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[Melkersson's-Rosenthal's syndrome].

R Sujić, J Potić, N Despotović

Srpski Arhiv Za Celokupno Lekarstvo

|September 1, 1994

Summary

Melkersson-Rosenthal syndrome, a rare neurological disorder, can present with unusual complications like stroke and vascular disease in young adults. This case highlights the syndrome's complexity and the need for ongoing patient monitoring.

Area of Science:

Neurology
Immunology
Vascular Medicine

Background:

Melkersson-Rosenthal syndrome (MRS) is a rare neurological disorder characterized by the triad of recurrent facial edema, facial palsy, and fissured tongue.
While typically diagnosed in younger individuals, its association with severe systemic complications, particularly in early adulthood, is less understood.

Observation:

A 33-year-old male patient with MRS presented with acute cerebral hemispheric ischemic infarction, a rare manifestation for his age.
The patient exhibited hypertension, persistent hematuria, and advanced cerebral stenoocclusive vascular disease, alongside the classic MRS symptoms.
Immunological investigations revealed slightly elevated serum immunoglobulin and B lymphocytes, with high blood histamine levels, suggesting an underlying immune dysregulation.

Findings:

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The patient displayed hypersensitivity to various external antigens, including exhaust, fumes, Coca-Cola, and the fungicide T.M.T.D.
This case underscores the potential for severe and atypical vascular complications in MRS patients.
Elevated histamine and immune markers may play a role in the pathogenesis of MRS and its associated complications.

Implications:

This case highlights the critical need for comprehensive diagnostic workups and long-term monitoring in patients diagnosed with Melkersson-Rosenthal syndrome.
Early identification and management of vascular risk factors and potential triggers are essential for improving patient outcomes.
Further research into the immunological underpinnings of MRS may reveal novel therapeutic targets for managing this complex syndrome.