Proband 007
This 77-year-old right-handed patient was evaluated in our Department after two years of language difficulty. He had endoprothetic treatment of the left hip at 67 years old and a right femoral fracture at the age of 75, without any obvious radiological or biological signs of PDB. Examination showed apraxia of speech characterized by an articulation planning deficit, dysarthria with hypokinetic features and buccofacial apraxia, associated with semantic and phonetic paraphasia. Phonemic and semantic fluency sores were low (respectively 12 and 2 words in two minutes). Syntactic and single-word comprehensions were spared. Mild grammatical deficit appeared in his spoken picture description and in writing. Neuropsychological testing showed executive dysfunction associated with severe visuo-constructional impairment when copying the Rey figure but preservation of memory (Fig. 2, Table 1). Careful interview of the patient’s caregiver revealed the absence of behavioral disorders. Deep tendon reflexes were increase in all limbs and plantar reflex was flexor bilaterally. He had no motor symptoms, no gaze limitation, and no parkinsonian signs. Brain magnetic resonance imaging (MRI) showed major diffuse cortical atrophy more marked on the frontal and temporal lobes. ECD single photon emission computed tomography (SPECT) of the brain revealed severe hypoperfusion of frontal and temporal lobes, predominantly on the right side, and hypoperfusion of basal ganglia (Fig. 3). Five years later, the patient displayed severe phonetic disintegration with worsening of agrammatism but comprehension was spared. There were no clinical or electromyographic signs of ALS.