Neurological Sciences

, Volume 23, Issue 5, pp 243–245

Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease

  • D. Testa
  • V. Tiranti
  • F. Girotti
CASE REPORT

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.

Key words Amyotrophic lateral sclerosis Mitochondria disorders Motor neuron disease Olivopontocerebellar atrophy 

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Copyright information

© Springer-Verlag Italia 2002

Authors and Affiliations

  • D. Testa
    • 1
  • V. Tiranti
    • 2
  • F. Girotti
    • 1
  1. 1.Department of Neurology, Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, I-20133 Milan, ItalyIT
  2. 2.Department of Biochemistry and Genetics, Istituto Nazionale, Neurologico Carlo Besta, Milan, ItalyIT

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