Guillain-Barré syndrome: rehabilitation outcome and recent developments.

Guillain-Barré syndrome is the most common polyneuropathy causing major disability and respiratory failure. Respiratory complications are the main cause of death. Improved respiratory care and new treatment strategies such as plasmaphoresis and immunoglobulin have been shown to improve outcome. We studied the course and outcome of 37 patients with Guillain-Barré syndrome who were admitted to a rehabilitation and respiratory care facility over a 10-year period. There were 21 males and 16 females with a mean age of 62+/-3 years. Fourteen patients developed respiratory failure requiring endotracheal intubation and mechanical ventilation. The mean duration of mechanical ventilation was 38+/-10 days. All patients were successfully liberated from the ventilator. However, 83 percent of the patients were moderately to severely disabled at the time of discharge. Thirteen out of 37 (35 percent) developed long-term disability. None of the patients died over the period of follow-up. These results indicate that early recognition and treatment of respiratory complications in Guillain-Barré syndrome could reduce the morbidity and mortality of this condition.     

Miller–Fisher syndrome associated with SARS-CoV-2: a case report

SARS-CoV-2 infections are increasingly associated with neurological complications, including immune-mediated neuropathies. Miller–Fisher syndrome is a rare variant of Guillain-Barré syndrome characterised by the triad of ataxia, ophthalmoplegia and areflexia.Here we present a case of Miller–Fisher syndrome following COVID-19 infection. The clinical presentation was a short history of a rapidly-progressive peripheral sensorimotor neuropathy with bulbar dysfunction and facial weakness following mild COVID infection. Examination revealed global areflexia and a broad-based ataxic gait. CSF analysis revealed albuminocytological dissociation and neurophysiological testing later supported the diagnosis. The patient required high flow nasal oxygen therapy for respiratory dysfunction in a level 2 care setting and received immunological treatment with intravenous immunoglobulins.We conclude that Miller–Fisher syndrome needs to be considered in patients presenting with new sensorimotor dysfunction following SARS-COV-2 infection. Early recognition is key given the propensity to cause life-threatening respiratory failure, and early administration of immunological treatment is associated with improved prognosis.     

Recent Advances in Animal Models of Zika Virus Infection

An infection by Zika virus(ZIKV), a mosquito-borne flavivirus, broke out in South American regions in 2015, and recently showed a tendency of spreading to North America and even worldwide. ZIKV was first detected in 1947 and only 14 human infection cases were reported until 2007. This virus was previously observed to cause only mild flu-like symptoms.However, recent ZIKV infections might be responsible for the increasing cases of neurological disorders such as GuillainBarre′ syndrome and congenital defects, including newborn microcephaly. Therefore, researchers have established several animal models to study ZIKV transmission and pathogenesis, and test therapeutic candidates. This review mainly summarizes the reported animal models of ZIKV infection, including mice and non-human primates.     

Chronic effects of Campylobacter infection

Campylobacter jejuni is one of the most common causes of bacterial gastroenteritis and chronic sequelae, such as reactive arthritis and Guillain-Barré syndrome (GBS), are known to follow uncomplicated infections. While little is known about reactive arthritis following Campylobacter infection, our knowledge on the pathogenesis of Campylobacter-induced GBS is expanding rapidly and is summarized in this review.     

Management of infection by the Zika virus

A panel of national experts was convened by the Brazilian Infectious Diseases Society in order to organize the national recommendations for the management of zika virus infection. The focus of this document is the diagnosis, both clinical and laboratorial, and appropriate treatment of the diverse manifestations of this infection, ranging from acute mild disease to Guillain-Barré syndrome and also microcephaly and congenital malformations.     

Neonatal complications in infants born to mothers with diabetes mellitus

Health status of 53 babies delivered by diabetic mothers are discussed. Neonatal period was uncomplicated only in 12 cases. The remaining babies suffered from respiratory disorders, edema, neurological disturbances, prolonged jaundice, infections etc. Metabolic disorders in diabetic female are unfavourable for the development of pregnancy and neonate health. it may be improved by the proper diagnostico-therapeutical management prior to and during pregnancy and by intensive care of neonates after delivery. It requires, however, the establishment of health institution with highly qualified teams well equipped which will be able to carry out diagnosis and therapy of diabetes mellitus in females in the reproductive age, during pregnancy as well as proper care of the neonates.     

Repetitive pseudoseizures incorrectly managed as status epilepticus.

Pseudoseizures should be considered in the differential diagnosis of intractable seizures. Incorrect diagnosis may result in incorrect management, with the patient unnecessarily exposed to side effects of drugs. The authors report on three patients who presented with uncontrolled seizures originally diagnosed and managed as status epilepticus. Electroencephalography performed during provoked attacks led to a diagnosis of pseudoseizures. Psychiatric assessment revealed psychologic disorders. The patients received supportive therapy, and the pseudoseizures stopped.     

An endogenous pentapeptide acting as a sodium channel blocker in inflammatory autoimmune disorders of the central nervous system

Reversible blockade of sodium channels by endogenous substances has been claimed to account for the fast exacerbations and relapses commonly seen in demyelinating autoimmune diseases. Evidence has been provided that in the cerebrospinal fluid of patients with multiple sclerosis or Guillain-Barré syndrome, a sodium-channel-blocking factor exists that has properties of local anesthetic agents. This factor could contribute to the nerve conduction block and paresis seen in these disorders. We describe here a previously unknown endogenous substance in human cerebrospinal fluid with distinct channel-blocking properties even at very low (0.00001 M) concentrations. The pentapeptide with the sequence Gln-Tyr-Asn-Ala-Asp exerted its blocking action by shifting the steady-state inactivation curve of the sodium channels to more-negative potentials, as most local anesthetics do. In the cerebrospinal fluid of healthy individuals, its concentration was about 3 microM, whereas in patients with multiple sclerosis and Guillain-Barré syndrome, it increased 300-1,400%. At these concentrations, the peptide's blocking efficacy was higher than that of 50 microM lidocaine. At a concentration of 10 microM, lidocaine is able to 'unmask' subclinical lesions in multiple sclerosis; thus, the endogenous pentapeptide may well contribute to the fast changes of symptoms. Furthermore, it may become valuable as a marker of disease activity.     

Children in Adult Intensive Therapy Units

From experience in the Northampton/Kettering area 9 to 10% of all patients in a general hospital requiring care in an intensive therapy unit were aged 12 years or under. Fifty-nine children were admitted to the intensive therapy unit of Northampton General Hospital between May 1967 and August 1969. Of these, 22 had been injured in road traffic accidents, five were surgical emergencies, five had meningitis, four status epilepticus, and four respiratory infections.All of the 30 families interviewed were in favour of their child being admitted to the unit, and none considered that the experience had had any lasting adverse psychological effect on the child. It is suggested that certain carefully selected child patients do benefit from the facilities of an intensive therapy unit, and for this reason such units must be designed, equipped, and staffed with this in mind.     

A critical review on brain and heart axis response in COVID-19 patients: Molecular mechanisms,mediators, biomarkers,and therapeutics

Since the outbreak of highly virulent coronaviruses, significant interest was assessed to the brain and heart axis (BHA) in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-affected patients. The majority of clinical reports accounted for unusual symptoms associated with SARS-CoV-2 infections which are of the neurological type, such as headache, nausea, dysgeusia, anosmia, and cerebral infarction. The SARS-CoV-2 enters the cells through the angiotensin-converting enzyme (ACE-2) receptor. Patients with prior cardiovascular disease (CVD) have a higher risk of COVID-19 infection and it has related to various cardiovascular (CV) complications. Infected patients with pre-existing CVDs are also particularly exposed to critical health outcomes. Overall, COVID-19 affected patients admitted to intensive care units (ICU) and exposed to stressful environmental constraints, featured with a cluster of neurological and CV complications. In this review, we summarized the main contributions in the literature on how SARS-CoV-2 could interfere with the BHA and its role in affecting multiorgan disorders. Specifically, the central nervous system involvement, mainly in relation to CV alterations in COVID-19-affected patients, is considered. This review also emphasizes the biomarkers and therapy options for COVID-19 patients presenting with CV problems.     

Tissue Binding Patterns and In Vitro Effects of <Emphasis Type="Italic">Campylobacter jejuni</Emphasis> DNA-Binding Protein from Starved Cells

Campylobacter jejuni (C. jejuni) is frequently associated with axonal Guillain-Barré syndrome (GBS). We reported that C. jejuni DNA-binding protein from starved cells (C-Dps) binds to and damages myelinated nerves in vivo. We studied the binding patterns of C-Dps to nervous tissues and its in vitro effects on neural cells. Immunohistochemically, C-Dps labeled the nodes of Ranvier, the outermost parts of internodal myelin and the basement membrane in the peripheral nerves, and neurons and myelin in the central nervous tissues. Its binding was blocked by sulfatide. C-Dps bound to the cell surfaces of nerve growth factor (NGF)-treated PC12 cells leading to dose-dependent LDH release, which was inhibited by either heat-denaturation of C-Dps or coincubation with an anti-C-Dps mAb. However, its binding to the surfaces of cultured NSC34 cells, S16 cells, or dorsal root ganglion cells, did not induce cytotoxicity. These findings suggest a possible involvement of C-Dps in C. jejuni-related GBS.     

Animal models for autoimmune demyelinating disorders of the nervous system

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) that takes a relapsing-remitting or a progressive course (reviewed in Refs 1,2). Its counterpart in the peripheral nervous system (PNS) is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) (reviewed in Ref. 3). In addition, there are acute, monophasic disorders, such as the inflammatory demyelinating polyradiculoneuropathy termed Guillain-Barré syndrome (GBS) in the PNS, and acute disseminated encephalomyelitis (ADEM) in the CNS. Both MS and GBS are heterogeneous syndromes. In MS different exogenous assaults together with genetic factors can result in a disease course that finally fulfils the diagnostic criteria. In both diseases, axonal damage can add to a primarily demyelinating lesion and cause permanent neurological deficits. No single animal model exists that mimics all the features of human demyelinating diseases; rather, the available models reflect specific facets. Here, we focus on experimental autoimmune encephalomyelitis (EAE) and neuritis (EAN) as models in rat and mouse strains, and discuss their distinct histopathology and the roles played by different autoantigens.     

“Complications” of the Landry-Guillain-Barré-Strohl syndrome

Prognosis for life in the Landry-Guillain-Barré-Strohl syndrome is dependent upon the development of respiratory and non-respiratory “complications” and their successful management. Review of the literature, a case history, and a study of 14 patients with this syndrome at the University Hospital, Edmonton, indicate that “complications” can be anticipated in virtually all areas of acute care management, including respiratory, gastrointestinal, urinary tract, central and autonomic nervous systems, metabolic, cardiovascular, and infectious disease. The proper management of patients with the Landry-Guillain-Barré-Strohl syndrome demands an awareness of the totality of care required and the presence of a hospital system that provides for vital system monitoring and support, and for ready interdisciplinary consultation.     

Wachstumsst?rungen als Leitsymptom

Short stature is a frequently encountered question in both the genetic and the pediatric clinic. The definition of short stature includes a body length/height below the 3rd percentile or of more than two standard deviations below the mean. The velocity of growth as well as growth patterns are fundamentally regulated by genetic factors, but may be modified by secondary effects. The spectrum of short stature syndromes includes disorders of growth hormone secretion or effect, skeletal dysplasias and complex malformation syndromes. Identifying the underlying defect greatly facilitates counseling about prognosis and possible management. The most relevant syndromes in daily clinical routine are discussed here: Turner syndrome, Léri-Weill syndrome, Russel-Silver syndrome, Noonan syndrome, and achondroplasia.     

Argininosuccinic aciduria. A new case revealed by psychiatric disorders

The case of a 4 years old boy, hospitalized for an unexplained coma, is reported. He is the first child of a non-consanguin couple. The psychomotor development of this child was considered as normal up to the age of 18 months; then, a delay in language development, behaviour disorders with an important instability interrupted by episodes of somnolence, were observed. This child was treated for psychotic disorders. At the age of 3 and half, he had two episodes of seizures associated with fever. He was hospitalized for a 24 hours coma (4 years old). An hepatomegaly and a dry, brittle hair were then observed. Hyperammonemia was made obvious by a protein tolerance test. The diagnosis of argininosuccinate lyase (ASAL) deficiency was based on the increased levels of ASA in plasma and urine. The deficiency was proved by a fibroblast culture. With protein restriction, hepatomegaly disappeared, hair became normal, the behaviour disorders and the delay in language development was improved. However, some school difficulties persist. This case shows that an hereditary metabolic syndrome can be revealed by psychotic like symptoms in childhood.     

Temperature-dependent phenotypic variation of <Emphasis Type="Italic">Campylobacter jejuni</Emphasis> lipooligosaccharides

Background  

Campylobacter jejuni is a major bacterial cause of food-borne enteritis, and its lipooligosaccharide (LOS) plays an initiating role in the development of the autoimmune neuropathy, Guillain-Barré syndrome, by induction of anti-neural cross-reactive antibodies through ganglioside molecular mimicry.     

Neurology of AIDS virus infection: a clinical classification

Infection with the AIDS virus itself (HIV, HTLV-III, LAV, ARV) is associated with a full spectrum of neurological disorders. The application of diagnostic studies for HTLV-III infection has demonstrated that these neurologic disorders can be the first manifestation of AIDS or occur in the absence of AIDS. The most common conditions associated with HTLV-III infection alone are a subacute encephalopathy (AIDS dementia) and peripheral neuropathy; however, vacuolar myelopathy and both acute and chronic aseptic meningitis are also common. Congenital (or neonatal) transmission of the virus can result in a mental retardation syndrome of delayed onset. The AIDS virus is neurotropic as well as targeting T-helper lymphocytes. The virus has been readily identified in neural tissues and cerebrospinal fluid, including instances in which other central nervous system infections, such as toxoplasmosis, coexist. Hence, recognition of an appropriate syndrome, neurodiagnostic studies, and exclusion (or treatment) of other infections, as well as evidence for HTLV-III infection are required for diagnosis. The development of successful therapy will require agents which cross the blood-brain barrier.     

Campylobacter jejuni infection in patient with Guillain-Barré syndrome: a case report

The Guillain-Barré syndrome is an acute inflammatory polineuropathy; it's frequency is established at the level of 1,3 cases/ 100 000 persons/ year. The main etiological factors concerned with the GBS occurrence are: Campylobacter jejuni, cytomegalovirus, Epstein-Barre virus, Mycoplasma pneumoniae. The authors present a case of the 15 years old boy with the clinical features of acute motor axonal polineuropathy and confirmed C. jejuni infection. Identification of C. jejuni isolate was based on colony morphology on CCDA plate (OXOID), characteristic motility, catalase, oxidase, hippurate hydrolysis and acetate hydrolysis. The identity of C. jejuni was also confirmed by a specific PCR. According to the authors' knowledge this is the first case of a patient with GBS with confirmed C. jejuni infection reported from Poland.     

代谢性疾病实验动物模型的建立与评价

代谢综合征是一组以多种代谢性疾病合并出现为临床特点的临床症候群。建立适合的MS动物模型对研究代谢性疾病的发病机理和相关药物筛选有十分重要的意义。本文就目前MS实验动物模型的建立做一综述,并对其进行评价。