Progressive Supranuclear Palsy Research Paper Example | Topics and Well Written Essays - 1500 words

Progressive Supranuclear Palsy - Research Paper Example Men are known to be affected by this condition to a greater extent as compared to the females (Golbe and Ohman-Strickland, 2007). Clinical Presentation The PSP encompasses distinguishing mild dementia, lack of memory, sluggishness of contemplation procedure, poignant or behavior modifications encompassing lethargy or hopelessness with infrequent outbreaks or petulance and weaken capability to maneuver attained information, the condition leads to subcortical dementia, the characteristic feature of PSP. The clinical features include parkinsonism, though with rigidity in extension rather than flexion and tremor is usually minimal. In addition there must be a supranuclear paralysis of eye movements, usually downgaze for the appropriate diagnosis. These features are accompanied by pyramidal signs and cognitive impairment. The condition belongs to the family of taupathies and engross both cortical and subcortical structures (Stamelou et al, 2010). PSP is a symmetric, akinetic-rigid syndrom e, frontal shortfalls, postural instability as well as falls. Early gait interruption with sub-zero, micrographia and hypophonia comprise an additional deviation entitled pure akinesia with gait freezing (Williams and Lee, 2009). Observations reveal that in case of progressive supranuclear palsy, cortical lesions play an imperative role in highlighting the symptoms and signs of the disease. The patient thus presents supranuclear opthalmoplegia. Apart from these features, additional clinical characteristics involve, progressive asymmetric dystonia, apraxia and cortical sensory loss (PSP-corticobasal syndrome) (Josephs et al, 2005). The heterogeneity of the medical management emerges to trail distinction in the anatomical allocation of diverse, PSP-specific histopathological amendments (Willians and Lees, 2009). These features are supported with retrospective neuropathological research. Neuropathology Neuropathologically, PSP is defined as an accumulation of neurofibrillary tangles an d thereby loss of neuronal cells encompasses cortical and subcortical constitution, in particular the subthalamic nuclei, globus pallidus, red nucleus, superior colliculi, pretectal regions, periaqueductal grey matter, substantia nigra, thalamus, cerebellum, the entire pontine tegmentum, oculomotor nucleus, medulla, dentate nucleus and the spinal cord (Williams and Lees, 2009). Cases which do not predispose these features are difficult to diagnose. Research studies reveal that genetic mutations in the tau gene present on the chromosome 17 is responsible for the frontotemporal dementias. Stanford et al (2000), have identified that a silent mutation (S305S) in the tau gene is responsible for noteworthy atrophy or cellular collapse of the frontal and temporal cortices. Histopathological tests display intracellular, somatodendritic tau-aggregates which are shown by the silver staining (Dickson, 1999). Presence of neurofibrillary tangles in neurons, neuropil threads in neuronal processes , coiled twisting bodies in oligodendrocytes, tufted astrocytes in the basal ganglia, amygdala and motor cortex as well as nonexistence of neuritic plaques aid to discriminate PSP from other tauopathies. Taupathies is a common terminology employed to neurodegenerative disorders displaying tau-pathology in the glial or neuronal cells. Tau protein is expressed in neurons, associated with the microtubule.