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International Orthopaedics Feb 2022International uniformity of definition and classification are crucial for diagnosis and management of cauda equina syndrome (CES). They are also useful for clinicians... (Review)
Review
PURPOSE
International uniformity of definition and classification are crucial for diagnosis and management of cauda equina syndrome (CES). They are also useful for clinicians when discussing CES with patients and relatives, and for medicolegal purposes.
METHODS
We reviewed published literature using PubMed on definition and classification of cauda equina syndrome since 2000 (21 years). Using the search terms 'cauda equina' and 'definition' or 'classification', we found and reviewed 212 papers.
RESULTS
There were 17 different definitions of CES used in the literature. There were three well-defined methods of classification of CES. The two-stage system of incomplete CES (CESI) versus CES with retention (CESR) is the most commonly used classification, and has prognostic value although the details of this continue to be debated.
CONCLUSION
We used the existing literature to propose a clear definition of CES. We also drew on peer-reviewed published literature that has helped to amplify and expand the CESI/CESR dichotomy, adding categories that are both less severe than CESI, and more severe than CESR, and we propose clear definitions in a table form to assist current and future discussion and management of CES.
Topics: Cauda Equina Syndrome; Humans; Polyradiculopathy; Prognosis
PubMed: 34862914
DOI: 10.1007/s00264-021-05273-1 -
The Canadian Journal of Neurological... Jan 2021Albeit primarily a disease of respiratory tract, the 2019 coronavirus infectious disease (COVID-19) has been found to have causal association with a plethora of... (Review)
Review
BACKGROUND
Albeit primarily a disease of respiratory tract, the 2019 coronavirus infectious disease (COVID-19) has been found to have causal association with a plethora of neurological, neuropsychiatric and psychological effects. This review aims to analyze them with a discussion of evolving therapeutic recommendations.
METHODS
PubMed and Google Scholar were searched from 1 January 2020 to 30 May 2020 with the following key terms: "COVID-19", "SARS-CoV-2", "pandemic", "neuro-COVID", "stroke-COVID", "epilepsy-COVID", "COVID-encephalopathy", "SARS-CoV-2-encephalitis", "SARS-CoV-2-rhabdomyolysis", "COVID-demyelinating disease", "neurological manifestations", "psychosocial manifestations", "treatment recommendations", "COVID-19 and therapeutic changes", "psychiatry", "marginalised", "telemedicine", "mental health", "quarantine", "infodemic" and "social media". A few newspaper reports related to COVID-19 and psychosocial impacts have also been added as per context.
RESULTS
Neurological and neuropsychiatric manifestations of COVID-19 are abundant. Clinical features of both central and peripheral nervous system involvement are evident. These have been categorically analyzed briefly with literature support. Most of the psychological effects are secondary to pandemic-associated regulatory, socioeconomic and psychosocial changes.
CONCLUSION
Neurological and neuropsychiatric manifestations of this disease are only beginning to unravel. This demands a wide index of suspicion for prompt diagnosis of SARS-CoV-2 to prevent further complications and mortality.
Topics: Ageusia; Alzheimer Disease; Angiotensin-Converting Enzyme 2; Anosmia; Brain Diseases; COVID-19; Cerebellar Ataxia; Cerebrovascular Disorders; Comorbidity; Delivery of Health Care; Demyelinating Diseases; Disease Management; Dizziness; Epilepsy; Guillain-Barre Syndrome; Headache; Humans; Hypoxia, Brain; Inflammation; Meningoencephalitis; Muscular Diseases; Myelitis, Transverse; Myoclonus; Nervous System Diseases; Parkinson Disease; Polyneuropathies; SARS-CoV-2; Seizures; Stroke; Viral Tropism
PubMed: 32753076
DOI: 10.1017/cjn.2020.173 -
JAMA Neurology Dec 2015Peripheral neuropathy is a prevalent condition that usually warrants a thorough history and examination but has limited diagnostic evaluation. However, rare... (Review)
Review
IMPORTANCE
Peripheral neuropathy is a prevalent condition that usually warrants a thorough history and examination but has limited diagnostic evaluation. However, rare localizations of peripheral neuropathy often require more extensive diagnostic testing and different treatments.
OBJECTIVE
To describe rare localizations of peripheral neuropathy, including the appropriate diagnostic evaluation and available treatments.
EVIDENCE REVIEW
References were identified from PubMed searches conducted on May 29, 2015, with an emphasis on systematic reviews and randomized clinical trials. Articles were also identified through the use of the authors' own files. Search terms included common rare neuropathy localizations and their causes, as well as epidemiology, pathophysiology, diagnosis, and treatment.
FINDINGS
Diffuse, nonlength-dependent neuropathies, multiple mononeuropathies, polyradiculopathies, plexopathies, and radiculoplexus neuropathies are rare peripheral neuropathy localizations that often require extensive diagnostic testing. Atypical neuropathy features, such as acute/subacute onset, asymmetry, and/or motor predominant signs, are frequently present. The most common diffuse, nonlength-dependent neuropathies are Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and amyotrophic lateral sclerosis. Effective disease-modifying therapies exist for many diffuse, nonlength-dependent neuropathies including Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and some paraprotein-associated demyelinating neuropathies. Vasculitic neuropathy (multiple mononeuropathy) also has efficacious treatment options, but definitive evidence of a treatment effect for IgM anti-MAG neuropathy and diabetic amyotrophy (radiculoplexus neuropathy) is lacking.
CONCLUSIONS AND RELEVANCE
Recognition of rare localizations of peripheral neuropathy is essential given the implications for diagnostic testing and treatment. Electrodiagnostic studies are an important early step in the diagnostic evaluation and provide information on the localization and pathophysiology of nerve injury.
Topics: Disease Management; Humans; Peripheral Nervous System Diseases; Treatment Outcome
PubMed: 26437251
DOI: 10.1001/jamaneurol.2015.2347 -
Journal of Neurology Apr 2021Since coronavirus disease-2019 (COVID-19) outbreak in January 2020, several pieces of evidence suggested an association between the spectrum of Guillain-Barré...
Since coronavirus disease-2019 (COVID-19) outbreak in January 2020, several pieces of evidence suggested an association between the spectrum of Guillain-Barré syndrome (GBS) and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Most findings were reported in the form of case reports or case series, whereas a comprehensive overview is still lacking. We conducted a systematic review and searched for all published cases until July 20th 2020. We included 73 patients reported in 52 publications. A broad age range was affected (mean 55, min 11-max 94 years) with male predominance (68.5%). Most patients showed respiratory and/or systemic symptoms, and developed GBS manifestations after COVID-19. However, asymptomatic cases for COVID-19 were also described. The distributions of clinical variants and electrophysiological subtypes resemble those of classic GBS, with a higher prevalence of the classic sensorimotor form and the acute inflammatory demyelinating polyneuropathy, although rare variants like Miller Fisher syndrome were also reported. Cerebrospinal fluid (CSF) albuminocytological dissociation was present in around 71% cases, and CSF SARS-CoV-2 RNA was absent in all tested cases. More than 70% of patients showed a good prognosis, mostly after treatment with intravenous immunoglobulin. Patients with less favorable outcome were associated with a significantly older age in accordance with previous findings regarding both classic GBS and COVID-19. COVID-19-associated GBS seems to share most features of classic post-infectious GBS and possibly the same immune-mediated pathogenetic mechanisms. Nevertheless, more extensive epidemiological studies are needed to clarify these issues.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; COVID-19; Child; Female; Guillain-Barre Syndrome; Humans; Male; Middle Aged; SARS-CoV-2; Young Adult
PubMed: 32840686
DOI: 10.1007/s00415-020-10124-x -
Deutsches Arzteblatt International Nov 2018The new German S3 guideline on Lyme neuroborreliosis is intended to provide physicians with scientifically based information and recommendations on the diagnosis and...
BACKGROUND
The new German S3 guideline on Lyme neuroborreliosis is intended to provide physicians with scientifically based information and recommendations on the diagnosis and treatment of this disease.
METHODS
The scientific literature was systematically searched and the retrieved publications were assessed at the German Cochrane Center (Deutsches Cochrane Zentrum) in Freiburg in the 12 months beginning in March 2014. In addition to the main search terms "Lyme disease," "neuroborreliosis," "Borrelia," and "Bannwarth," 28 further terms relating to neurological manifestations of the disease were used for the search in the Medline and Embase databases and in the Cochrane Central Register of Controlled Trials.
RESULTS
In the treatment of early Lyme neuroborreliosis, orally administered doxycycline is well tolerated, and its efficacy is equivalent to that of intravenously administered beta-lactam antibiotics (penicillin G, ceftriaxone, and cefotaxime) (relative risk [RR]: 0.98, 95% confidence interval [CI]: [0.68; 1.42], P = 0.93). 14 days of treatment suffice for early Lyme neuroborreliosis, and 14-21 days of treatment usually suffice for late (chronic) Lyme neuroborreliosis.
CONCLUSION
Lyme neuroborreliosis has a favorable prognosis if treated early. The long-term administration of antibiotics over many weeks or even months for putative chronic Lyme neuroborreliosis with nonspecific symptoms yields no additional benefit and carries the risk of serious adverse effects.
Topics: Anti-Bacterial Agents; Borrelia; Doxycycline; Humans; Lyme Neuroborreliosis; Polyradiculopathy; Prognosis; Treatment Outcome
PubMed: 30573008
DOI: 10.3238/arztebl.2018.0751 -
Journal of Clinical Medicine Nov 2022POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy,... (Review)
Review
POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.
PubMed: 36498588
DOI: 10.3390/jcm11237011 -
Current Opinion in Neurology Oct 2018To review the recent advances in the management and treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP). (Review)
Review
PURPOSE OF REVIEW
To review the recent advances in the management and treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP).
RECENT FINDINGS
Recent studies confirm the efficacy/safety of long-term intravenous immunoglobulin (IVIg) and short-term subcutaneous immunoglobulin (SCIg) therapy in CIDP. New outcome measures have been recently proposed and further studies evaluated the properties of those already in use. The presence of antibodies against proteins at the node of Ranvier was associated with specific clinical features and treatment response. Fingolimod adds to the list of immunosuppressive agents that failed to be effective in a controlled trial.
SUMMARY
Several studies evaluating the best strategy to provide maintenance IVIg treatment in CIDP are in progress. SCIg were shown to be an alternative to IVIg for maintenance treatment while their efficacy as initial therapy should be further addressed. New outcome measures have been shown to be effective in detecting treatment response in clinical trials, but their use in clinical practice remains uncertain. Similarly unsettled is the role of nerve imaging techniques as biomarker in CIDP. The discovery of antibodies against proteins at the node of Ranvier has rekindled a keen interest in the pathogenesis of CIDP and the potential therapeutic role of new agents.
Topics: Fingolimod Hydrochloride; Humans; Immunization, Passive; Immunoglobulins, Intravenous; Immunosuppressive Agents; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Treatment Failure; Treatment Outcome
PubMed: 30045075
DOI: 10.1097/WCO.0000000000000595