Introduction Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination. Methods We report a case series of 9 patients with CISMP, identified from 2 tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed. Results Patients presented with sensorimotor deficits and hypo-/areflexia, predominantly involving lower extremities. Three had cranial nerve involvement. Electrodiagnostic findings in all patients localized to roots proximal to dorsal root ganglia, without evidence of peripheral nerve demyelination. Cerebrospinal fluid examination revealed an albuminocytologic association. Eight patients exhibited gadolinium enhancement and thickening of multiple spinal nerve roots and/or cranial nerves. All patients demonstrated good responses to immunotherapies. Discussion CISMP is similar to CIDP in many aspects, but lacks typical electrodiagnostic findings of peripheral nerve demyelination. It is important to recognize this unusual and treatable entity. Muscle Nerve 59:658-664, 2019
机构:
Univ Tokyo, Dept Neurol, Bunkyo Ku, Tokyo 1138655, JapanNatl Def Med Coll, Dept Neurol Antiaging & Vasc Med, 3-2 Namiki, Tokorozawa, Saitama 3598513, Japan
机构:
Grant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Khadilkar, Satish
Patel, Bhagyadhan
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Grant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Patel, Bhagyadhan
Mansukhani, Khushnuma A.
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Bombay Hosp & Med Res Ctr, Dept Neurophysiol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Mansukhani, Khushnuma A.
Jaggi, Sunila
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Bombay Hosp & Med Res Ctr, Dept Radiol, Bombay, Maharashtra, India
Med Res Ctr, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
机构:
Univ Tokyo, Dept Neurol, Bunkyo Ku, Tokyo 1138655, JapanNatl Def Med Coll, Dept Neurol Antiaging & Vasc Med, 3-2 Namiki, Tokorozawa, Saitama 3598513, Japan
机构:
Grant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Khadilkar, Satish
Patel, Bhagyadhan
论文数: 0引用数: 0
h-index: 0
机构:
Grant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Patel, Bhagyadhan
Mansukhani, Khushnuma A.
论文数: 0引用数: 0
h-index: 0
机构:
Bombay Hosp & Med Res Ctr, Dept Neurophysiol, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India
Mansukhani, Khushnuma A.
Jaggi, Sunila
论文数: 0引用数: 0
h-index: 0
机构:
Bombay Hosp & Med Res Ctr, Dept Radiol, Bombay, Maharashtra, India
Med Res Ctr, Bombay, Maharashtra, IndiaGrant Med Coll & Sir JJ Grp Hosp, Dept Neurol, Bombay, Maharashtra, India