Abstract
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 language | English |
|---|---|
| Pages (from-to) | 243-245 |
| Number of pages | 3 |
| Journal | Neurological Sciences |
| Volume | 23 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Dec 2002 |
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Keywords
- Amyotrophic lateral sclerosis
- Mitochondria disorders
- Motor neuron disease
- Olivopontocerebellar atrophy
ASJC Scopus subject areas
- Neuroscience(all)
- Clinical Neurology
Cite this
Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease. / Testa, D.; Tiranti, V.; Girotti, F.
In: Neurological Sciences, Vol. 23, No. 5, 12.2002, p. 243-245.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease
AU - Testa, D.
AU - Tiranti, V.
AU - Girotti, F.
PY - 2002/12
Y1 - 2002/12
N2 - 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.
AB - 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.
KW - Amyotrophic lateral sclerosis
KW - Mitochondria disorders
KW - Motor neuron disease
KW - Olivopontocerebellar atrophy
UR - http://www.scopus.com/inward/record.url?scp=0036919466&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0036919466&partnerID=8YFLogxK
U2 - 10.1007/s100720200049
DO - 10.1007/s100720200049
M3 - Article
C2 - 12522682
AN - SCOPUS:0036919466
VL - 23
SP - 243
EP - 245
JO - Neurological Sciences
JF - Neurological Sciences
SN - 1590-1874
IS - 5
ER -