Cognitive disorders as the inaugural sign of amyotrophic lateral sclerosis: contribution of brain SPECT in intensive care

BACKGROUND: Cognitive disorders have been described in amyotrophic lateral sclerosis, but usually after the diagnosis has ben established. CASE REPORT: A 57-year-old man was intubated for acute respiratory distress subsequent to pneumonia and diaphragm palsy He had a 2-year history of dru g-resistant depression and deterioration of cognitive functions. A pyramida l syndrome associated with biopsy-proven chronic neurogenic atropy led to t he diagnosis of amyotrophic lateral sclerosis. The electromyogram did not c ontribute to diagnosis. Brain MRI only evidenced moderate bilateral frontal -temporal atrophy. Brain SPECT demonstrated major perfusion defects in the frontal lobes. DISCUSSION: This patient had amyotrophic lateral sclerosis and frontal-temp oral dementia with an unusually late onset clinical presentation: cognitive disorder was the inaugural sign. Brain SPECT and muscle biopsy enabled us to identify the cortical and peripheral motor neurone involvement in this u ncooperative intensive care patient totally dependent on mechanical ventila tion.