Oral Zinc Sulphate Supplementation for Six Months in SCA2 Patients: A Randomized, Double-Blind, Placebo-Controlled Trial

Velázquez‐Pérez, Luis; Rodriguez-Chanfrau, Jorge E.; García-Rodríguez, Julio César; Sánchez-Cruz, Gilberto; Aguilera-Rodríguez, Raúl; Rodríguez‐Labrada, Roberto; Rodríguez-Díaz, Julio Cesar; Canales-Ochoa, Nalia; Gotay, Dennis Almaguer; Mederos, Luis Enrique Almaguer; Mesa, José Miguel Laffita; Porto-Verdecia, Marlene; Triana, Consuelo González; Pupo, Noemí Rodríguez; Batista, Idania Hidalgo; López-Hernandez, Orestes D.; Polanco, Iverlis Díaz; Novas, Arelis Jayme

doi:10.1007/s11064-011-0496-0

Cited by 34 publications

(26 citation statements)

References 30 publications

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“…A mild decrease in SARA scores for gait, posture, stance and alternating hand movements and a reduced of saccadic latency were obseved. The treatment was also safe and well tolerated 25 .…”

Section: Symptomatic Treatmentmentioning

confidence: 86%

Current concepts in the treatment of hereditary ataxias

Neto

Pedroso

Kuo

et al. 2016

Arq. Neuro-Psiquiatr.

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Hereditary ataxias (HA) represents an extensive group of clinically and genetically heterogeneous neurodegenerative diseases, characterized by progressive ataxia combined with extra-cerebellar and multi-systemic involvements, including peripheral neuropathy, pyramidal signs, movement disorders, seizures, and cognitive dysfunction. There is no effective treatment for HA, and management remains supportive and symptomatic. In this review, we will focus on the symptomatic treatment of the main autosomal recessive ataxias, autosomal dominant ataxias, X-linked cerebellar ataxias and mitochondrial ataxias. We describe management for different clinical symptoms, mechanism-based approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis.

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“…A mild decrease in SARA scores for gait, posture, stance and alternating hand movements and a reduced of saccadic latency were obseved. The treatment was also safe and well tolerated 25 .…”

Section: Symptomatic Treatmentmentioning

confidence: 86%

Current concepts in the treatment of hereditary ataxias

Neto

Pedroso

Kuo

et al. 2016

Arq. Neuro-Psiquiatr.

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“…The treatment was safe and well tolerated. Zn supplementation was efficacious and safe in Cuban SCA2 patients [76]. SCA2 patients should be checked for lack of zinc concentration, and Zn supplementation may be started.…”

Section: Drug Therapy For Cerebellar Ataxiamentioning

confidence: 99%

Consensus Paper: Management of Degenerative Cerebellar Disorders

et al. 2013

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Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult. Yet there are recent developments that are likely to lead to significant improvements in the future. Most desirable would be a causative treatment of the underlying cerebellar disease. This is currently available only for a very small subset of cerebellar ataxias with known metabolic dysfunction. However, increasing knowledge of the pathophysiology of hereditary ataxia should lead to an increasing number of medically sensible drug trials. In this paper, data from recent drug trials in patients with recessive and dominant cerebellar ataxias will be summarized. There is consensus that up to date, no medication has been proven effective. Aminopyridines and acetazolamide are the only exception, which are beneficial in patients with episodic ataxia type 2. Aminopyridines are also effective in a subset of patients presenting with downbeat nystagmus. As such, all authors agreed that the mainstays of treatment of degenerative cerebellar ataxia are currently physiotherapy, occupational therapy, and speech therapy. For many years, well-controlled rehabilitation studies in patients with cerebellar ataxia were lacking. Data of recently published studies show that coordinative training improves motor function in both adult and juvenile patients with cerebellar degeneration. Given the well-known contribution of the cerebellum to motor learning, possible mechanisms underlying improvement will be outlined. There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed. Future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.

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“…Serum and cerebrospinal fluid levels of zinc are low in SCA2 patients. 37 A randomized, double-blind, placebo-controlled trial explored the role of zinc in the treatment of ataxia patients. Thirty-six SCA2 patients participated in the trial.…”

Section: Supplementsmentioning

confidence: 99%

Treatment Options in Degenerative Cerebellar Ataxia: A Systematic Review

Sarva

Shanker

2014

Movement Disord Clin Pract

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The etiology of cerebellar ataxia (CA) is heterogeneous and includes easily identified and often reversible causes (i.e., drug toxicity and vitamin B12 deficiency) as well as irreversible degenerative conditions. It is the latter that poses a significant therapeutic challenge for practitioners treating this population of patients. To date, there are no U.S. Food and Drug Administration-approved medications for the treatment of CA. The literature, consisting mostly of case reports, case series, and small clinical trials, is sparse and scattered. These studies are difficult to translate clinically because they often describe diverse study populations with various identified and unidentified genetic etiologies. In addition, the reported treatment duration is often brief, and it is uncertain whether any of these options provide substantially lasting benefits. In this article, we review published reports and studies to aid the practitioner counseling patients with degenerative ataxias.Degenerative cerebellar ataxias (CAs) are a group of disorders associated with progressive degeneration of the cerebellum, and its afferent and efferent pathways, resulting in the impairment of both appendicular and axial motor control. Patients often present with complaints of clumsiness, speech changes, and unsteady gait. These disorders can be classified into three major groups. The first group is the acquired ataxias resulting from causes such as toxins (e.g., alcohol), immune-mediated disorders (e.g., paraneoplastic cerebellar degeneration), vitamin deficiency (e.g., vitamin E), chronic central nervous system (CNS) infections (e.g., Creutzfeld-Jakob disease), and superficial siderosis. The other two groups are the hereditary and nonhereditary degenerative ataxias. 1 The inheritance pattern of the known hereditary ataxia disorders includes autosomal-dominant, autosomal-recessive, X-linked, and mitochondrial transmission. However, many patients present without a family history and have an unidentifiable etiology.The neurochemistry of CA is complex, suggesting a variety of possible targets for ataxia treatment. Though gamma-aminobutyric acid (GABA) and glutamate are the primary neurotransmitters associated with motor control, neurotransmitters such as serotonin, norepinephrine, acetylcholine, dopamine, and histamine are also responsible for normal cerebellar function, including motor learning. 2 Unfortunately, current practice offers no recommendations for first-line medical or physical therapy (PT).Several variables may influence the mixed outcomes of treatment studies, including the heterogeneity of the studied populations and the small sample size. The scattered publications addressing ataxia treatment, in addition to the lack of widely accepted guidelines, make it challenging for physicians to guide management in patients seeking comprehensive treatment, including PT. In this article, we offer the clinician an overview of the literature.To identify relevant publications, a PubMed search using the terms "treatments of cer...

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Oral Zinc Sulphate Supplementation for Six Months in SCA2 Patients: A Randomized, Double-Blind, Placebo-Controlled Trial

Cited by 34 publications

References 30 publications

Current concepts in the treatment of hereditary ataxias

Current concepts in the treatment of hereditary ataxias

Consensus Paper: Management of Degenerative Cerebellar Disorders

Treatment Options in Degenerative Cerebellar Ataxia: A Systematic Review

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