Ataxic Form of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Features and Pathological Study of the Sural Nerves

Abstract

We investigated clinical and pathological features of the sural nerves of 5 patients with the ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and compared these features with those of chronic ataxic neuropathies due to other causes. The CIDP patients presented with slowly progressive ataxia with deep sensory impairment. The durations of the symptoms from onset were relatively short in CIDP (4–8 months) and cancer (3 and 10 months), but long in chronic idiopathic ataxic neuropathy (24–260 months). Corticosteroid therapy elicited a good response in all the patients with CIDP, but a poor response in the patients with other ataxic neuropathies. Sural nerve biopsy of CIDP patients showed a slight or moderate loss of myelinated fiber. This report suggests that ataxic form of CIDP is a steroid-responsive ataxic neuropathy, and large myelinated fibers of the sural nerves in ataxic form of CIDP were better preserved than those in nerves with other chronic ataxic neuropathies.

Chronic sensorimotor polyradiculopathy with antibodies to P2: An electrophysiological and immunoproteomic analysis

“…The clinical picture of proximal weakness, in addition to severe sensory ataxia, resembles more a form of CIDP. Thus, pure sensory forms of CIDP,24, 25 classic CIDP,2, 32 or even the recently described chronic immune sensory polyradiculopathy,30 should be considered in the differential diagnosis.…”

Clinical assessment of the sensory ataxias; diagnostic algorithm with illustrative cases

“…Weakness may be mild or absent, by contrast with classical CIDP. Nerve conduction shows motor abnormalities typical of CIDP, and nerve biopsy shows demyelination and remyelination 12. It often responds to corticosteroids and/or intravenous immunoglobulins, and its management is along lines similar to typical CIDP.…”

“…Chronic sensory ataxia can result from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP),12 paraproteinaemia,13 14 diabetic neuropathy,15 coeliac disease,16 vitamin E deficiency17 and mitochondrial dysfunction 40. Chronic sensory ataxia can uncommonly be associated with anti-GD1b IgM antibody; it is worthwhile measuring antiganglioside antibodies in all patients with otherwise idiopathic sensory ataxic neuropathies, as intravenous immunoglobulin treatment is often effective 41…”

“…Nerve conduction studies may show either conduction slowed to the demyelinating range or partial conduction block 39. The uncommon sensory ataxic form manifests as prominent numbness in the extremities, ataxia, areflexia and impaired vibration and/or joint position sense 12. Weakness may be mild or absent, by contrast with classical CIDP.…”

Diagnostic criteria of chronic inflammatory demyelinating polyneuropathy in diabetes mellitus

“…If sensory symptoms predominate, deep sensation is more affected than superficial sensation -The sensory involvement in diabetic polyneuropathy is generally associated with damage to small, unmyelinated fibers leading to numbness, tingling sensations, and neuropathic pain in a stocking and glove distribution (4) while proprioceptive loss due to damage of the large myelinated fibers is much more indicative of CIDP (27,28,32).…”