Background We reviewed the literature on published cases of progressive facial hemiatrophy (Parry-Romberg syndrome) to identify possible pathophysiological mechanisms of the syndrome.
Objective To describe the somatosensory phenotype of a previously unreported patient with progressive facial hemiatrophy and facial pain.
Design Case report and 4-month follow-up period.
Setting University-based tertiary referral headache center.
Patient A 37-year-old woman with progressive facial hemiatrophy and strictly left-sided facial pain over 12 years.
Intervention Greater occipital nerve blockade with lidocaine, 2% (2 mL), and methylprednisolone sodium phosphate (80 mg).
Main Outcome Measures Trigeminal sensory phenotype on quantitative sensory testing using thermal threshold and Von Frey hairs. The case report includes patient photographs, neuroimaging, and neurophysiological findings.
Results On the left side, there was continuous pain in V1 and V2 and intermittent sharp shooting pains in V3. The sensory examination showed areas on the left side with pinprick hyperalgesia, cold and heat hyperalgesia, and dynamic mechanical allodynia. The pain in V1 and V3 and the allodynia dramatically improved after greater occipital nerve blockade. In the cases reported in the literature, a constant component of the pain was always part of the phenotype, and positive or negative trigeminal sensory signs were frequently described.
Conclusions The phenotype of our patient suggests neuropathic pain involving all 3 branches of the trigeminal nerve, and the patient fulfills newly defined stricter criteria for neuropathic pain. Similar to our case, phenotypes of the other published cases seem to agree with trigeminal neuropathic pain rather than trigeminal neuralgia specifically.