Related Experiment Videos
Degos' disease: radiological and immunological aspects
H Yoshikawa1, T Maruta, H Yokoji
1Department of Neurology, Kanazawa University, School of Medicine, Ishikawa, Japan.
Acta Neurologica Scandinavica
|November 1, 1996
Summary
Degos disease, a rare condition, can cause sudden paraplegia and neurological deficits in young patients. This case highlights its complex vascular and immunological involvement.
Area of Science:
- Neurology
- Vascular Medicine
- Immunology
Background:
- Degos disease is a rare systemic vasculopathy characterized by characteristic skin lesions and potential multi-organ involvement.
- Neurological manifestations are uncommon but can be severe, impacting the central and peripheral nervous systems.
Observation:
- An 18-year-old presented with acute paraplegia, rt-hemianopsia, paraparesis, and neurogenic bladder.
- Brain MRI revealed cerebral infarctions, hemorrhage, and dural enhancement.
- Cerebral and renal angiography showed arterial stenosis, ectasia, and aneurysms.
Findings:
- Skin biopsy demonstrated hyperkeratosis, epidermal atrophy, and collagen necrobiosis.
- Laboratory tests indicated elevated thrombin-antithrombin III complex (TAT), plasmin-alpha 2 plasmin inhibitor complex (PIC), and CD8+CD11- cytotoxic T cells.
Implications:
- This case underscores the potential for severe neurological complications in Degos disease.
- Findings suggest a complex interplay of prothrombotic, fibrinolytic, and immunological dysregulation.
- Early recognition and multidisciplinary management are crucial for patients with Degos disease and neurological involvement.