מאמר- הגדרה לתסמונת תחושתית חדשה

Author(s): Shahar Shelly, Kamal ShouAman, Pritikanta Paul, JaNean Engelstad, Kimberly K. Amrami, Robert J. Spinner, Divyanshu Dubey, Rocio Vazquez Do Campo, Peter J. Dyck, Christopher J. Klein, P. James B. DyckNeurology Apr 2021, 96 (16) e2078-e2089; DOI: 10.1212/WNL.0000000000011792

Objective Sensory loss with normal nerve conduction studies (NCS) from focal sensory root inflammatory demyelination is characteristic of chronic immune sensory polyradiculopathy (CISP). However, nonpure cases involving motor and distal sensory nerves exist (CISP-plus). We hypothesize that CISP-plus and CISP are fundamentally part of the same syndrome through comparison of clinical, neurophysiologic, and pathologic features.

Methods CISP-plus (primary dorsal root with lesser motor and sensory nerve involvement) and CISP cases were retrospectively analyzed (1986–2019).

Results We identified 44 CISP-plus and 28 CISP cases (n = 72) with 86% (38/44) of patients with CISP-plus and 79% (22/28) of patients with CISP experiencing imbalance. On examination, large fiber sensory loss was present in 98% (43/44) of patients with CISP-plus and 96% (27/28) of patients with CISP. Gait ataxia was evident in 93% (41/44) of patients with CISP-plus and 79% (22/28) of patients with CISP. Mild distal weakness was common in CISP-plus (75%, 33/44). NCS showed mild abnormalities in all patients with CISP-plus and were normal (by definition) in all patients with CISP. Elevated CSF protein, slowing of somatosensory evoked potentials, and MRI root enhancement occurred in most CISP-plus and CISP cases. Eleven CISP-plus nerve biopsies showed loss of large myelinated fibers and onion-bulb formations, most prominent in rootlet biopsies. Immunotherapy resulted in marked improvement of gait ataxia in 84% (27/32) of patients with CISP-plus and 93% (13/14) of patients with CISP with return to normal neurologic examination in half (25/46).

Conclusion The recognition of CISP-plus expands the spectrum of CIDP by combining CISP-plus (predominant sensory polyradiculopathy with mild motor and sensory nerve involvement) with pure CISP (focal sensory polyradiculopathy) together as proximal sensory CIDP.

Glossary

CI=confidence interval; 

CIDP= chronic inflammatory demyelinating polyradiculoneuropathy; 

CISMP= chronic immune sensorimotor polyradiculopathy; 

CISP= chronic immune sensory polyradiculopathy; 

EFNS= European Federation of Neurological Societies; 

IVIg=IV immunoglobulin; 

IVMP=IV methylprednisolone; 

MF=myelinated fibers; 

NCS=nerve conduction studies; 

NS=nonsignificant; 

OR=odds ratio; 

PLEX=plasmapheresis; 

PNS=Peripheral Nerve Society; 

QST=quantitative sensory testing; 

SSEP=somatosensory evoked potential

Although chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) usually presents with weakness,sensory-predominant forms have been described, including the recently recognized paranodal neuropathies (contactin-1–associated CIDP). In 2004, our group described a restricted form of sensory CIDP, chronic immune sensory polyradiculopathy (CISP), characterized by selective involvement of sensory nerve roots.⁷ A similar acute form of immune-mediated pure sensory polyradiculopathy has been described recently. CISP presents with sensory loss, gait ataxia, falls, large fiber sensory deficits, reduced reflexes, and preserved muscle strength. Because the pathology is confined to sensory roots (preganglionic) and motor nerves are spared, nerve conduction studies and EMG (NCS/EMG) are normal. The presence of slowed somatosensory evoked potentials (SSEP), CSF protein, and enlarged dorsal roots on MRI support the diagnosis of CISP. Dorsal lumbar rootlet biopsies confirm inflammatory demyelination. CISP has been recognized as atypical CIDP and is included in the European Federation of Neurologic Societies/Peripheral Nerve Society (EFNS/PNS) CIDP criteria. Since CISP's description, we have noted patients with a similar clinical syndrome but with mild distal weakness and mild abnormalities on NCS, implying the pathology is not restricted to sensory nerve roots (figure A, right panel). We call this nonpure form CISP-plus.

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