Progressive facial hemiatrophy revisited: a role for sympathetic dysfunction

Area of Science:

• Neurology
• Radiology
• Vascular Ultrasound

Background:

• Progressive facial hemiatrophy (PFH) is a rare condition characterized by gradual loss of tissue on one side of the face.
• The exact etiology of PFH remains unclear, with various theories proposed.

Observation:

• A 63-year-old male presented with a decade-long history of right-sided facial atrophy and recent onset of facial pain.
• Brain MRI revealed left frontoparietal atrophy, contralateral to the facial hemiatrophy.
• Transcranial Doppler (TCD) ultrasound showed evidence of autonomic dysfunction ipsilateral to the brain atrophy.

Findings:

• The case presents unusual findings of contralateral brain atrophy in the context of progressive facial hemiatrophy.
• Autonomic dysfunction, specifically indicated by TCD findings, was observed ipsilateral to the cerebral atrophy.

Implications:

• This case expands the known clinical and radiological spectrum of progressive facial hemiatrophy.
• The findings suggest a potential pathogenic role for sympathetic nervous system dysfunction in PFH.
• Further research into the neurovascular mechanisms underlying PFH is warranted.