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Relapsing ischemic encephaloenteropathy and cryoglobulinemia
P G Ince1, P Duffey, H R Cochrane
1Departments of Neuropathology, University of Newcastle upon Tyne, United Kingdom. paulince@hamsterley.u-net.com
Neurology
|November 30, 2000
Summary
Cryoglobulinemia, a rare condition, can cause recurrent encephalopathy and gastrointestinal ischemia. Autopsies revealed small vessel damage, highlighting the need for early diagnosis of this potentially fatal syndrome.
Area of Science:
- Neurology
- Immunology
- Gastroenterology
Background:
- Cryoglobulinemia is an uncommon cause of neurological dysfunction.
- Encephalopathy associated with gastrointestinal ischemia is poorly understood.
Observation:
- Three patients presented with recurrent encephalopathy and gastrointestinal ischemia.
- Cryoglobulinemia was diagnosed during life in only one patient.
Findings:
- Autopsies revealed diffuse cerebral, enteral, and systemic small vessel lesions.
- Lesions were immunoreactive for immunoglobulins, consistent with mixed essential cryoglobulinemia.
Implications:
- This syndrome can be misdiagnosed as nonorganic, delaying critical treatment.
- Early recognition of cryoglobulinemia is vital for managing this potentially fatal condition.