Chronic Inflammatory Polyneuropathy Research Articles

Differentiating recurrent Guillain-Barre syndrome and acute-onset chronic inflammatory polyneuropathy: literature review.

Guillain-Barre syndrome (GBS) is an acute-onset immune-mediated polyneuropathy characterized by ascending symmetrical muscle weakness, diminished reflexes, and sensory symptoms. While GBS typically follows a monophasic course, some patients experience treatment-related fluctuations or recurrences, posing diagnostic challenges in distinguishing GBS from acute-onset chronic inflammatory polyneuropathy (A-CIDP). A-CIDP, may present acutely, simulating GBS, with a nadir in less than 8weeks, subsequently evolving into a chronic or relapsing course. The distinction between recurrent GBS and A-CIDP is crucial, as A-CIDP necessitates long-term immunosuppression. A PubMed search was conducted using the search terms 'recurrent Guillain Barre syndrome' and 'acute onset CIDP' focusing on studies in theEnglish language, published between January 1, 2004 and April 30, 2023. Overlapping clinical features, particularly in the early stages, complicate differentiation between recurrent GBS and CIDP. Electrophysiological studies, ultrasonography, and immunological markers have been explored for discrimination; however, definitive criteria for differentiation remain elusive. Recent follow-up studies have further blurred the boundaries between recurrent GBS and A-CIDP, suggesting the persistence of underlying immune processes even in GBS patients without clinical deterioration. This emphasizes the necessity of reevaluating diagnostic criteria and treatment strategies. In conclusion, distinguishing recurrent GBS from A-CIDP remains an ongoing challenge. Existing evidence questions the categorization of recurrent GBS as a distinct entity, challenging its very existence. Continued research is necessary to refine diagnostic criteria and deepen our understanding of these conditions, ultimately advancing patient care. This review delves into the intricacies of recurrent GBS and A-CIDP differentiation and highlights the need for a reevaluation of therecurrent GBS concept.

The psychometric properties of the Six-Spot Step Test – a systematic review using the COSMIN guidelines

Objective Accurate and reliable balance measures are important for prescribing fall prevention treatments and monitoring their effectiveness. Thus, we aimed to systematically review the psychometric properties of the Six-Spot Step Test, an increasingly used measure of dynamic balance. Data sources A literature search using the free-text term “Six-Spot Step Test” was performed on 12 February 2024, in Medline, Embase, Rehabilitation & Sports Medicine and SPORTDiscus. Eligibility criteria were adults aged 18 or more, trials evaluating the psychometric properties of the Six-Spot Step Test, and English-language articles. Conference abstracts were excluded. Review methods Two investigators screened and selected data independently and assessed the methodological quality and evidence using the COSMIN Risk of Bias checklist and modified GRADE approach. One investigator extracted study characteristics such as design, population and psychometric properties. Results Of the 159 articles identified, 16, evaluating multiple measurement properties, were included in the final analysis. A total of 1319 people participated, including people affected by Stroke, multiple sclerosis, Parkison's disease, chronic inflammatory polyneuropathy and older adults with balance problems. Eight articles assessing reliability (n = 618, intraclass correlations coefficient ≥0.7, minimal detectable change = 22%) and 12 construct validity (n = 1082, 83% true hypothesis, area under the curve >0.7) exhibited sufficient methodological quality with high-level evidence, while two studies (n = 167) examining responsiveness showed very low evidence. Conclusion Apart from responsiveness, robust evidence supports the reliability and validity of the Six-Spot Step Test for assessing dynamic balance in a specific group of individuals with neurological diseases and older adults. Further, it is considered feasible for clinical use.

Neuropathic arthropathy of the shoulder associated with chronic inflammatory demyelinating polyneuropathy: A case report

Neuropathic arthropathy is a chronic degenerative arthropathy that results from a neurosensory deficit of the affected joint. According to neurovascular theory, the loss of sensation of the joint is responsible for its formation. Chronic inflammatory demyelination polyneuropathy (CIDP) is a rare disease with sensorimotor involvement in which demyelination and axonal damage occur in the peripheral nerves as a result of an abnormal immune response. A case is presented of a 64-year-old male patient with a diagnosis of CIDP who had Charcot arthropathy of the right shoulder.

Guillain-Barré Syndrome and It’s Effect on Child Population

Background: Guillian Barre Syndrome (GBS) is the most common cause of minor flaccid paralysis in the world and post-polio eradication is the most common cause on the Indian subcontinent as well. It affects 0.6-4 people per 1 lakh people per year. Guillian-Barre syndrome should be distinguished by a variation associated with inflammation of the cortical area of brain of chronic inflammatory polyneuropathy predicting prognosis and clinical course. Children have a better prognosis than adults and usually recover after a different period. However, in the Pressing event of the disease, the damage can be severe and can lead to mechanical failure and even death. There are a few studies in the indian subcontinent that show the effects of GBS in children and the effect of regional variability of the outcome. This study is therefore designed to study prospective clinicians, as well as the overall effect on children with GBS in India.
 Objective: Studying the short-term and long-term effect of Guillian Barre Syndrome in children.
 Methods: This is a health care infrastructurel-based study to be conducted at Acharya Vinoba Bhave Rural hospital (ABVRH) for a period of one year. All children under the age of 18 between August 2019 to July 2020 are admitted to the PICU and completing the comprehending criteria will be comprised in the study. Patient details regarding the progression of the disease, the results of the investigation, the PICU stay, clinical studies in the hospital, the treatment received and the state of discharge will be recorded in the pre-prescribed proforma. The data obtained will be analyzed simultaneously.
 Conclusion: Information on factors associated with positive and negative outcomes in children with GBS will serve as a guide for your treating pediatrician to plan a treatment plan and will also help them explain their predictions to parents.

Neuropathic pruritus-Evidence-based treatment recommendations

Neuropathic pruritus is apreviously neglected symptom of awide range of neurological diseases. Peripheral nerve or root compression syndromes, space-occupying lesions of the central nervous system, chronic inflammatory neurological diseases and polyneuropathy can cause neuropathic pruritus. Even when the identification of the underlying neurological disease is successful, adirect causal treatment is not always possible, hence an effective symptomatic treatment remains the only therapeutic option. The purpose of this review article is to present the current literature on various therapeutic agents and options in the treatment of neuropathic pruritus.

Myoclonus and tremor in chronic inflammatory demyelinating polyneuropathy: a multichannel electromyography analysis.

Information regarding involuntary movements in chronic inflammatory polyneuropathy (CIDP) is gradually increasing. Our goal is to identify the types of involuntary movements in CIDP. All patients who were followed with the diagnosis of CIDP were invited for clinical and electrophysiological evaluations. Demographic and clinical findings (age, gender, duration of illness, diagnosis, treatments) were noted. Clinical examination and multichannel surface electromyography were done. We also performed routine upper and lower extremity peripheral nerve conduction studies, F-waves, long latency reflexes, blink reflex, mixed nerve silent period and cutaneous silent period in all patients. Twenty-two patients accepted the invitation. Eleven patients with CIDP had involuntary movements. Ten (45.5%) patients with CIDP had tremor and seven (31.8%) had short-duration and high-amplitude myoclonus. Regarding demographic, clinical and electrophysiological features, there was no significant difference between patients with and without tremor. The latencies of R1, R2 and R2c components of BR were longer among CIDP patients without tremor compared to CIDP patients with tremor. Presence of myoclonus (p = 0.007) and delayed F-waves (p = 0.008) were associated with the presence of tremor. Tremor and myoclonus were frequent in CIDP. The fact that myoclonus was detected in the majority of patients only by multichannel surface EMG who were clinically evaluated as pure tremor suggests that a more detailed electrophysiological evaluation is required. There was no difference in the medications used or other clinical features between patients with and without tremor.

Neurofilament light chain and total tau in the differential diagnosis and prognostic evaluation of acute and chronic inflammatory polyneuropathies.

Background and PurposeTo investigate the diagnostic and prognostic value of axonal injury biomarkers in patients with inflammatory polyneuropathies.MethodsNeurofilament light chain (NfL) and total tau (T‐tau) were measured in the cerebrospinal fluid (CSF) and plasma in 41 patients with Guillain–Barré syndrome (GBS), 32 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 10 with paraproteinemia‐related demyelinating polyneuropathy (PDN), and 8 with multifocal motor neuropathy (MMN), in comparison with 39 disease‐free controls and 59 other controls. Outcome was measured with the GBS‐disability score (GBS‐ds) or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score.ResultsNeurofilament light chain levels in CSF and plasma were higher in GBS, CIDP, and PDN vs. disease‐free controls. Patients with MMN had higher NfL levels in plasma vs. disease‐free controls, but lower levels in CSF and plasma vs. patients with amyotrophic lateral sclerosis (ALS).T‐tau levels in plasma were higher in GBS, CIDP, PDN, and MMN vs. all control groups.Neurofilament light chain levels in CSF and plasma in patients with GBS correlated with GBS‐ds, as higher levels were associated with inability to run after 6 and 12 months. NfL levels in CSF and plasma in CIDP did not correlate significantly with outcome.ConclusionsAcute and chronic inflammatory neuropathies are associated with an increase in levels of NfL in CSF and plasma, but NfL is validated as a prognostic biomarker only in GBS. NfL could be used in differentiating patients with MMN from ALS. T‐tau in plasma is a novel biomarker that could be used in a diagnostic assessment of patients with acute and chronic inflammatory polyneuropathies.

Contactin 1: A New Antigen in Membranous Nephropathy Associated with Chronic Inflammatory Polyneuropathy

Contactin 1: A New Antigen in Membranous Nephropathy Associated with Chronic Inflammatory Polyneuropathy

Dynamics of the incidence of acute and chronic inflammatory polyneuropathies in adults in the Leningrad region

Background. Inflammatory polyneuropathies (IPNP) are diseases caused by an immune response against antigens in the peripheral nervous system. Epidemiological research is essential for health resource planning.Objective: to assess the clinical and epidemiological characteristics of acute and chronic IPNP in adults in the Leningrad region.Materials and methods. We analyzed the incidence of acute and chronic IPNP the Leningrad region for the last 24 years.Results. A gradual increase in the incidence rate since 2003 with slight fluctuations has been shown. A particularly significant increase was recorded in 2015 from 14 (2014) to 22 patients, while in previous years fluctuations ranged from 4 to 10 people. In 2016 and 2017, the number of cases increased even more to 26–27 per year (there are 1 600 000 residents over 18 years of age in the region). At the same time, an increase in the incidence of both Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) was noted. The incidence of GBS in the last 10 years was 0.18–0.88, the incidence of CIDP was the same 0.18–1.0 per 100 000 population. With GBS, women were more likely to get sick, with CIDP – men. With GBS, the debut was more often in winter (35 %), in summer and autumn, 25 % each, less often in spring – 17 %. The average age of GBS development in our study was 50.3 years. CIDP also fell ill at all age periods from 19 to 84 years (average age 55.6 years). The most common GBS triggers were acute respiratory viral infections in 36 % and enterocolitis in 13 %. Respiratory disorders requiring mechanical ventilation were observed in 8 % of patients with GBS.Conclusions. The incidence of IPNP in adults, both acute and chronic in the Leningrad region, is growing with some fluctuations. This should be taken into account for health resource planning.

Severe allo-immune antibody-associated peripheral and central nervous system diseases after allogeneic hematopoietic stem cell transplantation

Allogeneic hematopoietic stem cell transplantation (alloHSCT) is a curative treatment for hematologic malignancies. Acute and chronic graft-versus-host disease (GvHD) are the major immune-mediated complications after alloHSCT. However, there is controversy whether neurologic complications after alloHSCT might represent manifestations of GvHD. We report three patients who acquired distinct, severe immune-mediated peripheral or central nervous system diseases after alloHSCT without other, concomitant GvHD manifestations. One patient had been diagnosed with B-cell chronic lymphocytic leukemia and two patients with high risk myelodysplastic syndrome. Patient #1 presented as LGI1- and GAD-IgG positive immune-mediated encephalitis, patient #2 was diagnosed with MOG-IgG positive encephalomyelitis, and patient #3 had chronic inflammatory polyneuropathy associated with SSA(Ro)-IgG positive Sjögren’s syndrome. 100% donor chimerism was detectable in the peripheral blood in all three. The specific antibodies were undetectable in donors’ and patients’ blood before alloHSCT suggesting that the antibodies had arisen from the transplanted donor immune system. Early intensive immunotherapy led to improvement of clinical symptoms and stability of the neurological disease, however, at the cost of losing the graft-versus-malignancy effect in one patient. In conclusion, we provide evidence of isolated, severe allo-immune diseases of the peripheral and central nervous system as complications of alloHSCT (“neuro-GvHD”). Interdisciplinary surveillance and thorough diagnostic work-up are needed for early diagnosis and treatment of neuro-immunologic complications after alloHSCT to improve the otherwise poor outcome.

Causes of chronic neuropathies: a single-center experience.

Chronic neuropathies are a common cause of neurological disability worldwide. However, few reports have evaluated, in real life, the prevalence of the several conditions which can cause it. The authors reviewed informatic database for outpatient office to confirm identification of chronic neuropathy in a 3-year interval period. Among the 100 selected patients with chronic neuropathies, almost one fifth (19%) remained idiopathic. The most common etiologies were diabetes (17%), dysimmune neuropathies (38%), and vitamin B12 deficiency (9%). In the "dysimmune neuropathies" group, we distinguished various etiologies, including dysimmune neuropathies associated or not with systemic autoimmune diseases (7 and 3%, respectively), chronic inflammatory polyneuropathy (CIDP) (8%), multifocal motor neuropathy (MMN) (3%), paraproteinemic (8%), celiac disease-related (6%), and paraneoplastic (3%) neuropathies. In this report from a single neurological center, treatable causes of chronic neuropathies, such as dysimmune neuropathies, including CIDP, and celiac disease-associated neuropathy, were common. These findings suggest the utility of routine screening with blood testing for dysimmune neuropathy and celiac disease for all patients presenting with idiopathic chronic polyneuropathy in whom primary diagnostic testings had failed to identify an etiology for the disease. Our results indicate that patients with peripheral neuropathy could receive a benefit from being evaluated routinely in a specialized neurological center, as many of the conditions that were discovered represented potentially treatable causes of neuropathy.

Chronic Inflammatory Demyelination Polyneuropathy (CIDP) Associated with Colorectal Cancer

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) occurs in about 1-2 persons per 100,000 adults. It is a progressive relapsing distal neuropathy developing due to several humoral and cell-mediated mechanisms causing injury of the myelin sheet. We describe a rare case of a patient with CIDP. Case Description: A 32-year-old female with the history of CIDP and iron deficiency anemia presented with lightheadedness, malaise and black stools associated with episodic epigastric pain, SOB, nausea, and constipation. Vitals were stable and physical examination revealed conjunctival pallor with mild generalized abdominal discomfort. Laboratory workup revealed severe microcytic anemia. The endoscopic evaluation showed two large necrotic masses in the duodenum and ascending colon consistent with malignancy. Computed tomography of the abdomen showed an ascending colon mass directly invading into the duodenum and was confirmed to be an infiltrating colorectal adenocarcinoma. Discussion: CIDP is associated with human immunodeficiency virus infection and inflammatory bowel disease and malignancies including lymphoma, melanoma, renal, breast and lung cancers. However, its association with colorectal cancer is not well described. So far two cases of colorectal adenocarcinoma with distal acquired demyelinating sensory neuropathy and one case with motor neuropathy-CIDP phenotype have been reported. In two patients CIDP preceded the diagnosis of malignancy who presented with constipation and abdominal pain. Two patients showed resolution of CIDP after surgical treatment of cancer. All patients were found to have anti-myelin glycoprotein antibodies. Conclusion: CIDP can be a presentation of an underlying GI malignancy similar to our patient. Therefore, in patients with a new diagnosis of CIDP, consideration can be made to evaluate for colon cancer.

The six-spot-step test - a new method for monitoring walking ability in patients with chronic inflammatory polyneuropathy.

The aim of this study was to evaluate whether the six-spot-step test (SSST) is more suitable for monitoring walking ability in patients with chronic inflammatory polyneuropathy than the timed 25-foot-walking test (T25FW). In the SSST, participants have to walk as quickly as possible across a field measuring 1 × 5 m, while kicking blocks out of five circles on the floor. Sixty-two patients and 61 controls performed the SSST and T25FW. Patients also performed the overall disability sumscore, INCAT sensory sumscore, Medical Research Council sumscore, and 9-hole-peg-test. Twenty-one patients treated with intravenous immunoglobulin (IVIG) every 4-6 weeks were tested prior to and 2-3 weeks after treatment and judged change in their own clinical condition using the patient global impression of change (PGIC) scale. In patients, SSST ranged from 5.7 to 26.8 s and T25FW ranged from 3.6 to 12.9 s. Intra-class correlation between repeated tests was 0.97 for SSST and 0.95 for T25FW. Correlation with the additional tests was stronger for SSST than T25FW. In IVIG-treated patients, the mean change in walking time was -2.3 s for SSST and -0.6 s for T25FW. The SSST showed larger responsiveness in terms of effect size, standardized response means, and relative efficiency. Both ambulation tests correlated moderately to PGIC. The SSST may be superior to the T25FW in terms of dynamic range, floor effect, and responsiveness which makes the SSST a possible alternative for monitoring walking ability in patients with chronic inflammatory polyneuropathy.

Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelination polyneuropathy is a heterogeneous and treatable immune-mediated disorder that lacks biomarkers to support diagnosis. Recent evidence indicates that paranodal proteins (contactin 1, contactin-associated protein 1, and neurofascin-155) are the targets of autoantibodies in subsets of patients showing distinct clinical presentations. Here, we identified neurofascin-186 and neurofascin-140 as the main targets of autoantibodies in five patients presenting IgG reactivity against the nodes of Ranvier. Four patients displayed predominantly IgG4 antibodies, and one patient presented IgG3 antibodies that activated the complement pathway in vitro. These patients present distinct clinical features compared to those with anti-neurofascin-155 IgG4. Most patients had a severe phenotype associated with conduction block or decreased distal motor amplitude. Four patients had a subacute-onset and sensory ataxia. Two patients presented with nephrotic syndromes and one patient with an IgG4-related retroperitoneal fibrosis. Intravenous immunoglobulin and corticosteroids were effective in three patients, and one patient remitted following rituximab treatment. Clinical remission was associated with autoantibody depletion and with recovery of conduction block and distal motor amplitude suggesting a nodo-paranodopathy. Our data demonstrate that the pathogenic mechanisms responsible for chronic inflammatory demyelination polyneuropathy are broad and may include dysfunctions at the nodes of Ranvier in a subgroup of patients.

Arteriovenous access does not perform as well for plasmapheresis.

Access surgeons are occasionally asked to create arteriovenous access for non-dialysis functions. Subjectively noting overall poor results, we seek to present our experience with arteriovenous access creation for apheresis. Billing records were reviewed using Current Procedural Terminology (CPT) and International Classification of Diseases (ICD-9) codes to identify patients undergoing arteriovenous access creation for diseases other than renal failure from January 2007 to August 2014. Inpatient and outpatient records were reviewed to identify patient demographics, disease-specific medications/treatments, access-specific characteristics, patency data, and reinterventions required. A total of 16 access creation procedures were performed for 8 patients, accounting for just 1.6% of total access creations during the period. Treatment was for myasthenia gravis (n = 6), chronic inflammatory degenerative polyneuropathy (n = 9), and stiff man syndrome (n = 1). Access failure was by thrombosis (n = 7), non-maturation (n = 4), and infection/steal syndrome (n = 1), with four accesses still functional at conclusion of review. There was 50% autogenous access creation and overall maturation rate of 37.5%. Mean primary patency was 236 days (range 10-878), with secondary patency achieved in three patients adding a mean of 174 days (range 2-517). Cumulative 3-month and 1-year patency rates were 36.5% and 25%, respectively. Arteriovenous access creation for plasmapheresis represents a minority of access procedures. Though it remains unclear why, patency and maturation rates are significantly lower than expected when compared to access for hemodialysis access. These high failure rates must be taken into account when considering replacement of temporary catheters with surgical access for non-hemodialysis needs.

EP 13. Establishment and validation of neurophysiological parameter for early diagnosis in familiar Transthyretin Amyloid-Polyneuropathy (TTR-FAP)

Transthyretin-amyloidosis is a life-threatening disease which presents with polyneuropathy as an early sign. However, its diagnosis is often delayed for years. To facilitate the differential diagnosis, we analyzed data from patients with polyneuropathy of different etiology, characterized and aimed at identifying clinical and electrophysiological discriminators. Methods we analyzed 78 patients: 52 with diabetes polyneuropathy (dPNP) (age=62.94 ± SEM = 4,09; 32 male, 20 females) and 26 of patients with Transthyretin Familial Amyloid Polyneuropathy (TTR-FAP) (age = 4231 ± SEM = 425; 16 male, 10 females). Both groups were matched considering sex and presence of pain (2 dPNP patients per TTR-FAP-Patient) and the differences were characterized in the quantitative sensory testing (QST), quantitative autonomic testing (QAT) and nerve conduction velocity (NCV). The results were validated by using a cohort of patients with chemotherapy-induced polyneuropathy (CIN) ( n = 48; age = 54.13 ± SEM = 3.20) and CIDP ( n = 8, age = 4975 ± 4.50). Results We found statistically significant differences in the quantitative sensory testing (mean+SEM): CDT ( z = −1.06 ± 0.21 vs. −2.49 ± 0.51, p = 0.017) and MPT ( z = −0.11 ± 0.24 vs. 1.68 ± 0.71, p = 0.009) with TTR patients being more impaired. Autonomic function was also more affected in TTR-FAP (SSR-Amplitude (−557.76 ± 84.40 (dPNP) vs. −87.71 ± 92.91; p = 0.001) and SSR-Latency (1197.21 ± 87.35 (dPNP) vs. 798.35 ± 149.366 (TTR-FAP); p=0.027) in the upper limb, SSR-Latency in the lower limb (1549.7 ± 135.46 (dPNP) vs. 979.74 ± 234.66; p = 0.042), RMSSD of the heart rate variability ( z = −0.17 ± 0.12 vs. 2.85 ± 0,94; p = 0.006),variation coefficient of the respiratory sinus arrhythmia (RSA-VC) ( z = 0.094 ± 0.22 vs. −0.78 ± 0.24 p = 0.018), and difference of the respiratory sinus arrhythmia (RSA-max-min) ( z = 0.23 ± 0.25 vs. −0.67 ± 0.23; p = 0.028)). The nerve conduction studies showed bigger impairment of the ulnar nerve in TTR-FAP (sensory nerve conduction velocity (SNCV)( z = −100 ± 0.15 vs. −5.27 ± 1.05; p = 0.001) and distal motor latency ( z = −0,80 ± 0.16 vs. 1.80 ± 0.65; p = 0.001)). Discriminant analyses identified SNCV of the ulnar nerve as a significant discriminator between TTR-FAP and dPNP patients (883% of patients correctly classified). We validated our results with a cohort of patients with chemotherapy-induced polyneuropathy (CIN) and chronic inflammatory demyelinising polyneuropathy (CIDP) and found that this parameter also discriminated patients with these etiologies from TTR-FAP in 84.6% and 73.9% respectively. Conclusion Clinical scores (NDS) suggested a slightly bigger clinical severity in dPNP compared to TTR-FAP. In spite of this, TTR-FAP-patients appeared to be more affected in the quantitative sensory testing, autonomic function and nerve conduction studies in the upper limb in TTR- FAP compared with diabetic patients. The sensory nerve conduction velocity of the ulnar nerve was identified as a good electrophysiological marker to discriminate between TTR-FAP and polyneuropathies of different etiology (dPNP, CIN).

Cardiac Involvement in Peripheral Neuropathies.

Cardiac autonomic neuropathy (CAN) is the least recognized and understood complication of peripheral neuropathy. However, because of its potential adverse effects including sudden death, CAN is one of the most important forms of autonomic neuropathies. CAN presents with different clinical manifestations including postural hypotension, exercise intolerance, fluctuation of blood pressure and heart rate, arrhythmia, and increased risk of myocardial infarction. In this article, the prevalence, clinical presentations, and management of cardiac involvement in certain peripheral neuropathies, including diabetic neuropathy, Guillain-Barré syndrome, chronic inflammatory polyneuropathy, human immunodeficiency virus-associated neuropathy, hereditary neuropathies, and amyloid neuropathy are examined in detail.

Bilateral Sixth Nerve Palsies in Anti-Aquaporin 4 Antibody Syndrome

While optic neuritis and myelitis are classically described in neuromyelitis optic (NMO), Morrow and Wingerchuk (1) point out other sites within the central nervous system that may be affected. However, despite frequent involvement of the brainstem in NMO, detailed descriptions of the patterns of ophthalmoplegia are available in only a few case reports (Table 1). We report bilateral fascicular sixth nerve palsies in a patient with anti-aquaporin 4 (AQP4) antibody.TABLE 1: Ophthalmoplegia in anti-AQ4 antibody syndromeA 26-year-old woman complained of headache, vertigo, and unsteadiness. Examination showed spontaneous left-beating nystagmus, gaze-evoked nystagmus (GEN) and leftward falling while walking. Brain magnetic resonance imaging (MRI) revealed a lesion involving the dorsal medulla. Serum anti-AQP4 antibody test was positive. She experienced marked improvement after a 5-day course of corticosteroids. One year later, the patient experienced another episode of headache, somnolence, and visual blurring. Examinations 1 week later showed small amplitude, spontaneous nystagmus beating rightward and upward only without fixation, and impaired vertical smooth pursuit. Brain MRI showed newly developed lesions involving the hypothalamus, mammillary bodies, and optic tracts. Three months later, the patient had the third episode of headache, facial numbness, and diplopia. Examination showed limitation of abduction in both eyes, upbeat nystagmus during convergence, and horizontal and vertical GEN (See Supplemental Digital Content, Video, https://links.lww.com/WNO/A131). Brain MRI disclosed new lesions in the pontine tegmentum bilaterally (Fig. 1). She was treated with intravenous steroids for a week followed by oral maintenance.FIG. 1: Axial fluid-attenuated inversion recovery (FLAIR) magnetic resonance scans show symmetric lesions involving the periventricular pontine region.Our patient had recurrent neurological episodes due to lesions involving the dorsal medulla, hypothalamus, mammillary bodies, optic tracts, and dorsal pons, all of which highly express AQP4 (7). Since the discovery of anti-AQP4 antibody as a pathogenic marker of NMO, there has been increasing recognition of patients developing neurologic symptoms and signs attributable to anti-AQP4 antibody, but without optic nerve or spinal cord involvement. Those cases have been termed NMO spectrum disorder to which our patient belongs (8). We are unaware of previous reports of bilateral fascicular sixth nerve palsies in association with anti-AQP4 antibody. Demyelinating disorders may cause sixth nerve palsy, usually in the setting of multiple sclerosis or chronic inflammatory polyneuropathy (9). In addition to bilateral abduction defects, our patient reported facial numbness, indicating involvement of the spinal trigeminal nucleus and tract. The GEN and convergence-evoked upbeat nystagmus suggest that nearby pontine structures for gaze holding such as the nucleus prepositus hypoglossi and paramedian tract cell groups also were affected (10,11).

Chronic inflammatory polyneuropathy associated with ulcerative colitis: a reported case in literature

Abstract Background: Peripheral neuropathy is known to be related to inflammatory bowel disease and it is one of the most frequently reported neurologic complications. Various studies have found peripheral nervous system complications, rather than central nervous system involvement, to be predominant. In the literature, there are a few cases of inflammatory polyneuropathy developed in the course of ulcerative colitis : 5 cases of Guillain-Barre syndrome, one case of chronic inflammatory demyelinating polyneuropathy, 10 cases of neuropathy and one case of perineuritis. Case presentation: We describe a case of chronic inflammatory polyneuropathy associated with ulcerative colitis. Conclusion: Peripheral neuropathy is not a common manifestation of IBD, highlighting the need for careful exclusion of other causes of neuropathy when both conditions are encountered in clinical practice.

93. CANOMAD: Clinical and neurophysiological findings in two cases

The acronym CANOMAD (Chronic Ataxic Neuropathy, Ophthalmoplegia, IgM paraproteinemia, cold Agglutinin and Disialosyl antibodies) refers a rare immune-mediated peripheral neuropathy, whose clinical spectrum has still to be fully delineated. Herein we report the clinical and neurophysiological features of two CANOMAD patients. A 52-year-old man suffering from distal sensory symptoms and ataxia had received a first diagnosis of chronic inflammatory prominent-sensory polyneuropathy 11 years ago. High-dose i.v. steroid treatment resulted in paradoxical severe worsening with ophthalmoplegia and ambulation loss. Based on immunological and clinical–neurophysiological revaluation a diagnosis of CANOMAD was made. Since then IVIg periodic infusions had allowed good clinical remission up to-day, although minor degree of motor impairment and ataxia relapsed during last years. This 62-year-old man had a 5-year history of mild lower limb ataxia. More recently, a fluctuating bilateral ptosis was seen. Serum was positive for anti-disialosyl IgM antibodies and cold agglutinins. NCS/EMG showed a mild chronic sensory-motor neuropathy. Cortical somatosensory evoked potentials by tibial nerve stimulation at the ankle were deranged, whereas responses at the popliteal fossa and sural NCS were largely preserved. This suggests a proximal alteration of peripheral sensory conduction. These data further confirm the heterogeneous presentation of CANOMAD.