusion, the LSTA should be preferred to the LSTR. Three lactate determinations during 15 min have no advantage over a single lactate determination after a 5 min workload of 30 W...
  98. ncbi Mitochondriopathies
    J Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Eur J Neurol 11:163-86. 2004
    ..The rapidly increasing understanding of the pathophysiological background of MCPs may further facilitate the diagnostic approach and open perspectives to future, possibly causative therapies...
  99. ncbi Overview on visceral manifestations of mitochondrial disorders
    J Finsterer
    Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Neth J Med 64:61-71. 2006
    ..Although there is no causal therapy for MCPs, adequate symptomatic therapy, particularly of visceral manifestations, markedly improves quality of life and prognosis of these still often neglected or overlooked disorders...
  100. pmc Dropped head syndrome in mitochondriopathy
    J Finsterer
    Neurological Hospital Rosenhugel, Postfach 348, 1180, Vienna, Austria
    Eur Spine J 13:652-6. 2004
    ..Anti-Parkinson medication is of limited effect...
  101. ncbi Atypical paraneoplastic syndrome associated with anti-Yo antibodies
    J Finsterer
    Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria
    Clin Neuropathol 22:137-40. 2003
    ..Polyneuropathy, myopathy and spasticity have not been described as a manifestation of a neurologic paraneoplastic syndrome (NPS) associated with anti-Yo antibodies (anti-Yo)...