title: Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease.
abstract: 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.
authors.affiliation: Department of Neurology, Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, I-20133 Milan, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Testa
authors.forename: V
authors.initials: V
authors.lastname: Tiranti
authors.forename: F
authors.initials: F
authors.lastname: Girotti
mesh_terms: 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
chemical_list: C070354:Cox protein, Enterobacteria phage P2; D004268:DNA-Binding Proteins; D014764:Viral Proteins