Mandibular nerve involvement in diabetic polyneuropathy and chronic inflammatory demyelinating polyneuropathy

Sensory complaints in the area of the mandible and mouth often escape notice or remain undiagnosed. Using electromyographic recording of the trigeminal reflexes and motor responses, we sought trigeminal dysfunction in 50 patients with peripheral neuropathy, and tried to gain pathophysiological information on the mechanisms provoking trigeminal damage. Trigeminal reflex recordings (early and late blink reflex after supraorbital stimulation, early and late masseter inhibitory reflex after mental stimulation, and jaw jerk) disclosed abnormalities caused by sensory trigeminal neuropathy in 8 out of 15 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 13 out of 23 patients with severe diabetic polyneuropathy, and in none of 12 patients with mild diabetic polyneuropathy. Six patients had abnormal motor responses in facial or masseter muscles. The response affected most frequently was the masseter early inhibitory reflex (also called first silent period, SP1) after mental nerve stimulation, its latency being strongly delayed. We found these long delays not only in patients with CIDP, but also in diabetic patients with severe polyneuropathy. We conclude that peripheral polyneuropathies often cause subclinical damage to the trigeminal nerve, especially to its mandibular branch. We believe that the nerve fibers running along the alveolar–mandibular pathway are more exposed to damage because of their cramped anatomical route in the mandibular canal and below the internal pterygoid muscle and fascia. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1673–1679, 1998     

Electrophysiological entrapment syndromes in chronic inflammatory demyelinating polyneuropathy

We do not know if peripheral nerves are more susceptible to entrapment syndromes in chronic inflammatory demyelinating polyneuropathy (CIDP). We studied 31 prospectively recruited patients with CIDP. We determined whether entrapment zones were more frequently affected by demyelination than adjacent segments. The median, ulnar, and fibular nerves were studied at the wrist, elbow, and fibular head bilaterally. Motor conduction velocity and motor conduction block were evaluated at entrapment sites and compared with contiguous segments. Demyelination was significantly more frequent for ulnar and fibular nerves away from entrapment sites. No significant difference was observed for median nerves. CIDP is not associated with increased frequency of demyelination at entrapment sites. The presence of diffuse entrapment neuropathies at compression sites does not favor a diagnosis of CIDP. Although electrophysiological study of entrapment sites is not diagnostically useful in CIDP, it may help distinguish it from other neuropathies and confirm clinically relevant, surgically treatable compressions.     

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.     

A prospective evaluation of phrenic nerve conduction in multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy

The purpose of the study was to evaluate electrophysiologically phrenic nerve involvement in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). The response latencies following phrenic nerve stimulation were increased in 11 of 14 (80%) patients in the CIDP group but in only 1 of 14 (8%) patients in the MMN group. The mean diaphragmatic compound muscle action potential (CMAP) was significantly lower in amplitude in the CIDP group compared to the MMN group and to a control group of 8 subjects (P < 0.001). There were no significant differences between the MMN and control groups. Only the reduction in CMAP amplitude correlated with the presence of restrictive lung function. Phrenic nerve conduction measurement should be performed more systematically, especially in CIDP and, when diaphragmatic CMAPs are reduced in amplitude, pulmonary function tests should be performed to look for a restrictive lung syndrome.     

Clinical and electrophysiological parameters distinguishing acute‐onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy

Up to 16% of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely. We performed a retrospective chart review on 30 acute inflammatory demyelinating polyneuropathy (AIDP) and 15 acute‐onset CIDP (A‐CIDP) patients looking for any clinical or electrophysiological parameters that might differentiate AIDP from acutely presenting CIDP. A‐CIDP patients were significantly more likely to have prominent sensory signs. They were significantly less likely to have autonomic nervous system involvement, facial weakness, a preceding infectious illness, or need for mechanical ventilation. With regard to electrophysiological features, neither sural‐sparing pattern, sensory ratio >1, nor the presence of A‐waves was different between the two groups. This study suggests that patients presenting acutely with a demyelinating polyneuropathy and the aforementioned clinical features should be closely monitored as they may be more likely to have CIDP at follow‐up. Muscle Nerve, 2010     

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.     

Nerve root hypertrophy in chronic inflammatory demyelinating polyneuropathy

A patient with chronic inflammatory demyelinating polyneuropathy (ClDP) and centrel demyelinating disease is desoribed in whom striking nodular filling defects on multiple lumbar–sacral nerve roots, mimicking neurofibromata, were observed at myelography and magnetic resonance imaging. We suggest that these lesions are secondary to recurrent segmental demyelination and remyelination and that the differential diagnosis of this radiological feature should include CIDP. © 1994 John Wiley & Sons, Inc.     

Pathological findings in a patient with ventilatory failure and chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) may cause significant disability, but severe respiratory complications are uncommon. We describe the case of a 49-year-old man with clinical features of CIDP for 5 years who died of respiratory failure. Post-mortem findings of denervation in diaphragm muscle and axonal loss in phrenic nerve are presented. Severe ventilatory failure may occur in CIDP when neuropathy affects the respiratory muscles. Attention to early clinical features of respiratory insufficiency may facilitate the prevention of more severe features of respiratory failure.     

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.     

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.     

儿童慢性炎症性脱鞘性多神经病的临床和病理改变特点

目的探讨儿童慢性炎症性脱鞘性多神经病(chronic inflammatory demyelinating polyneuropathy,CI-DP)的临床及病理改变特点。方法根据欧洲神经肌肉病中心修订的儿童CIDP诊断标准诊断的10例17岁以下患者,收集其临床资料,进行周围神经电生理以及腓肠神经的病理检查。结果所有患者主要表现为肢体无力,分别有4例和3例出现四肢感觉减退和颅神经损害。9例有脑脊液蛋白细胞分离现象。10例均出现运动或感觉神经传导速度减慢及远端潜伏期延长,9例患者的动作电位波幅降低。所有患者的有髓神经纤维出现轻-重度减少,其中3例患者的纤维脱失程度在不同束间存在差异,6例患者以脱髓鞘为主;3例以轴索损害为主。1例患者仅出现轻微改变。9例患者存在炎细胞浸润。结论儿童CIDP以肢体无力为主。部分患者以轴索损害为主,神经纤维脱失程度可以存在束间差异。     

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.