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Published on: June 28, 2019
[Progressive atrophy of the globus pallidus--case report]
Anna Bochyńska1, Wanda Lipczyńska-Łojkowska, Jerzy Kulczycki
1I Kliniki Neurologicznej IPiN w Warszawie.
Abstract:
The authors report a case of atrophy of the globus pallidus in a woman aged 25 years, diagnosed alive. The diagnosis was based to a large extent on MRI findings. Atrophy of the globus pallidus (AGP) is a rare disease, recognized mostly in neuropathological examination. Its etiopathogenesis has not been explained so far. Since no specific abnormalities have been detected in laboratory tests, the clinical diagnosis of AGP is only probable. However, AGP should be suspected if such extrapyramidal symptoms are present as dystonia, choreoathetosis, muscular rigidity, and characteristic localisation of lesions in MRI. At present only comprehensive symptomatic treatment is possible.
Insights
This case study highlights a rare diagnosis of globus pallidus atrophy (AGP) in a young woman, identified using MRI. AGP diagnosis relies on clinical symptoms and imaging due to unexplained causes and lack of specific lab tests.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Atrophy of the globus pallidus (AGP) is a rare neurological condition.
- AGP is typically identified through neuropathological examination post-mortem.
- The etiopathogenesis of AGP remains largely unexplained.
Observation:
- A case of AGP was diagnosed in a 25-year-old woman during her lifetime.
- Magnetic Resonance Imaging (MRI) played a crucial role in the diagnosis.
- The patient presented with extrapyramidal symptoms including dystonia and choreoathetosis.
Findings:
- The diagnosis of AGP in vivo is challenging due to the absence of specific laboratory markers.
- Characteristic MRI findings, alongside clinical symptoms like muscular rigidity, support the diagnosis.
- The case demonstrates the utility of advanced imaging in diagnosing rare neurological disorders.
Implications:
- This case underscores the importance of considering AGP in patients with unexplained extrapyramidal symptoms and characteristic MRI findings.
- Early clinical suspicion and advanced imaging can facilitate probable diagnosis of AGP during a patient's lifetime.
- Current treatment for AGP is limited to symptomatic management.
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