Quadriplegic areflexic ICU illness: Selective thick filament loss and normal nerve histology
Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or muscle light microscopy. However, electron microscopy often documents a selective thick filament loss myopathy. Eight ICU patients who developed...
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| Published in | Muscle & nerve Vol. 26; no. 4; pp. 499 - 505 |
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| Main Authors | , , |
| Format | Journal Article |
| Language | English |
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01.10.2002
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| Abstract | Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or muscle light microscopy. However, electron microscopy often documents a selective thick filament loss myopathy. Eight ICU patients who developed areflexic quadriplegia underwent biopsy. Seven patients had received steroids, and 2 had also received paralytic agents. Electrodiagnostic studies revealed absent or low‐amplitude motor responses in 7. Sensory responses were normal in 5 of 6 and absent in 1. Initial electromyography revealed absent (n = 3), small (n = 3), or polyphasic (n = 1) motor unit potentials, and diffuse fibrillation potentials (n = 5). In all 8, light microscopy of muscle revealed numerous atrophic‐angulated fibers and corelike lesions, and electron microscopy revealed extensive thick filament loss. Morphology of sural and intramuscular nerves, and, in one autopsied case, of the obturator nerve and multiple nerve roots, was normal. Although clinical, electrodiagnostic, and light microscopic features mimicked denervating disease, muscle electron microscopy revealed thick filament loss, and nerve histology was normal. This suggests that areflexic ICU quadriplegia is a primary myopathy and not an axonal polyneuropathy. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 499–505, 2002 |
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| AbstractList | Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or muscle light microscopy. However, electron microscopy often documents a selective thick filament loss myopathy. Eight ICU patients who developed areflexic quadriplegia underwent biopsy. Seven patients had received steroids, and 2 had also received paralytic agents. Electrodiagnostic studies revealed absent or low‐amplitude motor responses in 7. Sensory responses were normal in 5 of 6 and absent in 1. Initial electromyography revealed absent (n = 3), small (n = 3), or polyphasic (n = 1) motor unit potentials, and diffuse fibrillation potentials (n = 5). In all 8, light microscopy of muscle revealed numerous atrophic‐angulated fibers and corelike lesions, and electron microscopy revealed extensive thick filament loss. Morphology of sural and intramuscular nerves, and, in one autopsied case, of the obturator nerve and multiple nerve roots, was normal. Although clinical, electrodiagnostic, and light microscopic features mimicked denervating disease, muscle electron microscopy revealed thick filament loss, and nerve histology was normal. This suggests that areflexic ICU quadriplegia is a primary myopathy and not an axonal polyneuropathy. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 499–505, 2002 Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or muscle light microscopy. However, electron microscopy often documents a selective thick filament loss myopathy. Eight ICU patients who developed areflexic quadriplegia underwent biopsy. Seven patients had received steroids, and 2 had also received paralytic agents. Electrodiagnostic studies revealed absent or low-amplitude motor responses in 7. Sensory responses were normal in 5 of 6 and absent in 1. Initial electromyography revealed absent (n = 3), small (n = 3), or polyphasic (n = 1) motor unit potentials, and diffuse fibrillation potentials (n = 5). In all 8, light microscopy of muscle revealed numerous atrophic-angulated fibers and corelike lesions, and electron microscopy revealed extensive thick filament loss. Morphology of sural and intramuscular nerves, and, in one autopsied case, of the obturator nerve and multiple nerve roots, was normal. Although clinical, electrodiagnostic, and light microscopic features mimicked denervating disease, muscle electron microscopy revealed thick filament loss, and nerve histology was normal. This suggests that areflexic ICU quadriplegia is a primary myopathy and not an axonal polyneuropathy. Abstract Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or muscle light microscopy. However, electron microscopy often documents a selective thick filament loss myopathy. Eight ICU patients who developed areflexic quadriplegia underwent biopsy. Seven patients had received steroids, and 2 had also received paralytic agents. Electrodiagnostic studies revealed absent or low‐amplitude motor responses in 7. Sensory responses were normal in 5 of 6 and absent in 1. Initial electromyography revealed absent ( n = 3), small ( n = 3), or polyphasic ( n = 1) motor unit potentials, and diffuse fibrillation potentials ( n = 5). In all 8, light microscopy of muscle revealed numerous atrophic‐angulated fibers and corelike lesions, and electron microscopy revealed extensive thick filament loss. Morphology of sural and intramuscular nerves, and, in one autopsied case, of the obturator nerve and multiple nerve roots, was normal. Although clinical, electrodiagnostic, and light microscopic features mimicked denervating disease, muscle electron microscopy revealed thick filament loss, and nerve histology was normal. This suggests that areflexic ICU quadriplegia is a primary myopathy and not an axonal polyneuropathy. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 499–505, 2002 |
| Author | Danon, Moris J. Golden, Marianna Sander, Howard W. |
| Author_xml | – sequence: 1 givenname: Howard W. surname: Sander fullname: Sander, Howard W. email: hws2001@med.cornell.edu organization: Department of Neurology, Peripheral Neuropathy Center, Weill College of Medicine of Cornell University, 635 Madison Ave., Suite 400, New York, New York 10022, USA – sequence: 2 givenname: Marianna surname: Golden fullname: Golden, Marianna organization: Burke Rehabilitation Hospital, White Plains, New York, USA – sequence: 3 givenname: Moris J. surname: Danon fullname: Danon, Moris J. organization: Department of Neurology, New York University School of Medicine, New York, New York, USA |
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| Keywords | Human Tetraplegia Nervous system diseases Filament Pathophysiology Motor system disorder Electron microscopy Abnormal reflex Intensive care unit Case study Pathology Electrodiagnosis Optical microscopy Tendinous areflexia Neurological disorder |
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| References_xml | – volume: 20 start-page: 316 year: 1997 end-page: 322 article-title: Acute quadriplegic myopathy: analysis of myosin isoforms and evidence for calpain‐mediated proteolysis publication-title: Muscle Nerve – volume: 17 start-page: 647 year: 1994 end-page: 654 article-title: Prolonged paralysis due to nondepolarizing neuromuscular blocking agents and corticosteroids publication-title: Muscle Nerve – volume: 19 start-page: 1057 year: 1996 article-title: Phrenic nerve conduction studies in the intensive care unit publication-title: Muscle Nerve – volume: 336 start-page: 1079 year: 1997 end-page: 1088 article-title: Case records of the Massachusetts General Hospital. 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| Snippet | Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon electrophysiology or... Abstract Areflexic quadriplegia that occurs in the intensive care unit (ICU) is commonly ascribed to critical illness polyneuropathy based upon... |
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| SubjectTerms | acute quadriplegic myopathy Adult Aged Biological and medical sciences Critical Care Critical Illness Electrodiagnosis Female Humans Male Medical sciences Microscopy, Electron Middle Aged Muscle, Skeletal - innervation Muscle, Skeletal - pathology Muscle, Skeletal - ultrastructure myopathy Myosins - metabolism Nervous system (semeiology, syndromes) Nervous system as a whole Neurology Neurons - pathology neuropathy Peripheral Nerves - pathology Peripheral Nerves - ultrastructure Plastic Embedding Quadriplegia - pathology Reflex - physiology Sural Nerve - pathology Sural Nerve - ultrastructure thick filament loss |
| Title | Quadriplegic areflexic ICU illness: Selective thick filament loss and normal nerve histology |
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