Differences between acute‐onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A‐CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Demographic characteristics, clinical manifestations, cerebrospinal‐fluid (CSF) findings, treatment and clinical evolution were analyzed. Nerve conduction studies were performed in all patients with at least 12 months follow‐up. A total of 91 patients were included (AIDP, n = 77; A‐CIDP, n = 14). The median age was 55.5 years in patients with A‐CIDP vs 43 years in AIDP (P = .07). The history of diabetes mellitus was more frequent in A‐CIDP (29% vs 8%, P = .04). No significant differences between groups were observed with respect to: human immunodeficiency virus (HIV) status, presence of auto‐immune disorder or oncologic disease. Cranial, motor and autonomic nerve involvement rates were similar in both groups. Patients in the A‐CIDP group showed higher frequency of proprioceptive disturbances (83% vs 28%; P < .001), sensory ataxia (46% vs 16%; P = .01), and the use of combined immunotherapy with corticoids (29% vs 3%; P = .005). There were no significant differences in CSF findings, intensive care unit (ICU) admission, or mortality rates. During the first 8 weeks both entities are practically indistinguishable. Alterations in proprioception could suggest A‐CIDP. Searching for markers that allow early differentiation could favor the onset of corticotherapy without delay.     

Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy

ObjectiveAim of this study was to develop and evaluate the applicability of an ultrasound score (Bochum ultrasound score – BUS) in distinguishing chronic (CIDP) from acute inflammatory demyelinating polyneuropathy (AIDP).Methods

• •Step 1: For the development of BUS 75 healthy-controls, 20 CIDP, 20 AIDP patients underwent US 4.55 ± 3.5 and 3.4 ± 2.91 years, respectively after onset. After comparing the distribution pattern and frequency of pathological US changes between the two study groups, we developed BUS, summarizing the cross sectional area (CSA) of: (1) the ulnar nerve in Guyons’ canal, (2) the ulnar nerve in upper-arm, (3) the radial nerve in spiral groove, (4) the sural nerve between the gastrocnemius muscle.
• •Step 2: The BUS underwent blinded evaluation in further 10 CIDP, 21 AIDP patients 3.8 ± 2.7 and 2.3 ± 1.5 years, respectively after onset.
• •Step 3: The BUS underwent blinded, prospective evaluation in 8 patients with acute/subacute polyradiculoneuropathy (5 CIDP, 3 AIDP) 2.6 ± 1.8 weeks after onset.

ResultsThe BUS showed a sensitivity of 90% and specificity of 90.4% (positive predictive value, PPV = 81.8%; negative predictive value, NPV = 95%) in distinguishing CIDP from AIDP, when they showed no differences in disease duration (p = 0.0551).In addition, the BUS distinguished subacute-CIDP from AIDP with a sensitivity of 80%, specificity of 100% (PPV = 100%, NPV = 75%).ConclusionThe BUS seems to allow a reliable distinction of CIDP from AIDP.SignificanceThe BUS may be helpful in distinguishing subacute-CIDP from AIDP.     

Acute‐onset chronic inflammatory demyelinating polyneuropathy: An electrodiagnostic study

Introduction: Acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) is an increasingly recognized CIDP subtype. Differentiating A‐CIDP from Guillain–Barré syndrome (GBS) is challenging but important, because there are different treatment outcomes. Methods: We report 3 patients with A‐CIDP who were initially diagnosed with severe GBS but were later confirmed to have CIDP based on their clinical course and electrodiagnostic (EDx) studies. We also report on the long‐term treatment of these patients and review the literature on EDx studies in this syndrome. Results: Three patients were initially diagnosed with GBS and responded to treatment. However, all 3 had arrest in improvement or deterioration during their rehabilitation phases. EDx studies showed prominent demyelinating changes many months after the initial presentation. All responded very well to immunotherapy. Conclusion: Although several features may suggest the diagnosis of A‐CIDP at initial presentation, close follow‐up of GBS patients during the recovery phase is also needed for accurate diagnosis. EDx studies may distinguish patients with A‐CIDP from GBS patients. Muscle Nerve 52 : 900–905, 2015     

Clinical heterogeneity in mild chronic inflammatory demyelinating polyneuropathy

We describe the clinical presentation, progression and electrodiagnostic features of three patients with a mild form of chronic inflammatory demyelinating polyneuropathy (CIDP). The unusually mild but also variable clinical picture was a cause of diagnostic uncertainty in all, but CIDP was eventually confirmed by extensive electrophysiological studies in each case, as well as by histology in one. Cerebrospinal fluid protein was raised in only one patient. Two patients were treated by intravenous immunoglobulins and both improved. Awareness of the existence of this relatively benign form of CIDP in its various presentations is essential as it can be functionally disabling, progress to more severe symptomatology, and as patients may benefit from immunomodulatory therapy.     

Sensory sparing patterns and the sensory ratio in acute inflammatory demyelinating polyneuropathy

The relative preservation (sparing) of sural sensory nerve action potentials (SNAPs) is a useful diagnostic finding in patients with acute inflammatory demyelinating polyneuropathy (AIDP). However, recording of sural SNAPs is not always technically feasible, especially in obese, edematous, or elderly individuals. Hence, we systematically evaluated the predictive values of the commonly employed SNAPs in the diagnosis of AIDP within 2 weeks from onset of symptoms. Sensory sparing patterns and sensory ratios of the sural, radial, median, and ulnar SNAPs of AIDP patients were included in a retrospective and blinded analysis, and compared to patients with diabetic polyneuropathy (DPN) and controls. Logistic regression models for the sural plus radial SNAPs/median plus ulnar SNAPs (sensory ratio) were constructed. A sural sparing pattern was present only in the AIDP group (34.4%, P < 0.001). A radial sparing pattern did not discriminate the AIDP from the DPN groups. The sural/radial sensory ratio was useful to ascertain DPN, but did not discriminate AIDP from controls. The sensory ratio was higher in AIDP compared to DPN and controls and was an independent predictor for AIDP. This study implies that the sensory ratio is a useful predictor for the diagnosis of AIDP and may substitute for sural sparing in technically difficult situations.     

Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy

We describe a patient with the previously unreported association of chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG). Immunosuppressive treatment with azathioprine and prednisone achieved clinical and electrophysiological remission of MG and improvement of CIDP. As ophthalmoplegia occurs infrequently in CIDP, the possibility of MG should be considered when this sign is present in a patient with CIDP. Since current therapy with corticosteroids, plasma exchange and other immunomodulating agents is effective against both diseases, their association may be undereported.     

The dropped head syndrome with chronic inflammatory demyelinating polyneuropathy

The dropped head syndrome occurs in a variety of neuromuscular disorders. We present a woman with chronic inflammatory demyelinating polyneuropathy who developed this syndrome, likely reflecting severe demyelination of nerves to cervical paraspinal muscles. © 1994 John Wiley & Sons, Inc.     

Subclinical electrophysiological alterations of phrenic nerve in chronic inflammatory demyelinating polyneuropathy

Alterations of the phrenic nerve (PN) and pulmonary function tests (PFTs) have been described in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). This study was aimed at assessing the relationship between PN and respiratory function in CIDP patients without clinical signs of respiratory failure. Bilateral PN and right median nerve conduction studies were carried out along with blood gas analysis and PFTs: maximal inspiratory pressure; maximal expiratory pressure; forced vital capacity. The amplitude of the compound muscle action potential of the PN was seen to be altered in 19/24 (79%) patients and latency in 22 (92%). Eighteen patients (75%) showed at least one abnormal PFTs or CO₂ partial pressure value. Electrophysiological alterations of the PN were observed in a high percentage of the CIDP patients studied. No statistically significant correlation was observed between PN and PFTs alterations.     

慢性炎性脱髓鞘性多发性神经病的治疗进展

慢性炎性脱髓鞘性多发性神经病（chronic inflammatory demyelinating polyradiculopathy,CIDP）是一种获得性的免疫介导的周围神经病.临床特征包括进展性或复发性的肢体无力、感觉缺失和腱反射消失等.     

Onset and course of chronic inflammatory demyelinating polyneuropathy

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.     

慢性炎症性脱髓鞘性多发性神经病临床及神经电生理研究

目的 分析慢性炎症性脱髓鞘性多发性神经病(CIDP)的临床及神经电生理表现.方法 选取2010-07-2012-07我院7例CIDP患者,对其临床资料进行回顾性研究,分析临床表现、脑脊液及神经电生理检测结果.结果 7例CIDP患者均有四肢或双下肢肌力下降,腱反射减弱或消失,脑脊液蛋白升高,神经电生理异常.出院后3例恢复较良好,另外4例出现2～4次复发.结论 CIDP的诊断应结合临床表现、脑脊液检查和神经电生理检查,应依据具体情况采用免疫球蛋白和(或)皮质激素治疗.     

Clinical manifestations of chronic inflammatory demyelinating polyneuropathy with anti-cardiolipin antibodies

OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune syndrome where certain autoantibodies define clinicopathologic subgroups. In the present study, serum anti-cardiolipin antibodies (aCL) were evaluated. MATERIALS AND METHODS: We investigated aCL in sera from 21 patients diagnosed with CIDP in our hospital between 1991 and 2001. The four CIDP patients with aCL (aCL+) were compared with 17 patients without aCL (aCL-). RESULTS: All aCL+ patients displayed sensory-motor polyneuropathy, with severity and distribution of weakness resembling those in aCL- patients. Anti-nuclear antibody titer of aCL+ patients were significantly higher than those in aCL- patients. None of aCL+ patients presented clinical manifestations of primary anti-phospholipid syndrome (APS), such as thromboses or recurrent abortion. Although the aCL+ patients were older and had more complications and more severe pathologic features than aCL- patients, they responded well to steroid pulse or intravenous immunoglobulin. CONCLUSION: The aCL in CIDP apparently differ from 'autoimmune' aCL in APS, instead being among the autoantibodies pathologically involved in CIDP subgroups.