• The common assumption that cerebellar ataxia of stance does not improve with visual feedback is true only of vestibulocerebellar lesions, not for ataxia resulting from atrophy of the anterior lobe of the cerebellum. (nih.gov)
  • A feature of cerebellar ataxia is dysmetria, which is characterized by inaccurate movements. (nih.gov)
  • Ramsay Hunt syndrome (RHS) type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome. (wikipedia.org)
  • Onset of symptoms usually occur in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks. (wikipedia.org)
  • A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. (bvsalud.org)
  • Myoclonic jerks of the trunk and the extremities, particularly on intention, occur in some patients with ataxia-telangiectasia but not before age 9 or 10 years. (medscape.com)
  • Intact sensation and a negative Romberg sign are helpful in differentiating the cerebellar ataxia of ataxia-telangiectasia from Friedreich ataxia, in which the ataxia is predominantly spinal or sensory and the Romberg sign is positive. (medscape.com)
  • Cerebellar tremor may be accompanied by other manifestations of ataxia , including dysarthria (speech problems), nystagmus (rapid, involuntary rolling of the eyes), gait problems and postural tremor of the trunk and neck. (wikipedia.org)
  • Cerebellar tremor appears to result from a central mechanism, but is modulated or provoked through increased long-loop EMG responses. (nih.gov)
  • Dyssynergia and intention tremor of the extremities become prominent features with age. (medscape.com)
  • Cerebellar tremor (also known as intention tremor ) is a slow, broad tremor of the extremities that occurs at the end of a purposeful movement, such as trying to press a button or touching a finger to the tip of one's nose. (wikipedia.org)
  • Cerebellar tremor is caused by lesions in or damage to the cerebellum resulting from stroke, tumor, or disease such as multiple sclerosis or some inherited degenerative disorder. (wikipedia.org)
  • In classic cerebellar tremor, a lesion on one side of the brain produces a tremor in that same side of the body that worsens with directed movement. (wikipedia.org)
  • Cerebellar damage can also produce a "wing-beating" type of tremor called rubral or Holmes' tremor - a combination of rest, action, and postural tremors. (wikipedia.org)
  • Titubation is tremor of the head and is of cerebellar origin. (wikipedia.org)
  • It is named for James Ramsay Hunt who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921. (wikipedia.org)
  • Myoclonic jerks commonly occur in persons with epilepsy, a disorder in which the electrical activity in the brain becomes disordered and leads to seizures. (malacards.org)
  • RHS type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease, dentatorubropallidoluysian atrophy, and celiac disease. (wikipedia.org)
  • Cerebellar cortical atrophy was present in 16 of 17 and cerebral atrophy in 17 of 17. (ajnr.org)
  • Human and animal experiments performed recently have resulted in a more detailed understanding of limb movement and body posture disorders associated with cerebellar dysfunction. (nih.gov)
  • A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. (ctdbase.org)
  • Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (bvsalud.org)
  • Fifteen of 17 patients showed symmetrically decreased T1 and increased T2 signal intensity in cerebellar white matter lateral, superior, and inferior to the dentate nuclei. (ajnr.org)
  • In this review, we have mainly discussed the cerebellar ataxic gait. (nih.gov)
  • The delay in movement initiation can be explained by a delay in onset of phasic motor cortex neural discharge owing to decreased input from the cerebellar hemispheres. (nih.gov)
  • Hypotonia can be observed only in acute cerebellar lesions. (nih.gov)
  • Oculomotor abnormalities in Dyssynergia cerebellaris myoclonica. (naver.com)
  • MR imaging studies of the brain of 17 male patients were characterized for signal intensity and for size of ventricles, cerebral and cerebellar sulci, and brain stem. (ajnr.org)
  • Slow initiation and performance of all voluntary activity and muscular hypotonia are characteristic and are also manifestations of cerebellar symptomatology. (medscape.com)
  • Fourteen of 17 had similar signal intensity alterations in the middle cerebellar peduncles. (ajnr.org)
  • SCAR18 features include progressive cerebellar atrophy, delayed psychomotor development, severely impaired gait, ocular movement abnormalities, and intellectual disability . (symptoma.com)
  • movement abnormalities , cerebellar atrophy on brain imaging, and intellectual disability (summary by Lise et al. (symptoma.com)
  • PMID: 25148684) Mas C … Collins BM (The Journal of biological chemistry 2014) 3 4 60 Mutations in SNX14 cause a distinctive autosomal-recessive cerebellar ataxia and intellectual disability syndrome. (symptoma.com)