Anikó Papp1,2, Anna Szűcs3
1 Neurologist, Department of Neurology/National Institute of Clinical Neuroscience, Budapest
2 Ph.D. Student, School of Ph.D. studies, Semmelweis University, Budapest
3 Neurologist, Department of Neurology/National Institute of Clinical Neuroscience, Budapest
Introduction: Posterior cortical atrophy (PCA) is a rare, under-diagnosed neurodegenerative condition characterized by early visouspatial/visuoperceptual decline, affecting additional skills, parietal or occipito-temporal regions. It has been underlain by various types of pathologies. The age of onset is typically between 50-65 years. Sleep disturbances or epileptic seizures have rarely been described with this syndrome.
Aim: We present our case for demonstrating clinical, neurophysiology and radiology features of PCA; characterised by insomnia and possible epilepsy.
Method: We diagnosed a 59-year-old female with possible PCA based on clinical and radiological signs. Her presentation supporting PCA included severe visuospatial impairment and parietal lobe dysfunction with cognitive decline about 1.5 years after disease-onset. Historically, the first signs were insomnia with inappropriate night activities and visual hallucinations. The first brain MRI scans showed centro-parietal cortical atrophy with the predominance of the left hemisphere. We performed 24-hour-long EEG-polysomnography.
Results: the long-term EEG showed decreased total sleep time, severe fragmented sleep without identifiable sleep stages, moreover we saw left temporal spikes suggesting interictal epileptic activity. The presence of epilepsy was supported by her confused sleep-related activities (possible complex partial seizures). Besides memantine, we started levetiracetam and zolpidem. 9 months after the first visit her sleep time increased and her night activities or hallucinations disappeared; we experienced a moderate global clinical improvement.
Conclusion: The clinical improvement is unusual in posterior cortical atrophy, even though there may occur better and worse periods. In our case the supposed epilepsy and sleep disturbance along with the underlying presumed PCA might have contributed to the cognitive decline slightly improving on medication. Long term follow-up is needed. Learning from this case it might be necessary collecting detailed information about sleep hygiene and possible epilepsy-like episodes; long-term EEG-polysomnography can provide new information about the attributes (complications) of the condition, e.g. epilepsy.
Doctoral School: Mental Health Sciences
Program: Mental Health Sciences
Supervisor: Anna Szűcs
E-mail address: firstname.lastname@example.org