Table 2. [TARDBP-Related Amyotrophic Lateral Sclerosis-Frontotemporal Dementia: Frequency of Disease Features]. - GeneReviews®

Feature	Frequency	Comment
Motor findings
Upper motor neuron disease	●			Weakness, spasticity, altered muscle tone
Lower motor neuron disease	●			Weakness, fasciculations, atrophy
Bulbar involvement	Dysarthria		●		Motor language deficit
Dysphagia		●		Problems swallowing food &/or liquids
Parkinsonism			●	Extrapyramidal findings such as resting tremor, rigidity, akinesia
Cognitive deterioration
Executive dysfunction			●	Issues w/planning, problem solving, organizing
Memory impairment			●	Amnesia, mostly recent memory
Language impairment			●	Deficits in speech production or comprehension
Apraxia			●	Impaired execution of learned motor tasks
Dyscalculia			●	Diminished mathematical reasoning
Behavioral & psychological manifestations of dementia
Disinhibition			●	Impulsivity, socially unacceptable behavior, risk taking
Apathy			●	Indifference, lack of interest
Delusions/hallucinations			●	Often bizarre delusions, mostly visual hallucinations
Psychosis			●	Psychosis, often as initial symptom
Anxiety			●	Generalized stress & apprehension
Repetitive, compulsive behavior			●	Often complex, ritualistic behaviors mimicking obsessive-compulsive disorder
Preference for sweet food			●	↑ craving for sweet foods

Feature

Frequency

Comment

Nearly all

Common ¹

Infrequent

Motor findings

Upper motor neuron disease

●

Weakness, spasticity, altered muscle tone

Lower motor neuron disease

●

Weakness, fasciculations, atrophy

Bulbar involvement

Dysarthria

●

Motor language deficit

Dysphagia

●

Problems swallowing food &/or liquids

Parkinsonism

●

Extrapyramidal findings such as resting tremor, rigidity, akinesia

Cognitive deterioration

Executive dysfunction

●

Issues w/planning, problem solving, organizing

Memory impairment

●

Amnesia, mostly recent memory

Language impairment

●

Deficits in speech production or comprehension

Apraxia

●

Impaired execution of learned motor tasks

Dyscalculia

●

Diminished mathematical reasoning

Behavioral & psychological manifestations of dementia

Disinhibition

●

Impulsivity, socially unacceptable behavior, risk taking

Apathy

●

Indifference, lack of interest

Delusions/hallucinations

●

Often bizarre delusions, mostly visual hallucinations

Psychosis

●

Psychosis, often as initial symptom

Anxiety

●

Generalized stress & apprehension

Repetitive, compulsive behavior

●

Often complex, ritualistic behaviors mimicking obsessive-compulsive disorder

Preference for sweet food

●

↑ craving for sweet foods

From: TARDBP-Related Amyotrophic Lateral Sclerosis-Frontotemporal Dementia

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Adam MP, Feldman J, Mirzaa GM, et al., editors.

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