Síndrome de Parry-Romberg: achados nas sequências avançadas de ressonância magnética - relato de caso

Abstract

Parry-Romberg syndrome is a rare disease characterized by progressive hemifacial atrophy associated with other systemic changes, including neurological symptoms. Currently, there are few studies exploring the utilization of advanced magnetic resonance sequences in the investigation of this disease. The authors report the case of a 45-year-old patient and describe the findings at structural magnetic resonance imaging and at advanced sequences, correlating them with pathophysiological data.

Parry-Romberg syndrome

“… 1 - 5 , 9 The extension of the atrophy is frequently limited to one side of the face, and ipsilateral facial involvement is rare (10-20% of cases were reported bilaterally). 2 - 5 , 8 , 9 The early hemifacial asymmetry progresses over years to extreme loss of subcutaneous tissues, resulting in a sunken and wrinkled face on one side. 5 , 8 , 9 The incidence of PRS is not well-established, the average onset of disease is around 10 years of age; however, onset can be found as late as 40-50 years in some patients; 8 - 10 our patient was a 48-year-old female.…”

“… 2 - 5 , 8 , 9 The early hemifacial asymmetry progresses over years to extreme loss of subcutaneous tissues, resulting in a sunken and wrinkled face on one side. 5 , 8 , 9 The incidence of PRS is not well-established, the average onset of disease is around 10 years of age; however, onset can be found as late as 40-50 years in some patients; 8 - 10 our patient was a 48-year-old female. The severity of PRS with late onset, including facial atrophy-asymmetry, neurological and ocular findings and so forth, may be less than PRS in younger ages, probably due to the complete development of craniofacial and nervous structures.…”

“…The severity of PRS with late onset, including facial atrophy-asymmetry, neurological and ocular findings and so forth, may be less than PRS in younger ages, probably due to the complete development of craniofacial and nervous structures. 5 , 8 - 10 To date around 150 cases of PRS had been reported in the literature. 1 - 10 This syndrome is more prevalent in females without any precise geographical distribution.…”

“… 1 - 4 , 6 The onset of this syndrome generally occurs in the first and second decades of life with skin changes resembling scleroderma, which is usually accompanied by this neurological effects of PRS, including seizures, migraine, trigeminal neuralgia and darkening of skin; partial seizures are the most common neurologic complication. 2 - 4 , 8 Ocular involvement is common, and the most frequent manifestation is enophthalmos. 2 - 5 , 9 Its origin is unclear without any known cure.…”

Chronic kernicterus: magnetic resonance imaging findings

“…Recent studies conducted in Brazil have highlighted the importance of MRI studies to improving the diagnosis of central nervous system disorders (1-5) . Kernicterus, also known as bilirubin encephalopathy, is a rare complication of hyperbilirubinemia in childhood, occurring when serum bilirubin levels in the neonate are in excess of 20 mg/dL at term or even lower values in premature infants, which result in bilirubin deposition in the globus pallidus, subthalamic nuclei, hippocampus, putamen, thalamus, and cranial nerves, primarily the third, fourth, and sixth cranial nerves (6) .…”