Examination was notable for moderate dysarthria, hypermetric saccades and axial appendicular ataxia.[mdsabstracts.org] According to Baloh and others (1997), EA2 is characterized by a progressive ataxia with interictal rebound nystagmus evoked by gaze, as well as downbeating nystagmus.[tchain.com] Symptoms progress slowly; appendicular ataxia, dysmetria, dysdiadochokinesia, and cerebellar or mixed dysarthria (cerebellar and bulbar) appear at later stages. 14–16,22,23[elsevier.es]
[…] progressive over weeks to months and stabilized within 6 months • The majority had both truncal and appendicular ataxia Brain (2003), 126, 1409 1418 66.[slideshare.net] . ; EA2 with interictal nystagmus , is long-lasting (hours) associated with attacks of vertigo, diffuse weakness, slurred speech, dystonia, seizures, truncal and limb ataxia[neuroweb.us] 30 See the list below: Clinical features Age of onset - 42-76 years (mean is 52 y) Relatively pure, slowly progressing gait and appendicular ataxia Dysarthria Lower limb[emedicine.medscape.com]
In those with persistent cerebellar ataxia, gait, speech, heel-shin test and the finger-nose test were the highest scoring elements on the SARA, suggesting both appendicular[academic.oup.com] Most patients also show various interictal nystagmus, such as gaze-evoked nystagmus (GEN), rebound nystagmus, or primary position downbeat nystagmus.[e-jmd.org] […] and midline ataxia.[academic.oup.com]
Symptoms progress slowly; appendicular ataxia, dysmetria, dysdiadochokinesia, and cerebellar or mixed dysarthria (cerebellar and bulbar) appear at later stages. 14–16,22,23[elsevier.es] 30 See the list below: Clinical features Age of onset - 42-76 years (mean is 52 y) Relatively pure, slowly progressing gait and appendicular ataxia Dysarthria Lower limb[emedicine.com] […] features Nonprogressive ataxia Nystagmus Dysarthria Psychomotor display Clumsiness Cognitive impairment Frequent falling early in disease Dysdiadochokinesis Spinocerebellar ataxia[emedicine.com]
appendicular ataxia.[scielo.br] 30 See the list below: Clinical features Age of onset - 42-76 years (mean is 52 y) Relatively pure, slowly progressing gait and appendicular ataxia Dysarthria Lower limb[emedicine.medscape.com] The patient's mother died in Japan, at the age of 70 years old, presenting gait ataxia for more than ten years.[scielo.br]
30 See the list below: Clinical features Age of onset - 42-76 years (mean is 52 y) Relatively pure, slowly progressing gait and appendicular ataxia Dysarthria Lower limb[emedicine.com] […] features Nonprogressive ataxia Nystagmus Dysarthria Psychomotor display Clumsiness Cognitive impairment Frequent falling early in disease Dysdiadochokinesis Spinocerebellar ataxia[emedicine.com]
appendicular ataxia.[scielo.br] The patient's mother died in Japan, at the age of 70 years old, presenting gait ataxia for more than ten years.[scielo.br] Neurological examination showed bilateral horizontal gaze-evoked nystagmus, ocular dysmetria and dysarthria; severe gait ataxia associated with less prominent truncal and[scielo.br]
30 See the list below: Clinical features Age of onset - 42-76 years (mean is 52 y) Relatively pure, slowly progressing gait and appendicular ataxia Dysarthria Lower limb[emedicine.medscape.com] […] features Nonprogressive ataxia Nystagmus Dysarthria Psychomotor display Clumsiness Cognitive impairment Frequent falling early in disease Dysdiadochokinesis Spinocerebellar ataxia[emedicine.medscape.com]
[…] in the impairment of both appendicular and axial motor control.[movementdisorders.org] Degenerative cerebellar ataxias (CAs) are a group of disorders associated with progressive degeneration of the cerebellum, and its afferent and efferent pathways, resulting[movementdisorders.org]
We describe three patients with the Machado-Joseph disease (MJD) genetic abnormality who had non-movement disorder neurological symptoms or signs that preceded the gait ataxia by several years. This implies that some clinical manifestations other than ataxia may be considered part of the herald symptoms of MJD,[…][ncbi.nlm.nih.gov]