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February 2001: A 74 year old man with a history over 3 months of increasing dyspnea and malaise
Abstract:
The February COM: A man of 78 years with idiopathic late-onset cerebellar ataxia developed renal failure in association with a high ESR and positive pANCA. This was complicated by a subclinical spinal subarachnoid hemorrhage which was related to necrotizing inflammation of small leptomeningeal vessels. Renal cortical infarcts were due to similar inflammation in arcuate and interlobular arteries. Spinal subarachnoid hemorrhage is rare and usually due to rupture of an arteriovenous malformation. However, an immunogenic connective tissue disorder should be considered in the differential diagnosis. In this case, the histology and results of an autoantibody screen support a diagnosis of microscopic polyangiitis.
Insights
A 78-year-old man with cerebellar ataxia developed kidney failure and spinal hemorrhage due to microscopic polyangiitis, an autoimmune disorder causing blood vessel inflammation.
Area of Science:
- Nephrology
- Neurology
- Rheumatology
Background:
- Idiopathic late-onset cerebellar ataxia can present with multisystemic complications.
- Renal failure and neurological events necessitate a thorough differential diagnosis.
Observation:
- A 78-year-old male with cerebellar ataxia presented with acute renal failure, elevated ESR, and positive pANCA.
- The patient developed a subclinical spinal subarachnoid hemorrhage and renal cortical infarcts.
Findings:
- Histopathology revealed necrotizing inflammation in leptomeningeal vessels and renal arteries.
- Autoantibody screening supported a diagnosis of microscopic polyangiitis.
Implications:
- Microscopic polyangiitis should be considered in the differential diagnosis of unexplained renal failure and neurological complications.
- This case highlights the rare association of spinal subarachnoid hemorrhage with vasculitis.
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