Multifocal motor neuropathy presenting with respiratory failure

Boonyapisit, Kanokwan; Katirji, Bashar

doi:10.1002/1097-4598(200012)23:12<1887::aid-mus16>3.0.co;2-y

Cited by 30 publications

(15 citation statements)

References 24 publications

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“…12 It is believed that respiratory failure may be due to chronic conduction blocks in the phrenic nerves and possible axonal damage, which is not rare in the course of CIDP. 11,13 In the literature, a pathologic study of one case who died of ventilatory failure revealed extensive areas of ongoing demyelination and remyelination associated with axonal loss in the phrenic nerve. 5 Dyck et al also noted segmental demyelination with and without hypertrophic changes in 3 patients.…”

Section: Discussionmentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy and Respiratory Failure Due to Phrenic Nerve Involvement

Tataroglu

Özkul²,

Şair³

2010

Journal of Clinical Neuromuscular Disease

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In this article, 2 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are presented. These patients developed respiratory failure which could not be explained by any cardiac or pulmonary pathology. The first case had pure motor involvement and probable CIDP, and the second case had severe sensorial dysfunction and an ataxic picture in addition to motor symptoms. His clinical picture was compatible with distal acquired demyelinating sensory neuropathy without M protein. Electrophysiologic investigations of both patients disclosed impaired phrenic nerve conduction and neurogenic motor unit changes in the diaphragm. Both patients showed a moderate response to immunotherapy. Distal acquired demyelinating sensory and pure motor variant of CIDP can be a cause of respiratory dysfunction. Different clinical patterns of CIDP should be evaluated for phrenic nerve involvement.

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Section: Discussionmentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy and Respiratory Failure Due to Phrenic Nerve Involvement

Tataroglu

Özkul²,

Şair³

2010

Journal of Clinical Neuromuscular Disease

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“…Life expectancy in MMN is normal but conversely spontaneous remission has not been reported 1,5,40. Life-threatening respiratory weakness associated with hypoventilation and respiratory insufficiency due to phrenic nerve involvement with diaphragmatic paresis has been described but is thought to be a rare manifestation of MMN 13,15…”

Section: Natural Historymentioning

confidence: 99%

“…Impairment is mainly in the upper limb function but, with progression of the disease, there can be a more generalized impact. Cranial nerve impairment is rare 12,13,84. Somewhat surprisingly, sensory symptoms such as pain and dysesthesia are not uncommon.…”

Section: Patient Focused Perspectivesmentioning

confidence: 99%

Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment

Lawson¹,

Arnold²

2014

NDT

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Multifocal motor neuropathy (MMN) is an uncommon, purely motor neuropathy associated with asymmetric deficits with predilection for upper limb involvement. Even in the early descriptions of MMN, the associations of anti-GM1 antibodies and robust response to immunomodulatory treatment were recognized. These features highlight the likelihood of an underlying autoimmune etiology of MMN. The clinical presentation of MMN can closely mimic several neurological conditions including those with more malignant prognoses such as motor neuron disease. Therefore early and rapid recognition of MMN is critical. Serological evidence of anti GM-1 antibodies and electrodiagnostic findings of conduction block are helpful diagnostic clues for MMN. Importantly, these diagnostic features are not universally present, and patients lacking these characteristic findings can demonstrate similar robust response to immunodulatory treatment. In the current review, recent research in the areas of diagnosis, pathogenesis, and treatment of MMN and needs for the future are discussed. The characteristic findings of MMN and treatment implications are reviewed and contrasted with other mimicking disorders.

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“…Prior reports describing phrenic involvement in the setting of multifocal motor neuropathy 6 led us to treat three of our patients with IV Ig. We found it difficult to exclude this potentially treatable disorder based solely on our clinical and electrophysiological findings since it may present focally 7 and because it is not possible to demonstrate conduction block by stimulating the phrenic nerve at only a single point.…”

Section: Results Illustrative Casementioning

confidence: 99%

Extensive brain calcification and dementia in postsurgical hypoparathyroidism

Adorni¹,

Lussignoli²,

Geroldi³

et al. 2005

Neurology

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Abstract-The authors report four patients with a syndrome of painless bilateral isolated phrenic neuropathy. Electrophysiologic testing demonstrated active denervation restricted to the diaphragm. Long-term recovery was poor. The authors conclude that bilateral isolated phrenic neuropathy is a cause of painless diaphragmatic paralysis distinguishable from immune brachial plexus neuropathy and other neuromuscular disorders with similar clinical presentation. Bilateral phrenic neuropathy is a rare cause of unexplained dyspnea. Current literature generally describes this presentation in the setting of arm weakness with neck or shoulder pain, often occurring after infectious illness or surgery. [1][2][3] It follows that these factors have led authors to place this phenomenon within the spectrum of immune brachial plexus neuropathy. [1][2][3][4] In contrast, bilateral isolated phrenic neuropathy (BIPN) unaccompanied by pain, arm weakness, or antecedent events has not been emphasized as a distinct entity. In contrast to immune brachial plexus neuropathy, this painless involvement may be difficult to recognize and distinguish from other neuromuscular disorders with similar respiratory symptoms and signs.Methods. We reviewed the records of consecutive patients referred to our neuromuscular centers between 1995 and 2005 for evaluation of bilateral diaphragmatic paralysis. These patients were initially evaluated medically and referred after cardiac, pulmonary, and chest wall disorders were excluded. Presenting symptoms ranged from dyspnea, orthopnea, and exercise intolerance to frank respiratory failure. Pulmonary function testing revealed a restrictive process, with reduction of forced vital capacity with a proportional reduction of forced expiratory volume in one second suggestive of a neuromuscular disorder. Chest radiographs demonstrated bilateral diaphragmatic elevation, whereas fluoroscopy showed paradoxical upward movement of the diaphragm with sniff maneuver. Results. Illustrative case.A 43-year-old man noticed shortness of breath that developed over several days.There was no history of pain, prodromal illness, trauma, or vaccinations. A chest radiograph demonstrated an elevated left hemidiaphragm. Breathing became more difficult 4 months later and he reported that he could no longer walk 30 feet without becoming short of breath. Forced vital capacity (FVC) was 22% of predicted and the FEV1/FVC ratio was 78%. A repeat chest radiograph showed bilateral hemidiaphragm elevations and fluoroscopy confirmed diaphragmatic paralysis. Spinal fluid protein level was 61 mg/dL with a normal cell count. Antiacetylcholine receptor antibodies, anti-GM-1 antibodies, and serum creatine kinase level were normal.Electrophysiologic testing revealed unobtainable phrenic nerve conduction responses bilaterally. Nerve conduction testing of the right upper limb and low-frequency repetitive stimulation of the nasalis (facial nerve), trapezius (accessory nerve), and first dorsal interosseous (ulnar nerve) were normal. EMG of the right diaph...

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Multifocal motor neuropathy presenting with respiratory failure

Cited by 30 publications

References 24 publications

Chronic Inflammatory Demyelinating Polyneuropathy and Respiratory Failure Due to Phrenic Nerve Involvement

Chronic Inflammatory Demyelinating Polyneuropathy and Respiratory Failure Due to Phrenic Nerve Involvement

Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment

Extensive brain calcification and dementia in postsurgical hypoparathyroidism

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