Subacute Idiopathic Demyelinating Polyradiculoneuropathy

Hughes, Richard; Sanders, E. A. C. M.; Hall, Susan; Atkinson, Penny F; Colchester, Alan C. F.; Payan, Peter

doi:10.1001/archneur.1992.00530300044009

Cited by 106 publications

(49 citation statements)

References 11 publications

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“…Acute onset can be followed by a chronic course, 12,15 and some patients have onset between 4 and 8 weeks. 4,8 Among our patients, 7% had an acute onset with progression over less than 4 weeks, and 18% had a subacute onset with progression for between 4 and 8 weeks. Acute onset was followed by a relapsing course in all patients.…”

Section: Discussionmentioning

confidence: 75%

“…The term "subacute inflammatory demyelinating neuropathy" has been suggested for these cases. 4,8 Patients with proximal weakness were more disabled at presentation, but had a better prognosis than patients with symptoms confined to distal regions (DADS). Among the patients with proximal weakness, 55% were in remission at follow-up, compared with none of the DADS patients.…”

Section: Discussionmentioning

confidence: 99%

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Onset and course of chronic inflammatory demyelinating polyneuropathy

2005

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is clinically heterogeneous. Our purpose was to determine whether initial progression time, clinical features, and distribution of nerve conduction slowing at presentation correlate with clinical course and prognosis. We examined how findings at presentation related to clinical course during an average follow-up time of 4.0 (range 1.0-9.0) years in 44 patients with CIDP. We calculated terminal latency index (TLI), a measure of differential slowing in distal relative to more proximal nerve segments. Patients with acute or subacute onset (progression over less than 8 weeks) had a higher remission rate (P = 0.012) than patients with chronic onset (progression over more than 8 weeks). Patients with proximal weakness had a higher remission rate than patients with the distal phenotype (P < 0.001). All 5 patients with a relapsing course had subacute onset. They had lower TLIs, suggesting a more distal pattern of demyelination, than patients with a monophasic or chronic course. In conclusion, subacute onset and presence of proximal weakness are good prognostic signs that correlate with a high rate of recovery to normal in CIDP. Distal accentuation of conduction slowing at presentation correlates with subacute onset and a relapsing course.

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Section: Discussionmentioning

confidence: 75%

Section: Discussionmentioning

confidence: 99%

Onset and course of chronic inflammatory demyelinating polyneuropathy

2005

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“…Mori et al [8] have described such patients who, after initial improvement with immunoglobulins, relapsed into a chronic phase and subsequently improved only after steroids were initiated [8] . Hughes et al [9] have described a subgroup of steroid-responsive patients who presented with a mild disease evolving over 4-8 weeks, whom they labeled as 'subacute inflammatory demyelinating polyneuropathy'. Our patient had all the features of GBS and did not respond to IVIG or to plasmapheresis, but instead responded dramatically to corticosteroids, suggesting a diagnosis of CIDP.…”

Section: Discussionmentioning

confidence: 99%

Corticosteroids Can Help Distinguish between Guillain-Barré Syndrome and First Attack of Chronic Inflammatory Demyelinating Neuropathy

Alexander

Al-Shubaili²,

Santhamoorthy³

et al. 2008

Med Princ Pract

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Objective: To report a case of acquired demyelinating neuropathy that failed to improve upon treatment with intravenous immunoglobulins and plasmapheresis but responded dramatically to corticosteroids, illustrating the occasional difficulty in distinguishing Guillain-Barré syndrome (GBS) from a first attack of chronic inflammatory demyelinating polyneuropathy (CIDP). Clinical Presentation and Intervention: A 25-year-old previously healthy man was admitted with a 5-day history of ascending areflexic paralysis of all 4 limbs and diagnosed with GBS. On admission, he was administered intravenous immunoglobulins at a dosage of 400 mg/kg/day for 5 days yet continued to worsen. He became quadriparetic by the second week. As there had been no improvement, he was plasmapheresed with 7 sessions of plasma exchange, 50 ml/kg of plasma at each session, including appropriate replacement fluid. After failing to improve within 8 weeks, he was started on intravenous methylprednisolone and a dramatic improvement was observed by the 5th day. He continued to get better on oral prednisolone, was ambulatory with support 4 weeks later and could walk without support on follow-up. Conclusion: This case illustrates that there is a subset of patients initially diagnosed with GBS who do not respond to immunoglobulins or plasmapheresis but do specifically well on steroids. Hence treatment with prednisolone should not be delayed in selective cases of GBS as it may actually be a first episode of CIDP.

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“…1 It is likely that CIDP is a heterogeneous disorder, 2 having a wide range of clinical expression ranging from subacute 3 to chronic progression, and a monophasic to a relapsing course. 2 4 5 In addition, it can include predominantly proximal to distal weakness, 6 and may involve neuropathies ranging from symmetric polyneuropathy to mononeuropathy multiplex.…”

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confidence: 99%

Footdrop after peroneal nerve lesion

Bendszus

2002

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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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. Results: Patients were classified as having a distal (n=10), intermediate (n=13), or diffuse (n=15) pattern, or were unclassified (n=4). Patients with the distal or diffuse pattern had common clinical features such as subacute onset, symmetric symptoms, and weakness involving proximal as well as distal muscles. Patients with the distal pattern had a good response to treatment and a monophasic remitting course, but the diffuse pattern was associated with a treatment dependent relapsing course, reflecting longer disease activity. The serum TNF-α concentrations increased only in the "diffuse" subgroup of patients, and this might be associated with breakdown of the blood-nerve barrier and therefore, involvement of the intermediate segments. The intermediate pattern was characterised by a chronic course, asymmetric symptoms, less severe disability, and refractoriness to treatments. Conclusions: CIDP consists of subtypes with varying predilections for lesions along the course of the nerve. The distribution patterns of conduction abnormalities may be useful in the prediction of outcome of patients with CIDP.

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Subacute Idiopathic Demyelinating Polyradiculoneuropathy

Cited by 106 publications

References 11 publications

Onset and course of chronic inflammatory demyelinating polyneuropathy

Onset and course of chronic inflammatory demyelinating polyneuropathy

Corticosteroids Can Help Distinguish between Guillain-Barré Syndrome and First Attack of Chronic Inflammatory Demyelinating Neuropathy

Footdrop after peroneal nerve lesion

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