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[Multineuritis in essential hypereosinophilia syndrome]
A Arnaud1, A Lagueny, E Hermosilla
1Service de Neurologie, CHU Hôpital Haut-Lévêque, Pessac.
Revue Neurologique
|August 1, 1998
Summary
A 72-year-old man with unexplained hypereosinophilia developed mononeuritis multiplex. Treatment with corticosteroids and immunosuppressants led to significant improvement, suggesting this syndrome can cause neuropathy.
Area of Science:
- Neurology
- Hematology
- Pathology
Background:
- Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent eosinophilia.
- Neurological manifestations in HES can be diverse, including peripheral neuropathy.
- Mononeuritis multiplex, a pattern of asymmetric nerve damage, is a less common but serious complication.
Observation:
- A 72-year-old male presented with significant weight loss, myalgia, and subacute onset of mononeuritis multiplex.
- Clinical examination revealed hypoesthesia and weakness in median and ulnar nerves bilaterally, and left femoral nerve weakness.
- Blood tests showed marked hypereosinophilia without an identifiable cause despite extensive investigation.
Findings:
- Electrodiagnostic studies indicated an axonal pattern of peripheral neuropathy.
- Nerve biopsy revealed acute myelino-axonal degeneration and endoneural edema, notably without vasculitis or eosinophils.
- The clinical presentation and laboratory findings supported a diagnosis of idiopathic hypereosinophilic syndrome with mononeuritis multiplex.
Implications:
- This case highlights the potential for idiopathic hypereosinophilic syndrome to manifest as axonal mononeuritis multiplex.
- The absence of eosinophils in nerve biopsy underscores the complexity of HES-associated neuropathies.
- Effective management involves corticosteroids and immunosuppressive therapy, leading to dramatic clinical improvement.