"Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy.

Abstract

The clinical electrophysiological and histological features of 10 cases of "chronic sensory demyelinating neuropathy" (CSDN) are reported. This entity is characterised by: 1) subacute or chronic progression; 2) pure sensory neuropathy; 3) high spinal fluid protein in the majority of cases; 4) electrophysiological evidence of demyelination affecting motor as well as sensory nerve fibres; 5) demyelination on sural nerve biopsy and 6) good response to immunotherapy in progressive phase. It is believed that this entity represents chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as pure sensory neuropathy. (7 Neurol Neurosurg Psychiatry 1992;55:677-680) the distal value (fig 1). Abnormal temporal dispersion was considered to be present when the CMAP shape was abnormal with multiple phases (more than four) and total duration longer than three standard deviations above the normal mean value (fig 1).3 In five patients, the plantar nerves were studied using the nearnerve needle sensory nerve conduction technique.4 Needle EMG examination was carried out in all cases.Sural nerve biopsy was performed in eight cases. Frozen sections were stained with modified trichrome, cresyl-fast violet, hematoxylin and eosin, and crystal violet stains. Paraffin sections were stained with hematoxylin and eosin and congo-red stains. Semithin EM sections in three cases were stained with toluidine blue. Teased nerve preparations were studied in four cases.To compare the degree of sensory impair-

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

“…Only a few reported cases of CIDP with ataxic neuropathy due to deep sensory disturbance responded well to steroid or immunotherapy. Oh et al [26]reported 10 patients with a sensory variant of CIDP ‘chronic sensory demyelinating neuropathy’ [26]. One of the 10 patients showed ataxic symmetrical polyneuropathy with a 2-year history and a favorable response to plasmapheresis followed by treatment with azathioprine and prednisolone.…”

Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: A long-term follow-up study

“…In 5-6% of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) only sensory symptoms are present, despite electrophysiological evidence of demyelination in motor nerve fibers [6,11,15]. Patients with CIDP and only sensory symptoms on clinical examination may fulfill the research criteria for CIDP as well [1,7].…”

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…Typical CIDP is characterized by chronically progressive, stepwise or recurrent symmetric proximal and distal weakness and sensory dysfunction associated with reduced or absent tendon reflexes in all 4 limbs with or without cranial nerve involvement developing over 2 months [1]. Atypical features in CIDP have been reported from time to time and can raise diagnostic difficulties [2,3,4,5]. 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].…”