Lineu Werneck | Universidade Federal do Paraná (original) (raw)

Papers by Lineu Werneck

Research paper thumbnail of Genetic screening for transthyretin familial amyloid polyneuropathy to avoid misdiagnosis in patients with polyneuropathy associated with high protein in the cerebrospinal fluid

Acta Neurologica Belgica, Feb 25, 2023

Research paper thumbnail of HLA Genotype in Patients with Neuromyelitis Optica (P05.120)

Research paper thumbnail of Progression of Idiopathic Late Onset Cerebellar Ataxia (S12.006)

Neurology, Apr 22, 2012

Objective: To analyze the follow-up of idiopathic late onset cerebellar ataxia (ILOCA) patients. ... more Objective: To analyze the follow-up of idiopathic late onset cerebellar ataxia (ILOCA) patients. Background ILOCA patients represent a group of neurodegenerative diseases of unknown cause affecting the cerebellum and its afferent and efferent connections. Design/Methods: Clinical and laboratory observations of the course of 50 adult patients initially evaluated in an cerebellar ataxia outpatient clinic over 20 years, with a diagnosis of ILOCA, and followed up regularly. The presence of REM sleep behavior disorder (RBD) was evaluated as probable RBD, based on clinical validated criteria, or definite RBD, based on polysomnographic confirmation. Results: From a total of 50 patients with ILOCA (excluding all patients with acquired or genetically defined cerebellar ataxia), 24 subsequently developed criteria for MSA type-C. 26 patients remained with the diagnosis of ILOCA, among them 8 with a probable diagnosis of late cerebellar atrophy of Marie-Foix-Alajouanine syndrome (MFAS). The group of patients with late cerebellar atrophy of MFAS had an older mean age (72.8 years) than MSA-C patients (59.5 years). Neurological examination, including a thorough eye movement assessment did not differentiate patients with MSA-C and ILOCA. Clinically or definite RBD was diagnosed in 46 % of patients. However, patients who developed MSA type C had a highly percentage of probable and definite previously diagnosis of RBD (83.4%). On the other hand, among patients with ILOCA, the percentage of RBD was significantly lower (11.5%, p Conclusions: In this Brazilian series of ILOCA patients, MSA-C was clinically diagnosed in 48 % of cases and 52 % remained with the diagnosis of ILOCA, including a 12 % with the diagnosis of late cerebellar atrophy of MFAS. RBD diagnosis, either clinical or polysomnographic should included as an important tool in the differentiation of MSA type C and ILOCA. Disclosure: Dr. Teive has nothing to disclose. Dr. Munhoz has nothing to disclose. Dr. Arruda has nothing to disclose. Dr. Werneck has nothing to disclose.

Research paper thumbnail of Hereditary neuropathy with liability to pressure palsies: a single-center experience in southern Brazil

Neurology International, Sep 30, 2016

Research paper thumbnail of Albuterol Treatment in Congenital Myastenic Syndrome with Dok-7 Mutation: An Open Prospective Study (IN1-1.002)

OBJECTIVE: This was an open prospective study to determined clinical response to albuterol in Dok... more OBJECTIVE: This was an open prospective study to determined clinical response to albuterol in Dok-7 CMS. BACKGROUND: Congenital myasthenic syndromes (CMS) are heterogeneous genetic diseases which can have different management in specific subgroups. In CMS related to Dok-7 deficiency (Dok-7 CMS), treatment can be accomplished with cholinesterase inhibitors, or according to the response, associate at 3-4 DAP. The ephedrine can be used in the treatment of Dok-7 CMS, but it cannot be prescribed in USA. Albuterol, or salbutamol, is a selective B2-adrenergic agonist, which was previous described associated with improvement of Dok-7 CMS. However, more prospective studies are necessary to determinate the most effective dosing regimen and long-term response. DESIGN/METHODS: Albuterol was prescribed at dose of 2 mg three times daily (6mg/day) for all patients. The response was assessment by QMG score at baseline, 3, 6, 9 and 12 months; ADL-MG score and 6 minutes walking test at baseline and after 12 months in follow-up clinic visits. Tolerance to albuterol was evaluated. RESULTS: The sample population consisted of 5 patients (2 male and 3 female), with a mean age of 27 + 11.06 years, who presented CMS with c.1124_1127dupTGCC and/or p.S45L mutations in DOK7 gene. We noted a progressive response to treatment in QMG score since 3 months of albuterol treatment. In ADL-MG scores and 6 minutes walking test, comparison between baseline and after 12 months reveals an important beneficial response to albuterol treatment. The side effects of albuterol were well tolerated in all patients. CONCLUSIONS: Albuterol was an effective treatment to Dok-7 CMS. This study provides class IV evidence that albuterol given at dose 6 mg/day improves measured by the QMG score, ADL-MG score and 6 minutes walking test. The dose of albuterol was well-tolerated and lesser than previous described to treat patients with Dok-7 CMS. Disclosure: Dr. Lorenzoni has nothing to disclose. Dr. Scola has received personal compensation for activities with Merck Serono as scientific advisor board. Dr. Kay has nothing to disclose. Dr. Paranhos has nothing to disclose. Dr. Pinheiro has nothing to disclose. Dr. Filla has nothing to disclose. Dr. Lochmuller has nothing to disclose. Dr. Werneck has nothing to disclose.

Research paper thumbnail of The muscle lesion in myasthenia gravis: a study of 17 cases with muscle histochemistry

Arquivos De Neuro-psiquiatria, Mar 1, 1982

Research paper thumbnail of Myasthenia gravis and pregnancy: A brief review of 37 pregnancies

Neuromuscular Disorders, Oct 1, 2015

Research paper thumbnail of Enzimas séricas e eletromiografia em doenças Neuromusculares: estudo comparativo de 817 casos

Arquivos De Neuro-psiquiatria, Jun 1, 1995

Research paper thumbnail of Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report

Arquivos De Neuro-psiquiatria, Sep 1, 2007

Research paper thumbnail of Thymus and Myasthenia Gravis: Long-Term Follow-Up Screening of Thymectomized and Non-Thymectomized Patients (P02.189)

Neurology, Feb 12, 2013

OBJECTIVE: We analyzed the recurrence rate and incidence of new thymoma in a series of patients w... more OBJECTIVE: We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. BACKGROUND: Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. DESIGN/METHODS: We study MG patients with follow-up for more than five years, well-controlled disease, a first contrast-enhanced chest computerized tomography (CT) at the beginning of the disease and a second contrast-enhanced chest CT after five years. MG was considered well-controlled according to the Myasthenia Gravis Foundation of America (MGFA) classification and myasthenia gravis composite (MGC). RESULTS: The sample consisted of 53 patients (44 female and 9 male), aged 17 to 72 years (median 41.8 years). The follow-up in our hospital varied between 75 and 472 months, with a mean time of 182.2±96.99 months. The MGFA classification and MGC score demonstrated that our patients9 conditions were well controlled at the time the second chest CT was performed. The chest CT detected thymus abnormalities in 8 patients at the initial screening and no abnormalities in all patients at a second screening after 5 years. Twenty-one patients were thymectomized. The suspicion of thymoma based on the initial chest CT screening of 8 patients was confirmed by histopathological study in all cases. CONCLUSIONS: These findings indicate that routine screening for thymoma at fixed follow-up intervals is not needed for patients with well-controlled MG. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease. Disclosure: Dr. Lorenzoni has nothing to disclose. Dr. Augusto has nothing to disclose. Dr. Kay has nothing to disclose. Dr. Scola has received personal compensation for activities with Merck Serono as scientific advisor board. Dr. Werneck has nothing to disclose.

Research paper thumbnail of Worsening of motor symptoms and gynecomastia during spironolactone treatment in a patient with Parkinson's disease and congestive heart failure

Research paper thumbnail of Toxic myopathies: muscle biopsy features

Arquivos De Neuro-psiquiatria, Mar 1, 2007

Research paper thumbnail of Muscle involvement in leprosy: study of the anterior tibial muscle in 40 patients

Arquivos De Neuro-psiquiatria, Sep 1, 1999

Research paper thumbnail of P1.065 Cervical dystonia: clinical and therapeutic features in 85 patients

Parkinsonism & Related Disorders, Dec 1, 2009

Research paper thumbnail of Diagnosis of limb-girdle muscular dystrophies in the molecular biology era: do clinical findings still matter?

Arquivos De Neuro-psiquiatria, Dec 1, 2015

Research paper thumbnail of Acquired cerebellar ataxia due to statin use

Arquivos De Neuro-psiquiatria, Feb 1, 2012

Research paper thumbnail of Malformação de Chiari tipo I: relato de dois casos com apresentações clínicas pouco usuais

Arquivos De Neuro-psiquiatria, Sep 1, 1999

Research paper thumbnail of Topiramate-induced psychosis: report of two cases

Expert Opinion on Drug Safety, Oct 18, 2006

1. Expert Opin Drug Saf. 2006 Nov;5(6):741-2. Topiramate-induced psychosis: report of two cases. ... more 1. Expert Opin Drug Saf. 2006 Nov;5(6):741-2. Topiramate-induced psychosis: report of two cases. Teive HA, Arruda WO, Munhoz RP, Werneck LC. PMID: 17044799 [PubMed - indexed for MEDLINE]. Publication Types: Case Reports; Letter. MeSH Terms: ...

Research paper thumbnail of Miastenia grave distal: relato de caso

Arquivos De Neuro-psiquiatria, Mar 1, 2003

Research paper thumbnail of P433: Clinical electromyography correlation in patients admitted to a university hospital

Clinical Neurophysiology, 2014

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S165 P433 C... more s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S165 P433 Clinical electromyography correlation in patients admitted to a university hospital C. Kay, R. Scola, P. Lorenzoni, L. Werneck Universidade Federal do Paraná, Curitiba, Brazil Introduction: Electromyography (EMG) is indicated to neurophysiological assessment of the peripheral nervous system. The interpretation of the results depend on the clinical correlation, but its contribution to the diagnosis and management of inpatients is not often reported in the literature. The aim of this study was correlate the clinical hypothesis with the results found in EMG, of inpatients at the Hospital de Clinicas, Universidade Federal do Paraná, within one year, to evaluate the contribution of the examination in the diagnosis of patients. Methods: A retrospective review of all EMG examination and medical records of patients admitted to HC-UFPR during one year period. In the medical records was obtained demographic data, specialty who request the exam, indication for the EMG, topography, result of exam and number of exams done in the same patient in the same year. Clinical hypothesis were grouped in topography. The results of EMG were correlated with clinical hypothesis as: confirmatory correlation, new diagnosis, incidental diagnosis, inconclusive diagnosis and normal. Results: We performed 406 EMG in one year period (210 exams in 204 inpatients). There were 123 males and 81 females, with mean age 38 year (range 4 months–91 years old). Five patients were submitted to additional EMG, one of them twice. Discussion: Confirmatory correlation found was similar to described in literature. However this study had a greater number of normal EMG. It occurred because in myopathy suspicion, the EMG sampling could be normal. In patients with peripheral neuropathy and normal EMG, the main symptom was pain. Since pain is conducted by small fiber caliber, the routine nerve conduction studies were unable to detect abnormalities of these fibers. In patients with neuromuscular junction dysfunction, repetitive stimulation test was normal in 47% of cases. P434 Ultrasonography support during the electrical evaluation of the peripheral respiratory muscle dysfunction J.M. Corredera, M. Salinas, E. Montes, S. Garcia Urquiza, C. Quijada Hospital Fundación Jimenez Diaz, Neurofisiologia Clínica, Madrid, Spain Question: Weakness of respiratory muscles has been found to be a frecuent symptom in the medical history of patients in Intensive Care, wich precede to flaccid paraylisis in some severe cases. Phrenic nerve stimulation, electrical (ES) or from cervical magnetic stimulation (CME), is an uncommon technique, often consider difficult to perform. We try to improve technique’s reliabilty by adding ultrasonography assessment during the evaluation of the peripheral respiratory muscle dysfunction at our institution. Methods: We performed 10 hemidiaphragms studies on 5 in-patients with clinical respiratory muscle dysfunction. Each phrenic nerve was estimulated transcutaneously in the neck at the posterior border of the sternocleidomastoid muscles, trying to minimize co-stimulation of the brachial plexus, wich could cause activation of the upper limb musculature. Square-wave impulses of 0.1-ms duration were delivered, using a conventional bipolar electrode. Diaphragmatic muscle action potentials were recorded with surface electrodes placed over the seventh intercostal space (active), at the costochondral juntion, and the xiphoid process (reference). Simultaneously ultranosography (Mmode traces) of the ipsilateral hemidiaphragm was performed. Results: Of the 10 studies, we obtained 5 normal hemidiaphragm potentials followed by normal motion (represented as a spikelike deflection) at the ultrasonography. In 3 cases, responses were markedly reduced in amplitude with increased latencies and decreased motion. One diaphragmatic response showed mild amplitude reduction but normal motion. We obtained no electrical response, with no motion in one study. Conclusion: Electrical stimulation can be difficult to accomplish as a consecuence of morphology of the subjet, anatomical variations of the nerve, and lack of uniformity establishing an standard protocol of recording and stimulation. Furthermore stimulation of many of the muscles of the upper thoracic cage, as well as other external artifacts, could be responsable for contaminating the electrical signal. We find ultranosonography support during electrical stimulation a realiable combination of techniques, that can helps to avoid false positive and negative results. P436 Assessing motor units with improved MUNIX A. Khomenko, I. Kobor, F. Stein, D. Baldaranov, S. Johannesen, T. Bruun, U. Bogdahn, W. Schulte-Mattler Department of Neurology, University Hospital Regensburg, Regensburg,

Research paper thumbnail of Genetic screening for transthyretin familial amyloid polyneuropathy to avoid misdiagnosis in patients with polyneuropathy associated with high protein in the cerebrospinal fluid

Acta Neurologica Belgica, Feb 25, 2023

Research paper thumbnail of HLA Genotype in Patients with Neuromyelitis Optica (P05.120)

Research paper thumbnail of Progression of Idiopathic Late Onset Cerebellar Ataxia (S12.006)

Neurology, Apr 22, 2012

Objective: To analyze the follow-up of idiopathic late onset cerebellar ataxia (ILOCA) patients. ... more Objective: To analyze the follow-up of idiopathic late onset cerebellar ataxia (ILOCA) patients. Background ILOCA patients represent a group of neurodegenerative diseases of unknown cause affecting the cerebellum and its afferent and efferent connections. Design/Methods: Clinical and laboratory observations of the course of 50 adult patients initially evaluated in an cerebellar ataxia outpatient clinic over 20 years, with a diagnosis of ILOCA, and followed up regularly. The presence of REM sleep behavior disorder (RBD) was evaluated as probable RBD, based on clinical validated criteria, or definite RBD, based on polysomnographic confirmation. Results: From a total of 50 patients with ILOCA (excluding all patients with acquired or genetically defined cerebellar ataxia), 24 subsequently developed criteria for MSA type-C. 26 patients remained with the diagnosis of ILOCA, among them 8 with a probable diagnosis of late cerebellar atrophy of Marie-Foix-Alajouanine syndrome (MFAS). The group of patients with late cerebellar atrophy of MFAS had an older mean age (72.8 years) than MSA-C patients (59.5 years). Neurological examination, including a thorough eye movement assessment did not differentiate patients with MSA-C and ILOCA. Clinically or definite RBD was diagnosed in 46 % of patients. However, patients who developed MSA type C had a highly percentage of probable and definite previously diagnosis of RBD (83.4%). On the other hand, among patients with ILOCA, the percentage of RBD was significantly lower (11.5%, p Conclusions: In this Brazilian series of ILOCA patients, MSA-C was clinically diagnosed in 48 % of cases and 52 % remained with the diagnosis of ILOCA, including a 12 % with the diagnosis of late cerebellar atrophy of MFAS. RBD diagnosis, either clinical or polysomnographic should included as an important tool in the differentiation of MSA type C and ILOCA. Disclosure: Dr. Teive has nothing to disclose. Dr. Munhoz has nothing to disclose. Dr. Arruda has nothing to disclose. Dr. Werneck has nothing to disclose.

Research paper thumbnail of Hereditary neuropathy with liability to pressure palsies: a single-center experience in southern Brazil

Neurology International, Sep 30, 2016

Research paper thumbnail of Albuterol Treatment in Congenital Myastenic Syndrome with Dok-7 Mutation: An Open Prospective Study (IN1-1.002)

OBJECTIVE: This was an open prospective study to determined clinical response to albuterol in Dok... more OBJECTIVE: This was an open prospective study to determined clinical response to albuterol in Dok-7 CMS. BACKGROUND: Congenital myasthenic syndromes (CMS) are heterogeneous genetic diseases which can have different management in specific subgroups. In CMS related to Dok-7 deficiency (Dok-7 CMS), treatment can be accomplished with cholinesterase inhibitors, or according to the response, associate at 3-4 DAP. The ephedrine can be used in the treatment of Dok-7 CMS, but it cannot be prescribed in USA. Albuterol, or salbutamol, is a selective B2-adrenergic agonist, which was previous described associated with improvement of Dok-7 CMS. However, more prospective studies are necessary to determinate the most effective dosing regimen and long-term response. DESIGN/METHODS: Albuterol was prescribed at dose of 2 mg three times daily (6mg/day) for all patients. The response was assessment by QMG score at baseline, 3, 6, 9 and 12 months; ADL-MG score and 6 minutes walking test at baseline and after 12 months in follow-up clinic visits. Tolerance to albuterol was evaluated. RESULTS: The sample population consisted of 5 patients (2 male and 3 female), with a mean age of 27 + 11.06 years, who presented CMS with c.1124_1127dupTGCC and/or p.S45L mutations in DOK7 gene. We noted a progressive response to treatment in QMG score since 3 months of albuterol treatment. In ADL-MG scores and 6 minutes walking test, comparison between baseline and after 12 months reveals an important beneficial response to albuterol treatment. The side effects of albuterol were well tolerated in all patients. CONCLUSIONS: Albuterol was an effective treatment to Dok-7 CMS. This study provides class IV evidence that albuterol given at dose 6 mg/day improves measured by the QMG score, ADL-MG score and 6 minutes walking test. The dose of albuterol was well-tolerated and lesser than previous described to treat patients with Dok-7 CMS. Disclosure: Dr. Lorenzoni has nothing to disclose. Dr. Scola has received personal compensation for activities with Merck Serono as scientific advisor board. Dr. Kay has nothing to disclose. Dr. Paranhos has nothing to disclose. Dr. Pinheiro has nothing to disclose. Dr. Filla has nothing to disclose. Dr. Lochmuller has nothing to disclose. Dr. Werneck has nothing to disclose.

Research paper thumbnail of The muscle lesion in myasthenia gravis: a study of 17 cases with muscle histochemistry

Arquivos De Neuro-psiquiatria, Mar 1, 1982

Research paper thumbnail of Myasthenia gravis and pregnancy: A brief review of 37 pregnancies

Neuromuscular Disorders, Oct 1, 2015

Research paper thumbnail of Enzimas séricas e eletromiografia em doenças Neuromusculares: estudo comparativo de 817 casos

Arquivos De Neuro-psiquiatria, Jun 1, 1995

Research paper thumbnail of Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report

Arquivos De Neuro-psiquiatria, Sep 1, 2007

Research paper thumbnail of Thymus and Myasthenia Gravis: Long-Term Follow-Up Screening of Thymectomized and Non-Thymectomized Patients (P02.189)

Neurology, Feb 12, 2013

OBJECTIVE: We analyzed the recurrence rate and incidence of new thymoma in a series of patients w... more OBJECTIVE: We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. BACKGROUND: Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. DESIGN/METHODS: We study MG patients with follow-up for more than five years, well-controlled disease, a first contrast-enhanced chest computerized tomography (CT) at the beginning of the disease and a second contrast-enhanced chest CT after five years. MG was considered well-controlled according to the Myasthenia Gravis Foundation of America (MGFA) classification and myasthenia gravis composite (MGC). RESULTS: The sample consisted of 53 patients (44 female and 9 male), aged 17 to 72 years (median 41.8 years). The follow-up in our hospital varied between 75 and 472 months, with a mean time of 182.2±96.99 months. The MGFA classification and MGC score demonstrated that our patients9 conditions were well controlled at the time the second chest CT was performed. The chest CT detected thymus abnormalities in 8 patients at the initial screening and no abnormalities in all patients at a second screening after 5 years. Twenty-one patients were thymectomized. The suspicion of thymoma based on the initial chest CT screening of 8 patients was confirmed by histopathological study in all cases. CONCLUSIONS: These findings indicate that routine screening for thymoma at fixed follow-up intervals is not needed for patients with well-controlled MG. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease. Disclosure: Dr. Lorenzoni has nothing to disclose. Dr. Augusto has nothing to disclose. Dr. Kay has nothing to disclose. Dr. Scola has received personal compensation for activities with Merck Serono as scientific advisor board. Dr. Werneck has nothing to disclose.

Research paper thumbnail of Worsening of motor symptoms and gynecomastia during spironolactone treatment in a patient with Parkinson's disease and congestive heart failure

Research paper thumbnail of Toxic myopathies: muscle biopsy features

Arquivos De Neuro-psiquiatria, Mar 1, 2007

Research paper thumbnail of Muscle involvement in leprosy: study of the anterior tibial muscle in 40 patients

Arquivos De Neuro-psiquiatria, Sep 1, 1999

Research paper thumbnail of P1.065 Cervical dystonia: clinical and therapeutic features in 85 patients

Parkinsonism & Related Disorders, Dec 1, 2009

Research paper thumbnail of Diagnosis of limb-girdle muscular dystrophies in the molecular biology era: do clinical findings still matter?

Arquivos De Neuro-psiquiatria, Dec 1, 2015

Research paper thumbnail of Acquired cerebellar ataxia due to statin use

Arquivos De Neuro-psiquiatria, Feb 1, 2012

Research paper thumbnail of Malformação de Chiari tipo I: relato de dois casos com apresentações clínicas pouco usuais

Arquivos De Neuro-psiquiatria, Sep 1, 1999

Research paper thumbnail of Topiramate-induced psychosis: report of two cases

Expert Opinion on Drug Safety, Oct 18, 2006

1. Expert Opin Drug Saf. 2006 Nov;5(6):741-2. Topiramate-induced psychosis: report of two cases. ... more 1. Expert Opin Drug Saf. 2006 Nov;5(6):741-2. Topiramate-induced psychosis: report of two cases. Teive HA, Arruda WO, Munhoz RP, Werneck LC. PMID: 17044799 [PubMed - indexed for MEDLINE]. Publication Types: Case Reports; Letter. MeSH Terms: ...

Research paper thumbnail of Miastenia grave distal: relato de caso

Arquivos De Neuro-psiquiatria, Mar 1, 2003

Research paper thumbnail of P433: Clinical electromyography correlation in patients admitted to a university hospital

Clinical Neurophysiology, 2014

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S165 P433 C... more s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S165 P433 Clinical electromyography correlation in patients admitted to a university hospital C. Kay, R. Scola, P. Lorenzoni, L. Werneck Universidade Federal do Paraná, Curitiba, Brazil Introduction: Electromyography (EMG) is indicated to neurophysiological assessment of the peripheral nervous system. The interpretation of the results depend on the clinical correlation, but its contribution to the diagnosis and management of inpatients is not often reported in the literature. The aim of this study was correlate the clinical hypothesis with the results found in EMG, of inpatients at the Hospital de Clinicas, Universidade Federal do Paraná, within one year, to evaluate the contribution of the examination in the diagnosis of patients. Methods: A retrospective review of all EMG examination and medical records of patients admitted to HC-UFPR during one year period. In the medical records was obtained demographic data, specialty who request the exam, indication for the EMG, topography, result of exam and number of exams done in the same patient in the same year. Clinical hypothesis were grouped in topography. The results of EMG were correlated with clinical hypothesis as: confirmatory correlation, new diagnosis, incidental diagnosis, inconclusive diagnosis and normal. Results: We performed 406 EMG in one year period (210 exams in 204 inpatients). There were 123 males and 81 females, with mean age 38 year (range 4 months–91 years old). Five patients were submitted to additional EMG, one of them twice. Discussion: Confirmatory correlation found was similar to described in literature. However this study had a greater number of normal EMG. It occurred because in myopathy suspicion, the EMG sampling could be normal. In patients with peripheral neuropathy and normal EMG, the main symptom was pain. Since pain is conducted by small fiber caliber, the routine nerve conduction studies were unable to detect abnormalities of these fibers. In patients with neuromuscular junction dysfunction, repetitive stimulation test was normal in 47% of cases. P434 Ultrasonography support during the electrical evaluation of the peripheral respiratory muscle dysfunction J.M. Corredera, M. Salinas, E. Montes, S. Garcia Urquiza, C. Quijada Hospital Fundación Jimenez Diaz, Neurofisiologia Clínica, Madrid, Spain Question: Weakness of respiratory muscles has been found to be a frecuent symptom in the medical history of patients in Intensive Care, wich precede to flaccid paraylisis in some severe cases. Phrenic nerve stimulation, electrical (ES) or from cervical magnetic stimulation (CME), is an uncommon technique, often consider difficult to perform. We try to improve technique’s reliabilty by adding ultrasonography assessment during the evaluation of the peripheral respiratory muscle dysfunction at our institution. Methods: We performed 10 hemidiaphragms studies on 5 in-patients with clinical respiratory muscle dysfunction. Each phrenic nerve was estimulated transcutaneously in the neck at the posterior border of the sternocleidomastoid muscles, trying to minimize co-stimulation of the brachial plexus, wich could cause activation of the upper limb musculature. Square-wave impulses of 0.1-ms duration were delivered, using a conventional bipolar electrode. Diaphragmatic muscle action potentials were recorded with surface electrodes placed over the seventh intercostal space (active), at the costochondral juntion, and the xiphoid process (reference). Simultaneously ultranosography (Mmode traces) of the ipsilateral hemidiaphragm was performed. Results: Of the 10 studies, we obtained 5 normal hemidiaphragm potentials followed by normal motion (represented as a spikelike deflection) at the ultrasonography. In 3 cases, responses were markedly reduced in amplitude with increased latencies and decreased motion. One diaphragmatic response showed mild amplitude reduction but normal motion. We obtained no electrical response, with no motion in one study. Conclusion: Electrical stimulation can be difficult to accomplish as a consecuence of morphology of the subjet, anatomical variations of the nerve, and lack of uniformity establishing an standard protocol of recording and stimulation. Furthermore stimulation of many of the muscles of the upper thoracic cage, as well as other external artifacts, could be responsable for contaminating the electrical signal. We find ultranosonography support during electrical stimulation a realiable combination of techniques, that can helps to avoid false positive and negative results. P436 Assessing motor units with improved MUNIX A. Khomenko, I. Kobor, F. Stein, D. Baldaranov, S. Johannesen, T. Bruun, U. Bogdahn, W. Schulte-Mattler Department of Neurology, University Hospital Regensburg, Regensburg,