Abstract

background activity but no interictal discharges suggestive of epilepsy. Neuropsychological evaluations with 2-year intervals showed progressivewordfinding difficulties, attentionand memory-deficits. He became nearly mute and apathetic and was unable to continue his work as a process-controller. The MRI was repeated 8-year after symptom-onset showing prominent atrophy of the left hemisphere (figure), consistent with the clinical diagnosis progressive non-fluent aphasia. Case-management was still lacking at referral to our memory clinic. The presence of epilepsy in the presented patient with PPA has led to a long-lasting delay in clinical diagnosis and adequate care. Patients with subtle speech difficulties usually seek medical attention on average 2-3 years after symptom-onset. Primary and secondary care physicians often do not recognise PPA-symptoms adding a further diagnostic delay. Complaints are often attributed to psychogenic factors (burnout, mood or anxiety) or to other comorbid conditions, which even led to a diagnostic delay of 8 years in our case. Conclusions: An atypical age-at-onset, slow disease progression, absence of cognitive or behavioural problems are all factors delaying referral to a memory clinic in PPA. Diagnostic delay is even more pronounced in the presence of comorbid conditions which may affect speech.

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