Neurol., May 1993, 50:489-491. 15. Ostermeyer-Shoaib, B. IgG subclass deficiency in amyotrophic lateral sclerosis. Acta Neurol. Scand., 1993, 87:192-194.
Olivopontocerebellar Atrophy Abstract Olivopontocerebellar Atrophy(OPCA), is characterized by neuronal degeneration of the cerebellar cortex, the inferior olive, and the pons. The symptoms associated with it are primarily cerebellar ataxia with disturbances in equilibrium and gait. However, broader symptomology is usually seen with OPCA. Current research is focusing on three primary systems thought to be responsible for the etiology of OPCA. They are excitatory amino acid disturbances, oligodendroglial microtubular tangles, and phospholipid metabolism disorders.
Genetically-altered, dopamine-producing tissues are currently being proposed as an alternative in transplant therapy of PD. As techniques become more refined, such "brain-grafting" may be the panacea for not only PD, but also for other debilitating diseases such as Huntington’s disease and Alzheimer’s disease. According to Fitzgerald (1992:215), the "cardinal pathological feature [of PD] is loss of neurons from the substantia nigra". Most of this loss occurs in the SNpc, of which approxima... ... middle of paper ... ...zgerald, M. J. T. Neuroanatomy: Basic and Clinical. London: Bailliere Tindall, 1992.
Niemann: Pick's Disease Niemann Pick disease consists of a group of genetic disorders in which the common feature is a varying degree of sphingomyelin storage in certain tissues of the body. According to the current classification based on the enzymatic defect underlying these disorders, two main groups are distinguished. The first group, which comprises type A, which is characterized by a severe deficiency in acid sphingomyelinase activity, includes infantile neuronopathic form; and type B, an adult chronic form without neurologic symptoms. In the second heterogeneous group called type C, neuro-visceral involvement is massive and lipid metabolism is affected. The sphingomyelin that accumulates in the lysosomes of the Niemann-Pick disease cells is thought to arise from the degradation of cells and their organelles since it is a major component of all mammalian cell membranes, the myelin sheath and the erythrocyte stroma.
Multiple sclerosis Multiple sclerosis interrupts the transmission of nerve impulses, resulting in a wide range of symptoms and decreased function. Comprehensive patient education about the nature and treatment of the disease can make a big difference for these patients. Multiple sclerosis (MS) is a persistent autoimmune disease of the central nervous system (CNS) in which the immune system erroneously attacks the myelin sheath that protects nerve cell fibers in the brain and spinal cord. This process, which is called demyelization, leads to diminished or lost CNS function, muscle weakness, complexity with synchronization and balance, visual disturbances, exhaustion, cognitive impairments, and an array of associated symptoms. (Bornstein M. Miller A, Slagle S, et al.
1992; 51: 371-77. Fredrico, A. "The Clinical Aspect of Adult Hexosaminidase Deficiencies." Developmental Neuroscience. 1991; 13: 280-287 Fukumizu, M. "Tay-Sachs Disease: Progression of Changes on Neuroimaging in Four Cases."
Parkinson’s Disease (from hereon PD) is an extrapyramidal disorder characterized primarily by massive idiopathic degeneration of dopaminergic neurons in the substantia nigra, resulting in greatly decreased levels of dopamine in the striatum. The diagnosis, which is essentially a clinical judgment due to the lack, thus far of a simple diagnostic test, has historically been on the basis of the presence of at least two of the three main features of PD: bradykinesia (or akinesia or hypokinesia), rigidity, and resting tremor. In addition to these symptoms, most PD patients also show postural disturbances, impaired righting reflexes, and abnormal ocular movements. The extent of the disease and its symptoms can be quantified by one of a few similar scales, including the motor examination of the Unified Rating Scale for Parkinson’s Disease, in which each of 14 motor aspects are scored from 0 to 4 and the scores totaled. The items in this particular scale are as follows: 1) speech; 2) facial expression; 3) tremor at rest; 4) action or postural tremor of hands; 5) rigidity; 6) finger taps; 7) hand movements; 8) rapid alternating movements of hands; 9) foot agility; 10) arising from chair; 11) posture; 12) postural stability; 13) gait; and 14) body bradykinesia or hypokinesia.
‘Huntington’s disease (HD) is an autosomal dominant progressive neurodegenerative disorder characterized by personality changes, motor impairment and subcortical dementia. It is associated with a selective neuronal cell death occurring primarily in the cortex and striatum.’ (Scherzinger et al, 1997). HD causes emotional problems, uncontrolled movements and the loss of thinking ability. It can lead to disability and death from the illness. There are two forms of this disease: adult-onset and early-onset (juvenile).