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Kon Chu

Publications and source records attributed to Kon Chu.

38 records · Page 3Linked to original sources

Phonological agraphia after superior temporal gyrus infarction.

BACKGROUND: Phonological agraphia refers to a condition in which the ability to write nonwords to dictation is impaired, while writing words to dictation is preserved, as is oral repetition of the words and nonwords. This condition has been regarded as reflecting a disconnection within the phonological writing system, and previous neurolinguistic correlations suggested that the anterior-inferior supramarginal gyrus was a crucial link within the system. SETTING: A neurology department of a university hospital. PATIENT: A 51-year-old right-handed man presented with speech disturbances. On initial evaluation of his language, his deficit was consistent with that of conduction aphasia, which improved rapidly to an apparently normal level. A subsequent detailed examination of oral and written repetition of words and nonwords revealed a rather selective form of phonological agraphia. A magnetic resonance imaging scan of his brain showed a focal ischemic lesion at the left posterior superior temporal gyrus and at the underlying white matter. CONCLUSIONS: In contrast to most previously described patients, this patient showed a selective impairment of phonological agraphia in association with a focal infarction restricted to the left posterior superior temporal gyrus, suggesting that this region of the brain is an important node within a wider network of areas that subserve the phonological route for writing.

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A patient with proximal myotonic myopathy and parkinsonism.

INTRODUCTION: There are two case reports of patients who had proximal myotonic myopathy (PROMM)/myotonic dystrophy (DM) Type 1 and parkinsonism. The combination of myotonic myopathy and parkinsonism is so rare that it may appear to be just a coincidence. However, previous neuropathological examinations of patients who had myotonic dystrophy showed that there were intracytoplasmic inclusion bodies in the nigra and striatum, which raises the possibility that myotonic myopathy may be associated with parkinsonism. In this report we describe a patient with PROMM and a clinically definite parkinsonism to highlight this possibility. CASE REPORT: A 65-year-old man developed proximal muscle weakness, myotonia and atrophy around the age of 55 and was diagnosed as having PROMM at the age of 62. Needle electromyography and muscle biopsy supported the diagnosis. A gene study of the DM Type 1 showed a normal CTG repeat length. At age 63, he developed rest tremor, bradykinesia, hypomimia, stooped posture, and gait disturbance. The postural instability worsened rapidly. The tremor and rigidity were much worse in his right side, where myotonia was more severe. Levodopa therapy was only partially effective. CONCLUSION: This is a case report of a patient with PROMM that shows an association with a rapidly progressive form of parkinsonism. We suggest that this may be a novel form of a neurodegenerative disorder, which we name 'Parkinsonism-Myotonic Myopathy-Complex'.

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