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5: Movement disorders I: parkinsonism and the akinetic-rigid syndromes.

The major features of akinetic-rigid syndromes are bradykinesia (small, slow movements), rigidity and tremor, often summarised as "parkinsonism". Approximately 80% of akinetic-rigid syndromes are due to Parkinson's disease. Treatment of Parkinson's disease should be determined by level of disability and handicap. The combination of levodopa and peripheral dopadecarboxylase inhibitor is the most efficacious symptomatic treatment for Parkinson's disease. Motor fluctuations appear after 2-3 years of levodopa treatment, and affect at least 50% of patients after five years. Surgery can relieve refractory tremor and dyskinesias, but does not alter disease progression or need for drug therapy.

Aged↗

Increased 8-oxo-dGTPase in the mitochondria of substantia nigral neurons in Parkinson's disease.

There is growing evidence for the involvement of oxidative stress and mitochondrial respiratory failure in nigral neuronal death in Parkinson's disease (PD). We report increased immunoreactivity of 8-oxo-dGTPase (8-oxo-7, 8-dihydrodeoxyguanosine triphosphatase [hMTH1]), an enzyme known to play an important role in controlling spontaneous mutagenesis, and 8-oxo-7, 8-deoxyguanosine (8-oxo-dG) in the mitochondria of the substantia nigra of 6 PD patients. Our results suggest that hMTH1 might be a useful marker of oxidative stress and can be used to explore the relation between such oxidative stress and genomic instability.

8-Hydroxy-2'-Deoxyguanosine↗

Parkinson disease and its differentials. Diagnoses made easy.

BACKGROUND: Parkinson disease is a common neurological disorder that is both underdiagnosed and inaccurately diagnosed. There is no reliable biological marker or test that can differentiate between causes of parkinsonism. Even for experienced clinicians, the clinical diagnostic accuracy compared to post mortem findings is about 80%. OBJECTIVE: To discuss the clinical features that differentiate Parkinson disease from other important causes of parkinsonism. DISCUSSION: Although Parkinson disease is a common cause of parkinsonism, other candidates such as drug reactions, benign essential tremor, vascular disease and Lewy body dementia need to be differentiated. Incorrect diagnosis can result in complications related particularly to the use of levodopa and antipsychotic agents. Diagnostic accuracy is important to ensure appropriate management, to avoid complications and to assist patients to have realistic expectations and prognostic information about their condition.

Adult↗

[Heterogeneity of Parkinson's disease].

Parkinson's disease was, until recently, a unique disease. The discovery of several genetic factors has emphasized the heterogeneity of the disease. Analyse of structure and function of these gene products points to their critical role in dopaminergic neurons death and pathophysiology of parkinson's disease. In the mean time the clinical constellation of parkinsonian syndromes have been lumped into "synucleinopathies" and "taupathies" that both share common pathologic lesions. It is likely that clarification of the combined genetic and environmental factors undelying these various disorders will lead to novel diagnostic and therapeutic strategies.

Diagnosis, Differential↗

Deep brain stimulation in movement disorders. The applications reconsidered.

In this study the role of DBS in advanced Parkinson disease (PD) is re-evaluated and new indications in the field of movement disorders are explored. The authors performed unilateral pallidal stimulation in 26 patients with advanced PD. At long-term follow-up review, the motor effect is unsatisfying. The authors conclude that unilateral pallidal stimulation is not a favourable treatment option for patients with advanced PD. Bilateral subthalamic nucleus stimulation was performed in twenty patients with advanced PD. After a minimum follow-up of 4 years, there was still a significant improvement in motor function and functional performance in all patients. One side-effect of the stimulation was however rather concerning: four patients presented with stimulation-induced hypomanic to manic characteristics. Bilateral subthalamic stimulation was performed in four patients with multiple system atrophy-parkinsonism. At long-term follow-up, the patients fared better with than without stimulation. The authors finally present a completely new indication for DBS: Tourette syndrome (TS). They review the literature on surgical procedures performed in TS, all of which consisted of making lesions. Three TS patients are presented on which bilateral thalamic stimulation was performed. At long-term follow-up, all major tics had disappeared, as well as associated behavioral disturbances.

Adult↗