Abstract
Mitsuyama variant in the association ALS-dementia. We report a case characterized by the association of an early-onset, rapidly progressive and severe dementia with the subsequent appearance of a motoneuron disease (bulbar ALS). The neuropsychological evaluation showed a global cognitive impairment, with a more profound impairment of executive, linguistic and mnestic functions, as in fronto-temporal dementia. SPECT and PET investigations demonstrated a bilateral reduction of blood perfusion and glucose metabolism in the frontal and temporal lobes. Brain MRI showed a diffuse cortical atrophy, more pronounced at the level of the frontal lobes. The characteristics of the present case are in agreement with the clinical variant first described by Mytsuyama in eastern patients. However, our case differs for the extremely rapid onset and progression of cognitive decline and this could suggest that the complexity of the association between fronto-temporal dementia and ALS is even more heterogeneous than reported up-to-date.
| Original language | English |
|---|---|
| Pages (from-to) | 62-78 |
| Number of pages | 17 |
| Journal | Nuova Rivista di Neurologia |
| Volume | 10 |
| Issue number | 2 |
| Publication status | Published - Mar 2000 |
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Keywords
- Bulbar ALS
- Fronto-temporal dementia
ASJC Scopus subject areas
- Clinical Neurology
Cite this
Mitsuyama variant in the association ALS-dementia. / Appollonio, Ildebrando; Cavarretta, Rossella; Melzi, Paola; Apale, Paolo; Curtò, Natale; Frattola, Lodovico.
In: Nuova Rivista di Neurologia, Vol. 10, No. 2, 03.2000, p. 62-78.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Mitsuyama variant in the association ALS-dementia
AU - Appollonio, Ildebrando
AU - Cavarretta, Rossella
AU - Melzi, Paola
AU - Apale, Paolo
AU - Curtò, Natale
AU - Frattola, Lodovico
PY - 2000/3
Y1 - 2000/3
N2 - Mitsuyama variant in the association ALS-dementia. We report a case characterized by the association of an early-onset, rapidly progressive and severe dementia with the subsequent appearance of a motoneuron disease (bulbar ALS). The neuropsychological evaluation showed a global cognitive impairment, with a more profound impairment of executive, linguistic and mnestic functions, as in fronto-temporal dementia. SPECT and PET investigations demonstrated a bilateral reduction of blood perfusion and glucose metabolism in the frontal and temporal lobes. Brain MRI showed a diffuse cortical atrophy, more pronounced at the level of the frontal lobes. The characteristics of the present case are in agreement with the clinical variant first described by Mytsuyama in eastern patients. However, our case differs for the extremely rapid onset and progression of cognitive decline and this could suggest that the complexity of the association between fronto-temporal dementia and ALS is even more heterogeneous than reported up-to-date.
AB - Mitsuyama variant in the association ALS-dementia. We report a case characterized by the association of an early-onset, rapidly progressive and severe dementia with the subsequent appearance of a motoneuron disease (bulbar ALS). The neuropsychological evaluation showed a global cognitive impairment, with a more profound impairment of executive, linguistic and mnestic functions, as in fronto-temporal dementia. SPECT and PET investigations demonstrated a bilateral reduction of blood perfusion and glucose metabolism in the frontal and temporal lobes. Brain MRI showed a diffuse cortical atrophy, more pronounced at the level of the frontal lobes. The characteristics of the present case are in agreement with the clinical variant first described by Mytsuyama in eastern patients. However, our case differs for the extremely rapid onset and progression of cognitive decline and this could suggest that the complexity of the association between fronto-temporal dementia and ALS is even more heterogeneous than reported up-to-date.
KW - Bulbar ALS
KW - Fronto-temporal dementia
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M3 - Article
AN - SCOPUS:3242820011
VL - 10
SP - 62
EP - 78
JO - Rivista di Neurologia
JF - Rivista di Neurologia
SN - 0035-6344
IS - 2
ER -