Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement

Abstract

We report a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves. Investigations showed a demyelinating polyradiculoneuropathy with axonal degeneration and depletion of postganglionic noradrenergic fibers in the rectal mucosa. Intravenous immunoglobulin and corticosteroid administration were effective in alleviating symptoms and improving electrophysiological abnormalities. This neuropathy may be a novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), in which autoimmunoreactivity is directed not only against myelin but also against axon- or ganglion-composing protein. Autonomic nerve involvement does not exclude a diagnosis of CIDP.

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…Dysautonomia in CIDP has been attributed to the involvement of postganglionic unmyelinated autonomic fibers. Autoimmunity in CIDP is not only directed against myelin but also against axon and autonomic ganglia [7]. In our study, 2 of the 3 patients with dysautonomia had diabetes mellitus suggesting the role of diabetes in producing dysautonomia in CIDP.…”

“…In the literature some studies have demonstrated minimal subclinical autonomic dysfunction [20, 21]. In the European Federation of Neurological Societies/Peripheral Nerve Society CIDP Guideline dysautonomia was not mentioned in atypical CIDP but we considered it atypical in the light of another report [7]. Dysautonomia in CIDP has been attributed to the involvement of postganglionic unmyelinated autonomic fibers.…”

“…In a retrospective review of 67 patients with CIDP, atypical features included predominantly distal involvement in 17%, predominantly cranial nerve involvement in 5% and amyotrophic-lateral-sclerosis (ALS)-like picture in 1.5% of patients [4]. There are also isolated case reports describing atypical findings in CIDP such as respiratory failure [6], dysautonomia [7], pure sensory involvement [4] and lumbar-canal-stenosis-like syndrome [8]. There is a paucity of reports comparing the clinical and electrodiagnostic features of typical and atypical CIDP patients.…”

Autonomic Involvement in Subacute and Chronic Immune-Mediated Neuropathies

“…Yamamoto and colleagues reported a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves suggesting that autonomic nerve involvement does not exclude a diagnosis of CIDP [61]. Severe autonomic dysfunction with abnormal autonomic tests function has been reported in a chronic axonal subtype of CIDP [62].…”

Chronic inflammatory demyelinating polyneuropathy – update on pathogenesis, diagnostic criteria and therapy

“…In cases with inflammation mainly affecting the dorsal roots sensory nerve-conduction studies may be normal while somatosensory-evoked potentials, elevated cerebrospinal fluid protein content and enlargement of lumbar nerve roots on MRI were the characteristic features of the syndrome termed chronic immune sensory polyradiculopathy [31 ]. Mild autonomic dysfunction is not uncommon in CIDP [33] and, in rare cases, severe autonomic disturbance may even be the presenting feature [34]. A comprehensive comparison of the different forms of sensory neuropathy including paraneoplastic, dysimmune, autoimmune, toxic and hereditary sensory neuropathy (=Friedreich's ataxia) has been provided by Kuntzer and coworkers [32].…”