Clinical and electrodiagnostic findings in copper deficiency myeloneuropathy
Introduction: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. Methods: Clinical, radiographic and electrodiagnostic findings...
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Published in | Journal of neurology, neurosurgery and psychiatry Vol. 80; no. 5; pp. 524 - 527 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
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London
BMJ Publishing Group Ltd
01.05.2009
BMJ Publishing Group BMJ Publishing Group LTD |
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Abstract | Introduction: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. Methods: Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded. Results: The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways. Conclusions: This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency. |
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AbstractList | Introduction: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. Methods: Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded. Results: The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways. Conclusions: This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency. Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency. Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded. The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways. This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency. INTRODUCTIONCopper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with copper deficiency.METHODSClinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of copper deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded.RESULTSThe predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways.CONCLUSIONSThis pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with copper deficiency. |
Author | Bosch, E P Hoffman-Snyder, C Smith, B E Dodick, D D Ross, M A Goodman, B P |
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Snippet | Introduction: Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and... Copper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings... INTRODUCTIONCopper deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and... |
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SubjectTerms | Aged Aminoacid disorders Ankle Ataxia Biological and medical sciences Copper - deficiency Electrodiagnosis Electromyography Errors of metabolism Evoked Potentials, Somatosensory - physiology Female Gait Disorders, Neurologic - physiopathology Gastrointestinal surgery Humans Magnetic Resonance Imaging Male Medical sciences Metabolic diseases Middle Aged Muscle, Skeletal - physiopathology Neural Conduction - physiology Neurologic Examination Neurology Patients Peripheral Nervous System Diseases - diagnosis Peripheral Nervous System Diseases - pathology Peripheral Nervous System Diseases - physiopathology Retrospective Studies Spinal cord Spinal Cord Diseases - diagnosis Spinal Cord Diseases - pathology Spinal Cord Diseases - physiopathology |
Title | Clinical and electrodiagnostic findings in copper deficiency myeloneuropathy |
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