|Year : 2022 | Volume
| Issue : 4 | Page : 257-258
Ischemic monomelic neuropathy
S Sheetal1, S Athira Vijayan Remadevi2, Ashna Sara Mathew2, Alna Merin George2
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 Submission||07-Apr-2022|
|Date of Decision||04-Jun-2022|
|Date of Acceptance||06-Jun-2022|
|Date of Web Publication||17-Oct-2022|
Dr. S Sheetal
16 FG, The Edge, Skyline Apartments, Thirumoolapuram, Thiruvalla - 689 115, Kerala
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
Ischemic monomelic neuropathy is an under-recognized type of ischemic neuropathy involving multiple nerves of a single limb. 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. It differs from dialysis-associated steal syndrome (DASS) in that there is no muscle or tissue necrosis. 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|| |
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|| |
Ischemic monomelic neuropathy (IMN) is a rare, albeit debilitating complication of arteriovenous fistula creation, with a reported incidence of 0.1%–3%. Although it was first reported by Bolton et al. in 1979, the term “ischemic monomelic neuropathy” was coined by Wilbourn in 1983., 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. 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. 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. 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.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]