By Hiroshi Mitsumoto MD
Amyotrophic Lateral Sclerosis provides the main accomplished, clinically-focused info on ALS (Lou Gehrig's sickness) in print. the 3 authors, widely recognized for his or her paintings during this sector, supply cohesive and balanced assurance of this syndrome, together with the heritage, scientific positive aspects, pathology and pathogenesis, therapy and administration of ALS. To facilitate figuring out, each one bankruptcy is more desirable by way of finished tables and figures, in addition to distinct bankruptcy summaries.
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Extra resources for Amyotrophic Lateral Sclerosis
Charcot12 setforth the clinical and pathologic characteristics of ALS in a form that has since been modified very little. His description of ALS was based on 20 patents History, Terminology, and Classification of ALS 7 Table 1-2. CLINICAL FINDINGS OF ALS ACCORDING TO CHARCOT Upper Motor Neuron Findings Spasmodic contracture Temporary or permanent rigidity Flexibilitas cerea (cataleptic state of limbs) Exaggerated tendon reflexes Spinal epilepsy or trepidation Tremor on movement Involuntary extension or flexion "Simple" or "pure" paralysis Lower Motor Neuron Findings Atrophy of muscles "en masse" Fibrillary contractions or "fascicular shaking" (indicating fasciculations) Preservation of contractility to faradic stimulation Bulbar Findings Transverse wrinkles on forehead Contracture of lower face Widened mouth Crying expression Weakness of lips Drooling Nasal voice Thickness to or loss of speech Small, tremulous, wrinkled tongue (or may be of normal size though weak) Difficulty in swallowing Respiratory and circulatory disturbances Sensory Findings No loss of sensation Onset with frequent lively pains, numbness and tingling Muscles tender on pressure and passive movement Mental Findings Intellect undisturbed Other Findings No paralysis of bladder or rectum No bed sores Source: Adapted from Goldblatt, D: Motor neuron disease: Historical introduction.
PBP is exceedingly rare; usually the limb and paraspinal muscles are affected and thus PBP may not be considered as an independent condition. tracts may be involved, resulting in dysphagia and dysarthria that aggravates the already established lower motor neuron involvement at the brain-stem level. These features define classic ALS, in the sense that it was described by Charcot, and stand in contrast to subsets of ALS. At onset, ALS presents with lower motor neuron (LMN) involvement (LMN-onset or the PMA form of ALS), upper motor neuron (UMN) involvement (UMN-onset or PLS form), or bulbar involvement (bulbar-onset or PBP form) (Fig.
Although usually similar clinically,125 these forms of ALS differ in epidemiology, pathology (see Chapter 11), and possibly in pathogenesis (see Chapter 12). , progressive bulbar palsy, progressive spinal muscular atrophy) in reporting the epidemiology of motor neuron disease. Here, we will use the term ALS to encompass all these types of motor neuron degeneration because most cases eventually progress to become classic ALS. 84,110,131 True progressive bulbar palsy is rare and progressive spinal muscular atrophy is uncommon (see Chapter 1).
Amyotrophic Lateral Sclerosis by Hiroshi Mitsumoto MD