Which clinical history findings are characteristic of frontotemporal lobe dementia with motor neuron disease (FTD/MND)?

Updated: Apr 11, 2017
  • Author: Jasvinder Chawla, MD, MBA; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Frontotemporal lobe dementia with motor neuron disease (FTD/MND) usually presents as a change in personality with deterioration in social conduct. Initial behavioral changes vary but include abulia, apathy, and reduced spontaneity and/or initiation. Some patients become strikingly disinhibited, overactive, and frankly inappropriate, with emotional lability. With disease progression, however, even those patients manifesting disinhibition and restlessness become increasingly apathetic.

Stereotypic behavior and repetitive rituals of hoarding, dressing, wandering, and toileting can be observed. In addition, patients may overeat, exhibit hyperoral tendencies, and develop food fads (although this is more exceptional). Some patients may hold food in their mouth for prolonged periods without swallowing.

Dynamic, spontaneous speech output progressively declines, resulting in anarthria and mutism. A subset of patients presents with rapidly progressive aphasia. Despite progression to anarthria, autopsy studies show that anarthria can occur in the absence of significant involvement of the hypoglossal nucleus. Although there is significant memory impairment, this is not as distinguishing as the frontal lobe or language features.

Posterior cortical functions (eg, visuospatial skills) are preserved and/or spared until the preterminal stages.

The clinical pattern reflects the topographic pattern of atrophy, often visible radiographically, with asymmetrical frontotemporal atrophy. If asymmetrically worse in the left (language-dominant) hemisphere, aphasia is a likely and prominent clinical feature.

Throughout the course of the disease, signs and symptoms of MND also progress. Cognitive changes often precede signs of MND. Limb weakness and dysphagia eventually become disabling, although some patients have a primarily bulbar pattern of weakness with relative sparing of limb strength.

Consensus clinical criteria detailing core and supportive features for FTD syndromes have been published.

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