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| Reports by JD Nutt |
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Cerebella Ataxia... |
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| Published 5:43 p.m. August 13th, 2019 | Updated 8.15.2019 | ||
| Index > ataxia | ||
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| Ataxia Explained... | ||
| Ataxia is a degenerative disease of the nervous system. Many symptoms of Ataxia mimic those of being drunk, such as slurred speech, stumbling, falling, and the lack of coordination. These symptoms are caused by damage to the cerebellum, the part of the brain that is responsible for coordinating movement. Ataxia treatment involves a combination of medication to treat symptoms and therapy to improve quality of life. | ||
| People affected by Ataxia may experience problems with using their fingers and hands, arms, legs, walking, speaking or moving their eyes. Ataxia affects people of all ages. Age of symptom-onset can vary widely, from childhood to late-adulthood. Complications from the disease are serious and oftentimes debilitating. Some types of Ataxia can lead to an early death. | ||
| No cures are possible for most patients who suffer debilitating movement disorders called cerebellar ataxias. | ||
| But in a few of these disorders, patients can be effectively treated with regimens such as prescription drugs, high doses of vitamin E and gluten-free diets, according to a review article in the journal Movement Disorders. "Clinicians must become familiar with these disorders, because maximal therapeutic benefit is only possible when done early. These uncommon conditions represent a unique opportunity to treat incurable and progressive diseases," first author Adolfo Ramirez-Zamora, MD, co-author José Biller, MD, and colleagues report. Dr. Ramirez-Zamora is an assistant professor of neurology and the Phyllis E. Dake Endowed Chair in Movement Disorders at Albany Medical Center Department of Neurology, and a former chief neurology resident at Loyola University Chicago. Dr. Biller is chair of the Department of Neurology of Loyola University Chicago Stritch School of Medicine. The word ataxia means without co-ordination. Ataxia symptoms include poor coordination, unsteady walk, difficulty speaking and swallowing, involuntary back-and-forth eye movements and difficulty performing fine-motor skills such as writing or buttoning a shirt. Hereditary ataxias are degenerative and progress over time. Ataxias usually are due to damage to the cerebellum, a part of the brain that controls muscle coordination. Chronic cerebellar injury due to alcohol or other commonly used drugs such as lithium can be treated by discontinuing the offending drugs, the review article said. Below is a sampling of other effective treatments for cerebellar ataxias detailed in the review article: Ataxia with vitamin E deficiency (AVED) impairs the body's ability to use vitamin E, resulting in ataxia and other debilitating symptoms. High doses of vitamin E (800 mg/d) typically stop disease progression and lead to neurological improvement -- although recovery may be slow and incomplete. "The results of vitamin E supplementation also seem to be most beneficial if started in patients with less than 15 years of disease duration; the sooner after diagnosis the supplementation is begun, the better," the article said. Cerebrotendinous xanthomatosis (CTX) is treated with chenodeoxycholic acid, which is made from naturally occurring bile acid. Beginning treatment early is crucial to preventing neurological deterioration. Gluten ataxia can be treated with a strict gluten-free diet. Glutose transporter type 1 deficiency can be treated with a high-fat, low-carb ketogenic diet. Glutamic acid decarboxylase ataxia can be treated with intravenous immunoglobulin (IVIG) or steroids. |
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| Treatable causes of cerebellar ataxia... | ||
| The cerebellar ataxia syndromes are a heterogeneous group of disorders clinically characterized by the presence of cerebellar dysfunction. Initial assessment of patients with progressive cerebellar ataxia is complex because of an extensive list of potential diagnoses. A detailed history and comprehensive examination are required for an accurate diagnosis and hierarchical diagnostic investigations. Although no cure exists for most of these conditions, a small group of metabolic, hereditary, inflammatory, and immune-mediated etiologies of cerebellar ataxia are amenable to disease-modifying, targeted therapies. Over the past years, disease-specific treatments have emerged. Thus, clinicians must become familiar with these disorders because maximal therapeutic benefit is only possible when done early. In this article, we review disorders in which cerebellar ataxia is a prominent clinical feature requiring targeted treatments along with specific management recommendations. | ||
| What imaging studies will be helpful in making or excluding the diagnosis of varicella-zoster virus infection? Imaging studies are not required in the routine management of either chickenpox or shingles. However, with both infections, complications can occur that require imaging. For example, chickenpox can cause a syndrome of cerebella ataxia or, rarely, encephalitis. With shingles, granulomatous arteritis can occur after the infection has resolved. In both circumstances, a magnetic resonance imaging (MRI) scan or a magnetic resonance angiogram (MRA) would be of value in delineating the pathology. The costs of MRI scans and MRAs vary dramatically from one institution to another. For adults, and especially pregnant women, with chickenpox, a chest radiograph is important to assess whether pneumonia is present. What consult service or services would be helpful for making the diagnosis and assisting with treatment? An infectious diseases consultation should be sought for an immunocompromised patient who has VZV infection. If you decide the patient has this disease, what therapies should you initiate immediately? Chickenpox in the normal host is usually a benign infection and is treated at the discretion of both the family and physician. Chickenpox in the immunocompromised host should always be treated. Shingles should be treated regardless of the age of the patient. 1. Anti-infective agents If I am not sure what pathogen is causing the infection what anti-infective should I order? Empiric therapy for the immune-competent adult without complications should be either valaciclovir or famciclovir. The oral suspension of aciclovir is used in children who are thought to require therapy. Table I summarizes treatment options. Of note, the second generation antiviral drugs, valaciclovir and famciclovir, are superior to aciclovir and have improved pharmacokinetics and pharmacodynamics. However, neither is available in a useful pediatric formulation. |
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| Granulomatous arteritis... | ||
| Granulomatous, or temporal, arteritis is a disease of older people and should be considered when any patient over 55 years old has complaints consistent with a multi-system disease and/or visual loss, especially if combined with a history of diplopia, amaurosis fugax, loss of vision, or with the findings of ischemic optic neuritis or central retinal artery occlusion. Treatment should be instituted immediately with high doses of corticosteroids. The sine qua non for the diagnosis is a positive temporal artery biopsy when ocular involvement is a feature of the disease. Treatment includes administration of systemic and retrobulbar steroids. The ESR is used as a guide in determining the level of maintenance steroid dosage. | ||
| Encephalitis... | ||
| Encephalitis (en-sef-uh-LIE-tis) is inflammation of the brain. There are several causes, but the most common is viral infection. Encephalitis often causes only mild flu-like signs and symptoms — such as a fever or headache — or no symptoms at all. Sometimes the flu-like symptoms are more severe. Encephalitis can also cause confused thinking, seizures, or problems with senses or movement. Rarely, encephalitis can be life-threatening. Timely diagnosis and treatment are important because it's difficult to predict how encephalitis will affect each individual. |
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| Sources: | ||
| https://www.infectiousdiseaseadvisor.com/home/decision-support-in-medicine/infectious-diseases/varicella-zoster-virus-vzv/ | ||
| https://ataxia.org/what-is-ataxia/ | ||
| https://www.sciencedaily.com/releases/2015/03/150323150536.htm | ||
| https://www.ncbi.nlm.nih.gov/pubmed/1222548 | ||
| https://onlinelibrary.wiley.com/doi/abs/10.1002/mds.26158 | ||
| https://www.mayoclinic.org/diseases-conditions/encephalitis/symptoms-causes/syc-20356136 |
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