Trigeminal Neuropathic Pain in a Patient With Progressive Facial Hemiatrophy (Parry-Romberg Syndrome)

TY - JOUR

T1 - Trigeminal Neuropathic Pain in a Patient With Progressive Facial Hemiatrophy (Parry-Romberg Syndrome)

AU - Viana, Michele

AU - Glastonbury, Christine M.

AU - Sprenger, Till

AU - Goadsby, Peter

PY - 2011/7

Y1 - 2011/7

N2 - 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 V(1) and V(2) and intermittent sharp shooting pains in V(3). The sensory examination showed areas on the left side with pinprick hyperalgesia, cold and heat hyperalgesia, and dynamic mechanical allodynia. The pain in V(1) and V(3) 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.

AB - 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 V(1) and V(2) and intermittent sharp shooting pains in V(3). The sensory examination showed areas on the left side with pinprick hyperalgesia, cold and heat hyperalgesia, and dynamic mechanical allodynia. The pain in V(1) and V(3) 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.

KW - SCLERODERMA EN-COUP

KW - HEMIFACIAL ATROPHY

KW - SABRE

KW - INVOLVEMENT

KW - MIGRAINE

KW - SYSTEM

U2 - 10.1001/archneurol.2011.126

DO - 10.1001/archneurol.2011.126

M3 - Article

SN - 0003-9942

VL - 68

SP - 938

EP - 943

JO - Archives of neurology

JF - Archives of neurology

IS - 7

ER -