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**27** 

*Italy* 

**How to Assess Disease's Severity and Monitor** 

**Patients with Amyotrophic Lateral Sclerosis:** 

*1Department of Neuroscience, Unit of Neurology, Pisa University Medical School,* 

Amyotrophic Lateral Sclerosis (ALS) is a fatal, neurodegenerative disorder affecting upper and lower motor neurons; it's the commonest of the motor unit diseases in Europe and North America, characterized by a broad spectrum of clinical presentations mimicking vertebral stenosis, motor polyradiculoneuropathies and myopathies (Juergens et al., 1980; Swash, 2001). Striking asymmetry and selective involvement of individual groups of muscles, especially of hand and forearm, are typical early features of the disease. On average, delay from onset of symptoms to diagnosis is about 14 months and expected

Clinical neurophysiology in ALS plays a fundamental role both in the diagnosis of suspected disease and in the assessment of its severity and progression, offering a promising perspective to quantify muscle involvement and evaluate response to therapy (Brooks et al., 2000; Olney and Lomen-Hoerth, 2000; Beghi et al., 2002). Neuroimaging using magnetic resonance imaging (MRI), magnetic resonance spectroscopy (1HMRS), positron emission tomography (PET) and functional MRI may prove valuable results (Pohl et al., 2001), although they are complex, expensive and not always available. On the other hand, blood tests are necessary: hypoglycaemia insulinoma-related and autoimmune hyperthyroidism can be mistaken for ALS as they cause generalized muscle weakness, sometimes accompanied by fasciculations without a significant sensory impairment. Spinal fluid analysis could be helpful to rule out rare conditions closely mimicking ALS, such as meningeal infiltration with lymphoma, multifocal motor neuropathy (MMN) or a motor variant of inflammatory demyelinating neuropathy (CIDP). EMG investigation, usually performed with concentric needle electrodes (Daube et al., 2000), plays an essential role in the diagnosis and monitoring of ALS (Bromberg et al., 1993; Eisen, 2001; de Carvalho et al.,

survival commonly ranges from months to a few years (Andersen et al., 2007).

**1. Introduction** 

**Lessons from Neurophysiology** 

Chiara Pecori1,3, Chiara Rossi1 and Fabio Giannini4

*2Institute of Neuroscience, CNR, Pisa,* 

*Pisa University Medical School, Pisa,* 

*Siena University Medical School, Siena,* 

Ferdinando Sartucci1,2,3, Tommaso Bocci1,4, Lucia Briscese1,

*3Department of Neuroscience, Unit Outpatients Neurological Activity,* 

*4Department of Neurological Neurosurgical and Behavioural Sciences,* 

