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המפגש האחרון של קבוצת התמיכה התקיים בתאריך 14.4.2016.

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cidp

 

Subarachnoid Block for a Patient with Progressive Chronic Inflammatory Demyelinating Polyneuropathy

Joy E. Schabel, MD

Department of Anesthesiology, State University of New York at Stony Brook, Stony Brook, New York

Address correspondence and reprint requests to Joy E. Schabel, MD, Department of Anesthesiology, State University of New York at Stony Brook, Stony Brook, New York 11794-8480.

 

Abstract

IMPLICATIONS: We report a case of successful administration of a spinal anesthetic to a patient with progressive chronic inflammatory demyelinating polyneuropathy (CIDP). There have been no reports of regional anesthetic management of patients with CIDP.

Chronic inflammatory demyelinating polyneuropathy (CIDP), first described by Dyck et al. (1) in 1975, is a polyneuropathy characterized by slowly progressive, symmetrical limb weakness, widespread areflexia, and loss of large-fiber (vibratory) sensibility of at least 2 mo duration (2). Sensory symptoms present as paresthesias and numbness. Weakness of the respiratory muscles can occur, requiring mechanical ventilation. There have been no reports of regional anesthetic management of patients with CIDP. We discuss successful administration of a spinal anesthetic to a patient with progressive CIDP undergoing cesarean delivery.

Case Report

A 31-yr-old G3P1 woman at 36-wk gestation presented for urgent cesarean delivery because of rapidly progressive CIDP. The patient was first diagnosed with CIDP at 33-wk gestation when she presented to the neurology clinic with a 5-mo history of progressive leg weakness. This resulted in significant gait difficulty. She denied any sensory symptoms. Extensive laboratory work-up revealed mild vitamin B12 deficiency. Vitamin B12 supplementation was begun and the patient’s weakness improved. Spinal magnetic resonance imaging and lumbar puncture were deferred until after delivery. Nerve conduction studies indicated an acquired demyelinating neuropathy. Hematological work-up for secondary causes from systemic disease, such as human immunodeficiency virus, monoclonal gammopathy, cytomegalovirus, chronic active hepatitis, Lyme disease, systemic lupus erythematosus, cryoglobulinemia, multiple myeloma, thyroid disease, diabetes mellitus, and neurosyphilis was negative. The 5-mo time course of weakness progression, absence of an antecedent viral infection or event, and absence of autonomic dysfunction made the diagnosis of acute Guillain-Barre Syndrome (GBS) or Recurrent GBS unlikely.

The patient’s weakness continued to improve over the next 17 days. On day 18, she began having left arm numbness and tingling in a C8 distribution. She denied any speech, visual, swallowing, breathing, bowel, or bladder disturbances. The patient’s neurologist believed that the new upper extremity paresthesias predicted a rapid deterioration to possible respiratory weakness and ventilator dependency. An urgent cesarean delivery was recommended.

On arrival to the obstetric suite, the patient’s chief complaints were left arm paresthesias and leg weakness. She denied any significant past medical, surgical or family history. Her medications were vitamin B12. The patient was 160 cm tall and weighed 70 kg. She had no respiratory complaints. Physical examination was unremarkable except for the neurological examination, which revealed mild weakness (5-/5) of the orbicular oculi bilaterally, mild weakness (5-/5) of neck flexors, and weakness (4/5) of bilateral leg flexors, extensors, adductors, and abductors. Sensation was intact to light touch and pinprick. Vibration sense was decreased in both feet. Deep tendon reflexes were 2/2 throughout. Bilateral ankle jerks were absent. Her gait was ataxic. The Romberg test was negative. Baseline negative inspiratory force was >−60 mm Hg and vital capacity was 4.3 L. The patient refused IV steroid administration that was recommended by the neurologist.

A spinal anesthetic was chosen for this patient. The neurologist requested that we collect 10 mL of cerebral spinal fluid (CSF) for analysis before drug injection. The subarachnoid space was entered on the first attempt at the L3-4 vertebral level. The CSF aspirate was clear and after 10 mL of CSF was collected, 1.6 mL of bupivacaine 0.75% with 8.25% dextrose, fentanyl 10 μg, and preservative-free morphine 0.2 mg were injected. A T4 sensory level to pinprick was obtained. The delivery and intraoperative course were uneventful. After recovery from the spinal anesthetic, the patient’s neurological examination was unchanged from that performed preoperatively. Serial vital capacities ranged from 2.8 L to 3.4 L and all negative inspiratory forces were >−60mm Hg. No additional medical therapy was initiated. On postoperative day 1, her upper extremity paresthesias resolved and her leg weakness improved. Because of such improvement, no additional medical therapy was initiated at that time. However, with any signs of neurological deterioration, IV steroids were to be administered. On postoperative day 3, she was ambulating on her own. On postoperative day 4, she was discharged home. Her CSF studies were all unremarkable.

Three-month follow-up revealed the patient to have continued improvement of symptoms, though there was still some residual lower extremity weakness. Two-year follow-up revealed the patient to have continued relapsing and remitting proximal leg weakness with mild proximal leg atrophy. Repeat nerve conduction studies continued to show the acquired demyelinating polyneuropathy.

 

Discussion

CIDP is a predominantly motor polyneuropathy of unknown etiology with progression over at least two months (1,3). Thereafter, the weakness may progress or may be stable during months or years, or the patient may improve spontaneously before experiencing a relapsing-remitting course (4). The onset may occur at any age, and the disease progression, although usually slow, might be rapid with features similar to those of GBS. By definition, the nadir of GBS is reached within four weeks (5). The time course of weakness progression is one of the criteria that contrasts GBS from CIDP. Physical examination generally reveals proximal symmetric muscle weakness with or without distal sensory loss. Deep tendon reflexes are usually reduced or absent, and there are no central nervous system manifestations. There is also no infectious prodrome, no autonomic dysfunction, and no other known cause for the polyneuropathy. Nerve conduction abnormalities, as seen in this patient, must meet four electrophysiologic criteria to provide a definitive diagnosis of CIDP: slowing of conduction velocity in distal nerve segments to at least 60% of normal, prolongation of F wave latency, prolonged distal motor latencies, and conduction block in one or more nerves (2). Typically, the CSF protein level is increased but not always. Depending on the severity of symptoms, treatments include IV steroid administration, plasma exchange and IV immune globulins (2). Many patients reach remission either spontaneously or through therapy (5). However, it appears that the longer patients are followed, the more likely they will be observed to relapse (3). There appears to be a tendency for relapses, or a worsening of symptoms during relapse, during the third trimester of pregnancy or postpartum (6). It is not known how immunological responses in pregnancy could cause or exacerbate CIDP.

A MEDLINE search revealed only one report of general anesthetic management of a patient with CIDP. In 2000, Hara et al. (7) showed a prolonged effect of vecuronium in a man with CIDP undergoing partial gastrectomy. Reports on patients with other lower motor neuron diseases show a hypersensitivity to nondepolarizing muscle relaxants such as d-tubocurare (8). Hyperkalemia has occurred after administration of succinylcholine (9) and cardiac arrest has occurred after succinylcholine administration in a pregnant patient recovered from GBS (10).

No reports regarding regional anesthesia and CIDP could be found in our search. One publication reported a patient with GBS requiring a decreased dose of local anesthetic for cesarean delivery under epidural anesthesia (11). Additionally, there is one report of a subarachnoid block being administered to a man with multiple system atrophy without complications (12).

In light of detrimental effects of depolarizing drugs and possible hypersensitivity to nondepolarizing drugs in a patient with full stomach precautions and progressive neuromuscular dysfunction, a regional anesthetic was chosen. A subarachnoid block was given to obtain CSF for studies, producing a T4 sensory level without any adverse sequelae. A combined spinal-epidural technique could have been performed that would have allowed CSF to be attained and titration of the anesthetic block in a more controlled fashion. The author admits that, in retrospect, this may have been a preferred approach.

Performing neuraxial blockade in a patient with neurological dysfunction is not without concerns. Historically, there has been controversy over the administration of regional anesthesia in patients with multiple sclerosis (MS) because the effect of local anesthetic on the course of the disease is unclear. MS is also a chronic progressive demyelinating disease with exacerbations and remissions but it involves the central nervous system, in contrast to CIDP, which is limited to the peripheral nervous system. Relapses have occurred in MS patients after spinal anesthesia (13), prolonged epidural analgesia, or after exposure to large local anesthetic dosage or concentrations (14). The relapses were thought to result from larger CSF levels of drug (14). However, the same authors reported no difference in the relapse rate among women with MS who delivered under epidural, local, or general anesthesia. Though the use of epidural blockade does not appear to have adverse effects on the course of MS, it is suggested that only the smallest effective concentration and volume of local anesthetic should be given (14).

Another concern regarding regional anesthesia administration to patients with neurological dysfunction is impaired respiratory function. High sensory levels required for adequate anesthesia during cesarean delivery might paralyze the intercostal muscles (15) and cause respiratory distress in patients dependent on intercostal function for adequate respiration.

There might also be unpredictable block height in patients with neurological dysfunction. One report indicated a higher-than-usual spread of epidural block in a patient with GBS (11). However, this finding was not seen in a more recent report of combined spinal-epidural administration in a patient with GBS (16).

Alternatives to neuraxial blockade with avoidance of depolarizing muscle relaxant use, such as awake intubation and nondepolarizing muscle relaxant administration, were considered in this case. However, the benefits of regional anesthesia in the pregnant patient were considered to outweigh the theoretical risk of further neurological damage. Also, because the patient’s respiratory function was normal preoperatively, it was felt that respiratory distress from intercostal muscle paralysis was unlikely.

The choice of anesthesia for a patient with CIDP is difficult, given the paucity of available literature for either general or regional techniques. We report our initial experience with spinal anesthetic administration to a pregnant patient with CIDP for cesarean delivery.

Footnotes

  • Accepted June 22, 2001.

 

References

1.Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975; 50: 621–37.

MedlineWeb of Science

2.Small GA, Lovelace RE. Chronic inflammatory demyelinating polyneuropathy. Semin Neurol 1993; 13: 305–12.

MedlineWeb of Science

3.Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory polyradiculoneuropathy. Clinical characteristics, course, recommendations for diagnostic criteria. Arch Neurol 1989; 46: 878–84.

Abstract/FREE Full Text

4.Dyck PJ, Prineas J, Pollard JD. Chronic inflammatory demyelinating polyneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 3rd ed. Philadelphia: WB Saunders, 1993: 1498–518.

5.Van der Meche FGA, van Doorn PA. Guillain-Barre syndrome and chronic inflammatory demyelination polyneuropathy: immune mechanisms and updates in current theories. Ann Neurol 1995; 37 (S1): 514–31.

6.McCombe PA, McManis PG, Frith SA, et al. Chronic inflammatory demyelinating polyneuropathy associated with pregnancy. Ann Neurol 1987:102–4.

7.Hara K, Minami K, Takamoto K, et al. The prolonged effect of a muscle relaxant in a patient with chronic inflammatory demyelinating polyneuropathy. Anesth Analg 2000 90 224 –6.

FREE Full Text

8.Rosenbaum KJ, Neigh JL, Strobel GE. Sensitivity to nondepolarizing muscle relaxants in amyotrophic lateral sclerosis: report of two cases. Anesthesiology 1971; 35: 638–41.

MedlineWeb of Science

9.Cooperman LH, Strobel GE Jr Kennel EM. Massive hyperkalemia after administration of succinylcholine. Anesthesiology 1970; 32: 161–4.

MedlineWeb of Science

10.Feldman JM. Cardiac arrest after succinylcholine administration in a pregnant patient recovered from Guillain-Barre syndrome. Anesthesiology 1990; 72: 942–4.

MedlineWeb of Science

11.McGrady EM. Management of labour and delivery in a patient with Guillain-Barre Syndrome [letter]. Anaesthesia 1987; 42: 899.

MedlineWeb of Science

12.McBeth C, Murrin K. Subarachnoid block for a case of multiple system atrophy. Anaesthesia 1997; 52: 884–5.

CrossRefMedlineWeb of Science

13.Bamford C, Sibley W, Laguna J. Anesthesia in multiple sclerosis. Can J Neurol Sci 1978; 5: 41–4.

MedlineWeb of Science

14.Bader AM, Hunt CO, Datta S, et al. Anesthesia for the obstetric patient with multiple sclerosis. J Clin Anesth 1988; 1: 21–4.

Medline

15.Harrop-Griffiths AW, Ravalia A, Browne DA, et al. Regional anesthesia and cough effectiveness. Anaesthesia 1991; 46: 173.

16.Vassiliev DV, Nystrom EV, Leicht CH. Combined spinal and epidural anesthesia for labor and cesarean delivery in a patient with Guillian-Barre Syndrome. Reg Anesth Pain Med 2001; 26: 174–6.

MedlineWeb of Science

 

Copyright © 2010 by the International Anesthesia Research Society

 

ברוכים הבאים

הגעתם לאתר  הרשמי של קבוצת התמיכה בישראל לחולי GBS ו CIDP. האתר מהווה מענה לאלו שאובחנו כסובלים מתסמיני המחלה, לחולים ועבור משפחות התומכות בחולים.

הערה חשובה: המידע באתר ו/או המענה דרך הפורום אינו מחליף בדיקה רפואית מוסמכת ואינו מהווה "אבחנה רפואית" אלא מהווה מקום להתייעצות, החלפת דעות, שיתוף במידע וקבלת סיוע ומשוב מגולשים בעלי עניין זהה במחלה והשלכותיה.

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