Appendicular Ataxia: Causes & Reasons

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Possible Causes for Appendicular Ataxia

Autosomal Recessive Spinocerebellar Ataxia 8

Examination was notable for moderate dysarthria, hypermetric saccades and axial>appendicular ataxia. [mdsabstracts.org]

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.medscape.com]

Cerebellar Ataxia - Neuropathy - Vestibular Areflexia Syndrome

ataxia, dysarthria and horizontal gaze-evoked nystagmus. [ncbi.nlm.nih.gov]

Cerebellar dysfunction DusanPavlovic.com  gaze evoked Ny, downbeat Ny  saccadic smooth pursuit  gait ataxia  dysarthria  appendicular ataxia March 30th 2015 6. [slideshare.net]

There was marked cerebellar dysarthria, appendicular ataxia, and a positive Romberg s test. [docplayer.net]

Adult-Onset Autosomal Recessive Cerebellar Ataxia

[…] 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]

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]

Clinical features of cerebellar disease  Ataxia (appendicular or axial)  Dysmetria  Dyssynergia  Dysdiadochokinesia  Rebound Phenomenon  Dysarthria  Tremor  Titubation [slideshare.net]

Spinocerebellar Ataxia Type 19

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]

Spinocerebellar Ataxia Type 13

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]

Spinocerebellar Ataxia Type 10

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]

Spinocerebellar Ataxia Type 23

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]

Spinocerebellar Ataxia Type 1

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]

Spinocerebellar Ataxia Type 5

[…] 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]

Spinocerebellar Ataxia Type 2

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]

Machado-Joseph Disease

Machado-Joseph disease (MJD) is an autosomal dominant neurodegenerative disorder originally described in families of Portuguese-Azorean ancestry. The cloning of the MJD1 gene allowed identification of the disease in many other populations, and MJD is now known to be the most common cause of dominant[…] [ncbi.nlm.nih.gov]

Spinocerebellar Ataxia Type 15

BACKGROUND: To guide time- and cost-efficient analyses of the increasing number of autosomal-dominant spinocerebellar ataxia genes (SCAs), more information about frequency distributions, phenotypic characteristics and optimal diagnostic strategies is warranted. OBJECTIVE: To assess the prevalence and phenotypic[…] [ncbi.nlm.nih.gov]

Spinocerebellar Ataxia Type 26

Talk to our genetiec expert 55 644 5590 All the samples are tested by 2 different teams to get accurate test results. The samples are processed at Genetic DNA Lab facility. Test Cost : 3000.00 AED Reporting Time: 4 Week Sample Type: EDTA blood or DNA Spinocerebellar ataxia type 26 autosomal dominant (EEF2)[…] [dnalabsuae.com]

Ataxia-Oculomotor Apraxia Syndrome

December 14, 2004 ; 63 (11) Brief Communications Late onset and variable phenotype C. Criscuolo, P. Mancini, F. Saccà, G. De Michele, A. Monticelli, L. Santoro, V. Scarano, S. Banfi, A. Filla First published December 13, 2004, DOI: https://doi.org/10.1212/01.WNL.0000145604.57000.36 This article requires a subscription to view the[…] [neurology.org]

Alpha-Mannosidosis

Alpha-mannosidosis is a rare lysosomal storage disorder with an autosomal recessive inheritance. Deficient alpha-mannosidase activity leads to lysosomal accumulation of mannose-rich oligosaccharides. The disease characterized by mental retardation, skeletal changes, hearing impairment, and recurrent infections. Stem[…] [ncbi.nlm.nih.gov]

Pilomyxoid Astrocytoma

Upon neurological examination, we found a Karnofsky Performance Status Scale (KPS) score of 70, a gait deviation to the left, an appendicular ataxia and palsy of the left [pagepress.org]

Progressive Myoclonic Epilepsy Type 8

Relatives initially noted appendicular ataxia at the age of 1‐year, followed by a progressive delay in developmental milestones, particularly in language. [onlinelibrary.wiley.com]

Neurological exam disclosed dysarthria, truncal and appendicular ataxia, bilateral horizonto‐torsional nystagmus, choreoatethoic movements in his fingers, slight dystonic [onlinelibrary.wiley.com]

Not described Not described Not described Not described Truncal and appendicular ataxia Gait impairement Wheelchair‐bound Unable to walk without support Not described Not [onlinelibrary.wiley.com]

Hereditary Cerebellar Degeneration

This is another indication of appendicular ataxia. Tests for Truncal Ataxia Truncal ataxia presents as an unsteady gait. [kenhub.com]

ataxia, dysarthria). [els.net]

ataxia). [6] These include: intention tremor (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the [en.wikipedia.org]

Marinesco-Sjögren Syndrome

Mitochondrial myopathy in Marinesco-Sjögren syndrome T Torbergsen et al. J Ment Defic Res. 1991 Apr. Abstract Myopathy represents one of the major features of Marinesco-Sjögren syndrome (MSS). Seven patients with MSS from three different families were studied with morphological and neurophysiological methods. In[…] [pubmed.ncbi.nlm.nih.gov]

Gerstmann-Straussler-Scheinker Disease

Autosomal Recessive Progressive External Ophthalmoplegia

Hum Mol Genet. 2012 Jan 1;21(1):66-75. doi: 10.1093/hmg/ddr438. Epub 2011 Sep 21. Tyynismaa H 1, Sun R, Ahola-Erkkilä S, Almusa H, Pöyhönen R, Korpela M, Honkaniemi J, Isohanni P, Paetau A, Wang L, Suomalainen A. Author information 1 Research Programs Unit, Molecular Neurology, Biomedicum Helsinki, Haartmaninkatu 8, Helsinki[…] [ncbi.nlm.nih.gov]

Cerebellar Mass Lesion

[…] cord — > gait abnormalities Truncal Ataxia versus Appendicular Ataxia Truncal Ataxia Caused by lesions confined to the cerebellar vermis Affect primarily the medial motor [brainaacn.org]

Cause of trunkal ataxia Cause of appendicular ataxia Appropriate tests? Positive signs? [quizlet.com]

and eye movement abnormalities Lesions of the intermediate part of the cerebellar hemisphere cause mainly ataxia of the limbs (i.e., appendicular ataxia) Ataxia is often [brainaacn.org]

Opsoclonus-Myoclonus Syndrome

The mildest cases of acute cerebellar ataxia represent a benign condition that is characterized by acute truncal and gait ataxia, variably with appendicular ataxia, nystagmus [ncbi.nlm.nih.gov]

Patient had severe truncal and appendicular ataxia, along with truncal and limb myoclonus. His myoclonic movements markedly increased on attempted movement. [ijo.in]

Spinocerebellar Ataxia with Rigidity and Peripheral Neuropathy

ataxia abulia, behavioural changes unilateral spatial neglect memory loss transcortical motor aphasia vertical gaze palsy third nerve palsy + contralateral limb movement [lifeinthefastlane.com]

He had no appendicular ataxia, dysarthria, resting limb tremor, bradykinesia for activities of daily living, appendicular or axial rigidity, facial grimacing, nuchal or truncal [pmj.bmj.com]

[…] 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]

Vestibulocerebellar Ataxia

It is involved in gait stability. [3] Truncal ataxia is different from appendicular ataxia. Appendicular ataxia affects the movements of the arms and legs. [en.wikipedia.org]

ataxia, dysarthria and horizontal gaze-evoked nystagmus... [read.qxmd.com]

ataxia). [6] These include: intention tremor (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the [en.wikipedia.org]

Foville Syndrome

Afferent fibers through the middle cerebellar peduncles: this can present with ipsilateral appendicular ataxia. [statpearls.com]

Opsoclonus

Patient had severe truncal and appendicular ataxia, along with truncal and limb myoclonus. His myoclonic movements markedly increased on attempted movement. [ijo.in]

She was unable to stand independently secondary to severe truncal ataxia. She also showed significant appendicular ataxia during finger to nose testing. [file.scirp.org]

Neurologic examination revealed spontaneous, involuntary, arrhythmic and conjugate rapid eye movements; facial, axial and appendicular myoclonus and gait ataxia. [academic.oup.com]

Subacute Cerebellar Degeneration

ataxia, dysarthria). [els.net]

It is characterised by subacute onset of gait instability which may progress to a pan-cerebellar syndrome with vertigo, diplopia, dysarthria, truncal and appendicular ataxia [karger.com]

Clinically it presents with appendicular and truncal ataxia, dysarthria, and nystagmus. [omicsonline.org]

Acute Cerebellar Ataxia

ataxia, dysarthria). [els.net]

[…] divided into syndromes that predominantly affect the midline cerebellar structures (gait and ocular dysfunction predominant) or the cerebellar hemisphere(s) (ipsilateral appendicular [els.net]

Axonal Neuropathy

Conclusions: The patient’s presentation with severe truncal and appendicular ataxia/myoclonus drove more attention, however the antecedent diarrheal illness, distal onset [n.neurology.org]