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Seminars in Neurology Dec 2015Although most neuromuscular disorders are recognized and treated in the outpatient clinic, a subset can present with rapidly advancing or severe weakness and constitute... (Review)
Review
Although most neuromuscular disorders are recognized and treated in the outpatient clinic, a subset can present with rapidly advancing or severe weakness and constitute true emergencies by threatening respiratory and bulbar function. This review focuses on the recognition, diagnosis, and management of neuromuscular diseases most likely to present in the inpatient setting: infectious motor neuronopathies, inflammatory polyradiculopathies, and disorders of neuromuscular transmission. In each, the prompt recognition, diagnosis, and institution of specific therapies or supportive care can prevent mortality and mitigate morbidity.
Topics: Disease Management; Emergencies; Humans; Neuromuscular Diseases
PubMed: 26595869
DOI: 10.1055/s-0035-1564303 -
British Journal of Hospital Medicine... Nov 2016
Topics: Diagnosis, Differential; Disease Management; Humans; Lumbosacral Region; Magnetic Resonance Imaging; Medical History Taking; Polyradiculopathy; Radiography; Sciatica; Symptom Assessment
PubMed: 27828743
DOI: 10.12968/hmed.2016.77.11.C180 -
Current Opinion in HIV and AIDS Nov 2014To present an overview of HIV-associated distal symmetric polyneuropathy (HIV-DSP) and other HIV-related peripheral neuropathies in the post-highly active retroviral... (Review)
Review
PURPOSE OF REVIEW
To present an overview of HIV-associated distal symmetric polyneuropathy (HIV-DSP) and other HIV-related peripheral neuropathies in the post-highly active retroviral therapy era.
RECENT FINDINGS
HIV-DSP has become the most common neurologic complication of HIV largely due to the prolonged survival of HIV-positive patients with the advent of highly active retroviral therapy. HIV-DSP can be attributed to the disease itself or to secondary effects of certain HAART agents, and often the two disease entities cannot be distinguished. HIV-DSP can lead to significant morbidity and interfere with daily activities. Diagnosis can be obtained from a detailed history and neurologic exam revealing absent ankle jerks and abnormal, vibratory perception or decreased pinprick or temperature. Supporting studies include nerve conduction studies and skin biopsy. Although there are no United States Food and Drug Administration-approved treatments for HIV-DSP, clinicians often use off-label medications, including antidepressants, anticonvulsants, topical agents and other analgesics.
SUMMARY
The prevalence of those affected by HIV-DSP will continue to grow with the aging population of HIV-infected individuals. Compared to the diabetic neuropathy drug trials, trials in both symptomatic and disease-modifying agents for HIV-DSP have had little success. Other forms of HIV-related peripheral neuropathies are discussed briefly, and include acute and chronic inflammatory demyelinating polyneuropathy, autonomic neuropathy, polyradiculopathy, mononeuropathies, mononeuritis multiplex, cranial neuropathies, and amyotrophic lateral sclerosis-like motor neuropathy.
Topics: Antiretroviral Therapy, Highly Active; HIV Infections; Humans; Peripheral Nervous System Diseases
PubMed: 25275705
DOI: 10.1097/COH.0000000000000103 -
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 -
Continuum (Minneapolis, Minn.) Feb 2021Cauda equina dysfunction (often referred to as cauda equina syndrome) is caused by a diverse group of disorders that affect the lumbosacral nerve roots. It is important... (Review)
Review
PURPOSE OF REVIEW
Cauda equina dysfunction (often referred to as cauda equina syndrome) is caused by a diverse group of disorders that affect the lumbosacral nerve roots. It is important to recognize dysfunction of the cauda equina quickly to minimize diagnostic delay and lasting neurologic symptoms. This article describes cauda equina anatomy and the clinical features, differential diagnosis, and management of cauda equina disorders.
RECENT FINDINGS
The diagnosis of disorders of the cauda equina continues to be a challenge. If a compressive etiology is seen, urgent neurosurgical intervention is recommended. However, many people with clinical features of cauda equina dysfunction will have negative diagnostic studies. If the MRI is negative, it is important to understand the diagnostic evaluation and differential diagnosis so that less common etiologies are not missed.
SUMMARY
Cauda equina dysfunction most often occurs due to lumbosacral disk herniation. Nondiskogenic causes include vascular, infectious, inflammatory, traumatic, and neoplastic etiologies. Urgent evaluation and surgical intervention are recommended in most cases of compressive cauda equina syndrome. Other types of treatment may also be indicated depending on the etiology.
Topics: Cauda Equina; Delayed Diagnosis; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Polyradiculopathy
PubMed: 33522743
DOI: 10.1212/CON.0000000000000903 -
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 -
Practical Neurology Feb 2022Chronic immune sensory polyradiculopathy (CISP) is a rare variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We describe a man with isolated...
Chronic immune sensory polyradiculopathy (CISP) is a rare variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We describe a man with isolated sensory ataxia whose initial investigations included normal nerve conduction studies and normal non-enhanced MR imaging of whole spine, but whose subsequent investigations showed delayed somatosensory evoked potential (SSEP) responses of the lower limbs, elevated cerebrospinal fluid (CSF) protein and lumbosacral nerve roots enhancement on MR imaging. We diagnosed CISP and he improved following intravenous immunoglobulin. Proposed diagnostic criteria for CISP are sensory symptoms with a polyneuropathic distribution without weakness, and normal motor and sensory nerve conduction and electromyography (EMG) studies, plus at least two of: abnormal SSEPs not due to central nervous system (CNS) involvement, MRI showing gadolinium enhancement or hypertrophy of the nerve roots, cauda equina or plexuses, and elevated CSF protein with normal cell count. Intravenous immunoglobulin is an effective treatment.
Topics: Contrast Media; Gadolinium; Humans; Magnetic Resonance Imaging; Male; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyradiculopathy
PubMed: 34740963
DOI: 10.1136/practneurol-2021-003070 -
International Orthopaedics Apr 2019Cauda equina syndrome is a devastating condition often following an innocent pathology in the form of a disc prolapse. The effect on sufferers, however, can be lifelong.... (Review)
Review
Cauda equina syndrome is a devastating condition often following an innocent pathology in the form of a disc prolapse. The effect on sufferers, however, can be lifelong. It is necessary to make a diagnosis as expeditiously as possible via adequate history, clinical examination and appropriate imaging to offer treatment, in the form of decompressive surgery within 48 hours. It is extremely important to communicate adequately with the patient and their family recording all the relevant details including those of expected outcome. National guidelines are likely to be of value to clinicians and patients.
Topics: Cauda Equina Syndrome; Decompression, Surgical; Humans; Intervertebral Disc Degeneration; Intervertebral Disc Displacement; Polyradiculopathy
PubMed: 30374638
DOI: 10.1007/s00264-018-4208-0 -
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