[P‐043]: FTD/ALS ‐ A rare case with cognitive impairment and variety of speech disorders

Background: Dementia rarely occurs in patients with ALS, although known associations include the Parkinson-ALS-dementia complex of Guam. In cases of sporadic forms of ALS-dementia complex, front temporal dementia is the most common clinical feature. We report a case of definite ALS with domination of progressive bulbar palsy and progressive cognitive impairment with variety of speech disorders. Methods: We studied 61 years old man with rapid progressive history of changes in mood and personality, and anxiety in the evening. One year later he developed attention loss, loss of verbal fluency leading to complete mutism, impairment of perceptual skills. Two years later he developed a full range of Motor Neuron Disease at the level of spinal cord and bulb as well as flaccid dysarthria manifested by breath, articulation, prosody and resonance disorders. We observed very rapid progression leading ultimately breath difficulties and respiratory failure. Patient underwent full general and neurological examination and neuropsychological assessment was carried out. Brain MRI and CT scans revealed cortical atrophy of frontal lobe. EEG was normal. Pattern in EMG was not different from that seen in ALS. El Escorial criteria were used to diagnose definite ALS. Patient was treated farmacologically as well as a bunch of rehabilitation methods were used. reported the sporadic case of MND and dementia with familial history of ALS and FTD.