Citations
Citations
To investigate the intra-rater and inter-rater reliability and usefulness of the Korean version of the Scale for the Assessment and Rating of Ataxia (K-SARA) in ataxic stroke patients.
The original SARA was translated into Korean, back translated to English, and compared to the original version. Stroke patients (n=60) with ataxia were evaluated using the K-SARA by one physiatrist and one occupational therapist. All subjects were rated twice. We divided the subjects into 5 groups by Functional Ambulation Category (FAC) and 3 groups based on the ataxia subscale of the National Institutes of Health Stroke Scale (NIHSS). The mean K-SARA scores representing each group of FAC and the ataxia subscale of NIHSS were compared.
The test-retest correlation coefficient of the K-SARA was 0.997 by the therapist and 1.00 by the physiatrist (p<0.001). The inter-rater correlation coefficient of the K-SARA was 0.985 (p<0.001). The ataxia subscale of NIHSS did not correlate with K-SARA. There was a significant difference in the mean K-SARA score by FAC (p<0.001).
K-SARA is a reliable and valid measure of ataxia in stroke patients in Korea.
Citations
Burkitt lymphoma is a type of B-cell lymphoma that occurs mostly in children, and rarely in adults. The sporadic type is known to occur mostly at the ileum and cecum. Cytarabine, which is used for central nervous system prophylaxis during chemotherapy for Burkitt lymphoma, has known neurotoxicity, and its side effects include motor ataxia due to cerebellar injury, ataxic dysarthria, dysfunction of ocular movement, confusion, somnolence and lethargy. This case report is about a patient diagnosed with Burkitt lymphoma who manifested motor ataxia after chemotherapy that included cytarabine.
To examine the usefulness of the Scale for the Assessment and Rating of Ataxia (SARA) in ataxic stroke patients.
This was a retrospective study of 54 patients following their first ataxic stroke. The data used in the analysis comprised ambulation status on admission and scores on the SARA, the Korean version of the Modified Barthel Index (K-MBI) and the Berg Balance Scale (BBS). The subjects were divided into four groups by gait status and into five groups by level of dependency in activities of daily living (ADLs) based on their K-MBI scores. Data were subjected to a ROC curve analysis to obtain cutoff values on the SARA for individual gait status and levels of activity dependency. The correlations between the SARA, K-MBI and BBS scores were also computed.
There was significant correlation between the SARA and the K-MBI scores (p<0.001), and this correlation (r=-0.792) was higher than that found between the BBS and the K-MBI scores (r=0.710). The SARA scores of upper extremity ataxia categories were significantly related to the K-MBI scores of upper extremity related function (p<0.001). The SARA scores were also significantly correlated negatively with ambulation status (p<0.001) and positively with ADL dependency (p<0.001). In the ROC analysis, patients with less than 5.5 points on the SARA had minimal dependency in ADL, while those with more than 23 points showed total dependency.
SARA corresponds well with gait status and ADL dependency in ataxic stroke patients and is considered to be a useful functional measure in that patient group.
Citations
Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive disorder characterized by dysmyelination of the central nervous system (CNS) caused by mutations in the proteolipid protein (PLP) gene. PLP is located at Xq22 and its mutation result in abnormal expression or production of PLP, the most abundant protein in CNS myelin. We present a case of PMD in the 7-year-old boy with nystagmus, ataxia, spastic quadriplegia and severe psychomotor delay. His brain MRI revealed totally dysmyelinated white matter
involving entire supratentorial region, atrophic change, and overaccumulation of the iron in both basal ganglia. He also showed soft-tissue contractures of the hip adductors, associated hip dislocations and equinovarus foot deformities due to severe spasticity of lower extremities. Orthopaedic surgery was performed on both hips. Antispastic medication and physical therapy were maintained for reduction of spasticity. We report this case with the review of literatures. (J Korean Acad Rehab Med 2002; 26: 108-112)
Dominantly inherited spinocerebellar ataxias (SCAs) are a group of the heterogenous neurodegenerative diseases that are characterized by chronic progressive cerebellar ataxia associated with various combinations of other neurological signs. Clinical classification is difficult because of the phenotypic overlap. With the evolution of molecular genetics, the loci and mutations for many of the ataxias have been identified, allowing more definitive molecular classification.
We experienced 42 years-old man who presented with progressive both lower leg weakness, dysarthria, ataxia, ophthalmoplegia, and nystagmus. The family history was remarkably suspicious. We could not observe the upper extremity weakness, definite evidences of peripheral neuropathy and myopathy in electrodiagnosis. No abnormal findings in blood chemistry and brain MRI. We performed polymerase chain reaction (PCR) and polyacrylamide gel electrophoresis (PAGE) analysis, found that his gene contained expanded CAG repeats (CAG repeat number was 72). Although no effective treatment exists for most the ataxic syndromes, the accurate diagnosis and the genetic counseling are often important to the patient's family for prognostication.
In 1965 Gillespie reported a new syndrome of bilateral aniridia, cerebellar ataxia, and oligophrenia (mental retardation). This new syndrome was named Gillespie syndrome. Since then only 17 cases of Gillespie syndrome have been reported in UK, Brazil, Ireland, Belgium, Australia, and US. A case of Gillespie syndrome was not reported in Korea.
A 4 year-old girl has triad of Gillespie syndrome, which are partial aniridia, cerebellar ataxia and mental retardation. We confirmed this with ophthalmologic examination, brain MRI, and developmental delay. We report the typical manifestation of Gillespie syndrome in a 4 year-old girl with the brief review of literature.
Early onset cerebellar ataxia with retained tendon reflexes is distinctive clinical syndrome characterized by progressive cerebellar ataxia of unknown etiology with an onset within the first two decades. This disorder was distinguished from Friedreich's ataxia by the preservation of the deep tendon reflexes. There is 22-year-old male with 13 year history of slowly progressive cerebellar ataxia and dysarthria. His elder brother, also, has milder clinical manifestations, electrophysiological and radiological abnormalities. We experienced two cases of early onset cerebellar ataxia with retained tendon reflexes developed in brothers which was diagnosed by clinical manifestations, electrophysiologic, radiologic studies and report with brief review of related literatures.
First
Prev
