**3.4 Assessment of the quality of life**

6 Amyotrophic Lateral Sclerosis

The patients know about the devastating nature of ALS with only a short survival time remaining at the point of time at diagnosis, with a deteriorating quality of life, and without a pharmacological treatment option towards "healing". Therefore, a high prevalence of depression and anxiety in ALS patients would be expected. On the other hand, prevalence rates for depression in ALS patients range from 0-44% in the literature, whereas structured interviewing according to DSM-IV criteria reveals rates of 9-11% (Averill et al., 2007; Ferentinos et al., 2011; Kurt et al., 2007). Prevalence rates for anxiety in ALS have been reported with 0-30% (Kurt et al., 2007). Interestingly, the rate of depression and anxiety is not as high as expected (Huey et al., 2010; McLeod & Clarke, 2007; Norris et al., 2010), which means that the quality of life addressed by an individual is not dependent on the remaining

The association of schizophrenia with ALS has been reported (Enns et al., 1993; Howland, 1990; Yase et al., 1972), but the pathophysiological interrelation remains unknown. There

The use of ALS assessment techniques such as rating scales is to monitor disease progression , to make outcome parameters in clinical trials comparable; and to predict

Typical ALS assessment techniques comprehend (i) global scales which are based on clinical observation, (ii) measuring of the muscular strength, (iii) electrophysiological testing, and

Global scales subsume (i) scores based on subjective or historic data, such as the ALS functional rating scale (ALSFRS) (Cedarbaum & Stambler, 1997; The ALS CNTF treatment study (ACTS) phase I-II Study Group, 1996), and the ALS severity scale (ALSSS) (Hillel et al., 1989), (ii) scores based on clinical tests such as the Norris scale (Norris et al., 1974), or the Appel scale (Appel et al., 1987), and (iii) scales used both for ALS and other diseases such as the Schwab and England global rating scale (Schwab & England, 1969), or the modified

Measuring of the muscular strength includes quantitative tests, maximum voluntary isometric contraction, handheld dynamometry, Jamar grip strength, manual muscle testing. Moreover, the spirometric measurement of the forced vital capacity and maximum

Electrophysiological testing includes compound muscle action potentials (CMAPs) and motor unit number estimate (MUNE) (Shefner et al., 2011) or the motor unit number index

have been reports on a familial association (Burnstein, 1981).

efficacy of therapeutic strategies (Cudkowicz et al., 2004).

Ashworth spasticity scale (Bohannon & Smith, 1987).

voluntary ventilation can also be included in muscle tests.

(MUNIX) (Nandedkar et al., 2010; Neuwirth et al., 2011).

**3.3 Electrophysiological measurements** 

**2.2.4 Depression and anxiety** 

lifetime.

**2.2.5 Psychosis** 

**3. Measuring the disease** 

(iv) the assessment of the quality of life.

**3.1 Clinical assessment scales** 

**3.2 Muscular function testing** 

The quality of life is assessed by questionnaires such as the Short form – 36 (SF-36), Short form – 12 (SF-12), ALSQ-40, or the Sickness Impact Profile (reviewed in Epton et al., 2009; McGuire et al., 1997; Williams et al., 2008).
