An atypical chronic inflammatory demyelinating polyradiculoneuropathy that radiologically mimicking neurofibromatosis: Case report
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon demyelinating disorder with a relapsing and remitting or continuously progressive course. Patients may have motor and sensory involvement, but generally motor involvement may be more prominent and more severe in lower extremities. CIDP is a treatable neuropathy that is challenging to diagnose and has a broad spectrum of presentations. When ranked by the descending frequency, postural tremor in the arms, peripheral nerve thickening, papilledema and facial or bulbar weakness, respiratory failure and autonomic dysfunction can be seen in patients with CIDP. Demyelinating neuropathy predominantly affects spinal roots, plexuses and proximal nerve trunks and thickened nerves can be palpable in about 10% of the patients. In patients with atypical presentation, the diagnosis of CIDP may be delayed and hypertrophic nerve roots have been reported in CIDP patients with delayed diagnosis. Magnetic Resonance Imaging (MRI) may be helpful in the diagnosis of CIDP by excluding the compressive or structural lesions that may lead to polyradiculopathy and MRI may also show hypertrophy of nerve roots and inflammatory processes in CIDP. We presented a patient with CIDP who had MRI findings of diffuse enlargement and mild enhancement of roots and extraforaminal segments of nerves in all segments.
Crino, P.B., Grossman, R.J., Rostami, A., 1993. Magnetic resonance imaging of the cauda equina in chronic inflammatory demyelinating polyneuropathy. Ann. Neurol. 33, 311-313.
Duggins, A.J., McLeod, J.G., Pollard, J.D., Davies, L., Yang, F., Thompson, E.O., Soper, J.R., 1999. Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy. Brain. 122, 1383-1390.
Goldstein, J.M., Parks, B.J., Mayer, P.L., Kim, J.H., Sze, G., Miller, R.G., 1996. Nerve root hypertrophy as the cause of lumbar stenosis in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 19, 892-896.
Matsuda, M., Ikeda, S., Sakurai, S., Nezu, A., Yanagisawa, N., Inuzuka, T., 1996. Hypertrofic neuritis due to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): A postmortem pathological study. Muscle Nerve. 19, 163-169.
Midroni, G., Dyck, P.J., 1996. Chronic inflammatory demyelinating polyradiculoneuropathy: Unusual clinical features and therapeutic responses. Neurology. 46, 1202-1212.
Mizuno, K., Nagamatsu, M., Hattori, N., Yamamoto, M., Goto, H., Kuniyoshi, K., Sobue, G., 1998. Chronic inflammatory demyelinating polyradiculoneuropathy with diffuse and massive peripheral nevre hypertrophy: Distinctive clinical and magnetic resonance imaging features. Muscle Nerve. 21, 805-808.
Morgan, G.W., Barohn, R.J., King, R.B., Klucznik, R.P., 1993. Nerve root enhancement with MRI in inflammatory demyelinating polyradiculoneuropathy. Neurology. 43, 618-620.
Pollard, J.D., 2002. Chronic inflammatory demyelinating polyneuropathy. Curr. Opin. Neurol. 15, 279-283.
Rentzos, M., Anyfanti, C., Kaponi, A., Pandis, D., Ioannou, M., Vassilopoulos, D., 2007. Chronic inflammatory demyelinating polyneuropathy: A 6-year retrospective clinical study of a hospital-based population. J. Clin. Neurosci. 14, 229-235.
Rotta, F.T., Sussman, A.T., Bradley, W.G., Ram Ayyar, D., Sharma, K.R., Shebert, R.T., 2000. The spectrum of chronic inflammatory demyelinating polyneuropathy. J. Neurol. Sci. 173, 129-139.
Schady, W., Goulding, P.J., Lecky, B.R., King, R.H., Smith, C.M., 1996. Massive nerve root enlargement in chronic inflammatory demyelinating polyneuropathy. J. Neurol. Neurosurg. Psychiatry. 61, 636-640. the lower extremities, increased tonus and brisk deep tendon reflexes. MRI may be helpful in the diagnosis of CIDP by excluding the compressive or structural lesions that may lead to polyradiculopathy and MRI may also show hypertrophy of nerve roots and inflammatory processes in CIDP (Crino et al., 1993; Morgan et al., 1993; Goldstein et al., 1996; Midroni and Dyck, 1996; Mizuno et al., 1998). Duggins et al (1999) demonstrated that all patients with spinal root and plexus hypertrophy had a relapsing–remitting course and a significantly longer duration of disease than those without hypertrophy.
Our patient also had at least five years duration of complaints and on MRI of the cervicothoracal and lumbosacral spine, the nerve roots were prominently enlarged leading the radiologist to suspect neurofibromatosis. Mizuno et al. (1998) also showed nerve damage and subsequent hypertrophic nerve development due to demyelination between relapses in patients not receiving treatment.
In cases with CIDP, though not always, abnormal contrasting and hypertrophy are observed in cauda equina and lumbar nerve roots. In the literature, a few CIDP cases with wellcircumscribed and contrasted lesions displaying expansion in cervicothoracic and lumbosacral foramina have been reported. In the differential diagnosis of nerve root hypertrophy, CIDP should be considered in the differental diagnosis beside neurofibromatosis, Guillian-Barre syndrome, neoplastic lesions, lymphoma, meningeal carcinomatosis, sarcoidosis, and CMV polyradiculopathy.