Guillain-Barre syndrome in northeastern Yunnan Province， China： A single-center retrospective analysis of 112 cases / 中风与神经疾病杂志

@#Objective To explore the clinical characteristics of patients with Guillain-Barré syndrome （GBS） in northeastern Yunnan Province， China， and to provide a basis for clinical diagnosis and treatment of GBS in this region. Methods We retrospectively analyzed the clinical data of inpatients with GBS in the Department of Neurology， Qujing No. 1 Hospital from February 2018 to October 2022， who were mainly from the northeastern regions of Yunnan Province. We compared the clinical characteristics of GBS reported in different regions of China in recent years. Results A total of 112 patients meeting the criteria were retrospectively included in this study， including 49 cases of acute inflammatory demyelinating polyneuropathy （AIDP） and 33 cases of acute motor axonal neuropathy （AMAN）. In this cohort of patients， GBS occurred frequently in spring and winter， especially in spring （38 cases， 33.9%）. AIDP was more common in winter （15 cases， 30.6%）， while AMAN was more common in spring （14 cases， 42.4%）. The median age of onset was 56 years （range： 8 to 83 years）， peaking at the age of 60 to 69 years. The male-to-female ratio was 1.60 （69∶43）. Upper respiratory tract infection （25 cases， 22.3%） and gastroenteritis （14 cases， 12.5%） were the most common among prodromal infections. AIDP was the most common subtype （49 cases， 51.6%）. There were no significant differences between the AIDP and AMAN groups in age of onset， prodromal infection， initial symptoms， clinical manifestations， cerebrospinal fluid protein levels， or therapeutic effects （P>0.05）. Twelve patients （10.7%） required mechanical ventilation at nadir. The patients with mechanical ventilation had higher proportions of cranial nerve involvement compared with those without mechanical ventilation， especially in the proportion of dysphagia （75.0% vs 32.0%， P=0.008 1）. Upper respiratory tract infection was the main prodromal event of GBS in Dali of Yunnan Province and the southern and eastern regions of China， where the proportions ranged from 22% to 35%. Gastroenteritis was more common in the northern region of China， accounting for 24%. AMAN was the predominant GBS subtype in northern （40%） and eastern （35%） China， while AIDP was the predominant subtype in southern China （49%）. Conclusion GBS showed disparities in different regions， with the demyelinating subtype dominant in northeastern Yunnan Province. GBS mainly occurred in men and at the age of 60-69 years， with a double-peak distribution. Gastrointestinal and respiratory infections remained the major prodromal events in this region. Cranial nerve involvement， especially bulbar muscle involvement， might be associated with the risk of mechanical ventilation.

Diagnostic value of F wave changes in patients with Charcot-Marie-Tooth1A and chronic inflammatory demyelinating polyneuropathy / 北京大学学报(医学版)

OBJECTIVE@#To analyze and compare the characteristics and causes of F wave changes in patients with Charcot-Marie-Tooth1A (CMT1A) and chronic inflammatory demyelinating polyneuropathy (CIDP).@*METHODS@#Thirty patients with CMT1A and 30 patients with CIDP were enrolled in Peking University Third Hospital from January 2012 to December 2018. Their clinical data, electrophysiological data(nerve conduction velocity, F wave and H reflex) and neurological function scores were recorded. Some patients underwent magnetic resonance imaging of brachial plexus and lumbar plexus, and the results were analyzed and compared.@*RESULTS@#The average motor conduction velocity (MCV) of median nerve was (21.10±10.60) m/s in CMT1A and (31.52±12.46) m/s in CIDP. There was a significant difference between the two groups (t=-6.75, P < 0.001). About 43.3% (13/30) of the patients with CMT1A did not elicit F wave in ulnar nerve, which was significantly higher than that of the patients with CIDP (4/30, 13.3%), χ2=6.65, P=0.010. Among the patients who could elicit F wave, the latency of F wave in CMT1A group was (52.40±17.56) ms and that in CIDP group was (42.20±12.73) ms. There was a significant difference between the two groups (t=2.96, P=0.006). The occurrence rate of F wave in CMT1A group was 34.6%±39%, and that in CIDP group was 70.7%±15.2%. There was a significant difference between the two groups (t=-5.13, P < 0.001). The MCV of median nerve in a patient with anti neurofascin 155 (NF155) was 23.22 m/s, the latency of F wave was 62.9-70.7 ms, and the occurrence rate was 85%-95%. The proportion of brachial plexus and lumbar plexus thickening in CMT1A was 83.3% (5/6) and 85.7% (6/7), respectively. The proportion of brachial plexus and lumbar plexus thickening in the CIDP patients was only 25.0% (1/4, 2/8). The nerve roots of brachial plexus and lumbar plexus were significantly thickened in a patient with anti NF155 antibody.@*CONCLUSION@#The prolonged latency of F wave in patients with CMT1A reflects the homogenous changes in both proximal and distal peripheral nerves, which can be used as a method to differentiate the CIDP patients characterized by focal demyelinating pathology. Moreover, attention should be paid to differentiate it from the peripheral neuropathy caused by anti NF155 CIDP. Although F wave is often used as an indicator of proximal nerve injury, motor neuron excitability, anterior horn cells, and motor nerve myelin sheath lesions can affect its latency and occurrence rate. F wave abnormalities need to be comprehensively analyzed in combination with the etiology, other electrophysiological results, and MRI imaging.

Pain abdomen and cardiac arrest as peculiar presenting symptoms in AIDP- AMSAN variety

AIDP (Acute Inflammatory Demyelinating Polyneuropathy) is a heterogenous condition encompassing several variants. It is a post infectious neurological disorder with an autoimmune pathogenesis with molecular mimicry mechanism. They present commonly with symmetrical ascending type paralysis and absent or diminished deep tendon reflexes. Cranial nerve palsies may or may not be present. The diagnosis is based on the clinical signs and symptoms, nerve conduction studies and cerebrospinal fluid analysis. We herewith report a rare case of AIDP, where the patient came walking to the emergency room with pain abdomen as the only complaint and had no neurological deficits at the time of presentation, hours later, the patient went into cardiac arrest, the cause of which was later thought to be dysautonomia and respiratory failure. The next day, patient developed motor weakness and multiple cranial nerve palsies which is an overlap of AMSAN and Acute Ophthalmoplegia. It is extremely uncommon to present with pain abdomen and cardiac arrest as presenting features in AIDP, AMSAN (Acute Motor Sensory Axonal Neuropathy) variety.

A rare presentation of Guillain-Barre syndrome with GQ1b positivity: A case report

Rationale: To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity. Patient concern: A 35-year-old female, 68 days postpartum, presented with headache, vomiting, and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking, 13 days after ChAdOx1 nCoV-19 vaccination. Diagnosis: Guillain-Barre syndrome with GQ1b positivity. Intervention: Five cycles of plasmapheresis were given. Outcome: The patient's clinical condition improved. Palatal weakness improved and she could walk without support. There were mild sensory symptoms involving upper limbs which gradually improved. Lessons: AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination. Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.

Eficacia de la movilización de células madre en pacientes con Polineuropatía Desmielinizante Inflamatoria Crónica refractaria / Efficacy of mobilization of stem cells in patients with Refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Introducción: La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad desmielinizante e inflamatoria de mediación autoinmune. El tratamiento convencional es basado en la inmunomodulación e inmunosupresión. El uso de células madre es una terapia novedosa en los trastornos autoinmune, siendo incluida como terapia. Objetivo: Determinar la eficacia de la movilización de células madre mediante la aplicación del factor estimulador de colonias granulocíticas (F-ECG) en pacientes con CIDP que han recibido otras líneas de tratamiento. Material y Métodos: Se realizó un estudio aleatorizado, doble ciego sobre una cohorte de 45 pacientes con CIDP, donde se administró el (F-ECG) en 25 pacientes y 20 continuaron con el tratamiento habitual, tratados anteriormente con otras variantes terapéuticas por más de tres años, sin respuesta satisfactoria. Resultados: Predominio de los hombres para 64,4 por ciento, la Diabetes Mellitus tuvo mayor asociación y la medicación más usada fueron los esteroides. Los síntomas y signos clínicos mejoraron significativamente tras el tratamiento. Los valores de la puntuación del TCSS al mes y 3 meses después del tratamiento disminuyeron significativamente; pero este decremento no se mantuvo al final del estudio. La velocidad de conducción y el potencial de acción de los nervios sensoriales y motores mejoraron considerablemente después del tratamiento. Conclusiones: La efectividad de la aplicación del (F-ECG) para la mejoría de los síntomas clínicos y resultados de estudios neurofisiológicos evolutivamente son mayores que otras variantes terapéuticas en los primeros meses, con buena seguridad y tolerabilidad, por lo que se puede incluir en la terapéutica convencional para la CIDP(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune demyelinating disease. Conventional treatment is based on immunomodulation and immunosuppression. The use of stem cells is a novel therapy in autoimmune disorders, so it is included as therapy. Objective: To determine the efficacy of mobilization of stem cells by applying granulocyte colony-stimulating factor (G-CSF) in patients with CIDP who have followed other lines of treatment. Material and Methods: A randomized, double-blind study was carried out on a cohort of 45 patients with CIDP. G-CSF was administered to 25 patients and 20 of them continued with the usual treatment. These patients were previously treated with other therapeutic variants for more than three years without satisfactory response. Results: There was a prevalence of men (64.4 percent), Diabetes Mellitus had a greater association, and the most used medications were steroids. Clinical symptoms and signs improved significantly after treatment. TCSS scores significantly decreased at one and three months after treatment, but this decrease was not maintained at the end of the study. The conduction velocity and action potential of sensory and motor nerves improved considerably after treatment. Conclusions: The effectiveness of the use of G-CSF shows an improvement of clinical symptoms. The results of neurophysiological studies have a better course than other therapeutic variants during the first months, with good safety and tolerability, so it can be included in the conventional therapy for the CIDP(AU)

Polineuropatía desmielinizante inflamatoria crónica como forma de presentación de lupus eritematoso sistémico / Chronic inflammatory demyelinating polyneuropathy as a form of presentation of systemic lupus erythematosus

Se describe el caso de una mujer de 35 años que presenta polineuropatía desmielinizante inflamatoria crónica como compromiso neurológico en su diagnóstico inicial de lupus eritematoso sistémico (LES). Si bien el compromiso neurológico es de una prevalencia variable en lupus, la asociación que se describe no es frecuente y tiene importantes connotaciones en el tratamiento.

We described a 35 years old female, who developed Chronic inflammatory demyelinating polyneuropathy as neurologic commitment during the early diagnosis in Systemic Lupus Erithematosus (SLE). While the neuropsychiatric commitment has a variable prevalence in SLE, the association that we describe is infrequent and it has important concerns during its treatment.

Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy

OBJECTIVE: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency.MATERIALS AND METHODS: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained.RESULTS: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy.CONCLUSION: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

Polineuropatía desmielinizante inflamatoria crónica como forma de presentación de lupus eritematoso sistémico / Chronic inflammatory demyelinating polyneuropathy such as presentation of systemic lupus erythematosus

Se describe el caso de una mujer de 35 años que presenta polineuropatía desmielinizante inflamatoria crónica como compromiso neurológico en su diagnóstico inicial de lupus eritematoso sistémico (LES). Si bien el compromiso neurológico es de una prevalencia variable en lupus, la asociación que se describe no es frecuente y tiene importantes connotaciones en el tratamiento.

We described a 35 years old female, who developed Chronic inflammatory demyelinating polyneuropathy as neurologic commitment during the early diagnosis in Systemic Lupus Erithematosus (SLE). While the neuropsychiatric commitment has a variable prevalence in SLE, the association that we describe is infrequent and it has important concerns during its treatment.

Polineuropatía desmielinizante inflamatoria crónica en paciente diabética con sífilis / Chronic inflammatory demyelinating polyneuropathy in a diabetic patient with syphilis

La polineuropatía desmielinizante inflamatoria crónica (PDIC) se presenta generalmente con síntomas motores, debilidad tanto en los músculos proximales como en los distales con reflejos globalmente disminuidos o ausentes. La neuritis insulínica es un trastorno caracterizado por dolor agudo de las extremidades, y daño de los nervios periféricos con afectación predominante de las fibras pequeñas, en los pacientes diabéticos sometidos a un rápido control glucémico. El dolor es raro en la PDIC clásica. Describimos el caso de una mujer de 54 años con diabetes mellitus (DB) tipo II, en tratamiento reciente con insulina, que consultó por un cuadro de debilidad e hiperalgesia de los cuatro miembros de dos meses de evolución. Al examen físico presentaba dolor de intensidad 10/10 y alodinia en los cuatro miembros, a niveles proximal y distal, con fuerza muscular reducida de los músculos proximales y arreflexia patelar y aquilea bilateral. Se realizó un estudio electrofisiológico, el cual mostró una polineuropatía sensitiva y motora desmielinizante. Se indicó tratamiento con inmunoglobina humana recombinante, con total remisión del cuadro. Estudios realizados posteriormente demostraron positividad débil de los anticuerpos GM1, GD1a, GD1b y anti-asialo GM1. Previo al alta hospitalaria se recibieron los resultados de VDRL sérica positiva, y FTA-Abs. VDRL en líquido cefalorraquídeo fue negativa por lo que se descartó neurosífilis, indicándose tratamiento con penicilina benzatínica.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder characterized by motor symptoms such as weakness in both proximal and distal muscles with globally diminished or absent reflexes. Insulin neuritis is referred as an acute pain in the extremities, due to the damage of peripheral nerves affecting mainly small fibers, in diabetic patients treated with insulin who achieved rapid glycemic control. Pain is unusual in classic CIDP. We report the case of a 54-year-old female patient with type II diabetes mellitus, and a recent onset of insulin therapy, who presented at the emergency room with a 2-month history of weakness and hyperalgesia of extremities. Physical examination showed marked pain and proximal and distal allodynia in the 4 limbs, with reduced muscle strength of the proximal muscles and patellar and achillear areflexia. Electrophysiological study showed sensory and motor polyneuropathy with a demyelinating predominance. Treatment with recombinant human immunoglobin was started, and the patient presented a total remission of the condition. Complementary studies confirmed weak serum positivity of GM1, GD1a, GD1b and anti-asialo GM1. Prior to hospital discharge, results of positive serum VDRL and FTA-Abs were received. VDRL in cerebrospinal fluid was negative, so neurosyphilis was ruled out, and treatment with benzathine penicillin was indicated.

Clinical characteristics of patients with recurrent Guillain-barré syndrome / 中国神经精神疾病杂志

Objective To investigate the clinical features of recurrent Guillain-barré syndrome. Methods The data on demographic information and clinical features of 286 inpatients with Guillain-barré syndrome were collected. Patients were divided into recurrent group and non-recurrent group based on the number of episodes. The data from those patients with recurrent Guillain-barré syndrome whose information was missing was collected by telephone. Results we identified 22 (7.7%) cases with recurrent Guillain-barré syndrome including one case of Miller fisher syndrome. The total episodes were 55. Sixteen patients had 2 episodes, 3 patients had 3 episodes, 1 patients had 4 episodes and 2 patients had 5 episodes. Compared with non-recurrent group, patients with recurrent Guillain-barré syndrome had younger onset [(36.36 ±14.51)y vs. (45.72 ±16.13)y, t=-2.633, P=0.009], the shorter interval between the initial manifestations to peak [(2.18±0.73)d vs.(4.24±2.98)d, t=-8.537, P=0.000], the lower GBS disability score (68.2% vs. 31.8% , χ2=4.209, P=0.040) and less involvement in the cranial nerve (13.6% vs. 37.5% , χ2=5.040, P=0.025). Conclusion The recurrent risk may be considered in patients with Guillain-barré syndrome when younger patients, have lower GBS disability score and the shorter interval between the initial manifestations to peak.

Delayed Relaxation (Pseudomyotonia) as the Only Clinical Manifestation of Chronic Inflammatory Demyelinating Polyneuropathy

No abstract available.

Corticosteroids Treatment in Spinal Cord and Neuromuscular Disorders

Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of the vertebrates, as well as the synthetic analogs of these hormones that are synthesized in the laboratories. Two main classes of corticosteroids, glucocorticoids, and mineralocorticoids, are involved in a wide range of physiologic processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Corticosteroids have been used for almost 60 years in medicine and their roles in patients have always been discussed by researchers and clinicians dedicated in the related field. Currently, they are still used in the treatment of patients with neurological disorders. Usually, corticosteroids are used in the treatment of various inflammatory diseases and conditions. In this review, we present five key indications, i.e., neuromyelitis optica, acute spinal cord injury, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, polymyositis/dermatomyositis for the systemic use of corticosteroids in neurology based on a mix of quality of evidence, prevalence, and impact on disease management.

Estudio electrodiagnóstico en síndrome de Guillain Barré en adultos / Electrophysiological assessment in Guillain-Barré syndrome in adults

El síndrome de Guillain Barré es una poliradiculoneuropatía aguda con varias formas de presentación y actualmente la principal causa de parálisis aguda arrefléctica en el mundo, su diagnóstico es clínico y los exámenes de apoyo como el electrodiagnóstico son utilizados para realizar el diagnóstico diferencial, clasificar entre las distintas variantes (desmielinizantes o axonales) y definir pronóstico. El propósito de este trabajo es realizar una revisión narrativa de la literatura disponible, haciendo énfasis en el estudio electrofisiológico, hallazgos precoces y describir los errores más frecuentes, como también destacar la importancia de realizar la clasificación del cuadro para orientar las acciones de rehabilitación hacia la recuperación funcional.

Guillain Barré syndrome is an acutepolyradiculoneuropathy with differentforms ofpresentation and is currently the leading cause ofacute arreflexicparalysis in the world, its diagnosis is clinical and additional assesment as electrodiagnosis are used for differential diagnosis, rank among the variants (demyelinating or axonal) and define prognosis. The purpose of this paper is to make a narrative review of the literature, emphasizing the electrophysiological study, describing early findings, the most common mistakes, while highlighting the importance of classification chart to guide actions towards rehabilitation and functional recovery.

Leprous neuromyositis: A rare clinical entity and review of the literature.

Mycobacterium leprae, the causative agent of leprosy (Hansen’s disease), is a slow growing intracellular acid‑fast bacillus that affects the skin, peripheral nerves and respiratory tract. In patients with suppressed cell‑mediated immunity, the infiltration of the Bacilli can produce disseminated illness such as leprous neuromyositis. We reported a case of 56‑year‑old gentleman presenting with pyrexia of unknown origin, asymmetric sensory motor axonal polyneuropathy and was on chronic exogenous steroid therapy. On evaluation, his skin, muscle, nerve and bone marrow biopsy showed numerous globi of acid‑fast Bacilli suggestive of leprous neuromyositis, a rare form of disseminated Hansen’s disease. We reported this case in view of its rarity, atypical manifestation of a relatively rare disease and literature review on poor electrophysiological correlation in the diagnosis of leprous neuromyositis as compared to the histopathological examination.

Early Electrodiagnostic Features of Upper Extremity Sensory Nerves Can Differentiate Axonal Guillain-Barré Syndrome from Acute Inflammatory Demyelinating Polyneuropathy

BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.

Clinical outcome of Gullian-Barre Syndrome in a tertiary care teaching hospital A prospective observational study

Background: Gullian-Barre Syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP) with autoimmune background. The clinical management of GBS is by nerve conduction velocity (NCV) and supportive care, intravenous immunoglobulin’s (IVIG) and Plasmapheresis. We have studied the clinical outcome of Gullian-Barre Syndrome patients visiting to the tertiary care hospital in Andhra Pradesh. Material and methods: A cross sectional study was conducted in a tertiary care teaching hospital at Andhra Pradesh in 50 patients over the period of 2 years. Neurological examination like higher mental functions, cranial nerves, motor system, sensory system and autonomic system was done for all patients. Descriptive analysis of clinical presentation, type of GBS, occurrence of complications and final outcome was also done. Results: A total of 50 participants were included in the study. Majority (52%) of the study participants were aged below 40 years. Diabetes mellitus (DM) and hypertension (HTN) were the Vasa VK, Chowdary DB, Kalyani OM. Clinical outcome of Gullian-Barre Syndrome in a tertiary care teaching hospital – A prospective observational study. IAIM, 2016; 3(1): 105-109. Page 106 most common co-existing illnesses reported in 8% and 6% of study population respectively. Conclusion: The majority of the Guillain-Barre Syndrome patients recovered smoothly without going for complications. Prognostic outcome was poor in our study with increasing age and co-existing illness like diabetes mellitus or ischemic heart disease.

Catastrophic antiphospholipid syndrome – A case report.

Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially life-threatening condition, acute in onset , characterized by diffuse vascular thrombosis, leading to multiple organ failure in a short period of time in the presence of positive antiphospholipid antibodies (aPL). Lupus anticoagulant and anticardiolipin antibodies are the predominant antibodies associated with CAPS. Treatment options for CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. The high rate of mortality warrants greater awareness among clinicians for early diagnosis and treatment of CAPS. In this case report, 30-yearold post-partum female presented with progressive weakness, shortness of breath of grade IV and swelling of all the four limbs of 15 days duration with an episode of seizure. Investigations revealed MRV - cortical sinus venous thrombosis (CSVT) of transverse and sigmoid sinus, Raised anti-ds DNA anticardiolipin and lupus anticoagulant, 24 hour urinary proteins – 540 mg/day indicating clinical lupus nephritis. Weakness of all the limbs with areflexia indicated acute inflammatory demyelinating polyneuropathy (AIDP). 2D echocardiography- post partum dilated cardiomyopathy (DCMP).

Comparison of clinical manifestations and electrophysiological features in patients with chronic inflamma-tory demyelinating polyneuropathy and Type-I Charcot Marie Tooth Disease / 中国神经精神疾病杂志

Objectives To compare clinical manifestations and electrophysiological features in patients with chron?ic inflammatory demyelinating polyneuropathy (CIDP) and Type-I Charcot Marie Tooth Disease (CMT-I) for guiding dif?ferential diagnosis. Methods Data including clinical manifestations and electrophysiological indexes was collected from thirty-one CIDP cases and 28 CMT-I cases. Correlation analysis was used to assess the association of the severity of electrophysiology with the severity of clinical symptoms. Results There were statistically significant differences in onset site, sensory dysfunction, foot deformity and cerebrospinal fluid protein between these two groups (P0.05). Conclusions Differential diagnoses of CIDP and CMT-I can be made based on clinical manifestations and electro?physiological features.

Diplejía facial como variante inusual del síndrome de Guillain-Barré: primer caso reportado en Venezuela / Facial diplegia as unusual variant of Guillain-Barré syndrome: first case reported in Venezuela

El síndrome de Guillain-Barré (SGB) es la causa más común de parálisis generalizada aguda. El SGB es una polirradiculoneuropatia aguda inflamatoria desmielinizante que generalmente se presenta como una parálisis que inicia en miembros inferiores y luego progresa de forma ascendente y simétrica. El presente trabajo, tiene como objetivo, informar un caso de diplejía facial como manifestación inicial de SGB. Paciente masculino, 37 años de edad, diabético tipo 2, que luego de ocho días de haber padecido una sinusitis aguda, presentó de forma gradual, cefalea hemicraneana derecha, derramamiento salival y disartria. En la exploración neurológica se observó ausencia bilateral de los pliegues frontales, lagoftalmos bilateral acompañado de epífora, signo de Bell bilateral y derramamiento salival a través de ambas comisuras labiales. A las 48 horas de su ingreso hospitalario, presentó paresia en ambos miembros superiores. El estudio del líquido cefalorraquídeo reportó 1,1 células/mm³ representadas en su totalidad por linfocitos de aspecto normal, y proteínas totales 196,9mg/dL. La electromiografía fue compatible con polineuropatía desmielinizante aguda de predominio motor con mayor afectación facial. Con los hallazgos clínicos y paraclínicos se realizó el diagnóstico de SGB. Se inició tratamiento a base de plasmaféresis e inmunoglobulina endovenosa, con posterior mejoría de la clínica. La diplejía facial forma parte de las variantes regionales del SGB. A pesar que cerca del 60% de los pacientes con SGB presentan debilidad facial en el curso del trastorno, ésta habitualmente es precedida por debilidad en extremidades. El presente caso permite evidenciar que el SGB puede debutar clínicamente como una diplejía facial.

The Guillain-Barré syndrome (GBS) is the most common cause of acute generalized paralysis. GBS is an acute inflammatory demyelinating polyradiculoneuropathy. It usually presents as a paralysis that starts in the lower limbs and then progresses symmetrically upward. The present study reports a case of bilateral facial palsy as the initial manifestation of GBS. This is a report of a case of a 37-year-old male, diabetic, that eight days after having suffered acute sinusitis, gradually presented with right hemicranial headache, dysarthria and sialorrhea. The neurological examination disclosed the absence of the bilateral frontal folds, accompanied by epiphora, bilateral lagophthalmos, bilateral Bell sign and salivary drooling through both commissures of lips. At 48 hours after hospital admission the patient showed paresis in both upper limbs. The cerebrospinal fluid analysis reported 1.1cells/mm³, fully represented by lymphocytes of normal aspect and total proteins were 196.9 mg/dL. The electromyography was consistent with acute demyelinating polyneuropathy, with a predominant motor component and a major facial involvement. With the clinical and laboratory findings, a diagnosis of GBS was established. Treatment was started with plasmapheresis and intravenous immunoglobulin, with the subsequent improvement of the clinic. The facial diplegia is part of the regional variants of GBS. Although about 60% of GBS patients present with facial weakness, it is usually preceded by weakness in the limbs. This case makes evident that GBS may present clinically as a facial diplegia.

Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses / 소아과

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-Barre syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.