|
Spastic Tetraparesis Due to CIDP with Nerve Root Hypertrophy
|
Abstract
Chronic inflammatory demyelinating polyneuropathy (CIDP) with nerve root hypertrophy is a relatively common association, reported in approximately 16 - 54% of cases based on imaging studies. However, it may present a diagnostic challenge, particularly in atypical clinical presentations. We report the case of a 43-year-old patient diagnosed with CIDP who presented with pyramidal syndrome in the lower limbs. Spinal MRI revealed marked hypertrophy of the nerve roots, underscoring the critical role of imaging in supporting the diagnosis and excluding differential diagnoses. Treatment with rituximab, an anti-CD20 monoclonal antibody, has shown promising outcomes in cases of CIDP resistant to conventional therapies, leading to significant clinical improvement and sustained remission in many reported cases, including ours.
Hayani, O. E. , Benabdeljlil, M. , Bnouhanna, W. , Rahmani, M. , Errguig, L. , Birouk, N. and Aidi, S. (2025). Spastic Tetraparesis Due to CIDP with Nerve Root Hypertrophy —A Case Report and Literature Review. Open Access Library Journal, 12, e3429. doi: http://dx.doi.org/10.4236/oalib.1113429.
References| [1] | Akpinar, C.K., Dogru, H., Balci, K. and Terzi, M. (2014) An Atypical Chronic Inflammatory Demyelinating Polyradiculoneu-ropathy That Radiologically Mimicking Neurofibromatosis: Case Report. Journal of Experimental and Clinical Medicine, 31, 111-113. https://doi.org/10.5835/jecm.omu.31.02.011 |
| [2] | Rentzos, M., Anyfanti, C., Kaponi, A., Pandis, D., Ioannou, M. and Vassilopoulos, D. (2007) Chronic Inflammatory Demyelinating Polyneuropathy: A 6-Year Retrospective Clinical Study of a Hospital-Based Population. Journal of Clinical Neuroscience, 14, 229-235. https://doi.org/10.1016/j.jocn.2006.11.001 |
| [3] | Pinto, M.V., Spinner, R.J. and Staff, N.P. (2018) A Treatable Hyper-trophic Neuropathy. Practical Neurology, 19, 80-82. https://doi.org/10.1136/practneurol-2018-002083 |
| [4] | Hasan, M.T., Patil, S., Chauhan, V., Gosal, D., Ealing, J., Du Plessis, D., et al. (2021) Spinal Cord Compression from Hypertrophic Nerve Roots in Chronic Inflammatory Demyelinating Polyradiculoneuropathy—A Case Report. Surgical Neurology Interna-tional, 12, Article 114. https://doi.org/10.25259/sni_35_2021 |
| [5] | Stino, A.M., Naddaf, E., Dyck, P.J. and Dyck, P.J.B. (2020) Chronic Inflammatory Demyelinating Polyradiculoneuropathy—Diagnostic Pitfalls and Treatment Approach. Muscle & Nerve, 63, 157-169. https://doi.org/10.1002/mus.27046 |
| [6] | Laughlin, R.S., Dyck, P.J., Melton, L.J., Leibson, C., Ran-som, J. and Dyck, P.J.B. (2009) Incidence and Prevalence of CIDP and the Association of Diabetes Mellitus. Neurology, 73, 39-45. https://doi.org/10.1212/wnl.0b013e3181aaea47 |
| [7] | Dziadkowiak, E., Koszewicz, M., Podgórski, P., Wieczorek, M., Budrewicz, S. and Zimny, A. (2024) Central Nervous System Involvement in Chronic Inflammatory Demyelinating Poly-radiculoneuropathy—MRS and DTI Study. Frontiers in Neurology, 15, Article 1301405. https://doi.org/10.3389/fneur.2024.1301405 |
| [8] | Broers, M.C., Bunschoten, C., Nieboer, D., Lingsma, H.F. and Jacobs, B.C. (2019) Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Re-view and Meta-Analysis. Neuroepidemiology, 52, 161-172. https://doi.org/10.1159/000494291 |
| [9] | Ye, L., Yu, W., Liang, N., Sun, Y. and Duan, L. (2022) Spinal Canal Decompression for Hypertrophic Neuropathy of the Cauda Equina with Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Case Report. World Journal of Clinical Cases, 10, 4294-4300. https://doi.org/10.12998/wjcc.v10.i13.4294 |
| [10] | VandenBergh, P.Y.K., et al. (2021) European Academy of Neurology/Peripheral Nerve Society Guideline on Diagnosis and Treatment of Chronic Inflammatory Demyelinating Poly-radiculoneuropathy: Report of a Joint Task Force—Second Revision. European Journal of Neurology, 28, 3556-3583. |
| [11] | Ripellino, P., Ventura, E., Querol, L. and Gobbi, C. (2021) Chronic Inflammatory Demyelinating Polyneu-ropathy with Hypertrophic Nerves. Journal of the Peripheral Nervous System, 26, 227-230. |
| [12] | Dalakas, M.C. (2011) Advances in the Diagnosis, Pathogenesis and Treatment of CIDP. Nature Reviews Neurology, 7, 507-517. https://doi.org/10.1038/nrneurol.2011.121 |
| [13] | Wang, Y., Chen, H., Zhuang, W. and Li, H. (2018) The Clinical Features of Combined Central and Peripheral Demyelination in Chinese Patients. Journal of Neuroimmunology, 317, 32-36. https://doi.org/10.1016/j.jneuroim.2018.02.006 |
| [14] | Ogata, H., Matsuse, D., Yamasaki, R., Kawamura, N., Matsushita, T., Yonekawa, T., et al. (2015) A Nationwide Survey of Combined Central and Peripheral Demyelination in Japan. Journal of Neurology, Neurosurgery & Psychiatry, 87, 29-36. https://doi.org/10.1136/jnnp-2014-309831 |
| [15] | Aktas, O. (2017) Shifting Borders, Crossing Boundaries: The Case of Combined Central and Peripheral Demyelination. Multiple Sclerosis Journal, 24, 550-551. https://doi.org/10.1177/1352458517726386 |
| [16] | Matsuda, M., Ikeda, S., Sakurai, S., Nezu, A., Yanagisawa, N. and Inuzuka, T. (1996) Hypertrophic Neuritis Due to Chronic Inflammatory Demyelinating Polyradiculo-neuropathy (CIDP): A Postmortem Pathological Study. Muscle & Nerve, 19, 163-169. https://doi.org/10.1002/(sici)1097-4598(199602)19:2<163::aid-mus6>3.0.co;2-c |
| [17] | Duggins, A.J., McLeod, J.G., Pol-lard, J.D., Davies, L., Yang, F., Thompson, E.O., et al. (1999) Spinal Root and Plexus Hypertrophy in Chronic Inflammatory Demyelinating Polyneuropathy. Brain, 122, 1383-1390. https://doi.org/10.1093/brain/122.7.1383 |
| [18] | Tanaka, K., Mori, N., Yokota, Y. and Suenaga, T. (2013) MRI of the Cervical Nerve Roots in the Diagnosis of Chronic Inflammatory De-myelinating Polyradiculoneuropathy: A Single-Institution, Retrospective Case-Control Study. BMJ Open, 3, e003443. https://doi.org/10.1136/bmjopen-2013-003443 |
| [19] | Shaefer, A., Alsharabati, M., Claussen, G. and Oh, S. (2014) Spinal Nerve Root Hypertrophy as Etiology of Back Pain in CIDP (P1.072). Neurology, 82. https://doi.org/10.1212/wnl.82.10_supplement.p1.072 |
| [20] | Matsuoka, N., Kohriyama, T., Ochi, K., Nishitani, M., Sueda, Y., Mimori, Y., et al. (2004) Detection of Cervical Nerve Root Hypertrophy by Ultrasonography in Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Journal of the Neurological Sciences, 219, 15-21. https://doi.org/10.1016/j.jns.2003.11.011 |
| [21] | Tazawa, K., Matsuda, M., Yoshida, T., Shimojima, Y., Gono, T., Morita, H., et al. (2008) Spinal Nerve Root Hypertrophy on MRI: Clinical Significance in the Diagnosis of Chronic Inflammatory Demye-linating Polyradiculoneuropathy. Internal Medicine, 47, 2019-2024. https://doi.org/10.2169/internalmedicine.47.1272 |
| [22] | Schady, W., Goulding, P.J., Lecky, B.R., King, R.H. and Smith, C.M. (1996) Massive Nerve Root Enlargement in Chronic Inflammatory Demyelinating Polyneuropathy. Journal of Neurology, Neurosurgery & Psychiatry, 61, 636-640. https://doi.org/10.1136/jnnp.61.6.636 |
| [23] | Mythri, A. (2021) CIDP Present-ing as Myeloradiculopathy. International Journal of Scientific Research, 10, 50-51. |
| [24] | Staff, N.P., Figueroa, J.J., Parisi, J.E. and Klein, C.J. (2010) Hypertrophic Nerves Producing Myelopathy in Fulminant CIDP. Neurology, 75, 750. https://doi.org/10.1212/wnl.0b013e3181eee4ed |
| [25] | Guibord, N., Chalk, C., Wein, F., Richardson, J., Snipes, G.J. and Del Carpio, R. (1998) Trigeminal Nerve Hypertrophy in Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Neurol-ogy, 51, 1459-1462. https://doi.org/10.1212/wnl.51.5.1459 |
| [26] | Zhao, H., Zheng, Y., Meng, L., Yu, M., Zhang, W., Lv, H., et al. (2021) Chronic Inflammatory Demyelinating Polyneuropathy with Hypoglossal Nerve Involvement and Inverted Bee-vor’s Sign: Case Report. BMC Neurology, 21, Article No. 244. https://doi.org/10.1186/s12883-021-02287-5 |
| [27] | Aïdi, S., El Alaoui Faris, M., Amarti, A., Belaïdi, H., Jiddane, M., Guezzaz, M., Medjel, A. and Chkili, T. (2002) Polyradiculoneuropathie inflammatoire démyélinisante chronique hypertrophique avec atteinte des nerfs craniens. Revista de Neurología (Paris), 158, 819-823. |
| [28] | Amrami, K.K., Felmlee, J.P. and Spinner, R.J. (2008) MRI of Peripheral Nerves. Neurosurgery Clinics of North America, 19, 559-572. https://doi.org/10.1016/j.nec.2008.07.019 |
| [29] | Preisner, F., Pitarokoili, K., Lueling, B., Motte, J., Fisse, A.L., Grüter, T., et al. (2023) Quantitative Magnetic Resonance Neurography in Chronic Inflammatory De-myelinating Polyradiculoneuropathy: A Longitudinal Study over 6 Years. Annals of Clinical and Translational Neurology, 11, 593-606. https://doi.org/10.1002/acn3.51978 |
| [30] | Su, X., Kong, X., Lu, Z., Zhou, M., Wang, J., Liu, X., et al. (2020) Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy. Korean Journal of Radiology, 21, 483-493. https://doi.org/10.3348/kjr.2019.0739 |
| [31] | Zaidman, C.M. and Pestronk, A. (2014) Nerve Size in Chronic Inflammatory Demyelinating Neuropathy Varies with Disease Activity and Therapy Response over Time: A Retrospective Ultrasound Study. Muscle & Nerve, 50, 733-738. https://doi.org/10.1002/mus.24227 |
| [32] | Niu, J., Zhang, L., Hu, N., Cui, L. and Liu, M. (2023) The Distribution Pattern of Nerve Enlargement in Clinical Subtypes of Chronic Inflammatory Demyelinating Polyneuropathy. Journal of Neuroimaging, 34, 127-137. https://doi.org/10.1111/jon.13162 |
| [33] | Schaik, I.N.v. and Eftimov, F. (2011) Chronic Inflammatory Demy-elinating Polyradiculoneuropathy—An Overview of Existing Treatment Options and Prospects for the Future. European Neurological Review, 6, 45-51. https://doi.org/10.17925/enr.2011.06.01.45 |
