An Unexpected Schwannoma of the Phrenic Nerve
Wickii T. Vigneswaran in Thoracic Surgery, 2019
Schwannomas are most commonly benign but can be slow-growing and have the potential for malignant transformation [6]. They are frequently asymptomatic at presentation, often discovered incidentally on imaging. When they have symptoms, most commonly, it is related to paralysis of the affected nerve. CT or MRI will often demonstrate the mass, sometimes with cystic areas apparent if IV contrast is given. A PET/CT may show variable levels of uptake [7]. Histologically, schwannomas show a mixture of two distinct patterns: Antoni A and B. Antoni A areas are highly cellular consisting of spindle cells, while Antoni B areas are hypocellular in a connective tissue stroma. In addition, Antoni B areas are noted to be prone to degeneration. Immunohistochemically, schwannomas often show strong S-100 positivity [6].
Enteroviruses
Avindra Nath, Joseph R. Berger in Clinical Neurovirology, 2020
Acute flaccid paralysis usually begins with fever, flu-like symptoms, and sometimes muscle cramps followed by muscle weakness in one or more limbs. The prodromal symptoms may not occur with EV70. Paralysis is usually asymmetrical, flaccid, more proximal than distal, and often patchy [70]. The reflexes are lost as paralysis progresses. Over the next several days, paralysis may develop in other extremities and bulbar involvement with impaired respiration may occur. Extension of paralysis is unlikely to occur after the fifth or sixth day. Paralysis caused by coxsackievirus and ECHO are usually mild compared with that seen with PV, EV70, and EV71. Generally, the weakened muscles regain some strength. The differential diagnosis of paralytic disease should thus include all of the enteroviruses that can cause paralysis (Table 17.4). In addition, several other viruses, e.g., rabies [64], herpes zoster [71], and West Nile virus [72], can occasionally cause acute lower motor neuron paralysis. Other entities in the differential include acute inflammatory polyradiculitis (Guillain–Barré syndrome), botulism, acute toxic neuropathies, acute intermittent porphyria, acute transverse myelitis, and acute spinal cord compression from epidural abscess [73–75].
Polio
Rae-Ellen W. Kavey, Allison B. Kavey in Viral Pandemics, 2020
This was one of many similar outbreaks in western Europe and North America reported at around this time, characterized by the same insidious onset, primarily in young children in the summer months and with the same serious residua of localized paralysis in up to half of symptomatic survivors. In 1907 and in 1911, after devastating polio epidemics in Sweden, Ivar Wickman published three important facts based on his careful observation of polio outbreaks in families in isolated rural communities. First, he confirmed the seasonal occurrence of the disease in late summer and early fall. More importantly, he reported that polio was spread directly from person to person and that there was subclinical infection, based on his observations in a small, isolated community that a new case in a family without polio often occurred after a visit from an asymptomatic individual whose family had an affected member. Finally, he identified the community school as the common source of the infections and deduced that apparently healthy children carried the disease home, where family members developed varied clinical presentations. He was the first to recognize that polio could be present in completely asymptomatic people and identified the importance of these cases in the spread of the disease.11 In addition, if infected individuals could be asymptomatic, the actual rates of paralytic disease and mortality were far lower than in original descriptions of the disease. Nonetheless, each summer brought terror to the hearts of parents all over the developed world.
Overview of stem cells therapy in amyotrophic lateral sclerosis
Published in Neurological Research, 2021
Goun Je, Kiandokht Keyhanian, Mehdi Ghasemi
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by degeneration of upper and lower motor neurons (Figure 1) [1]. Typically, it begins with focal muscle weakness and relentlessly progresses to affect most of the skeletal muscles, causing complete paralysis. Rapid disease progression limits the survival to 3–5 years after disease onset, mainly due to respiratory paralysis [2]. Both familial and sporadic forms of ALS are reported [3]. The familial component is shown in approximately 5–10% of the disease with associated genetic abnormalities, including copper-zinc superoxide dismutase 1 (SOD1), chromosome 9 open reading frame 72 (C9orf72), fused in sarcoma (FUS), TAR DNA-binding protein 43 (TARDBP) genes, and other related genes [4,5]. However, the vast majority of ALS patients are sporadic (90–95%) with unclear pathogenic mechanisms, which makes it difficult to develop effective therapies for ALS. Currently, ALS is considered as an incurable disease. Treatment options are limited to respiratory supports and symptomatic management. So far, only two disease-modifying therapies are U.S. Food and Drug Administration (FDA) approved; Riluzole and Edaravone, providing modest benefits in some patients with prolonging median survival by about 2–3 months or slightly reducing the disease progression rate in the early stages of disease [6–8].
SOD1-targeting therapies for neurodegenerative diseases: a review of current findings and future potential
Published in Expert Opinion on Orphan Drugs, 2020
John P. Franklin, Mimoun Azzouz, Pamela J. Shaw
Amyotrophic lateral sclerosis (ALS) is a devastating adult-onset neurodegenerative disorder characterized by progressive loss of upper and lower motor neurons. Clinically, this manifests as atrophy and spasticity of skeletal muscle, leading to paralysis. Dysphagia and dysarthria may also occur when bulbar muscles are affected. The prognosis is invariably fatal: survival time from symptom onset to death from respiratory weakness is typically between 2 and 4 years [1]. There is no cure currently available: one medication, Riluzole, has been used for many years and has been shown to modestly prolong life [2]. Another drug, Edaravone has since been licensed and may prolong functional independence in a subgroup of patients [3,4]. Recently, early phase clinical trials of two gene silencing therapies targeting SOD1 have been published with some preliminary evidence of disease-modifying activity in patients with ALS caused by mutations in the SOD1 gene [5,6]. It is hoped that these breakthroughs may herald a new era of personalized medicine for monogenic causes of ALS and other neurodegenerative diseases [7].
Inpatient management and post-discharge outcomes of hyperkalemia
Published in Hospital Practice, 2021
Jill Davis, Rubeen Israni, Fan Mu, Erin E. Cook, Harold Szerlip, Gabriel Uwaifo, Vivian Fonseca, Keith A. Betts
Hyperkalemia is an electrolyte disorder characterized by elevated levels of serum potassium [1]. Symptoms include muscle weakness, paralysis, and cardiac dysfunction (e.g., arrhythmia), which may be life-threatening [2,3]. Risk factors for hyperkalemia include comorbidities (e.g., chronic kidney disease [CKD], hypertension, type 2 diabetes), medications (e.g., renin-angiotensin-aldosterone system inhibitors [RAASi]), and high potassium intake [4,5]. In 2014, the annual prevalence of hyperkalemia among adults in the general population of the United States (US) was estimated at 1.55% (3.7 million) [6]. However, the prevalence of hyperkalemia can vary by condition. For example, 6.35% of adults with CKD and/or heart failure were estimated to have hyperkalemia and 41.2% of pre-dialysis patients with end-stage kidney disease (ESKD) were estimated to have hyperkalemia [6,7]. The clinical and economic burden associated with hyperkalemia is considerable. Patients with hyperkalemia were reported to incur two to three times the all-cause health costs compared to patients without hyperkalemia ($31,844 vs. 15,861 USD), a difference which persisted after adjustment for comorbid conditions, age, and gender [8]. In that same analysis, patients with hyperkalemia had twice as many hospital visits and longer lengths of stay compared to patients without hyperkalemia [8]. The clinical and economic burden associated with hyperkalemia is expected to increase as the prevalence of associated risk factors continues to rise [9].
Related Knowledge Centers
- Nerve Injury
- Nervous System
- Stroke
- Spinal Cord
- Skeletal Muscle
- Peripheral Neuropathy
- Cerebral Palsy
- Motor Skill
- Injury
- Polio