Treatable causes of cerebellar ataxia

  title={Treatable causes of cerebellar ataxia},
  author={Adolfo Ramirez-Zamora and Warren Zeigler and Neeja Desai and Jose Biller},
  journal={Movement Disorders},
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… 
Current concepts in the treatment of hereditary ataxias.
This review describes management for different clinical symptoms, mechanism-based approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis for Hereditary ataxias.
Drugs in Selected Ataxias
No symptomatic drug treatment is available which ameliorates the clinical signs and symptoms of ataxia, and the only exceptions are aminopyridines for treatment of downbeat nystagmus, and acetazolamide and aminocyanine for Treatment of episodic ataxias.
Cerebellar ataxia with sensory ganglionopathy; does autoimmunity have a role to play?
This case series highlights that amongst patients with the unusual combination of cerebellar ataxia and SG, immune pathogenesis plays a significant role.
Cerebellar ataxia and sensory ganglionopathy associated with light-chain myeloma
The rarity of these conditions and the absence of an alternative aetiology for the neurological dysfunction argue in favour of a paraneoplastic phenomenon.
Three Adult-Onset Autosomal Recessive Ataxias: What Adult Neurologists Need to Know.
This review will cover the genetics, clinical presentation, and necessary diagnostic steps required to identify 3 causes of Autosomal Recessive Cerebellar Ataxia that manifest differently in adults vs children.
Cerebellar involvement in patients withprimary Sjögren’s syndrome: diagnosis and treatment
The clinical features of cerebellar involvement in patients with primary Sjögren’s syndrome (pSS) are described and early diagnosis and empirical aggressive glucocorticoid treatment is warranted.
Neurological gait disorders in childhood Authors
This review focuses on some of the more common movement disorders resulting in inserted postures, inserted movements, and impairment of motor control (including ataxia and neuro-muscular disorders).
A case of acute cerebellar ataxia with two possible etiologies: viral cerebellitis and paraneoplastic antibody-positive ovarian cancer
A case of a 47-year-old female presenting with acute cerebellar ataxia diagnosed with both Epstein-Barr virus (EBV)-associated cerebellitis and anti-Yo antibody-positive ovarian cancer is reported.
Neurological gait disorders in childhood


Consensus Paper: Management of Degenerative Cerebellar Disorders
There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed, and future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.
Sporadic ataxia with adult onset: classification and diagnostic criteria
Primary episodic ataxias: diagnosis, pathogenesis and treatment.
This work summarizes current knowledge of clinical and genetic diagnosis, genotype-phenotype correlations, pathophysiology and treatment of episodic ataxia syndromes, and focuses on unresolved issues including phenotypic and genetic heterogeneity.
An approach to the patient with late-onset cerebellar ataxia
An 83-year-old man presented with hypertension, hyperlipidemia, and a previous basal cell carcinoma, having developed progressive worsening of his balance and difficulty walking at the age of 78 years, which was subsequently confirmed by gene testing as spinocerebellar ataxia type 6 (SCA6).
Familial episodic ataxia: Clinical heterogeneity in four families linked to chromosome 19p
Clinical and oculographic findings in 4 families with episodic ataxia and interictal nystagmus (EA‐2) linked to chromosome 19p showed localizing to the vestibulocerebellum and posterior vermis, suggesting involvement of the cerebellum, brainstem, and cortex.
Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis
Cerebellar degeneration in these patients with raised antithyroid antibodies may be immune mediated, and “Hashimoto's associated ataxia” seems to represent a recognisable and not uncommon condition; a trial of immunomodulating therapy should be considered.
Treatment of Paraneoplastic Cerebellar Degeneration
  • J. Greenlee
  • Medicine, Biology
    Current Treatment Options in Neurology
  • 2013
Evidence-based treatment strategies for paraneoplastic cerebellar degeneration do not exist, but cyclophosphamide, tacrolimus, rituximab, or possibly mycophenolate mofetil may warrant consideration in patients who fail to stabilize or improve on less aggressive therapies.
The aetiology of sporadic adult-onset ataxia.
The study performed in 112 patients who met the following inclusion criteria: progressive ataxia; onset after 20 years; informative and negative family history; and no established symptomatic cause.
Hashimoto’s Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration
It is suggested that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of Hashimoto’s encephalopathy, but that nystagmus and severe Cerebellar atrophy are uncommon.
Hashimoto encephalopathy presenting with progressive cerebellar ataxia
A patient with Hashimoto encephalopathy, who presented with progressive cerebellar ataxia with mild abnormality on electroencephalography (EEG) and who showed marked improvement after steroid administration, is described, emphasises that HashimotoEncephalopathy can present with isolated cerebellary ataxIA and is responsive to immunosupression.