|LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 3 | Page : 239-240
The woman who lost her words
Thomas Gregor Issac1, Sadanandavalli Retnaswami Chandra2
1 Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
|Date of Web Publication||11-Aug-2016|
Thomas Gregor Issac
No. 13, New Kabini Hostel Complex, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
|How to cite this article:|
Issac TG, Chandra SR. The woman who lost her words. Saudi J Med Med Sci 2016;4:239-40
Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous syndrome with a prevalence of 15.1/100,000 population. FTD is more commonly diagnosed in patients <65 years of age. Original cases were described by Alzheimer more than a century ago and later found to have tau-positive inclusions. FTD can be classified into three clinical syndromes depending on the early and predominant symptoms. The more common syndrome is a behavioral variant (bvFTD) with two language variants namely semantic dementia and progressive nonfluent aphasia (PNFA), which is very rare compared to bvFTD. Behavioral disturbances in the language variants of FTD, especially PNFA, are not frequently described in literature., The majority of patients who present with PNFA are females and display decreased verbal output, slow effortful speech, impaired production and comprehension of grammar (agrammatism), and motor speech deficits with retained comprehension for single words. These patients often have normal reading skills, unlike other variants of FTD.
A 60-year-old homemaker presented with features of cognitive decline and behavioral problems for the past 1 year duration. When her illness started, she became aloof and disinterested in household activities with a decreased interest in cooking and interacting with her family members, especially her grandchildren. Later, her relatives noticed that she would get irritated easily with occasional angry outbursts, even for trivial issues which she used to manage quite efficiently in her premorbid self. Gradually, she started repeating questions and could not recognize close relatives and neighbors. Three months after the onset of her illness, she started showing disinhibition, which manifested itself in terms of improper dressing, using vulgar terms in public, showing stimulus-bound behavior, tendency to wander, emotional incontinence, and becoming doubly incontinent without displaying any embarrassment. Her appetite also increased with a preference for carbohydrate rich food items and sweets (food faddism) and occasionally she drank cooking oil. She was admitted in a local hospital and diagnosed as having Bipolar affective disorder. She was conservatively managed with antipsychotics and benzodiazepines, without any significant improvement. In spite of her illness, she was able to dress, bathe, and navigate inside her house without any confusion. Gradually, she started having decreased verbal output often resorting to gestures. Her vocabulary dwindled, and she started using the same pair of words again and again. Her vocabulary became restricted to the following six words “Pants-Shirt,” “Milk-Jaggery,” and “inside-outside” which she would interchangeably use. She would use these terms as responses to all questions, including when she was asked to provide her name and address. Her routine investigations and specific investigations to rule out acquired and reversible causes of dementia were noncontributory. Magnetic resonance imaging showed atrophy of the inferior frontal and temporal lobes with asymmetrical widening of the left sylvian fissure and knife cut atrophy of the anterior temporal and inferior frontal gyrus, indicating PNFA type of FTD [Figure 1]a,[Figure 1]b,[Figure 1]c. She was prescribed Memantine, Fluoxetine, and symptomatic management for incontinence with Tolterodine. She is currently continent with decreased agitation and anger, but after 3 months, her vocabulary is still restricted.
|Figure 1: Magnetic resonance imaging demonstrating (a) dilated temporal horn, left >right. (b) Knife cut atrophy of anterior temporal lobe with perisylvian atrophy. (c) Lateral and medial temporal atrophy|
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Even though PNFA is seen in only 20% of patients with FTD, it often begins by deficits in speech or language and social decorum is almost always intact with some patients progressing to the bvFTD., In this patient, the illness followed a reverse course involving the orbitofrontal and dorsolateral frontal lobes first in terms of behavior, higher executive functions, and working memory, and then later affecting the expressive language area with limited vocabulary and absent insight causing distress to her family members. The words used interchangeably in pairs for communication by the patient are also only nouns and pronouns with the complete omission of verbs. The rare occurrence of PNFA has to be considered in elderly patients, especially females, who in addition have cognitive decline, behavioral problems and absent insight, also have limited vocabulary and frequently use sets of nouns to express themselves.
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