2017 Resarch Forum

PSY: C-1

Applicant & Principal Investigator: Sabina Bera MD MS

Losing control: A rare presentation of behavioral variant of frontotemporal dementia

Sabina Bera MD MS, Naila Maniar MD, Mohammed Molla MD

INTRODUCTION: Frontotemporal dementia (FTD) is a collection of neurodegenerative diseases involving frontotemporal lobar degeneration. Diagnosing FTD can be challenging with a wide variety of presenting symptoms. A behavioral variant frontotemporal dementia (bvFTD) commonly presents with the onset of psychiatric symptoms and diagnosis as a primary psychiatric disorder. The patient presented here was first diagnosed with bipolar disorder with catatonia and was later found to have probable bvFTD. PURPOSE: This patient is a 33 year old Caucasian male with no past psychiatric history and a medical history of migraines with two years of gradual behavioral and cognitive changes. His family first noticed ritualistic behaviors then insomnia and social withdrawal. He also experienced difficulties performing tasks at work, and left employment. These symptoms were initially attributed to several close deaths in the family. After a neurologist relayed that the problem was likely not neurological in nature, the patient’s primary care physician started the patient on antidepressants. A psychiatrist later diagnosed him with bipolar disorder with catatonic features (irritability, sleep difficulty, perseveration, some obsessive preoccupation with compulsive behavior interpreted as increase goal directed activity and selective mutism). Antipsychotics, antidepressants, and benzodiazepines did not improve symptoms. The patient later developed severe difficulty with speech, complex tasks and problem solving. A second psychiatrist requested a repeat neurological evaluation. The patient’s neurologist ordered a comprehensive workup (imaging, a lumbar puncture, and laboratory tests), results of which were unremarkable. The patient was admitted post lumbar puncture (headache) and was seen by the psychiatry consult service: a likely an underlying neurocognitive disorder was identified. The patient was referred to a memory and aging specialty clinic, and had an extensive neuropsychological evaluation. The patient was found to meet criteria for probable bvFTD. DISCUSSION: A review of the literature reveals a significant overlap between the symptoms of behavioral variant frontotemporal dementia and psychiatric disorders. These patients are often incorrectly diagnosed and treated. Patients with symptoms resembling bvFTD require a collaborative approach between neurology and psychiatry. Treatment is generally aimed at targeting the patient’s symptoms: selective serotonin reuptake inhibitors can be used to target compulsive and ritualistic behaviors, medications such as trazodone can be used to decrease irritability, and antipsychotics and mood stabilizers are reserved for education and aggression. CONCLUSION: This is a rare and atypical presentation of probable bvFTD. The patient was outside the normal age range for bvFTD (45-64 years; the picture was further complicated by the psychiatric symptoms alongside a coincidental psychosocial stressor. This case report may encourage the psychiatric community to include bvFTD in their differential when evaluating behavioral changes in a young adult patient with no past psychiatric history. The atypical presentation of this patient may also stimulate a discussion surrounding the current DSM-V diagnostic criteria for frontotemporal neurocognitive disorder.