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Primary Progressive Aphasia Presenting With Neuropsychiatric Symptoms

We describe a case of primary progressive aphasia (PPA) with an underlying neurodegenerative motor disorder (possible ALS or PSP), presenting with symptoms of irritability and frustration, that were misdiagnosed and treated as a primary psychiatric disorder, i.e. depression. PPA is a rare neurodegenerative disorder characterized by insidious onset and gradual progression of speech and language impairment. We emphasize that PPA can initially masquerade as or be accompanied by neuropsychiatric symptoms potentially leading to misdiagnosis. Most prevalent neuropsychiatric symptoms reported in the PPA literature are agitation, depression, anxiety, apathy, irritability, abnormal appetite and disinhibition. To ensure early diagnosis of PPA, if a patient presents with new psychiatric symptoms accompanied by new onset speech and/or language impairment, referral to a specialist (i.e., neurologist and/or speech-language pathologist) is recommended.