Amyotrophy

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Nens Van Alfen - One of the best experts on this subject based on the ideXlab platform.

  • phrenic neuropathy and diaphragm dysfunction in neuralgic Amyotrophy
    Neurology, 2018
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Jonne Doorduin, Marieke H Van Rosmalen, Yvonne F Heijdra, Andrea J Boon, Michael A Gaytant, Ries J M Van Den Biggelaar, Roy T M Sprooten, Peter J Wijkstra
    Abstract:

    OBJECTIVE: To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic Amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. METHODS: This observational cohort study recruited adult patients with neuralgic Amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic Amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. RESULTS: Phrenic neuropathy occurs in 7.6% of patients with neuralgic Amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. CONCLUSION: We recommend screening every patient with neuralgic Amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    textabstractObjective Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. Methods In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. Results Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. Conclusions Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    OBJECTIVE: Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. RESULTS: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. CONCLUSIONS: Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

  • clinical and pathophysiological concepts of neuralgic Amyotrophy
    Nature Reviews Neurology, 2011
    Co-Authors: Nens Van Alfen
    Abstract:

    Neuralgic Amyotrophy is a distinct, painful focal neuropathy with a broad spectrum of clinical phenotypes and a complex pathophysiology. The extent of disability experienced varies from patient to patient, but many individuals are left with residual disabilities affecting their everyday lives. In this Review, Nens van Alfen provides an overview of the current clinical and pathophysiological concepts and research topics in neuralgic Amyotrophy.

  • sensory nerve conduction studies in neuralgic Amyotrophy
    American Journal of Physical Medicine & Rehabilitation, 2009
    Co-Authors: Nens Van Alfen, Willem J Huisman, Sebastiaan Overeem, B G M Van Engelen, M J Zwarts
    Abstract:

    Neuralgic Amyotrophy is a painful, episodic peripheral nerve disorder localized to the brachial plexus. Sensory symptoms occur in 80% of the patients. We assessed the frequency of abnormalities in sensory nerve conduction studies of the lateral and medial antebrachial cutaneous, radial sensory, median sensory, and ulnar sensory nerves in 112 patients. Sensory nerve conduction studies showed abnormalities in <20% of nerves, even when the nerve was clinically affected. The lateral and medial antebrachial cutaneous nerves were most often abnormal, in 15% and 17% of nerves. No correlation with the presence or localization of clinical deficits was found. Brachial plexus sensory nerve conduction studies seem to be of little diagnostic value in neuralgic Amyotrophy. Our findings also indicate that some sensory lesions may be in the nerve roots instead of the plexus. An examination of normal sensory nerve conduction studies does not preclude neuralgic Amyotrophy as a diagnosis.

Floris A Van De Laar - One of the best experts on this subject based on the ideXlab platform.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    textabstractObjective Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. Methods In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. Results Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. Conclusions Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    OBJECTIVE: Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. RESULTS: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. CONCLUSIONS: Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

Jeroen J J Van Eijk - One of the best experts on this subject based on the ideXlab platform.

  • phrenic neuropathy and diaphragm dysfunction in neuralgic Amyotrophy
    Neurology, 2018
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Jonne Doorduin, Marieke H Van Rosmalen, Yvonne F Heijdra, Andrea J Boon, Michael A Gaytant, Ries J M Van Den Biggelaar, Roy T M Sprooten, Peter J Wijkstra
    Abstract:

    OBJECTIVE: To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic Amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. METHODS: This observational cohort study recruited adult patients with neuralgic Amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic Amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. RESULTS: Phrenic neuropathy occurs in 7.6% of patients with neuralgic Amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. CONCLUSION: We recommend screening every patient with neuralgic Amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    textabstractObjective Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. Methods In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. Results Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. Conclusions Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    OBJECTIVE: Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. RESULTS: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. CONCLUSIONS: Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

Shinya Kawai - One of the best experts on this subject based on the ideXlab platform.

  • Distal-Type Cervical Spondylotic Amyotrophy
    Spine, 2004
    Co-Authors: Kazuo Kaneko, Toshihiko Taguchi, Kouichiro Toyoda, Yoshihiko Kato, Y. Azuma, Shinya Kawai
    Abstract:

    Study Design.Six cases with distal-type cervical spondylotic Amyotrophy are reported.Objective.To investigate the pathophysiology of distal-type cervical spondylotic Amyotrophy from magnetic resonance imaging and intraoperative evoked spinal cord responses.Summary of Background Data.Cervical spondyl

  • distal type cervical spondylotic Amyotrophy assessment of pathophysiology from radiological findings on magnetic resonance imaging and epidurally recorded spinal cord responses
    Spine, 2004
    Co-Authors: Kazuo Kaneko, Toshihiko Taguchi, Kouichiro Toyoda, Yoshihiko Kato, Y. Azuma, Shinya Kawai
    Abstract:

    STUDY DESIGN: Six cases with distal-type cervical spondylotic Amyotrophy are reported. OBJECTIVE: To investigate the pathophysiology of distal-type cervical spondylotic Amyotrophy from magnetic resonance imaging and intraoperative evoked spinal cord responses. SUMMARY OF BACKGROUND DATA: Cervical spondylotic Amyotrophy had a characteristic clinical symptom of severe muscular atrophy with no or insignificant sensory deficit. Selective ventral root lesions or intrinsic spinal cord lesions have been proposed as the pathophysiology of cervical spondylotic Amyotrophy, but they have not been well understood. METHOD: Six patients with distal-type cervical spondylotic Amyotrophy were described, and their magnetic resonance imaging and evoked spinal cord potentials after median nerve, motor cortex, and spinal cord stimulation were investigated. RESULTS: Sagittal T2-weighted magnetic resonance imaging showed high signal intensity change within the spinal cord at C4-C5, C5-C6, and C6-C7. All patients underwent laminoplasty. The attenuation of postsynaptic potentials with preserved presynaptic potentials at C4-C5, C5-C6, and C6-C7 was characteristic in the evoked spinal cord potentials after median nerve stimulation. The amplitude of the evoked spinal cord potentials after median nerve stimulation was preserved at C2-C3. This means that lateral posterior column in the spinal cord had less or no involvement in distal-type cervical spondylotic Amyotrophy. Concomitant hyperactivity of the patellar tendon reflex was correlated with the abnormality in the evoked spinal cord potentials after transcranial electric stimulation. CONCLUSIONS: The results suggest a longitudinal gray matter lesion as one pathophysiologic feature, and that less damage to the lateral posterior column is the reason for the preservation of sensory function in the patients with distal-type cervical spondylotic Amyotrophy described in this study.

Sigrid Pillen - One of the best experts on this subject based on the ideXlab platform.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    textabstractObjective Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. Methods In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. Results Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. Conclusions Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

  • incidence of neuralgic Amyotrophy parsonage turner syndrome in a primary care setting a prospective cohort study
    PLOS ONE, 2015
    Co-Authors: Nens Van Alfen, Jeroen J J Van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A Van De Laar
    Abstract:

    OBJECTIVE: Neuralgic Amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic Amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic Amyotrophy. Neuralgic Amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic Amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. RESULTS: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic Amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic Amyotrophy of 1 per 1000. CONCLUSIONS: Our findings suggest that neuralgic Amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.