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CASE REPORT
Year : 2022  |  Volume : 20  |  Issue : 4  |  Page : 257-258

Ischemic monomelic neuropathy


1 Department of Neurology, Pushpagiri Institute of Medical Sciences and Research Centre, Thiruvalla, Kerala, India
2 Department of Internal Medicine, Pushpagiri Institute of Medical Sciences and Research Centre, Thiruvalla, Kerala, India

Date of Submission07-Apr-2022
Date of Decision04-Jun-2022
Date of Acceptance06-Jun-2022
Date of Web Publication17-Oct-2022

Correspondence Address:
Dr. S Sheetal
16 FG, The Edge, Skyline Apartments, Thirumoolapuram, Thiruvalla - 689 115, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cmi.cmi_39_22

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  Abstract 


Ischemic monomelic neuropathy is a rare complication associated with arteriovenous fistula creation for hemodialysis. It is an acutely developing ischemic neuropathy involving multiple nerves of a single limb. The pathogenesis is thought to be decreased blood flow to the distal axons, due to the shunting of blood away from the distal limb. It is characterized by severe pain and muscle weakness along with multiple nerve distributions, confined to the limb. It is different from steal syndrome in that there is no muscle or skin necrosis. It is often a debilitating condition warranting immediate closure of the fistula. Hence, prompt recognition of this entity is essential. Hereby, we report the case of a 60-year-old male with chronic kidney disease, who underwent a brachiocephalic fistula creation in the left upper limb and developed features of ischemic monomelic neuropathy.

Keywords: Brachiocephalic fistula, hemodialysis, ischemic monomelic neuropathy


How to cite this article:
Sheetal S, Remadevi S A, Mathew AS, George AM. Ischemic monomelic neuropathy. Curr Med Issues 2022;20:257-8

How to cite this URL:
Sheetal S, Remadevi S A, Mathew AS, George AM. Ischemic monomelic neuropathy. Curr Med Issues [serial online] 2022 [cited 2022 Dec 1];20:257-8. Available from: https://www.cmijournal.org/text.asp?2022/20/4/257/358640




  Introduction Top


Ischemic monomelic neuropathy is an under-recognized type of ischemic neuropathy involving multiple nerves of a single limb.[1] It has been described in patients undergoing vascular access surgery for hemodialysis, due to shunting of arterial blood away from the distal extremity, leading to ischemia in multiple nerves of the limb. It usually develops acutely, after the creation of the arteriovenous fistula, and is characterized by severe pain and muscle weakness, confined to the limb.[1] It differs from dialysis-associated steal syndrome (DASS) in that there is no muscle or tissue necrosis.[2] It is important to recognize this entity early and take necessary action since it can lead to a permanent disability. Hereby, we report the case of a 60-year-old male with chronic kidney disease, who underwent a brachiocephalic fistula creation in the left upper limb and developed features of ischemic monomelic neuropathy. An informed written consent from the patient was obtained, for reporting this case.


  Case Report Top


A 60-year-old man, with a history of long-standing type 2 diabetes mellitus, systemic hypertension, and chronic kidney disease stage 5, was planned on initiation of hemodialysis. He underwent a radiocephalic fistula for vascular access for hemodialysis, however, it failed and a brachiocephalic fistula was created on the left side [Figure 1]. Few hours later, he developed severe pain, paresthesia, and burning sensation over his left forearm and hand. His left radial pulse was palpable, and the limb was warm to palpation. Benediction sign or pointing index finger sign was noted on the left side [Figure 2]. He had weakness of hand grip, weakness of thenar and hypothenar muscles, lumbricals, interossei and finger extensors of the left upper limb, and impaired sensation over the dorsum and palmar aspect of the left hand indicating a median, radial, and ulnar nerve involvement. Deep tendon reflexes were present in the upper limbs and were absent in the lower limbs, and the plantar response was bilaterally flexor. Vibration and position sense were impaired in bilateral lower limbs, up to the knee. A nerve conduction study showed reduced compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) in the left median, ulnar, and radial nerves and absent CMAPs and SNAPs in bilateral lower limb nerves, suggesting the diagnosis of ischemic monomelic neuropathy of the left upper limb and coexistent diabetic peripheral neuropathy. He underwent closure of the brachiocephalic fistula, 1 week later. On review after 1 month, he had only minimal improvement in power.
Figure 1: Scar of failed radiocephalic fistula and a brachiocephalic fistula in the left upper limb.

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Figure 2: Patient attempting to make a fist with both hands, but the left hand shows “pointing index finger sign.”

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  Discussion Top


Ischemic monomelic neuropathy (IMN) is a rare, albeit debilitating complication of arteriovenous fistula creation, with a reported incidence of 0.1%–3%.[3] Although it was first reported by Bolton et al. in 1979, the term “ischemic monomelic neuropathy” was coined by Wilbourn in 1983.[4],[5] It refers to the ischemia of multiple nerves of a single extremity, resulting in axonal neuropathies. Female sex, diabetes, associated neuropathy, and peripheral vascular occlusive disease are reported to increase the risk of development of this syndrome.[1] Our patient had diabetes mellitus, with associated peripheral neuropathy. The symptoms are usually acute in onset, developing immediately after placement of the fistula and include pain, numbness, paresthesia, and weakness in the distribution of multiple nerves of the limb.[6] The limb is usually warm, with the palpable peripheral pulse, which helps in differentiation from DASS. In DASS, there is associated muscle and skin necrosis, resulting in a cold, cyanosed limb. In IMN, shunting of the blood away from the distal limb results in distal axonal infarction without muscle necrosis. It is hypothesized to result from the nerve's lower threshold for ischemia than that of skin or muscle. A proximally placed fistula, mainly the brachiocephalic type, predisposes to the development of IMN. Our patient also developed IMN after the placement of a brachiocephalic fistula. It has been rarely reported in the lower limb after femoral artery–vein grafting.[2] Nerve conduction studies show low or absent compound muscle action potential and SNAPs. The recommended management is restoring of arterial supply by either surgical or medical intervention; however, the results are variable, and often there is significant residual morbidity.[7]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sheetal S, Byju P, Manoj P. Ischemic monomelic neuropathy. J Postgrad Med 2017;63:42-3.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Thimmisetty RK, Pedavally S, Rossi NF, Fernandes JA, Fixley J. Ischemic monomelic neuropathy: Diagnosis, pathophysiology, and management. Kidney Int Rep 2017;2:76-9.  Back to cited text no. 2
    
3.
Miles AM. Upper limb ischemia after vascular access surgery: Differential diagnosis and management. Semin Dial 2000;13:312-5.  Back to cited text no. 3
    
4.
Bolton CF, Driedger AA, Lindsay RM. Ischaemic neuropathy in uraemic patients caused by bovine arteriovenous shunt. J Neurol Neurosurg Psychiatry 1979;42:810-4.  Back to cited text no. 4
    
5.
Wilbourn AJ, Furlan AJ, Hulley W, Ruschhaupt W. Ischemic monomelic neuropathy. Neurology 1983;33:447-51.  Back to cited text no. 5
    
6.
Redfern AB, Zimmerman NB. Neurologic and ischemic complications of upper extremity vascular access for dialysis. J Hand Surg Am 1995;20:199-204.  Back to cited text no. 6
    
7.
Ramdon A, Breyre A, Kalapatapu V. A case of acute ischemic monomelic neuropathy and review of the literature. Ann Vasc Surg 2017;42:301.e1-5.  Back to cited text no. 7
    


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