Parry-Romberg syndrome presenting as status migrainosus

Area of Science:

• Neurology
• Dermatology
• Rare Diseases

Background:

• Parry-Romberg syndrome (PRS) is a rare disorder causing progressive hemiatrophy of facial tissues.
• Its etiology remains unknown, with theories suggesting trigeminal nerve sympathetic fiber injury.
• PRS can present with neurological complications like epilepsy and hemiparesis.

Observation:

• Facial linear scleroderma and en coup de sabre syndrome share overlapping features with PRS, complicating diagnosis.
• Central nervous system involvement in en coup de sabre syndrome further blurs diagnostic lines.
• A child initially diagnosed with en coup de sabre syndrome presented with severe status migrainosus.

Findings:

• Pathological, clinical, and neuroimaging findings confirmed a diagnosis of Parry-Romberg syndrome.
• The case underscores the diagnostic difficulties and controversies surrounding PRS and en coup de sabre syndrome.
• Similarities and contradictions between these conditions necessitate careful differential diagnosis.

Implications:

• Accurate diagnosis of PRS is crucial for appropriate management and understanding of associated neurological deficits.
• Distinguishing PRS from en coup de sabre syndrome requires comprehensive clinical and radiological assessment.
• Further research is needed to clarify the relationship and distinct features of these overlapping syndromes.