8.1 Clinical History – Main Complaint

ALLG is a 57-year-old married woman, with 15 years of schooling (graduated in pedagogy).

At the age of 50 her husband noted that ALLG became more participative in church. Once restrained and timid, ALLG began to use the microphone to preach during church services, manifesting an unprecedented religious fanaticism. She suddenly quit eating meat and only accepted what she considered “healthy food.” She started to collect stuff without obvious actual utility, including plastic bags and empty butter pots and soda cans. She also engaged in repetitively tapping on things (tables, chairs) without any reasonable purpose. Her abilities to deal with administrative and financial tasks, housekeeping, and cooking became impaired. Before neurological evaluation at our unit, the patient had consulted a psychiatrist, who established the diagnosis of depression.

ALLG became progressively more inappropriate, especially in social events, when she started embarrassing her family relatives. ALLG would easily call someone she barely knew as a sinner and order the person to seek the Lord. However, she kept working until the age 53, when she got her first medical disability due to behavioral and personality changes, and difficulty in planning her daily activities.

Despite her husband’s compelling complaints, ALLG made no remarks regarding her condition during first evaluation, except for insomnia. She denied all her husband’s descriptions.

8.2 General History

ALLG was born in the countryside of Minas Gerais, Brazil. She graduated in pedagogy from University of São Paulo, where she lived until she turned 30 years old. Then she went back to her hometown and worked as a pedagogue until her retirement. ALLG is married and has one son.

The patient was previously healthy, with no comorbidities. She had no history of developmental learning or language problems, previous psychiatric or neurological problems.

8.3 Family History

There were six cases of early-onset dementia in her family, including her brother, father, grandfather, and three uncles. Her husband could not provide clinical details on their presentation but stressed they presented remarkable behavioral changes.

8.4 Examination

8.4.3 Neuroimaging Findings

Brain MRI (Figure 8.1) shows severe anterior right temporal atrophy, including frontoinsular region. There was mild atrophy in left temporal lobe and in frontal lobes, especially medial frontal (red circle). Severe anterior right temporal atrophy, including frontoinsular region. There was mild atrophy in left temporal lobe and in frontal lobes, especially medial frontal (red circle). Posterior regions were preserved. There were no focal or vascular lesions.

Figure 8.1 Brain MRI.

8.5 Diagnosis

Based on the clinical, neuropsychological, and neuroimaging data a diagnosis of probable behavioral variant frontotemporal dementia (bvFTD) was established. The definitive diagnosis was confirmed by genetic analysis.

8.6 Follow-Up

The patient was submitted to symptomatic pharmacological treatment. Trazodone was chosen, due to the evidence of benefits.⁷ After 2 weeks the patient was evaluated with improvement in repetitive movements and hoarding. Disinhibition was still an issue and doses of trazodone were gradually increased until 300 mg/day.

Two months after use of trazodone on maximum dosage, the patient was putting herself in risk, walking from the country house to the city by herself, being lost from the family, talking loudly during church, and refusing treatment. After a failed trial with quetiapine, risperidone (1 mg/day) was initiated.⁸

The patient kept herself stable for a year. When she tried to misrepresent a document in doctor’s name in order to stop treatment (Figure 8.2), risperidone was increased to 2 mg/day and then to 3 mg/day. ALLG became severely apathetic, with overt extrapyramidal symptoms. However, drug regimen was maintained due to severity of her behavioral symptoms, and 1 year later it was reduced.

Figure 8.2 Patient’s misrepresented document.

Figure 8.2 translation: “Dear Seventh-day Adventist Church Sir we declare that Patient A… is great and does not need to take any more medicines, if she needs to take sleeping pills she could seek another Physician. You can let her participate in church services, Singing, praying and preaching the word of God. She is free to drive a car. You must consider her with great affection. We declare she is cured.” This is an ipsis litteris translation of her letter including several grammar mistakes.

Almost 7 years after symptoms onset and 3 years after our first evaluation, the patient remains on trazodone (300 mg/day) and risperidone (1 mg/day), and also duloxetine (30 mg/day). She presents severe apathy, puerility, and mild disinhibition, with no hoarding or dietary changes. Regardless of her clinical presentation, she is still partially independent for basic activities of daily living.