Clinical History: A 30 year-old male who was involved in a high-speed motor vehicle accident 10 months prior. Axial T2-weighted (1A) and T1-weighted (1B) images are provided. What are the findings? What is your diagnosis?
Traumatic avulsion of the right C2 nerve root.
The peripheral nerves are frequently compromised due to degenerative disc and facet disease in the spine. However, there are other less common causes of peripheral nerve disease that can present with similar clinical findings. This web clinic reviews several non-degenerative causes of peripheral nerve pathology.
Nerve root avulsion is a severe injury which occurs when the nerve roots are subjected to significant traction. In adults, avulsion is usually due to direct trauma or high-speed motor vehicle accidents, with motorcycle accidents commonly cited. In children, avulsion is most frequently seen as a result of birth trauma. When the nerve root is torn, the meninges are also frequently torn, resulting in leakage of CSF and an associated pseudomeningocele.1On MRI, these changes result in an “empty” or featureless nerve root sheath which is fluid-filled with no neural elements; expansion and remodeling of the neural foramen is common. In some cases, a post-traumatic reactive neuroma can develop at the end of the damaged nerve.2,3
Nerve root avulsion presents clinically with variable neurological deficits, including partial or complete motor loss, and can significantly impact activities of daily living. Cervical nerve root avulsion is associated with brachial plexus dysfunction, as the C5 through C7 nerve roots are part of the brachial plexus. If recovery of function does not occur within 3 months, surgical repair is indicated in an attempt to preserve or restore function.4,5
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired demyelinating disease of the peripheral nerve roots. Patients typically present with gradual onset of weakness and loss of reflexes. The clinical presentation can mimic Guillian-Barre syndrome, but CIDP has a more chronic course. Over time, inflammation results in significant thickening of the nerve roots. On MRI, the nerve roots demonstrate fusiform enlargement and generally enhance; associated changes of denervation, such as atrophy or enhancement, can be seen in the paraspinous musculature, and abnormal signal can also be seen in the adjacent spinal cord. Treatment for CIDP consists of steroids and other immunosuppressive drugs.6,7
The hereditary motor and sensory neuropathies (HMSN) are a group of genetic disorders that present as variable progressive motor weakness and sensory deficits. Charcot-Marie-Tooth (CMT) disease is one of the more common HMSNs and is usually transmitted via an autosomal dominant inheritance pattern. In CMT, increased myelination causes hypertrophy of the peripheral nerves with a classic “onion bulb” appearance, described originally as a histopathologic finding but also seen on MRI. Associated muscle atrophy and scoliosis can be present. Enhancement is not usually a common finding. There is currently no treatment for CMT.8,9
Schwannoma is a common benign neoplasm of the peripheral nerve which can present with pain or parasthesias. On MRI imaging, schwannomas are well-circumscribed masses that are hyperintense on T2-weighted images and hypointense on T1-weighted images. They may have a rim of peripheral fat or T2 hyperintensity and are not associated with significant edema. Schwannomas can mimic perineural cysts but are generally more heterogeneous on T2-weighted imaging and enhance after the administration of gadolinium. Schwannomas can be intradural, extradural, or peripheral.10
The peripheral nerves can be involved by leptomeningeal spread of a primary malignancy, most commonly breast cancer, lung cancer, or melanoma. Leptomeningeal spread of cancer is a late-stage complication that is generally associated with a poor prognosis. Although CSF cytology is needed for definitive diagnosis of leptomeningeal disease, T1-weighted post gadolinium images are abnormal in 70-80% of patients with leptomeningeal disease and are helpful in diagnosis. Enhancement, nodularity, and clumping of peripheral nerve roots are seen on MR imaging.11
A perineural cyst is a benign meningeal cyst of the nerve root sheath. Perineural cysts in the sacrum are usually referred to as Tarlov cysts and can uncommonly cause back pain. On MRI, perineural cysts are thin-walled and follow CSF signal intensity on all sequences; the nerve roots are closely associated with the cyst and are preserved. Remodeling of the neural foramen can be seen. Perineural cysts are very common and should not be mistaken for nerve root avulsion or tumors.12,13
In addition to degenerative disease, a variety of processes can involve the nerves in the spine, including trauma, inflammation/demyelination, congenital disease, benign or malignant neoplasm, and non-pathologic cysts. Proper identification of these different entities on MRI examinations has significant clinical implications for treatment and prognosis.
- Haider AS, Watson IT, Sulhan, S, LeonardnD, Arrey EN, Khan, U, Nguyen, P, Layton, KF. Traumatic Cervical Nerve Root Avulsion with Pseudomeningocele Formation. Cureus. 2017 Feb; 9(2): e1028. ↩
- Medina LS, Laylali I, Zurakowski D, Ruiz J, Altman NR, Grossman JAI. Diagnostic performance of MRI and MR myelography in infants with a brachial plexus birth injury. Pediatr Radiol. 2006 Dec; 36(12):1295-9. ↩
- Doai M, Tonami H, Munetaka M, Tamase A, Akane A. Traumatic Neuroma of an Intradural Cervical Nerve Root, Without Trauma. Appl Radiol 2020; 49(1): 54-55. ↩
- Thatte MR, Babhulkar S, Hiremath A. Brachial plexus injury in adults: Diagnosis and surgical treatment strategies. Ann Indian Acad Neurol. 2013 Jan-Mar; 16(1): 26-33. ↩
- Yoshikawa T, Hayashi N, Yamamoto S, Tajiri Y, Yoshioka N, Tomohiko M, Mori H, Abe O, Aoki S, Ohtomo K. Brachial Plexus Injury: Clinical Manifestations, Conventional Imaging Techniques, and the Latest Imaging Techniques. RadioGraphics. 2006; 26:S133-S143. ↩
- Thawait SK, Chaudry V, Thawait GK, Wang, KC, Belzberg, A, Carrino JA, Chhabram A. High-Resolution MR Neurography of Diffuse Peripheral Nerve Lesions. AJNR. Sept 2011; 32(8) 1365-1372. ↩
- Deng F, Gaillard F. Chronic inflammatory demyelinating polyneuropathy. www.radiopaedia.org. ↩
- Aho TR, Wallace RC, Pitt AM, Sivakumar S. Charcot-Marie-Tooth Disease: Extensive Cranial Nerve Involvement on CT and MR Imaging. AJNR. March 2004; 25(3) 494-497. ↩
- Bell DJ, Gaillard, F. Charcot-Marie-Tooth Disease. www.radiopaedia.org. ↩
- Crist J, Hodge JR, Frick M, Leung FP, Hsu E, Ming TG, Venkatesh SK. Magnetic Resonance Imaging Appearance of Schwannomas from Head to Toe: A Pictorial Review. J Clin Imaging Sci. 2017; 7:38. ↩
- Nayar G, Ejikeme T, Chongsathidkiet P, Elsamadicy AA, Blackwell K, Clarke JM, Lad SP, Fecci PE. Leptomeningeal disease; current diagnostic and therapeutic strategies. Oncotarget. 2017; 8(42):73312-73328. ↩
- Bell DJ, Jones J. Tarlov cyst. www.radiopaedia.org ↩
- Tarlov Cyst: A diagnostic of exclusion. Andrieux C, Poglia P, Laudato P. Int J Surg Case Rep. 2017; 39:25-28. ↩