Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease

被引:5
作者
Testa, D
Tiranti, V
Girotti, F
机构
[1] Ist Nazl Neurol Carlo Besta, Dept Neurol, I-20133 Milan, Italy
[2] Ist Nazl Neurol Carlo Besta, Dept Biochem & Genet, I-20133 Milan, Italy
关键词
amyotrophic lateral sclerosis; mitochondria disorders; motor neuron disease; olivopontocerebellar atrophy;
D O I
10.1007/s100720200049
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Sporadic olivopontocerebellar atrophy (OPCA) is a neurodegenerative disorder that presents a wide clinical spectrum. Motor neuron disease (MND) is characterized by a selective degeneration of motor neurons. A 60-year-old man developed slurred speech and unsteadiness of gait. He had also noticed difficudlty in holding his head upright and shoulder weakness. The disease had a rapid progression. At the age of 63 years, magnetic resonance imaging supported a diagnosis of OPCA, and a diagnosis of MND was suggested by clinical and electrophysiological findings. He also had upward gaze palsy. A muscular biopsy showed sporadic ragged red and Cox deficient fibers. The present case could define a unique disorder, as the occasional occurrence of two degenerative disorders appears unlikely.
引用
收藏
页码:243 / 245
页数:3
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