a pure sensory neuropathy . demyelinating polyneuropathy presenting as neuropathy " : chronic inflammatory " Chronic sensory demyelinating (original) (raw)
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Atypical pure sensory forms of chronic inflammatory demyelinating polyneuropathies
2019
Background: There are still not enough data on clinical and laboratory peculiarities of atypical chronic inflammatory demyelinating polyneuropathy (CIDP), ranging from only sensitive symptoms without weakness to asymmetric motor deficit. Recent epidemiological data do not clearly elucidate the percentage of cases with atypical CIDP from total CIDP types. Nerves conduction study, the gold standard in diagnosing demyelinating polyneuropathies has low sensibility for atypical forms of CIDP. The purpose of this study was determining the criteria for clinical and laboratory diagnosis of atypical sensory CIDP. Material and methods: Two groups of study were identified: 30 patients with typical CIDP and 30 patients with atypical CIDP. All patients underwent nerves conduction studies, blood was drawn for biochemical tests, also electrophoresis and serum protein immunofixation were done. Fibular nerve biopsy was performed in 9 patients. Overall Neuropathy Limitation Scale (ONLS) questionnaire was used for the assessment of functional disability. Results: Nerves conduction studies in cases with sensory CIDP show normal motor conduction velocity in 10 cases, and diminished only in 4 cases. Total ONLS in patients with sensory CIDP is equal to 1.85 ± 0.21 points compared to total 4.17 ± 0.240 points in patients with typical CIDP (p <0.001). Conclusions: Nerves conduction study is not a gold standard for diagnosis atypical sensory CIDP. According to functional scores results, sensory CIDP is less disabling compared with typical CIDP.
Sensory chronic inflammatory demyelinating polyneuropathy: an under-recognized entity?
Muscle & nerve, 2013
Sensory chronic inflammatory demyelinating polyneuropathy (CIDP) can be difficult to diagnose. We report 22 patients with chronic sensory polyneuropathy with ≥1 clinical sign atypical for chronic idiopathic axonal polyneuropathy (CIAP) but no electrodiagnostic criteria for CIDP. Clinical signs atypical for CIAP were: sensory ataxia (59%), generalized areflexia (36%), cranial nerve involvement (32%), rapid upper limb involvement (40%), and age at onset ≤55 years (50%). Additional features were: normal sensory nerve action potentials (36%), abnormal radial/normal sural pattern (23%), abnormal somatosensory evoked potentials (SSEPs) (100%), elevated cerebrospinal fluid (CSF) protein (73%), and demyelinating features in 5/7 nerve biopsies. Over 90% of patients responded to immunotherapy. We conclude that all patients had sensory CIDP. Sensory CIDP patients can be misdiagnosed as having CIAP. If atypical clinical/electrophysiologic features are present, we recommend performing SSEPs and ...
Sensory pathophysiology in chronic acquired demyelinating neuropathy
Brain, 1996
Pathophysiological changes in sensory fibres in chronic acquired demyelinating neuropathy (CADP) are poorly understood, and it is not known to what extent sensory loss may be due to axonal loss or to conduction block. Motor and sensory nerve conduction were studied in 18 patients with CADP to delineate abnormalities in the compound sensory action potential (CSAP) recorded proximally along the limb. To distinguish small CSAPs from noise, near-nerve needle electrodes and electronic averaging were used. In all, 58 motor and 78 sensory nerves in the upper and lower limbs were studied, and in 29 nerves, motor and sensory conduction was compared over the same proximal and distal segments of the upper limbs. The proximal/distal amplitude ratio (P/D ratio) of the compound muscle action potential (CMAP) was reduced in 76% of the nerves compared with only 21% of the CSAPs. The amplitudes of CMAPs evoked and of CSAPs recorded distally were reduced to the same extent. The prolongation of the distal motor latency (DML) was linearly related to the reduction in amplitude of the CMAP whereas reduction of the distal sensory conduction velocity (SCVj) mainly occurred if the amplitude of the CSAP was reduced more than 70%. The proximal motor nerve conduction velocity (MCV p) was reduced by 40-50%, twice as much as the reduction in distal MCV (MCV d) (calculated from the reciprocal DML), and related to the reduction in the P/D ratio of the CMAP. The proximal SCV (SCV p) decreased-20%, similar to the reduction in SCVj, and out of proportion to the marked reduction of the MCVp. The results suggest different pathophysiological changes in sensory and motor fibres in CADP. Thus, nerve fibre loss could account for most of the abnormal parameters in sensory conduction, whereas demyelination was the dominating cause of motor nerve dysfunction.
Muscle & Nerve, 2003
The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) relies primarily on clinical and electrophysiologic examination, but the nerve biopsy findings may be supportive, especially in atypical cases. In order to define the usefulness of nerve biopsy in this disease, we retrospectively studied 44 consecutive patients whom we classified as having CIDP on pathological grounds. We found that 8 of these 44 patients had pathological findings indicative of CIDP but did not meet any of the usually accepted electrophysiological criteria for its diagnosis. Among these eight patients, five responded favorably to conventional therapy. All of these eight patients had an electrophysiological pattern of generalized axonopathy with additional subtle findings suggestive of demyelination that prompted us to perform a nerve biopsy. Our data suggest that a significant number of patients with unrecognized CIDP are erroneously classified as having chronic idiopathic axonal polyneuropathy. CIDP should be suspected if the electrophysiological examination displays subtle abnormalities suggestive of demyelination, even in the presence of a prominent axonal pattern. Nerve biopsy in these patients may reveal abnormalities suggestive of CIDP and guide therapeutic options.
Journal of Neurology, Neurosurgery & Psychiatry, 2002
Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide clinical range, and is characterised by multifocal demyelination that can involve the distal nerve terminals, intermediate nerve segments, and nerve roots. Objective: To investigate whether the distribution patterns of demyelination along the course of the nerve correlate with clinical profiles in patients with CIDP. Methods: Motor nerve conduction studies were carried out on 42 consecutive patients. According to the physiological criteria for demyelination, the presence of a demyelinative lesion was determined in the distal nerve segments (distal pattern) or intermediate nerve segments (intermediate pattern), or in both (diffuse pattern). The serum concentration of tumour necrosis factor (TNF)-α was measured by immunoassay.
Muscle & Nerve, 2017
Introduction: We report here the diagnostic value of the near-nerve needle sensory nerve conduction study (NNN-SNCS) in sensory inflammatory demyelinating polyneuropathy (IDP) in which the routine nerve conduction study was normal or nondiagnostic. Methods: The NNN-SNCS was performed to identify demyelination in the plantar nerves in 14 patients and in the median or ulnar nerve in two patients with sensory IDP. Results: In 16 patients with sensory IDP, routine NCSs were either normal or non-diagnostic for demyelination. Demyelination was identified by NNN-SNCS by dispersion and/or slow nerve conduction velocity (NCV) below the demyelination marker. Immunotherapy was initiated in 11 patients, 10 of whom improved or remained stable. Discussion: NNN-SNCS played an essential role in identifying demyelinaton in 16 patients with sensory IDP, leading to proper treatment.
Diagnostic value of sural nerve demyelination in chronic inflammatory demyelinating polyneuropathy
Brain, 2001
The objective of the study was to determine the diagnostic value of features of demyelination and inflammation in sural nerve biopsy specimens of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The features of (i) demyelination, (ii) axonal de-and regeneration and (iii) inflammation were investigated by measuring the number of onion bulbs, g ratio (axon diameter/total nerve fibre diameter), myelinated nerve fibre density, number of clusters and endoneurial area in 21 patients with CIDP, as well as in 13 patients with chronic idiopathic axonal polyneuropathy (CIAP) and six autopsy controls. In addition, teased fibres were classified and lengths of internodes measured. We found no difference in demyelinating features between patients with CIDP and CIAP, as the percentage of fibres with segmental de-and remyelination and the number of onion bulbs Abbreviations: CIAP ϭ chronic idiopathic axonal polyneuropathy; CIDP ϭ chronic inflammatory demyelinating polyneuropathy