Idiopathic very late-onset cerebellar ataxia: a Brazilian case series. 2015

Hélio A G Teive, and Mariana Moscovich, and Adriana Moro, and Marina Farah, and Walter O Arruda, and Renato P Munhoz
Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.

The authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy. METHODS 26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG. RESULTS 62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients. CONCLUSIONS We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D008609 Mental Status Schedule Standardized clinical interview used to assess current psychopathology by scaling patient responses to the questions.
D001938 Brazil A country located on the eastern coast of South America, located between Colombia and Peru, that borders the Atlantic Ocean. It is bordered on the north by Venezuela, Guyana, Suriname, and French Guiana, on the south by Uruguay, and on the west by Argentina. The capital is Brasilia.
D002531 Cerebellum The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills. Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D002819 Chorea Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as CHOREATIC DISORDERS. Chorea is also a frequent manifestation of BASAL GANGLIA DISEASES. Choreatic Disorders,Choreiform Movement,Chorea Disorders,Chorea Syndromes,Chorea, Benign Hereditary,Chorea, Chronic Progressive,Chorea, Rheumatic,Chorea, Senile,Chorea, Sydenham,Choreatic Syndromes,Choreic Movement,Dyskinesias, Paroxysmal,Hereditary Chorea,Hereditary Progressive Chorea Without Dementia,Paroxysmal Dyskinesias,Rheumatic Chorea,Senile Chorea,St. Vitus's Dance,Sydenham Chorea,Sydenham's Chorea,Benign Hereditary Chorea,Benign Hereditary Choreas,Chorea Disorder,Chorea Syndrome,Chorea, Hereditary,Chorea, Sydenham's,Choreas,Choreas, Benign Hereditary,Choreas, Chronic Progressive,Choreas, Hereditary,Choreas, Rheumatic,Choreas, Senile,Choreas, Sydenham,Choreatic Disorder,Choreatic Syndrome,Choreic Movements,Choreiform Movements,Chronic Progressive Chorea,Chronic Progressive Choreas,Disorder, Chorea,Disorder, Choreatic,Disorders, Chorea,Disorders, Choreatic,Dyskinesia, Paroxysmal,Hereditary Chorea, Benign,Hereditary Choreas,Hereditary Choreas, Benign,Movement, Choreic,Movement, Choreiform,Movements, Choreic,Movements, Choreiform,Paroxysmal Dyskinesia,Progressive Chorea, Chronic,Progressive Choreas, Chronic,Rheumatic Choreas,Senile Choreas,St. Vitus Dance,St. Vitus's Dances,St. Vituss Dance,Sydenham Choreas,Sydenhams Chorea,Syndrome, Chorea,Syndrome, Choreatic,Syndromes, Chorea,Syndromes, Choreatic
D004576 Electromyography Recording of the changes in electric potential of muscle by means of surface or needle electrodes. Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

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