Primary lateral sclerosis

Verma A, Berger IR (2008) Primary lateral sclerosis with HIV-1 infection. Neurology 70(7) 575-577... 

Singh et al. (2003) put forward a more latest predictive model (design) for the cytochrome P-450 (CYP) 3A4 metabolism. This method exclusively rests upon the primary lateral sclerosis (PLS), however, one of the descriptors is totally based on acute myocardial infarction (AMl)-calculated H-atom abstraction process. 

Frontotemporal dementia involves an early and primary degenerative process of frontal and/or temporal cortex. Several disorders fall under this rubric, such as Pick s disease and the dementia associated with amyotrophic lateral sclerosis (ALS). ALS is a degenerative disease of upper motor neurons that is sometimes accompanied by a frontal lobe dementia (Vercelletto et al. 1999 Abe et al. 1997). ALS has been associated with mutations in the free radical scavenging enzyme superoxide dismutase 1 (Price et al. 1997). Pick s disease is associated histologically with a loss of neurons and cytoplasmic Pick bodies in surviving neurons. 

AD is a primary degenerative dementia affecting humans as young as in their forties. The German physician Alois Alzheimer first described the disease in 1906. It is characterized by senile plaques and paired helical filaments (PHFs), and the severity of the condition directly parallels their number.63 The involvement of aluminum in this and related dementias (dialysis encephalopathy and amyotrophic lateral sclerosis — Parkinson dementia in Guam) is currently a highly contested issue in neurological research. 

The disease models can be grouped into four primary categories (Fig. 20.1). (1) Motor neuron diseases, in which the death of motor neuron somata in the spinal cord results in denervation of the muscles, progressive flaccid paralysis, and usually premature death. In humans, examples of such diseases would include amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). (2) Peripheral neuropathies, in which axonal integrity or conduction is not maintained, resulting in axon degeneration and impaired connectivity of the nervous system and the musculature. 

Pathological activation of glutamate receptors is a common feature and one of the primary causes of neuronal death in acute neuronal injury (such as trauma, epilepsy, and brain ischemia) and chronic neurodegenerative diseases (such as Parkinson s disease, Alzheimer diseases, amyotrophic lateral sclerosis, and AIDS dementia) (Choi, 1988 Doble, 1999 Lipton and Rosemberg, 1994). In particular, elevation of extracellular glutamate level is a key factor in the development of neuronal damage under ischemic conditions. 

Electromyography (EMG) and nerve conduction velocities (NCVs) are used to assess the function of the peripheral nerves, neuromuscular junction, and muscles. NCVs are measured by stimulating the nerve and recording the speed of conduction of the impulse. NCVs can be used to detect the presence of localized peripheral nerve inj uries (carpal tunnel, etc.) or diffuse symmetric neuropathies (which may be inherited or acquired). EMG assesses muscle dysfunction due to primary muscle disease or secondary to nerve injury. This test is used to diagnose peripheral neuropathies (inherited and acquired), GuiUain-Barre syndrome, myasthenia gravis, amyotrophic lateral sclerosis, radiculopathies, and muscle diseases. 

Liu D, Bao F, Wen J, Liu J (2007) Mutation of superoxide dismutase elevates reactive species comparison of nitration and oxidation of proteins in different brain regions of transgenic mice with amyotrophic lateral sclerosis. Neuroscience 146 255-264 Loberto N, Prioni S, Bettiga A, Chigomo V, Prinetti A, Sonnino S (2005) The membrane environment of endogenous cellular prion protein in primary rat cerebellar neurons. J Neurochem 95 771-783... 

The Multiple Sclerosis Functional Composite (MSFC) was developed by the MS Consortium (Whitaker et al., 1995) for evaluating disabilities ranging from motor function to cognitive changes and to replace the EDSS. The three tools included in the MSFC are a timed 25 foot walk, the 9-hole peg test for hand dexterity and the Paced Auditory Serial Addition Test (PASAT). The MSFC is a quandfiable measure of disability but ten years later, it is sdll infrequently incorporated as a primary outcome measure because of the increased time element necessary to validate the individual tests and the dme necessary for their adminisd adon. 

D-penicillamine is so named because it was first isolated as an amine, from the degradation products of penicillin by Abraham et al [87]. Later studies showed the characteristic chemical behavior of D-penicillamine which involve three types of reactions, formation of disulphide links, formation of thiazolidine rings, and formation of metal complexes and chelates [67]. It was first used in 1956 in the treatment of Wilson s disease [88]. D-penicillamine has since been used in the treatment of many diseases, such as cystinuria [89], rheumatoid arthritis [90-92], systemic sclerosis [93], primary bdiary cirrhosis [94], heavy metal poisoning due to lead [95], cadmium [%], and mercury [97], and hyperviscosity syndrome [99]. In rheumatoid arthritis, D-peni-cdlamine has been widely accepted as an effective second line treatment. Despite of its effectiveness, it causes many adverse effects, such as skin rashes [99,100], taste abnormalities [100,101], hepatic dysfunction [102-104], gastrointestinal toxiciiy [99,105], proteinuria [100,106], hematuria [107, 108], thrombocytopenia [92, 109], aplastic anemia [110], lupus-like syndrome [111, 112], Goodpasture s-tike pulmonary renal syndrome [113-115], vasculitis [116,117], myasthenia gravis [118-122], polymyositis [123, 124], and dermatomyositis [125]. 

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