**8.1 Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD)**

There is icreasing clinical, imaging and neurophysiological evidence that ALS represents a multisystem neurodegerative disease. Neurodegeneration is not restricted to motor neurons, but also includes parts of the brain other than the motor cortex, especially the preforntal and/or anterior temporal lobe, that contribute to the clinical syndrome. In some cases an evident dementia that resembles frontotemporal degeneration (FTD) was observed. It is now suggested that ALS and FTD are closely related conditions with overlapping clinical, pathological, radiological, and genetic characteristics. The presence of a frontal dementia in ALS has also crucial practical consequences for management of the patients, whose disorder requires critical life decisions for enteral nutrition and respiratory complications. (Zago, et al. 2010).

#### **8.2 The new classification of cognitive and behavioral disorder in ALS**

In 2009, Strong and colleagues articulated new guidelines to direct ongoing investigations of cognitive and behavioral syndromes in ALS (Strong et al., 2009). To define the


Table 3. Neuropsychological test for the evaluation of cognitive/behavioral impairment in ALS (modifed from Strong et al. 2009), with permission.

system, including cortical areas which are consistently involved in FTD. It comes as no surprise, therefore, that a proportion of patients presenting with MND manifest cognitive and/or behavioural changes which may be severe enough in some instances to reach criteria

There is icreasing clinical, imaging and neurophysiological evidence that ALS represents a multisystem neurodegerative disease. Neurodegeneration is not restricted to motor neurons, but also includes parts of the brain other than the motor cortex, especially the preforntal and/or anterior temporal lobe, that contribute to the clinical syndrome. In some cases an evident dementia that resembles frontotemporal degeneration (FTD) was observed. It is now suggested that ALS and FTD are closely related conditions with overlapping clinical, pathological, radiological, and genetic characteristics. The presence of a frontal dementia in ALS has also crucial practical consequences for management of the patients, whose disorder requires critical life decisions for enteral nutrition and respiratory complications. (Zago, et

In 2009, Strong and colleagues articulated new guidelines to direct ongoing investigations of cognitive and behavioral syndromes in ALS (Strong et al., 2009). To define the

> Association, FAS, D Words, Animal fluency, Written Word Fluency Design Fluency, California Card Sorting Test, Stroop

Learning Test II, Warrington Recognition Memory Test, Wechsler Logical Memory and Visual Reproduction, Wechsler Paired Associate Learning. Kndrik Object Learning Test

Digit Modit Modality Test, Paced Auditory Serial Addition

Trees, Peabody Picture Vocabulary Test, British Picture Vocabulary Test, Test for the Reception of grammar

Block Design, Motor-Free Visual Perception Test-Revised,

Neuropsychiatric inventory, frontal Behavoural Inventory,

Colour-Word Interference Test, Trail Making Test

**8.1 Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD)** 

**8.2 The new classification of cognitive and behavioral disorder in ALS** 

**Executive measure** Wisconsin Card Sorting Test, Controlled Oral Word

**Memory/Learning** Rey Auditory Verbal Learning Test, California Verbal

Task, Digit Span

ALS (modifed from Strong et al. 2009), with permission.

**Attention/Concentration** Verbal Serial Attention Test, Consonant trigrams Test, Symbol

**Language** Boston Naming Test, Graded Naming Test, Pyramid and palm

**Visual/Spatial** Judgement of line Orientation, Benton Facial Recognition Test,

Frontal Systems Behavioural Scale Table 3. Neuropsychological test for the evaluation of cognitive/behavioral impairment in

Visual Object and Space Perception Battery

for frank FTD.(Irwin, et al, 2007).

**Domain Test**

**Emotional/ Behavioral** 

**functioning** 

al. 2010).

neuropsychological status of ALS patients a framework was based on four different axes. Axis I is based on the EL Escorial criteria proposed in 1998, that includes possible, probable, and definite ALS clinical subtypes. This multidimensional approach incorporates several criteria (Brooks et al, 2000). The novelty of the classification lies primarily in Axis II with the proposal of five categories which classify ALS patients along a continuum: (1) ALS patients cognitively and behaviorally intact: (2) ALS patients with mild cognitive impairments; (3) ALS patients with mild behavioral impairment; (4) ALS with a full-fledged fronto-temporal dementia; (5) ALS with other non FTD-forms of dementia.

Axis III indicates the presence, in addition to frontotemporal impairments, of additional non-motoneuronal disease manifestations such as extrapyramidal signs, cerebellar degenerations, autonomic dysfunctions, sensory impairments, and ocular motility abnormalities. The absence of the above indicates a "pure form," while their presence defines "complicated forms" with additional pathological motor aspects. Axis IV, instead, provides the search for factors which could modify the course of the disease. Several disease modifiers have been reported in literature associated with longer survival, age at symptom onset (< 45 years), gender (male/sex), and site of the disease onset (bulbar or limb).
