Moria and Witzelsücht from Frontotemporal Dementia

A 57-year-old right-handed female had a 2-year personality change described as increased gregariousness, excitement, and a tendency to indiscriminately approach strangers without apprehension. She had become the life of the party and would laugh, joke, and sing all the time. The patient had decreased self-care and hygiene and wore the same clothes every day. In addition, she had developed a compulsive tendency, particularly with hoarding of money, and an addiction to ice cream with marked weight gain.

Her past medical history was negative, but her family history was positive for dementia and amyotrophic lateral sclerosis (ALS). Her paternal grandmother expressed dementia in her 50’s, and her paternal uncle died at 58 with ALS.

On examination, her general demeanor was slightly euphoric and unconcerned. She was very talkative, animated, and disinhibited. Her most salient behaviors were almost continuous silly laughter and excitement (moria of Jastrowitz) and frequent childish jokes and puns (Witzelsücht). The patient would also make frequent personal comments about the examiner or touch the examiner.

The rest of her examination revealed cognitive changes primarily in language. She was fluent except for word-finding pauses, and she had good auditory comprehension and repetition but had difficulty with naming and made literal paraphasic errors. The patient’s performance on a 10-item verbal memory test was zero spontaneously but 8 on recognition. The patient was able to do praxis tasks and simple drawings, and the rest of her neurological examination was entirely normal.

Most of her laboratory results, including syphilis serology, were unremarkable. Magnetic resonance imaging showed prominent atrophy in the anterior temporal lobes. Single-photon emission tomography scan showed circumscribed areas of hypoperfusion in both temporal lobes, much larger on the right. The patient was treated with citalopram with some behavioral improvement, and the family was informed of specialized genetic testing and genetic counseling.

Frontotemporal dementia (FTD) is a neuropsychiatric syndrome with progressive degeneration of the frontal lobes, anterior temporal lobes, or both.^3,4 The core clinical criteria for diagnosing FTD include progressive declines in social conduct, personal regulation, insight, and emotional reactivity.³ This patient met criteria for FTD, and her family history suggested a familial form of the FTD-ALS spectrum.

Frontal temporal dementia is a variable and often asymmetric disorder with a range of neuropsychiatric symptoms.⁴ Previously described from vascular lesions and tumors involving the orbitofrontal region and from neurosyphilis, moria and Witzelsücht can also be the most prominent symptoms of FTD. Moria includes childish euphoria and cheerful excitement,¹ and Witzelsücht includes excessive and inappropriate facetiousness, jokes, and pranks.² These behaviors may be specifically related to disturbances in the right anterior temporal orbitofrontal region,⁵ as evidenced in this patient. Furthermore, moria and Witzelsücht may respond to serotonin selective reuptake inhibitor and other psychoactive medications.