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a.k.a. paralysis agitans Characterized pathologically by degeneration of pigmented & other brain stem nuclei, particularly the substancia nigra Associated with formation of eosinophilic neuronal inclusions called LEWY BODIES
Etiology
1. 2. 3. 4.
4 most prominent hypotheses Theory of accelerated aging Theory of toxin exposure Theory of genetic predisposition Theory of oxidative stress
Clinical signs & symptoms Tremors- most common symptom & initial complaint Rigidity- characterized as lead pipe or cogwheel Bradykinesia- slowness of movement Postural instability- least specific but the most disabling of all symptoms
6. 7. 8.
Prognosticating factors
Positive prognosticating features 1. early tremor 2. rigidity 3. family history Negative prognosticating features 1. bradykinesia 2. akinesia 3. postural instability 4. gait difficulties 5. cognitive defects 6. late age of onset
Treatment
There is no known cure for parkinsons disease. There methods of management include: a. pharmacology b. rehabilitation c. neurosurgical procedure
Pharmacology
Dopamine - does not cross the blood brain barrier L-DOPA - is the preferred tx for Parkinsons Dse. - administered orally with carbidopa; combination of these two is marketed as sinemet.
Rehabilitation
Relaxation exercises to decrease rigidity ROM exercises, stretching maneuvers Mat activities PNF techniques Frenkels exercise
Neurosurgical
Unsuccessful in relieving parkinsonian symptoms
Huntingtons Disease
Inherited in an autosomal dominant fashion The gene responsible for the disease has been located on the short arm of chromosome no. 4 Clinically onset is ussually between 30 and 50 years of age Disease is progressive and usually leads to fatal outcome within 15-20 years Presents with chorea, dementia and behavioral and mood disorders.
Hereditary ataxias
Can result from disorders of the cerebellum or its connections, the brainstem, the vestibular system, the dorsal columns and spinocerebellar tracts of the spinal cord, the dorsal root ganglia or the peripheral nerves. Some of these disorders also have associated systemic involvement (e.g. cardiomyopathy and diabetes mellitus in Friedrichs ataxia) Mainstay of treatment if the provision of physical therapy and occupational therapy directed at maintaining function for as long as possible. Gait training and instruction in the use of assistive devices to prevent falls and enhance mobility are useful.
Dystonia
A syndrome characterized by sustained muscle contractions resulting in abnormal movements or sustained postures. Result of sustain co-contraction of both the agonist and antagonist. Can be focal, multifocal, segmental or generalized.
Myoclonus
Common in pxs with idiopathic epilepsy Especially prominent in certain hereditary disorders characterized by seizures and progressive intellectual decline, such as lipid storage disease
Wilsons Disease
Hepatolenticular degeneration which is a rare familial disorder that is inherited in an autosomally recessive manner Occurs in both males and females between the 1st and 3rd decades Condition is characterized by excessive deposition of copper in the liver and brain Abnormal movements and postures of all sorts may occur with or without coexisting signs of liver involvement
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