Respiratory Disorders Expert Forum
Left diaphragm paralysis
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Left diaphragm paralysis

My father is 78 and was diagnosed with CIDP Jan of 2012.  He has undergone IVIG for over a year, and Imuran - he has declined to the point of now on a ventilator.  He moved to Florida from Georgia in December, and went into respiratory failure in January 2 days after starting PE.  2 months later after ICU stay in the hospital, his legs are the strongest part of his body [he was wheelchair bound in December] - however, has shown favorable response to this treatment he is still being affected in the right hand with contracture [left hand has contracted over the past year and very weak in that arm] The diaphram paralysis was the symptom that sent him to the hospital resulting in the CIDP diagnosis.  They are crediting the disease for the phrenic nerve damage.  Is there anything available for help with his diaphragm in order to wean from the ventilator?  He is currently at an Acute care hospital....in wean mode...however, it is very slow progress and I fear they will give up, I am sure due to lack of knowledge of this rare disease...any help or advise?  I have also been in contact with the GBS/CIDP foundation, and they have been great...each case is different, and it appears my father has the chronic version of this disease...
242587_tn?1355427710
I regret that I have no expertise or even much general knowledge of CIDP.  But, I know it to be a dreadful disease and I am so sorry that you and your father have been subjected to this ordeal.  The following is an abstract that briefly describes electrophrenic pacing in a variety of neurologic settings. The reporting physician, Dr. Bach could and should be reached by phone or E-mail, ***@**** , and might prove to be a valuable resource to you and the physicians..  

Authors Full Name Bach, John R.
Institution Department of Physical Medicine and Rehabilitation, University of Medicine and Dentistry of New Jersey, B403, 150 Bergen Street, Newark, New Jersey 07103, USA. ***@****

Title Noninvasive respiratory management and diaphragm and electrophrenic pacing in neuromuscular disease and spinal cord injury.

Source Muscle & Nerve. 47(2):297-305, 2013 Feb.
Abstract The purpose of this monograph is to describe noninvasive management of respiratory muscle weakness/paralysis for patients with neuromuscular disease (NMD) and spinal cord injury (SCI). Noninvasive ventilation (NIV) assists and supports inspiratory muscles, whereas mechanically assisted coughing (MAC) simulates an effective cough. Long-term outcomes will be reviewed as well as the use of NIV, MAC, and electrophrenic pacing (EPP) and diaphragm pacing (DP) to facilitate extubation and decannulation. Although EPP and DP can facilitate decannulation and maintain alveolar ventilation for high-level SCI patients when they cannot use NIV because of lack of access to oral interfaces, there is no evidence that they have any place in the management of NMD. Copyright 2012 Wiley Periodicals, Inc.
Following it is another report that may be of interest to you and your father’s physicians.  I give the citation and the discussion.  This too might be shared with his physicians and consideration given to contacting the authors of both reports.  I realize that, unless you are a physician or have familiarity with neurologic disease, these may not be of interest to you, but they might serve to prompt his physicians to consider alternative approaches.

Journal of Clinical Neuromuscular Disease
Issue: Volume 12(1), September 2010, pp 42-46
Copyright: © 2010 Lippincott Williams & Wilkins, Inc.
Publication Type: [Short Report]
DOI: 10.1097/CND.0b013e3181d80bdc
ISSN: 1522-0443
________________________________________
Chronic Inflammatory Demyelinating Polyneuropathy and Respiratory Failure Due to Phrenic Nerve Involvement
Tataroglu, Cengiz MD; Ozkul, Ayca MD; Sair, Ahmet MD
Author Information
From the Adnan Menderes University, Faculty of Medicine, Department of Neurology, Aydin, Turkey.
Reprints: Ayca Ozkul, MD, Adnan Menderes University Medicine Faculty, Neurology Department, 09100 Aydin, Turkey (e-mail:***@****).
Abstract:
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.
DISCUSSION
In this article, 2 patients are reported with different clinical features of CIDP with respiratory failure due to phrenic nerve involvement. The first patient had a nerve conduction study concordant with demyelinating neuropathy and fulfilled the electrophysiologic criteria of CIDP. He complained of slowly progressive symmetrical weakness and needed respiratory support because of phrenic nerve palsy. He was therefore accepted as a case of pure motor variant of CIDP with phrenic nerve involvement. In addition, he had moderate response to plasmapheresis and prednizolon treatment, but still needed ventilatory support in spite of having no other symptom reflecting bulbar dysfunction. The motor variant of CIDP has been observed in only 10% of patients with CIDP.9,10 It is not a commonly seen variant and motor CIDP associated with phrenic nerve involvement causing respiratory failure had not been reported previously in the literature.
The second patient had a diagnosis of definite CIDP according to the diagnostic criteria of the Ad Hoc Subcommittee of the American Academy of Neurology (1991).7 He had severe sensorial dysfunctions, and motor involvement was seen especially on the distal extremities. His clinical and laboratory findings were concordant with DADS polyneuropathy.1 This variant can be present as distal motor involvement and primary axonal degeneration.1 Electrophysiologic investigations of the patient demonstrated phrenic nerve involvement in spite of moderate motor involvement of peripheral nerves. Although this patient did not need ventilatory support, he had dyspnea which showed moderate improvement following corticosteroid therapy. Our patient is the first case in the literature with a diagnosis of DADS neuropathy associated with phrenic nerve involvement.
Respiratory failure due to phrenic nerve palsy which may be responsive to treatment with IVIG or steroids can be seen in CIDP patients even with relatively mild limb involvement.11 High incidence of subclinical involvement in CIDP patients has also been reported.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.14 Extensive axonal loss replaced by endoneurial fibrosis has been seen as well.15 Different clinical patterns may have different pathologic findings.
Here 2 different variants of CIDP with respiratory failure due to phrenic nerve involvement are presented. The absence of pathologic evaluation of these patients limits the possibility of commenting on this subject. However, it was felt that pathologic studies of different CIDP variants may help the understanding of the pathophysiology of phrenic nerve involvement and hence may help to obtain novel treatment strategies.
In conclusion, phrenic nerve involvement should always be considered in CIDP patients with respiratory failure, and clinicians should always be aware of phrenic nerve involvement in different CIDP variants. Respiratory failure in the pure motor variant of CIDP and DADS may be due to phrenic nerve involvement, which shows a moderate response to immune therapies. Neurophysiologic evaluation of the phrenic nerve and pulmonary functional tests in different subgroups of CIDP may clarify the pathogenesis and hence help to prevent the more severe features of respiratory failure.

There is another, a surgical technique called Plication of the diaphragm that has been used with some success in persons whose only problem was diaphragmatic paralysis resulting in respiratory failure.  

A caveat for any of the above would be that his respiratory physician(s) would have to be reasonably certain that the diaphragm is the main problem and not involvement of other respiratory muscles with CIDP.

I wish I could be more helpful.

Good luck
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