Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease.
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 difficulty 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.
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Department of Neurology, Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, I-20133 Milan, Italy.
Tiranti
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Girotti
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D004268:DNA-Binding Proteins / Q000172:deficiency; D003680:Deglutition Disorders / Q000209:etiology; D004576:Electromyography / Q000379:methods; D020234:Gait Ataxia / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000473:pathology; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D009849:Olivopontocerebellar Atrophies / Q000150:complications* / Q000473:pathology; D014764:Viral Proteins
C070354:Cox protein, Enterobacteria phage P2; D004268:DNA-Binding Proteins; D014764:Viral Proteins