Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease

D. Testa, V. Tiranti, F. Girotti

Research output: Contribution to journalArticlepeer-review


Sporadic olivopontocerebellar atrophy (OPCA) is a neurodeggenerative 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.

Original languageEnglish
Pages (from-to)243-245
Number of pages3
JournalNeurological Sciences
Issue number5
Publication statusPublished - Dec 2002


  • Amyotrophic lateral sclerosis
  • Mitochondria disorders
  • Motor neuron disease
  • Olivopontocerebellar atrophy

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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