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10 marksDrNB 2025NeurologyMovement Disorders
Describe the approach to a young patient with extrapyramidal and cognitive dysfunction.
Extrapyramidal and cognitive dysfunction in a young patient refers to a clinical syndrome characterized by movement disorders (rigidity, tremors, dystonia, chorea) alongside cognitive decline or dementia occurring typically before 40 years of age (Bradley's Neurology, 8e, Ch. 23).
Extrapyramidal-cognitive syndromes in young patients arise from diverse etiologies affecting basal ganglia and cortical networks involved in motor control and cognition. Key mechanisms include:
- Genetic neurodegenerative disorders (e.g., Huntington’s disease, Wilson’s disease)
- Metabolic and mitochondrial dysfunction
- Neuroinflammation and autoimmune disorders
- Toxic and drug-induced causes
| Classification | Examples | Pathophysiology |
|---|---|---|
| Genetic | Huntington’s disease, Wilson’s disease, Neurodegeneration with Brain Iron Accumulation (NBIA) | Protein aggregation, copper accumulation, iron overload |
| Metabolic | Wilson’s disease, mitochondrial cytopathies | Copper/metal accumulation, energy failure |
| Autoimmune/Inflammatory | Susac syndrome, autoimmune basal ganglia encephalitis | Immune-mediated neuronal injury |
| Toxic/Drug-Induced | MPTP toxicity, antipsychotic-induced Parkinsonism | Dopamine receptor blockade or mitochondrial toxicity |
(Harrison's 21e, Ch. 342; Bradley's Neurology, 8e, Ch. 23)
References
Bradley's Neurology, 8e, Ch. 23Harrison's 21e, Ch. 342Harrison's 21e, Ch. 342; Bradley's Neurology, 8e, Ch. 23Harrison's 21e, Ch. 342; Bradley’s Neurology, 8e