Severe Anxiety and Frontal Cognitive Dysfunction Heralding a Progressive Supranuclear Palsy-Corticobasal Overlap Syndrome (P07.190)

Abstract

Objective: To describe the evolution of behavioral, cognitive, and motor symptoms and ancillary testing in a case of progressive supranuclear palsy-corticobasal overlap syndrome. Background Progressive supranuclear palsy (PSP) and corticobasal degeneration have genetic and pathologic similarities, but different phenotypes. Occasionally, the clinical diagnosis between PSP and corticobasal syndrome (CBS) is blurred, and patients are felt to have an overlap syndrome. In such cases, less is known about the spectrum of behavioral and cognitive features that can be seen, especially those preceeding the movement symptoms. Design/Methods: We review the clinical evolution, brain MRI and brain PET scans and videotaped examination findings in this case. Results: This patient is a 76-year-old woman who developed severe and constant anxiety and obsessive-compulsive features including strict rule-bound activities for herself and family, self-harm threats if separated from her spouse, mental rigidity, and bizarre and time-consuming rituals. This was followed by impaired problem solving and perspective-taking, childlike behavior, and pathological laughing and crying. One and a half years later, she developed repeated falls, slow and soft speech, largely symmetric bradykinesia, and axial symptoms of neck rigidity and worsened postural instability, but only slightly slowed downward saccades. Subsequently, right-sided predominant rigidity, myoclonus, and mild apraxia developed. Brain MRI at 3 months and brain PET at 1 year after motor onset, revealed left-sided predominant parietal, medial prefrontal, and anterior temporal mild atrophy and hypometabolism, respectively. Anxiety and obsessive-compulsive features were somewhat responsive to sertraline and behavioral strategies were also employed. Conclusions: Severe anxiety with frontal cognitive symptoms may preceed a PSP-CBS overlap disorder but often go unrecognized, yet may respond to pharmacotherapy and family education. This symptom cluster requires additional study to determine its abillty early on to predict PSP-CBS syndromes. Disclosure: Dr. Smyth has nothing to disclose. Dr. Riley has received personal compensation for activities with Allergan, Ipsen, Lundbeck, Merz and Teva as a consultant.Dr. Riley has received research support from Cleveland Medical Devices. Dr. Lerner has nothing to disclose.